Adrenal Medullary Hyperplasia: Hyperplasia‐pheochromocytoma Sequence

Abstract

We present a case of unilateral adrenal medullary hyperplasia in a 63-year-old woman with clinical signs and symptoms of pheochromocytoma unassociated with multiple endocrine neoplasia. The surgically removed adrenal gland revealed diffuse medullary hyperplasia with multiple micronodules measuring up to 2 mm. The micronodules were composed of enlarged chromaffin cells with atypia, histologically similar t o those of pheochromocytoma, forming small solid alveolar patterns separated by a fibrovascular stroma. Removal of the hyperplastic adrenal gland resulted in disappearance of paroxysmal nocturnal hypertension and palpitation. These results suggest that diffuse and nodular medullary hyperplasia is the precursor of pheochromocytoma. Acta Pathol Jpn 40 : 683-686,1990.A-63-year-old woman presented with a 3-year history of paroxysmal nocturnal hypertension and palpitation. Because she was a nurse, she had examined her own blood pressure during the nocturnal attack, and found it had risen to 200 mmHg. She had no positive family history of MEN. Ultrasonography and computed tomography (CT) revealed no morphological abnormalities of the thyroids and parathyroids, except for a small adenomatous goiter. The normal blood values of calcium (4.6 mEq/l), calcitonin (80 pg/ml) and carcinoembryogenic antigen (CEA) (3.3 ng/ml) also ruled out the possibility of MEN.Laboratory data under asymptomatic conditions were as follows: slight elevation of plasma norepinephrine

Laparoscopic Adrenalectomy for Nonfamilial Adrenal Medullary Hyperplasia

“…1 Attempts at proving the existence of AMH as a clinicopathologic entity have appeared since 1933, but perhaps the most convincing example based on clinical and postoperative grounds is that reported by Montalbano et al in 1962. 2,3 This clinicopathologic entity has been widely reported to be associated with familial or type 2 MEN syndrome. 2,4 In most cases AMH—usually bilateral—is a component of type 2 MEN together with medullary thyroid cancer and parathyroid hyperplasia.…”

“…2,3 This clinicopathologic entity has been widely reported to be associated with familial or type 2 MEN syndrome. 2,4 In most cases AMH—usually bilateral—is a component of type 2 MEN together with medullary thyroid cancer and parathyroid hyperplasia. 5 Up to now, sporadic AMH has been documented in a relatively small number of cases.…”

“…1 Carney et al 2 described the likely sequence of events during AMH. Initially, hypertrophy and hyperplasia of the medullary cells occur, which cause a slight increase in the volume of the medulla without a significant alteration in weight.…”

“…2 Some authors consider the diagnosis of AMH only when there is a significant decrease in the corticomedullary ratio in the head and body of the gland with or without extension of the medulla into the tail. 1 …”

The value of plasma markers for the clinical behaviour of phaeochromocytomas

“…Notably, all of these latter cases had normal urinary CAT metabolites as well, but PCCs were more or less incidentally diagnosed by CT and MIBG scans in three sporadic cases and during family screening in three MEN2 cases. In four of these six cases, tumours were smaller than 1 cm, this has been arbitrarily referred to as micronodular medullary hyperplasia, the PCC-precursing lesion in the adrenal medulla in MEN2 (35) and sporadic (36,37) cases. The following correlations of plasma markers and clinicopathological features of PCCs could be established.…”