Background: Proliferating trichilemmal tumors (PTTs) are rare benign neoplasms that arise from the outer sheath of a hair follicle. Group III PTTs are high-grade malignant tumors reported to exhibit a high recurrence rate, lymph nodes, and a tendency to develop distant metastasis. Case Presentation: A 25 years old female patient was presented to us with a history of recurring soft tissue scalp lesions. The lesion recurred three times within 1 year. There was no history of trauma. Every time the lesion was excised but no histopathology of the specimen was done. Upon the third recurrence, it came out to be a malignant proliferating trichilemmal tumor (MPTT). Considering the young age of the patient and keeping in view the history of multiple recurrences with positive deep margin even after the third surgery, the patient was planned external beam radiotherapy (RT) 50 Gy/25 fx to scalp with 9 Mev electron using 0.5 cm bolus. The post-RT follow-up currently of 2 years is uneventful. Conclusion: The mainstay of treatment is surgery with wide surgical margins as local recurrence is most common in MPTT. Adjuvant RT was used in this case to prevent recurrence.
Keywords: Proliferating trichilemmal tumors, scalp lesion.