scholarly journals CD10-Positive Cutaneous PEComa: An Extremely Rare Skin Tumour

2020 ◽  
Vol 12 (3) ◽  
pp. 192-198
Author(s):  
Simon Ueberschaar ◽  
Matthias Goebeler ◽  
Hermann Kneitz
Keyword(s):  
Clear Cell ◽  
Subcutaneous Tissue ◽  
Epithelioid Cell ◽  
Tumour Cells ◽  
Cell Tumour ◽  
Surrounding Tissue ◽  
Neoplastic Cells ◽  
Cell Renal Cell Carcinoma ◽  
Perivascular Epithelioid Cell ◽  
Clear Cytoplasm

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.

Pigmented Perivascular Epithelioid Cell Tumor of the Kidney

2009 ◽  
Vol 133 (12) ◽  
pp. 1981-1984 ◽  
Author(s):  
Masaharu Fukunaga ◽  
Tohru Harada
Keyword(s):  
Tumor Cells ◽  
Spindle Cell ◽  
S100 Protein ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Cell Renal Cell Carcinoma ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Spindle Cell Proliferation ◽  
Clear Cytoplasm

Abstract A case of pigmented perivascular epithelioid cell tumor of the kidney in a 57-year-old woman with a clinically indicated tuberous sclerosis is presented. The lesion was a 3.0-cm, well-demarcated, black-colored mass. The tumor was histologically characterized by an epithelioid arrangement of round to polygonal cells with round nuclei and clear cytoplasm containing abundant melanin. Tumor cells showed mild atypia and low mitotic activity. A spindle cell proliferation was focally observed. There were no adipose elements or thick-walled vascular vessels. The stroma demonstrated intervening, thin, fibrovascular septa. Immunohistochemically, the tumor cells were strongly positive for HMB-45 but negative for epithelial and muscle markers, vimentin, and S100 protein. The patient had no evidence of disease 3 months after surgery. Pathologists and clinicians should know about the existence of pigmented perivascular epithelioid cell tumor of the kidney. This type of tumor should be differentiated from clear cell renal cell carcinoma or malignant melanoma.

Malignant Perivascular epithelioid cell tumour (Pecoma) of the gastrointestinal tract

Pathology ◽  
2013 ◽  
Vol 45 ◽  
pp. S76
Author(s):  
K. Manoharan ◽  
P. Cropley ◽  
A. Gal ◽  
G. Wong ◽  
S. McCarthy ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Epithelioid Cell ◽  
Cell Tumour ◽  
Perivascular Epithelioid Cell

scholarly journals A case of perivascular epithelioid cell tumour (pecoma)-NOS

Pathology ◽  
2016 ◽  
Vol 48 ◽  
pp. S84
Author(s):  
Sagarika Tripathy ◽  
Anthony Kelmann ◽  
Yuen Chan
Keyword(s):  
Epithelioid Cell ◽  
Cell Tumour ◽  
Perivascular Epithelioid Cell

Epithelioid Angiomyolipoma: A Morphologically Distinct Variant That Mimics a Variety of Intra-abdominal Neoplasms

2011 ◽  
Vol 135 (5) ◽  
pp. 665-670
Author(s):  
Ozgur Mete ◽  
Theodorus H. van der Kwast
Keyword(s):  
Correct Diagnosis ◽  
Smooth Muscle Tumor ◽  
Epithelioid Cell ◽  
Cell Phenotype ◽  
Renal Oncocytoma ◽  
Nerve Sheath Tumor ◽  
Epithelioid Angiomyolipoma ◽  
Perivascular Epithelioid Cell ◽  
Show Evidence ◽  
Clear Cytoplasm

Abstract This review examines the histopathologic, immunohistochemical, ultrastructural, and molecular biologic features of epithelioid angiomyolipoma (EAML), with an emphasis on the differential diagnosis of intra-abdominal EAML. Epithelioid angiomyolipoma is an uncommon mesenchymal tumor with malignant potential, frequently associated with tuberous sclerosis complex. Histologically, EAML is characterized by sheets or nests of large polygonal epithelioid cells with abundant eosinophilic or occasionally clear cytoplasm, often with prominent nucleoli, and EAML may include multinucleated and markedly pleomorphic forms. As these tumors share a distinctive perivascular epithelioid cell phenotype, they belong to the PEComa tumor family. Nearly all EAMLs show immunoreactivity for both melanocytic and myoid markers. Ultrastructurally, EAMLs show evidence of melanogenesis by the presence of premelanosomes. Epithelioid angiomyolipoma can pose significant diagnostic challenges as it mimics morphologically a variety of neoplasms including renal cell carcinoma, renal oncocytoma, adrenal cortical neoplasm, epithelioid smooth muscle tumor, epithelioid peripheral nerve sheath tumor, epithelioid gastrointestinal stromal tumor, epithelioid melanoma, hepatoblastoma, and hepatocellular carcinoma. The variation in immunophenotype in these tumors requires a prudent use of immunohistochemistry, which may occasionally need complementation by electron microscopy to establish the correct diagnosis.

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

2020 ◽  
Vol 16 (5) ◽  
pp. 1-4
Author(s):  
Massimiliano Silveri ◽  
Gian Luigi Natali ◽  
Giovanni Cobellis ◽  
Agnese Roberti ◽  
Sabrina Rossi ◽  
...  
Keyword(s):  
Epithelioid Cell ◽  
Cell Tumour ◽  
Perivascular Epithelioid Cell ◽  
Paediatric Case ◽  
Organ Sparing

Malignant perivascular epithelioid cell tumour with rhabdomyosarcomatous differentiation of the pelvic cavity

2015 ◽  
Vol 68 (5) ◽  
pp. 763-766
Author(s):  
Huijuan Shi ◽  
Teng Zou ◽  
Hui Li ◽  
Tiantian Zhen ◽  
Fenfen Zhang ◽  
...  
Keyword(s):  
Epithelioid Cell ◽  
Cell Tumour ◽  
Pelvic Cavity ◽  
Perivascular Epithelioid Cell

scholarly journals An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Zofia Tynski ◽  
Way Chiang ◽  
Albert Barrett
Keyword(s):  
Clear Cell ◽  
Middle Age ◽  
Epithelioid Cell ◽  
Ligamentum Teres ◽  
Cell Tumor ◽  
The Third ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasms ◽  
First Case ◽  
Sugar Tumor

Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.

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