IntroductionSolitary fibrous tumors are rare neoplasms of mesenchymal origin. They are often of low malignant potential and rarely metastasize. While they frequently arise from the pleura, they can occur at any soft tissue site in the body. We present a case of a large (28 × 21cm) malignant solitary fibrous tumor arising from the bladder serosa. In addition, the clinicopathologic features, differential diagnosis, cytogenetics and management of this rare disease are discussed, along with a review of the existing literature on this topic.Case presentationAn otherwise healthy 41-year-old Caucasian man presented with weight loss and progressive abdominal bloating. A subsequent computed tomography scan of his chest, abdomen and pelvis revealed a 26.8 × 21cm intra-abdominal mass occupying most of his abdominal cavity. The inferior vena cava was compressed, and the mass extended inferiorly to his upper pelvis abutting the superior dome of his bladder. He underwent operative resection and the resected mass measured 28 × 21 × 18cm and weighed 4.8kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern, with randomly assorted tan fleshy nodules. A histologic evaluation revealed variable, alternating hypercellular and hypocellular areas, with areas of necrosis. The tumor cells varied from spindle to epithelioid within a hyalinized stroma. In the hypercellular areas, the tumor cells showed moderate atypia with high mitotic activity. The histological features combined with immunophenotyping were suggestive of a malignant solitary fibrous tumor that grossly appeared to be growing from the bladder serosa, specifically the intraperitoneal superior dome of the bladder. Our patient is currently eight months post-surgery without evidence of recurrence.ConclusionsExtrapleural occurrences of solitary fibrosis tumors are being increasingly observed. Malignant solitary fibrosis tumors of the urinary bladder, however, are very rare. As there are no pathognomonic features of malignancy, surgical resection is often both diagnostic and therapeutic, as was the case in our report.
A 51-year-old man with a history of stage IV angioimmunoblastic T-cell lymphoma was diagnosed with osteomyelitis of the patella. Legionella anisa was identified by 16S rRNA gene sequencing and culture. The patient had pneumonia 2 months prior to this osteomyelitis episode. L. anisa was retrospectively detected in his lung tissue by 16S rRNA gene sequencing and was considered the source of the L. anisa that caused his patella osteomyelitis. CASE REPORTA 51-year-old man with a history of stage IV angioimmunoblastic T-cell lymphoma presented to the emergency department of Hackensack University Medical Center (Hackensack, NJ) with a 3-week history of right knee pain. The pain was associated with a decreased range of motion of the knee. He denied any other signs or symptoms, including fever, chills, or trauma. Five months prior to the reported onset of knee pain, he was found to have recurrence of the lymphoma, for which he underwent a left-sided tonsillectomy and was treated with alemtuzumab (anti-CD52 monoclonal antibody) and lenalidomide. Subsequently, 2 months prior to his current admission, he was also hospitalized with multifocal pneumonia and underwent a bronchoscopy during which a transbronchial lung biopsy specimen was obtained. The latter showed bronchiolitis obliterans with organizing pneumonia, and he was started on oral prednisone. Routine cultures of blood, sputum, and lung tissue for bacteria, mycobacteria, and fungi were negative. He was empirically treated with vancomycin at 1 g every 12 h, ceftazidime at 2 g intravenously every 8 h, azithromycin at 500 mg intravenously every 24 h, and voriconazole at 200 mg per os every 12 h. He recovered clinically with resolution of his pulmonary infiltrates as determined by a computerized axial tomography scan of the chest.On physical examination, he was afebrile and thermodynamically stable. The right knee had no appreciable effusion but was tender over the anterior and lateral aspects of the patella, as well as the patellar tendon. The rest of his physical examination was unremarkable.Magnetic resonance imaging of the right knee revealed moderate marrow edema and enhancement involving the anterior aspect of the patella. An indium whole-body scan revealed intense uptake of activity in the right patella. The patient underwent debridement of the patella. Gram staining of the tissue demonstrated numerous white blood cells, red blood cells, and large numbers of Gram-negative rods. He was initially treated with imipenemcolistin at 500 mg intravenously every 6 h. After L. anisa was identified (see microbiology investigation), the patient was treated for 8 weeks with moxifloxacin at 400 mg intravenously daily and had an uneventful course.Microbiology investigation. A bone (patellar) biopsy specimen showed numerous extracellular and intracellular slender Gram-negative rods (Fig. 1). Routine aerobic and anaerobic cultures were negative. To rapidly identify the pathogen, DNA was extracted directly from the biopsy specimen with the MagneSiI Genomic Fix Tissue System (Prome...
Objectives Primary cutaneous clear cell sarcoma (PCS) is a rare malignancy and difficult to differentiate from melanoma. We investigated factors influencing survival and recurrence. Methods An institutional cancer registry and literature search were used for a retrospective study. Only clear cell sarcoma cases with a primary site of skin and subcutaneous tissue were included. Kaplan-Meier and Cox regression analyses were used to assess survival time and hazard ratios. Results Three eligible cases were identified at our institution. In addition, the PubMed and Google Scholar reviews identified 1,878 items, with 23 patients with PCS. The median age was 25 years with 62% female. The tumors ranged in size from 0.4 to 4.5 cm. Cytogenetics showed t(12;22)(q13;q12) in all cases and a unique variant of t(2;22)(q32.3;q12) in one case. Surgery was the most common treatment, followed by chemotherapy/radiation. PCS recurred in 46% of patients with a median relapse-free survival time of 15 months. Only two known PCS-related mortalities were recorded, at 38 and 60 months following initial diagnosis. Smaller tumor size and negative sentinel lymph node biopsy (SLNB) status were significantly associated with a better disease-free survival. Conclusions Tumor size and SLNB status influence PCS survival and recurrence. More research is needed due to the rarity of this disease.
Background Embryonal tumor with multilayered rosettes (ETMR) is a deadly grade IV pediatric brain tumor. Despite an intensive multimodal treatment approach that includes surgical resection, high‐dose chemotherapy, and radiotherapy, the progression‐free survival at 5 years is less than 30%. Case We report a case of long‐term survival in a 5‐month old female with a large mass in the posterior fossa, diagnosed as ETMR, which subsequently underwent treatment‐induced maturation. Prior to chemotherapy, histopathology revealed an abundance of highly proliferative, undifferentiated cells and multilayered rosette structures. Conversely, post‐treatment histopathology revealed cell populations that differentiated into neuronal and ganglionic phenotypes. At 5‐year follow‐up, the patient remains progression‐free. Conclusion This finding contributes to the few reports to date of post‐treatment differentiation/maturation of ETMR cell populations, with an implication for less cytotoxic therapeutic interventions aimed at differentiation.
Disorders of carbohydrate metabolism resulting in accumulation of polyols in body fluids were first described in the early 1990s by van der Knapp et al, but this diagnosis remains rare with very few patients reported with primary neurological phenotypes. We present a child with a presumed polyol disorder whose clinical course was similar to two of the original five patients and who had similar findings in quantification of urinary polyols. In addition, we document details of central nervous system pathology in this rare metabolic disorder. The basic pathology is that of a neurodegenerative disorder with hypomyelination leukodystrophy consequent to a defect of oligodendroglia and resultant axonal and neuronal loss.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.