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J Neurol Surg Rep 2016; 77(03): e113-e117
DOI: 10.1055/s-0036-1585091
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome

Audrey E. Arzamendi
1   Division of Endocrinology, Diabetes & Metabolism, Department of Internal Medicine, UC Davis Medical Center, Sacramento, California, United States
,
Kiarash Shahlaie
2   Department of Neurological Surgery, UC Davis Medical Center, Sacramento, California, United States
,
Richard E. Latchaw
3   Department of Radiology, UC Davis Medical Center, Sacramento, California, United States
,
Mirna Lechpammer
4   Department of Pathology, UC Davis Medical Center, Sacramento, California, United States
,
Hasmik Arzumanyan
1   Division of Endocrinology, Diabetes & Metabolism, Department of Internal Medicine, UC Davis Medical Center, Sacramento, California, United States
› Author Affiliations
Further Information

Publication History

29 August 2015

30 May 2016

Publication Date:
22 July 2016 (online)

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Abstract

Objective To describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS).

Methods We report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature.

Results A 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum.

Conclusion This report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations.

Indexing Acromegaly, ESS, pituitary adenoma, sphenoid sinus septum.

Keywords

acromegaly - empty sella syndrome - pituitary adenoma - sphenoid sinus septum
 

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