Primary Carcinosarcoma of the Skin in an African Albino: Case Report and Review of Literature

Abstract

Albinism results from a deficiency of the protein responsible for melanin production in melanocytes. Albinos are at an increased risk of dermatoses and malignancies. Primary carcinosarcoma of the skin is an exceedingly rare biphasic tumor composed of malignant epithelial and mesenchymal elements with an unclear histogenesis. To our knowledge, this has not been reported in an African albino. Here, we report the index case of this rare tumor in an albino of African descent.

A Rare Cutaneous Malignancy: A Primary Cutaneous Carcinosarcoma with Adnexal Epithelial Component (Trichoblastic Carcinosarcoma) of Left Temporal Region

“…Histologically the epithelial component is epidermal-derived (basal or squamous cell carcinoma- BCC/SCC) or adnexal (spiradenocarcinoma, porocarcinoma, trichoblastic, or pilomatrical carcinoma) and the mesenchymal one can be homologous or heterologous - undifferentiated sarcoma, osteosarcoma, chondrosarcoma, rarely leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, angiosarcoma or giant cell tumour of soft parts. [ 4 ] The heterogeneity of this tumor necessitates large sampling, as insufficient sampling may cause misdiagnosis a carcinoma or sarcoma simply. [ 2 ]…”

“…IHC shows cytokeratin highlighting the epithelial elements, while vimentin positivity in the mesenchymal elements and co-expression of p63 in both. [ 4 ] p63 positivity in skin lesions is seen in cutaneous sweat gland carcinoma, SCC and BCC, and in contrast metastatic breast and other adenocarcinomas to skin are p63 negative. In addition D2-40 and P40 were positive and BerEP4, S100, Melan A and MITF were negative in present case indicating primary skin adnexal tumor rather than epidermal.…”

“…Complete excision of the lesion with 10 mm-free circumferential margin is the treatment of choice; adjuvant chemotherapy or radiotherapy has not been shown to be beneficial. [ 4 ] With Mohs micrographic surgery a cure rate of ≥98% has been reported; in contrast to a recurrence rate of 33% without it. The prevalence of metastasis in PCCS with a BCC epithelial component has been reported upto 2% of cases and between 12 and 50% in SCC/adnexal ones.…”

The Rarity in the Rarity: Presentation of Three Cases of Cutaneous Carcinosarcoma with Clinical and Histopathological Insights

“…Patients with a cCS typically present with a rapidly growing, ulcerated nodule or plaque on sun-exposed areas of the skin, such as the head, neck, and extremities [ 6 ], and the lesion is often painful and may bleed or become infected. Due to its aggressive nature, the tumor can invade surrounding tissues and metastasize to distant sites if not treated promptly [ 6 , 7 ]. Histopathologically, a cCS can be composed of different epithelial and mesenchymal components, of which the first one can be represented by a squamous cell carcinoma (SCC), basal cell carcinoma (BCC), Merkel cell carcinoma (MCC), pilomatrix carcinoma, spiradenocarcinoma, porocarcinoma, and also a trichoblastic carcinoma [ 6 ].…”

“…Histopathologically, a cCS can be composed of different epithelial and mesenchymal components, of which the first one can be represented by a squamous cell carcinoma (SCC), basal cell carcinoma (BCC), Merkel cell carcinoma (MCC), pilomatrix carcinoma, spiradenocarcinoma, porocarcinoma, and also a trichoblastic carcinoma [ 6 ]. Regarding the sarcomatous component, usually, the cells are undifferentiated, similar to these one constituting Atypical Fibroxanthoma (AFX), with epithelioid and spindle morphology, brisk and atypical mitotic activity, and with foci of necrosis [ 7 ]. Furthermore, the heterologous component can include angiosarcomatous, leiomyosarcomatous, rhabdomyosarcomatous, chondroblastic, or osteoblastic differentiation [ 8 , 9 ].…”