Clinical features, diagnosis and therapy of pituicytoma: an update
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Pituicytomas (PTs) are extremely rare, low-grade glial tumors closely related to the neurohypophyseal axis. Definite conclusions concerning the optimal diagnostic and therapeutic approach to these neoplasms are lacking to date, as most of this information has been presented as case reports.
Retrospective review of case reports published in the scientific literature to date, including a new illustrative example treated in our department.
116 cases were collected. PTs had a higher prevalence in the fifth and sixth decades of life, with a slight male predominance. Main symptoms, which tended to be progressive, included visual field defects and pituitary–hypothalamic dysfunction. Radiologically, PTs were found anywhere along the hypothalamic–pituitary axis mimicking other, more frequent tumors growing in this anatomical region. Surgical treatment included both transcranial or transsphenoidal approaches, and resulted in gross total resection and morbidity rates of 46.8 and 59%, respectively; the latter essentially consisted in anterior and posterior pituitary dysfunction, with limited impact on daily quality of life.
Due to both low frequency and the absence of pathognomonic clinical and/or radiological features, formulating a suspicion diagnosis of PT represents a considerable challenge even for experienced professionals. The indication for treatment should be made on an individual basis, but it is inescapable in the presence of a visual field defect. The surgical approach has to be tailored according to the topography of the tumor and preoperative symptoms; the greatest challenges in accomplishing a gross total removal are represented by the degree of adherence and vascularization of the PT.
KeywordsHypothalamus Pituicytoma Pituitary Tumor Transsphenoidal surgery
We would like to acknowledge the assistance of Prof. Eduardo E. Espinosa during the elaboration of the manuscript in English language.
Compliance with ethical standards
Conflict of interest
The authors report no conflicts of interest or financial disclosures/nothing to declare.
This study was ellaborated in accordance with the ethical standards currently in force, including those from our institutional research committee and the 1964 Helsinki declaration (and its later amendments). For this type of study formal consent is not required.
Informed consent was obtained from all individuals participants included in the study.
Online Resource 1. Video showing a description of most important steps in the surgical approach of a PT through an endonasal endoscopic transsphenoidal corridor (AVI 13570 kb)
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