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The skull base (SB) is the osseous foundation of the cranial vault. It contains many openings that allow communication between the extracranial and intracranial structures. This communication is crucial in normal physiologic processes yet may also arrow spread of disease. This article provides a comprehensive review of SB anatomy including important landmarks and anatomic variants relevant to SB surgery. We also illustrate the diverse pathologies affecting the SB., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: To report on the outcomes of immunotherapy in patients with locally advanced periorbital squamous cell carcinoma., Methods: We performed a retrospective chart review of seven consecutive patients with locally advanced periorbital cutaneous squamous cell carcinoma treated with anti-PD-1 immunotherapy. Treatments and therapeutic outcomes were reviewed., Results: Of the seven patients, six were treated with cemiplimab, and one was treated with pembrolizumab. Five patients were treated with immunotherapy as neoadjuvant therapy before planned surgical resection; two patients received immunotherapy for treatment of advanced recurrent lesions deemed unresectable following multiple previous excisions and radiation therapy. In all seven patients, measurable clinical and/or radiologic response was observed., Conclusions: Our findings support the emerging role of anti-PD-1 immunotherapy in the management of locally advanced periorbital cutaneous squamous cell carcinoma., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Graves' disease is an autoimmune disorder in which hyperthyroidism results in various systematic symptoms, with about 30% of patients presenting with Graves' eye disease (GED). The majority of patients with GED develop mild symptoms, including eyelid retraction, exposure of the globe, superior rectus-levator muscle complex inflammation, and fat expansion, leading to exophthalmos. More severe cases can result in extraocular muscle enlargement, restricted ocular movement, eyelid and conjunctival edema, and compression of the optic nerve leading to compressive optic neuropathy (CON). GED severity can be classified using the Clinical Activity Score, European Group on Graves' Orbitopathy scale, NO SPECS Classification system, and VISA system. CT and MRI aid in the diagnosis of GED through the demonstration of orbital pathology. Several recent studies have shown that MRI findings correlate with disease severity and can be used to evaluate CON. Mild cases of GED can be self-limiting, and patients often recover spontaneously within 2-5 years. When medical treatment is required, immunomodulators or radiotherapy can be used to limit immunologic damage. Surgery may be needed to improve patient comfort, preserve the orbit, and prevent vision loss from optic nerve compression or breakdown of the cornea.

Objective: The aim of the study is to determine whether multiphase multidetector computed tomography (4D-MDCT) can differentiate between intrathyroid parathyroid adenomas (ITPAs), colloid nodules, and papillary thyroid carcinoma (PTC)., Methods: We studied 22 ITPAs, 22 colloid nodules, and 11 PTCs in 55 patients. Hounsfield unit (HU) values of the nodules were measured on 4D-MDCT in the precontrast, arterial, venous, and delayed phases. Raw HU values, phase with peak enhancement, and washout percentages between the phases were evaluated., Results: Regardless of size, all ITPAs (22/22) showed peak enhancement in the arterial phase, which was significantly greater than both colloid nodules (15/22) and PTC (6/11, P = 0.002); thus, nodules with peak enhancement in the venous or delayed phase were not ITPAs (specificity = 1). For nodules with peak enhancement in the arterial phase, the percentage washout in the arterial-to-venous phases separated ITPAs from PTC and colloid nodules (P < 0.001) with greater than or equal to 23.95% loss of HU value implying IPTA (area under curve, 0.79). This left a subset of colloid nodules or PTC that either peaked in the venous or delayed phase or had an arterial-to-venous phase washout of less than 23.95%. From this subset, PTC measuring 1 cm or greater could be separated from colloid based on HU values in the arterial phase with a cutoff HU value less than 81.4 for PTC (area under curve, 0.72) and an HU value greater than 164.5 suggested colloid., Conclusions: Intrathyroid parathyroid adenomas can be distinguished from colloid nodules and PTC by peak enhancement in the arterial phase and rapid washout. A subset of colloid and PTC measuring 1 cm or greater can be separated using arterial phase HU values., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To evaluate the frequency and clinical course of residual orbital masses on imaging studies after multimodality treatment for orbital rhabdomyosarcoma., Design: Retrospective case series., Methods: We reviewed records of patients with primary orbital rhabdomyosarcoma who underwent chemotherapy and radiotherapy after surgical biopsy or debulking at 4 US centers during 1998-2019. Demographics, histologic subtype, tumor response 12 weeks after chemotherapy initiation and after completion of all treatment, and imaging findings were analyzed., Results: Thirty-two patients met inclusion criteria. Twenty-two were male, and 30 were younger than 18 years. Histologic subtype was embryonal in 22 patients, alveolar in 8, and mixed embryonal/alveolar in 2. Median follow-up time was 46 months (range, 4.9-199 months). Two patients died. Twenty-seven patients had reliable end-of-treatment imaging findings, of whom 9 had a residual mass. Three residual masses disappeared spontaneously (by 4, 32, and 53 months), 2 remained at last contact, at 2 and 7 years of follow-up, and 3 were excised; 1 progressed and underwent an exenteration. Complete response at 12 weeks was associated with complete response at the end of treatment (P < .001). Patients with T1 or T2 tumor at presentation were more likely to have complete response at last contact than were those with T3 or T4 tumor (P < .05). Biopsy type (incisional or excisional) was not associated with response to treatment at any time point., Conclusion: A residual orbital mass on imaging may be present after multimodality treatment in approximately one-third of patients. Resolution without biopsy or excision varied from months to years., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Thyroglossal duct cysts (TGDCs) are the most common congenital midline cystic lesions in the neck, and they are often evaluated by fine-needle aspiration. Recognizing the cytomorphologic features of TGDCs and their mimics is important for clinical management., Methods: This study examined the clinical, radiological, and cytopathological features of 86 ultrasonography-guided fine-needle aspiration (US-FNA) specimens from clinically suspected TGDCs or malignancies arising from TGDCs and correlated the findings with surgical follow-up and/or imaging studies., Results: According to ultrasound examinations of 66 lesions, 17 (25.8%) were cystic, 8 (12.1%) were cystic with septations, 21 (31.8%) were cystic with solid nodules, and 20 (30.3%) were solid or cystic with internal debris. Cytopathologically, 81 lesions (94%) were categorized as benign, 2 (2%) were categorized as atypical, and 3 (3%) were categorized as malignant. In benign lesions, proteinaceous material (63%), histiocytes (63%), colloid (37%), squamous cells (35%), columnar cells (32%), follicular cells (15%), inflammatory cells (9%), and multinucleated giant cells (9%) were noted. Diagnoses in the benign category included TGDC in 64 patients (75%), TGDC or mimics (colloid nodule/epidermoid cyst) in 14 patients (17%), a colloid nodule in 1 patient, and thyroiditis in 1 patient. Surgical resection, performed in 23 patients, confirmed TGDCs in 12, benign mimics in 7, and carcinoma in 4., Conclusions: Cytopathological features, in conjunction with imaging, allowed a definite diagnosis of TGDC in most patients (75%). The presence of mature squamous cells, thyroid follicular cells, with or without colloid and/or lymphocytes alone allowed a differential diagnosis of TGDC and its mimics in 17%. US-FNA findings could not distinguish primary carcinomas arising from TGDCs from metastatic tumors., (© 2021 American Cancer Society.)

Background: To describe the clinical presentation, treatment, and overall prognosis in eight patients with primary Ewing's sarcoma (ES) involving the orbit., Methods: A retrospective interventional study of all biopsy-proven cases of primary ES involving the orbit was done., Results: There were seven males and one female with a median age of 14 years. Imaging showed osseous involvement in all eight cases with extraorbital extension in four. Complete tumor resection was done in four, partial resection in three, and biopsy followed by sinus surgery in one. EWSR1 gene rearrangement analysis was done to confirm diagnosis. All patients received multidrug systemic chemotherapy and seven patients received adjuvant radiotherapy. Eye salvage was achieved in all patients. At a mean follow-up duration of 52.63 months, seven patients were doing well with no evidence of disease., Conclusions: ES involving the orbit is sensitive to chemotherapy and radiation. Aggressive multimodality treatment can help salvage the globe and improve overall survival., (© 2021 Wiley Periodicals LLC.)

Purpose: To study the multidisciplinary management and survival outcomes of orbital metastasis (OM)., Methods: All patients with a diagnosis of OM treated during 1999-2019 were included. Clinical data were retrospectively collected and analyzed., Results: The study included 118 patients, 71 females and 47 males, with a median age of 61 years. The most common primary tumor types were breast carcinoma (43 patients), melanoma (17), and lung (13), thyroid (7), renal cell (6), and neuroendocrine carcinoma (6). Ninety-six patients had a known history of cancer at OM diagnosis. The median time from diagnosis of primary cancer to OM was 31 months (range, 0-304). In 22 patients, OM was the first sign of cancer. In 47 patients, the orbit was the only site of metastasis. The most common presenting features were restricted by extraocular motility (77 patients) and proptosis (61). Eight patients had enophthalmos. OM was diagnosed based on clinical history and imaging studies in 81 patients and orbital biopsy in 37. One hundred nine patients were treated with chemotherapy and immunotherapy, 75 with radiation, and 21 with palliative surgical resection. Eighty-two patients died during follow up. The median overall survival (OS) time after diagnosis of OM was 17 months (95% CI: 12-28). OM from renal cell carcinoma was associated with the best and OM from thyroid cancer with the worst OS. Patients with breast cancer had longer median survival (28 months; 95% CI: 15-60) than patients with lung, melanoma, neuroendocrine, or thyroid cancer., Conclusion: In this large series, breast cancer and melanoma were the most common causes of OM. Most patients had a known history of cancer at OM diagnosis and did not require orbital biopsy to confirm the diagnosis. Patients with renal cell carcinoma and breast carcinoma had the best prognosis after diagnosis of OM., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Graves disease is an autoimmune disorder caused by the breakdown of immune tolerance to thyroid antigens against the TSH receptor. In approximately 25% of patients, an inflammatory condition, Graves eye disease (GED), affects the orbital soft tissues. About 60% of patients develop mild symptoms including fat expansion and inflammation of the levator muscle complex with resultant proptosis, eyelid retraction, and exposure of the globe. The remaining patients experience enlargement of one or more of the extraocular muscles, leading to conjunctival and eyelid edema and congestion, restricted ocular movement with resultant diplopia, and optic nerve compression leading to compressive optic neuropathy., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background/aims: To identify prognostic factors for local recurrence, distant metastasis and disease-specific survival (DSS) for lacrimal gland carcinoma., Methods: All consecutive patients with lacrimal gland carcinoma treated from January 1998 through December 2018 were included. Log-rank tests and univariate Cox proportional hazards regression models were used to study risk factors and survival., Results: Overall, 55 patients were included in this study, and 5 patients were excluded from the survival analysis. Median age was 46 years (range: 10-76). 43 patients (78%) had adenoid cystic carcinoma (ACC). 31 patients (56%) had T2 disease at presentation. 28 patients (51%) underwent orbital exenteration with or without adjuvant radiotherapy or chemoradiation, 26 (47%) underwent eye-sparing surgery with or without adjuvant radiotherapy or chemoradiation, and 1 received palliative chemoradiation. 11 patients (22%) experienced local recurrence; 14 (29%) experienced distant metastasis. Five- and 10-year local-recurrence-free survival rates were 0.71 (95% CI 0.58 to 0.88), and 5- and 10-year distant-metastasis-free survival rates were 0.67 (95% CI 0.53 to 0.85) and 0.49 (95% CI 0.30 to 0.81), respectively. There was no significant difference in risks of local recurrence, distant metastasis or DSS between ACC patients who had orbital exenteration and those who had eye-sparing surgery. Perineural invasion was negatively associated with local-recurrence-free survival (p=0.02). Among patients with ACC, basaloid/solid histologic type was associated with significantly worse DSS than non-basaloid/solid histologic type (p<0.01)., Conclusions: For lacrimal gland carcinoma, orbital exenteration with adjuvant therapy and eye-sparing surgery with adjuvant therapy are associated with similar recurrence outcomes. Eye-sparing surgery is associated with better DSS. Perineural invasion is a risk factor for local recurrence. ACC with basaloid/solid subtype correlates with worse DSS., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Neurolymphomatosis involving the cranial nerves (CNs) is rare. We sought a better understanding of the primary disease sites and patterns of spread in neurolymphomatosis of the orbit and retro-orbital cranial nerves., Methods: Patients with lymphoma and MRI evidence of neurolymphomatosis of CN II, III, IV, V 1 , or V 2 were retrospectively reviewed. Demographics and primary disease site and sites of neurolymphomatosis on MRI were recorded. Wilcoxon rank sum test was used to compare number of sites of neurolymphomatosis with lymphoma type and survival., Results: The study included 18 patients. The most frequent types of lymphoma were diffuse large B-cell (DLBCL) (n = 9) and marginal zone (n = 3). In 9 patients, lymphoma presented as a mass (n = 7) or infiltrative disease (n = 2) directly involving the orbit; in 6, a maxillofacial mass spread directly to CNs; and in 3, lymphoma at remote sites spread to orbital CNs. Overall, 81 sites of neurolymphomatosis were noted. The most common sites were the maxillary nerve (V 2 ) including at the infraorbital fissure or foramen rotundum (17 patients; 19 nerves), pterygopalatine fossa (16 patients; 19 nerves), and cavernous sinus (9 patients; 12 nerves). Number of sites of neurolymphomatosis was significantly lower for DLBCL than for other lymphoma types (p = 0.007). Number of sites of neurolymphomatosis did not affect survival (p = 0.26). The mean interval between the pathologic diagnosis and MRI documentation of the full extent of neurolymphomatosis was 39 days after pathologic diagnosis., Conclusions: Based on our study results, neurolymphomatosis in the orbit appears to be frequently associated with an orbital and/or maxillofacial mass and commonly involves CN V 2 , the pterygopalatine fossa, and the cavernous sinus. DLBCL may be associated with fewer sites of neurolymphomatosis than other lymphomas. In patients with lymphoma, a systematic search for neurolymphomatosis is imperative for early detection.

Purpose: Retinoblastoma (RB) is the most common intraocular cancer and is associated with lifelong risks of developing a second primary cancer, especially in patients with hereditary RB and/or childhood exposure to radiotherapy., Methods: The study included all consecutive patients with a history of RB treated for a second primary cancer during 1994-2018. Patient demographics and characteristics of the primary RB and second primary cancer were examined. The associations among radiation or chemotherapy exposure as a treatment for RB, unilateral versus bilateral status, types and multiplicity of second primary cancers, and survival after diagnosis of second primary cancer were analyzed., Results: A wide spectrum of second primary cancer types was identified from 62 eligible patients (30 males and 32 females), including sarcoma, breast cancer, various skin cancers, gastrointestinal and genitourinary cancers, and endocrine cancers. Of all patients who had second primary cancers, 40 patients (65%) had bilateral RB and 17 patients (27%) had unilateral RB. Thirty-five patients (56%) who developed second primary cancers received radiation therapy during childhood as the treatment of RB, and 17 patients (27%) received chemotherapy for the treatment of RB. The 5-year and 10-year survival rates for RB patients diagnosed with a second primary cancer were 54.0% and 36.0%, respectively. The median age of onset of second primary cancer among RB survivors was 36.6 years., Conclusions: In contrast to previous studies, we found a broader spectrum of second primary cancer types. All RB survivors, regardless of unilateral or bilateral status, should undergo strict cancer surveillance particularly as they approach the fourth decade of life., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2021 Asia-Pacific Academy of Ophthalmology. Published by Wolters Kluwer Health, Inc. on behalf of the Asia-Pacific Academy of Ophthalmology.)

Magnetic resonance imaging (MRI) offers outstanding soft tissue contrast that may reduce uncertainties in target and organ-at-risk delineation and enable online adaptive image-guided treatment. Spatial distortions resulting from non-linearities in the gradient fields and non-uniformity in the main magnetic field must be accounted for across the imaging field-of-view to prevent systematic errors during treatment delivery. This work presents a modular phantom and software application to characterize geometric distortion (GD) within the large field-of-view MRI images required for radiation therapy simulation. The modular phantom is assembled from a series of rectangular foam blocks containing high-contrast fiducial markers in a known configuration. The modular phantom design facilitates transportation of the phantom between different MR scanners and MR-guided linear accelerators and allows the phantom to be adapted to fit different sized bores or coils. The phantom was evaluated using a 1.5 T MR-guided linear accelerator (MR-Linac) and 1.5 T and 3.0 T diagnostic scanners. Performance was assessed by varying acquisition parameters to induce image distortions in a known manner. Imaging was performed using T1 and T2 weighted pulse sequences with 2D and 3D distortion correction algorithms and the receiver bandwidth (BW) varied as 250-815 Hz pixel -1 . Phantom set-up reproducibility was evaluated across independent set-ups. The software was validated by comparison with a non-modular phantom. Average geometric distortion was 0.94 ± 0.58 mm for the MR-Linac, 0.90 ± 0.53 mm for the 1.5 T scanner, and 1.15 ± 0.62 mm for the 3.0 T scanner, for a 400 mm diameter volume-of-interest. GD increased, as expected, with decreasing BW, and with the 2D versus 3D correction algorithm. Differences in GD attributed to phantom set-up were 0.13 mm or less. Differences in GD for the two software applications were less than 0.07 mm. A novel modular phantom was developed to evaluate distortions in MR images for radiation therapy applications.

Purpose: To review the clinical presentation, treatment, and prognosis of patients with malignant mixed tumor (carcinoma ex pleomorphic adenoma) of the lacrimal gland., Methods: Clinical records and radiographic images were reviewed for patients with malignant mixed tumor of the lacrimal gland treated at the center during 2008-2019., Results: The study included 9 patients (6 men, 3 women) aged 17-66 years (median age, 56 years). Six had primary malignant mixed tumor with no history of orbital lesions, and 3 had previously been diagnosed with pleomorphic adenoma. Tumor, Node, Metastasis classification per the eighth edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual were T1aN0M0 in 2 patients, T2aN0M0 in 3 patients, T4bN0M0 in 2 patients, and T4cN0M0 in 2 patients. Two patients underwent orbital exenteration, 6 patients underwent eye-sparing surgery, and 1 patient had an unresectable tumor because of cavernous sinus extension. All patients received radiotherapy (intensity-modulated radiotherapy in 3 and proton therapy in 6). All patients received chemotherapy, 8 concurrently with radiotherapy and 1 after radiotherapy. The median follow-up time was 70 months. At last contact, 6 patients were alive without evidence of disease; 2 had died of disease, 1 of distant metastasis, and the other of cavernous sinus invasion., Conclusions: The findings suggest that de novo malignant mixed tumor of the lacrimal gland is more common than disease that results from transformation after incomplete resection of lacrimal gland pleomorphic adenoma. Most cases can be treated with eye-sparing surgery and radiation unless skull base extension is present.

Background: To test the hypothesis that intraventricular ADC values can be used to determine the presence of neoplastic leptomeningeal disease (LMD)., Materials and Methods: ADC values were measured at multiple sites in the ventricular system in 32 patients with cytologically-proven LMD and 40 control subjects. Multiple linear regression analysis was used to determine the mean difference of ADCs between the LMD and control groups after adjusting for ventricle size and tumor type. Receiver operating characteristics (ROC) analysis was performed and optimal ADC value cut-off point for predicting the presence of LMD. ADC was compared to T1 enhancement and FLAIR signal hyperintensity for determining the presence of LMD., Results: After adjusting for ventricular volume and tumor type, the mid body of lateral ventricles showed no significant difference in ventricular volume and a significant difference in ADC values between the control and LMD groups (p > 0.05). In the mid-body of the right lateral ventricle the AUC was 0.69 (95% CI 0.57-0.81) with an optimal ADC cut off point of 3.22 × 10 - 9  m 2 /s (sensitivity, specificity; 0.72, 0.68). In the mid-body of left lateral ventricle the AUC was 0.7 (95% CI 0.58-0.82) with an optimal cut-off point of 3.23 × 10 - 9  m 2 /s (0.81, 0.62). Using an average value of HU measurements in the lateral ventricles the AUC was 0.73 (95% CI 0.61-0.84) with an optimal cut off point was 3.11 × 10 - 9  m 2 /s (0.78, 0.65). Compared to the T1 post-contrast series, ADC was predictive of the presence of LMD in the mid-body of the left lateral ventricle (p = 0.036)., Conclusion: Complex interactions affect ADC measurements in patients with LMD. ADC values in the lateral ventricles may provide non-invasive clues to the presence of LMD.

Background and Purpose: During thyroidectomy incomplete resection of the thyroid gland may occur. This complicates the imaging surveillance of these patients as residual thyroid needs to be distinguished from local recurrence. Therefore, the purpose of this study was to determine if multiphasic multi-detector computed tomography (4D-MDCT) can differentiate residual nonmalignant thyroid tissue and recurrent thyroid carcinoma after thyroidectomy., Materials and Methods: In this retrospective study, Hounsfield unit values on multiphasic multidetector CT in precontrast, arterial (25 seconds), venous (55 seconds), and delayed (85 seconds) phases were compared in 29 lesions of recurrent thyroid cancer, 29 with normal thyroid, and 29 with diseased thyroid (thyroiditis/multinodular thyroid). The comparison of Hounsfield unit values among lesion types by phase was performed using ANOVA. The performance of Hounsfield unit values to predict recurrence was evaluated by logistic regression and receiver operating characteristic analysis., Results: All 3 tissue types had near-parallel enhancement characteristics, with a wash-in-washout pattern. Statistically different Hounsfield unit density was noted between the recurrence (lowest Hounsfield unit), diseased (intermediate Hounsfield unit), and normal (highest Hounsfield unit) thyroid groups throughout all 4 phases ( P  < .001 for each group and in each phase). Dichotomized recurrence-versus-diseased/normal thyroid tissue with univariate logistic regression analysis demonstrated that the area under the receiver operating characteristic curve for differentiating benign from malignant thyroid for the various phases of enhancement was greatest in the precontrast phase at 0.983 (95% CI, 0.954-1), with a cutoff value of ≤62 (sensitivity/specificity, 0.966/0.983) followed by the arterial phase., Conclusions: Recurrent thyroid carcinoma can be distinguished from residual nonmalignant thyroid tissue using multiphasic multidetector CT with high accuracy. The maximum information for discrimination is in the precontrast images, then the arterial phase. An optimal clinical protocol could be built from any number of phases but should include a precontrast phase., (© 2020 by American Journal of Neuroradiology.)

The use of transoral sonography-guided fine-needle aspiration for intraoperative localization of retropharyngeal masses has been described by Fornage et al. The purpose of this study was to assess the accuracy of this technique. We reviewed the images and medical records of 26 patients with a retropharyngeal lesion suspicious for a metastatic lymph node of Rouviere identified on CT and/or PET/CT. There were 14 patients with a history of thyroid cancer, 7 with mucosal squamous cell carcinoma, 1 with renal cell carcinoma, 1 with parotid acinic cell cancer, 1 with metastatic colon adenocarcinoma, and 2 with no history of cancer. Intraoperative transoral sonography was performed using a commercially available endovaginal transducer. A transoral sonography-guided fine-needle aspiration was performed with a 25-cm-long 20-ga Chiba needle through a needle guide attached to the transducer shaft. Cytopathologic results were categorized as malignant, benign, or nondiagnostic. Transoral sonography and transoral sonography-guided fine-needle aspiration were performed in all patients. A diagnostic specimen was obtained in 25 of 26 (96%) patients with a 100% overall accuracy. Twelve patients underwent subsequent transoral resection of the retropharyngeal mass. In each patient, surgical pathology confirmed the fine-needle aspiration biopsy result. In 4 patients, transoral sonography-guided injection of methylene blue was used to facilitate intraoperative localization of the metastatic retropharyngeal mass. Transoral sonography and transoral sonography-guided fine-needle aspiration of suspicious masses in the retropharyngeal space are highly accurate procedures for identification and cytologic evaluation of benign and metastatic lymph nodes of Rouviere and for presurgical localization., (© 2019 by American Journal of Neuroradiology.)

Background: The purpose of this study was to test the hypothesis that there is no significant difference in vascular flow patterns between cytopathologically-proven colloid nodules and papillary thyroid carcinoma (PTC) even when adjusting for nodule size., Methods: Doppler vascular flow patterns in 200 colloid nodules and 166 nodules with PTC were retrospective reviewed independently by 2 neuroradiologists blinded to the cytopathological results. Absence of vascular flow, perinodular and/or intranodular flow, and diffuse vascular flow were recorded. The vascular flow patterns were compared without (Fisher exact test) and with (Kruskal-Wallis test) an adjustment for nodular size. Using the most common flow pattern as the reference group, multiple logistic regression was used to compare the flow patterns. Sample skewness was calculated to determine degree of symmetry of the size distribution for each vascular flow category., Results: No significant difference was found in the tested vascular flow patterns between colloid nodules and PTC both without and with an adjustment for nodular size (P>0.05). Intranodular flow only was the largest group (n=111/366) and used as the reference for multiple logistic regression. No significant difference was noted between the vascular flow patterns (P>0.05). Sample skewness showed that nodules were generally smaller in size with outliers of larger size on the opposite end of the spectrum., Conclusions: Independent of nodule size the absence or presence of vascular flow is not significantly different between colloid nodules and PTC. Therefore, vascular flow may not be useful in distinguishing between colloid nodules and PTC., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare., (2019 Gland Surgery. All rights reserved.)

Background: The objective of the current study was to characterize the incidence, pattern, and impact on oncologic outcomes of retropharyngeal lymph node (RPLN) involvement in HPV-associated oropharyngeal cancer (OPC)., Methods: Data regarding patients with HPV-associated OPC who were treated at The University of Texas MD Anderson Cancer Center with intensity-modulated radiotherapy from 2004 through 2013 were analyzed retrospectively. RPLN status was determined by reviewing pretreatment imaging and/or reports. Outcomes analysis was restricted to patients with lymph node-positive (+) disease. Kaplan-Meier survival estimates were generated and survival curves were compared using the log-rank test. Bayesian information criterion assessed model performance changes with the addition of RPLN status to current American Joint Committee on Cancer staging. Competing risk analysis compared modes of disease recurrence., Results: The incidence of radiographic RPLN involvement was 9% (73 of 796 patients) and was found to vary by primary tumor site. The 5-year rates of freedom from distant metastases (FDM) and overall survival were lower in patients with RPLN(+) status compared with those with RPLN-negative (-) status (84% vs 93% [P = .0327] and 74% vs 87% [P = .0078], respectively). RPLN(+) status was not found to be associated with outcomes on multivariate analysis. Bayesian information criterion analysis demonstrated that current American Joint Committee on Cancer staging was not improved with the inclusion of RPLN. Locoregional and distant disease recurrence probabilities for those patients with RPLN(+) status were 8% and 13%, respectively, compared with 10% and 6%, respectively, for those with RPLN(-) status. RPLN(+) status portended worse 5-year FDM in the low-risk subgroup (smoking history of <10 pack-years) and among patients who received concurrent chemotherapy but not induction chemotherapy., Conclusions: RPLN(+) status was associated with worse overall survival and FDM on univariate but not multivariate analysis. In subgroup analyses, RPLN(+) status was associated with poorer FDM in both patients with a smoking history of <10 pack-years and those who received concurrent chemotherapy, suggesting that RPLN(+) status could be considered an exclusion criteria in treatment deintensification efforts seeking to omit chemotherapy., (© 2019 American Cancer Society.)

Purpose: To describe thyroid eye disease (TED)-like orbital inflammatory syndrome in 3 cancer patients treated with immune checkpoint inhibitors., Methods: All consecutive patients treated by the senior author who were receiving immune checkpoint inhibitors and developed TED-like orbital inflammation were included., Results: Three cancer patients treated with immune checkpoint inhibitors developed orbital inflammation. The first patient was treated with a combination of a cytotoxic T-lymphocyte antigen-4 inhibitor and a programmed cell death protein 1 inhibitor and developed TED-like orbital inflammation with normal thyroid function and antibody levels. The second patient had a previous diagnosis of Graves disease without TED, and developed TED soon after initiating treatment with a programmed cell death protein 1 inhibitor. The third patient developed acute hyperthyroidism with symptomatic TED following treatment with an investigational cytotoxic T-lymphocyte antigen-4 inhibitor agent. All 3 patients were managed with either systemic steroids or observation, with resolution of their symptoms and without the need to halt immune checkpoint inhibitor treatment for their cancer., Discussion and Conclusions: TED-like orbital inflammation may occur as a side effect of immune checkpoint inhibitor therapy with anti-cytotoxic T-lymphocyte antigen-4 or anti-PD-1 inhibitors. To the best of their knowledge, this is the first reported case of TED as a result of programmed cell death protein 1 inhibitor monotherapy. All 3 patients were treated with systemic steroids and responded quickly while continuing treatment with immune checkpoint inhibitors for their cancer. With increasing use of this class of drugs, clinicians should be familiar with the clinical manifestations and treatments for this adverse reaction.

Objective: The objective of this study was to compare multidetector computed tomography (MDCT) images with volume-rendered translucent display (VRTLD) series to plain radiographs for evaluating spinal surgical instrumentation after resection and reconstruction for spinal malignancies., Methods: In 44 patients with tumor resection and spinal reconstruction, 17 with complications, 3 neuroradiologists evaluated plain radiographs, MDCT images alone, VRTLD images alone, and MDCT images with VRTLD images for identifying complications in 3 categories: subsidence/migration, construct fracture, and screw loosening. Each category was scored as 1 (complications), 2 (no complications), or 3 (not sure), and the minimum score was used for analyses. Clinical/surgical outcomes were the reference standard., Results: Sensitivity, specificity, and accuracy (95% confidence interval), respectively, were as follows: MDCT/VRTLD, 100%, 100%, 100% (91.96%-100.00%); MDCT alone, 88.24%, 100%, 95.45% (84.53%-99.44%); VRTLD alone, 82.35%, 96.3%, 90.91% (78.33%-97.47%); plain radiographs, 52.94%, 100%, 81.82% (67.29%-91.81%)., Conclusions: Multidetector computed tomography with VRTLD series seems best for evaluation of spinal instrumentation after tumor resection and reconstruction.

Background: The aim of this study was to determine the interdisciplinary agreement in identifying the post-operative tumor bed., Methods: Three radiation oncologists (ROs), four surgeons, and three radiologists segmented post-operative tumor and nodal beds for three patients with oral cavity cancer. Specialty cohort composite contours were created by STAPLE algorithm implementation results for interspecialty comparison. Dice similarity coefficient and Hausdorff distance were utilized to compare spatial differentials between specialties., Results: There were significant differences between disciplines in target delineation. There was unacceptable variation in Dice similarity coefficient for each observer and discipline when compared to the STAPLE contours. Within surgery and radiology disciplines, there was good consistency in volumes. ROs and radiologists have similar Dice similarity coefficient scores compared to surgeons., Conclusion: There were significant interdisciplinary differences in perceptions of tissue-at-risk. Better communication and explicit description of at-risk areas between disciplines is required to ensure high-risk areas are adequately targeted.

Purpose: To evaluate the clinical and anatomical location of orbital plasmacytomas and assess local control following therapy., Methods: The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried to identify patients diagnosed with orbital plasmacytoma. These patients' records were reviewed for demographic characteristics, clinical and radiologic findings, treatments, and outcomes., Results: Thirty patients from 4 institutions (24 from MD Anderson Cancer Center, 3 from SUNY Downstate Medical Center, 2 from University of California, Los Angeles, and 1 from Oregon Health and Science University) were identified. Eighteen patients (60%) were diagnosed with multiple myeloma (MM) before and 11 (37%) were diagnosed with MM immediately after orbital plasmacytoma. Based on imaging, 4 distinct anatomical patterns were identified: 1) bony plasmacytoma affecting the superotemporal orbit, epidural space, and temporal fossa (15 patients; 50%); 2) discrete orbital plasmacytoma (7 patients; 23%); 3) infiltrative plasmacytoma either originating from a sinus (4 patients; 13%); or 4) originating from the orbital floor and infiltrating facial soft tissue (4 patients; 13%). Of the 29 patients with available treatment data, 2 had radiation only, 3 had chemotherapy only, 6 had chemoradiation, and 18 had stem cell transplant following chemoradiation (n = 17) or only chemotherapy (n = 1). Following treatment, 10 patients achieved complete and 11 achieved partial responses., Conclusion: Orbital plasmacytomas were found exclusively in patients with MM diagnosed before or immediately after orbital plasmacytoma. Plasmacytomas can have 4 distinct anatomical patterns of origin. Following treatment, all patients had good to excellent local control of their orbital lesions.

Anaplastic thyroid carcinoma is fatal if unresectable. However, improved survival has been reported after gross total resection and multimodality therapy. In this report, we describe the contrast-enhanced high-resolution CT characteristics of anaplastic thyroid carcinoma in 57 patients. Anaplastic thyroid carcinoma presented as a large neck mass with necrosis in 82% of cases. The tumors demonstrated common extrathyroidal extension (91%). Sixty-two percent of tumors demonstrated calcification. Visceral space invasion involved the esophagus (62%), trachea (57%), and larynx (29%). Carotid artery encasement was present in 42%, and 43% involved the internal jugular vein. Sixty-three percent had lateral compartment lymphadenopathy; 58% of these nodes were necrotic, and 11% were cystic. No metastatic nodes had calcification. Central compartment lymphadenopathy was seen in 56% of cases, and lateral retropharyngeal lymphadenopathy was detected in 12%. Knowledge of these imaging features aids in guiding the approach to the initial tissue diagnosis with either fine-needle aspiration or core biopsy, assessing the feasibility of surgical resection, and determining prognosis., (© 2018 by American Journal of Neuroradiology.)

Neoplastic leptomeningeal disease (LMD) represents infiltration of the leptomeninges by tumor cells. Knowledge of the frequencies of locations of LMD on MRI may assist in early detection, help elucidate the process of leptomeningeal spread of cancer and understand how LMD affects the central nervous system. Our goal was to identify intracranial sites of neoplastic LMD predilection on MRI in patients with cytologically-proven LMD. The presence of FLAIR signal hyperintensity and T1-weighted post-contrast enhancement in the sulci of the supratentorial compartment and cerebellum and enhancement of the cranial nerves (CNs), basal cisterns, pituitary stalk, and ependymal surface of the lateral ventricles, as well as the presence of parenchymal metastasis were recorded. Within each imaging sequence, sites were ordered by prevalence and compared using McNemar's test. The study included 270 patients. Positive MRI findings were present in 185/270 (68.5%) patients. FLAIR signal hyperintensity was significantly more common (p≤0.003) in the cerebellum (n=96) and occipital lobe (n=92) relative to the other lobes. Leptomeningeal enhancement was also significantly more common (p≤0.009) in the cerebellum (n=82) and occipital lobe (n=67) relative to the other lobes. Enhancement was most commonly found involving CN VII/VIII and the ependymal surface of the lateral ventricles compared to other sites. Parenchymal metastases were present in 110 (40.1%) of the patients. In conclusion, neoplastic LMD predominantly involves the cerebellum and occipital lobes, CN VII/VIII, and the ependymal lining of the lateral ventricles. Parenchymal metastases are frequently present in patients with neoplastic LMD., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Purpose: The aim of this study was to determine rates of positive findings on positron emission tomography (PET) and bone marrow biopsy performed during staging workup for ocular adnexal lymphoma (OAL)., Methods: A retrospective review of OAL patients was conducted. Demographics, primary versus secondary OAL, histologic subtype, and findings on PET and bone marrow biopsy performed as part of the initial staging workup for OAL were recorded., Results: The study included 119 patients with OAL. There were 85 primary and 34 secondary OALs. The main histologic subtypes of lymphoma were mucosa-associated lymphoid tissue (n = 61), follicular (n = 26), diffuse large B-cell (n = 17), and mantle cell (n = 10). Positive PET findings were seen in 42 of 68 patients (62%) with primary OAL and 19 of 24 (79%) with secondary OAL. Positive PET findings were seen in 24 of 47 patients (51%) with mucosa-associated lymphoid tissue, 13 of 17 (76%) with follicular, 14 of 15 (93%) with diffuse large B-cell, and 9 of 10 (90%) with mantle cell lymphoma. Positive findings on bone marrow biopsy were seen in 7 of 59 patients (12%) with mucosa-associated lymphoid tissue, 4 of 23 (17%) with follicular, 1 of 17 (6%) with diffuse large B-cell, and 2 of 9 (22%) with mantle cell lymphoma., Conclusions: Our findings suggest that a significant proportion of patients with primary and secondary OAL have positive findings on PET and bone marrow biopsy at initial diagnosis, suggesting a reasonable yield for these tests as part of the initial staging workup in patients with a new diagnosis of OAL.

Leptomeningeal disease (LMD) represents disseminated intracranial metastatic disease that requires early detection and initiation of therapy. Patients with LMD typically present with a variety of neurologic problems, including ocular disturbances. However, little is reported on LMD presenting exclusively with ocular-related disturbances in the absence of any other central nervous system (CNS) dysfunction. Our goal was to describe the workup for ocular disturbances in the setting of known cancer diagnosis. Retrospective case study utilizing prospectively collected database at a tertiary cancer care center for all patients with diagnosis of LMD between 2001 and 2009. Main outcome was descriptive analysis of ocular findings by primary or admitting service with or without formal ophthalmology exam in workup for LMD. 34 patients demonstrated ocular disturbances without any other CNS manifestations. Our findings demonstrate that 71% of ocular disturbances were detected by the primary admitting services. Formal consultation with ophthalmology resulted in the detection of the remaining cases. The most common findings were cranial nerve deficits, papilledema, and optic disc or retinal infiltration by tumor. These findings supported a further work-up for CNS disease. Therefore, it is appropriate to refer cancer patients with visual complaints or findings on exam to ophthalmology to evaluate for evidence suggestive of LMD that may support a further work-up., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Objectives: Intrathyroid metastases from extrathyroid primary tumors are rare. Clinical findings may be subtle, but detection of intrathyroid metastases has improved with sonography. The objective of this study was to evaluate the sonographic appearance of intrathyroid metastases., Methods: Patients with thyroid masses with cytopathologic features matching those of an extrathyroid primary tumor were retrospectively identified. The appearances of intrathyroid metastases on sonography were reviewed for the following features: size, margin regularity, echogenicity, echotexture, vascularity on power or color Doppler ultrasonography, and the presence or absence of any associated cervical adenopathy., Results: The study included 52 patients. The most frequent primary tumor sites were lung, head and neck, and breast. Intrathyroid metastases presented as a discrete nodule in 34 patients and as diffuse infiltration of the gland in 18 patients. The discrete nodules ranged in size from 1.1 to 5.6 cm (mean ± SD, 2.5 ± 1.2 cm). Thirty-three lesions (63%) had irregular margins, and 19 (37%) had well-defined margins. Most of the lesions were heterogeneously hypoechoic (n = 50, 96%). Vascularity was present in 32 of 50 measured lesions (64%) that were evaluated with Doppler sonography. Cervical adenopathy was present in 37 patients (71%)., Conclusions: Intrathyroid metastases have sonographic characteristics similar to those described for both benign and malignant thyroid diseases. In patients with a previous or current extrathyroid malignancy, thyroid nodules or diffuse infiltration of the thyroid gland on sonography should be viewed as a potential intrathyroid metastasis and evaluated via ultrasound-guided fine-needle aspiration regardless of the site of the primary tumor., (© 2016 by the American Institute of Ultrasound in Medicine.)

Purpose: While metastasis to the thyroid from a primary cancer remote to the thyroid is uncommon, current imaging techniques have improved detection of these intrathyroid metastases. The purpose of this study was to evaluate the 18 F-PET/CT appearance of intrathyroid metastases and assess the impact of detection on patient management., Methods: The 18 F-PET/CT appearance of intrathyroid metastasis, including standardized uptake value (SUV), disease extent, and the effect on patient management following diagnosis were retrospectively reviewed. Inclusion criteria included 18 F-PET/CT imaging and diagnosis of the intrathyroid metastasis matching the remote primary tumor., Results: Intrathyroid metastasis were detected in 24 patients. The intrathyroid metastases presented on 18 F-PET/CT as focal nodular uptake (n = 21), multiple nodular uptake (n = 2), or diffuse uptake/infiltration of the thyroid gland (n = 1). The SUV ranged between 3.9 and 42 (median 12.5 ± 7.5); in 2 patients, the FDG-avidity was minimal. On 18 F-PET/CT, distant metastases were present outside the neck (n = 18), or limited to the neck (n = 6). In 2 of these 6 patients, the thyroid was the only site of metastatic disease. Due to the metastatic disease, the therapy was changed in 23 of 24 patients; 1 patient was lost to follow-up., Conclusion: In any patient with a previous or current history of an extrathyroid malignancy, an 18 FDG-avid thyroid mass or diffuse infiltration of the thyroid on 18 F-PET/CT should be considered a potential intrathyoid metastasis until proven otherwise. Knowledge of an intrathyroid metastasis may impact patient management, especially if the thyroid or neck are the only sites of metastatic disease., Competing Interests: Conflicts of Interest Disclosure The authors declare that there is no conflict of interest statement.

The purpose of this study was to present the imaging features of primary and secondary malignant lacrimal sac and nasolacrimal duct tumors and their pattern of tumor spread in 18 patients. The most common tumor histology in our series was squamous cell carcinoma. In 15/18 patients, tumor involved both the lacrimal sac and duct at the time of diagnosis. In 11/16 patients on CT, the nasolacrimal bony canal was smoothly expanded without erosive changes. The medial canthus region (16/18) was a frequent site of direct tumor spread. Two patients had intraconal orbital spread of tumor. Tumor spread to the sinus or nasal cavity was observed in 5/13 primary tumors. Only 1 patient presented with nodal metastasis. There was no intracranial tumor extension, perineural tumor spread along the infraorbital nerve, distant metastasis, or dacryocystocele formation in any of the patients at the time of diagnosis., (© 2016 by American Journal of Neuroradiology.)

Objective: Leptomeningeal disease (LMD), the presence of metastasis in the subarachnoid space, has devastating implications if left untreated. The gold standard for LMD diagnosis is cytologic analysis of cerebrospinal fluid (CSF); MRI is also used to evaluate suspected LMD. The purpose of this study was to compare the appearance of LMD in the spinal canal on 18 F-FDG PET/CT imaging with the appearance of LMD on MRI and with CSF cytology., Methods: In twenty-one patients with cytologically-proven spinal LMD, findings on 18 F-FDG PET/CT, MRI, and CSF cytology at diagnosis of LMD and after the initiation of treatment for LMD were retrospectively reviewed., Results: At diagnosis of LMD, abnormal 18 F-FDG avidity was demonstrated in the spinal canal in six patients, and the anatomic distribution of 18 F-FDG activity corresponded to the sites of LMD on MRI. All six of these patients were then treated with intrathecal chemotherapy. Follow-up 18 F-FDG PET/CT and MRI were obtained in four of the six cases. In all four cases, normalization of 18 F-FDG activity in the spinal canal and reduction of enhancement on MRI corresponded to the cytologic response to treatment, as determined by CSF analysis., Conclusion: 18 F-FDG avidity in the spinal canal greater than the normal contents of the canal can suggest spinal LMD. This abnormal avidity may be detected before the diagnosis of LMD has been established with MRI or CSF cytology. The spinal canal should be routinely evaluated on 18 F-FDG PET/CT in patients with suspected LMD so that appropriate treatment is initiated., Competing Interests: Conflicts of Interest Disclosure The author declares that there is no conflict of interest statement.

The histiocytoses are a rare group of varied but related disorders characterized by abnormal tissue proliferation of macrophages and dendritic cells within tissues. The purpose of this article was to review the imaging findings in patients presenting with CNS and with head and neck manifestations of these disorders. Histiocytoses include but are not limited to Rosai-Dorfman disease, Erdheim Chester disease, Langerhans cell histiocytosis, histiocytic sarcoma, and juvenile xanthogranuloma. A review of the literature was performed to determine the sites of disease involvement. This article includes the demographics, histopathologic criteria for diagnosis, and imaging features of these histiocytoses, and describes the manifestations in locations known to harbor disease: intraaxial and extra-axial intracranial regions, the calvaria, skull base, hypothalamopituitary axis, orbits, paranasal sinuses, spine, and the head and neck region. Histiocytoses have variable imaging appearances in the CNS and in the head and neck region, and radiologists should be aware of the spectrum of findings to avoid mistaking them for other disease processes., Learning Objective: To understand the general pathophysiology, clinical presentation, and typical imaging characteristics of the most common histiocytoses; comprehend the morphologic and immunohistochemical characteristics of these histiocytoses and the hallmark findings on pathology; and be able to differentiate between these disorders based on their most common presentations.

Introduction: Neurofibromatosis type 1 (NF-1) may involve the spine as various abnormalities including bony dysplasia, scoliosis, and nerve sheath tumors. Surgery may be performed for stabilization of the spine. We have seen an increase in requests for multidetector CT (MDCT) imaging with the (three-dimensional) 3D-volume rendered (VR) images in patients evaluated at our institution. We, therefore, investigated how MDCT could be best utilized in this patient population., Methods: Seventy-three patients with NF-1 were identified in whom MDCT imaging was performed for diagnostic, pre-operative, or post-operative evaluation of spinal abnormalities. True axial source images and two dimensional (2D) orthogonal reconstructed MDCT images, as well as the VR images, were compared with plain radiographs and MRI. In addition, the MDCT study was compared to the VR images. These studies were reviewed to compare assessment of A) bony abnormalities such as remodeling from dural ectasia, dysplasia, and fusion, B) abnormal spinal curvature, C) nerve sheath tumors, and D) surgical instrumentation., Results: When compared to plain radiographs, the MDCT and VR images were rated as helpful for evaluating the abnormalities of the spine in 19 of 24 patients for a total of 30 findings. This included the following categories A) (n = 6), B) (n = 5), C) (n = 7), and D) (n = 12). Compared to MR, the MDCT and VR study was helpful in evaluating the findings of NF-1 in 24 of 36 patients for a total of 40 findings. This included the following categories A) (n = 12), B) (n = 10), C) (n = 3), and D) (n = 15). When the VR images were compared to the orthogonal MDCT, the VR images was rated as helpful in 41 of 73 patients for a total of 60 findings, including the following categories: A) (n = 11), B) (n = 24), C) (n = 0), and D) (n = 25)., Conclusion: MDCT has distinct advantages over plain radiographs and MR imaging, and the VR images over MDCT in the evaluation of the spine in patients with NF-1, especially for the assessment of bony abnormalities, abnormal spinal curvature, and spinal instrumentation.

Purpose: Tumors that recur following orbital exenteration may not be evident on clinical examination, highlighting the need for imaging surveillance. The goal of this study was to report the imaging characteristics of recurrent tumors following orbital exenteration and free flap reconstruction., Methods: The authors retrospectively reviewed the records of 48 patients who underwent orbital exenteration for the treatment of orbital malignancy and identified 17 recurrent tumors in 17 patients. The lesions were assessed for the presence of a soft tissue mass, imaging characteristics, and fluorodeoxyglucose avidity., Results: The recurrent tumors were detected 1 month to 6 years 10 months (median, 1 year 3 month) after orbital exenteration. On both CT and MRI, all 17 lesions were soft tissue masses at presentation. On CT, the lesions demonstrated heterogeneous to homogeneous to centrally necrotic enhancement; on MRI, the lesions were T1 hypointense to isointense and T2 hypointense to hyperintense. Twelve of the 15 recurrent tumors with available preoperative imaging had an enhancing appearance similar to that of the original tumor. Thirteen of the 17 recurrent tumors were at the margin of a flap placed for reconstruction; the other 4 lesions were remote from the operative site., Conclusion: Recurrent tumors following orbital exenteration and free flap reconstruction demonstrate a wide range of imaging appearances but most often appear as a soft tissue masses often similar in appearance to the primary tumor and arising near the flap margin. Awareness of the imaging features of recurrent disease is important because failure to diagnose recurrence can delay appropriate treatment.

Purpose: To facilitate detection of tumor recurrence, the authors reviewed the MRI characteristics of myocutaneous and fasciocutaneous free flaps following orbital exenteration for treatment of orbital or maxillofacial tumors., Methods: The authors retrospectively reviewed the MRI characteristics, including T1 and T2 signal intensity, and enhancement pattern of 28 such flaps., Results: The study included 17 myocutaneous flaps and 11 fasciocutaneous flaps placed in 28 patients. For 23 flaps, additional imaging was performed after baseline imaging (range, 2-65 months after surgery). On precontrast T1 imaging, 15 of 17 myocutaneous flaps demonstrated a striated appearance similar to that of native muscle. Twenty-six of the 28 flaps in the series were T2 hyperintense. On baseline imaging, 26 flaps showed linear (n = 5), patchy (n = 10), or homogeneous (n = 11) enhancement. No flaps demonstrated mass-like enhancement. Five fasciocutaneous and 5 myocutaneous flaps showed decreased enhancement on follow-up imaging, while 4 myocutaneous flaps showed increased enhancement. Fourteen patients received postoperative radiation, 4 of which demonstrated increased enhancement, which subsequently decreased in 3 flaps. Fourteen of 23 followed flaps became smaller over time., Conclusions: On MRI, both myocutaneous and fasciocutaneous flaps placed after orbital exenteration generally demonstrate persistent non-mass-like enhancement and T2 hyperintensity, and both types of flaps may become smaller over time. Head and neck radiologists, ophthalmologic and plastic surgeons, and oncologists should be aware of the range of imaging features of these flaps to avoid misinterpreting the postoperative appearance as tumor recurrence.

Abstract The objective of this study was to assess the value of magnetic resonance imaging (MRI) of the orbit for conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma. The yield of other staging tests at baseline were also evaluated. Twenty-one consecutive patients treated for conjunctival MALT lymphoma were retrospectively studied. Lymphoma was staged according to both the Ann Arbor system and the seventh edition of the AJCC [American Joint Committee on Cancer] cancer staging manual. Findings on MRI of the orbit, whole-body positron emission tomography/computed tomography (PET/CT), CT of the chest/abdomen/pelvis, bone marrow (BM) biopsy and gastrointestinal (GI) endoscopy were recorded. Seventeen patients had orbital MRI. Fourteen of 17 patients (82%) with obvious conjunctival MALT lymphoma on clinical examination had a negative MRI scan. Only three patients had subtle conjunctival enhancement on orbital MRI. Ann Arbor stage at presentation was as follows: stage IE (15 patients), stage IIE (two patients) and stage IV (four patients). Eighteen of 21 patients had total-body PET/CT; four patients (22%) had hypermetabolic activity evident on PET scan. All 21 patients had bilateral BM biopsies. Fifteen of 21 patients (71%) had GI endoscopy. None of the patients had a positive BM biopsy or findings on GI endoscopy. Our data suggest that orbital MRI has a very low yield for identification of conjunctival MALT lymphoma. Clinical examination is critical in diagnosing and assessing treatment response in conjunctival MALT lymphoma. The yield for GI endoscopy and BM biopsy may also be low in staging of conjunctival MALT lymphoma.

Background and Purpose: Dermatofibrosarcoma protuberans is a rare, locally aggressive sarcoma of the skin in children and adults, usually involving the trunk and extremities and less commonly the head and neck. Despite clinical reports in the literature on the management of dermatofibrosarcoma protuberans, there are limited articles describing its imaging features., Materials and Methods: We retrospectively reviewed the demographics and imaging findings in all 24 patients with pathologically proven dermatofibrosarcoma protuberans of the head and neck seen at a tertiary cancer center between 2001 and 2010., Results: Twenty-two of the 24 lesions were nodular and well circumscribed; 19 of the 24 were located on the scalp. On imaging, all 24 lesions involved subcutaneous tissues. The lesions ranged in size from 0.6-9.5 cm (mean, 3.7 cm; standard deviation, 2.3 cm). Twelve lesions involved the soft tissues either at or extending directly to the midline. Thirteen lesions were associated with bulging of the skin surface. Fourteen lesions were imaged with CT and 14 with MR imaging. Whereas variable enhancement patterns were noted on CT and MR imaging, dermatofibrosarcoma protuberans was usually T2-hyperintense and demonstrated marked enhancement. None of the lesions was associated with bone invasion, perineural spread, or nodal/distant metastasis., Conclusions: Knowledge of the imaging characteristics of dermatofibrosarcoma protuberans may alert neuroradiologists to include dermatofibrosarcoma protuberans in the differential diagnosis of lesions about the head and neck with similar imaging characteristics.

Background: We performed this study to define the incidence of radiographic retropharyngeal lymph node (RPLN) involvement in oropharyngeal cancer (OPC) and its impact on clinical outcomes, neither of which has been well established to date., Methods: Our departmental database was queried for patients irradiated for OPC between 2001 and 2007. Analyzable patients were those with imaging data available for review to determine radiographic RPLN status. Demographic, clinical, and outcome data were retrieved and analyzed., Results: The cohort consisted of 981 patients. The median follow-up was 69 months. The base of the tongue (47%) and the tonsil (46%) were the most common primary sites. The majority of patients had stage T1 to T2 primary tumors (64%), and 94% had stage 3 to 4B disease. Intensity-modulated radiation therapy was used in 77% of patients, and systemic therapy was administered in 58% of patients. The incidence of radiographic RPLN involvement was 10% and was highest for the pharyngeal wall (23%) and lowest for the base of the tongue (6%). RPLN adenopathy correlated with several patient and tumor factors. RPLN involvement was associated with poorer 5-year outcomes on univariate analysis (P<.001 for all) for local control (79% vs 92%), nodal control (80% vs 93%), recurrence-free survival (51% vs 81%), distant metastases-free survival (66% vs 89%), and overall survival (52% vs 82%) and maintained significance on multivariate analysis for local control (P = .023), recurrence-free survival (P = .001), distant metastases-free survival (P = .003), and overall survival (P = .001)., Conclusions: In this cohort of nearly 1000 patients investigating [corrected] radiographic RPLN adenopathy in OPC, RPLN involvement was observed in 10% of patients and portends [corrected] a negative influence on disease recurrence, distant relapse, and survival. In this cohort of nearly 1000 patients investigating radiographic RPLN adenopathy in OPC, RPLN involvement was observed in 10% of patients and portends a negative influence on disease recurrence, distant relapse, and survival., (Copyright © 2013 American Cancer Society.)

Diffusion tensor imaging (DTI) data has traditionally been displayed as a grayscale functional anisotropy map (GSFM) or color coded orientation map (CCOM). These methods use black and white or color with intensity values to map the complex multidimensional DTI data to a two-dimensional image. Alternative visualization techniques, such as V max maps utilize enhanced graphical representation of the principal eigenvector by means of a headless arrow on regular nonstereoscopic (VM) or stereoscopic display (VMS). A survey of clinical utility of patients with intracranial neoplasms was carried out by 8 neuroradiologists using traditional and nontraditional methods of DTI display. Pairwise comparison studies of 5 intracranial neoplasms were performed with a structured questionnaire comparing GSFM, CCOM, VM, and VMS. Six of 8 neuroradiologists favored V max maps over traditional methods of display (GSFM and CCOM). When comparing the stereoscopic (VMS) and the non-stereoscopic (VM) modes, 4 favored VMS, 2 favored VM, and 2 had no preference. In conclusion, processing and visualizing DTI data stereoscopically is technically feasible. An initial survey of users indicated that V max based display methodology with or without stereoscopic visualization seems to be preferred over traditional methods to display DTI data.

Intricate resection and complex reconstructive procedures often required for primary and metastatic orbital tumors are facilitated by accurate imaging. A three-dimensional (3D) image can be reconstructed from source axial multidetector computed tomography (MDCT) images to visualize orbital tumors. To assess the utility of 3D images in this setting, the 3D images were reconstructed retrospectively for 20 patients with an orbital tumor and compared to two-dimensional (2D) orthogonal MDCT studies. Both types of images were assessed for their capacity to show the bony orbital walls and foramina, extraocular muscles, and optic nerve in the orbit contralateral to the tumor and, in the affected orbit, the extent of the tumor and its relationship to normal orbital contents and associated bone destruction. 3D imaging is most informative when axial images are acquired at 1.25 mm collimation. The optic nerve, extraocular muscles, and well-circumscribed orbital tumors were well visualized on 3D images. On 3D imaging, tumor-associated destruction of the lateral and superior orbital walls was fairly well demonstrated and that of the inferior and medial walls was not. The 3D images provide the surgeon with a comprehensive view of well-circumscribed orbital tumors and its relationship to extraocular muscles, exiting foramina, and the superior and lateral walls.

Cross-sectional imaging plays an important role in the evaluation of the retropharyngeal space (RPS) and the prevertebral space (PVS). Because of their deep location within the neck, lesions arising within these spaces are difficult, if not impossible, to evaluate on clinical examination. This article details the cross-sectional anatomy and imaging appearances of primary and secondary diseases involving the RPS and PVS, including metastasis and spread from adjacent spaces. The role of image-guided biopsy is also discussed., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background. Abnormal spinal curvature is routinely assessed with plain radiographs, MDCT, and MRI. MDCT can provide two-dimensional (2-D) orthogonal as well as reconstructed three-dimensional volume-rendered (3-D VR) images of the spine, including the translucent display: a computer-generated image set that enables the visualization of surgical instrumentation through bony structures. We hypothesized that the 3-D VR series provides additional information beyond that of 2-D orthogonal MDCT in the evaluation of abnormal spinal curvature in patients evaluated at a major cancer center. Methods. The 3-D VR series, including the translucent display, was compared to 2-D orthogonal MDCT studies in patients with an abnormal spinal curvature greater than 25 degrees and scored as being not helpful (0) or helpful (1) in 3 categories: spinal curvature; bony definition; additional findings (mass lesions, fractures, and instrumentation). Results. In 38 of 48 (79.2%) patients assessed, the 3-D VR series were scored as helpful in 63 of 144 (43.8%) total possible categories (32 spinal curvature; 14 bony definition; 17 additional findings). Conclusion. Three-dimensional MDCT images, including the translucent display, are complementary to multiplanar 2-D orthogonal MCDT in the evaluation of abnormal spinal curvature in patients treated at a major cancer center.

Sarcomas developing after radiation treatment for primary malignancies of the head and neck are often detected clinically when they are very aggressive. We reviewed the patient demographics and imaging findings in 21 patients with radiation-associated sarcomas (RAS) of the head and neck treated at our institution. Twenty-one RAS of the head and neck were retrospectively reviewed. The lesions were assessed for presence of a soft tissue mass, enhancement pattern, bone destruction, characteristics of tumor matrix, and FDG avidity. The RAS developed 4.5-25 years (mean 12.7 years) after irradiation. On both CT and MRI, all 21 lesions presented with a soft tissue mass. A variable imaging appearance was noted on CT, MR, and PET/CT, most, but not all, demonstrated aggressive features. At a median follow-up time of 19.1 months, 11 of the 21 patients had died from the sarcoma. RAS have variable imaging appearances. While most demonstrate aggressive features, some appear benign, which can lead to misdiagnosis. Head and neck radiologists, surgeons and oncologists who manage patients after radiation treatment should be aware of the wide range of clinical presentations and imaging features of RAS, because failure to diagnose can delay appropriate treatment., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Background and Purpose: ECD is a rare non-Langerhans-cell histiocytosis, which can involve the CNS; therefore, CNS imaging findings have been described in only a small number of patients. To gain additional insight into the CNS manifestations of ECD, we reviewed the findings on imaging of the brain, head and neck, and spine in patients with ECD who presented to our institution. Here, we illustrate manifestations that have not, to our knowledge, been previously described., Materials and Methods: CT, MR imaging, and PET/CT studies of the brain, maxillofacial region, and spine were reviewed in 11 patients with ECD., Results: Four new manifestations of ECD were present, including the following: a stellate appearance of intracranial extra-axial lesions, ependymal enhancement along the lateral ventricle with deep linear extension to the lentiform nucleus, irregular enhancement in the pons, and diffuse involvement of the vertebral column on PET/CT., Conclusions: ECD has a variety of imaging appearances in the CNS, including new manifestations described herein. Neuroradiologists should be aware of these manifestations to avoid mistaking them for other disease processes.