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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 -
Asian Journal of Surgery Mar 2023
Topics: Humans; Pituitary Neoplasms; Glioma; Magnetic Resonance Imaging
PubMed: 36180358
DOI: 10.1016/j.asjsur.2022.08.116 -
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 -
World Neurosurgery Feb 2020To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy... (Review)
Review
OBJECTIVE
To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy for these rare tumors and add to the limited existing literature.
METHODS
Retrospective review of 8 cases (5 pituicytomas, 2 spindle cell oncocytomas, and 1 granular cell tumor) managed at our institution between 2004 and 2019. The patients' clinical course, histologic features, and radiologic findings were reviewed. Their management and long-term follow-up is presented and compared with the literature.
RESULTS
Long-term follow-up ranged from 1 to 9 years. There was 1 recurrence in a patient with spindle cell oncocytoma, and this was treated with radiotherapy. The endoscopically managed cases resulted in complete tumor excision with no recurrence.
CONCLUSIONS
Epidemiologic data on primary tumors of the neurohypophysis is limited because of the rarity of these tumors. This study adds to the literature that these tumors behave as World Health Organization grade I tumors, although close follow-up is recommended as a few cases have shown recurrence. The endoscopic approach resulted in better gross total tumor resection rate in this series.
Topics: Adenoma, Oxyphilic; Adult; Aged; Aged, 80 and over; Cerebral Intraventricular Hemorrhage; Cytoreduction Surgical Procedures; Female; Glioma; Granular Cell Tumor; Hemianopsia; Humans; Hypogonadism; Incidental Findings; Male; Microsurgery; Middle Aged; Neoplasm, Residual; Neuroendoscopy; Pituitary Gland, Posterior; Pituitary Neoplasms; Sphenoid Bone
PubMed: 31734425
DOI: 10.1016/j.wneu.2019.11.043 -
Pathology Apr 2023
Topics: Humans; ACTH-Secreting Pituitary Adenoma; Pituitary ACTH Hypersecretion; Pituitary Neoplasms; Adenoma; Glioma
PubMed: 36351865
DOI: 10.1016/j.pathol.2022.08.005 -
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 -
British Journal of Neurosurgery Oct 2020Pituicytomas and granular cell tumours (GCTs) of the neurohypophysis are considered non-adenomatous neoplasms in the sellar region. The association between hormone...
Pituicytomas and granular cell tumours (GCTs) of the neurohypophysis are considered non-adenomatous neoplasms in the sellar region. The association between hormone hypersecretion and the tumours is seldom discussed and unclear. Therefore, we attempt to investigate this association based on our experience and a review of the literature. We report three patients who presented with Cushing's syndrome- or acromegaly-like symptoms at our institution. They underwent transsphenoidal surgery for suspected pituitary adenomas, which were subsequently diagnosed as pituicytomas or hypophyseal GCTs following histological and immunohistochemical analysis. We also review previously reported relevant cases of pituitary non-adenomatous tumours in the literature. Four cases of Cushing's syndrome with pituicytoma and one case of acromegaly with a GCT have recently been reported. In the three cases presented here, one patient with Cushing's syndrome and one patient with acromegaly also had a pituicytoma, while the second patient with acromegaly had a GCT. Rather than mere coexistence of non-adenomatous pituitary tumours with hypersecretory adenomas or hyperplasia, alternative causes for the observed symptoms maybe the presence of some unidentified substances produced by the tumours that stimulate the adenohypophysis to secrete pituitary hormones. The glial cells of the pituitary gland may play an important role in oncogenic differentiation and regulation of the release of hormones. Therefore, attention should be focused on investigating the origin and functions of glial cells.
Topics: Acromegaly; Adenoma; Cushing Syndrome; Humans; Pituitary Gland; Pituitary Neoplasms
PubMed: 29667493
DOI: 10.1080/02688697.2018.1464121 -
International Journal of Surgery Case... Jun 2023Pituicytomas are extremely rare cancers of the sellar and suprasellar region that appear from the infundibulum or posterior pituitary. World Health Organization in 2007,...
INTRODUCTION AND IMPORTANCE
Pituicytomas are extremely rare cancers of the sellar and suprasellar region that appear from the infundibulum or posterior pituitary. World Health Organization in 2007, described pituicytoma as a low-grade tumour (Grade I) in the taxonomy of CNS cancers. The tumour can frequently simulate a pituitary adenoma and is also linked with hormonal disorders. Distinguishing a pituitary adenoma from a pituicytoma can be challenging. We present a rare case report where an elderly female showed high levels of prolactin mainly due to mass effects along with diagnostic, imaging, and immunohistochemical characteristics of pituicytoma.
CASE PRESENTATION
A 50-year-old female known case of hypothyroidism, complained of headache associated with dizziness and blurry vision. Her prolactin levels were high which led to the suspicion of pituitary involvement and underwent MRI. The imaging study revealed a well-defined, completely suprasellar, homogenously enhancing mass lesion arising from the left lateral aspect of the pituitary infundibulum. The initial differential diagnosis from the imaging included an ectopic pituitary gland, adenoma, pituicytoma, or hypothalamic glioma. She underwent a right supra-orbital craniotomy for debulking of the pituitary stalk lesion. The histopathological diagnosis was pituicytoma, WHO grade I.
CLINICAL DISCUSSION
The clinical manifestations are mostly depended upon the tumour mass and position. They typically present due to mass effects leading to hormonal disorders. The imaging studies are the backbone of the clinical diagnosis along with the histopathological findings. Surgical resection is the preferred treatment for pituicytoma, with an exceptionally low recurrence rate (4.3 %) following complete removal.
CONCLUSION
Pituicytomas are slow-growing, benign glial growths. It is challenging to diagnose before surgery as its clinical manifestations and imaging findings look like those of non-functional pituitary adenomas. The effective treatment for pituicytoma is gross total resection by the endoscopic method or transcranial technique.
PubMed: 37269767
DOI: 10.1016/j.ijscr.2023.108348 -
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 -
World Neurosurgery: X Jul 2023Pituicytoma (PTs) is a rare tumor of the sella and suprasellar region, derived from the pituicytes of the neurohypophysis, having distinct histological characteristics...
BACKGROUND
Pituicytoma (PTs) is a rare tumor of the sella and suprasellar region, derived from the pituicytes of the neurohypophysis, having distinct histological characteristics of glial neoplasms. We reported, the clinical data, neuroimaging studies, surgical approaches and pathology in five patients with PTs and also, we reviewed the literature.
METHODS
Retrospective chart from five consecutive patients with PTs treated at one University Hospital from 2016 to 2021 were reviewed. In addition, we conducted a search in PubMed/Medline databases using the term "Pituicytoma". Data regarding age, gender, pathological findings, and treatment modality applied were extracted.
RESULTS
All patients were female, aged 29-63, complaining of headaches, visual loss and field defects, dizziness and normal or abnormal levels of circulating pituitary hormones. Magnetic Resonance Imaging (MRI) showed in all patients a sellar and suprasellar mass, which was removed through an endoscopic transsphenoidal approach. Our third patient had a subtotal resection followed by close observation. Histopathology showed a glial non-infiltrative tumors with spindle cells, and a final diagnosis of pituicytoma was made. After surgery, visual field defects in all patients were normalized, and in two patients normal levels of plasma hormones were restored. After a mean of three years follow-up, the patients were managed post-operatively through close clinical observation and serial MRI. None of the patients had recurrence of the disease.
CONCLUSION
PTs is a rare glial tumor of the sellar and suprasellar region that arises from neurohypophyseal pituicytes. Disease control may be achieved by total excision.
PubMed: 37026087
DOI: 10.1016/j.wnsx.2023.100186