Pituicytoma: A Report of Two Cases and Literature Review

Abstract

Abstract Introduction Pituicytoma is a rare form of primary benign tumor of the neurohypophysis and infundibulum. There have been 140 reported cases in world literature that could be found in our search. Here we discuss about the presentation, radiological features, treatment, histopathology, and immunohistochemical markers of two cases, along with a brief review of literature. Case 1 A 48-year-old female presented with headache and painless visual loss for 4 years. Examination revealed bitemporal hemianopia and normal fundus. Hormonal profile was normal. Magnetic resonance imaging (MRI) showed 2.6 × 2.8 × 3.3 cm, thin-walled, cystic sellar and suprasellar lesions stretching the optic chiasm superiorly. Endoscopic transnasal, transsphenoidal gross total excision was done. Histopathological examination (HPE) and immunohistochemistry (IHC) were suggestive of pituicytoma. Case 2 A 55-year-old male presented with intermittent headache and vomiting for 3 months. Examination was unremarkable. Hormonal profile was normal. MRI showed lobulated mass of size 1.4 × 1.9 × 2.0 cm, arising from anterior pituitary and extending to the suprasellar cistern. Lesion was hyperintense in T1-weighted and heterointense in T2-weighted images, and Fluid attenuated inversion recovery images (FLAIR) with homogenous contrast enhancement pushed the optic chiasm superiorly. Endoscopic transnasal partial excision of the lesion was done. HPE and IHC were suggestive of pituicytoma. Discussion Total number of cases was 142, of which 71 were males (50%) and 71 were females (50%). Age ranged between 7 and 83 years (mean 49.2, median 48). Brain imaging was available in 110 cases. Size of the lesion varied from 4 to 72 mm (mean 27 mm). Location was either pure sellar (24.3%) or with extension into the suprasellar cistern (34.3%) and/or the cavernous sinuses (7.1%). Lesions in MRI were solid (84%) with few areas of cystic changes, while contrast enhancement of solid portion was in 92% (homogenous in 80% and heterogeneous in 20%) of the cases. One case showed features of hemorrhage while no case showed calcification. Treatment was primarily surgical (120 out of 130 cases): transsphenoidal excision in 70% of the cases and craniotomy and excision in 30%. Preoperative embolization of arterial feeders was done in two cases and postoperative radiation was given in one case. Gross total resection was done in 46.3%, subtotal resection in 40%, partial resection in 12.6%, and biopsy was done in 1.1% of the cases in the study. Follow-up ranged from 2 to 134 months (average 31.2, median 19). Recurrence or regrowth was documented in 23 patients, treatment in 18 patients, and resurgery was done in 12 cases, followed by radiotherapy in 5. In six cases, radiotherapy was used in isolation. Improvement in the visual deficit was seen in 26 patients, and remained unchanged in 3. Among the patients with preoperative endocrine dysfunction, 10 improved while 11 suffered a worsening. Ten patients had new postoperative endocrine alteration. Only one fatal case was registered. Conclusion Pituicytoma is a rare World Health Organization grade I tumor of the sellar and suprasellar region, presenting with varied clinical, radiological, and hormonal features. Histopathology and immunohistochemistry form the mainstay in diagnosis. Surgical excision with transsphenoidal approach is ideal. Prognosis in general is good after surgical resection alone or in conjunction with radiotherapy.