Eighty-two cases of neoplasms arising in Paget's disease of bone from the Mid-America Bone Tumor Registry, accessioned between 1958 and 1983, were reviewed. There were 77 osteosarcomas, 3 fibrosarcomas, 1 chondrosarcoma, and 1 giant cell tumor. The male/female ratio was 2:1. The age distribution was 18%, 29%, 36%, and 17% for the groups less than 51 years, 51-60 years, 61-70 years, and greater than 70 years, respectively. The femur was involved by tumor in 22%; the humerus in 21%; the pelvis in 21%; the calvarium in 12%; the tibia in 10%; and other bones in 15% of the cases. There were 48% survivors after 1 year; 17% after two; and 5% after three and five years; two additional patients with tumor lived for more than seven and 11 years. Radiographs available for simultaneous study in 43 cases revealed predominant tumor patterns that were mixed in 69%, osteoblastic in 21%, and osteolytic in 10%. Other radiographic data and the clinical and histopathologic data are in agreement with five earlier major studies. While the prognosis of neoplasms arising in Paget's disease generally is not good, a small fraction of long-term survivors after aggressive therapy makes this complication a challenge for timely radiographic diagnosis and histopathologic confirmation.

A case of multiple hamartoma syndrome (Cowden's disease) associated with renal cell adenocarcinoma and primary neuroendocrine carcinoma of the skin is described. Neither of these neoplasms has been documented previously in association with this genodermatosis. A search for epidermal growth factor receptor (c-erb-B protooncogene) gene abnormalities in the kidney, liver, and thyroid, as well as in tissue of the primary neuroendocrine carcinoma, was negative. Serum obtained from the patient before his death contained elevated levels of both chromogranin A (2641 ng/mL; normal level, less than 20 ng/mL) and calcitonin (517 pg/mL; normal level, less than 200 pg/mL), suggesting that the patient's principal tumor was neuroendocrine in origin.

This report describes a case of homicide by insulin administration and a study of the effects of storage conditions on insulin in serum. The study revealed insulin to be remarkably stable at refrigerator temperatures. Therefore, for forensic science purposes, insulin immunoassay data are interpretable even when serum is not stored by the standard laboratory method of freezing.