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.

Superior sagittal sinus (SSS) thrombosis has high morbidity and mortality, and urgent recanalization is critical for severe cases. Standard endovascular techniques for thrombolysis and thrombectomy use retrograde venous access, an approach that may be unsuccessful in cases with extensive firm clot burden involving the dural sinuses distal to the SSS. An anterior open transcranial approach to the SSS for catheter sheath placement to facilitate antegrade mechanical thrombectomy and thrombolysis of the SSS and more distal sinuses has not been previously described. Here we describe a case in which multiple unsuccessful attempts at retrograde endovascular access were attempted. Thus, a burr hole over the anterior SSS was performed for daily endovascular antegrade procedures using the Angiojet rheolytic catheter device and chemical thrombolysis. Near-complete recanalization of the SSS was achieved with venous outflow via dilated left transverse and left sigmoid sinuses, along with significant collateral flow in multiple cerebral veins.