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SMJ // Article

Case Report

Coincidental Consort Clear Cell Cutaneous Carcinom: Facial Squamous Cell Carcinoma In Situ Containing Human Papillomavirus and Cancer Cells with Clear Cytoplasm in an Octogenarian Couple

Authors: Philip R. Cohen, MD, Keith E. Schulze, MD, Peter L. Rady, MD, PhD, Stephen K. Tyring, MD, PhD, MBA, Qin He, MD, Paul T. Martinelli, MD, Bruce R. Nelson, MD

Abstract

Clear cell squamous cell carcinoma in situ, also referred to as pagetoid or clear cell Bowen disease, is a rare pathologic variant of this neoplasm. It is characterized by neoplastic cells with clear or pale cytoplasm. An octogenarian husband and wife concurrently developed new facial skin lesions which demonstrated squamous cell carcinoma in situ consisting of cancer cells with clear cytoplasm. Cutaneous human papillomavirus (HPV) typing detected HPV Type 5 and HPV Type 21 in the tumors of the husband and wife, respectively. HPV is a potential etiologic factor in the oncogenesis of nonmelanoma skin cancer, and HPV DNA has been demonstrated in extragenital squamous cell carcinoma in situ. The detection of DNA from different HPV types in the tumors of our patients suggests that the concurrent occurrence of their skin cancers may have been coincidental. However, the presence of HPV DNA in their tumors introduces the possibility of a viral-associated oncogenesis for clear cell squamous cell carcinoma in situ.


Key Points


* Clear cell squamous cell carcinoma in situ, characterized by neoplastic cells with clear or pale cytoplasm, is a rare pathologic variant of this neoplasm.


* The pathologic differential diagnosis of clear cell squamous cell carcinoma in situ includes extramammary Paget disease, mammary Paget disease, benign and malignant tumors characterized by intraepidermal pagetoid cells, and other clear cell neoplasms of the skin.


* Human papillomavirus is a potential etiologic factor in the oncogenesis of nonmelanoma skin cancer.


* Human papillomavirus DNA has been demonstrated in extragenital squamous cell carcinoma in situ.


* The detection of HPV DNA in our patients’ clear cell squamous cell carcinomas in situ introduces the possibility that a viral-associated oncogenesis may contribute to the development of this pathologic variant of skin cancer.

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