Gigantism in a McCune-Albright's syndrome with calcified GH-releasing pituitary adenoma: Case report and literature review

Miguel Vega-Arroyo; Martha Lilia Tena-Suck; Celia Teresa de Jesús Álvarez-Gamiño; Citlaltepetl Salinas-Lara; Juan Luis Gómez-Amador

doi:10.1016/j.ijscr.2018.10.030

Gigantism in a McCune-Albright's syndrome with calcified GH-releasing pituitary adenoma: Case report and literature review

Int J Surg Case Rep. 2018:53:61-65. doi: 10.1016/j.ijscr.2018.10.030. Epub 2018 Oct 23.

Authors

Miguel Vega-Arroyo¹, Martha Lilia Tena-Suck², Celia Teresa de Jesús Álvarez-Gamiño³, Citlaltepetl Salinas-Lara², Juan Luis Gómez-Amador³

Affiliations

¹ Neurosurgery Department, National Institute of Neurology and Neurosurgery "Manuel Velasco Suárez", Mexico City, Mexico. Electronic address: miguelvega@neurocirugia-innn.com.
² Neuropathology Department, National Institute of Neurology and Neurosurgery "Manuel Velasco Suárez", Mexico City, Mexico.
³ Neurosurgery Department, National Institute of Neurology and Neurosurgery "Manuel Velasco Suárez", Mexico City, Mexico.

Abstract

Background: McCune-Albright's syndrome (MAS) is a rare disorder that is characterized by café-au-lait macules, fibrous dysplasia of the skull and endocrinopathies like excessive secretion of growth hormone by a hyper-functional pituitary adenoma (PA).

Case: We describe the case of a 43-year-old male with history of Gigantism in 1990 secondary to a GH-secreting pituitary macroadenoma that was treated via microscopic transsphenoidal surgery at that time. He was reported as asymptomatic for 26 years until he developed headache and right temporal hemianopia with left amaurosis. Also ptosis and proptosis was found caused by a re-growth of the tumor on the follow up MRI. A second surgical procedure was performed via a dorsolateral craniotomy. Gross total resection was also achieved with a Neuropathology report of a pituitary adenoma tissue accompanied by extended dystrophic calcification and bone formation.

Conclusion: This is a rare case of MAS. Gigantism within the context of a pituitary calcification raises special diagnostic and therapeutic challenges. The cause of the excessive secretion of GH in MAS is not well understood concluding that it seems to be a different etiology of patients with Acromegaly and Gigantism in non-MAS patients.

Keywords: Adenoma; Calcification; Gigantism; MAS; McCune-Albright’s; Pituitary.