Heavily melanotic perivascular epithelioid clear cell tumor of the kidney

Adachi, Shiro; Hanada, Masato; Kobayashi, Yoshiyuki; Tsutahara, Kohichi; Fukuhara, Shinichiro; Mori, Nobuhiro; Hara, Tsuneo; Mukai, Hideyo; Shimasaku, Emi; Kawai, Masao; Kishikawa, Naoto; Yamaguchi, Seiji

doi:10.1111/j.1440-1827.2004.01617.x

Cited by 24 publications

(23 citation statements)

References 24 publications

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“…PEComas were found in various tissues, such as gynecologic, soft, cutaneous, and visceral tissues [1,6,13,15,16,18,20,21,23]. In addition, the following rare two cases have been reported.…”

Section: Discussionmentioning

confidence: 99%

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Perivascular epithelioid cell tumor of the rib

et al. 2008

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We present a rare case of perivascular epithelioid cell tumor (PEComa) in the right 6th rib of a 28-year-old man. A plain computed tomography scan showed a round osteolytic lesion in the right 6th rib. The resected tissue contained a globular-shaped, soft tumor. Histologically, the tumor was rich in vasculature and exclusively composed of perivascular epithelioid cells with clear cytoplasm. Immunohistochemically, the tumor expressed diffusely a melanocyte marker, human melanoma black-45, and focally a myogenic marker, alpha-smooth muscle actin, but not an epithelial marker, AE1/AE3. Fontana-Masson-positive melanin pigments were present and c-kit receptor tyrosine kinase (CD117), involved in the development of melanocytes but not myogenic cells, was expressed in tumor cells. These findings indicate that the tumor is PEComa with some differentiation into melanocytes. Notably, owing to the unique location of the occurrence, the tumor occupied bone marrow tissues of the rib, resulting that the tumor has the potential for hematogenous metastasis. In spite of the lack of cells with severe atypia, necrosis, and numerous mitoses, tumor cells invaded into surrounding tissues and overexpressed cyclin D1. To the best of our knowledge, this is the first case report of PEComa arising from the rib with the signs of malignant potential.

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“…PEComas were found in various tissues, such as gynecologic, soft, cutaneous, and visceral tissues [1,6,13,15,16,18,20,21,23]. In addition, the following rare two cases have been reported.…”

Section: Discussionmentioning

confidence: 99%

“…PEComas with abundant Fontana-Masson-positive melanin pigments and definite melanosome have been reported [1,21,22]. Moreover, the expression of c-kit receptor tyrosine kinase (CD117), which is involved in the development of melanocytes but not myogenic cells, is reported in AML [13,17].…”

Section: Discussionmentioning

confidence: 99%

Perivascular epithelioid cell tumor of the rib

et al. 2008

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“…However, pigmented PEComas were reported in organs other than the skin and subcutaneous tissue. They are exceedingly rare neoplasms and have been reported in kidney, [30][31][32] pelvis, 33 liver, 34 and orbit. 35 Here, we describe the first case of cutaneous PEComa with focal melanin pigmentation histologically and discuss differential diagnoses of other entities, such as melanoma, renal clear cell carcinoma, clear hidradenoma, and paraganglioma.…”

Section: Introductionmentioning

confidence: 99%

Pigmented Perivascular Epithelioid Cell Tumor of the Skin

Navale

Asgari

Chen

2015

The American Journal of Dermatopathology

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The authors present a first case of pigmented perivascular epithelioid cell tumor (PEComa) of the skin, which was misdiagnosed histopathologically as metastatic melanoma. PEComa are exceedingly rare neoplasms, and only a few cases have been reported in kidney, liver, and pelvis. Herein, we describe this very rare entity in the subcutaneous tissue of the flank with very similar if not identical morphology of renal clear cell carcinoma. Because of the presence of focal melanin pigmentation and strong positivity for HMB-45, the lesion was mistaken for metastatic melanoma. To the best of authors' knowledge, this is the first case of cutaneous PEComa with melanin pigmentation. Awareness about occurrence of the lesion in the subcutaneous tissue and its unique immunoprofile would be helpful to reach correct diagnosis and to distinguish this lesion from melanoma or metastatic renal clear cell carcinoma.

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“…A case of a heavily melanotic perivascular epithelioid clear cell tumor of the kidney was recently reported [1]. A large amount of cystoplasmic melanin was reported as a striking feature and the histochemical and immunohistochemical profiles support the extreme melanocytic differentiation.…”

Section: Discussionmentioning

confidence: 87%

Malignant clear-cell myomelanocytic tumor of broad ligament—a case report

et al. 2006

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Clear-cell myomelanocytic tumors (CCMT) of the perivascular epithelioid cell tumor (PEComa) family have been recently reported. We report a case involving a 12-year-old girl. The tumor (9 x 7.5 x 7 cm) was a firm, tan-gray mass with heavily dark pigmentation, massive hemorrhage, and necrosis, and was located in the right broad ligament attached to the right ovary. Histologically, the tumor was composed of polygonal cells exhibiting diffuse hemorrhage, multifocal necroses, and vascular invasion. Most of the tumor cells contained melanin pigments with Fontana-Masson positivity and ultrastructurally suspicious, membrane-bound premelanosomes. Immunohistochemical staining was positive against HMB-45 and focally positive for smooth muscle actin. The tumor recurred in the form of multiple conglomerated masses of the right iliac fossa, with the greatest measuring up to 3.8 cm in dimension, within 1 year. Most CCMT are believed to originate from falciform ligament/ligamentum teres. To the best of our knowledge, this is the second report of a CCMT arising in the broad ligament with typical morphology and contributory ancillary results. Further study for proper subclassification of the PEComa family should be validated, not by anatomic site but by clinical behavior.

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Heavily melanotic perivascular epithelioid clear cell tumor of the kidney

Cited by 24 publications

References 24 publications

Perivascular epithelioid cell tumor of the rib

Perivascular epithelioid cell tumor of the rib

Pigmented Perivascular Epithelioid Cell Tumor of the Skin

Malignant clear-cell myomelanocytic tumor of broad ligament—a case report

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