Low-grade Malignant Proliferating Pilar Tumor Simulating a Squamous-Cell Carcinoma in an Elderly Female: A Case Report and Immunohistochemical Study

A 65-year-old lady presented with an ulcerated lesion over the occipital region of nine-year duration, an incisional biopsy of which was reported as squamous-cell carcinoma. A wide local excision was performed and the tissue was sent for histopathological examination which revealed a low-grade malignant pilar tumor. Focal invasion and atypia were noted. Immunohistochemical (IHC) analysis revealed positivity for CD34 and calretinin immunomarkers favoring outer root sheath origin. Ki67 immunostains revealed a relatively low immunoreactivity indicating the low-grade nature of the tumor; however, p53 immunostain showed strong diffuse nuclear staining confirming the malignant nature of the tumor. Proliferating pilartumors (PPT) are rare tumors and less than 100 well-documented cases of malignant PPT have been reported so far in the literature. These tumors have been recently classified into benign, low- and high-grade malignant tumors and statistically significant difference was found in their biological behavior. However, we propose that IHC can be of immense value in assisting the subtyping of the tumor, so that the behavior and role of adjuvant therapy can be validated in future studies.