Acromegaly with empty sella syndrome.

Area of Science:

• Endocrinology
• Rare Diseases
• Pituitary Disorders

Background:

• Acromegaly is a chronic disorder caused by excess growth hormone (GH), typically from pituitary adenomas.
• Primary empty sella (PES) is a rare condition, and its association with acromegaly is exceptionally uncommon.
• Identifying the source of GH hypersecretion can be challenging, especially in atypical cases.

Purpose of the Study:

• To describe a rare case of acromegaly in a patient with primary empty sella (PES).
• To illustrate the diagnostic process and successful medical management in this unusual presentation.
• To contribute to the limited literature on acromegaly associated with PES.

Main Methods:

• Clinical presentation and biochemical evaluation of acromegaly (elevated GH and IGF-1).
• Pituitary MRI to assess for pituitary adenoma and empty sella.
• 68Gallium DOTATATE PET/CT to confirm GH source within the empty sella.
• Initiation of medical therapy with octreotide acetate and testosterone replacement.

Main Results:

• The patient presented with overt acromegaly features and biochemical evidence of GH excess.
• Pituitary MRI revealed an empty sella, with PET/CT confirming it as the likely GH source.
• Medical treatment with octreotide acetate led to normalization of GH and significant reduction in IGF-1.
• Hormone replacement therapy addressed associated hypogonadism.

Conclusions:

• Acromegaly can rarely be associated with primary empty sella, posing diagnostic challenges.
• Medical therapy, such as octreotide acetate, is a viable primary treatment option when a resectable mass is absent.
• Comprehensive biochemical and imaging evaluation is crucial for diagnosing and managing rare endocrine disorders.