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Leiomyoma of the bladder is an unusual cause of benign smooth muscle tumor, only reported about 250 times in the literature. Imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI) scans are useful in defining the extent and location of the tumor, however, immunohistology samples must be obtained to rule out similar appearing cancerous masses. In this article, we report the case of a 50-year-old male diagnosed with leiomyoma of the bladder that initially presented as dysuria and raised suspicion of a bladder transitional cell carcinoma (TCC). We focus on the utilization of radiological imaging modalities for diagnosis, management, and preoperational planning with pathological findings for an optimized diagnosis.

Endometrioid carcinoma is the second most common ovarian tumor, classified as an epithelial-stromal ovarian tumor, and is usually characterized by a cystic tumor with partial solid components on magnetic resonance (MR) images. In this case report, we discuss an 81-year-old female who presented with atypical genital bleeding and distended abdomen, for which she underwent abdominal computed tomography and MR imaging. Solid endometrioid carcinoma of the ovary is very rare but was confirmed in our patient during the histological examination after surgery.

Background: The bacteria, Achromobacter are present ubiquitously in the moist environment. Infections due to Achromobacter have mostly been reported in immunocompromised patients. However, the microorganism has also been associated with nosocomial infections even in healthy subjects. Report: We report chronic osteomyelitis caused by Achromobacter denitrificans in a 44-year-old male with a history of femoral intramedullary nail in situ. The organism was confirmed by matrix assisted laser desorption ionization-time of flight mass spectrometry (MADI-TOF MS). Clinical management: The patient was managed with implant removal, surgical debridement and culture-directed antibiotic therapy that led to clinical cure. Conclusion: Identification of implant associated infection poses a serious challenge to surgeons and clinical microbiologists. Atypical pathogens must always be considered with a history of any implant in a case of chronic osteomyelitis.

Background/Objective: Olanzapine is a second-generation antipsychotic medication with increased side effects of weight gain, hyperglycemia, and insulin resistance. Here we describe a case of diabetic ketoacidosis in a patient who started taking olanzapine 12 weeks before she presented. Case Report: A 73-year-old African-American female presented with a 1-week history of confusion, polyuria, and polydipsia. Her past medical history included type 2 diabetes mellitus, hyperlipidemia, and severe depression with psychotic features. Her medications were olanzapine 5 mg, duloxetine 90 mg, and rosuvastatin 5 mg daily. Three weeks prior, she was diagnosed with COVID-19 and treated for a urinary tract infection. Her physical exam upon admission included severely dry mucous membranes and labored respirations. The circulating glucose was 748 mg/dL (70-110), anion gap 39 mmol/L (7-16), and hemoglobin A1c (HgbA1c) 11.8% (105 mmol/mol). Three months prior, her HgbA1c was 6.7% (50 mmol/mol). She was treated with intravenous fluids and continuous insulin infusion followed by subcutaneous basal-bolus glargine and lispro after an anion gap of 13 mmol/L (7-16) was obtained. Two weeks into her hospitalization, olanzapine was discontinued. She was discharged on 10 units of glargine and metformin 500 mg twice daily. Five months after discharge, she indicated not taking any of the prescribed insulin or metformin. At this follow-up, her HgbA1c was 6.7%. Discussion: Olanzapine may impair insulin secretion by causing pancreatic beta-cell apoptosis. Conclusion: Increased awareness of the generalized metabolic effects and risk of diabetic ketoacidosis associated with antipsychotic medications is needed to develop a safe treatment plan for patients.

Dravet syndrome (DS) is characterized by recurrent convulsive seizures, including status epilepticus, and intellectual disability as a comorbidity. Seizures associated with DS are commonly resistant to antiseizure medications. Typical features of DS are recurrent episodes of status epilepticus, the presence of genetic mutations, and no abnormal magnetic resonance imaging (MRI) findings. Here, we report a rare case of DS in a 14-year-old girl who was negative for genetic mutations, had experienced status epilepticus only once, and had abnormal findings on brain MRI. Although our patient’s case features are atypical of DS, they do not contradict the diagnostic criteria. Despite the difficulty in diagnosing DS because of the negative genetic testing results, we started our patient on fenfluramine (FFA). Long-term treatment with low-dose FFA effectively controlled our patient’s seizures and resulted in cognitive and functional improvements.

Introduction: Bisphosphonates are commonly used to prevent osteoporotic fractures. Many randomized controlled trials have proved the efficacy of bisphosphonates, showing their ability to increase bone mineral density and decrease the risk of hip and vertebral fractures. Atypical, bisphosphonate-related fractures concerning the femur have been widely described and a list of primary and secondary clinical and radiographic criteria are used in order to achieve diagnosis. Research question: To identify clinical and radiographic findings for a possible association of vertebral fractures to prolonged bisphosphonate therapy. Material and methods: In this paper we present a case of an atraumatic bilateral pedicle lumbar fracture, assuming the hypothesis of an insufficiency atypical fracture due to prolonged Alendronate therapy for osteoporosis. We highlight the various aspects for diagnosis, treatment and a review of the existing literature was carried out. Discussion and conclusion: Atypical fractures in the spine are rarely reported in the literature. Pedicle anatomy is partially similar to a long bone, described as a strong bridge between the posterior arch and the vertebral body composed of a cortical bone and a cancellous core. Stress fractures are reported as a result of an underlying bone disease, trauma, post-surgery and stress fractures. In our opinion, the criteria described for atypical femoral fractures are a useful tool to also classify these rare pedicle lesions as insufficiency, bisphosphonate-correlated fractures.

Posterior reversible encephalopathy syndrome (PRES) is an uncommon, but important, pathology affecting primarily the posterior cerebral circulation. Typical imaging features include vasogenic edema involving the bilateral occipital and parietal lobes. We report 4 cases of atypical PRES: The first one is a 59-year-old woman with a medical history of diabetes and hypertension who presented a consciousness disorder. MRI of this patient revealed signal abnormalities in the thalamus, the cerebellum, the brainstem and the corpus callosum with microbleeds and vascularization disorders. The second patient is a 41-year-old woman admitted for right hemiparesis and facial paralysis. Physical examination found a hypertension and the atypical imaging features on MRI were low ADC values with a pearl necklace appearance on ARM sequences. The third patient is a 32-year-old woman who undergone a surgery for an ectopic pregnancy and went in a coma for 8 days. Physical and biological parameters were normal. Diagnosis was delayed because of atypical signal abnormalities in caudal and lentiform nuclei that worsened on follow-up MRI, resulting in a dramatic evolution to bilateral cecity and psychosis. The last case is a 34-year-old woman with terminal renal failure hospitalized for spontaneously resolving tonic-clonic seizures. Biology showed high uremia and brain MRI revealed a gyriform enhancement at gadolinium injection with unilateral putamen lesion. The patient improved after blood purification. Knowledge of these atypical findings can help the radiologist make an accurate, timely diagnosis, and improve patient care.

Background and Objective: Meningiomas are non-malignant neoplasms primarily originating from arachnoid cells and are classified into three grades [1, 2, and 3] based on histological features according to the WHO classification. However, this classification system is imperfect especially for grade 1 and 2 meningiomas as many grade 1 tumors recur. Meningiomas are hence a histologically diverse class of tumors exhibiting more unpredictable behaviour. Therefore, more improved classification is required, possibly using novel and more dependable biomarkers. In this study, we aim to investigate the role of the H3K27me3 and Ki67 labelling index in assessing the biological behaviour of meningiomas. The study was conceived, with the primary objective of examining the expression of H3k27me3 and Ki67 labelling index in grade 1/2 meningiomas with atypical features to ascertain if this potentially impacts patient prognosis., Methodology: Upon obtaining clearance from the Institutional Ethical Committee (IEC), the authors studied 81 cases of meningiomas including 11 recurrent cases. The study used immunohistochemistry to evaluate the Ki67 index and H3K27me3 immunohistochemistry. The Ki67 labelling Index was determined by counting the positively stained MIB-1 cells and categorizing them into <5%, 5-10% and > 10%. The H3k27me3 staining was evaluated by finding the product of the tumor cells showing positive staining and the intensity of staining. Based on the product of the two, the cases were subdivided into Negative [0], Low [1-4] and high expression [5-9] of H3K27me3., Results: The results showed that presence of atypical morphological features including necrosis and prominent nucleoli in grade 1 meningioma and low expression of H3K27me3 was significantly associated with higher grade, recurrence, and shorter progression-free survival (Kaplan Meier curves showed higher negative slope). The study also found that a higher Ki67 labelling Index was associated with recurrence and poor prognosis. This suggest that the H3K27me3 and Ki67 labelling Index can be useful prognostic markers in meningiomas, particularly in challenging grade 1 and 2 cases and recurrent meningiomas., Conclusion: The study highlights the importance of the H3K27me3 and Ki67 Labelling Index in assessing the biological behaviour of meningiomas. The findings provide valuable insights into the prognosis and treatment of meningiomas, emphasizing the need for further research to validate these markers and develop targeted therapeutic strategies., (Copyright © 2024. Published by Elsevier Inc.)

Leiomyoma of the bladder is an unusual cause of benign smooth muscle tumor, only reported about 250 times in the literature. Imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI) scans are useful in defining the extent and location of the tumor, however, immunohistology samples must be obtained to rule out similar appearing cancerous masses. In this article, we report the case of a 50-year-old male diagnosed with leiomyoma of the bladder that initially presented as dysuria and raised suspicion of a bladder transitional cell carcinoma (TCC). We focus on the utilization of radiological imaging modalities for diagnosis, management, and preoperational planning with pathological findings for an optimized diagnosis., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Esophageal granular cell tumors (GCTs), or also called Abrikossoff's tumor are rare benign tumors originating from Schwann cells most commonly found in the skin, subcutaneous tissue, and tongue. Approximately 5% –8% arise in the gastrointestinal tract, and one-third of these tumors arise in the esophagus [1]. This neoplasm has a benign behavior usually, but there have been described a malignant transformation in 2% –3% of the cases. Herein, we discuss a case of a 70-year-old male patient with no pathological background, admitted for dysphagia evolving in 3 months that was explored with endoscopy and CT, the diagnosis at this level was challenging but the histopathology and Immunohistochemistry confirmed the presence of granular cells thus confirm the diagnosis.The purpose of our work is to report the uncommon evolution of an Abrikossoff ‘s tumor located in the esophagus, as a warning of the possible malignant transformation of this tumor mostly benign; also we made a review of the literature.

Adenoid cystic carcinoma, also known as cylindroma, is one of the rare and unexplored clinical presentations of lung cancer, for which existing knowledge is scarce. This case report discusses a presentation of this tumor in the right lung, which subsequently extended to the left atrium through the right superior pulmonary vein. The extension of this rare tumor into the left atrium makes this case both uniquely distinctive and clinically relevant. The management strategy opted for this case was a right posterolateral thoracotomy and right pneumonectomy with partial resection of the left atrium. The desired outcome of this report is to shed light on the unusual clinical pathophysiology, register its atypical extensions, and navigate surgeons who may encounter this manifestation in the future.

Pityriasis rosea is a common, self-limited disease which in its typical form should not raise diagnostic doubts. Nevertheless, its atypical presentations can be a challenge for the clinician, and hence it is important to be aware of them. Recognition of these forms may enable a correct diagnosis to be made and avoid unnecessary procedures. We present two patients for whom we believe atypical PR was the most likely the diagnosis., Competing Interests: Declaration of competing interest The authors declare there is no conflict of interests., (Copyright © 2024 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Serous fluids offer crucial diagnostic insights, but inconsistent analysis hampers reporting quality, especially in indeterminate (ID) categories like atypia of undetermined significance (AUS) and suspicious for malignancy (SFM). The 2020 International System for reporting Serous Fluid Cytopathology (TIS) aims to standardize communication and reduce reporting disparities. This study evaluates TIS's role in AUS and SFM categories within our institution., Materials and Methods: A 4-year retrospective search of cytopathology reports from December 2015 to December 2019 for AUS and SFM diagnoses in pleural, ascitic, pericardial fluids, and peritoneal washings was performed and results reclassified using TIS definitions. The risk of malignancy (ROM) was calculated for existing and reclassified diagnoses., Results: Over 4 years, we received 2998 serous fluid specimens. AUS constituted 2.3% (70 cases), while SFM constituted 0.5% (16 cases). Excluding repeats, 80 cases were TIS-reviewed. Sixteen cases of ID diagnoses were reclassified. Two cases of AUS were changed to negative for malignancy (NFM) and 12 to SFM. Two SFM cases were upgraded to malignancy. ROM shifted from 63% to 60% for AUS and 100% to 85% for SF (TIS's ROM range: AUS: 66% ± 10%; SFM: 82% ± 4.8%)., Conclusions: This institution's ID diagnosis rate is low. AUS ROM is challenging but aligns with TIS, primarily favoring benign. All SFM diagnoses are highly suspicious but quantitatively inadequate for definitive malignancy, explaining the elevated ROM. AUS rate should gauge quality, not serve as a catch-all category. Algorithmic cytology with cell blocks and ancillary studies aids reclassification. TIS is user-friendly and is a consistent methodology for standardized reporting. Further studies are needed to evaluate ROM and define reproducible diagnostic criteria for each category for better system utilization., (Published by Elsevier Inc.)

The most common manifestations of the 2019 novel coronavirus disease (COVID-19) include fever, cough, dyspnea. Nevertheless, many atypical forms of presentation might be present, delaying a correct diagnosis.Acute kidney injury (AKI) is one of the important complications of COVID-19, occurring in 0.5–7% of cases and in 2.9–23% of ICU patients. The exact mechanisms by which COVID-19 induces AKI in different clinical settings is still a matter of debate.We present the case of a 53-year old woman, without any prior renal pathology, admitted to a Cardiology Department for atypical thoracic pain and oligo-anuria, without respiratory symptoms, who was diagnosed with SARS-CoV-2 infection. The patient had a significant rise in high-sensitivity cardiac troponin (from 304 ng/L to 889 ng/L in one hour) and mild systolic dysfunction (LVEF 45%), which led to the initial misdiagnosis of an acute myocardial infarction. Blood tests confirmed the diagnosis of acute kidney injury (creatinine 8.8 mg/dL in two different samples). She received hydro-electrolytic rebalancing treatment, with good clinical and biological evolution. To our knowledge this is one of the first reports, that highlights the existence of myocardial injury secondary to acute kidney injury caused by SARS-CoV-2 infection, in a patient without respiratory symptoms.

Background: Tumor infections are uncommon, and often misdiagnosed entity, with no pathognomonic signs or characteristic clinical presentation. A useful tool for its diagnostic approach is the magnetic resonance image. Treatment should include tumor removal plus infection management. Case description: We present a patient who arrives at the emergency department with rapid neurological deterioration with no specific signs and symptoms, radiological behaved as multiple brain abscess associated with subdural hematoma. In the operating room, we diagnosed a tumor with an associated abscess. Conclusions: It should be useful to consider in some scenarios, multiple alternative diagnoses when the clinical picture does not match with the classical presentation and exhaust the radiological armamentarium before planning a surgery when feasible. Also, we present the first case of an abscessed anaplastic (malignant) meningioma.

Insulinoma is a rare endocrine tumor that causes hypoglycemia, with incidence of only 1–4 cases per 1 million population. Various clinical manifestations of hypoglycemia may resemble neurological, psychiatric and cardiac problems, which result in diagnosis delay. In this case, we reported a 30-year-old woman experiencing deterioration of consciousness and history of repeated seizures, despite normal EEG, brain CT and MRI. Multiphase contrast-enhanced abdominal CT showed hypovascular nodule, which is not typical for insulinoma. Multiphase contrast-enhanced MRI showed hypointense T1WI, hyperintense T2WI pancreatic nodule which enhance in arterial phase. Laboratory and histopathological examination confirm the diagnosis of neuroendocrine tumor. We described the atypical clinical manifestations, as well as atypical imaging appearance of pancreatic insulinoma in this patient. Lesion characteristics and its differential diagnosis based on CT and MRI were discussed, with several features to help differentiating pancreatic insulinoma from adenocarcinoma in accordance with the current literature.

Purpose: To report the clinical and histopathological findings of atypical intravitreal growth of a retinoblastoma with a multi-branching configuration. Observations: A 7-month-old boy was referred to our hospital due to leukocoria in the right eye. Ophthalmic examinations identified multi-branching vessels surrounded by diaphanous tissue behind the lens in the right eye. Imaging modalities showed microphthalmos, band-shaped calcification, and cystic lesions in that eye. Because it was difficult to rule out congenital anomalies such as persistent fetal vasculature due to the atypical clinical features of retinoblastoma, we performed a biopsy using a limbal approach. An intraoperative rapid pathological examination led to the definitive diagnosis of retinoblastoma. The right eye was enucleated and postoperative adjuvant chemotherapy was administered. Immunohistochemical staining of the enucleated eyeball showed that the tumoral cells and diaphanous tumoral tissue around the vessels were positive for neuron-specific enolase and Ki-67 and partially positive for glial fibrillary acidic protein (GFAP). The vessels of the diaphanous tissues near the tumoral mass were stained by GFAP and those behind the lens were stained faintly. Conclusions and importance: We described an atypical retinoblastoma of pseudo-persistent fetal vasculature with a multi-branching configuration, which expanded the clinical spectrum of retinoblastoma. Such a specific growth pattern of the embryonic tumor might occur with a combination of retinal development, retinal vasculature, and hyaloid vascular system.

We present a case of a bisphosphonate-related femur fracture in an elderly woman, who failed treatment with both cephalomedullary nail and proximal femoral locking plate, leading to successful treatment with total hip arthroplasty. Hardware failure should be included in the differential of patients with previous internal fixation of bisphosphonate-related femur fracture that present with hip or groin pain. Arthroplasty can be an acceptable salvage option in an active elderly patient with a bisphosphonate-related femur fracture.

Choroid plexus papillomas are highly vascular tumors, and such tumors causing subarachnoid hemorrhage have been reported in literature. Similarly, few articles have reported atypical fourth ventricular choroid plexus tumors in adults. However, such an atypical tumor presenting with grossly hemorrhagic transformation without any acute symptoms could not be found in the literature., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Globally, there are more than 25 licensed antipsychotic medications. Antipsychotics are commonly described as either typical or atypical, but this dichotomous classification does not reflect the diversity of their pharmacological and clinical profiles. There is a need for a data-driven antipsychotic classification scheme suitable for clinicians and researchers that maps onto both pharmacological and clinical effects. Receptor affinity provides one starting point for such a scheme., Methods: We analyzed affinities of 27 antipsychotics for 42 receptors from 3325 in vitro receptor binding studies. We used a clustering algorithm to group antipsychotics based on receptor affinity. Using a machine learning model, we examined the ability of this grouping to predict antipsychotic-induced clinical effects quantified according to an umbrella review of clinical trial and treatment guideline data., Results: Clustering resulted in 4 groups of antipsychotics. The predominant receptor affinity and clinical effect "fingerprints" of these 4 groups were defined as follows: group 1, muscarinic (M 2 -M 5 ) receptor antagonism (cholinergic and metabolic side effects); group 2, dopamine (D 2 ) partial agonism and adrenergic antagonism (overall low side-effect burden); group 3, serotonergic and dopaminergic antagonism (overall moderate side-effect burden); and group 4, dopaminergic antagonism (extrapyramidal side effects and hyperprolactinemia). Groups 1 and 4 were more efficacious than groups 2 and 3. The classification was shown to predict out-of-sample clinical effects of individual drugs., Conclusions: A receptor affinity-based grouping not only reflects compound pharmacology but also detects meaningful clinical differences. This approach has the potential to benefit both patients and researchers by guiding treatment and informing drug development., (Copyright © 2023 Society of Biological Psychiatry. Published by Elsevier Inc. All rights reserved.)

The most common organisms causing prosthetic knee joint infections are staphylococci. However, arthroplasty infections with atypical microbial pathogens, such as Mycobacteria can occur. Due to the rarity of mycobacterial prosthetic joint infections, diagnosis, treatment, and management of these atypical infections represent a clinical challenge. A 71-year old female post-operative day 40 after a left total knee arthroplasty was hospitalized secondary to left knee pain and suspected arthroplasty infection. She had failed outpatient oral antimicrobial treatment for superficial stitch abscess; and outpatient IV/Oral antimicrobials for a clinical postoperative septic bursitis. Ultimately, resection arthroplasty with operative tissue acid fast bacterial cultures demonstrated growth of the Mycobacterium smegmatis group. Post-operatively, she completed a combination course of oral doxycycline and levofloxacin and successfully completed a replacement arthroplasty with clinical and microbial resolution of the infection. To our knowledge, literature review demonstrates three case of knee arthroplasty infection caused by the Mycobacterium smegmatis group. Correspondingly, optimal surgical procedures and antimicrobial management including antimicrobial selection, treatment duration are not well defined. Presently, the best treatment options consists of two step surgical management including prosthesis hardware removal followed by extended antimicrobial therapy, followed by consideration for re-implantation arthroplasty. Our case illustrates importance of considering atypical mycobacterial infections in post-operative arthroplasty infections not responding to traditional surgical manipulations and antimicrobials. For an arthroplasty infection involving the atypical Mycobacterium smegmatis group, two step arthroplasty revision, including arthroplasty resection, with a combination of oral doxycycline and levofloxacin can lead to successful infection resolution, allowing for a successful replacement arthroplasty.

Purpose: Women with breast cancer are at increased risk for femur fracture. Contributing factors include estrogen deficiency, cancer-related therapies, or direct bone involvement. This study examines fracture subtypes in women with prior breast cancer experiencing a femur fracture. Methods: Women age ≥50 years old with a history of invasive breast cancer who experienced a femur fracture were identified during 2005–2012. Fracture site was classified by hospital diagnosis (for hip) and/or radiologic findings (for femoral diaphysis), with subtype classification as pathologic, atypical or fragility fracture. Clinical characteristics were ascertained using health plan databases and disease registries. Results: There were 802 women with prior breast cancer who experienced a femur fracture. The mean age at fracture was 80.5±9.6 years, with most fractures (93.8%) occurring in the hip and only 6.2% in the femoral diaphysis. However, diaphyseal fractures accounted for 23.6% of fractures in younger women (age ≤65 years). Pathologic fractures comprised 9.6% of total fractures (56.0% of diaphyseal fractures) and accounted for half the fractures in younger women. An atypical fracture pattern was seen in 1% of all femur fractures and 16.0% of diaphyseal fractures, with prior bisphosphonate exposure in all atypical fracture cases. Conclusion: Most femur fractures in women with prior breast cancer occurred in the hip. Among younger women and those experiencing diaphyseal fractures, a larger proportion were pathologic and some were found to be atypical. Further studies should examine risk factors for femur fracture in women with breast cancer with specific attention to fracture subtype and pharmacologic exposures.

Purpose: Borderline prostatic lesions, with insufficient histomorphologic features, to be definitely diagnosed as prostatic adenocarcinoma (PCa) are often signed out as “atypical glands suspicious for carcinoma” or atypical small acinar proliferation (ASAP). These findings that eventually warrant either immunohistochemical (IHC) studies or a repeat biopsy, prove to be more burdensome to patients in developing countries (such as the Philippines), where health care is not as progressive nor is it an utmost priority. At the same time, in countries like the Philippines, there is a shortage of urological pathologists. Methods: In this study, we compared the transrectal ultrasound-guided prostate (TRUS) biopsies signed out by general surgical pathologists in St. Luke's Medical Center Quezon City from 2008–2010, and the TRUS Biopsies primarily signed out by a urologic pathologist in both St. Luke's Medical Center Quezon City and Global City from July 2013 to July 2014, and from September 2013 to July 2014, respectively. Results: From 2008 to 2010, 30.6% (129 of 421) of the cases were signed out as atypical. Of these, 79 underwent IHC staining, 21 (26.6%) of which were eventually signed out as PCa. Compared to those signed out in 2013 to 2014 by our genitourinary pathologist, only 16.6% (39 of 235) of the cases were signed out as atypical. Of these, 16 underwent IHC staining, with 15 (93%) of them being definitively diagnosed as PCa. Among the 21 cases wherein a repeat biopsy was recommended, only three followed and two of these had findings of PCa on repeat biopsy. Looking at our 16.6% rate of atypicals and subtracting those that were eventually established as PCa after IHCs, our atypicals would be down to 10% (24/235) in 2013–2014 compared to 25.7% (108/421) in 2008–2010. Conclusions: These results highlight the critical role a specialist has in the field of urological pathology, especially in developing countries. It is in the diagnosis of PCa in needle biopsies that a urological pathologist impacts the use of an atypical diagnosis, by ensuring its judicious use. This ultimately benefits the patients, by lessening unwarranted expenses through the decreased dependence on IHC staining and if necessary, a repeat biopsy.

Intestinal pseudo-obstruction in atypical Kawasaki disease (KD) is rare. A boy aged 2 years and 6 months presented with a 7-day history of fever, coffee-ground vomit, and abdominal pain. Abdominal radiography and ultrasound showed a dilated duodenum. Peeling of the skin on his fingers and toes developed on hospitalization day 9. Echocardiogram revealed right and left coronary artery dilatation compatible with KD. He was treated with 2 g/kg intravenous immunoglobulin (IVIG), with rapid resolution of fever and relief of abdominal pain. Follow-up abdominal radiography and ultrasound showed improvement of bowel dilatation. This case illustrates that atypical KD can present with intestinal pseudo-obstruction. A high index of suspicion is required for early diagnosis, and prompt treatment with IVIG is recommended.

Atypical anti-glomerular basement membrane (anti-GBM) disease is characterized by linear immunoglobulin G (IgG) deposition along the GBM without circulating IgG anti-GBM antibodies. Compared to classic anti-GBM disease, atypical anti-GBM disease tends to be milder with a more indolent course in certain cases. Moreover, pathologic disease pattern is much more heterogenous in atypical anti-GBM disease than in the classic type, which is uniformly characterized by diffuse crescentic and necrotizing glomerulonephritis. Although there is no single well-established target antigen in atypical anti-GBM disease, the target antigen (within the GBM) and the autoantibody type are hypothesized to be different from the classic type. Some patients have the same antigen as the Goodpasture antigen that are detected only by a highly sensitive technique (biosensor analysis). Some cases of atypical anti-GBM disease have autoantibodies of a different subclass restriction like IgG4, or of monoclonal nature. Antibodies targeting antigen/epitope structure other than the Goodpasture antigen can be detected using modified assays in some cases. Patients with IgA- and IgM-mediated anti-GBM disease are known to have negative circulating antibodies because conventional assays do not detect these classes of antibodies. A significant proportion of cases with atypical anti-GBM disease do not have any identifiable antibodies despite extensive evaluation. Nevertheless, extensive evaluation of atypical autoantibodies using modified assays and sensitive techniques should be attempted, if feasible. This review summarizes the recent literature on atypical anti-GBM disease., (© 2023 International Society of Nephrology. Published by Elsevier Inc.)

Objective: Anxiety is a prominent concern in Parkinson's disease (PD) that negatively impacts quality of life, increases functional disability, and complicates clinical management. Atypical presentations of anxiety are under-recognized and inadequately treated in patients with PD, compromising global PD care., Methods: This systematic review focuses on the prevalence, symptomology and clinical correlates of atypical presentations of PD-related anxiety following PRISMA guidelines., Results: Of the 60 studies meeting inclusion criteria, 14 focused on 'Anxiety Not Otherwise Specified (NOS)' or equivalent, 31 reported on fluctuating anxiety symptoms, and 22 reported on 'Fear of Falling (FOF)'. Anxiety NOS accounted for a weighted mean prevalence of 14.9%, fluctuating anxiety for 34.19%, and FOF for 51.5%. These latter two exceeded the average reported overall prevalence rate of 31% for anxiety disorders in PD. We identified a diverse array of anxiety symptoms related to motor and non-motor symptoms of PD, to complications of PD medication (such as "on" and "off" fluctuations, or both), and, to a lesser extent, to cognitive symptoms., Conclusion: Atypical anxiety is common, clinically relevant, and heterogeneous in nature. A better understanding of the phenomenology, clinical course, and pathophysiology of varied forms of atypical anxiety in PD is needed to improve recognition, advance therapeutic development and ultimately optimize quality of life in PD., (Copyright © 2022. Published by Elsevier Inc.)

Ossifying fibromyxoid tumor (OFMT) is a rare mesenchymal neoplasm of uncertain line of differentiation that can be subdivided into typical, atypical, and malignant tumors. Cytogenetically, OFMT is characterized by recurrent gene rearrangement involving PHF1 in up to 85% of cases. The most common PHF1 fusion partner is EP400, present in approximately half of cases. Most recently, a novel fusion of PHF1-TFE3 was identified in about 10% of PHF1-rearranged OFMTs. Herein, we report a unique case of PHF1-TFE3 fusion atypical OFMT with prominent collagenous rosettes. A 50-year-old male patient presented with a slowly growing, painless mass in the right foot for 4 years. Gross examination showed a 3.5-cm, subcutaneous well-circumscribed, lobulated mass. Microscopic examination revealed a well-demarcated but un-encapsulated tumor without a peripheral bony shell. The neoplasm was composed of mildly atypical spindle to ovoid cells with increased mitosis (2 mitoses per 10 high-power fields) arranged in a multinodular manner within a fibromyxoid stroma, which contained numerous small, irregular collagenous rosettes surrounded by radiating growth of tumor cells. The neoplastic cells were diffusely positive for TFE3 and CD10. RNA sequencing revealed an in-frame fusion between PHF1 exon 12 and TFE3 exon 7. Subsequent Fluorescence in-situ hybridization analyses demonstrated positive for rearrangements of both the PHF1 and TFE3 loci. The patient was free of disease at 63 months' follow-up. Our case exhibits atypical features and prominent collagenous rosettes, expanding the morphological spectrum of OFMT with PHF1-TFE3 fusion., (Copyright © 2021 Elsevier Inc. All rights reserved.)