Malignant adnexal carcinomas of the skin are rare but associated with high propensity for local recurrence, and for some of the distinct subgroups they are known to metastasize regionally or distant. Biopsy is necessary for correct diagnosis, as the lesions cannot be separated from other tumour types entirely on their clinical appearance. The histologic diagnosis is troublesome, and the lesions are often mistaken for their benign counterpart, basal cell carcinoma or squamous cell carcinoma. The lesions are treated with surgery. Radiotherapy and chemotherapy may play a role in treatment, although the evidence is limited.
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Virchows Arch
January 2025
CARADERM Network, Tours, France.
Trichogerminoma (TG) is a rare adnexal tumor with hair follicle differentiation with less than 50 cases reported in the literature. In 2022, by investigating the genetic profile of 21 cases, our group identified recurrent rearrangements of the GRHL1/2/3 genes in this tumor entity, suggesting such alteration as the main oncogenic driver in TG. Up to now, only one TG case of malignant transformation has been reported.
View Article and Find Full Text PDFAm J Dermatopathol
February 2025
Departments of Dermatology and Pathology, School of Medicine, Wake Forest University, Medical Center BLVD, Winston Salem, NC.
Primary vulvar carcinomas are rare and constitute a diverse group of neoplasms. These primary tumors are typically classified based on their presumed tissue of origin or histological characteristics. Among these, carcinomas of sweat gland origin are particularly significant.
View Article and Find Full Text PDFIndian Dermatol Online J
December 2024
Department of Dermatology, Father Muller Medical College, Mangalore, Karnataka, India.
Microcystic adnexal carcinoma (MAC) is a rare, slow-growing, locally aggressive malignant, and recurring appendageal tumor. Prolonged UV exposure, immunosuppression, and radiotherapy are a few frequently associated risk factors. MAC classically presents as an asymptomatic skin coloured plaque on the face.
View Article and Find Full Text PDFVirchows Arch
January 2025
Department of Pathology, Université de Tours, Centre Hospitalier Universitaire de Tours, 37044, Tours, France.
A case of cutaneous adnexal neoplasm with unusual squamoid morphology and harboring an in frame ACTB::ZMIZ2 fusion transcript was recently described. Herein, we report a second case of adnexal carcinoma harboring similar morphology and an identical in frame ACTB::ZMIZ2 fusion transcript. This 2.
View Article and Find Full Text PDFArch Dermatol Res
January 2025
Premier Dermatology, Ashburn, VA, USA.
Pilomatrix carcinoma (PC) is a rare malignant adnexal tumor originating from follicular matrix cells primarily impacting Caucasian males. This review provides a comprehensive analysis of scientific literature on PC through an exploration of 206 cases reported between 1980 and 2024. We discuss the epidemiology, clinical presentation, histopathology, and diagnostic challenges of PC, and explore various treatment methods for this rare malignancy as well as their associated outcomes.
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