Objective: To investigate the clinicopathological features and treatment strategies of pituicytoma. Methods: Twenty-one cases of pituicytoma were collected at the First Affiliated Hospital of Nanjing Medical University and Jinling Hospital, Nanjing, China from 2009 to 2020. The clinical data of 21 pituicytoma patients was retrospectively analyzed, and the relevant literature was reviewed. Results: Twenty-one patients aged 4 to 68 years, including 8 males and 13 females. All patients underwent surgical treatment. Histologically, the tumor was consisted almost entirely of elongate, bipolar spindle cells arranged in a fascicular or storiform pattern. Mitotic figures were rare. Immunohistochemically, tumor cells were diffusely positive for S-100 protein (21/21), vimentin (15/15) and TTF1 (14/14), while they were weakly or focally positive for GFAP (13/16) and EMA (6/12). CKpan was negative in all cases and Ki-67 proliferation index was low (<5%). Among the 18 patients with follow-up, all survived and 2 relapsed after surgery. Conclusions: Pituicytoma is a rare low-grade glioma of the sellar area. It is easily confused with other sellar tumors. Preoperative diagnosis is difficult. It needs to be confirmed by histopathology and immunohistochemistry. Microsurgery is the main treatment method at present.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Craniopharyngioma ; Female ; Glioma/pathology* ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Pituitary Neoplasms/pathology* ; Retrospective Studies ; Young Adult

Craniopharyngioma ; Humans ; Parapharyngeal Space ; Pharyngeal Neoplasms ; Pituitary Neoplasms

This is a case of a 15-year-old Filipino male with a history of craniopharyngioma who underwent trans-sphenoidal surgery with panhypopituitarism as its sequelae. The biological factors of the patient's disease contributed to his persistent depression and were perpetuated by psychosocial and cognitive factors. A biopsychosocial approach was used in understanding this case to arrive at individualized treatment and management.
Craniopharyngioma ; Depression

Idiopathic granulomatous hypophysitis (IGH), a rare disease, requires differentiation from more common mass lesions of the sella such as pituitary adenoma, craniopharyngioma, Rathke's cleft cyst, or pituitary tuberculoma. IGH usually presents with an insidious onset of visual defects and headaches. On the other hand, rapid onset of neurologic and visual symptoms in an IGH patient is exceptionally rare. Here, we present a biopsy-proven case of IGH with rapid onset and satisfactory outcome after high dose steroid treatment.
Adult ; Autoimmune Hypophysitis ; Craniopharyngioma ; Endoscopy ; Glucocorticoids ; Hand ; Headache ; Humans ; Pituitary Gland ; Pituitary Neoplasms ; Rare Diseases ; Sella Turcica ; Tuberculoma

Adipsic hypernatremia is a rare disease where patients do not feel thirst even in the increased serum osmotic pressure and results in electrolyte imbalance, severely increased osmotic pressure and neurologic symptoms like nausea, vomiting, and seizures. We report a 12-year-old male patient who had underwent a trans-sphenoidal surgery for craniopharyngioma newly diagnosed with adipsic hypernatremia after having growth hormone replacement for growth hormone deficiency. The patient visited emergency room complaining of generalized weakness, tremor in both legs, and poor oral intake including water after starting growth hormone replacement therapy. Laboratory test revealed serum sodium 168 mmol/L and serum osmolality 329 mOsm/kg, despite the patient didn't feel any thirst at all. We treated him with scheduled water intake of 2.5 L a day with intranasal vasopressin. He admitted to Soonchunhyang University Gumi Hospital and Soonchunhyang University Seoul Hospital for 4 times during the following 8 months and serum sodium level and osmolality was controlled by scheduled water intake combined with intranasal vasopressin treatment. It is still unclear whether growth hormone replacement worked as a trigger of hypernatremia.
Child ; Craniopharyngioma ; Drinking ; Emergency Service, Hospital ; Growth Hormone ; Gyeongsangbuk-do ; Humans ; Hypernatremia ; Leg ; Male ; Nausea ; Neurologic Manifestations ; Osmolar Concentration ; Osmotic Pressure ; Rare Diseases ; Seizures ; Seoul ; Sodium ; Thirst ; Tremor ; Vasopressins ; Vomiting ; Water

Superficial siderosis of the central nervous system (CNS) is a progressive and debilitating neurological disease manifesting sensorineural hearing loss, cerebellar ataxia, and pyramidal tract signs. Chronic extravasation of blood into the subarachnoid space results in the accumulation of hemoglobin derivate in the subpial layer of the CNS, which is toxic to the neural tissues. Craniopharyngioma is a benign third ventricle tumor, which rarely presents with tumor bleeding. We report a rare case of superficial siderosis associated with craniopharyngioma with intratumoral hemorrhage in a patient with no history of prior trauma or CNS surgery.
Central Nervous System ; Cerebellar Ataxia ; Craniopharyngioma ; Hearing Loss, Sensorineural ; Hemorrhage ; Humans ; Magnetic Resonance Imaging ; Pyramidal Tracts ; Siderosis ; Subarachnoid Space ; Third Ventricle

Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD) and diabetes mellitus (DM). We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection. Two years later, the craniopharyngioma recurred, and she underwent a second resection. Three years after her second operation, she was diagnosed with type 2 DM, after which she did not visit the outpatient clinic for 2 years and then suddenly reappeared with a weight loss of 25.8 kg that had occurred over 21 months. One month later, she presented to the Emergency Department with dyspnea. Laboratory findings revealed liver dysfunction and hypoxia with increased alveolar artery oxygen gradient. Liver biopsy showed portal hypertension and micronodular cirrhosis. Echocardiography and a lung perfusion scan demonstrated a right to left shunt. She was finally diagnosed with hepatopulmonary syndrome and is currently awaiting a donor for liver transplantation. Patients surviving craniopharyngioma need to be followed up carefully to detect signs of hypothalamic obesity and monitored for the development of other comorbidities such as DM, NAFLD, and hepatopulmonary syndrome.
Ambulatory Care Facilities ; Anoxia ; Arteries ; Biopsy ; Comorbidity ; Craniopharyngioma* ; Diabetes Mellitus ; Dyspnea ; Echocardiography ; Emergency Service, Hospital ; Female ; Fibrosis ; Hepatopulmonary Syndrome* ; Humans ; Hypertension, Portal ; Hypothalamus ; Liver ; Liver Diseases ; Liver Transplantation ; Lung ; Non-alcoholic Fatty Liver Disease* ; Obesity* ; Oxygen ; Perfusion ; Tissue Donors ; Weight Loss ; Young Adult

Extremely massive sellar xanthogranuloma (XG) are rare, and the surgical outcome and prognosis are not well known. XG remain unknown whether they are derived from Rathke's cleft cysts (RCCs) or craniopharyngiomas (CPs) following extensive inflammation and metaplasia, to the point that no epithelium is readily identifiable. These lesions usually tend to occur in younger patients (mean 28.3 years), have a smaller diameter, and remain primarily intrasellar region with infrequent calcification. This 36-year-old man presented our hospital with visual deterioration. At the time of visit, there were no neurological problems other than visual field defect and hormonal disorder. He visited our hospital in 2007 due to headache and decreased vision, and underwent transphenoid surgery for pituitary RCC. Since then, he has received treatment at our hospital for postoperative hormonal disorders. Through preoperative imaging study, the author suspected CP and underwent surgery. During the operation, the adhesion of the tumor to the surrounding major neurovascular structures was severe in the naked eyes, but the tumor could be removed more easily than expected. The postoperative histological findings were confirmed as XG. The postoperative course was uneventful. Compared to the previous literature, this case is a case where the size of XG is very large in a sellar region and it can be proved that it originated from the RCC. And regular follow-up is necessary to confirm the prognosis after surgery.
Adult ; Central Nervous System Cysts ; Craniopharyngioma ; Epithelium ; Follow-Up Studies ; Headache ; Humans ; Inflammation ; Metaplasia ; Prognosis ; Visual Fields

Growth hormone (GH) is an essential element for normal growth. However, reports of normal growth without GH have been made in patients who have undergone brain surgery for craniopharyngioma. Normal growth without GH can be explained by hyperinsulinemia, hyperprolactinemia, elevated leptin levels, and GH variants; however, its exact mechanism has not been elucidated yet. We diagnosed a female patient aged 13 with combined pituitary hormone deficiency (CPHD) caused by pituitary stalk interruption syndrome (PSIS). The patient has experienced recurrent hypoglycemic seizures since birth, but reached the height of 160 cm at the age of 13, showing normal growth. She grew another 8 cm for 3 years after the diagnosis, and she reached her final adult height of 168 cm which was greater than the midparental height, at the age of 16. The patient's blood GH and insulin-like growth factor-I levels were consistently subnormal, although her insulin levels were normal. Her physical examination conducted at the age of 15 showed truncal obesity, dyslipidemia, and osteoporosis, which are metabolic features of GH deficiency (GHD). Herein, we report a case in which a PSIS-induced CPHD patient attained her final height above mid parental height despite a severe GHD.
Adult ; Brain ; Craniopharyngioma ; Diagnosis ; Dyslipidemias ; Female ; Growth Hormone* ; Humans ; Hyperinsulinism ; Hyperprolactinemia ; Insulin ; Leptin ; Obesity ; Osteoporosis ; Parents ; Parturition ; Physical Examination ; Pituitary Gland* ; Seizures

Craniopharyngiomas exhibiting histologic malignancy are extremely rare. Herein, we report the case of a 26-year-old male patient who underwent suprasellar mass excision via an interhemispheric transcallosal approach. Histopathological examination indicated that the craniopharyngioma was of the adamantinomatous subtype. The patient received postoperative medical treatment for endocrine dysfunction and diabetes mellitus without radiation treatment. Two years after the operation, he presented with progressive visual disturbance and altered mentality. Magnetic resonance imaging revealed a huge mass in the suprasellar cistern and third ventricle. He underwent a second operation via the same approach. The histopathological examination showed an adamantinomatous craniopharyngioma with sheets of solid proliferation in a spindled pattern, indicating malignant transformation. Malignant transformation of craniopharyngioma in the absence of radiation therapy has been reported in only five cases, including this one. We present a case of malignant transformation of craniopharyngioma with a brief review of relevant literature.
Adult ; Craniopharyngioma* ; Diabetes Mellitus ; Humans ; Magnetic Resonance Imaging ; Male ; Radiotherapy ; Third Ventricle