UNLABELLED Perivascular epithelioid cell neoplasms (PEComas) of the urinary bladder are extremely rare and the published cases were comprised predominantly of middle-aged patients. Herein, the authors present the first urinary bladder PEComa occurring in an adolescent. This 16-year-old Chinese girl present with a 3-year history of abdominal discomfort and a solid mass was documented in the urin...

Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB45 and/or Melan-A) and smooth muscle (actin and/or desmin) markers. A 36-year-old male was admitt...

Dear Editor, Perivascular epithelioid cell tumor, also known as PEComa, is a type of mesenchymal neoplasm. Here, we encountered an interesting case of primary PEComa in a cervical lymph node (LN) that was associated with leukoderma. A 63-year-old Japanese man was referred to us with a 3month history of a swollen LN in the left neck. The patient was a white collar worker belonging to an advertis...

A 60-year-old male patient presented with complaints of loss of appetite and bloated abdomen of two weeks duration. The radiological examination revealed large mass involving left kidney. With a clinical and radiological diagnosis of renal cell carcinoma, radical nephrectomy was done. Histopathological examination revealed a neoplasm composed purely of epithelioid cells. Based on histopathologi...

症例は48歳女性．生理不順を主訴に当院婦人科受診，肝腫瘍を指摘された．入院時身体所見は腹部に腫瘤を触知するも圧痛なし．HBs-Ag陰性，HCV-Ab陰性で，腫瘍マーカーはCA19-9のみ軽度上昇していた．腹部CT検査では肝外側区域に境界明瞭な14cm大の単発性腫瘍を認めた．内部は多血性で，不均一に造影され，複数の発達した肝静脈にドレナージされていた．消化管には明らかな悪性所見は認めなかった．以上より，肝血管筋脂肪腫（angiomyolipoma：AML）を最も疑ったが，悪性腫瘍が否定できないことと破裂の可能性を考慮し肝左葉切除術を施行した．術後の病理組織学所見ならびに免疫組織化学所見よりperivascular epithelioid cell tumor（PEComa）と診断した．肝PEComaは稀な腫瘍であり，その悪性度評価，治療法には現状では一定の見解はない．文献的考察を加えて...