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Military Medical Research Jul 2021Pituicytoma is an extremely rare low-grade glial tumor that is closely related to the neurohypophysis axis. Most studies of pituicytomas include only several cases. To...
Clinical features, radiological profiles, pathological features and surgical outcomes of pituicytomas: a report of 11 cases and a pooled analysis of individual patient data.
BACKGROUND
Pituicytoma is an extremely rare low-grade glial tumor that is closely related to the neurohypophysis axis. Most studies of pituicytomas include only several cases. To better understand this disease, we reviewed a series of cases of pituicytomas. The diagnosis and treatment of pituicytoma must be further elucidated.
METHODS
Eleven patients with pituicytoma admitted to Beijing Tiantan Hospital from 2012 to 2019 were selected. The clinical features, including radiological and histological examination, surgical records and prognosis were reviewed. Sixty-eight other previously published cases of pituicytoma also were used to analyze the predictive factors for the results. The Cox regression model was used for univariate and multivariate analyses.
RESULTS
Our patients included 5 males (45.5%) and 6 females (54.5%), with a mean age of 49.3 years. The tumor was located in the suprasellar region in 5 patients (45.5%), intrasellar region in 4 patients (36.4%), and intrasellar-suprasellar region in 2 patients (18.2%). All patients were misdiagnosed with other common tumors in the sellar region before the operation. During the operation, gross total resection (GTR) of the tumor was achieved in 6 patients (54.5%), and subtotal resection (STR) was achieved in 5 patients (45.5%). The mean progression-free survival (PFS) time was 29.82 months. Tumor progression after surgical resection occurred in 4 patients (36.4%). Among them, 60.0% of the patients (cases 4, 5, 7) with STR experienced progression, while 16.7% of the patients (case 2) with GTR experienced progression. Combined with the 68 cases in the literature, GTR was an independent risk factor for PFS time (P < 0.05).
CONCLUSIONS
Pituicytomas are more common in middle-aged people and the sellar region. The clinical manifestations of pituicytomas are different, but no diagnostic clinical features have been identified other than an abnormally abundant blood supply. Currently, GTR is the best approach for the treatment of pituicytomas. More patients and longer follow-up periods were needed to further elucidate the biological features of pituicytomas.
Topics: Adult; Beijing; Female; Glioma; Humans; Male; Middle Aged; Outcome Assessment, Health Care; Pituitary Gland; Proportional Hazards Models; Radiography; Retrospective Studies; Treatment Outcome
PubMed: 34210357
DOI: 10.1186/s40779-021-00332-5 -
Pituitary Oct 2021Preoperative diagnosis of pituicytomas is difficult, and management and prognostic factors remain ambiguous. The purpose of this study was to elucidate the radiological... (Review)
Review
PURPOSE
Preoperative diagnosis of pituicytomas is difficult, and management and prognostic factors remain ambiguous. The purpose of this study was to elucidate the radiological characteristics of pituicytoma, to assess the risk factors affecting tumor progression, and to propose the optimal treatment regimen based on comprehensive analysis.
METHODS
We reviewed the clinical data of 22 patients with pituicytoma confirmed pathologically in our institution. In addition, 93 cases of pituicytoma in the previous literature were recruited. The individual data of 115 patients were analyzed to evaluate the adverse factors affecting pituicytoma progression.
RESULTS
In the combined cohort, 3 of 61 patients who underwent gross-total resection (GTR) developed recurrence (4.9%); of the 54 patients who received non-GTR, 19 progressed (35.2%). Univariate and multivariate Cox regression analysis verified male gender (HR 2.855, 95% CI 1.008-8.089; p = 0.048), TS (transsphenoidal surgery; HR 3.559, 95% CI 1.015-12.476; p = 0.047), and non-GTR (HR 4.388, 95%CI 1.240-15.521; p = 0.022) were independent unfavorable factors for pituicytoma progression. A multivariate logistic regression model verified that tumor diameter ≥ 1.85 cm (OR 4.859, 95% CI 1.335-17.691; p = 0.016) was independent adverse factors for GTR. Compared with TS, OT (open transcranial) is more likely to have postoperative complications (OR 3.185, 95% CI 1.020-9.944; p = 0.046), especially vision deterioration (OR 37.267, 95% CI 4.486-309.595; p = 0.001).
CONCLUSION
Based on our findings, GTR was advocated as an optimal treatment for pituicytomas. However, in order to avoid damage to important structures, partial resection is acceptable. After that, adjuvant radiotherapy is recommended for male patients with high Ki-67 index, and the remaining patients can be followed up closely. When the tumor recurs or progresses, it is recommended to re-operate and remove the lesion completely as far as possible. If GTR is still not possible, postoperative radiotherapy for the residual tumor is recommended.
Topics: Craniopharyngioma; Glioma; Humans; Male; Pituitary Neoplasms; Prognosis; Retrospective Studies
PubMed: 33982223
DOI: 10.1007/s11102-021-01152-5 -
Posterior pituitary tumours: patient outcomes and determinants of disease recurrence or persistence.Endocrine Connections Apr 2021Posterior pituitary tumours (PPTs) are rare neoplasms with the four recognised subtypes unified by thyroid transcription factor -1 (TTF-1) expression, according to the...
OBJECTIVE
Posterior pituitary tumours (PPTs) are rare neoplasms with the four recognised subtypes unified by thyroid transcription factor -1 (TTF-1) expression, according to the 2017 WHO classification. Though traditionally defined as low-grade neoplasms, a substantial proportion of them show recurrence/persistence following surgery.
METHODS
We selected patients with PPTs in our cohort of 1760 patients operated for pituitary tumours over the past 10 years (2010-2019). The clinical, radiological, hormonal, histopathological profiles and long-term outcomes of the three cases identified (two pituicytomas and one spindle cell oncocytoma, SCO) were analysed. Following a literature review, data of all published cases with documented TTF-1 positive pituicytomas and SCOs were analysed to determine the predictors of recurrence/persistence in these tumours.
RESULTS
Patients presented with compressive features or hypogonadism. Two had sellar-suprasellar masses. One had a purely suprasellar mass with a pre-operative radiological suspicion of pituicytoma. Two were operated by transsphenoidal surgery and one transcranially guided by neuronavigation. Histopathology confirmed spindle cells in a storiform arrangement and low Ki67 index. Immunohistochemistry showed positive TTF-1, S-100 expression and variable positivity for EMA, vimentin and GFAP. Re-evaluation showed recurrence/persistence in two patients. A literature review of recurrent/persistent pituicytoma (n = 17) and SCO (n = 9) cases revealed clinical clues (headache for pituicytomas, male gender for SCO), baseline tumour size (≥20.5 mm with sensitivity exceeding 80%) and longer follow-up duration as determinants of recurrence/persistence.
CONCLUSION
PPTs are rare sellar masses with quintessential TTF-1 positivity. Recurrent/persistent disease following surgery is determined by greater tumour size at baseline and duration of follow-up. This warrants intensive and long-term surveillance in these patients.
PubMed: 33709954
DOI: 10.1530/EC-20-0621 -
Pituitary Apr 2021Sellar/parasellar tumors (SPTs) very rarely become symptomatic during pregnancy. No specific guidelines exist for their management, that is extremely challenging as...
INTRODUCTION
Sellar/parasellar tumors (SPTs) very rarely become symptomatic during pregnancy. No specific guidelines exist for their management, that is extremely challenging as mother and fetus health can be jeopardized.
MATERIALS AND METHODS
Data of patients with SPTs becoming symptomatic during pregnancy treated at two Italian referral Centers were retrospectively collected. Systematic literature review was also performed.
RESULTS
Our series consisted of 6 cases, 3 meningiomas, 1 ACTH-secreting adenoma, 1 pituicytoma and 1 craniopharyngioma. Mean age at presentation was 33.6 ± 6.0 years. Five patients complained of visual disturbances, associated with headache in one case, that occurred between gestation week (GW) 22 and 34. In 5 cases, pregnancy was uneventful with the delivery of a healthy baby between GW 33 and 35, followed by endoscopic surgical tumor exeresis (n = 4) or proton bean therapy (n = 1). Another patient presented with stigmata typical of Cushing's syndrome and rapidly worsening pre-eclampsia, that required pregnancy interruption and adenomectomy. Based on personal and literature cases, a practical algorithm was proposed to help clinicians dealing with these patients.
CONCLUSIONS
SPTs becoming symptomatic in pregnancy deserve careful monitoring and multidisciplinary management. Overall, wait-and-see approach is suggested, reserving surgery to patients with rapidly progressive/life-threatening situations, significant risk of permanent neurological impairment or malignant lesions.
Topics: Female; Humans; Pregnancy; Algorithms; Retrospective Studies; Central Nervous System Neoplasms; Pregnancy Complications, Neoplastic
PubMed: 33200305
DOI: 10.1007/s11102-020-01107-2 -
Journal of Neurological Surgery. Part... Oct 2020To identify perioperative factors that may predict postoperative cerebrospinal fluid (CSF) leak and meningitis following expanded endoscopic transsphenoidal surgery...
To identify perioperative factors that may predict postoperative cerebrospinal fluid (CSF) leak and meningitis following expanded endoscopic transsphenoidal surgery (EETS). This is a retrospective study. This study was set at the Cedars-Sinai Medical Center, Los Angeles. A total of 78 patients who underwent EETS between January 2007 and November 2018 were participated. The main outcome measures were CSF leak and meningitis. A total of 78 patients underwent a total of 100 EETS procedures; 17.9 and 10.3% of patients developed postoperative CSF leaks and meningitis, respectively. Out of eight, three patients with meningitis did not develop an observable CSF leak. The risk of developing meningitis in patients with a CSF leak was significantly higher than those without a leak, with an odds ratio (OR) of 11.48 (95% confidence interval, 2.33-56.47; = 0.004). Pituicytomas were significantly associated with meningitis compared with other pathologies. No other patient-specific factors were identified as risks for leak or meningitis, including method of skull base repair, sex, tumor volume, or body mass index, although there was a strong trend toward reduced CSF leak rates in patient with nasoseptal flaps used for skull base repair, compared with those without (9.5 vs. 25%). CSF protein was consistently elevated on the first CSF values obtained when meningitis was suspected. CSF leak and meningitis are common complications of expanded endonasal surgery No statistically significant risk factors for developing a postoperative leak other than the pathology of pituicytoma were identified, including method of skull base repair, although the use of a vascularized nasoseptal flap did trend toward a reduced CSF leak rate. CSF protein is the most sensitive marker for the presumptive diagnosis and timely treatment of meningitis.
PubMed: 33134016
DOI: 10.1055/s-0039-1696999 -
Journal of Surgical Case Reports Jun 2020Pituicytoma is a rare tumor that has been recently recognized and described, where only few reported cases of pituicytoma associated with Cushing's disease. We describe...
Pituicytoma is a rare tumor that has been recently recognized and described, where only few reported cases of pituicytoma associated with Cushing's disease. We describe a case of a 47 years old female with a history of high cortisol levels and a diagnosis of Cushing's disease was made. Brain magnetic resonance imaging showed lesion in pituitary gland compatible with microadenoma and tumor resection was carried out. The histopathological findings were of a pituicytoma with positive thyroid transcription factor-1 immunostain.
PubMed: 32577204
DOI: 10.1093/jscr/rjaa104 -
Surgical Neurology International 2020Granular cell tumor (GCT) of the sellar region is a rare tumor of the sellar and suprasellar regions that originate from the neurohypophysis. This tumor is very...
BACKGROUND
Granular cell tumor (GCT) of the sellar region is a rare tumor of the sellar and suprasellar regions that originate from the neurohypophysis. This tumor is very difficult to differentiate from other pituitary neoplasms, such as pituitary adenoma, pituicytoma, and spindle cell oncocytoma. We report a rare case of GCT arising from the posterior pituitary of the sellar region and suggest a useful indicator for accurate diagnosis and pitfalls for surgical procedures.
CASE DESCRIPTION
A 42-year-old woman was admitted to our hospital with bitemporal hemianopsia. Neuroimaging showed a large pituitary tumor in the sellar and suprasellar regions with a hypointense part on T2-weighted magnetic resonance imaging, and the enhanced anterior pituitary gland was displaced anteriorly. Laboratory findings showed mild hyperprolactinemia. Subtotal resection of the tumor was achieved using an endoscopic endonasal transsphenoidal approach. Histological findings showed round or polygonal cells with abundant granular eosinophilic cytoplasm staining strongly for thyroid transcription factor 1. The tumor was, therefore, diagnosed as a GCT of the sellar region, belonging to tumors of the posterior pituitary. After surgery, visual impairment and anterior pituitary function were improved. Follow-up neuroimaging after 1 year showed no signs of recurrence.
CONCLUSION
GCT of the sellar region is difficult to diagnose on routine neuroimaging. Therefore, accurate diagnosis requires careful identification of clinical signs, magnetic resonance imaging including hypointensity on T2-weighted imaging, and analysis of combined morphological and immunohistochemical studies.
PubMed: 32494380
DOI: 10.25259/SNI_111_2020 -
World Journal of Surgical Oncology May 2020Pituicytoma is a rare pituitary non-neuroendocrine tumour. The awareness of pituitary non-neuroendocrine tumours has gradually increased over the past several decades,...
BACKGROUND
Pituicytoma is a rare pituitary non-neuroendocrine tumour. The awareness of pituitary non-neuroendocrine tumours has gradually increased over the past several decades, but the knowledge of some histological variants of the tumours is limited, particularly in clinicopathological significance. Here, we report a rare case of pituicytoma variant.
CASE PRESENTATION
A 71-year-old man presented with sudden symptoms of stroke including urinary incontinence, weakness in right lower limb, and trouble speaking. Physical examinations showed a right facial paralysis. The radiological examinations eventually found a 1.7 × 1.4 × 1.3 cm sellar occupied lesion. After symptomatic treatment improved the symptoms, the patient underwent transsphenoidal resection of the pituitary mass. Histologically, the tumour contained hypocellular area and hypercellular area. The hypocellular area showed elongated spindle cells arranged in a fascicular pattern around small vessels and scattered Herring bodies; the hypercellular area showed a large number of pseudorosettes. Immunohistochemistrically, the tumour cells were positive for thyroid transcription factor-1, S100, and neuron-specific enolase. Neurofilament only showed a little positive in the hypocellular area, and silver impregnation was only noted in a perivascular distribution. The patient had no recurrence 4 months after the surgery.
CONCLUSIONS
The rare variant of pituicytoma has a favourable prognosis. Moreover, it needs to be distinguished pituicytomas with pseudorosettes from ependymomas because of different prognosis. Lastly, Herring bodies may occasionally be seen in the pituicytoma, which could be a potential diagnostic pitfall.
Topics: Aged; Biomarkers, Tumor; Humans; Magnetic Resonance Imaging; Male; Pituitary Gland, Posterior; Pituitary Neoplasms
PubMed: 32460843
DOI: 10.1186/s12957-020-01889-6 -
Annals of the Royal College of Surgeons... Apr 2020Pituicytoma is a rare; slow-growing primary tumour originating from the glial cells of the neurohypophysis. It was classed as a low-grade (grade I) central nervous...
BACKGROUND
Pituicytoma is a rare; slow-growing primary tumour originating from the glial cells of the neurohypophysis. It was classed as a low-grade (grade I) central nervous system tumour by the World Health Organization in 2007. We present the case of a 71-year-old man with a previous history of sellar meningioma who underwent surgical resection of pituicytoma by transphenoidal hypophysectomy.
METHOD
We conducted a literature search using Medline EMBASE and the Cochrane Database of Systematic Reviews using the Ovid search engines. Key words searched were 'pituitary gland', 'pituicytoma', 'neurohypophysis'. Patient records and imaging were obtained from the Royal Stoke University Hospital database.
RESULTS
A 71-year-old man with a background of sellar meningioma 16 years previously was found to have a pituitary fossa tumour on surveillance magnetic resonance imaging. Differential diagnosis was pituitary adenoma. Endoscopic transphenoidal hypophysectomy was performed. Histology was consistent with pituicytoma. As a result of this histology from his initial tumour was re-examined and was amended to pituicytoma, indicating a recurrence.
CONCLUSION
Pituicytoma is a benign, slow growing lesion of the posterior pituitary. Brat diagnosed it as a distinct entity in 2000 and it was listed as a World Health Organization classification grade I tumour in 2007. It can be mistaken for pituitary adenoma owing to its similar clinical presentation. Complete excision of the tumour by transphenoidal hypophysectomy is an effective management for pituicytoma as demonstrated in this case.
Topics: Aged; Diagnostic Errors; Humans; Magnetic Resonance Imaging; Male; Meningioma; Neoplasm Recurrence, Local; Pituitary Gland, Posterior; Pituitary Neoplasms; Transanal Endoscopic Surgery; Treatment Outcome
PubMed: 31964155
DOI: 10.1308/rcsann.2020.0004 -
Endocrine Regulations Oct 2019Pituicytomas are rare, solid, well-circumscribed, low grade (grade I), non-neuroendocrine, and noninfiltrative tumors of the neurohypophysis or infundibulum, which...
OBJECTIVES
Pituicytomas are rare, solid, well-circumscribed, low grade (grade I), non-neuroendocrine, and noninfiltrative tumors of the neurohypophysis or infundibulum, which appear in the sellar/suprasellar regions. Herein, we present a case with Cushing's disease (CD) caused by an ACTH-secreting pituitary adenoma in association with an infundibular pituicytoma. Subject and Results. A 37-year-old male patient presented to the hospital with a six-month history of blurry vision. Physical examination demonstrated plethora, excessive sweating, weight gain, moon facies, and acne. Basal serum cortisol and ACTH levels were 16 µg/dl and 32 pg/ml, respectively. The results of screening tests were suggestive of Cushing syndrome. It was also 1.97 µg/dl following 8 mg dexamethasone suppression test which was consistent with CD. Pituitary MR imaging revealed a single lesion measuring 6x6.5 mm on the pituitary stalk. Infundibular mass excision and pituitary exploration by extended endoscopic endonasal approach were applied. On immunohistochemistry, strong diffuse immunolabeling for both S100 and TTF-1 was noted for the cells of infundibular mass, diagnosed as pituicytoma. Because the developed panhypopituitarism postoperatively, patient was discharged with daily desmopressin, levothyroxine, hydrocortisone, and intramuscular testosterone, once a month.
CONCLUSIONS
Pituicytoma is an uncommon noninvasive tumor of the sellar and suprasellar regions. In this case report, we described a patient with Cushing's disease to whom MRI displayed only an infundibular well-circumscribed lesion, but not any pituitary adenoma. Despite the absence of any sellar lesion, awareness of other undetected possible lesion and exploring hypophysis during the transsphenoidal surgery is mandatory for the correct diagnosis.
Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Adrenocorticotropic Hormone; Adult; Humans; Male; Neoplasms, Multiple Primary; Pituitary ACTH Hypersecretion; Pituitary Gland; Pituitary Neoplasms
PubMed: 31734654
DOI: 10.2478/enr-2019-0026