Case Report: Paraganglioma in the sellar region: longitudinal observation and surgical outcome

Abstract

BackgroundParaganglioma in the sellar region is an extremely rare entity, with a limited number of cases reported in the literature. Due to the paucity of clinical evidence, the diagnosis and treatment of paragangliomas in the sellar region remain challenging. Herein, we reported a case of sellar paraganglioma with parasellar and suprasellar extension. Particularly, the dynamic evolution of this benign tumor within a 7-year longitudinal observation was presented. Additionally, the relevant literature regarding sellar paraganglioma was comprehensively reviewed.Case descriptionA 70-year-old woman presented with progressive visual deterioration and headache. Brain magnetic resonance imaging demonstrated a mass in the sellar region with parasellar and suprasellar extension. The patient refused surgical treatment. Seven years later, brain magnetic resonance imaging showed the lesion significantly progressed. Neurological examination revealed bilateral tubular contraction of visual fields. Laboratory examinations showed endocrine hormone levels were normal. Surgical decompression was performed via a subfrontal approach, and subtotal resection was achieved. Histopathological examination confirmed a diagnosis of paraganglioma. Postoperatively, she developed hydrocephalus, and ventriculoperitoneal shunting was performed. Eight months later, cranial CT showed no recurrence of the residual tumor, and the hydrocephalus had been relieved.ConclusionParaganglioma occurring in the sellar region is rare, and the preoperative differential diagnosis is difficult. Owing to the infiltration to the cavernous sinus and internal carotid, complete surgical resection is usually impracticable. There has been no consensus regarding postoperative adjuvant radiochemotherapy for the tumor residue. In-situ recurrence and metastasis have been reported in the literature, and close follow-up is warranted.

Gamma Knife Radiosurgery: An Adjuvant Therapy for Primary Sellar Paraganglioma

“…SP is predominantly seen in males (65.63%), with a male-to-female ratio of 1.9:1, contrasting with the female-predominant carotid, vagal, tympanic, and laryngeal paragangliomas [ 34 , 35 ]. SP tumors are lobulated, firm, and highly vascular, with or without a capsule [ 1 , 33 ]. They are adherent to the carotid artery, cavernous sinus, and optic nerve, which extends to the extra sellar locations [ 2 , 3 , 33 ].…”

“…SP tumors are lobulated, firm, and highly vascular, with or without a capsule [ 1 , 33 ]. They are adherent to the carotid artery, cavernous sinus, and optic nerve, which extends to the extra sellar locations [ 2 , 3 , 33 ]. Although the exact origin of the SP is unknown, in the current case, the border between the tumor and the pituitary gland was clear, and the tumor extended into the cavernous sinus, suggesting that it may have originated from within the cavernous sinus.…”

“…Although the exact origin of the SP is unknown, in the current case, the border between the tumor and the pituitary gland was clear, and the tumor extended into the cavernous sinus, suggesting that it may have originated from within the cavernous sinus. MRI of SP shows heterogeneous intensity on T2 with gadolinium enhancement, indicating high vascularity [ 12 , 32 , 33 ]. However, the findings were atypical for the present case, with a weak contrast effect and minimal enhancement in dynamic studies.…”

“…In the late 20th-century case reports, SP patients were treated via both the TC [8][9][10][11][12][13] and TS [1,7,11,[14][15][16] routes equally (n=6 each). However, since 2000, more patients with SP have been treated via the TC route (13 patients, 65%) [3,[18][19][20][21][22]24,28,[30][31][32][33] rather than the TS route (seven patients, 35%) [2,17,23,[25][26][27]29]. The primary treatment choices for SP are biopsy (12.5%), subtotal resection (65.6%), or gross total resection (21.9%).…”