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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 -
Neuropathology and Applied Neurobiology Feb 2018This short review highlights significant changes and recent findings incorporated to varying extent in the WHO 2016 definition of a variety of tumours, including... (Review)
Review
This short review highlights significant changes and recent findings incorporated to varying extent in the WHO 2016 definition of a variety of tumours, including peripheral nerve sheath tumours, meningiomas, mesenchymal nonmeningothelial tumours, melanocytic tumours, lymphomas and histiocytic tumours, germ cell tumours and non-neuroendocrine pituitary tumours. Most notable classification changes include: adding 'hybrid nerve sheath tumours' to the spectrum of benign nerve sheath tumours; an updated definition of atypical meningioma (WHO grade II), including cases with brain invasion; recognizing dural solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) as a single tumour entity characterized by NAB2 and STAT6 gene fusions for which the term SFT/HPC was chosen; recognizing that pituitary granular cell tumour, spindle cell oncocytoma, and pituicytoma all share nuclear expression of TTF-1, possibly representing a spectrum of a single nosological entity derived from posterior pituitary glial cells. The most significant diagnostic markers which have emerged include: inactivation of NF1, CDKN2A, and PRC2 components, SUZ12 and EED in MPNST, leading to neurofibromin and H3K27me3 expression loss; GNAQ and GNA11 mutations in CNS primary melanocytic tumours; BRAFV600E mutation in histiocytic tumours (Langerhans cell histiocytosis and Erdheim-Chester disease) and papillary craniopharyngioma, which provides both a diagnostic marker in the appropriate pathological setting and a therapeutic target. The WHO 2016 Classification has balanced cutting-edge knowledge on the molecular characteristics of the miscellaneous CNS tumours reviewed here with a practical approach for their daily diagnostic work-up. Much more progress can be expected in the classification of these neoplasms in the near future.
Topics: Central Nervous System Neoplasms; Humans; World Health Organization
PubMed: 28295484
DOI: 10.1111/nan.12397 -
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 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 -
Brain Tumor Pathology Apr 2018The fourth edition of the World Health Organization classification of endocrine tumors (EN-WHO2017) was released in 2017. In this new edition, changes in the... (Review)
Review
The fourth edition of the World Health Organization classification of endocrine tumors (EN-WHO2017) was released in 2017. In this new edition, changes in the classification of non-neuroendocrine tumors are proposed particularly in tumors arising in the posterior pituitary. These tumors are a distinct group of low-grade neoplasms of the sellar region that express thyroid transcription factor-1, and include pituicytoma, granular cell tumor of the sellar region, spindle cell oncocytoma, and sellar ependymoma. This short review focuses on the classification of posterior pituitary tumors newly proposed in EN-WHO2017, and controversies in their pathological differential diagnosis are discussed based on recent cases.
Topics: Adenoma, Oxyphilic; Biomarkers, Tumor; Diagnosis, Differential; Ependymoma; Granular Cell Tumor; Humans; Neoplasm Staging; Pituitary Gland, Posterior; Pituitary Neoplasms; Sella Turcica; Thyroid Nuclear Factor 1; World Health Organization
PubMed: 29500747
DOI: 10.1007/s10014-018-0311-6 -
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 -
Brain Tumor Research and Treatment Oct 2021Pituicytoma is a rare solid benign tumor of the sellar and/or suprasellar region originating from the pituicytes of the neurohypophysis or infundibulum, which is not...
Pituicytoma is a rare solid benign tumor of the sellar and/or suprasellar region originating from the pituicytes of the neurohypophysis or infundibulum, which is not differentiated from a pituitary adenoma that is diagnosed mostly in the sellar and/or suprasellar region. In addition, cystic tumors are very rare and have not been reported due to their solid and hypervascular natures. A 33-year-old man presented with a chronic headache which exacerbated recently. MRI was performed and revealed a cystic tumor in the sellar and suprasellar regions with a small parenchymal island in the cyst compressing the optic chiasm. The endoscopic endonasal transsphenoidal approach was used to remove the tumor. Immunohistochemical staining was positive for thyroid transcription factor 1, S-100 protein, and glial fibrillary acidic protein. The pituicytoma was diagnosed based on histologic findings. The authors review herein the literature on clinical presentation, diagnosis, surgical management, and outcome.
PubMed: 34725987
DOI: 10.14791/btrt.2021.9.e11 -
Acta Neuropathologica Dec 2021Pituicytoma (PITUI), granular cell tumor (GCT), and spindle cell oncocytoma (SCO) are rare tumors of the posterior pituitary. Histologically, they may be challenging to...
Pituicytoma (PITUI), granular cell tumor (GCT), and spindle cell oncocytoma (SCO) are rare tumors of the posterior pituitary. Histologically, they may be challenging to distinguish and have been proposed to represent a histological spectrum of a single entity. We performed targeted next-generation sequencing, DNA methylation profiling, and copy number analysis on 47 tumors (14 PITUI; 12 GCT; 21 SCO) to investigate molecular features and explore possibilities of clinically meaningful tumor subclassification. We detected two main epigenomic subgroups by unsupervised clustering of DNA methylation data, though the overall methylation differences were subtle. The largest group (n = 23) contained most PITUIs and a subset of SCOs and was enriched for pathogenic mutations within genes in the MAPK/PI3K pathways (12/17 [71%] of sequenced tumors: FGFR1 (3), HRAS (3), BRAF (2), NF1 (2), CBL (1), MAP2K2 (1), PTEN (1)) and two with accompanying TERT promoter mutation. The second group (n = 16) contained most GCTs and a subset of SCOs, all of which mostly lacked identifiable genetic drivers. Outcome analysis demonstrated that the presence of chromosomal imbalances was significantly associated with reduced progression-free survival especially within the combined PITUI and SCO group (p = 0.031). In summary, we observed only subtle DNA methylation differences between posterior pituitary tumors, indicating that these tumors may be best classified as subtypes of a single entity. Nevertheless, our data indicate differences in mutation patterns and clinical outcome. For a clinically meaningful subclassification, we propose a combined histo-molecular approach into three subtypes: one subtype is defined by granular cell histology, scarcity of identifiable oncogenic mutations, and favorable outcome. The other two subtypes have either SCO or PITUI histology but are segregated by chromosomal copy number profile into a favorable group (no copy number changes) and a less favorable group (copy number imbalances present). Both of the latter groups have recurrent MAPK/PI3K genetic alterations that represent potential therapeutic targets.
Topics: Adenoma, Oxyphilic; Epigenesis, Genetic; Granular Cell Tumor; Humans; Pituitary Neoplasms
PubMed: 34661724
DOI: 10.1007/s00401-021-02377-1 -
Endocrine Pathology Dec 2021Between 1996 and 2020, 12,565 cases were enrolled in the German Registry of Pituitary Tumors including 10,084 PitNETs (10,067 adenomas and 19 carcinomas obtained...
Between 1996 and 2020, 12,565 cases were enrolled in the German Registry of Pituitary Tumors including 10,084 PitNETs (10,067 adenomas and 19 carcinomas obtained surgically and 193 adenomas diagnosed at autopsy) as well as 69 spindle cell tumors of the neurohypophysis (64 surgical specimens and 5 autopsies). In six patients (1 post mortem and 5 surgical specimens), PitNETs as well as posterior lobe tumors were found in the specimens. Two of the PitNETs were sparsely granulated prolactin-producing tumors, combined in one case with a granular cell tumor and in one case with a pituicytoma. One of the PitNETs revealed that autopsy was a sparsely granulated GH tumor combined with a neurohypophyseal granular cell tumor. Two PitNETs were null cell adenomas combined with a pituicytoma and a spindle cell oncocytoma, respectively. Further, one Crooke cell tumor was combined with a spindle cell oncocytoma. In five cases, the PitNETs were larger than the posterior lobe tumors and accounted for the clinical symptoms. Previously, four cases of co-existing pituitary anterior and posterior lobe tumors were described in the literature, comprising two ACTH PitNETs, one gonadotrophic PitNET and one null cell PitNET, each in combination with a pituicytoma. PitNETs and concomitant granular cell tumor or spindle cell oncocytoma, as observed in our cohort, have not been reported before.
Topics: Adenoma; Adult; Aged; Cohort Studies; Female; Germany; History, 20th Century; History, 21st Century; Humans; Male; Middle Aged; Neoplasms, Multiple Primary; Neuroendocrine Tumors; Pituitary Gland, Posterior; Pituitary Neoplasms; Registries; Retrospective Studies
PubMed: 34129177
DOI: 10.1007/s12022-021-09677-y -
Der Pathologe May 2015Pituitary adenomas have to be studied in detail for structural characteristics, especially regarding the degree of granulation and immunohistochemical hormone... (Review)
Review
Pituitary adenomas have to be studied in detail for structural characteristics, especially regarding the degree of granulation and immunohistochemical hormone expression, such as growth hormone (GH), prolactin, adrenocorticotropic hormone (ACTH), thyroid stimulating hormone (TSH), follicle stimulating hormone (FSH), luteinizing hormone (LH) and proliferation markers (e.g. Ki-67 and p53) for correlation to clinical data and assessment of the prognosis. If histological and immunostaining data do not correlate to the patient data, explanations for the discrepancies must be found. All active adenoma types can also be present as inactive, so-called silent adenomas showing the same features. An increased Ki-67 index (> 3%), significant nuclear expression of protein p53 and mitoses are characteristic of atypical adenomas. Up to now the biological relevance of these atypical adenomas, especially their role as preneoplasms for pituitary carcinomas has not been fully elucidated. The only proof of a pituitary carcinoma is the existence of metastases. Extensive local invasion and a greatly increased Ki-67 index are not sufficient for this diagnosis. Craniopharyngiomas have to be classified into adamantinomatous types (intrasellar and suprasellar) and papillary types (only suprasellar). Regressive changes are found in adamantinomatous types only. Strong regression may lead to difficulties in the differential diagnosis of Rathke's cleft cysts with squamous metaplasia. Demonstration of nuclear expression of beta-catenin in these cases enables the diagnosis of craniopharyngioma. Papillary craniopharyngiomas are characterized by BRAF mutations that may be helpful in the differential diagnosis. All pituicytomas of the neurohypophysis, all spindle cell oncocytomas of the anterior pituitary and all granular cell tumors of the posterior pituitary express thyroid transcription factor 1 (TTF-1) and are thought to be variants of a common uniform spindle cell tumor of the pituitary.
Topics: Adenoma; Biomarkers, Tumor; Craniopharyngioma; DNA-Binding Proteins; Ki-67 Antigen; Pituitary Gland; Pituitary Hormones; Pituitary Neoplasms; Prognosis; Transcription Factors; Tumor Suppressor Protein p53
PubMed: 25947224
DOI: 10.1007/s00292-015-0025-z