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Medicine Mar 2016Pituicytoma is a rare, low-grade glial neoplasm that arises in the neurohypophysis or infundibulum and usually presents as pituitary gland enlargement. They are often... (Review)
Review
Pituicytoma is a rare, low-grade glial neoplasm that arises in the neurohypophysis or infundibulum and usually presents as pituitary gland enlargement. They are often misdiagnosed as pituitary adenomas. Causes have varied for high serum adrenocorticotropic hormone level reported in a few patients with pituicytoma.We report a rare case of pituicytoma accompanied by corticotroph hyperplasia-a challenging diagnosis guided by clinical presentations, radiological signs, and biopsy.We present a case of pituicytoma with corticotroph hyperplasia in a 46-year-old woman with typical Cushing syndrome. Magnetic resonance imaging revealed a lesion in the sellar area with equal T1 and T2 signals and marked homogeneous enhancement. We present detailed analysis of the patient's disease course and review pertinent literature. Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary.The patient underwent a surgical exploration and tumor resection through a trans-sphenoidal approach. Pathologic results revealed pituicytoma and corticotroph hyperplasia. As adrenocorticotropic hormone and cortisol levels did not decrease to normal, the patient received radiotherapy and recovered uneventfully. No recurrence was found over 8 years of follow-up.Pituicytoma is a rare type of sellar tumor. Pituicytomas in patients with Cushing syndrome are rarer still. To our knowledge, this is the first report of Cushing syndrome caused by corticotroph hyperplasia in a pituicytoma patient.
Topics: Corticotrophs; Diagnosis, Differential; Disease Progression; Female; Glioma; Humans; Hyperplasia; Magnetic Resonance Imaging; Middle Aged; Pituitary Neoplasms
PubMed: 26962837
DOI: 10.1097/MD.0000000000003062 -
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 Sep 2017This review focuses on discussing the main changes on the upcoming fourth edition of the WHO Classification of Tumors of the Pituitary Gland emphasizing... (Review)
Review
This review focuses on discussing the main changes on the upcoming fourth edition of the WHO Classification of Tumors of the Pituitary Gland emphasizing histopathological and molecular genetics aspects of pituitary neuroendocrine (i.e., pituitary adenomas) and some of the non-neuroendocrine tumors involving the pituitary gland. Instead of a formal review, we introduced the highlights of the new WHO classification by answering select questions relevant to practising pathologists. The revised classification of pituitary adenomas, in addition to hormone immunohistochemistry, recognizes the role of other immunohistochemical markers including but not limited to pituitary transcription factors. Recognizing this novel approach, the fourth edition of the WHO classification has abandoned the concept of "a hormone-producing pituitary adenoma" and adopted a pituitary adenohypophyseal cell lineage designation of the adenomas with subsequent categorization of histological variants according to hormone content and specific histological and immunohistochemical features. This new classification does not require a routine ultrastructural examination of these tumors. The new definition of the Null cell adenoma requires the demonstration of immunonegativity for pituitary transcription factors and adenohypophyseal hormones Moreover, the term of atypical pituitary adenoma is no longer recommended. In addition to the accurate tumor subtyping, assessment of the tumor proliferative potential by mitotic count and Ki-67 index, and other clinical parameters such as tumor invasion, is strongly recommended in individual cases for consideration of clinically aggressive adenomas. This classification also recognizes some subtypes of pituitary neuroendocrine tumors as "high-risk pituitary adenomas" due to the clinical aggressive behavior; these include the sparsely granulated somatotroph adenoma, the lactotroph adenoma in men, the Crooke's cell adenoma, the silent corticotroph adenoma, and the newly introduced plurihormonal Pit-1-positive adenoma (previously known as silent subtype III pituitary adenoma). An additional novel aspect of the new WHO classification was also the definition of the spectrum of thyroid transcription factor-1 expressing pituitary tumors of the posterior lobe as representing a morphological spectrum of a single nosological entity. These tumors include the pituicytoma, the spindle cell oncocytoma, the granular cell tumor of the neurohypophysis, and the sellar ependymoma.
Topics: Humans; Pituitary Neoplasms; World Health Organization
PubMed: 28766057
DOI: 10.1007/s12022-017-9498-z -
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 -
Human Pathology Jan 2016Pituicytoma is a rare benign neoplasm arising in the sellar region, usually found in the posterior lobe and/or pituitary stalk. Here, we report the case of a 67-year-old...
Pituicytoma is a rare benign neoplasm arising in the sellar region, usually found in the posterior lobe and/or pituitary stalk. Here, we report the case of a 67-year-old woman who presented with bitemporal hemianopsia and visual impairment accompanied by mildly elevated prolactin. Pathologic and molecular examination of the tissue removed transsphenoidally revealed 2 distinct tumors: pituitary adenoma and pituicytoma. To the best of our knowledge, histologically proven pituicytoma and pituitary adenoma have never been reported together.
Topics: Adenoma; Aged; Biomarkers, Tumor; Biopsy; DNA Methylation; Endoscopy; Female; Hemianopsia; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Neoplasms, Multiple Primary; Pituitary Neoplasms; Prolactin; Visual Fields
PubMed: 26476569
DOI: 10.1016/j.humpath.2015.08.017 -
Oncology Letters Nov 2016Pituicytoma is a rare tumor of the sellar and suprasellar regions, arising from the pituicytes, which are specialized glial cells in the neurohypophysis and...
Pituicytoma is a rare tumor of the sellar and suprasellar regions, arising from the pituicytes, which are specialized glial cells in the neurohypophysis and infundibulum. Due to its rarity, ambiguity persists over the diagnosis, management and prognosis of pituicytoma. The current study presents a case series of three patients, each with a histopathological diagnosis of pituicytoma. A summary of the clinical manifestations, radiological characteristics, histopathological features, treatment strategies and prognoses are presented. In addition, 78 cases of pituicytoma, identified in a search of the published literature in Pubmed, are profiled. Pituicytoma typically presents with dysfunction of the optic nerve and pituitary. The radiological characteristics are nonspecific; diagnosis is typically made on the basis of histopathological results. The tumor is slow growing and benign and is amenable to surgical treatment by gross total resection; subsequent tumor recurrence is rare. A definitive assessment of prognosis requires an extended follow-up in a larger cohort.
PubMed: 27900014
DOI: 10.3892/ol.2016.5119 -
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 -
CNS Neuroscience & Therapeutics Jul 2017Pituicytoma is a rare neoplasm of the sella region. Tumor resection is the primary treatment option, but remains subtotal due to excessive bleeding in many cases. The...
INTRODUCTION
Pituicytoma is a rare neoplasm of the sella region. Tumor resection is the primary treatment option, but remains subtotal due to excessive bleeding in many cases. The search for alternative or additional treatment regimens is necessary.
AIMS
We aimed to determine the receptor expression of pituicytoma to find alternatives or supplements to surgical therapy in the use of targeted therapies.
METHODS
Pituicytoma samples were collected from three institutions between 2006 and 2015 and were stained for vascular endothelial growth factors (VEGF), thyroid transcription factor (TTF1), and somatostatin receptors (SSTR 2/3/5). The stains were classified from 0=no staining to +++=strong staining. A complementary retrospective analysis of the patient charts regarding sex, age, and primary symptoms, pituitary function, and perioperative complications was performed.
RESULTS
Ten samples were analyzed; mean patient age was 57.8 years SD 16.3 years. Seven samples were acquired from male patients (one relapse) and three from female. All tumors stained strongly positive (+++) for VEGF-R. Six samples stained positive for TTF1. As for somatostatin receptors, three samples were slightly positive for SSTR 2; seven were negative. SSTR 3 was + in one, three were ++, three were +++, and three were 0. SSTR 5 stained +++ in 1, ++ in 5, + in 1, and 0 in three patients.
CONCLUSIONS
Pituicytomas were generally positive for VEGFR and showed regular expression of SSTR 3 and 5 indicating a possible treatment option through targeted therapies in cases where resection remains insufficient. Further research is necessary as to whether tumor growth can be inhibited using these pathways.
Topics: Adult; Aged; DNA-Binding Proteins; Female; Humans; Intraoperative Complications; Male; Middle Aged; Neoplasm Recurrence, Local; Pituitary Neoplasms; Postoperative Complications; Receptors, Somatostatin; Retrospective Studies; Transcription Factors; Vascular Endothelial Growth Factor A; Young Adult
PubMed: 28556544
DOI: 10.1111/cns.12709