Adrenal medullary hyperplasia with coexistent cerebral angiomas

Sporadic adrenal medullary hyperplasia (AMH) is a rare entity and mimics pheochromocytoma clinically as well as pharmacologically. It is characterized by increase in adrenal medullary cells with the expansion of cells into areas normally not seen. A 59-year-old male presented with chronic hypertension and raised 24-h urinary normetanephrine levels. Radiological and clinical possibility of pheochromocytoma led to left transperitoneal laparoscopic adrenalectomy. Histopathology, however, showed increase in adrenal medullary to cortical ratio, further confirmed by immunohistochemistry. The absence of any well-defined lesion led to the diagnosis of AMH. Furthermore, on routine imaging, two asymptomatic cavernous hemangiomas were seen. We present this case to reiterate that AMH is an entity which should be considered as a differential for pheochromocytoma. Furthermore, the presence of asymptomatic cavernous hemangiomas in the cerebrum, in this case, makes it rarer since this sporadic association is seldom seen.

Adrenal Medullary Hyperplasia with Coexisting Adrenal Cortical Adenoma / 대한내분비외과학회지

Adrenal medullary hyperplasia is an increase in the mass of the adrenal medullary cells. We report a case of a 38-year-old man presenting with pheochromocytoma-like symptoms who was preoperatively misdiagnosed with pheochromocytoma. Hypertension was associated with an intracranial hemorrhage evident in a brain computed tomography scan, in which no obvious pituitary gland enlargement was detected. An abdominopelvic CT revealed a solitary tumor in the right adrenal gland with no obvious enlargement of the contralateral adrenal gland or sympathetic chains. Lab results showed increased levels of urinary metanephrines. Based on clinical data, the patient underwent a laparoscopic right adrenalectomy bases on a diagnosis of pheochromocytoma. The patient was finally diagnosed with adrenal medullary hyperplasia with coexisting ipsilateral non-functioning adrenal cortical adenoma. Postoperatively, blood pressure and lab results were maintained in the normal range and the patient was symptomatically free during the follow-up period.

An Adrenal Cortical Adenoma with Adrenal Medullary Hyperplasia / 대한내과학회지

The relationship between the adrenal cortex and medulla has been studied since the 1960s. Rarely, a patient with an adrenal cortical adenoma presents with the findings of pheochromocytoma. However, there has been no report of a case with the clinical features of pheochromocytoma showing the pathological features of an adrenal cortical adenoma with medullary hyperplasia on histological examination. We report a 59-year-old-man who was shown to have an adrenal cortical adenoma, with medullary hyperplasia, during a diagnostic work up for pheochromocytoma.

Hypercatecholaminism(report of 95 cases) / 中华泌尿外科杂志

Objective To evaluate the improvement of diagnosis and treatment of hypercatecholaminism. Methods A total of 95 cases of hypercatecholaminism were reviewed.Of them 74 cases were of pheochromocytoma and 21 cases were of adrenal medullary hyperplasia (AMH).In pheochromocytoma group,67 cases (90.5%) had symptom of hypertension.Increase in 24 h urinary VMA occurred in 62 cases (83.8%) and elevated urinary catecholamine level in 67(90.5%).All thses had positive findings of ultrasound,CT scan and MRI.In AMH group,all the patients were hypertensive and their 24 h urinary VMA was increased.Most of them (15/16) had higher urinary catecholamine level than normal;14 out of the 21 cases had positive findings of B-ultrasound examination. Results Surgical operations were performed in the 74 patients with pheochromocytoma including 9 cases of extra-drenal pheochromocytoma.Of them 5 cases were confirmed as malignant pheochromocytoma.In the 21 cases of AMH,18 undewent surgical operation.Of them 12 received unilateral resection and 6 received bilateral resection of adrenal gland.The pathological examination confirmed the diagnosis of AMH. Conclusions The diagnosis of pheochromocytoma should be focused on endocrinological examination,ie,qualitative examination,whereas it is diffucult to localize the lesion in AMH compared with pheochromocytoma.The establishment of notion of AMH as an isolated clinicopathological entity broadens and perfects the theory of hypercatecholaminism.

ADRENAL MEDULLARY HYPERPLASIA: CLINICAL ANALYSIS OF 4 CASES / 解放军医学杂志

Adrenal medullary hyperplasia (AMH) is a rare cause of secondary hypertension. Herein we analysed clinical manifestation of 4 cases. The clinical and biochemical features of AMH were similar to those of pheochromocytoma. 131 I MIBG scintigram and CT scanning were helpful to make differential diagnosis. Etiology, diagnosis and treatment of AMH were discussed.