Giant Pilomatrix Carcinoma of the Thorax: An Uncommon and Clinically Misdiagnosed Tumor

Abstract

Summary:. Pilomatrix carcinoma is an unusual and aggressive malignant tumor deriving from follicular matrix cells and exhibiting a metastatic potential to lungs and regional lymph nodes in 10% of cases. We report the case of a 54-year-old male patient who presented with a biopsy-proven malignant pilomatrixoma of the thoracic region, which recurred multiple times after previous excisions. Due to the size of the tumor (28 by 22 cm), wide excision and axillary lymphadenectomy of levels I and II were performed, followed by reconstruction with a free deep inferior epigastric artery perforator flap and adjuvant radiotherapy. Owing to its rarity, this tumor can initially be misdiagnosed, resulting in delayed treatment and recurrences if inadequately excised. Also, large tumor size is correlated with a higher incidence of metastasis. High index of clinical suspicion and wide excision are recommended, along with the need of establishing oncological guidelines for better prognosis.