Perivascular epithelioid cell tumors (PEComas) of the pancreas are extremely rare mesenchymal tumors and to our knowledge, only 17 cases have been reported in the English literature to date. We report our experience with a new case of primary pancreatic PEComa diagnosed preoperatively by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) complemented by tissue cell block and immunohistochemistry. The patient was a 54-year-old female whose chief complaint was intermittent severe right upper quadrant abdominal pain. Computed-tomography (CT) imaging revealed a mass between the head and the body of the pancreas. EUS-FNA smear preparation was obtained but was nondiagnostic. However, examination of the tissue cell block showed sheets of epithelioid cells with abundant eosinophilic cytoplasm and immunohistochemistry studies revealed positivity for both melanocytic (HMB-45 and Melan-A) and smooth muscle markers (actin and desmin). A diagnosis of PEComa was made and an uncomplicated middle pancreatectomy was performed. Our case and review of the literature demonstrates that EUS-FNA complemented with tissue cell block increases cellular yield, improved preoperative diagnostic accuracy, and may assist the surgeon in planning conservative surgical management. Diagn. Cytopathol. 2017;45:59-65. © 2016 Wiley Periodicals, Inc.
Patient: Female, 68-year-old Final Diagnosis: Richter transformation of CLL to HL Symptoms: Abdominal pain • ascites • diffuse lymphadenopathy • fever • hepatosplenomegaly • pleural effusion • weakness Medication: — Clinical Procedure: — Specialty: Forensic Medicine • Hematology • General and Internal Medicine • Oncology • Pathology Objective: Rare disease Background: Richter transformation (RT) is an uncommon clinicopathological condition referring to the development of aggressive lymphoma from chronic lymphocytic lymphoma/small lymphocytic lymphoma (CLL/SLL) and characterized by sudden clinical deterioration with marked multifocal lymphadenopathy. Transformation of CLL/SLL to diffuse large B-cell lymphoma is the most common (2–9%), but T-cell and Hodgkin transformation (HL) (0.4%) occur, although less frequently. Case Report: A 68-year-old woman initially diagnosed with CLL/SLL presented to the hospital with fever, weakness, abdominal pain, and vomiting. The physical examination showed hepatosplenomegaly and extensive abdominal lymphadenopathy on radiological imaging. The laboratory work-up revealed pancytopenia and a markedly increased alkaline phosphatase. In the setting of extensive granulomatous hepatitis, the development of aggressive Hodgkin lymphoma Richter transformation with multi-organ involvement within a few months led to the patient’s sudden death. Autopsy findings led to the post-mortem diagnosis of Richter transformation of CLL. Conclusions: Here, we describe a rare case of Hodgkin lymphoma RT from progressive CLL, with transformation occurring at approximately 12 years after initial diagnosis, despite treatment. Our case report underscores the diagnostic challenges and pitfalls associated with the granulomatous presentation masking RT transformation of CLL to Hodgkin lymphoma. The purpose of this report is to raise suspicion for the clinicopathological signs of Richter transformation in the presence of an atypical granulomatous presentation.
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