Primary Gastric Histiocytic Sarcoma Reminiscent of Inflammatory Pseudotumor: A Case Report with Review of the Literature

No abstract available.

Diagnostic Utility of the JAZF1/JJAZ1 Gene Fusion in Endometrial Stromal Sarcomas and Their Histologic Variants

BACKGROUND: The diagnosis of endometrial stromal sarcoma (ESS) is often difficult in cases showing diverse histological differentiation or in undifferentiated endometrial sarcoma (UES). Recently, JAZF1/JJAZ1 gene fusion has been described as a defining feature of low-grade ESS (LGESS). However, its prevalence is variably reported, and the diagnostic utility has rarely been examined for cases showing various histological differentiation. METHODS: To test the diagnostic utility of JAZF1/JJAZ1 gene fusion in difficult cases, we compared the prevalence of the JAZF1/JJAZ1 fusion gene in LGESS with and without histological differentiation. RESULTS: The JAZF1/JJAZ1 fusion transcript was detected in 18 of 21 LGESS (85.7%), including 14 classical LGESS (93%), four LGESS with diverse histological differentiation (67%), and two with UES (28.6%). Positive cases included two LGESS with sex cord-like differentiation, one with osseous differentiation, and two UES. LGESS showing smooth muscle differentiation revealed the fusion transcript only in the classic area. Direct sequencing analysis of two LGESS revealed a previously reported breakpoint at t(7;17)(p15;q21). CONCLUSIONS: The JAZF1/JJAZ1 fusion gene was identified in a significant proportion of LGESS showing secondary histological differentiation except in cases with smooth muscle differentiation. Thus, this fusion gene may be useful to confirm the diagnosis in difficult cases of LGESS.

Fine Needle Aspiration Cytology of Langerhans Cell Histiocytosis of Mandible: A Case Report

We present a case of mandibular involvement with Langerhans cell histiocytosis (LCH), diagnosed by ultrasound-guided aspiration and subsequently confirmed by incisional biopsy and immunohistochemistry in an eight-year-old boy. The cytologic findings included the presence of characteristic Langerhans cells of both mononucleate and multinucleate form. Diagnostic confirmation was obtained by immunopositivity for S-100 protein and CD1a of Langerhans histiocytes on paraffin-embedded sections obtained during incisional biopsy of the right mandibular area. By reporting a case of childhood LCH, we correlate the cytologic findings with histologic features and discuss the role of aspiration cytologic diagnosis in such a rare and cytomorphologically characteristic case.

Two Cases of Pheochromocytoma Presented with Hypertension and Weight Loss / 대한소아내분비학회지

Pheochromocytoma is a rare disease in children, arising from chromaffin cells of adrenal medullary or extra-adrenal paraganglionic tissues. Typical clinical features include hypertension, diaphoresis, headache, and weight loss. It should therefore be considered that patients associated with hypertension might have pheochromocytoma. The diagnostic approach is based on typical clinical manifestations and biochemical test. Pheochromocytoma is completely curable by surgical resection. Early diagnosis and total excision are the most important aspects of accurate treatment of pheochromocytoma in children. We report two patients with pheochromocytoma presented with hypertension and weight loss both of which were improved after tumor resection.

Accidental Hanging in Children / 대한법의학회지

Accidental hanging in infancy and early childhood is not common and the autopsy findings have not been well-known. We experienced five cases of accidental hanging in children in a variety of situations. All cases were concluded as accidental hanging after a comprehensive examination based on the death scene investigation, autopsy findings, and investigation of their background and past history. Two cases happened at slides in a playground and in a kindergarten, the other cases related with by various kinds of cords in their house. As it is not always possible to predict every dangerous circumstance, these cases will be helpful to prevent the cases of accidental hanging.

Splenic Littoral Cell Angioma with Hepatitis C Associated Liver Cirrhosis

Littoral cell angioma (LCA) of spleen is an uncommon vascular neoplasm of littoral cell origin. It is well established that LCA may be associated with other malignancy or autoimmune disorders. We describe a 34-year-old woman with viral hepatitis C associated liver cirrhosis who presented with the incidental finding of LCA. She showed ascites, esophageal varix and drowsy mentality. Abdominal computed tomography (CT) showed multiple benign looking nodules in both hepatic lobes, but no abnormality in spleen. Liver transplantation and splenectomy were performed. Microscopic findings revealed narrow anastomosing vascular channels lined with plump cells that exfoliated into the lumen. Immunohistochemically the lining cells were positive for CD31, CD68 and negative for CD34, consistent with LCA. Herein, a second case of an incidentally detected LCA with cirrhosis, viral hepatitis C associated in Korea is reported.