Hereditary multiple fibrofolliculomas with trichodiscomas and acrochordons.

In a sibship of nine, six members had hereditary medullary carcinoma of the thyroid. Two of those with thyroid neoplasms and two without had numerous small papular skin lesions. These proved to be a type of pilar tumor that we named fibrofolliculoma. Further investigation of the total kindred of 70 showed no other evidence of thyroid neoplasm. Skin tumors only appeared after the age of 25 years. Fifteen of 37 members older than the age of 25 years exhibited the typical skin lesions. Obviously, the original sibship was the repository of two dominantly inherited traits. The fibrofolliculoma is characterized by abnormal hair follicles with epithelial strands extending out from the infundibulum of the hair follicle into a hyperplastic mantle of specialized firbrous tissue. Associated skin lesions in this kindred were trichodiscomas and acrochordons.

Effects of phosphorus supplementation on serum parathyroid hormone and bone morphology in osteoporosis.

The effect of phosphorus (inorganic phosphate) supplementation was studied in seven postmenopausal women with osteoporosis. Prior to supplementation, all chemical parameters studied in serum and urine were normal. Bone density was below the fifth percentile for age in all but one patient, and the percentage of bone surface involved in resorption was higher than normal. During administration of the phosphorus supplement, fasting serum concentrations of calcium and immunoreactive parathyroid hormone showed no significant changes, while serum phosphorus, urinary calcium, and tubular reabsorption of phosphorus decreased. In four patients studied by balance techniques, calcium balance became positive or less negative. Bone-forming surface decreased and bone-resorbing surface increased in all patients. Bone-resorbing surface was highly correlated with total phosphorus intake. Density of the distal radius changed variably, while density of the midradius increased slightly in all patients.

Comparative ultrastructure of thyroid, tongue and eyelid lesions in the neuroma phenotype of medullary carcinoma of the thyroid: association of amyloid with fibroblasts in thyroid tumor and in mucosal neuromas.

Electron-microscopic and histochemical studies of thyroid tumor, tongue neuromas and eyelid neuromas from the lesions of a patient with medullary thyroid carcinoma were compared. In the thyroid tumor, a significant number of the C cells showed heterogeneity of granule types; no C cells, however, were identified in the tongue and eyelid neuromas. Amyloid was clearly shown by Congo red staining in the thyroid neoplasm and in the tongue neuromas. In all tissues, amyloid fibrils were found to be ultrastructurally closely associated with fibroblasts. These fingings suggest that the fibroblast rather than the C cell may have played the important role in the deposition of amyloid in this patient's thyroid carcinoma.

Hypercalcemia in small cell (oat cell) carcinoma of the lung. Coincident parathyroid adenoma in one case.

Hypercalcemia is very uncommon in small cell (oat cell) carcinoma of the lung. Two cases of this neoplasm associated with symptomatic hypercalcemia are described. Despite normal skeletal roentgenograms, metastatic bone disease was demonstrated by abnormal bone scans and bone biopsies in both patients. The combination of conventional antihypercalcemia therapy, cytotoxic cancer chemotherapy, and synthetic salmon calcitonin corrected the hypercalcemia despite progression of the small cell carcinoma. One patient with elevated serum immunoreactive parathyroid hormone (PTH) had a parathyroid adenoma at autopsy. This association emphasizes that in cases of bronchogenic small cell carcinoma with hypercalcemia, conincidental primary hyperparathyroidism should be considered.