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1.
Am J Med Sci ; 363(1): 64-68, 2022 01.
Article in English | MEDLINE | ID: mdl-32732076

ABSTRACT

Adrenal medullary hyperplasia is a cause of increased secretion of catecholamines by the adrenal gland that is rarely considered among the differential diagnoses of endocrine hypertension. We report the case of a 48-year-old Hispanic woman who presented for evaluation of resistant hypertension with several episodes of hypertensive crisis. The clinical presentation, biochemical results, and abdominal computed tomography scan suggested the possibility of a pheochromocytoma; however, an iodine-123-meta-iodobenzylguanidine (123I-MIBG) uptake study combined with single-photon emission computed tomography (SPECT) and computed tomography (CT) scan showed diffusely increased metabolic activity in both adrenal glands. The patient underwent left adrenalectomy, and the pathology study revealed the presence of adrenal medullary hyperplasia. After surgery, blood pressure control was achieved with one antihypertensive drug, and the patient did not have recurrent hypertensive crisis. Relevant findings obtained from a whole genomic sequence done on a whole blood DNA sample from the patient are discussed.


Subject(s)
Adrenal Gland Neoplasms , Hypertension , Pheochromocytoma , Adrenal Gland Neoplasms/pathology , Adrenalectomy , Female , Humans , Hyperplasia , Hypertension/etiology , Middle Aged , Pheochromocytoma/pathology
2.
BMJ Case Rep ; 12(1)2019 Jan 10.
Article in English | MEDLINE | ID: mdl-30635305

ABSTRACT

Somatostatinomas are rare neuroendocrine tumours, mostly located in the pancreas or duodenum, with an estimated incidence of 1 in 40 million. Duodenal somatostatinomas (DSs) are usually found in association with neurofibromatosis type 1 (NF1), tuberous sclerosis and Von Hippel-Lindau syndrome. Gastrointestinal stromal tumours (GIST) have also been described in NF1, but the association with somatostatinoma is very uncommon. We report the case of a patient with NF1 who presented with obstructive jaundice due to multiple firm nodules around the ampulla of Vater. A pancreaticoduodenectomy was performed and revealed a 1 cm duodenal/ampullary mass which stained positive for somatostatin, together with a GIST also found on the duodenal wall. Despite its rarity, ampullary somatostatinomas should be considered in the differential diagnosis of biliary tract dilation in patients with NF1.


Subject(s)
Gastrointestinal Stromal Tumors/complications , Jaundice, Obstructive/diagnosis , Neurofibromatosis 1/complications , Somatostatinoma/pathology , Adult , Aftercare , Diagnosis, Differential , Duodenal Neoplasms/pathology , Duodenal Neoplasms/surgery , Female , Gastrointestinal Stromal Tumors/pathology , Gastrointestinal Stromal Tumors/surgery , Humans , Jaundice, Obstructive/etiology , Jaundice, Obstructive/surgery , Neuroendocrine Tumors/epidemiology , Neurofibromatosis 1/diagnosis , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/surgery , Pancreaticoduodenectomy/methods , Somatostatin/metabolism , Somatostatinoma/complications , Somatostatinoma/surgery , Treatment Outcome
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