Malignant proliferating trichilemmal tumor in neck: A rare clinical presentation and review of 50 cases from literature

Background: Proliferating trichilemmal tumor is a rare cutaneous neoplasm derived from the outer root sheath of the hair follicle which usually presents as a solitary lesion, in fourth to eighth decades of life, and has a predilection for women. The scalp is the most common site of occurrence of this tumor. Malignant proliferating trichilemmal tumor (MPTT) invades surrounding tissues or metastasizes with features of cellular pleomorphism and nuclear atypia. Local recurrences have been occurred in about 3.7%–6.6%. Complete surgical excision with a margin of normal tissue is the standard treatment. Aim: To present a rare case of MPTT over the upper part of the neck which presented to our ENT department and to review about 50 cases of this entity in terms of age, sex, location, treatment, and outcome. Methods: The detailed description of the case of MPTT presented over the upper part of the neck to our ENT department will be given. Literature was reviewed for similar cases of this entity and was studied in terms of age, sex, location of tumor, treatment, and outcome. Results: Out of the 50 cases reviewed, majority of the patients were females, between the age group 40 and 80 years. The scalp was the common site of tumor. All the cases were treated surgically. Conclusion: MPTT is a rare neoplasm which carries a diagnostic dilemma and is often confused with squamous cell carcinoma. Wide excision of MPTT with an adequate clearance margin is the treatment of choice.