A case of basal cell nevus syndrome complicated by upper jaw squamous cell carcinoma.

The patient was a 39-year-old male. Family history revealed that his sister and her second son were diagnosed as having jaw cysts. The patient himself underwent cystectomy twice, in December of 1971 and in February of 1986, under the diagnosis of the bilateral jaw cysts of the mandible and maxilla. In May of 1991, a fistula communicating between the left maxillary sinus and oral cavity emerged and within several months the patient lost 10 kg. of body weight. In 1988, he noticed a black spot at the right root of the nose, which enlarged gradually with manifesting bleeding and crust formation. He was referred to us on August 19. An 8×6mm-sized, black nodule was recognized and up to 3mm. diameter similar nodules were also scattered on the other parts of the face. Almost all nodules consisted of multiple, miliary-sized fused nodules. Up to 2mm diameter infundibuliform pits were seen on the palm and planter areas. Under the diagnosis of BCNS, brain X-ray CT was performed. In addition to the calcification of falx cerebri and tentorium cerebelli, a water-dense tumor with bone destruction was disclosed at the left maxillary sinus. The histopathology of the biopsy specimen from the left maxillary sinus was compatible with well-differenti-ated squamous cell carcinoma. On September 28, the partial resection of the light maxillary sinus and resection of the facial neoplasms was performed simultaneously. Six out of nine facial nodules were histologically diagnosed as basal cell epithelioma (solid type) .This case is considered as a typical case of BCNS. It is important to recognize that squamous cell carcinoma can arise from the jaw cysts of BCNS, because it might affect the prognosis of the patients. Only three similar cases have been reported in the world.