ADENOCARCINOMA COMBINED WITH MALIGNANT PERIPHERAL-NERVE SHEATH TUMOR OF THE GALLBLADDER

Adenocarcinoma of the gallbladder combined with a malignant peripheral nerve sheath tumor (MPNST) in the gallbladder in an 81-year-old woman is reported. The resected gallbladder showed two distinct tumor compo nents, the epithelioid type of MPNST and adenocarcinoma with areas of mucin production. Although the immediate postoperative course was unev entful, a pathologic fracture of her right upper femur developed 4 mon ths after the cholecystectomy. The pathology was determined to be a fe ature of metastatic MPNST rather than of adenocarcinoma. A whole body bone scan revealed multiple metastases, including the left parietal sk ull, left ninth rib, seventh thoracic vertebra, and right upper third of the femur. Despite cholecystectomy and postoperative irradiation th erapy, she died 6 months after diagnosis of the tumor. Without an auto psy the primary site of the MPNST was unknown. We found that the progn osis was very poor in patients with distal metastatic MPNST, especiall y in older patients.