Colonic angiomyolipoma with a monotypic expression and a predominant epithelioid component.

Queens Medical Centre University Hospital, Nottingham NG7 2UH, UK.
Journal of Clinical Pathology (Impact Factor: 2.55). 11/2005; 58(10):1107-9. DOI: 10.1136/jcp.2004.025130
Source: PubMed

ABSTRACT Angiomyolipomas are rare lesions, often arising in the kidney, and are part of a group of tumours with a diverse appearance and evidence of dual melanocytic and smooth muscle differentiation known as PEComas (tumours of perivascular epithelioid cell origin). This report describes an unusual case of a colonic PEComa in a 40 year old woman. Unlike most of the previous colonic angiomyolipomas/PEComas reported in the literature, this case formed a large, mainly extrinsic mass and was monotypic, and composed entirely of the myomatous component with no adipose tissue or typical vasculature.

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    ABSTRACT: Neoplasms of the perivascular epithelioid cell (PEComas) represent a recently described heterogeneous group of mesenchymal tumors characterized by the presence of specific histological, immunohistochemical, and ultrastructural findings. The PEComas encompass a family of neoplasms that include angiomyolipomas, clear cell sugar tumors, and lymphangioleiomyomatosis. The PEComas demonstrate a wide spectrum of clinicobiological behavior and imaging findings. Perivascular epithelioid cell, as the name implies, is a unique cell that is characterized by perivascular distribution and epithelioid morphology. Perivascular epithelioid cell consistently shows immunoreactivity to melanocytic and smooth muscle markers including HMB-45 and actin. Abdominopelvic PEComas are found at a variety of somatic and visceral locations including kidney, liver, pancreas, gastrointestinal tract, genitourinary tract, peritoneum, and retroperitoneum. A subset of patients with abdominopelvic PEComas manifests tuberous sclerosis complex. In this paper, we review the histological spectrum and discuss the imaging findings of the PEComas that involve the abdomen and pelvis.
    Journal of Computer Assisted Tomography 08/2007; 31(5):688-96. DOI:10.1097/rct.0b013e318031912f · 1.60 Impact Factor
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    ABSTRACT: Angiomyolipoma is apparently a part of a family of neoplasms that derive from perivascular epitheloid cells. It is a rare mesenchymal tumor, usually found in the kidney. Extrarenal angiomyolipoma is uncommon and the most common extrarenal site is the liver. Only two cases of adrenal angiomyolipoma are reported in English literature. Authors wish to add one more case to world literature. Because of its large size and symptomatic presentation of extremely rare tumor merits documentation.
    Indian Journal of Urology 08/2007; 23(3):319-20. DOI:10.4103/0970-1591.33734
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    ABSTRACT: Angiomyolipoma of stomach is extremely rare. Only 1 case has been reported earlier. We report a case of 40-year-old female who presented with gastrointestinal bleeding. Upper gastrointestinal endoscopy suggested a polypoidal growth in the stomach. Biopsy from the growth was suggestive of mesenchymal tumor. The contrast-enhanced computed tomography of abdomen revealed a pedunculated 4×3 cm, intramural lesion at the junction of first and second parts of duodenum, likely lipoma. The stalk seemed to be arising from distal stomach. At laparoscopy, a highly mobile lesion was observed arising from the posterior wall of stomach. Wedge resection of the lesion was performed laparoscopically after doing an anterior gastrotomy. The final diagnosis on histopathology was a gastric angiomyolipoma.
    Surgical laparoscopy, endoscopy & percutaneous techniques 02/2012; 22(1):e21-4. DOI:10.1097/SLE.0b013e31823cd1f5 · 0.94 Impact Factor

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