Authors: Vieira Neto, L.¹; Taboada, G.¹; Corrêa, L.¹; Polo, J.¹; Nascimento, A.²; Chimelli, L.³; Rumilla, K.⁴; Gadelha, M.⁵

Source: Endocrine Pathology, Volume 18, Number 1, May 2007 , pp. 46-52(7)

Publisher: Humana Press

Abstract:

Ectopic growth hormone-releasing hormone (GHRH)-secreting tumors are rare and cause acromegaly with somatotroph hyperplasia. We report a case of acromegaly secondary to GHRH secretion by an incidentally discovered pheochromocytoma in a normotensive patient. A 23-year-old man presented with signs and symptoms of acromegaly. Laboratory evaluation confirmed the diagnosis and magnetic resonance imaging (MRI) revealed a sellar mass which was thought to be a macroadenoma and surgically resected. The patient was not cured and medical treatment was indicated. An abdominal ultrasound performed before initiation of medical treatment showed a solid/cystic lesion superiorly to the right kidney. An abdominal MRI confirmed an adrenal tumor. Hormonal workup of the adrenal incidentaloma revealed elevated urinary cathecolamine and total metanephrines findings strongly suggestive of a pheochromocytoma. Acromegaly was then suspected to be due to ectopic secretion of GHRH by the tumor. Patient underwent surgical resection and histopathologic examination confirmed a pheochromocytoma which stained positively for GHRH. Also, review of the pituitary specimen confirmed somatotrophic hyperplasia. Genetic analysis of the ret proto-oncogene showed no mutation. Pituitary MRI was repeated 10 months after pheochromocytoma resection and revealed a slightly enlarged pituitary and partial empty sella. The diagnosis of acromegaly caused by ectopic production of GHRH is a challenging task. A careful histopatological examination of the surgically excised pituitary tissue has a key role to arouse the suspicion and guide the investigation of a secondary cause of acromegaly.

Keywords: acromegaly; GHRH; pheochromocytoma

Document Type: Research article

DOI: http://dx.doi.org/10.1007/s12022-007-0006-8

Affiliations: 1: Endocrinology Section, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 2: Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA, 3: Department of Pathology, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 4: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, USA, 5: Endocrinology Section, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, Email: mgadelha@hucff.ufrj.br

Publication date: 2007-05-01

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