Epithelioid sarcoma of the hand: a wolf in sheep's clothing

Epithelioid sarcoma (ES) of the hand is a rare, aggressive cutaneous malignancy with high rates of recurrence, metastases and mortality. With an incidence rate of 0.4 cases/y per one million population, which compromise for approximately 1–1.4% of all soft tissue sarcoma, ES accounts for 10% of soft tissues sarcomas of the hand and foot. Its aggressiveness and propensity to spread and metastases without being noticed, makes it unique and potentially lethal. Missed or delayed diagnosis are often encountered as this tumor can mimic variety of different entities and due to the infrequent nature of this lesion, treatment options are still controversial. The authors provide systemic review of the current literature on epidemiology, etiology, pathogenesis, management and outcomes of this disease as well as a case presentation and a proposed treatment algorithm. The choice of treatment option depends on disease characteristics, staging at presentation, regional lymph node involvement, comorbidities and performance status of the patient. Emphasis on a multidisciplinary coordinated care is crucial as early diagnosis and treatment can decrease morbidity and mortality rates.