Two Cases of Nevoid Basal Cell Carcinoma Syndrome

Abstract

Two cases of nevoid basal cell carcinoma syndrome were reported with a review of pertinent literature. The first case was a 59‐year‐old man, whose autopsy was warranted. Signs and symptoms manifested in this case were basal cell carcinoma, generalized multiple nevi, multiple cysts in the jaws and long bones, pits in the palm and sole, frontal and occipital bossing, ossification of the falx cerebri, a bifid rib, renal fibroma and a patent foramen ovale. The family history revealed a hereditary predisposition as to the syndrome. The patient in the second case was a 25‐year‐old man who is still alive. Manifestations in this case included basal cell carcinoma, multiple nevi, multiple jaw cysts, pits in the palm and sole, frontal bossing, calcification of the falx cerebri, cervical vertebral fusion and high‐arched palate. ACTA PATH. JAP. 27: 713 ˜ 727, 1977.

Complete ossification of the human falx cerebri

“…Some medical disorders seem to have a higher than normal incidence of falx cerebri ossification. These include endocrine disorders, basal cell nevus syndrome, brachyolmia Maroteaux type, hypertelorism, and pseudoxanthoma elasticum (Satoh et al, 1977;Shohat et al, 1989;Cohen et al, 1995). Our specimen did not have a history of any of these disorders or findings.…”

A partially ossified falx cerebri

“…It has been reported that patients with nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome), hypertelorism, brachyolmia, pseudoxanthoma elasticum, renal failure, chondrocalcinosis, prostatic adenocarcinoma, and some endocrine disorders have an increased prevalence of FC ossification [4,7,8,20,29,30].…”