Extra-anogenital giant cutaneous squamous cell carcinomas require multidisciplinary management.

Cutaneous squamous cell carcinoma (cSCC) is a highly prevalent cancer and the majority of cSCC have a good prognosis. However, a subset of cSCC can progress to advanced disease. We present the first reported case of a giant cSCC located on the breast. In addition, a systematic literature search on extra-anogenital giant (EAG) cSCC was performed using Pubmed and MEDLINE databases. Thirty-one articles could be retained which were relevant for this review. A total of 42 well-described cases were identified. Median age at presentation was 70 years (range 9-100 years). Twenty-four (57%) patients were male, eighteen were female (43%). The estimated median delay of treatment was 12 months (range 1 to >240 months). In 27 patients (64%) the giant cSCC was localized on the neck, face or scalp, 6 on the thoracic wall or back (14%), 4 on the lower limb (10%), 2 on the hip or buttock (5%), 2 on the upper limb (5%), one (2%) on the breast. Tumor stage at presentation was T2, T3 and T4 in respectively 26 (62%), 11 (26%) and 5 (12%) cases. Lymph node metastases were identified in 1 patient (2%) and distant metastases in another patient (2%). In 34 out of 42 cases (81%) primary radical surgical excision was performed, 3 received radiotherapy, 2 chemotherapy and 3 palliative care. In the cases with reported follow-up, four patients (4/30: 13%) died of disease. The treatment of EAG cSCC poses many problems, making a multidisciplinary approach of paramount importance.