Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematopoietic neoplasm, which in the past was also known variously as blastic NK cell lymphoma, agranular CD4+ natural killer cell leukemia, and CD4+/CD56+ hematodermic neoplasm. BPDCN is now believed to arise from plasmacytoid dendritic cells, but its exact etiology is still unknown. We report here on the cerebrospinal fluid (CSF) cytology of a BPDCN, a hypercellular specimen comprised of malignant, singly dispersed cells with scant to moderate amounts of pale blue, agranular cytoplasm, and uniform round to oval nuclei, fine chromatin, prominent nucleoli, occasional cytoplasmic microvacuoles, and pseudopodia. Neither mitoses nor karyorrhexis were identified. Flow cytometry of the CSF demonstrated that the malignant cells expressed bright CD45, HLA-DR and CD33, dim CD4, heterogeneous CD56, and partial CD123. The importance of clinical, histopathological, and phenotypic correlation is emphasized. Clinical and histopathological correlation and a literature review are also presented. The poor clinical outcome makes it important to accurately report this rare tumor in a CSF specimen.
Background: Sarcoidosis is a multisystem inflammatory disease of uncertain etiology that is histopathologically characterized by non-necrotizing granulomas. It typically involves the lungs and lymph nodes, but has been reported in many other organ systems as well. Sarcoidosis involving the female genital tract, the uterus in particular, is a rare event with isolated accounts in the medical literature. Case: We present a rare occurrence of cervical sarcoidosis in a Pap test of a 31-year-old woman with a history of biopsy-proven mediastinal lymph node sarcoidosis and mediastinal pleomorphic liposarcoma with status post chemotherapy. The Surepath smear showed crowded cellular groups with abundant epithelioid histiocytes and lymphocytes consistent with granulomatous inflammation. No granular necrotic debris or polarizable foreign body material was identified. There were no microorganisms present. The cytomorphologic features and differential diagnoses are discussed. Conclusion: Uterine cervical sarcoidosis, although rare, can be an overlooked cause of non-necrotizing granulomatous inflammation in a cervical Pap test. Sarcoidosis should be a diagnosis of exclusion; other common conditions with similar morphologic presentation such as non-necrotizing tuberculosis, foreign body-related granulomatous inflammation, and neoplastic/chemotherapy-related sarcomatoid reaction need to be excluded in conjunction with the patient’s clinical history.
Background: Signet ring carcinoma is an exceedingly rare and aggressive variant of primary bladder carcinoma. The cytomorphologic features of this rare entity in urinary specimens have not been well characterized. Methods: Twenty-seven cases of signet ring cell carcinoma of the urinary bladder were identified from the pathology archives of The Johns Hopkins Hospital (1989–2011). Of these, 6 cases with prior urinary cytology specimens were studied. Results: There were 5 males and 1 female, with a mean age of 62 years. The presenting complaints included hematuria with or without symptoms of bladder irritation. Histopathologically, there were 5 cases of primary bladder carcinoma and 1 case of metastatic colonic signet ring cell carcinoma. The salient cytomorphologic features included scattered malignant epithelial cells, displaying distinct cell borders, abundant cytoplasm with a single large, discrete mucin vacuole, and eccentric irregular nuclei with prominent nucleoli. Primary adenocarcinoma additionally revealed a few intact malignant glandular epithelial fragments. Metastatic colonic signet ring cell carcinoma displayed predominantly singly dispersed malignant cells with eccentrically placed, oval nuclei with occasional small nucleoli and a moderate amount of vacuolated cytoplasm. Conclusion: Signet ring cell carcinomas are rarely encountered in urinary cytology. The differential diagnosis includes distended histiocytes or degenerated urothelial cells, primary signet ring cell carcinoma, and metastatic adenocarcinoma.
We present a rare occurrence of metastatic proximal epithelioid sarcoma (PES) in the pleural effusion of a 23-year-old man, developed within one year of diagnosis in his gluteal soft tissue. The cytologic and immunoperoxidase findings are described. PES, due to its epithelioid morphology, can be confused with more common cancers in effusions such as adenocarcinoma and mesothelioma. PES is an aggressive neoplasm that differs clinically and pathologically from conventional epithelioid sarcoma. Knowledge of its cytomorphology in serous cavity effusions, a patient's clinical history and ancillary studies may lead to an accurate diagnosis.
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