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Polish Archives of Internal Medicine Dec 2023
Topics: Humans; Neuroendocrine Tumors; Pituitary Neoplasms; Magnetic Resonance Imaging; Growth Hormone
PubMed: 37943187
DOI: 10.20452/pamw.16599 -
Surgical Neurology International 2023Pituicytomas are rare tumors of the pituitary gland derived from the ependymal cells and line the pituitary stalk and posterior lobe. These tumors are located in the...
BACKGROUND
Pituicytomas are rare tumors of the pituitary gland derived from the ependymal cells and line the pituitary stalk and posterior lobe. These tumors are located in the vulnerable regions of the brain: Either in the sellar or suprasellar area. The location marks the difference in the clinical features of the tumor. Here, we report a case of histopathologically diagnosed pituicytoma of the sellar region. Literature is also reviewed and discussed to gain a better understanding of this rare disease.
CASE DESCRIPTION
A 24-year-old female presented to the outpatient department with complaints of headache, diplopia, dizziness, and decreased vision in the right eye for 6 months. Computed tomography scan brain without contrast showed a well-defined hyperdense lesion in the sella without associated bony erosion. Her magnetic resonance imaging showed well defined rounded lesion in the pituitary fossa which was isointense on T1-weighted image and hyperintense on T2-weighted images. A presumptive diagnosis of pituitary adenoma was made. She underwent endoscopic endonasal transsphenoidal resection of pituitary mass. Intraoperatively, normal pituitary gland was visualized and there was a grayish-green-colored, jelly like tumor which was pulled gently. On 9 postoperative day, she presented with cerebrospinal fluid (CSF) rhinorrhea. She underwent endoscopic CSF leak repair. Her histopathology was concluded to be Pituicytoma.
CONCLUSION
Pituicytoma is an uncommon diagnosis. The surgical aim is to completely excise the tumor which results in complete cure, but incomplete resection may be performed due to high vascularity of this tumor. In case of incomplete excision, recurrence is common and adjuvant radiotherapy may be administered.
PubMed: 37404513
DOI: 10.25259/SNI_248_2023 -
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 -
Neuro-oncology Aug 2023"Primary papillary epithelial tumor of the sella (PPETS)" is a recently described rare tumor entity of the central nervous system (CNS) with stereotypic location in the...
BACKGROUND
"Primary papillary epithelial tumor of the sella (PPETS)" is a recently described rare tumor entity of the central nervous system (CNS) with stereotypic location in the sella. Comprehensive molecular investigations and epigenetic profiles of PPETS have not been performed to date.
METHODS
We report a comprehensive clinical, histopathologic, and molecular assessment of 5 PPETS cases in comparison with a cohort composed of 7 choroid plexus papilloma (CPP), 7 central neurocytoma (CN), 15 posterior pituitary tumor (PPT) including 4 pituicytoma, 6 granular cell tumors of the sellar region (GCT), and 5 spindle cell oncocytoma.
RESULTS
All PPETS had good outcomes. Immunohistochemically, PPETS tumors showed positive staining with TTF1, EMA, AE1/AE3, MAP2, and Vimentin, but were negatively stained with Syn, GFAP, CgA, and S100, and sporadically stained with Ki-67. In unsupervised hierarchical clustering and t-distributed stochastic neighbor embedding analyses of DNA-methylation data, PPETS and PPT tumors formed a distinct cluster irrespective of their histologic types. However, PPETS tumors did not cluster together with CPP and CN samples. Similar findings were obtained when our samples were projected into the reference cohort of the brain tumor classifier. Substantial fractions of the PPETS and PPT tumors shared broadly similar chromosomal copy number alterations. No mutations were detected using targeted next-generation sequencing.
CONCLUSIONS
Though more cases are needed to further elucidate the molecular pathogenesis of these tumors, our findings indicate that PPETS and PPT tumors may constitute a single neurooncological entity.
Topics: Humans; Pituitary Neoplasms; Neoplasms, Glandular and Epithelial; Adenoma, Oxyphilic; DNA Methylation; Central Nervous System
PubMed: 37058118
DOI: 10.1093/neuonc/noad067 -
No Shinkei Geka. Neurological Surgery Jul 2023Pituitary tumors or tumors of the sella turcica include pituitary neuroendocrine tumors, Rathke's cleft cysts, craniopharyngiomas, tuberculum sellae, planum sphenoidale...
Pituitary tumors or tumors of the sella turcica include pituitary neuroendocrine tumors, Rathke's cleft cysts, craniopharyngiomas, tuberculum sellae, planum sphenoidale meningiomas, germ cell tumors, and hypophysitis. In addition, some rare tumors, such as pituicytomas, granular cell tumors, spindle cell oncocytomas, and chordomas or chondrosarcomas, arise from the parasellar regions. The treatment strategy is completely different for each lesion; therefore, accurate diagnosis is essential.
Topics: Humans; Pituitary Neoplasms; Pituitary Gland; Sella Turcica; Meningioma; Meningeal Neoplasms
PubMed: 37491055
DOI: 10.11477/mf.1436204791 -
Acta Neurologica Belgica Jun 2023Pituicytomas (PT) are rare benign sellar and/or supra sellar tumors which surgical treatment might be challenging, owing to the hypervascularity of the tumor. Here, we...
OBJECTIVE
Pituicytomas (PT) are rare benign sellar and/or supra sellar tumors which surgical treatment might be challenging, owing to the hypervascularity of the tumor. Here, we report our experience with PTs, describe their clinical and radiological features, and propose an optimal therapeutic strategy.
METHODS
This is a retrospective single-center study, reporting the clinical manifestations, radiological characteristics, histopathological features, treatment strategies and long-term outcomes of four patients who have been treated for a PT at Bicêtre University Hospital in Paris, France, over the past 7 years.
RESULTS
Four patients were operated mean age at presentation was 60.25 years. Main symptoms, which tended to be progressive, included visual field defects and pituitary-hypothalamic dysfunction. Radiologically, all our cases resented with sellar and suprasellar localization with cavernous invasion in 75% of cases. Endoscopic transphenoidal approach was performed as first surgery for all cases. Transcranial route was done in one case. During surgery, important bleeding was observed in all cases. Gross total resection was obtained with a single surgery in two cases and a second surgery was necessary in the two remaining cases. Pathological diagnosis was confirmed in all cases. No recurrences were noted after an average follow-up of 3.6 years.
CONCLUSIONS
PT is a rare differential diagnosis of sellar and/or parasellar tumors, whose hemorrhagic nature can become a challenge during surgery. We added four more cases to the literature to make physicians establish Piticytomas at suspicion diagnosis for sellar and/or suprasellar masses. Knowing the diagnosis, a preparation should be made preoperatively to avoid possible complications peroperatively.
Topics: Humans; Middle Aged; Retrospective Studies; Pituitary Neoplasms; Craniopharyngioma; Glioma; Diagnosis, Differential
PubMed: 35301693
DOI: 10.1007/s13760-022-01917-0 -
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 -
Pediatrics Dec 2015Pituicytoma is a tumor extremely rare in childhood, with only 4 cases reported in literature. It is thought to arise from the specialized glial elements called...
Pituicytoma is a tumor extremely rare in childhood, with only 4 cases reported in literature. It is thought to arise from the specialized glial elements called "pituicytes." The association of pituicytoma and Cushing's disease (CD) has been described only once so far, in an adult patient. A 7-year-old girl was referred for clinical signs of hypercortisolism, and a diagnosis of CD was made. MRI revealed 2 pathologic areas in the pituitary gland. The patient underwent surgery, with microscopic transsphenoidal approach, and a well-circumscribed area of pathologic tissue was identified and removed. Surprisingly, histologic and immunohistochemical study provided unequivocal evidence of pituicytoma. No pituitary adenoma could be identified. For persistent hypercortisolism, the patient necessitated transsphenoidal endoscopic reintervention and 2 other lesions were removed. By immunohistological examination, these lesions were confirmed to be corticotropin-secreting adenoma. Unfortunately, there was no postoperative decrease in corticotropin and cortisol levels, and the patient underwent bilateral laparoscopic adrenalectomy. Considering that we report a second case of association of pituicytoma and corticotropin-secreting adenoma, that CD is infrequent, and pituicytoma is extremely rare in childhood, the coexistence of these 2 tumors should not be considered a mere coincidence. To date, there is no conclusive evidence about the origin of these different subtypes of pituitary tumors. This case supports the hypothesis that these tumors share a common progenitor cell, which could be the folliculostellate cell.
Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Child; Female; Humans; Pituitary ACTH Hypersecretion; Pituitary Neoplasms
PubMed: 26553184
DOI: 10.1542/peds.2015-0638 -
Radiographics : a Review Publication of... 2021Primary tumors of the pituitary gland are the second most common histologic category of primary central nervous system tumors across all age groups and are the most...
Primary tumors of the pituitary gland are the second most common histologic category of primary central nervous system tumors across all age groups and are the most common in adolescents to young adults, despite originating from a diminutive endocrine gland that is often described as "about the size of a pea." The vast majority of these represent primary tumors of the adenohypophysis, specifically pituitary adenomas, which can be either functional or silent with regard to hormone hypersecretion. According to the fourth edition of the World Health Organization classification of endocrine tumors, published in 2017, cellular lineage and immunohistochemical stains for pituitary hormones and/or transcription factors help with making the correct pathologic diagnosis. From a radiologic standpoint, microadenomas pose challenges for accurate detection and avoiding false-negative or false-positive results, while macroadenomas pose challenges from local mass effect on surrounding structures. Pituitary carcinoma and pituitary blastoma also arise from the adenohypophysis and are characterized by metastatic disease and infantile presentation, respectively. While primary tumors of the adenohypophysis are common, a second category comprising primary tumors of the Rathke pouch (ie, craniopharyngioma) are uncommon, and a third category comprising primary tumors of the neurohypophysis (eg, pituicytoma) are rare. The authors review all three categories of pituitary tumors, with emphasis on radiologic-pathologic correlation, including the typical neuroimaging, histologic, and molecular features that may point toward a specific diagnosis. Work of the U.S. Government published under an exclusive license with the RSNA.
Topics: Adenoma; Adolescent; Glioma; Humans; Pituitary Gland, Posterior; Pituitary Neoplasms; World Health Organization; Young Adult
PubMed: 34597177
DOI: 10.1148/rg.2021200203 -
Medicine Nov 2019Pituicytomas are exceptional rare tumors in the sellar and suprasellar regions with clinical manifestations, such as headache, visual disturbance, hypopituitarism, and... (Review)
Review
RATIONALE
Pituicytomas are exceptional rare tumors in the sellar and suprasellar regions with clinical manifestations, such as headache, visual disturbance, hypopituitarism, and decreased libido. Unlike that of common pituitary adenoma, the association between pituicytoma and Cushing disease (CD) is extremely rare. There were only 6 reported cases till now. In the current study, we describe an unusual case of pituicytoma associated with severe CD with a recurrence-free follow-up period of 49 months.
PATIENT CONCERNS
A 32-year-old woman was referred to our hospital with moon face, central obesity, and purple stripes on the lower limbs.
DIAGNOSES
The plasma cortisol level was 1122 nmol/L. The low-dose dexamethasone suppression test failed to suppress plasma cortisol. This test provided evidence of nonpituitary-dependent CD. However, magnetic resonance imaging demonstrated a sellar mass measuring approximately 7.6 × 5.7 mm. The patient was diagnosed with pituitary microadenoma. Histopathological analysis of the tissue sections based on the findings from the immunohistochemical staining diagnosed it as pituicytoma.
INTERVENTIONS
Transsphenoidal surgery was performed to remove the pituitary mass.
OUTCOMES
Within 2 months postoperatively, the patient's blood pressure and cortisol level decreased gradually and normalized on the 6th month when other symptoms of CD also disappeared. The patient is presently free from recurrence 49 months after the initial diagnosis.
LESSONS
Based on the postoperative remission, CD was caused by pituitary disorders. A reasonable assumption is that an extremely small coexisting adenoma was not detected by the surgeon and washed out during the dissection. Another possible explanation might be the mass effect caused by this intrasellar lesion.
Topics: Adenoma; Adult; Female; Humans; Pituitary ACTH Hypersecretion; Pituitary Neoplasms
PubMed: 31689841
DOI: 10.1097/MD.0000000000017772