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2 "Pigmentation"
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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
  • 7,554 View
  • 62 Download
  • 9 Crossref
AbstractAbstract 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.

Citations

Citations to this article as recorded by  
  • 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
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.
  • 1,600 View
  • 23 Download
AbstractAbstract PDF
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.

J Pathol Transl Med : Journal of Pathology and Translational Medicine