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Heavily pigmented perivascular epithelioid cell tumors (PEComa) are rare, only eight cases of which have been reported. Unlike typical epithelioid angiomyolipoma, most of these tumors have been encountered in female patients without tuberous sclerosis. The long-term prognosis thereof is undetermined. Cytological similarity and heavy melanin pigment make it difficult for pigmented PEComa to be differentiated from pigmented clear cell renal cell carcinoma or malignant melanoma. The immunoprofile of tumor cells, such as human melanoma black-45 expression, as well as the absence or presence of other melanocytic or epithelial markers, are helpful in determining a differential diagnosis. Here we report a case of heavily pigmented PEComa of the right kidney and review the literature describing this tumor. In this case, the immunoprofile and clinical features corresponded well to those described in the literature. Since the prognosis of such disease has not yet been established, close follow-up of this patient was recommended.

Citations

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Plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach is a recently recognized entity. Because of its rarity, only 22 cases have been reported in the English-language literature and most of these are single case reports. We report two cases of gastric PAMT. The tumor cells were bland and plexiform arranged in a myxoid stroma, which was positive for alcian blue. Immunohistochemically, the tumor cells were positive for smooth muscle actin, but negative for c-kit, CD34, desmin, S-100 protein, epithelial membrane antigen, neurofilament, and protein kinase C-theta. Mutation analyses for exon 9, 11, 13, and 17 of KIT genes and 12, 14, and 18 of the platelet-derived growth factor receptor alpha (PDGFRA) genes were performed and the tumors were wild-type for mutation.

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  Michael W. Morris, Lisa Sullivan, David E. Sawaya, Michael A. Steiner, Michael J. Nowicki
  Journal of Pediatric Surgery Case Reports.2016; 4: 38.     CrossRef
• Unusual focal keratin expression in plexiform angiomyxoid myofibroblastic tumor
  Giuseppe Quero, Teresa Musarra, Alfredo Carrato, Michelangelo Fici, Maurizio Martini, Angelo Paolo Dei Tos, Sergio Alfieri, Riccardo Ricci
  Medicine.2016; 95(28): e4207.     CrossRef
• Laparoscopy endoscopy cooperative surgery for gastric plexiform fibromyxoma: a case report
  Yoshikage Inoue, Shutaro Gunji, Kazutaka Obama, Hiroshi Okabe, Yoshiharu Sakai
  Surgical Case Reports.2016;[Epub]     CrossRef
• Plexiform fibromyxoma with cotyledon-like serosal growth: A case report of a rare gastric tumor and review of the literature
  JOSHUA ROBERT KANE, NATASHA LEWIS, REBECCA LIN, CELINA VILLA, ALEXANDRA LARSON, JEFFREY D. WAYNE, ANJANA V. YELDANDI, WILLIAM B. LASKIN
  Oncology Letters.2016; 11(3): 2189.     CrossRef
• A case of gastric plexiform fibromyxoma: radiological and pathological findings
  Katsumi Sakamoto, Masakazu Hirakawa, Kazushige Atsumi, Koshi Mimori, Kohei Shibata, Taro Tobo, Hidetaka Yamamoto, Hiroshi Honda
  Japanese Journal of Radiology.2014; 32(7): 431.     CrossRef
• Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach: Report of a Case and Review of the Literature
  Soo-Heui Baek, Jung-Hee Yoon, Ji-Yeon Kim
  Journal of the Korean Society of Radiology.2014; 70(1): 47.     CrossRef
• Plexiform Fibromyxoma: Report of Two Pediatric Cases and Review of the Literature
  Lizette Vila Duckworth, Raul S. Gonzalez, Matthew Martelli, Chen Liu, Cheryl M. Coffin, John D. Reith
  Pediatric and Developmental Pathology.2014; 17(1): 21.     CrossRef