Primary cutaneous perivascular epithelioid cell tumor (<scp>PEComa</scp>): Five new cases and review of the literature

Stuart, Lauren N.; Tipton, Russell G.; DeWall, Michael R.; Parker, Darien; Stelton, Christina D.; Morrison, Annie O.; Coleman, Landon W.; Fosko, Scott W.; Vidal, Claudia I.; Hurley, M. Yadira; Deeken, Amy; Gardner, Jerad M.

doi:10.1111/cup.12972

Cited by 25 publications

(56 citation statements)

References 20 publications

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“…Histologically, primary cutaneous PEComas are characterized by a typical perivascular and occasionally radial arrangement of epithelioid cells with pale eosinophilic to finely granular clear cytoplasm and small central nuclei. While they mostly present with a perivascular architecture [6, 7], variants have been reported without a direct relationship to blood vessels. The cellular composition of PEComas may vary substantially; besides purely epithelioid forms, mixed cell and even spindle cell variants may be found [3, 13].…”

Section: Discussionmentioning

confidence: 99%

“…PEComas are defined by characteristic histological and immunohistochemical properties [4, 5]. Their histogenesis is uncertain and there are no known cellular counterparts to the distinctive neoplastic cell [6, 7]. The perivascular epithelioid cells (PEC) have an epithelioid shape, a clear to pale eosinophilic cytoplasm, a predominantly perivascular location, and are characterized by the co-expression of melanocytic markers (HMB-45, MART-1, MiTF) and smooth muscle actin/desmin.…”

Section: Introductionmentioning

confidence: 99%

“…While immunohistochemical positivity for CD10 had been considered as a reliable marker for cutaneous metastases of clear cell renal cell carcinoma, in 2016 Fernandez-Flores et al [7] reported 3 cases of cutaneous PEComa that were marked by positivity for CD10 as well. Stuart et al [6] described another 5 cases of CD10-positive cutaneous PEComas.…”

Section: Introductionmentioning

confidence: 99%

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CD10-Positive Cutaneous PEComa: An Extremely Rare Skin Tumour

Ueberschaar¹,

Goebeler²,

Kneitz³

2020

Case Rep Dermatol

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We here present the case of a 67-year-old woman with a history of a slowly progressive, polypous nodule on her left wrist. The lesion was excised, and the histological analysis revealed a clear cell tumour that was relatively sharply demarked from the surrounding tissue extending into the subcutaneous tissue. The tumour showed a characteristic trabecular pattern in which the tumour cells were arranged around numerous vessels. The neoplastic cells had a predominantly epithelioid shape, granular eosinophilic to clear cytoplasm and prominent centrally located nucleoli. The histological differential diagnosis included a metastatic clear-cell renal cell carcinoma and a primary cutaneous perivascular epithelioid cell tumour (PEComa). Immunohistochemically, the tumour cells revealed homogenous expression of HMB-45, MiTF and CD10, whereas MART-1 and S100 were negative. Antibodies against actin marked the trabecularly arranged vessels, and the neoplastic cells yielded a patchy positivity against actin and desmin. Additional immunohistochemical stains against pan-cytokeratin, CAIX, PAX-8 and EMA were negative. Based on the morphologic and immunophenotypic findings, the histological diagnosis of a CD10-positive cutaneous PEComa was made.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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CD10-Positive Cutaneous PEComa: An Extremely Rare Skin Tumour

Ueberschaar¹,

Goebeler²,

Kneitz³

2020

Case Rep Dermatol

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“…PEComa also has a unique immunophenotype with expression of both myogenic and melanocytic markers. 18 In summary, we present the first case description…”

Section: Case Reportmentioning

confidence: 95%

Salivary gland hyalinizing clear‐cell carcinoma with cutaneous metastasis: A rare and deceptive tumor

et al. 2020

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Clear-cell carcinoma (CCC) is an uncommon malignant tumor of minor salivary glands. It characteristically has a low-grade morphology and a favorable outcome by most reports. An EWSR1-ATF1 fusion can be detected in the majority of cases. We present a rare case of CCC, which had an aggressive course with the development of cutaneous metastases. Practicing dermatopathologists should be aware of this tumor given its low-grade appearance and histopathologic resemblance to other primary cutaneous adnexal and metastatic neoplasms.

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“…PEComas show a wide anatomical distribution, but most often arise in the retroperitoneum, uterus, gastrointestinal tract, or abdominopelvic region. Primary cutaneous PEComas are relatively rare, have a wide age range, and arise predominantly in lower extremities [124][125][126]. Clinically, these lesions usually present as a single painless, slow-growing nodule or plaque.…”

Section: Pecomamentioning

confidence: 99%

Epithelioid Cutaneous Mesenchymal Neoplasms: A Practical Diagnostic Approach

Choi

2020

Diagnostics

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Epithelioid cells are rounded or polygonal cells with abundant eosinophilic or clear cytoplasm and ovoid to round nuclei, superficially resembling epithelial cells. Cutaneous mesenchymal neoplasms composed predominantly or exclusively of epithelioid cells are relatively uncommon and can cause considerable diagnostic difficulties due to overlapping histologic features among heterogeneous groups of tumors. Familiarity with practical diagnostic approaches and recognition of key histopathologic features are important for correct diagnosis and management. This review summarizes the histologic features of epithelioid cutaneous mesenchymal neoplasms and discusses their differential diagnoses from malignant melanomas and carcinomas.

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Primary cutaneous perivascular epithelioid cell tumor (PEComa): Five new cases and review of the literature

Cited by 25 publications

References 20 publications

CD10-Positive Cutaneous PEComa: An Extremely Rare Skin Tumour

CD10-Positive Cutaneous PEComa: An Extremely Rare Skin Tumour

Salivary gland hyalinizing clear‐cell carcinoma with cutaneous metastasis: A rare and deceptive tumor

Epithelioid Cutaneous Mesenchymal Neoplasms: A Practical Diagnostic Approach

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