From the Guidelines
Pituicytoma is a rare, benign brain tumor that originates from pituicytes, which are specialized glial cells in the neurohypophysis (posterior pituitary gland), and the standard treatment for pituicytoma is surgical resection, with the goal of complete tumor removal when possible, as recommended by recent guidelines 1.
Diagnosis and Treatment
The diagnosis of pituicytoma usually involves MRI imaging showing a solid, well-circumscribed mass in the sellar or suprasellar region, followed by histopathological confirmation.
- Patients with pituicytoma may present with headaches, visual disturbances, or hormonal abnormalities depending on the tumor's size and location.
- The prognosis for patients with pituicytoma is generally favorable, with low recurrence rates after complete resection, though long-term follow-up with periodic imaging is recommended to monitor for potential regrowth.
Management Recommendations
According to recent consensus guidelines 1, the management of pituicytoma should involve a multidisciplinary team, including neurosurgeons, endocrinologists, and radiologists.
- Surgical resection is the primary treatment for pituicytoma, with the goal of complete tumor removal when possible 1.
- Radiation therapy might be considered for residual or recurrent tumors, though its efficacy is not well-established due to the rarity of these lesions 1.
Genetics and Family Screening
Genetic assessment is recommended for all children and young people with pituitary adenomas, including pituicytoma, to inform management and family surveillance 1.
- Genetic testing should be offered to all children and young people with growth hormone and prolactin excess, as these tumors are more likely to have a genetic cause 1.
From the Research
Definition and Characteristics of Pituicytoma
- Pituicytomas are rare, low-grade glial neoplasms originating in the neurohypophysis or infundibulum, appearing as solid, noninfiltrative masses occupying the sella turcica, the suprasellar space, or both 2, 3.
- They have a high tendency to bleed and can be firm and adherent to surrounding structures, making preoperative diagnosis and surgical removal challenging 3, 4.
Clinical Features and Diagnosis
- Pituicytomas tend to occur in the fifth and sixth decades of life, with a slight male predominance, and often present with progressive visual field defects and pituitary-hypothalamic dysfunction 4.
- Radiologically, they can mimic other tumors growing in the hypothalamic-pituitary axis, making diagnosis difficult without histological and immunohistochemical analysis 3, 4.
Surgical Management and Outcome
- Gross total surgical resection is the preferred treatment for pituicytomas, with the goal of achieving complete removal of the tumor 2, 5.
- The surgical approach can be transsphenoidal or transcranial, with the choice depending on the tumor's location and the patient's symptoms 2, 4.
- Expanded endoscopic endonasal transsphenoidal and transplanum (EETS-TP) approaches have been associated with higher rates of gross total removal and lower rates of neurological complications compared to craniotomy 2.
- Postoperative complications, including visual loss and pituitary dysfunction, can occur, especially with transcranial approaches 4, 5.
Prognostic Factors and Recurrence
- Male gender, tumor diameter ≥ 1.85 cm, and non-gross total resection have been identified as independent unfavorable factors for pituicytoma progression 5.
- Recurrence can occur after subtotal resection, and adjuvant radiotherapy may be recommended for male patients with high Ki-67 index or for residual tumors after partial resection 5.
- Close follow-up is essential for monitoring tumor recurrence or progression, and re-operation may be necessary to achieve complete removal of the tumor 5.