Epithelioma Adenoides Cysticum: Basal Cell Nevi, Agenesis of the Corpus Callosum and Dental Cysts

Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome

“…The incidence of polydactyly (3%) was similar to that obtained by Shanley et al [1994]. Absent or abnormal corpus callosum was seen as an autopsy finding in the first reported case of NBCC [Binkley and Johnson, 1951]. Other associated anomalies such as asymmetric ventricles (not associated with tumors or surgery) and cerebral atrophy have not previously been reported.…”

Radiation‐Induced Basal‐Cell Carcinoma

“…Did this child have a “forme fruste” of NBCCS, or was it like the cases in this report and manifested some hitherto unreported genetic problem? Radiation therapy for jaw cysts in another patient with NBCCS and epithelioma adenoides cysticum resulted in a fibrosarcoma that metastasized to the lungs and bone 25 . In Case 3 in this report, one daughter of the patient had tuberous sclerosis, and although it has not been reported to my knowledge, perhaps both these diseases may share some genetic susceptibility to the development of multiple basal‐cell cancers.…”

The roots of the naevoid basal cell carcinoma syndrome*

“…When I examined the boy, it dawned on me that this instance of multiple skin cancers in father and son was similar to the cases of the mother and daughter reported by Binkley & Johnson (1951). I was familiar with their paper, 'Epithelioma adenoides cysticum: basal cell nevi, agenesis of the corpus callosum and dental cysts', in which they described the developmental defects associated with the skin tumours in one of their patients, the mother.…”