Abstract
In more than 95% of cases acromegaly is due to GH hypersecretion by a pituitary adenoma. GHRH hypersecretion accounts for about 0.5% of cases of acromegaly. Intracranial GHRH-secreting tumors are extremely rare and only a few well-documented cases have been reported. The clinical features of acromegaly due to intracranial GHRH-secreting tumor are indistinguishable from those of other patients with “classical acromegaly”. In cases of intrasellar gangliocytomas, not even radiological findings help to make the correct diagnosis, which can only be made with the hystological study. We present the case of a woman with acromegaly; the magnetic resonance demonstrated a 2×1.8×1.2 cm mass in the jugum sphenoidalis region, associated with a partial empty sella. There was a partial response to high-dose lanreotide therapy, so surgical treatment was decided, although only part of the tumor could be removed. Histopathological diagnosis was consistent with gangliocytoma, and immunostaining in the ganglionic cells was positive for GHRH. After surgery, hormone hypersecretion persisted, so medical treatment was reintroduced. In summary, we report a well-documented case of an intracranial GHRH-secreting gangliocytoma, an exceedingly rare cause of acromegaly. Clinical and biochemical data did not allow to make the correct diagnosis, which was only made on the pathological study. This case underscores that acromegaly can be due to causes other than a GH-secreting adenoma, and underlines that finding an image not typical of a pituitary adenoma should raise the suspicion that an unusual cause subsides the acromegaly.
Similar content being viewed by others
References
Reichlin S. Functional aspects of endocrine neoplasms. In: Kovacs K, Asa SL eds. Functional endocrine pathology. Boston: Blackwell Scientific Publications. 1991, 898–923.
von Werder K, Losa M, Stalla GK, et al. Measurement of human growth hormone- and corticotropin-releasing hormone. In: Faglia G, Beck-Peccoz P, Ambrosi B eds. Pituitary adenomas: new trends in basic and clinical research. Amsterdam: Elsevier Science BV. 1991, 215–29.
Faglia G, Arosio M, Bazzoni N. Ectopicacromegaly. Endocrinol Metab Clin North Am 1992, 21: 575–95.
Losa M, von Werder K. Pathophysiology and clinical aspects of the ectopicGH-releasing hormone syndrome. Clin Endocrinol (Oxf) 1997, 47: 123–35.
Asa SL, Scheithauer BW, Bilbao JM, et al. A case for hypothalamic acromegaly: a clinicopathological study of six patients with hypothalamic gangliocytomas producing growth hormone releasing factor. J Clin Endocrinol Metab 1984, 58: 796–803.
Kurosaki M, Saeger W, Ludecke DK. Intrasellar gangliocytomas associated with acromegaly. Brain Tumor Pathol 2002, 19: 63–7.
Geddes JF, Jansen GH, Robinson SF, et al. “Gangliocytomas” of the pituitary: a heterogeneous group of lesions with differing histiogenesis. Am J Surg Pathol 2000, 24: 607–13.
McCowen KC, Glickman JN, Black PM, Zervas NT, Garber JR. Gangliocytoma masquerading as a prolactinoma. Case report. J Neurosurg 1999, 91: 490–5.
Sano T, Asa SL, Kovacs K. Growth hormone-releasing hormone-producing tumors: clinical, biochemical, and morphological manifestations. Endoc Rev 1988, 9: 357–73.
Puchner MJ, Ludecke DK, Saeger W, Riedel M, Asa SL. Gangliocytomas of the sellar region- a review. Exp Clin Endocrinol Diabetes 1995, 103: 129–49.
Luna V, Morales F, Luengo LM, Sanz A, Diaz J. Pituitary gangliocytoma-adenoma presenting with acromegaly: response to treatment. Arch Intern Med 2001; 161: 1010–1.
Cavalla P, Schiffer D. Neuroendocrine tumors in the brain. Ann Oncol 2001, 12(Suppl. 2): S131–4.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Isidro, M.L., Díaz, P.I., Matías-Guiu, X. et al. Acromegaly due to a growth hormone-releasing hormone-secreting intracranial gangliocytoma. J Endocrinol Invest 28, 162–165 (2005). https://doi.org/10.1007/BF03345360
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF03345360