Metastatic insidious super giant basal cell carcinoma

Abstract

A middle-aged woman presented with a mechanical fall. Physical examination revealed a massive purulent ulcerated lesion spanning her entire back and axilla, which had reportedly been brewing for over a decade. Punch biopsy revealed infiltrative basal cell carcinoma. She was treated with Vismodegib, with a remarkable decrease in the size of the cancer. However, surveillance imaging after 6 months of treatment revealed new bone metastases. In the setting of progressive disease, medical therapy was switched to cemiplimab instead. Basal cell carcinoma is commonly known as a benign tumour of the skin, rarely larger than 5 cm. Here, we discuss the entity of metastatic super giant basal cell carcinoma; this case is one of the largest reported lesions. As locoregional therapy, such as surgery is sometimes not appropriate for such advanced lesions, we discuss the current forefront of therapy including oral hedgehog pathway and check point inhibitors.

Super giant basal cell carcinoma: a comprehensive systematic review

“…The American Joint Committee on Cancer classification defines BCCs as T1, if they are 2 cm or less in greatest diameter, T2, if they are more than 2 cm but not more than 5 cm in greatest diameter, and T3 or giant BCCs, if they are 5 cm or more in greatest diameter 1 . SGBCC described with diameter of larger than 20 cm, are known to be an aggressive yet extraordinarily rare oncological entity 8 , 9 .…”

“…Additionally, physical or mental disability may impede healthcare access and the disease progresses unnoticed 23 . Severe autism, schizophrenia, self-neglect, lack of insurance, inadequate treatment of previous tumors, chronic alcoholism, and avoidance of medical care have been reported 1 , 4 , 8 , 9 , 20 – 24 . In some of these cases, patients’ noncompliance has made management challenging 20 .…”

“…Our study found that the average age of presentation is in the sixth decade of life, with an average duration of evolution is greater than 10 years and it is more prevalent in Caucasian males. In African Americans, it is virtually nonexistent 9 . In the absence of any identifiable specific histologic factors in the reported specimens, we believe self-neglect is the likely etiology of the large size of these lesions.…”

“…Because of the rarity of such extensive lesions, there is no consensus on treatment 2 , 9 . When possible, the surgical approach is the treatment of choice 4 , 19 .…”

“…The literature links GBCC with male sex, alcohol use, poor socioeconomic status, and delay in access to medical attention 5 – 7 . On the other hand, Super Giant Basal Cell Cancer (SGBCC), described with a diameter of larger than 20 cm, is known to be an aggressive oncological entity, yet is extraordinarily rare 8 , 9 . Our goal was to conduct a comprehensive review of existing literature and help characterize SGBCC, specifically with regards to age, sex predilection, risk factors, geographical location, body site, metastasis, and treatment.…”