Bao Yang1, Chenlong Yang2, Yilin Sun3, Jiang Du4, Pinan Liu1, Guijun Jia1, Wang Jia1, Yazhuo Zhang1, Jisheng Wang1, Yulun Xu1, Shuo Wang5. 1. Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing, 100050, China. 2. Department of Orthopedics, Peking University Third Hospital, North Garden Street No. 49, Haidian District, Beijing, 100191, China. 3. Department of Ultrastructural Pathology, Beijing Neurosurgical Institute, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing, 100050, China. 4. Department of Neuro-pathology, Beijing Neurosurgical Institute, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing, 100050, China. 5. Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing, 100050, China. wangshuo_tth@outlook.com.
Abstract
BACKGROUND: Mixed gangliocytoma-pituitary adenoma is an extremely rare tumor occurring in the sellar region, histologically composed of both gangliocytic and pituitary adenomatous architectures. The histogenesis of these tumors remains unknown, and the diagnosis, treatment, and prognosis are yet to be fully understood. METHODS: We retrospectively reviewed the clinical, radiological, and histopathological profiles from a series of 20 patients with sellar mixed gangliocytoma-pituitary adenomas. All patients underwent surgical tumorectomy via an endoscopic or microscopic transsphenoidal approach. Perioperative magnetic resonance imaging (MRI) and computed tomography (CT) data were reviewed. Immunohistochemical stains and electron microscopy examination were performed. Follow-up outcomes were presented. RESULTS: This case series consisted of 13 females and seven males (age range, 20-59 years; mean age, 42.3 ± 11.2 years). Preoperative endocrine examination showed elevated growth hormone (GH) in nine patients and hyperprolactinemia in eight patients. The tumors were positive for GH in 15 cases, prolactin in 13 cases, adrenocorticotropic hormone in three cases, and thyroid-stimulating hormone in one case. Gross total resection was achieved in 15 patients, and subtotal resection in five patients. During an average follow-up period of 42.5 ± 29.0 months, no recurrence was noted. CONCLUSIONS: The clinical and neuroimaging features of sellar mixed gangliocytoma-pituitary adenomas are non-specific, and invasion into the cavernous sinus is common. Surgical resection via a transsphenoidal approach is the preferred treatment, and the surgical outcomes are favorable. Moreover, our histopathological findings are more likely to support the theory that mixed gangliocytoma-pituitary adenoma originates from the neuronal transdifferentiation of adenomatous cells.
BACKGROUND: Mixed gangliocytoma-pituitary adenoma is an extremely rare tumor occurring in the sellar region, histologically composed of both gangliocytic and pituitary adenomatous architectures. The histogenesis of these tumors remains unknown, and the diagnosis, treatment, and prognosis are yet to be fully understood. METHODS: We retrospectively reviewed the clinical, radiological, and histopathological profiles from a series of 20 patients with sellar mixed gangliocytoma-pituitary adenomas. All patients underwent surgical tumorectomy via an endoscopic or microscopic transsphenoidal approach. Perioperative magnetic resonance imaging (MRI) and computed tomography (CT) data were reviewed. Immunohistochemical stains and electron microscopy examination were performed. Follow-up outcomes were presented. RESULTS: This case series consisted of 13 females and seven males (age range, 20-59 years; mean age, 42.3 ± 11.2 years). Preoperative endocrine examination showed elevated growth hormone (GH) in nine patients and hyperprolactinemia in eight patients. The tumors were positive for GH in 15 cases, prolactin in 13 cases, adrenocorticotropic hormone in three cases, and thyroid-stimulating hormone in one case. Gross total resection was achieved in 15 patients, and subtotal resection in five patients. During an average follow-up period of 42.5 ± 29.0 months, no recurrence was noted. CONCLUSIONS: The clinical and neuroimaging features of sellar mixed gangliocytoma-pituitary adenomas are non-specific, and invasion into the cavernous sinus is common. Surgical resection via a transsphenoidal approach is the preferred treatment, and the surgical outcomes are favorable. Moreover, our histopathological findings are more likely to support the theory that mixed gangliocytoma-pituitary adenoma originates from the neuronal transdifferentiation of adenomatous cells.