CD10-Positive Cutaneous PEComa: An Extremely Rare Skin Tumour

Abstract

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.

Primitive Cutaneous (P)erivascular (E)pithelioid (C)ell Tumour (PEComa): A New Case Report of a Rare Cutaneous Tumor

“…A second step consisted in defining this lesion in the spectrum of benignity or malignancy. In this regard, the scheme of Folpe et al was used [3][4][5][6][7][8][9][10][11]. was partially positive.…”

“…A second step consisted in defining this lesion in the spectrum of benignity or malignancy. In this regard, the scheme of Folpe et al was used [3][4][5][6][7][8][9][10][11]. The cPEComa had only one characteristic suggestive of possible malignancy, the infiltrative growth pattern, but other parameters such as necrosis, tumour size greater than 5 cm and vascular invasion were absent.…”

“…For many years, clear cell tumours have been called sugar tumours in the lung and myomelanocytomas at other sites [ 12 , 13 ]. On the other hand, cPEComa is a rare tumour, first described in 2003 [ 6 ] and later confirmed by other reports [ 7 , 8 , 9 , 10 , 11 , 14 , 15 , 16 , 17 ]; it is not yet clear what the real histogenesis of this entity is, as the benign cell counterpart of origin of this neoplasm is not known [ 10 ]. The concept of PEComa was coined in a unifying sense for a series of different entities that has been named in the most diverse ways [ 3 ].…”

“…Differential diagnoses with entities such as malignant melanomas (MMs) and clear cell and balloon cell melanomas are equally important, considering the extensive polymorphic picture with which MMs can present [ 8 , 10 , 15 , 19 , 25 , 26 ]. In this case, a correct immunophenotypic investigation may prove sufficient since S-100 protein and Sry-related HMg-box gene 10 (SOX-10) will be positive in one case and not the other.…”

Malignant lung PEComa (clear cell tumor): rare case report and literature review

“…Furthermore, other markers such as S-100, vimentin, CD34, Melan-A, microphthalmia factor, neuron-specific enolase, cathepsin B, and CD1a can be expressed ( 18 , 20 , 28 ). CD10 may be expressed in other types of non-pulmonary PEComas ( 83 , 84 ). Our patient tested positive for HMB-45, Melan-A, smooth muscle actin, and CD10, and tested negative for cytokeratins.…”