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Background: Spectral localizer images from photon-counting detector (PCD) CT can be used for bone mineral density (BMD) evaluation given their 2D-projectional nature and material decomposition capability. As all CT examinations include localizer images, this approach could allow opportunistic osteoporosis screening in patients undergoing clinically indicated imaging by PCD CT. Objective: To assess the utility of PCD-CT spectral localizer images for opportunistic derivation of area BMD (aBMD) values and T-scores, using dual-energy X-ray absorptiometry (DXA) as the reference standard. Methods: This prospective study included patients ≥18 years old scheduled for clinically indicated lumbar spine CT between October 2023 and February 2024 and who underwent DXA within the 13 prior months or were scheduled for DXA within the subsequent 13 months. Participants underwent lumbar spine CT by PCD CT including spectral localizer images. Lumbar spine aBMD was extracted from clinical DXA reports. ROIs were placed on lumbar vertebral bodies and background soft tissues to extract areal densities from spectral localizer images using material decomposition; areal densities were used to derive lumbar spine aBMD values. The aBMD values were used to derive T-scores, which were classified as representing normal (≥-1) or abnormal (<-1) bone mass. DXA-derived and PCD-CT derived measurements were compared. Results: The study included 51 participants (mean age: 62 years [range, 28-83 years]; 31 female, 20 male). Mean DXA-derived T-score was 0.39±1.64; mean PCD-CT derived T-score was 0.28±1.77 (p=.29). Lin's concordance correlation coefficient between DXA-derived and PCD-CT T-scores was 0.90. The difference between DXA-derived and PCD-CT derived T-scores showed a small correlation with patient age (r=-0.13), absolute interval between DXA and PCD CT (r=.15), and BMI (r=0.28); this difference in scores did not show a significant difference between male and female patients (0.08 vs 0.13, respectively; p=.81). PCD-CT T-scores had sensitivity of 97%, specificity of 71%, PPV of 90%, and NPV of 91% for detecting abnormal bone mass using DXA-derived T-scores as the reference standard. Conclusion: PCD-CT spectral localizers showed clinical utility for deriving aBMD values and, consequently, T-scores. Clinical Impact: The T-score derived from PCD-CT spectral localizers may serve as an opportunistic screening tool for low bone mass and osteoporosis.

Objective: Retrospectively evaluate multimodality imaging features of perinephric myxoid pseudotumor of fat (PMPTF)., Methods: Institutional cases of PMPTF with CT, MRI and/or ultrasound evaluation from 1/1/2020 to 9/1/2023 were retrospectively reviewed. Patient demographics and clinical history were reviewed, and imaging features recorded., Results: 14 patients with pathologically-proven PMPTF were identified (11 M, 3 F; mean age 66.7 ± 17.0 years; range 40-87 years). Three patients (18%) had bilateral lesions; a total of 17 PMPTFs were reviewed. 15/17 (88%) were biopsy-proven; two cases were diagnosed by imaging only in patients with a contralateral biopsy-proven PMPTF. All evaluable specimens were negative for MDM2 amplification. 11/17 (65%) occurred in patients with renal disease, including 4/17 (24%) in patients with renal transplant. 100% (17/17) had CT, 11/17 (65%) MRI, and 6/17 (35%) ultrasound. The mean largest lesion dimension was 10.9 ± 4.6 cm (range 4.3-17.0 cm). Of cases involving native kidneys, 7/13 (54%) presented as multifocal perinephric masses and 5/13 (38%) as a solitary perinephric mass. All four transplant cases presented as infiltrative-appearing masses involving the renal sinus with lesser perinephric involvement. 14/17 (82%) lesions contained macroscopic fat on CT and MRI and 3/17 (18%) showed no macroscopic fat, all involving renal transplants. All cases with MRI demonstrated T2 hyperintensity with signal dropout on opposed-phase imaging. 11/13 (85%) PMPTF showed no or equivocal CT enhancement. Enhancement was better seen on MRI in all cases evaluated by both CT and MRI. Of the six PMPTFs imaged by ultrasound, four (67%) were heterogeneously hypoechoic and two (33%) had mixed regions of hypo-, iso- and hyperechogenicity relative to adjacent renal parenchyma., Conclusions: PMPTF is a rare, benign, and underrecognized lesion that may mimic malignancy, particularly retroperitoneal well-differentiated liposarcoma. The imaging features of this unusual pseudosarcoma differ in native and transplanted kidneys. Improved awareness of this entity will facilitate appropriate patient management and avoid unnecessary intervention., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: We have recently described circumferential nerve involvement of neuromuscular choristoma associated with desmoid-type fibromatosis (NMC-DTF) in cases involving the sciatic nerve, supporting a nerve-derived mechanism for the DTF. We wondered whether a similar growth pattern occurs in cases involving the brachial plexus (BP)., Methods: We reviewed all available magnetic resonance (MR) imaging in patients diagnosed at our institution with NMC or NMC-DTF of the BP. We also performed a literature search of patients with NMC or NMC-DTF of the BP., Results: In our clinical records, four patients with NMC of the BP were identified, and three developed NMC-DTF. All three patients had MR imaging evidence of circumferential encasement of the BP. In the literature, we identified 15 cases of NMC of the BP, of which 12 had identified NMC-DTF. Four published cases included MR images, and only two were of sufficient quality for review. The single provided image in both cases demonstrated a similar pattern of circumferential encasement of the BP by the NMC-DTF. One additional case report was published without MR images but described circumferential involvement in the surgical findings. One unpublished case of NMC-DTF of the BP from an international radiology meeting also had this circumferential pattern pattern on MRI., Conclusions: The MRI findings of circumferential nerve involvement in patients with NMC-DTF of the BP are similar to our previously reported data in patients with NMC-DTF of the sciatic nerve, providing further imaging-based support of a nerve-driven mechanism. Clinical implications are presented based on the proposed pathogenetic mechanism., (Copyright © 2024 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Normocalcemic hyperparathyroidism (NHPT) is variably defined, and information regarding complications and natural history are scarce. We aimed to describe the phenotype of NHPT in relation to hypercalcemic hyperparathyroidism (PHPT) and controls, to determine risk of progression, and to develop a predictive model for progression to PHPT. This is a retrospective chart review of 232 patients at a tertiary medical center, comparing 75 controls, 73 patients with NHPT, and 84 with PHPT. NHPT was intermediate in biochemical profile between controls and PHPT with respect to cCa, iPTH, intraindividual coefficient of variant of cCa, phosphorus, and 25(OH)D. NHPT patients had an increased adjusted risk of urolithiasis (OR 5.34, 95%CI, 2.41-12.71, P  < .001) and fragility fractures (OR 4.53, 95%CI, 1.63-14.84, P  = .006) versus controls, after adjustment for age, sex, and BMI. Fewer NHPT compared with PHPTH patients achieved cure with parathyroidectomy ( P  = .001). NHPT more often had nonlocalizing imaging or polyglandular disease ( P  = .005). Parathyroidectomy improved biochemical but not BMD parameters in NHPT. Over a median follow-up of 4.23 (IQR 1.76-5.31) years, NHPT patients managed expectantly experienced no change in iPTH, and progression to PHPT occurred in 9%. An XGBoost model combining 6 factors for progression (mean index 2 iPTH, mean index 2 cCa, 24-h urinary calcium, age, 25(OH)D, and presence of urolithiasis) had an area under the curve 1.00 (95%CI, 1.00-1.00, P  < .001) for predicting combined progression. NHPT is a mild variant of PHPT at intermediate risk of urolithiasis and fragility fractures. Cure was less often achieved with parathyroidectomy, which did not improve BMD parameters. Progression was infrequent with conservative management. Because only a minority progressed to PHPT, in addition to lower surgical success rates, we suggest conservative management for the majority of NHPT unless risk factors for progression are identified., Competing Interests: The authors declare no conflicts of interest., (© The Author(s) 2024. Published by Oxford University Press on behalf of the American Society for Bone and Mineral Research.)

Background: Peripheral neurolymphomatosis (NL) is an often-misdiagnosed condition characterized by lymphomatous infiltration within the peripheral nerves. Its rarity and complexity frequently result in delayed diagnosis and suboptimal patient outcomes. This study aims to elucidate the role of the paraneurium (circumneurium) in NL, emphasizing its diagnostic and therapeutic significance., Observations: A 72-year-old man presented with lesions on his right lower eyelid. Initial diagnostics were inconclusive until an excisional biopsy confirmed extranodal marginal zone lymphoma. Following a complete metabolic response to rituximab treatment, the patient relapsed 14 months later with progressive lymphoma and bilateral sciatic nerve involvement, as confirmed by positron emission tomography-computed tomography and magnetic resonance imaging., Lessons: This paper underscores the critical role of the paraneurium in NL, enhancing understanding of its pathophysiology. Integrating advanced imaging techniques have proved essential in accurately identifying neurolymphomatous involvement within the paraneurium. This study paves the way for more effective management strategies in NL and similar conditions, focusing on improving patient care and outcomes.

The past 25 years have seen significant growth in the role of positron emission tomography/computed tomography (PET/CT) in musculoskeletal oncology. Substantiative advances in technical capability and image quality have been paralleled by increasingly widespread clinical adoption and implementation. It is now recognized that PET/CT is useful in diagnosis, staging, prognostication, response assessment, and surveillance of bone and soft tissue sarcomas, often providing critical information in addition to conventional imaging assessment. As individualized, precision medicine continues to evolve for patients with sarcoma, PET/CT is uniquely positioned to offer additional insight into the biology and management of these tumors., Competing Interests: Disclosure The authors have no relevant financial disclosures or conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Spine pain is highly prevalent and costly, but evaluation with clinical features and anatomic imaging remain limited. Fat-suppressed MR imaging and molecular imaging (MI) may help identify inflammatory, lesional, and malignant causes. Numerous MI agents are available, each with advantages and disadvantages. Herein, FDG PET, prostate-specific membrane antigen (PSMA), bone radiotracers, and others are highlighted. No specific pain MI agents have been identified, but mechanisms of key agents are shown in video format, and the mechanism of PSMA as a theranostic agent is displayed. A multidisciplinary approach is needed to master this topic., Competing Interests: Disclosure None to declare for the authors., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Measures of muscle and adipose tissue mass have been associated with outcomes in several malignancies, but studies in multiple myeloma (MM) are inconsistent. The aim of this study was to evaluate the association between muscle and fat areas and radiodensity, and overall survival (OS) in patients with newly diagnosed MM. We included 341 patients diagnosed with MM from 2010-2019 who had an 18 F-fluorodeoxyglucose positron emission tomography/computed tomography at diagnosis. A cross-sectional image at the third lumbar vertebrae was segmented into muscle and fat components. Median follow up was 5.7 years. There was no association between sarcopenia and baseline disease characteristics or OS. Low muscle radiodensity was associated with higher disease stage, anemia, and renal failure. OS was 5.6 vs. 9.0 years in patients with muscle radiodensity in the lower vs. middle/upper tertiles, respectively (P = 0.02). High subcutaneous adipose tissue (SAT) radiodensity was associated with higher stage, anemia, thrombocytopenia, hypercalcemia, renal failure, and high LDH. OS was 5.4 years vs. not reached in patients with SAT radiodensity in the upper vs. middle/lower tertiles, respectively (P = 0.001). In conclusion, sarcopenia was not associated with OS in MM patients. High SAT radiodensity and low muscle radiodensity were associated with advanced disease stage and adverse laboratory characteristics., (© 2023. The Author(s).)

PET/MRI is a relatively new imaging modality with several advantages over PET/CT that promise to improve imaging of the abdomen and pelvis for specific diagnostic tasks by combining the superior soft tissue characterization of MRI with the functional information acquired from PET. PET/MRI has an established role in staging and response assessment of multiple abdominopelvic malignancies, but the modality is not yet established for non-oncologic conditions of the abdomen and pelvis. In this review, potential applications of PET/MRI for non-oncologic conditions of abdomen and pelvis are outlined, and the available literature is reviewed to highlight promising areas for further research and translation into clinical practice., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Abstract: Familial expansile osteolysis is an exceedingly rare autosomal dominant bone dysplasia, which can have overlapping features with Paget disease and expansile skeletal hyperphosphatasia. We present a novel case of familial expansile osteolysis evaluated on 99mTc-MDP bone scan with correlative radiographs and CT., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Abstract: Leucine-rich glioma inactivated 1 autoimmune encephalitis is a treatable cause of autoimmune epilepsy associated with faciobrachial dystonic seizures-a rare form of epilepsy with frequent brief seizures primarily affecting the arm and face. We report a case with characteristic imaging findings. 18 F-FDG PET/CT demonstrated severe hypometabolism in the left basal ganglia, a regional abnormality associated with leucine-rich glioma inactivated 1 encephalitis., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

The evolving classification of rhabdomyosarcoma (RMS) now includes spindle cell RMS (SRMS). Bone/soft tissue SRMS often harbor TFCP2, or less often MEIS1 rearrangements. We studied 25 fusion-driven SRMS involving bone (n = 19) and soft tissue (n = 6). Osseous SRMS occurred in 13 women and 6 men (median age: 41 years) and involved the pelvis (5), sacrum (2), spine (4), maxilla (4), mandible (1), skull (1), and femur (2). Follow-up (median: 5 months) demonstrated local recurrence in 2/16 and distant metastases in 8/17 patients (median time to metastasis: 1 month). Eight patients died of disease; 9 were alive with disease. Soft tissue SRMS occurred in 4 men and 2 women (median: 50 years). Follow-up (median: 10 months) revealed distant metastasis at diagnosis (1), alive with unresected tumor (1), and no evidence of disease (4). Next-generation sequencing demonstrated FUS::TFCP2 (12), EWSR1::TFCP2 (3) and MEIS1::NCOA2 (2); FISH identified EWSR1 (2) rearrangements. Most TFCP2-rearranged SRMS (13/17) showed spindled/epithelioid morphology, rarely with rhabdomyoblasts. The bone tumors were diffusely desmin and MyoD1 positive with limited myogenin; 10/13 were ALK -positive and 6/15 were keratin positive. Soft tissue SRMS harbored EWSR1::TFCP2, MEIS1::NCOA2, ZFP64::NCOA2, MEIS1::FOXO1, TCF12::VGLL3 and DCTN1::ALK, and displayed spindled/epithelioid, leiomyomatous, and myxofibrosarcoma-like morphologies. Immunohistochemistry (IHC) was positive for MyoD1 (6/6), focal desmin (5/6), myogenin (3/6), and keratin (1/6). We conclude that TFCP2-rearranged SRMS of bone and soft tissue show consistent morphologic and IHC features, likely representing a distinct subset of RMS. Non-TFCP2 fusion-positive SRMS could represent a single RMS subset, multiple subtypes of RMS, or "fusion-defined" sarcomas with rhabdomyoblastic differentiation., (Copyright © 2023 United States & Canadian Academy of Pathology. Published by Elsevier Inc. All rights reserved.)

Objective: Describe imaging features of intraosseous hemangiomas located outside of the mobile spine and calvarium., Materials and Methods: Imaging and medical records were retrospectively reviewed for cases of intraosseous hemangiomas located outside of the calvarium and mobile spine. Evaluation included patient demographics, histologic confirmation, and imaging characteristics., Results: Thirty-six patients were included (25 F, 11 M; mean age 54 ± 17 years, range 10-84 years) with 37 total lesions (70% axial and 30% appendicular skeleton). Mixed lytic and sclerotic features were identified on 83-85% radiographs and CTs. Amorphous increased density mimicking osteoid matrix was present on 38-45% radiographs and CTs. Classic honeycomb or radial pattern was identified on 45% of CTs. Osseous expansion and cortical permeation were common features. CT identified periosteal reaction in 24% of lesions. All hemangiomas had heterogeneous MRI signal and most moderately or avidly enhanced. Intralesional fat was identified on 78% MRIs, often as a minor component and only detected on 24% of CTs. A soft tissue mass was present on 52% of MRIs. FDG PET/CT mean SUVmax of 3.2 ± 0.6 (range 1.9-5.0). Lesional FDG activity relative to background marrow was increased in 75% of lesions. Lesions with cortical permeation had higher metabolic activity versus those without (3.5 ± 0.7 versus 2.2 ± 0.3, p = 0.041)., Conclusion: Intraosseous hemangiomas outside of the mobile spine and calvarium demonstrate more aggressive imaging features compared to vertebral hemangiomas, including cortical permeation, soft tissue mass, amorphous increased density mimicking osteoid matrix, and increased FDG activity., (© 2023. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Objective: To examine the multimodality imaging characteristics of parosteal lipomas., Materials and Methods: With IRB approval, our institutional imaging database and medical record were retrospectively reviewed from 1990-2020 for cases of pathologically-proven and/or imaging diagnosed parosteal lipomas., Results: There were 22 patients (12 males, 10 females) with a mean age of 57.1 ± 12.7 years (range 31-80 years). 11/22 cases (50%) were pathologically-confirmed on biopsy or surgical resection and 11/22 (50%) had imaging features compatible with parosteal lipoma. Lesions occurred most commonly along the femur (8/22, 36%), followed by the forearm (3/22, 14%). All cases demonstrated a juxtacortical fatty mass containing an osseous excrescence that was firmly attached to the cortical surface. The osseous excrescences were characterized as pedunculated in 16/22 (73%) and sessile in 6/22 (27%). The average largest dimension of the osseus excrescences was 2.4 ± 1.6 cm (range 0.8-6.1 cm) and the lipomatous portions 7.8 ± 3.8 cm (range 2.0-19.5 cm). The excrescences contained mature bone in 12/22 (55%) cases and a mixture of mature bone and radiating bone spicules in 10/22 (45%). There were non-lipomatous elements in the fatty portion of the mass in 13/22 (59%) of cases. Most cases (19/22, 85%) had cortical thickening/periostitis near the base of the osseous stalk. Two patients had a bone scan that demonstrated uptake in the osseous excrescence, and two patients had an FDG PET/CT that demonstrated no uptake., Conclusion: Parosteal lipomas are a rare benign lipomatous tumor with pathognomonic multimodality imaging features that may obviate the need for biopsy., (© 2023. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Objective: Neuromuscular choristoma (NMC) is a rare developmental malformation of peripheral nerve that is frequently associated with the development of a desmoid-type fibromatosis (DTF). Both NMC and NMC-DTF typically contain pathogenic CTNNB1 mutations and NMC-DTF develop only within the NMC-affected nerve territory. The authors aimed to determine if there is a nerve-driven mechanism involved in the formation of NMC-DTF from the underlying NMC-affected nerve., Methods: Retrospective review was performed for patients evaluated in the authors' institution with a diagnosis of NMC-DTF in the sciatic nerve (or lumbosacral plexus). MRI and FDG PET/CT studies were reviewed to determine the specific relationship and configuration of NMC and DTF lesions along the sciatic nerve., Results: Ten patients were identified with sciatic nerve NMC and NMC-DTF involving the lumbosacral plexus, sciatic nerve, or sciatic nerve branches. All primary NMC-DTF lesions were located in the sciatic nerve territory. Eight cases of NMC-DTF demonstrated circumferential encasement of the sciatic nerve, and one abutted the sciatic nerve. One patient had a primary DTF remote from the sciatic nerve, but subsequently developed multifocal DTF within the NMC nerve territory, including 2 satellite DTFs that circumferentially encased the parent nerve. Five patients had a total of 8 satellite DTFs, 4 of which abutted the parent nerve and 3 that circumferentially involved the parent nerve., Conclusions: Based on clinical and radiological data, a novel mechanism of NMC-DTF development from soft tissues innervated by NMC-affected nerve segments is proposed, reflecting their shared molecular genetic alteration. The authors believe the DTF develops outward from the NMC in a radial fashion or it arises in the NMC and wraps around it as it grows. In either scenario, NMC-DTF develops directly from the nerve, likely arising from (myo)fibroblasts within the stromal microenvironment of the NMC and grows outward into the surrounding soft tissues. Clinical implications for patient diagnosis and treatment are presented based on the proposed pathogenetic mechanism.

"Inflammatory rhabdomyoblastic tumor" (IRMT) is a recently coined name for a distinctive soft tissue neoplasm characterized by slow growth, a dense histiocytic infiltrate, scattered, bizarre-appearing tumor cells with morphologic and immunohistochemical evidence of skeletal muscle differentiation, a near-haploid karyotype with retained biparental disomy of chromosomes 5 and 22, and usually indolent behavior. There are 2 reports of rhabdomyosarcoma (RMS) arising in IRMT. We studied the clinicopathologic and cytogenomic features of 6 cases of IRMT with progression to RMS. Tumors occurred in the extremities of 5 men and 1 woman (median patient age, 50 years; median tumor size, 6.5 cm). Clinical follow-up (6 patients: median, 11 months; range 4-163 months) documented local recurrence and distant metastases in 1 and 5 of 6 patients, respectively. Therapy included complete surgical resection (4 patients) and adjuvant/neoadjuvant chemo/radiotherapy (6 patients). One patient died of disease, 4 were alive with metastatic disease, and one was without evidence of disease. All primary tumors contained conventional IRMT. Progression to RMS appeared as follows: (1) overgrowth of monomorphic rhabdomyoblasts with diminished histiocytes, (2) monomorphic spindle cell morphology with variably pleomorphic rhabdomyoblasts and low mitotic activity, or (3) morphologically undifferentiated spindle cell and epithelioid sarcoma. All but one were diffusely desmin-positive, with more limited MyoD1/myogenin expression. All RMS arising in IRMT, either primary or metastatic, demonstrated widespread loss of heterozygosity with retained heterozygosity of chromosomes 5 and 20, and all but one displayed additional gains and losses involving loci containing oncogenes/ tumor suppressor genes, most often CDKN2A and CDKN2B. RMS arising in IRMT have unique clinicopathologic and cytogenomic features, warranting classification as a distinct, potentially aggressive RMS subtype. It should be distinguished from other RMSs, particularly fusion-driven spindle cell RMS and pleomorphic RMS., (Copyright © 2023 United States & Canadian Academy of Pathology. Published by Elsevier Inc. All rights reserved.)

Background: Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion characterized by abnormal presence of muscle within nerve. Associated desmoid-type fibromatosis (NMC-DTF) often develops. We report 18 F-fluorodeoxyglucose positron emission tomography (FDG PET) characteristics of NMC and NMC-DTF and propose that increased FDG activity within NMCs may be associated with subclinical NMC-DTF or NMC-DTF "precursor" tissue., Methods: Our institutional database was searched for all NMC cases. Inclusion criteria were 1) confirmed diagnosis of NMC with or without biopsy, and 2) available PET and MRI studies. PET data included SUVmax and SUVmean of NMCs, contralateral limb normal skeletal muscle and unaffected nerves, and SUVmax of NMC-DTF if present. SUV values were compared using paired t-test. A p value of < 0.05 was considered statistically significant., Results: Our cohort consisted of 9 patients with NMC, 8 cases involving sciatic nerve and 1 of brachial plexus. On PET imaging, all NMC-affected nerve segments showed significantly higher FDG uptake (SUVmax/mean) compared to both contralateral normal nerve and normal skeletal muscle (all P < 0.05). Similar to sporadic DTF, NMC-DTF was highly FDG-avid (average SUVmax of 4.2). SUVmax in NMC with or without concurrent NMC-DTF did not differ (p = 0.76). Within NMC-affected nerve segment, FDG activity was relatively higher in areas with low T1/T2 MR signal., Conclusion: All NMCs were more FDG avid compared to both normal skeletal muscle and contralateral unaffected nerve, arguing against the presence of heterotopic muscle in NMC as the source of FDG avidity. FDG avidity within NMC may reflect subclinical NMC-DTF or a precursor lesion, as NMC-DTF are highly FDG-avid, and the highest regions of FDG avidity in NMC occurred in regions with MR characteristics associated with NMC-DTF (i.e., lower T1/T2 signal). We believe that the integration of FDG PET with serial MR imaging in patient follow up will clarify its utility in both detection and surveillance of NMC-DTF., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Abstract: PET/CT plays a crucial role in the management of prostate cancer with several emerging and established radiopharmaceuticals, including 18 F-piflufolastat and 11 C-choline. These radiotracers are thought to be relatively specific to prostate cancer; however, uptake has also been demonstrated in other benign and malignant lesions. Nodular fasciitis is a rapidly growing benign soft tissue neoplasm that is typically self-limiting. Although a few case reports describe 68 Ga-PSMA uptake in nodular fasciitis, uptake of 11 C-choline and other PSMA-targeted PET probes, including 18 F-piflufolastat, have not previously been reported. We present a novel case of nodular fasciitis demonstrating both 18 F-piflufolastat and 11 C-choline avidity., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Since the discovery of USP6 gene rearrangements in aneurysmal bone cysts nearly 20 years ago, we have come to recognize that there is a family of USP6-driven mesenchymal neoplasms with overlapping clinical, morphologic, and imaging features. This family of neoplasms now includes myositis ossificans, aneurysmal bone cyst, nodular fasciitis, fibroma of tendon sheath, fibro-osseous pseudotumor of digits, and their associated variants. While generally benign and in many cases self-limiting, these lesions may undergo rapid growth, and be confused with malignant bone and soft tissue lesions, both clinically and on imaging. The purpose of this article is to review the imaging characteristics of the spectrum of USP6-driven neoplasms, highlight key features that allow distinction from malignant bone or soft tissue lesions, and discuss the role of imaging and molecular analysis in diagnosis., (© 2022. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Background: Neuromuscular choristoma (NMC) is a rare congenital lesion in which muscle tissue is admixed with nerve fascicles within a peripheral nerve. Patients commonly present in early childhood with neuropathy, plexopathy, or chronic undergrowth in the distribution of the affected nerve., Observations: The authors present the case of a 35-year-old man with unrecognized neuromuscular NMC of the sciatic nerve, which resulted in recurrent, multicentric NMC-associated desmoid-type fibromatosis (NMC-DTF) within the nerve territory in association with a Marjolin ulcer, a cutaneous malignancy., Lessons: Based on anatomical and pathophysiological findings described in this case report, the authors support the association between NMC-DTF and Marjolin ulcer.

ADPKD is caused by pathogenic variants in PKD1 or PKD2 , encoding polycystin-1 and -2 proteins. Polycystins are expressed in osteoblasts and chondrocytes in animal models, and loss of function is associated with low bone mineral density (BMD) and volume. However, it is unclear whether these variants impact bone strength in ADPKD patients. Here, we examined BMD in ADPKD after kidney transplantation (KTx). This retrospective observational study retrieved data from adult patients who received a KTx over the past 15 years. Patients with available dual-energy X-ray absorptiometry (DXA) of the hip and/or lumbar spine (LS) post-transplant were included. ADPKD patients (n = 340) were matched 1:1 by age (±2 years) at KTx and sex with non-diabetic non-ADPKD patients (n = 340). Patients with ADPKD had slightly higher BMD and T-scores at the right total hip (TH) as compared to non-ADPKD patients [BMD: 0.951 vs. 0.897, p < 0.001; T-score: -0.62 vs. -0.99, p < 0.001] and at left TH [BMD: 0.960 vs. 0.893, p < 0.001; T-score: -0.60 vs. -1.08, p < 0.001], respectively. Similar results were found at the right femoral neck (FN) between ADPKD and non-ADPKD [BMD: 0.887 vs. 0.848, p = 0.001; T-score: -1.20 vs. -1.41, p = 0.01] and at left FN [BMD: 0.885 vs. 0.840, p < 0.001; T-score: -1.16 vs. -1.46, p = 0.001]. At the LS level, ADPKD had a similar BMD and lower T-score compared to non-ADPKD [BMD: 1.120 vs. 1.126, p = 0.93; T-score: -0.66 vs. -0.23, p = 0.008]. After adjusting for preemptive KTx, ADPKD patients continued to have higher BMD T-scores in TH and FN. Our findings indicate that BMD by DXA is higher in patients with ADPKD compared to non-ADPKD patients after transplantation in sites where cortical but not trabecular bone is predominant. The clinical benefit of the preserved cortical bone BMD in patients with ADPKD needs to be explored in future studies., Competing Interests: PCH reports receiving grants and/or research reagents from Amgen, Inc., Bayer AG, Genzyme Corporation, GlaxoSmithKline, Mitobridge Inc., Otsuka Pharmaceuticals, Palladio Biosciences, Regulus Therapeutics, and Vertex Pharmaceuticals, all outside of the submitted work. PCH also reports a position on the Clinical Advisory Board of Mironid, honoraria from Otsuka Pharmaceuticals and Vertex Pharmaceuticals, and other fees from Otsuka Pharmaceuticals. VET reports grants and/or other fees from Blueprint Medicines, Mironid, Otsuka Pharmaceuticals, Palladio Biosciences, Sanofi Genzyme, and Regulus Therapeutics, all outside the submitted work. ZMZ reports grants from Kadmon Inc. and consulting fees from Chronisense Medical, LTD. FTC reports research grant support from Otsuka Pharmaceuticals. All the other authors declared no competing interests., (© 2023 The Authors.)

Objective: Patients with malignant peripheral nerve sheath tumors (MPNSTs) of major motor nerves typically present with muscle weakness and pain. We aimed to analyze and characterize patients with MPNSTs of major motor nerves but without muscle weakness at initial presentation., Methods: We performed a retrospective search of MPNSTs in a major nerve evaluated and/or treated at our institution from 1994 to 2019. Patients with no muscle weakness and available magnetic resonance imaging were analyzed. Clinical materials and magnetic resonance imaging and positron emission tomography scans were reviewed for features of malignancy. This group of patients was compared with patients who presented with MPNSTs and muscle weakness., Results: Of 26 patients with MPNSTs who presented with no muscle weakness, 21 (81%) had a positive family history for malignancy. Only 16 (62%) magnetic resonance imaging scans were highly suspicious for malignancy. All 7 available positron emission tomography scans were highly suspicious for malignancy. Patients who presented with muscle weakness (n = 36) were more likely to have paresthesias and a history of neurofibromatosis 1 or radiation to the MPNST location (P < 0.05)., Conclusions: MPNSTs of major motor nerves without muscle weakness represent an underappreciated subset of cases that have potential treatment and outcome implications. These patients presented with fewer symptoms and had fewer risk factors than patients with muscle weakness. Positron emission tomography should be considered as an additional method to try to anticipate the diagnosis of an MPNST., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Abstract: Ewing sarcoma is the second most common primary bone tumor in children. Typical Ewing sarcoma most frequently occurs in long bones and within the pelvis. ALES (adamantinoma-like Ewing sarcoma) is a rare subtype of Ewing sarcoma that is characterized by epithelial differentiation in addition to small round blue cells. Unlike typical Ewing sarcoma, ALES has been described in several cases in the head and neck. Herein, we describe a case of a 9-year-old boy with ALES of the mandible evaluated on 18F-FDG PET/CT with correlative MRI scans., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Clinical success with intravenous (IV) oncolytic virotherapy (OV) has to-date been anecdotal. We conducted a phase 1 clinical trial of systemic OV and investigated the mechanisms of action in responding patients. A single IV dose of vesicular stomatitis virus (VSV) interferon-β (IFN-β) with sodium iodide symporter (NIS) was administered to patients with relapsed/refractory hematologic malignancies to determine safety and efficacy across 4 dose levels (DLs). Correlative studies were undertaken to evaluate viremia, virus shedding, virus replication, and immune responses. Fifteen patients received VSV-IFNβ-NIS. Three patients were treated at DL1 through DL3 (0.05, 0.17, and 0.5 × 1011 TCID50), and 6 were treated at DL4 (1.7 × 1011 TCID50) with no dose-limiting toxicities. Three of 7 patients with T-cell lymphoma (TCL) had responses: a 3-month partial response (PR) at DL2, a 6-month PR, and a complete response (CR) ongoing at 20 months at DL4. Viremia peaked at the end of infusion, g was detected. Plasma IFN-β, a biomarker of VSV-IFNβ-NIS replication, peaked between 4 hours and 48 hours after infusion. The patient with CR had robust viral replication with increased plasma cell-free DNA, high peak IFN-β of 18 213 pg/mL, a strong anti-VSV neutralizing antibody response, and increased numbers of tumor reactive T-cells. VSV-IFNβ-NIS as a single agent was effective in patients with TCL, resulting in durable disease remissions in heavily pretreated patients. Correlative analyses suggest that responses may be due to a combination of direct oncolytic tumor destruction and immune-mediated tumor control. This trial is registered at www.clinicaltrials.gov as #NCT03017820., (© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Multiple myeloma (MM) is a plasma cell malignancy that is characterized by diverse clinical presentations. Although biochemical assessment of disease activity is commonly used to monitor treatment response, findings on magnetic resonance imaging and positron emission tomography (PET)/computed tomography (CT), among other imaging modalities, have proven to harbor prognostic value. We sought to corroborate these findings by examining the prognostic significance of fluorodeoxyglucose PET/CT scanning in the setting of newly diagnosed MM. We retrospectively analyzed 195 patients with a PET/CT available at diagnosis and at 6 months posttreatment to examine their value as an adjuvant metric to conventional hematologic responses in terms of time to next treatment (TTNT) and overall survival (OS). The median TTNT and OS for the entire cohort were 24.6 months (95% confidence interval [CI], 20.4-29.1) and 79 months (95% CI, 63.1-119.1), respectively. When comparing PET/CT negative (-) with PET/CT positive (+) patients, we found significantly prolonged median TTNT (55.2 vs 17.8 months, P < .0001) and OS (unreached vs 60.8 months, P < .0001) in the former group. We then examined the additive value of PET/CT on the hematologic response achieved at 6 months and found that PET/CT (-) is associated with significantly increased median TTNT and OS for the very good partial response (VGPR) group and the less than VGPR group. Importantly, PET/CT retained prognostic significance after adjusting for multiple other predictive variables. We conclude that a PET/CT (-) at 6 months confers a significant prognostic advantage for patients with newly diagnosed MM and adds significant value to the hematologic response assessment., (© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Salivary gland tumors (SGTs) are a heterogeneous group of neoplasms arising from the 3 pairs of major salivary glands (parotid, submandibular, and sublingual) or numerous minor salivary glands located throughout the oral cavity. This review discusses the role of PET/computed tomography (CT) in evaluation of SGTs, including staging, restaging, prognostication, and response assessment. 18F-fluorodeoxyglucose (FDG) PET/CT is useful for staging and restaging malignant SGTs and offers important prognostic information in these patients. It is less useful for differentiating benign and malignant SGTs. Non-FDG PET radiotracers, perineural spread, parotid incidentalomas, and interpretative pitfalls are discussed as well., Competing Interests: Disclosure The authors have no relevant financial disclosures or conflicts of interest., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Mimics of primary and secondary bone tumors may result from a variety of processes. These can range from normal variants or developmental lesions that require no further work-up, to findings that require more urgent management, or may be indicative of a more serious systemic disease that necessitates further evaluation and treatment. It is important to be familiar with the spectrum of bone tumor mimics to avoid unnecessary tests, minimize patient morbidity, and reduce patient anxiety. This article discusses numerous nonneoplastic bone tumor mimickers, including their characteristic multimodality imaging features, differential diagnosis, and important aspects with which radiologists should be familiar., Competing Interests: Disclosure The authors have no relevant financial disclosures or conflicts of interest., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: Perineural spread of breast cancer to the brachial plexus can lead to pain, sensory alterations, and upper-extremity weakness. Although rare, perineural spread is an often-misdiagnosed long-term complication following breast cancer diagnosis. The objective of this study was to critically review the clinical, radiological, and pathological findings of biopsy-proven perineural spread of breast cancer to the brachial plexus., Methods: This is a retrospective study from a single institution in which a total of 19 patients with brachial plexus involvement from perineural spread of breast cancer who underwent fascicular biopsy between 1999 and 2021 were identified. Clinical, radiographic, and pathological data were retrospectively collected. Descriptive statistics were calculated for the cohort., Results: The mean age of patients at the time of diagnosis of breast cancer perineural spread was 60.6 ± 11.5 years. The diagnosis of brachial plexopathy due to perineural spread was on average 12 years after the primary diagnosis of breast cancer. There was also a delay in diagnosis due to the rarity of this disease, with a mean time from initial symptom onset to diagnosis of perineural spread of 25 ± 30 months. All patients at the time of presentation had upper-extremity weakness and pain. Nearly all patients demonstrated T2 signal change and nodular so-called sugar-coating contrast enhancement on brachial plexus MRI. Similarly, all patients who underwent PET/MRI or PET/CT had increased FDG uptake in the involved brachial plexus. Breast cancer perineural spread has an overall poor prognosis, with 16 of 19 patients dying within 5.9 ± 3.0 years after diagnosis of perineural spread., Conclusions: Perineural spread should be considered in patients with a history of breast cancer, even 10 years after primary diagnosis, especially in patients who present with arm pain, weakness, and/or sensory changes. Further diagnostic workup with electrodiagnostic studies; brachial plexus MRI, PET/CT, or PET/MRI; and possibly nerve biopsy is warranted to ensure accurate diagnosis.

BACKGROUND. COVID-19 vaccination may trigger reactive lymphadenopathy, confounding imaging interpretation. There has been limited systematic analysis of PET findings after COVID-19 vaccination. OBJECTIVE. The purpose of this study was to evaluate the frequency and characteristics of abnormal FDG and 11 C-choline uptake on PET performed after COVID-19 vaccination. METHODS. This retrospective study included 67 patients (43 men and 24 women; mean [± SD] age, 75.6 ± 9.2 years) who underwent PET examination between December 14, 2020, and March 10, 2021, after COVID-19 vaccination and who had undergone prevaccination PET examination without visible axillary node uptake. A total of 52 patients received the BNT162b2 mRNA COVID-19 vaccine (Pfizer-BioNTech; hereafter referred to as the Pfizer-BioNTech vaccine), and 15 received the SARS-CoV-2 mRNA-1273 vaccine (Moderna; hereafter referred to as the Moderna vaccine). Sixty-six of the patients underwent PET/CT, and one underwent PET/MRI. Fifty-four PET examinations used FDG, and 13 used 11 C-choline. PET was performed a median of 13 and 10 days after vaccination for patients who had received one ( n = 44) and two ( n = 23) vaccine doses, respectively. Two nuclear medicine physicians independently reviewed images and were blinded to injection laterality and the number of days since vaccination. Lymph node or deltoid SUV max greater than the blood pool SUV max was considered positive. Interreader agreement was assessed, and the measurements made by the more experienced physician were used for subsequent analysis. RESULTS. Positive axillary lymph node uptake was observed in 10.4% (7/67) of patients (7.4% [4/54] of FDG examinations and 23.1% [3/13] of 11 C-choline examinations); of the patients with positive axillary lymph nodes, four had received the Pfizer vaccine, and three had received the Moderna vaccine. Injection laterality was documented for five of seven patients with positive axillary lymph nodes and was ipsilateral to the positive node in all five patients. PET was performed within 24 days of vaccination for all patients with a positive node. One patient showed extraaxillary lymph node uptake (ipsilateral supraclavicular uptake on FDG PET). Ipsilateral deltoid uptake was present in 14.5% (8/55) of patients with documented injection laterality, including 42.9% (3/7) of patients with positive axillary lymph nodes. Interreader agreement for SUV measurements (expressed as intraclass correlation coefficients) ranged from 0.600 to 0.988. CONCLUSION. Increased axillary lymph node or ipsilateral deltoid uptake is occasionally observed on FDG or 11 C-choline PET performed after COVID-19 vaccination with the Pfizer-BioNTech or Moderna vaccine. CLINICAL IMPACT. Interpreting physicians should recognize characteristics of abnormal uptake on PET after COVID-19 vaccination to guide optimal follow-up management and reduce unnecessary biopsies.

Objective: Neurolymphomatosis (NL) is a rare manifestation of lymphoma confined to the peripheral nervous system that is poorly understood. It can be found in the cauda equina, but extraspinal disease can be underappreciated. The authors describe how extraspinal NL progresses to the cauda equina by perineural spread and the implications of this on timely and safe diagnostic options., Methods: The authors used the Mayo Clinic medical records database to find cases of cauda equina NL with sufficient imaging to characterize the lumbosacral plexus diagnosed from tissue biopsy. Demographics (sex, age), clinical data (initial symptoms, cerebrospinal fluid, evidence of CNS involvement, biopsy location, primary or secondary disease), and imaging findings were reviewed., Results: Ten patients met inclusion and exclusion criteria, and only 2 of 10 patients presented with cauda equina symptoms at the time of biopsy, with 1 patient undergoing a cauda equina biopsy. Eight patients were diagnosed with diffuse large B-cell lymphoma, 1 with low-grade B-cell lymphoma, and 1 with mantle cell lymphoma. Isolated spinal nerve involvement was identified in 5 of 10 cases, providing compelling evidence regarding the pathophysiology of NL. The conus medullaris was not radiologically involved in any case. Lumbosacral plexus MRI was able to identify extraspinal disease and offered diagnostically useful biopsy targets. FDG PET/CT was relatively insensitive for detecting disease in the cauda equina but was helpful in identifying extraspinal NL., Conclusions: The authors propose that perineural spread of extraspinal NL to infiltrate the cauda equina occurs in two phases. 1) There is proximal and distal spread along a peripheral nerve, with eventual spread to anatomically connected nerves via junction and branch points. 2) The tumor cells enter the spinal canal through corresponding neural foramina and propagate along the spinal nerves composing the cauda equina. To diffusely infiltrate the cauda equina, a third phase occurs in which tumor cells can spread circumdurally to the opposite side of the spinal canal and enter contralateral nerve roots extending proximally and distally. This spread of disease can lead to diffuse bilateral spinal nerve disease without diffuse leptomeningeal spread. Recognition of this phasic mechanism can lead to identification of safer extraspinal biopsy targets that could allow for greater functional recovery after appropriate treatment.

Objective: Examine the 18 F-FDG PET/CT and MRI imaging characteristics of chordoma., Materials and Methods: Biopsy-proven chordoma with a pre-therapy 18 F-FDG PET/CT from 2001 through 2019 in patients > 18 years old were retrospectively reviewed. Multiple PET/CT and MRI imaging parameters were assessed., Results: A total of 23 chordoma patients were included (16 M, 7 F; average age of 60.1 ± 13.0 years) with comparative MRI available in 22 cases. This included 13 sacrococcygeal, 9 mobile spine, and one clival lesions. On 18 F-FDG PET/CT, chordomas demonstrated an average SUVmax of 5.8 ± 3.7, average metabolic tumor volume (MTV) of 160.2 ± 263.8 cm 3 , and average total lesion glycolysis (TLG) of 542.6 ± 1210 g. All demonstrated heterogeneous FDG activity. On MRI, chordomas were predominantly T2 hyperintense (22/22) and T1 isointense (18/22), contained small foci of T1 hyperintensity (17/22), and demonstrated heterogeneous enhancement (14/20). There were no statistically significant associations found between 18 F-FDG PET/CT and MRI imaging features. There was no relationship of SUVmax (p = 0.53), MTV (p = 0.47), TLG (p = 0.48), maximal dimension (p = 0.92), or volume (p = 0.45) to the development of recurrent or metastatic disease which occurred in 6/22 patients over a mean follow-up duration of 4.1 ± 2.0 years., Conclusion: On 18 F-FDG PET/CT imaging, chordomas demonstrate moderate, heterogeneous FDG uptake. Predominant T2 hyperintensity and small foci of internal increased T1 signal are common on MRI. The inherent FDG avidity of chordomas suggests that 18 F-FDG PET/CT may be a useful modality for staging, evaluating treatment response, and assessing for recurrent or metastatic disease.

Multiple myeloma (MM) is a heterogeneous disease that may be evaluated by a broad array of imaging and laboratory techniques to measure disease activity and predict prognosis. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) scanning has been shown to be predictive of patient outcomes throughout the disease course. We sought to corroborate these findings by examining the prognostic impact of PET/CT scanning in the posttransplant setting. We retrospectively analyzed PET/CT scans in 229 MM patients receiving an autologous stem cell transplant (ASCT) near day 100, and correlated these findings with time to progression(TTP) and overall survival (OS) to assess the impact of day 100 PET/CT scan findings as an independent prognostic factor. The median OS for the entire cohort was 61.5 months (95% confidence interval [CI], 49-75) and the median TTP was 18.5 months (95% CI, 15.4-21.8). Among patients with abnormal day 100 PET findings (PET+), median TTP was 12.4 months vs 24 months among those with normal PET findings (PET-) (P < .0001). The median OS in the PET+ group was 46 months compared with 99 months in the PET- group (P < .0001). We conclude that an abnormal PET/CT scan near day 100 post-ASCT is predictive of shorter TTP and OS, with prognostic significance retained after adjusting for disease response and other prognostic variables in MM., (© 2021 by The American Society of Hematology.)

BRAF and MEK inhibitor combination therapy is the standard treatment for patients with BRAF V600E mutant metastatic melanoma. Neutrophilic panniculitis is a known rare complication of BRAF inhibitor therapy and can act as a potential mimic of melanoma metastases on 18 F-FDG PET/CT. In this case series, we present three cases of BRAF inhibitor-induced panniculitis in patients being treated for BRAF-mutant metastatic melanoma and emphasize the use of ultrasound to differentiate between panniculitis lesions, which are typically ill-defined echogenic masses and subcutaneous soft tissue melanoma metastases, which present as hypoechoic vascular masses.

Purpose: The purpose of this study was to evaluate the imaging characteristics of epithelioid hemangioendothelioma (EHE) on staging 18 F-FDG PET/CT., Materials and Methods: An IRB-approved retrospective review was conducted for patients with biopsy-proven EHE who underwent FDG PET/CT at our institution between 2005 and 2019. Patients with a history of surgery, chemotherapy, or radiotherapy prior to PET/CT were excluded. PET/CT exams were analyzed, noting metabolic activity, distribution of involvement, and CT morphologic features. PET/CT findings were correlated with comparative CT and MRI performed within three months., Results: There were 35 patients [21 females, 14 males; average age 55.1±16.9 years (range 15-82 years)]. 18/35 patients (52%) had more than one organ affected on PET/CT. The most common sites were liver [21/35 (60%)], lung [(19/35 (54%)], bone [5/35 (14%)], lymph nodes [4/35 (11%)], and vasculature [4/35 (11%)]. Most patients [30/35, (86%)] presented with multiple lesions. The average largest lesion dimension was 4.0±3.6 cm (range 0.6-15.0 cm). The average SUVmax of the most metabolically active lesion at any site was 5.3±3.3 (range 1.2-17.1), and for bone was 7.9±5.4 (range 3.5-17.1), liver was 5.1±2.1 (range 2.6-10.5), and lung was 3.0±1.9 (range 1.2-8.5). Of patients with pulmonary lesions, 9/19 (47%) showed calcification, and 4/19 (21%) had nodules that were either non FDG-avid or too small for accurate SUV assessment. Of patients with hepatic lesions, 11/21 (52%) demonstrated capsular retraction, and 12/21 (57%) were found to have additional hepatic lesions on contrast-enhanced CT or MRI that were occult on PET/CT., Conclusion: EHE demonstrates variable, but most commonly moderate FDG activity on PET/CT. The most common sites of disease are the liver, lungs, and bones, and most patients present with multiple lesions and more than one organ involved. Given the intrinsic metabolic activity and multi-organ involvement, FDG PET/CT represents an attractive modality for EHE evaluation. However, it may be best used in combination with CT or MRI given that EHE pulmonary or hepatic lesions may be missed by PET/CT., Competing Interests: None., (AJNMMI Copyright © 2021.)

Purpose: The aim of this study was to examine the MRI and FDG PET/CT imaging features of pathologically proven schwannomas., Patients and Methods: This institutional review board-approved retrospective study examined biopsy-proven schwannomas that underwent FDG PET/CT and/or MRI at our institution between January 1, 2002, and April 1, 2018. PET/CT features analyzed included SUVmax, metabolic ratios, volumetric metabolic measures, presence of calcification, and pattern of FDG activity. MRI features included T1/T2 signal, enhancement pattern, margins, perilesional edema, presence of muscular denervation, and size., Results: Ninety-five biopsy-proven schwannomas were identified (40 with both PET and MRI, 35 with PET only, and 20 with MRI only), 46 females and 49 males, average age of 57.7 ± 15.3 years. The average largest dimension was 4.6 ± 2.7 cm, the average SUVmax was 5.4 ± 2.7, and lesion SUVmax/liver SUVmean was 2.2 ± 1.2. Eleven (15%) of 75 lesions had SUVmax greater than 8.1, 26/75 (35%) had SUVmax greater than 6.1, and 14/75 (19%) had lesion SUVmax/liver SUVmean greater than 3.0. On MRI, 29/53 (55%) demonstrated internal nonenhancing areas. Twenty-eight (70%) of 40 lesions with both MRI and PET demonstrated at least 1 imaging feature concerning for malignant peripheral nerve sheath tumor (irregular margins, internal nonenhancement, perilesional edema, heterogeneous FDG uptake, or SUVmax >8.1). Lesions with heterogeneous FDG activity had higher SUVmax (6.5 ± 0.5 vs 4.7 ± 0.4, P = 0.0031) and more frequent internal nonenhancement on MRI (P = 0.0218)., Conclusions: Schwannomas may be large, be intensely FDG avid, and demonstrate significant heterogeneity, features typically associated with malignant peripheral nerve sheath tumors. A significant proportion exhibit FDG activity above cutoff levels previously thought useful in differentiating malignant from benign peripheral nerve sheath tumors., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Multiple mononeuropathy is a rare presentation of primary (AL) amyloidosis and nerve biopsy is usually needed for diagnosis. Conventional imaging is useful to identify proximal nerve involvement but may be inadequate. We report a patient with multiple mononeuropathy whose presentation was suggestive of AL amyloid neuropathy and in whom repeated tissue biopsies were negative for amyloid (including two sensory nerves and one muscle)., Methods: The patient underwent magnetic resonance imaging (MRI) and whole body 18 F-florbetapir positron emission tomography (PET)/MRI., Results: Whole body 18 F-florbetapir PET/MRI revealed abnormal low-level florbetapir uptake in the right proximal tibial and peroneal nerves, which provided a target for a sciatic bifurcation fascicular nerve biopsy that was diagnostic of AL amyloidosis., Conclusions: 18 F-florbetapir PET/MRI imaging is a promising diagnostic tool for patients with suspected peripheral nerve amyloidosis (including multiple mononeuropathy) in whom conventional imaging and nerve and muscle biopsies miss the pathology., (© 2020 Wiley Periodicals LLC.)

A variety of non-neoplastic diseases of the spine, including herniated/sequestered intervertebral discs, synovial cysts, and degenerative or post-traumatic changes, may present as mass lesions. Over the past several years, we have seen a large number of such paraspinal pseudoneoplasms in consultation, referred out of concern for malignancy on the part of the clinician, pathologist, or both. Herein, we report our experience with these specimens, emphasizing the clinical, radiologic, and histopathological features that allow their confident distinction from various mesenchymal tumors. Fifty-eight cases were identified within our consultation archives, referred in consultation to exclude malignancy and diagnosed as non-neoplastic disease involving the intervertebral disc, ligamentum flavum, or paraspinal soft tissues (2006-2019). Available radiologic studies were reviewed by 2 musculoskeletal radiologists. The histologic features of all cases were re-evaluated. Available clinical records were reviewed. The masses occurred in adults (median age 62 years, range 20-86 years) with a male predominance (35 males and 23 females). Sites included lumbar spine (N = 33), thoracic spine (N = 15), cervical spine (N = 6), paraspinal region (N = 3), and sacral spine (N = 1). In 44 cases (76%), the referring pathologist regarded the specimen as representing a benign or malignant neoplasm, either primary or metastatic. Fifteen cases (26%) were sent for second opinion at the request of the treating clinician, following an initial malignant diagnosis. Advanced imaging studies were available for re-review in 37 cases (64%) and showed herniated/extruded disc (N = 17), compression fracture (N = 9), synovial cyst (N = 8), and degenerative joint disease (N = 7). Multiple radiologic findings were seen in 9 patients. Histologically, the specimens showed a spectrum of often florid reactive changes involving degenerating disc material, ligamentum flavum, and bone. Awareness that non-neoplastic spinal processes may form pseudoneoplastic mass lesions, and careful clinical-radiologic-pathologic correlation should allow their confident distinction from potential morphologic mimics., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The role of quantitative magnetic resonance imaging (MRI) and positron emission tomography/computed tomography (PET/CT) techniques continues to grow and evolve in the evaluation of musculoskeletal tumors. In this review we discuss the MRI quantitative techniques of volumetric measurement, chemical shift imaging, diffusion-weighted imaging, elastography, spectroscopy, and dynamic contrast enhancement. We also review quantitative PET techniques in the evaluation of musculoskeletal tumors, as well as virtual surgical planning and three-dimensional printing., Competing Interests: Kay M. Pepin reports personal fees from Resoundant, Inc., outside the submitted work., (Thieme. All rights reserved.)

Background: Standard bone marrow biopsy (BMB) and bone involvement with follicular lymphoma (FL) on positron emission tomography (PET)/computed tomography (CT) both predict early clinical failure in FL. The key clinical question is whether PET/CT findings can obviate the need for BMB. The goal of this study was to determine the value of PET/CT in determining bone involvement in FL, using posterior iliac crest BMB as the gold standard., Materials and Methods: A total of 548 patients with newly diagnosed grade 1-3A FL were included. The presence, pattern, and location of bone involvement, spleen involvement, and standardized uptake values (SUVs) in the L3 vertebral body were recorded for all patients and compared with the BMB report., Results: Excluding patients with focal bone lesions on PET/CT, the sensitivity and specificity of PET/CT in detecting bone or marrow involvement, compared with BMB, were 53% and 88%, respectively. The sensitivity and specificity of spleen involvement on PET/CT in predicting a positive BMB were 55% and 86%, respectively. An L3 SUV max of less than 2.0 resulted in a negative predictive value (NPV) of 96% for marrow involvement on BMB; an L3 SUV mean below 1.4 resulted in an NPV of 100%., Conclusion: In newly diagnosed FL, PET/CT-detected bone and splenic involvement is highly specific for a positive BMB, and very low SUV values (<2.0 SUV max and < 1.4 SUV mean ) in the lumbar spine have a high NPV for a negative BMB. Routine BMB may be obviated in these patients. BMB remains necessary to definitively exclude bone marrow involvement in a large majority of patients with a negative PET., Implications for Practice: Predicting early clinical failure in follicular lymphoma (FL) is important but difficult. Bone marrow involvement by FL is associated with early clinical failure, and determining this involvement is a key component of the initial staging. This study highlights that in certain patients, positron emission tomography/computed tomography is sufficient in determining bone or marrow involvement, without the need for a confirmatory bone marrow biopsy (BMB). An algorithm is provided based on these findings to help clinicians determine which patients would benefit from BMB and when it can be avoided., (© AlphaMed Press 2020.)

With rapid advancements in the diagnosis and treatment of multiple myeloma (MM), imaging has become instrumental in detection of intramedullary and extramedullary disease, providing prognostic information, and assessing therapeutic efficacy. Whole-body low dose computed tomography (WBLDCT) has emerged as the study of choice to detect osteolytic bone disease. Positron emission tomography/computed tomography (PET/CT) combines functional and morphologic information to identify MM disease activity and assess treatment response. Magnetic resonance imaging (MRI) has excellent soft-tissue contrast and is the modality of choice for bone marrow evaluation. This review focuses on the imaging modalities available for MM patient management, highlighting advantages, disadvantages, and applications of each., (© 2020 Wiley Periodicals, Inc.)

Monoclonal gammopathy of undetermined significance (MGUS) is a common finding in clinical practice. The risk for developing MGUS increases with aging in parallel with age-associated increases in fracture risk. Although there is good evidence that patients with MGUS suffer from increased fracture risk, no standardized guidelines exist for the evaluation and/or management of skeletal health in patients with MGUS. Trabecular bone score (TBS), a texture index derived from lumbar spine (LS) dual-energy x-ray absorptiometry (DXA) images, provides information about trabecular microarchitecture independent of bone mineral density (BMD). We retrospectively identified 155 adult patients diagnosed with MGUS between 2005 and 2018. This group was matched 1:1 to a control group for sex, age and BMI. TBS was performed retrospectively, and values categorized as low (≤1.23), intermediate (1.23-1.31) or normal (>1.31). Patients had a mean ± SD age of 69.6 ± 10.0. BMD was performed within a median of 28 months (IQR 1-78) of MGUS diagnosis. Cases had a non-statistically significant higher rate of fractures compared to control subjects (27 vs. 17, respectively, p = 0.1). Patients with MGUS had a significantly lower TBS (1.31 ± 0.13 vs. 1.34 ± 0.12, respectively, p < 0.05) and lower LS BMD (1.215 ± 0.223 vs. 1.275 ± 0.247, p < 0.05) compared to controls. Although fractures occurred more commonly in those control subjects with significantly lower TBS values, this was not the case in subjects with MGUS (TBS 1.299 vs. 1.313 in cases with vs. without fractures p = 0.313). Similarly, there was no difference in T-scores in cases with or without fractures (-1.33 vs. -1.37, respectively, p = 0.56). Despite patients with MGUS having a significantly increased fracture risk compared to age-, sex- and BMI-matched control subjects, neither assessment of BMD nor TBS, obtained within two years of MGUS diagnosis, were able to accurately risk stratify MGUS patients. Unlike control subjects, patients with MGUS tend to fracture despite normal BMD and intermediate or normal TBS values, suggesting that deterioration of cortical rather than trabecular skeletal components may be more important for the increased fracture risk seen in MGUS., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Desmoid-type fibromatosis (DTF) frequently arises in patients with neuromuscular choristoma (NMC). We hypothesize that NMC-associated DTF occurs in soft tissues innervated by the NMC-affected nerve, and arises from CTNNB1-mutated (myo) fibroblasts within or directly adjacent to the NMC., Materials and Methods: A retrospective review of patients treated at our institution was performed for patients with biopsy-confirmed diagnosis of NMC-DTF. Clinical presentation, physical examination, electrodiagnostic findings and radiological features (MR and FDG PET/CT images for each NMC-DTF) and pathologic re-review of available materials were analyzed. A literature review was also performed., Results: Eight patients from our institution met the inclusion criteria. All patients presented with neuropathic symptoms and soft tissue or bone changes in the nerve territory innervated by the NMC. All MR images (N=8 cases) showed the characteristic features of NMC, and also showed direct contact between unifocal (N=5) or multifocal (N=3) DTF(s) and the NMC-affected nerve NMC. FDG PET/CT (N=2 cases) showed diffuse, increased FDG uptake along the entire affected nerve segment, contiguous with the FDG-avid DTF. In all cases, the DTFs arose in the soft tissues of the NMC-affected nerve's territory. No patient developed DTF at any other anatomic site., Conclusions: These data demonstrate that NMC-DTF arises solely within the NMC-affected nerve territory, and has direct contact with the NMC itself. Based on all these findings and the multifocality of NMC in several cases, we recommend imaging and surveillance of the entire NMC-affected nerve (from spine to distal extremity) to identify clinically-occult DTF in patients with NMC.

Diffuse-type tenosynovial giant cell tumor (TSGCT) is a rare, locally aggressive neoplasm. It most commonly occurs in the knee, followed by the hip, and has distinctive imaging features, including mass-like foci of low T2 signal intensity, "blooming" on gradient-echo MRI, and pronounced uptake on FDG PET/CT. Histologically, TSGCT demonstrates a neoplastic population of mononuclear cells admixed with hemosiderin-laden macrophages, foamy histiocytes, inflammatory cells, and osteoclast-like giant cells. In cases where diffuse-type TSGCT presents in an uncommon location or with atypical features, the imaging diagnosis may be challenging. Furthermore, because of its polymorphous appearance, it may be mistaken microscopically for other neoplastic and non-neoplastic histiocytic lesions. Herein, we present two cases of diffuse-type TSGCT presenting as large masses, and underscore the importance of radiologic-pathologic correlation for accurate diagnosis.

The objective was to evaluate the 18 F-FDG PET/CT characteristics of lipomatous tumors, and examine features helpful in differentiating benign from malignant tumor subtypes. Patients undergoing 18 F-FDG PET/CT from 01/2005 to 03/2018 with subsequent pathologically confirmed liposarcoma, lipoma, or hibernoma were retrospectively reviewed with IRB approval. A variety of imaging features, including metabolic activity and tumor morphology were noted. 67 tumors were included: 13 lipomas, five hibernomas, 16 atypical lipomatous tumors, 16 dedifferentiated liposarcomas, 15 myxoid liposarcomas, and two pleomorphic liposarcomas. There were 42 males and 23 females, mean age 58.8 ± 13.6 years. Mean SUVmax of lipomas measured 0.8 ± 0.2, atypical lipomatous tumors 2.3 ± 1.2, myxoid liposarcomas 3.0 ± 1.0, hibernomas 11.9 ± 8.4, pleomorphic liposarcomas 13.5 ± 2.9, and dedifferentiated liposarcomas 16.3 ± 11.4. There was no significant difference in metabolism between benign and malignant subtypes (SUVmax 3.9 ± 6.5 versus 7.6 ± 9.2, P = 0.13). There was a significant difference in metabolism between low- and high-grade liposarcoma (SUVmax 2.5 ± 1.2 versus 12.8 ± 10.8, P = 0.0001). 10/13 lipomas, 2/5 hibernomas, and 2/16 atypical lipomatous tumors had no internal soft tissue content. There are overlapping 18 FDG PET/CT features of benign and malignant lipomatous tumors. While liposarcoma grade correlates with SUVmax, malignant lesions (myxoid liposarcomas and atypical lipomatous tumors) may present with low FDG uptake and benign lesions (hibernomas) may demonstrate high metabolic activity. In some instances, a combination of metabolic and morphologic characteristics may narrow the differential diagnosis, or even be diagnostic., Competing Interests: None., (AJNMMI Copyright © 2020.)

Objective: To examine the CT and MRI characteristics of extraneural perineuriomas., Materials and Methods: With IRB approval, our institutional imaging database was retrospectively reviewed for cases of pathologically proven extraneural perineuriomas. CT and MRI features were recorded, correlative imaging analyzed, and the electronic medical record cross-referenced., Results: We identified ten patients [(seven males, three females, mean age 49.4 ± 18.3 years (range, 16-70 years)]. All cases were pathologically confirmed. Nine cases were conventional soft tissue extraneural perineuriomas, including one with "reticular" features and one with histologic features of malignancy; the tenth case contained admixed Schwann cells (hybrid perineurioma/schwannoma). Six out of ten patients underwent CT and ten of ten MRI evaluation. Nine out of ten MRIs were performed with IV contrast. Five lesions were subcutaneous, four intermuscular, and one intramuscular. Mean lesion diameter was 4.3 ± 2.7 cm (range, 0.9-10.2 cm). Nine out of ten lesions were well circumscribed; one had irregular margins. On CT, five of six were hypodense and one isodense compared to skeletal muscle. Most lesions were T1 isointense (5/10) or hypointense (4/10) and T2 hyperintense (7/10) relative to skeletal muscle, and demonstrated solid enhancement (6/9). There was no evidence of muscular denervation on any MRI exam, and a nerve of origin was identified in two out of ten cases., Conclusions: Extraneural perineuriomas have a distinctly different imaging appearance from intraneural perineuriomas, manifesting as rounded or ovoid soft tissue masses, without evidence of muscular denervation, and usually without an apparent nerve of origin. Because these features mimic other benign and malignant soft tissue lesions, including sarcomas, biopsy or excision is needed for definitive diagnosis.