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- Pigmented Perivascular Epithelioid Cell Tumor (PEComa) of the Kidney: A Case Report and Review of the Literature
- Hyeyoon Chang, Wonkyung Jung, Youngran Kang, Woon Yong Jung
- Korean J Pathol. 2012;46(5):499-502. Published online October 25, 2012
- DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.5.499
- 7,997 View
- 71 Download
- 9 Crossref
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Abstract
PDF 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.
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Citations
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- Malignant Pigmented Epithelioid Angiomyolipoma of the Kidney in a Child with Tuberous Sclerosis Complex
Thu Dang Anh Phan, Nhi Thuy To, Diem Thi Nhu Pham
Fetal and Pediatric Pathology.2023; 42(2): 285. CrossRef - Perivascular epithelioid cell tumor (PEComa) of the cystic duct
Takeshi Okamoto, Takashi Sasaki, Yu Takahashi, Manabu Takamatsu, Hiroaki Kanda, Makiko Hiratsuka, Masato Matsuyama, Masato Ozaka, Naoki Sasahira
Clinical Journal of Gastroenterology.2023; 16(1): 87. CrossRef - PEComa of the Adrenal Gland
Craig B. Wakefield, Peter M. Sadow, Jason L. Hornick, Christopher D.M. Fletcher, Justine A. Barletta, William J. Anderson
American Journal of Surgical Pathology.2023; 47(11): 1316. CrossRef - Recurrence of Pigmented Epithelioid Angiomyolipoma of the Kidney With Xp11 Translocation: A Case Report
Mahmoud D Srour, Andrew Harris
Cureus.2023;[Epub] CrossRef - Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney
Hexi Du, Jun Zhou, Lingfan Xu, Cheng Yang, Li Zhang, Chaozhao Liang
Medicine.2016; 95(44): e5248. CrossRef - PEComas of the kidney and of the genitourinary tract
Guido Martignoni, Maurizio Pea, Claudia Zampini, Matteo Brunelli, Diego Segala, Giuseppe Zamboni, Franco Bonetti
Seminars in Diagnostic Pathology.2015; 32(2): 140. CrossRef - Pigmented Perivascular Epithelioid Cell Tumor of the Skin
Pooja Navale, Masoud Asgari, Sheng Chen
The American Journal of Dermatopathology.2015; 37(11): 866. CrossRef - Clear Cell Melanoma: A Cutaneous Clear Cell Malignancy
Maria A. Pletneva, Aleodor Andea, Nallasivam Palanisamy, Bryan L. Betz, Shannon Carskadon, Min Wang, Rajiv M. Patel, Douglas R. Fullen, Paul W. Harms
Archives of Pathology & Laboratory Medicine.2014; 138(10): 1328. CrossRef - Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases
Dae Hyun Song, In Ho Choi, Sang Yun Ha, Kang Min Han, Jae Jun Lee, Min Eui Hong, Yoon-La Choi, Kee-Taek Jang, Sang Yong Song, Chin A Yi, Joungho Han
Korean Journal of Pathology.2014; 48(3): 188. CrossRef
- Malignant Pigmented Epithelioid Angiomyolipoma of the Kidney in a Child with Tuberous Sclerosis Complex
- Pediatric Pigmented Dermatofibrosarcoma Protuberans (Bednar Tumor): A Case Report.
- Dae Woon Eom, Gil Hyun Kang, Key Won Lee, Soo Jung Choi
- Korean J Pathol. 2007;41(3):183-186.
- 1,707 View
- 23 Download
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Abstract
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- A Bednar tumor is a rare neoplasm of intermediate malignant potential that accounts for 1-5% of all cases of dermatofibrosarcoma protuberans (DFSP). This tumor is considered a pigmented variant of DFSP, because the clinical and histological findings resemble DFSP. The diagnosis is commonly made in early to middle adult life except in cases with melanin containing cells. In the case presented here, the patient was a 3-year-old male who presented with a painless slow-growing 2.0x1.5x1.0 cm mass on the dorsal aspect of his right hand. Histological examination of the biopsy specimen revealed typical features of a Bednar tumor, which was composed of CD34 positive monomorphous spindle shaped cells arranged in a storiform fashion with moderate mitotic activity (up to 5 per 10 HPF) and scattered pigmented cells with dendritic processes. We report a rare case of Bednar tumor affecting a pediatric patient and review the medical literatures.
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