Malignant proliferating trichilemmal tumor (MPTT) is a dermal adnexal tumor originated from the external root sheath of hair follicles. In this article, we reported a rare case of auricular MPTT in a 65-year-old male with gradually enlarging and bleeding mass on the right auricle measuring 30 × 20 × 12 mm in size and detailed the surgical strategy for therapy. The diagnosis of MPTT was established primarily by histopathological observation with abrupt keratinization, cytological atypia, abnormal mitoses, and infiltrating margins. Following extensive excision, no recurrence was noted for 14 months. We also discuss its clinicopathological features, differential diagnoses, and treatment methods and review the relevant literature in this regard.

Malignant proliferating trichilemmal tumor (MPTT) is an infrequent malignant neoplasm originating from cutaneous appendages, with only a handful of documented cases. This report delineates a unique instance of MPTT situated in the neck, accompanied by lymph node metastasis. A comprehensive exposition of its clinical trajectory and imaging manifestation is presented, aiming to enhance comprehension and management of this atypical ailment.

Patient concerns: A 79-year-old male presented with a longstanding right neck mass persisting for over six decades, exhibiting recent enlargement over the past year. Diagnoses: Enhanced magnetic resonance imaging of the neck unveiled an elliptical mass on the right neck side, characterized by an ill-defined border and a heterogeneous signal pattern. The mass exhibited subdued signal intensity on T1-weighted imaging (T1WI) and a heterogeneous high signal on T2-weighted imaging (T2WI), interspersed with a lengthy T1 and T2 cystic signal motif. Close anatomical association with the submandibular gland joint was noted, and intravenous gadolinium diethylene triamine pentaacetic acid administration facilitated conspicuous enhancement. Substantial enhancement of the solid segment prompted an initial preoperative diagnosis of malignant nerve sheath tumor. However, post-surgery histopathological and immunohistochemical analysis conclusively confirmed the diagnosis as malignant hyperplastic external hair root sheath tumor. Intervention: Complete excision of the tumor was successfully executed. Outcomes: The patient experienced a favorable postoperative recovery.

Malignant proliferative trichilemmal tumor external hair root sheath tumor is a cystic-solid lesion, appearing as low signal on T1WI images or high signal on T2WI with enhancement of the solid component. Suspicions of malignancy are heightened when the tumor border is indistinct, tissue planes are breached, or when linear or patchy high signals are observed in the subcutaneous tissue on T1 liver acquisition with volume acceleration enhanced images along with intermediate signal on T2WI and restricted diffusion on diffusion-weighted imaging images. Strong consideration for malignancy should arise if there are signs of compromised adjacent tissue relationships or direct invasion evident on imaging. We have incorporated the above-mentioned content into the entire manuscript.

Malignant proliferating trichilemmal tumor (MPTT) is thought to represent the malignant counterpart of benign proliferative pilar cyst, a lesion originating from the outer hair root sheath. We report a case of an 86-year-old woman with an exophytic nodule containing an ulcerated surface in the temporal region. Histopathologically confirmed MPTT without metastasis, and considering the risk of metastasis and recurrence, we used single local narrow margin excision combined with modified 5-aminolevulinic acid-mediated photodynamic therapy (ALA-PDT). No recurrence of skin lesions was found in the 2-year follow-up after combined therapy. In conclusion, local narrow margin excision sequential with modified ALA-PDT may be a particularly promising and effective treatment option for MPTT.