Association between Acromegaly and Chordomas of the Skull Base. Case Report and Review of the Literature

Acromegaly is a disease described amongst the neuroendocrinologic syndromes derived from pituitary adenomas. It has been shown that acromegaly increases the risk for developing other types of tumors like colorectal, breast, and thyroid tumors. Based on its physiopathologic mechanism, the use of hormone replacement therapy with RhGH analogues in adults has suggested a role of the Growth Hormone in the pathogenesis of different epitelial tumors and leukemia.However, there is still lacking evidence that supports its role as a significant risk factor. Also, there are other patients whose tumors may progress in spite of RhGH treatment with a higher rate of incidence of new tumors like chordomas,endocrinologically silent pituitary adenomas, growth hormone secreting tumors, Meningiomas and craniopharyngiomas.

We report the case of a female patient, 57 years old, with a prior history of headache and physical changes compatible with acromegaly secondary to an invasive pituitary macroadenoma shown in MR imaging. The lesion eroded the sellar floor and occupied the dorsum sellae and extended to the prepontic and suprasellar cisterns with compression of adjacent structures. We performed an endoscopic transesphenoidal approach with which we obtained a subtotal resection and the inmunohistochemistry and pathology report were compatible with a chordoma. During the 12 month follow-up, there is tumoral progression and we perform a second resection where the pathology report is compatible with a growth hormone secreting pituitary adenoma. We perform complementary treatment with stereotactic fractionated radiotherapy achieving adequate control of the residual tumor during a 3 year follow-up.

The relationship of extraaxial tumors and acromegaly and hormone replacement therapy with somatotropine analogues is a rare one with case series that describe some types of related tumors not associated with a primary lesion like colorectal or breast cáncer. There is still lacking evidence that suggests a clear carcinogenic effect of the somatotropine analogues in relation to intracranial extraaxial tumors, with data that only suggests a positive effect on tumoral progression of preexisting lesions like chordomas and Meningiomas.