Pituicytoma: A rare tumor of the sella. A case report and review of literature for diagnosis and management
- Department of Neurosurgery, Patel Hospital, Karachi, Pakistan.
- Department of Histopathology, Aga Khan University Hospital, Karachi, Pakistan.
Areesha Syeda Shakeel, Department of Neurosurgery, Patel Hospital, Karachi, Pakistan.
DOI:10.25259/SNI_248_2023Copyright: © 2023 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Umaima Zaki1, Areesha Syeda Shakeel1, Yaseen Rauf1, Muhammad Raza2. Pituicytoma: A rare tumor of the sella. A case report and review of literature for diagnosis and management. 23-Jun-2023;14:220
How to cite this URL: Umaima Zaki1, Areesha Syeda Shakeel1, Yaseen Rauf1, Muhammad Raza2. Pituicytoma: A rare tumor of the sella. A case report and review of literature for diagnosis and management. 23-Jun-2023;14:220. Available from: https://surgicalneurologyint.com/surgicalint-articles/12372/
Background: Pituicytomas are rare tumors of the pituitary gland derived from the ependymal cells and line the pituitary stalk and posterior lobe. These tumors are located in the vulnerable regions of the brain: Either in the sellar or suprasellar area. The location marks the difference in the clinical features of the tumor. Here, we report a case of histopathologically diagnosed pituicytoma of the sellar region. Literature is also reviewed and discussed to gain a better understanding of this rare disease.
Case Description: A 24-year-old female presented to the outpatient department with complaints of headache, diplopia, dizziness, and decreased vision in the right eye for 6 months. Computed tomography scan brain without contrast showed a well-defined hyperdense lesion in the sella without associated bony erosion. Her magnetic resonance imaging showed well defined rounded lesion in the pituitary fossa which was isointense on T1-weighted image and hyperintense on T2-weighted images. A presumptive diagnosis of pituitary adenoma was made. She underwent endoscopic endonasal transsphenoidal resection of pituitary mass. Intraoperatively, normal pituitary gland was visualized and there was a grayish-green-colored, jelly like tumor which was pulled gently. On 9th postoperative day, she presented with cerebrospinal fluid (CSF) rhinorrhea. She underwent endoscopic CSF leak repair. Her histopathology was concluded to be Pituicytoma.
Conclusion: Pituicytoma is an uncommon diagnosis. The surgical aim is to completely excise the tumor which results in complete cure, but incomplete resection may be performed due to high vascularity of this tumor. In case of incomplete excision, recurrence is common and adjuvant radiotherapy may be administered.
Keywords: Ependymal tumor, Pituicytoma, Pituitary tumor, Sellar tumor, Transnasal endoscopic tumor resection
Pituicytomas are rare tumors of the posterior part of the pituitary gland-neurohypophysis or infundibulum.[
Embryologically, neurohypophysis is derived from the lower part of diencephalon and continues with the hypothalamus. The hypophysis is surrounded by these glial cells known as pituicytes.[
These are derived from the ependymal cells and line the pituitary stalk and posterior lobe.[
Here, we report a case of histopathologically diagnosed pituicytoma of the sellar region. Literature is also reviewed and discussed to gain a better understanding of this rare disease.
A 24-year-old female presented to the outpatient department with complaints of headache, diplopia, dizziness, and decreased vision in the right eye for 6 months. Due to these complaints, she even fell multiple times and injured herself. She did not report any episodes of fits, altered level of consciousness, nausea vomiting, or any history of trauma. On examination, she was a young female, oriented to time, place, and person. Her cranial nerves’ examination was unremarkable. The vision in the right eye was reduced to finger counting. The vision in the left eye was also similar. However, extraocular movements were normal bilaterally. She had normal tone, bulk, and power in all four limbs. Her sensations were also intact in all four limbs. She did not have any cerebellar signs. Her hormonal status was normal. Computed tomography (CT) scan brain without contrast showed a well-defined hyperdense lesion in the sella without associated bony erosion. Her magnetic resonance imaging (MRI) was showed well defined rounded lesion in the pituitary fossa which was isointense on T1-weighted image and hyperintense on T2-weighted images [
A presumptive diagnosis of pituitary adenoma was made. She underwent endoscopic endonasal transsphenoidal resection of pituitary mass. Intraoperatively, normal pituitary gland was visualized and there was a grayish-green-colored, jelly like tumor which was pulled gently. During surgery, diaphragma sella was damaged and there was cerebrospinal fluid (CSF) leak. The sella was packed with fat and fascia which was harvested from abdomen. This was further strengthened with bone and nasoseptal flap. Postoperatively, she developed panhypopituitarism.
On 9th postoperative day, she presented with CSF rhinorrhea. She underwent endoscopic CSF leak repair with fat, flap, bone, and Hadad flap. Fibrin glue was also applied. Nasal pack was inserted.
Unfortunately, she developed severe headache, altered sensorium, and fever 2 days later and meningitis was suspected. She was managed with antibiotics. She gradually improved but reported CSF leak again at 5th post-CSF leak repair day. For this, a lumbar drain was inserted. The lumbar drain was kept till 6th postprocedure day and the CSF leak was resolved. She was observed for one more day. Her symptoms improved and she was discharged and her nasal pack was removed on 8th postoperative day in outpatient department. On subsequent follow-up, she has recovered completely.
Her histopathology showed abundant hemorrhage and proteinaceous material along with spindle cells. This was concluded to be pituicytoma. It was glial fibrillary acidic protein (GFAP) negative and thyroid transcription factor 1 (TTF1) positive [
On pathological examination, the sample showed a hypocellular lesion, composed of ovoid to spindle cells, arranged in short bundles and sheets [
Adenomas arising from adenohypophysis are the most common pituitary tumors. Pituicytomas, germinomas, meningiomas, hamartomas, and craniopharyngiomas are categorized among posterior pituitary tumors originating from neurohypophysis.[
CNS tumors of glial origin are benign and were classified by the WHO in 2006 where it termed them pituicytoma differently from astrocytomas under low Grade-1 glial tumors.[
A cohort study on 93 diagnosed cases of pituicytoma from 1958 to 2020 concluded the most favorable findings suggestive of the diagnosis includes: (1) presence of hypopituitarism, (2) CT showing isodense tumor without calcification, (3) MRI revealing a globular mass located in the suprasellar region separating the pituitary gland, and lastly (4) an isointense image on T1-weighted MRI due to marked vascular proliferation and intense contrast-enhancement.[
The histological criteria for diagnosis of pituicytoma was proposed by Brat et al. in 2000.[
The preoperative clinical presentation includes mass effect symptoms including visual impairment such as bitemporal hemianopsia, headache, endocrine disorders like hypopituitarism being the most common,[
The mainstay of treatment for these tumors is surgical excision either by trans-sphenoidal or transcranial route.[
Gross total resection (GTR) is the cure for the disease based on a large series of cases reviewed in 2021.[
Brat et al., proved that complete excision of the tumor was the most significant predictor of recurrence.[
Pituicytoma remains an uncommon tumor in neurosurgical specimens. The tumor may be detected incidentally or may present with a spectrum of symptoms ranging from mass effect symptoms to endocrine abnormalities. The surgical aim is to completely excise the tumor which results in complete cure, but incomplete resection may be performed due to high vascularity of this tumor. In such cases, it is recommended to follow patient with MRI and offer either re-operation or radiotherapy if any recurrence is identified.
Patient’s consent not required as patient’s identity is not disclosed or compromised.
There are no conflicts of interest.
The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.
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