ECE2019 Poster Presentations Pituitary and Neuroendocrinology 1 (72 abstracts)
1Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv Sourasky Medical Center, Tel-Aviv, Israel; 2Sackler School of Medicine, Tel Aviv University, Tel-Aviv, Israel.
Introduction: Spindle cell oncocytoma (SCO), pituicytoma, granular cell tumor, and sellar ependymoma are rare TTF-1 positive posterior pituitary tumors classified as WHO Grade I, indicating low proliferative potential and high rates of surgical cure.
Aims: To report a case of SCO and review the pertinent literature.
Methods: English and non-English literature was searched, clinical, data were retrieved and summarized.
Results: A 39 years old male presented with complaints of headaches. MRI identified a suprasellar tumor that was initially followed conservatively. Vision and pituitary function were preserved. Symptom exacerbation and evidence of tumor growth led to transsphenoidal surgery. Tumor resection was compromised by profuse bleeding. He developed post-surgical pan-hypopituitarism. 42 publications describing 54 cases of SCO were identified (median age 60 y, 57% males). Presenting symptoms were mostly secondary to mass effect. 67% of tumors were sellar with suprasellar extension, 23% and 9.6% were exclusively intra or suprasellar respectively. All patients underwent surgery, 83% of which was trans-sphenoidal. Mean Ki-67 was 3.4%. Gross total resection was achieved in only 47.8% of cases, with recurrence-free survival time of 116 months (95% CI, 65167). After partial resection, event-free survival time was 23 months (95% CI, 1234). Nine patients received radiation therapy with mixed results.
Conclusions: Surgical resection of these rare tumors is challenging; surgical cure is infrequent and recurrence rates are high. Based on this literature review, we suggest that WHO classification of these tumors as grade 1 is not accurate, and that a revision should be considered.