A black female with inherited medullary thyroid carcinoma and pheochromocytoma was a mosaic for glucose-6-phosphate dehydrogenase types A and B in normal tissues (blood, thyroid, and adrenal gland); both the medullary carcinoma and pheochromocytoma tissue showed a B pattern only.
We determined the activity of DBH in the plasma of 8 patients with pheos, secondary to multiple endocrine neoplasia Type 2 (MEN II) (medullary carcinoma of the thyroid [MCT], pheochromocytoma(s), and parathyroid hyperplasia).
In contrast, of the 14 members in the other 4 family branches, only 4 have had pathologically elevated plasma calcitonin concentrations following alcohol provocation, probably representing C-cell hyperplasia or very early neoplasia and apparently not progressing.None has evidence of phaeochromocytoma.
This probe hybridizes specifically to a species of poly(A)-RNA from adrenal medulla and human pheochromocytoma, (1400--1450 bases), and also to [Met]enkephalin-containing pro-opiomelanocortin mRNAs from bovine pituitary (1200 bases) and from mouse pituitary tumor cell (1100 bases).
Although PPHP does not have the biochemical features of hypocalcemia and elevated parathyroid hormone levels as seen in pseudohypoparathyroidism, it seems from this case to share the potential for multiple endocrine neoplasia seen in a number of metabolic disorders in which pheochromocytoma may be a prominent manifestation.
The cells which comprise the adrenal nodules resemble those in the parent tumor of the rat PC12 pheochromocytoma cell line in that they show varying degrees of spontaneous or nerve growth factor-induced neurite outgrowth in culture and they contain little or no epinephrine.
An mRNA of approximately 2.3Kb was detected with the cDNA probes in human cell lines from MTC and lung cancers that were shown to produce CgA and in human pheochromocytoma and bovine adrenal medulla tissue.
Different opioid peptides, related to the enkephalins, dynorphins and beta-endorphin have also been detected in tissues outside the CNS including the adrenal medulla and in pheochromocytomas.
Expression of the calcitonin (CT)/calcitonin gene related peptide (CGRP) gene and the proopiomelanocortin (POMC) gene has been demonstrated by Northern blot hybridization analysis of RNA extracted from human medullary thyroid carcinoma (MTC), pheochromocytoma and lung carcinoma.
The length of POMC mRNA in MTCs (present in all 4 metastases investigated but not in 7 primary tumors) and pheochromocytomas is about 100 nucleotides more than pituitary POMC RNA.