An unusual pheochromocytoma associated with an androgen secreting adrenocortical adenoma. Evaluation of its polypeptide hormone, catecholamine, and enzyme characteristics

Abstract

Our patient had a left suprarenal mass. His blood pressure was normal, but his urinary catecholamines (CA), vanillylmandelic acid (VMA), total metanephrines (TMn) and 5-hydroxyindolacetic acid (SHIAA) were elevated. In addition, he had elevated, nonsuppressible urinary 17-ketosteroids (17KS) and androsterone, but his urinary 17-hydroxycorticoids (170HCS) and free cortisol were normal, as were his plasma cortisol and ACTH. After resection of the suprarenal mass, the patient's urinary hormone values reverted to normal. The mass contained a pheochromocytoma and an adrenocortical adenoma. The pheochromocytoma was unusual in that it contained very little norepinephrine (NE) and dopamine (DA) and an abundance of epinephrine (E) despite normal enzyme concentrations. Electron micrographs showed primarily E granules with few of the NE-type. The immunoperoxidase histochemical stains for vasoactive intestinal peptide (VIP) and serotonin (S) were strongly positive. The patient's blood pressure may have been normal because his pheochromocytoma secreted E, VIP, or S. The associated adrenocortical adenoma produced no symptoms and was probably coincidental.

Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabdomyosarcomatous differentiation in a patient without von Recklinghausen disease

“…The exact incidence of composite phaeochromocytoma is not known due to the small number of cases reported 2. The mixed phaeochromocytomas, on the other hand, include tumours with different histological elements having no common embryological derivation like phaeochromocytoma with adrenal cortical tumour or spindle cell sarcoma reminiscent of hemangiopericytoma 3 4…”

“…The non-chromaffin element may have a common embryological progenitor as phaeochromocytoma (composite phaeochromocytoma) or be an unrelated tumour (mixed). The category of composite phaeochromocytoma includes admixture of phaeochromocytoma with ganglioneuroma, ganglioneuroblastoma, neuroblastoma or malignant schwannoma, while mixed category includes those containing adrenal cortical tumour or hemangiopericytoma 2 – 4. No case of a composite phaeochromocytoma with components of malignant peripheral nerve sheath tumour and rhabdomyosarcoma (malignant Triton tumour) was found in the available literature following an extensive search.…”

Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report

“…Regarding manifestation, the coexistence of catecholamine-secreting tumors and adrenal cortical tumors can manifest as ipsilateral or contralateral tumors, and each tumor can manifest as unilateral or bilateral. From the literature reported after 1985, there were 38 cases of pheochromocytoma coexisting with adrenal cortical tumors, including 13 cases of hyperaldosteronism [18][19][20][21][22][23][24][25], 8 cases of Cushing's or subclinical Cushing's tumors [14,18,[26][27][28], 1 case of hyperandrogenism [29], 6 cases of nonfunctioning adrenal tumors [30][31][32][33][34], and 10 cases of unknown functional entities [18,35]. Furthermore, there have been only 4 cases of PGL coexisting with unilateral adrenal cortical tumor, including 3 cases of hyperaldosteronism [23,36,37] and one case of adrenal cortical carcinoma [38].…”

Feocromocitoma

“…Em um dos pacientes, o tumor cortical era produtor de andrógenos, e nos outros dois o tumor não era funcionante. Associações desse tipo, embora já tenham sido relatadas na literatura, são raramente descritas (34)(35)(36).…”