Abstract
Pituitary adenomas rarely originate outside the sella turcica. Ectopic locations include the suprasellar region, sphenoid sinus, cavernous sinus and clivus. We describe a 50-year-old female who presented with clinical signs and biochemical evidence of acromegaly. Pituitary MRI demonstrated a 2 mm hypointense lesion on the right side of the pituitary gland. However upon drilling of the upper clival bone to expose the sella during endoscopic transsphenoidal surgery, soft tumor-like tissue was encountered within the clivus. Exploration of the sella, including the area of hypointensity noted on preoperative imaging, did not identify any other abnormality. Immunohistochemical examination of the fully resected tumor demonstrated growth hormone immunoreactivity. Failed preoperative diagnosis of this rare ectopic GH-producing tumor was compounded by the presence of a misleading pituitary abnormality consistent with a microadenoma. The epidemiology and pertinent literature of this uncommon condition is discussed.
References
Melmed S, Colao A, Barken A, Molitch M, Grossman AB, Kleinberg D, Clemmons D, Chanson P, Laws E, Schlechte J, Vance ML, Ho K, Giustina A (2009) Guidelines for acromegaly management: an update. J Clin Endocrinol Metab 94:1509–1517
Colao A, Ferone D, Marzullo P, Lombardi G (2004) Systemic complications of acromegaly:epidemiology, pathogenesis, and management. Endocr Rev 25:102–152
Melmed S (2006) Medical progress: acromegaly. N Engl J Med 355:2558–2573
Faglia G, Arosio M, Bazzoni N (1992) Ectopic acromegaly. Endocrinol Metab Clin North Am 21(3):575–595
Biswal S, Srinivasan B, Dutta P, Ranjan P, Vaiphei K, Singh RS, Thingnam SS (2008) Acromegaly caused by ectopic growth hormone: a rare manifestation of a bronchial carcinoid. Ann Thorac Surg 85(1):330–332
Beuschlein F, Strasburguer CJ, Siegerstetter V et al (2000) Acromegaly caused by secretion of growth hormone by non-Hodgkin’s lymphoma. N Engl J Med 342:1871–1876
Platts JK, Child DF, Meadows P, Harvey JN (1997) Ectopic acromegaly. Postgrad Med J 73(860):349–351
Melmed S, Ezrin C, Kovasc K, Goodman RS, Frohman LA (1985) Acromegaly due to secretion of growth hormone by ectopic pancreatic islet-cell tumor. N Engl J Med 312:9–17
Thorner MO, Perryman RL, Croinin MJ et al (1982) Somatotroph hyperplasia: successful treatment of acromegaly by removal of a pancreatic islet tumor secreting a growth hormone-releasing factor. J Clin Invest 70:965–977
Hori A, Schimidt D, Rickels E (1999) Pharyngeal pituitary: development, malformation and tumorigenesis. Acta Neuropathol 98:262–272
Mitsuya K, Nakasu Y, Nioka H, Nakasu S (2004) Ectopic growth hormone-releasing adenoma in the cavernous sinus—case report. Neurol Med Chir (Tokyo) 44:380–385
Kurowska M, Tarach JS, Zgliczuski W, Malicka J, Zieliuski G, Janczarek M (2008) Acromegaly in a patient with normal pituitary gland and somatotropic adenoma located in the sphenoid sinus. Pol J Endocrinol 59(4):348–351
Guerrero CA, Krayenbuhl N, Husain M, Krisht AF (2007) Ectopic Suprasellar growth hormone secreting pituitary adenoma: case report. Neurosurgery 61:E879
Hori E, Akai T, Kurimoto M et al (2002) Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. J Clin Neurosci 9:196–199
Gondim JA, Schops M, Ferreira E et al (2004) Acromegaly due to an ectopic pituitary adenoma in the sphenoid sinus. Acta Radiol 45:689–691
Matsuno A, Katayama H, Okazaki R et al (2001) Ectopic pituitary adenoma in the sphenoid sinus causing acromegaly associated with empty sella. ANZ J Surg 71:495–498
Chan MR, Ziebert M, Maas DL et al (2005) My rings won’t fit anymore. Ectopic growth hormone-secreting tumor. Am Fam Physician 71:1766–1767
Madonna D, Kendler A, Soliman AM (2001) Ectopic growth hormone-secreting pituitary adenoma in the sphenoid sinus. Ann Otol Rhinol Laryngol 110:99–101
Choin JH, Park MK, Choi SS, Kim KU (2006) Ectopic growht hormone-secreting pituitary adenoma of the clivus. J Korean Neurosurg Soc 39:306–309
Bhatoe HS, Kotwal N, Badwal S (2007) Clival pituitary adenoma with acromegaly: case report and review of literature. Skull Base 17:265–268
Corenblum B, Le Blanc FE, Watanable M (1980) Acromegaly with an adenomatous pharyngeal pituitary. JAMA 234:1456–1457
Erdheim J (1909) Uber einen hypophysen tumor van ungewohnlichem sitz. Beitr Pathol Anat 46:223–249
Ballaux D, Verhelst J, Pickut B, De Deyn PP, Mahler C (1999) Ectopic macroprolactinoma mimicking a chordoma: a case report. Endocr Relat Cancer 6:122–177
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Appel, J.G., Bergsneider, M., Vinters, H. et al. Acromegaly due to an ectopic pituitary adenoma in the clivus: case report and review of literature. Pituitary 15 (Suppl 1), 53–56 (2012). https://doi.org/10.1007/s11102-011-0345-9
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DOI: https://doi.org/10.1007/s11102-011-0345-9