Advanced Basal Cell Cancer: Concise Review of Molecular Characteristics and Novel Targeted and Immune Therapeutics

M. Nikanjam; P. R. Cohen; S. Kato; J. K. Sicklick; R. Kurzrock

Disclosures

Ann Oncol. 2018;29(11):2192-2199. 

In This Article

Clinical Characteristics

Metastatic BCC is considered an ultra-rare malignancy. It accounts for 0.0028%–0.55% of all BCCs[7] with an incidence of approximately 0.35–6.8 per 100 000 per year in the United States. The median age of patients with a primary lesion is around 45 years and metastatic disease appears a median of 9 years after initial diagnosis.[8] Male gender, primary lesions in the head and neck, large or locally invasive lesions, and recurrence after surgery or radiation have been predictive of metastatic spread. Squamous cell carcinomas and immunosuppression may also correlate with the occurrence of metastatic disease.[9] The most common site of metastatic spread is the lymph nodes. For disease limited to the lymph nodes, mean survival has been reported as 87 months; however, spread to other sites such as bone, liver, and lungs can reduce mean survival to 24 months.[7]

Gorlin Syndrome

Nevoid BCC syndrome or Gorlin syndrome is a multisystem, hereditary disorder that has been associated with the development of BCCs. It occurs as a result of loss of germline heterozygosity of the PTCH1 gene in the Hedgehog signaling pathway.[10] The estimated prevalence is 1 in 57 000 to 164 000 people.[11] It is characterized by developmental anomalies and early onset of BCCs and medulloblastomas. The major findings of BCCs, jaw cysts, macrocephaly, and palmoplantar pits occur in 75%–80% of patients.[12] BCCs may not occur until the teens or young adulthood, can vary from a few to thousands, and rarely lead to locally advanced disease or metastatic spread.[13,14]

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