gms | German Medical Science

Objective: The sellar and suprasellar region (SSR) is a crowded area with vital and complex neurovascular anatomy, containing variable tissues from a cytogenetic point of view. Therefore, a wide range of histologically different tumors, among them also rare tumors, can develop in this area. The differential diagnosis of these rare tumors is challenging, due to the paucity of specific clinical und radiological features. This report provides an overview of the clinical and surgical management of 17 patients with rare tumors of the SSR within a 6-year period in a high load neurosurgical department.

Method: Patients were operated between June 2006 and April 2012 using microsurgical and/or neuroendoscopic technique and neuronavigation in some cases. In 7 cases tumor resection was performed through a transnasal/transsphenoidal approach and in 10 cases through a transcranial frontolateral/pterional approach. The mean age of the patients was 43.1 years (2–72 years). There were 10 men (58.8%) and 7 women (41.2%). Clinical follow-up time was 3–32 months (mean: 5.5 months).

Results: The most common leading symptom was visual deficit (9 cases, 53%) and the second most common was headache and anterior pituitary dysfunction (4 cases, 24%). Moreover, diabetes insipidus occurred in 3 cases (18%). Two cases presented with proptosis, facial pain and oculomotor symptoms (11.8%). The tumor was hyperintense on T1-weighted images in 12 cases (71%) and heterogeneous on T2-weighted images in 11 cases (65%). The tumor localisation was intrasellar in 3 cases, intra-/suprasellar in 9 cases and only suprasellar in 5 cases. Histopathologically, a xanthogranuloma was diagnosed in 6 cases, metastatic tumor in 5 cases and pilocytic astrocytoma in 2 cases. A gangliocytoma, colloid cyst, abscess and dysgerminoma was found in other patients. Clinical outcome was good in all cases (mean Karnofsky-Score: 80%). Hormonal substitution could be withdrawn or reduced in 6 patients (35%) after 3 months. In 1/3 of the cases with visual disturbance, the visual deficit improved after surgery (33%) and was stable in 67%.

Conclusions: The sellar and suprasellar region is indeed a hideout of rare lesions. A better understanding of the special features of tumors in this region is helpful for the surgical decision-making and further clinical treatment.