Cutaneous leiomyosarcoma of penile prepuce: A rare case

Cutaneous Leiomyosarcoma (atypical smooth muscle tumor) is an uncommon sarcoma of skin which typically presents in elderly Caucasian males as painful nodule over trunk and lower extremities. In view of its rarity, these cases go underdiagnosed in most of the patients. In this case report, the patient presented with phimosis and painful nodule over prepuce. This rare presentation made the clinical diagnosis even more challenging. Precise histopathology diagnosis with mention on location of lesion is of paramount importance. Based on location of the lesion, cLMS is categorized into three distinct groups with varying clinicopathological behavior and prognosis. The location of the lesion in the skin is the single most important prognostic factor. Pathological grading of tumor does not contribute to prognostication. Although superficial cLMS has good prognosis, dermal leiomyosarcoma has lower risk of local recurrence and distant metastasis than subcutaneous leiomyosarcoma. Cutaneous metastasis of extracutaneous (deep) leiomyosarcoma, which has the worst prognosis and least overall disease free survival, needs to be excluded in all cases of cLMS. Our case report highlights the significance and role of histopathological examination in the diagnosis of a very rare presentation of a rare disease.