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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 -
Clinical Neurology and Neurosurgery Apr 2021Pituicytoma is a rare subtype of WHO grade I glioma that originates in the neurohypophysis or infundibulum. Here we presented 3 cases of histopathologically diagnosed... (Review)
Review
Pituicytoma is a rare subtype of WHO grade I glioma that originates in the neurohypophysis or infundibulum. Here we presented 3 cases of histopathologically diagnosed pituicytoma and subsequently performed a corresponding systematic literature review. A comprehensive literature search of the PubMed database was conducted. A total of 77 studies were eventually reviewed and 168 pituicytoma cases were identified. The epidemiology, clinical manifestations, radiological features, treatment, and pathological findings of all previous pituicytoma cases were summarized, and a "portrait" of this rare tumor was shown. It is hoped that the current study will afford a broader and more adequate understanding upon this rare disease.
PubMed: 33971477
DOI: 10.1016/j.clineuro.2021.106650 -
Der Pathologe May 2021The 2017 WHO classification of pituitary tumors is still based on structural analyses and expression of various pituitary hormones. Three innovations have to be...
The 2017 WHO classification of pituitary tumors is still based on structural analyses and expression of various pituitary hormones. Three innovations have to be considered: (1) The expression of pituitary transcription factors Pit‑1, T‑Pit and SF‑1. (2) The term "atypical adenoma" was replaced by "aggressive adenoma". (3) The three tumor types of the neurohypophysis (pituicytoma, spindle cell oncocytoma, granular cell tumor) are defined by their common expression of TTF‑1. Craniophyryngiomas are identified as adamantinomatous type by focal nuclear expression of β‑catenin or as papillary type by demonstration of BRAF V600E mutation. Further primary tumors of the pituitary are extremely rare. These and also the other tumors of the sellar region can be structurally very similar to pituitary adenomas but can be-nearly without exception-differentiated by immunocytochemistry.
Topics: Adenoma; Craniopharyngioma; Humans; Pituitary Gland, Posterior; Pituitary Neoplasms; World Health Organization
PubMed: 33877399
DOI: 10.1007/s00292-021-00932-x -
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 -
Neurosurgery Apr 2021The pituitary gland is the site of numerous neoplastic and inflammatory processes. The overwhelmingly most frequent tumors arise from cells of the anterior lobe, the... (Review)
Review
The pituitary gland is the site of numerous neoplastic and inflammatory processes. The overwhelmingly most frequent tumors arise from cells of the anterior lobe, the pituitary neuroendocrine tumors (PitNETs). Immunohistochemistry assay staining for pituitary hormones is the core tool for classifying PitNETs, resulting in the diagnosis of somatotroph PitNETs, lactotroph PitNETs, and so on. For cases showing no hormonal expression, the updated WHO classification system now considers the assessment of several transcription factors: PIT-1 (pituitary-specific POU-class homeodomain transcription factor); T-PIT (T-box family member TBX19); and SF-1 (steroidogenic factor regulating gonadotroph cell differentiation) before rendering a diagnosis of null cell adenoma. Other tumors and disease processes of this site often mimic PitNETs radiographically and sometimes even clinically (ie, compression of the optic chiasm). These potpourri of processes include germ cell neoplasms (especially germinomas), tumors that originate from Rathke's pouch (craniopharyngiomas, Rathke's cleft cyst), tumors that originate from the posterior lobe of the pituitary (pituicytoma, spindle cell oncocytoma, granular cell tumor), and tumors that originate from the meninges (especially meningiomas). In addition to neoplasms, several described inflammatory and related conditions exist that need to be distinguished from PitNETs. These include lymphocytic hypophysitis and Langerhans cell histiocytosis, a neoplastic disorder of histiocytes. In this review, we aim to briefly describe the main pituitary and sellar lesions, with emphasis on the most common tumors, the PitNETs.
Topics: Adenoma; Adolescent; Adult; Child; Female; Humans; Male; Middle Aged; Pituitary Gland; Pituitary Neoplasms; Young Adult
PubMed: 33476394
DOI: 10.1093/neuros/nyaa548 -
Endocrine Pathology Mar 2021Molecular pathology has advanced our understanding of many tumors and offers opportunities to identify novel therapies. In the pituitary, the field has uncovered several... (Review)
Review
Molecular pathology has advanced our understanding of many tumors and offers opportunities to identify novel therapies. In the pituitary, the field has uncovered several genetic mutations that predispose to pituitary neuroendocrine tumor (PitNET) development, including MEN1, CDKN1B, PRKRIα, AIP, GPR101, and other more rare events; however, these genes are only rarely mutated in sporadic PitNETs. Recurrent genetic events in sporadic PitNETs include GNAS mutations in a subset of somatotroph tumors and ubiquitin-specific peptidase mutations (e.g., USP8, USP48) in some corticotroph tumors; to date, neither of these has resulted in altered management, and instead, the prognosis and management of PitNETs still rely more on cell type and subtype as well as local growth that determines surgical resectability. In contrast, craniopharyngiomas have either CTNNB1 or BRAF mutations that correlate with adamantinomatous or papillary morphology, respectively; the latter offers the opportunity for targeted therapy. DICER1 mutations are found in patients with pituitary blastoma. Epigenetic changes are implicated in the pathogenesis of the more common sporadic pituitary neoplasms including the majority of PitNETs and tumors of pituicytes.
Topics: Epigenesis, Genetic; Epigenomics; Genomics; Humans; Pituitary Neoplasms
PubMed: 33433883
DOI: 10.1007/s12022-021-09663-4 -
Indian Journal of Pathology &... 2021Pituicytoma is a distinct sellar or supracellar tumor which originates from specialized glial cells of neurohypophyses and infundibulum known as pituicytes. Because of...
Pituicytoma is a distinct sellar or supracellar tumor which originates from specialized glial cells of neurohypophyses and infundibulum known as pituicytes. Because of its sellar location patients present with headache, visual disturbance, and endocrine abnormalities. Pituicytoma is difficult to diagnose on neuroimaging as radiological features overlap with other more common tumors of this region. Thus, diagnosis is established by histopathology and immunohistochemistry of resected tumor only. Pituicytomas are composed of bipolar spindle cells arranged as fascicles and are immunoreactive for TTF-1, S100p, and vimentin. These tumors are extremely rare and only around 70 published cases are known in literature. We report a case of suprasellar SOL in a 58-year-old male who presented with headache and gradual visual deterioration in both eyes. He was diagnosed as a case of pituicytoma based on light microscopy findings and immunohistochemical expression of TTF-1, vimentin, S100p, and bcl-2.
Topics: Biomarkers, Tumor; Calcium-Binding Proteins; DNA-Binding Proteins; Diagnosis, Differential; Genes, bcl-2; Glioma; Headache; Histological Techniques; Humans; Immunohistochemistry; Male; Middle Aged; Neoplasm Proteins; Pituitary Neoplasms; Transcription Factors; Vimentin
PubMed: 33433426
DOI: 10.4103/IJPM.IJPM_430_20 -
Journal of Neuropathology and... Jan 2021Posterior pituitary tumors are supposed to represent the morphological spectrum of a single entity. Herein, we report the clinical-pathological, immunohistochemical, and...
Posterior pituitary tumors are supposed to represent the morphological spectrum of a single entity. Herein, we report the clinical-pathological, immunohistochemical, and genetic features of 5 spindle cell oncocytomas (SCOs), 3 pituicytomas, and 1 granular cell tumor (GCT). SCOs had the highest local invasiveness and affected older subjects. The 3 histotypes differed in the content of spindle cells (predominant in pituicytoma and absent in GCT), presence of lymphocytic infiltrate (in SCO and GCT, but not in the pituicytoma) and EMA/GFAP staining (negative in GCT; EMA-positive/GFAP-negative in 4/5 SCO and GFAP-positive in 3/3 pituicytomas). Three SCOs and 1 pituicytoma analyzed with next-generation sequencing had no mutations in 409 genes. However, 1 SCO had previously unreported homozygous deletion of CDKN2A/B and another of SMARCA4, SMARCB1, and NF2. All 3 SCOs had loss of heterozygosity of chromosome 1p, while the pituicytoma had chromosome 19 homozygous loss and chromosomes 10, 13q, and 18q loss of heterozygosity. Since 1p and 13q losses were previously reported in 1 pituicytoma and 1 SCO, respectively, our data demonstrate that posterior pituitary tumors share common genetic alterations. The possibility that posterior pituitary tumors are SMARCA4/SMARCB1-deficient should be kept in mind in the differential diagnosis toward other entities.
Topics: Adenoma, Oxyphilic; Adult; Aged; DNA Mutational Analysis; Female; Granular Cell Tumor; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Male; Middle Aged; Mutation; Pituitary Gland, Posterior; Pituitary Neoplasms; Sequence Deletion; Young Adult
PubMed: 33212494
DOI: 10.1093/jnen/nlaa139 -
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