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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 -
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 -
Endocrine Pathology Sep 2019The 2017 World Health Organization classification of central nervous system and endocrine tumors have introduced significant changes in the diagnostic criteria for... (Review)
Review
The 2017 World Health Organization classification of central nervous system and endocrine tumors have introduced significant changes in the diagnostic criteria for pituitary lesions. The aim of our paper is to describe the epidemiological, clinico-pathological, and radiological features of a single consecutive institutional surgical series of rare pituitary lesions, using these new criteria. Of the 316 endoscopic endonasal trans-sphenoidal approaches performed for pituitary lesions between 2010 and 2018, 15 rare lesions were encountered. These included metastases, pituitary carcinomas, pituicytomas, granular cell tumor, primary pituitary lymphomas, germinoma, mixed gangliocytoma-adenoma, hypophysitis, and pituitary hyperplasia. Their clinical, radiological, and pathological features are herewith presented along with a literature review that enabled us to propose an algorithm to facilitate a diagnosis for rare pituitary lesions.
Topics: Diagnostic Techniques, Endocrine; Humans; Pituitary Diseases; Rare Diseases; Retrospective Studies; Tertiary Care Centers; Thyroid Function Tests
PubMed: 31209729
DOI: 10.1007/s12022-019-09581-6 -
Journal of Neurosurgery. Pediatrics Mar 2022The purpose of this study was to investigate the efficacy and safety of endoscopic transcortical intraventricular biopsy in pediatric patients with isolated thickened...
OBJECTIVE
The purpose of this study was to investigate the efficacy and safety of endoscopic transcortical intraventricular biopsy in pediatric patients with isolated thickened pituitary stalk lesions.
METHODS
From 1994 to 2018, 16 pediatric patients (8 males and 8 females) underwent endoscopic transcortical intraventricular biopsy at a single institution to establish an accurate pathological diagnosis for appropriate treatment strategies.
RESULTS
Preoperatively, the median diameter of the pituitary stalks was 5.31 ± 1.67 mm (range 3.86-9.17 mm). Overall, the diagnostic yield at endoscopic intraventricular biopsy was 93.8%. Histopathological diagnosis included germinoma (n = 10, 62.5%), Langerhans cell histiocytosis (n = 4, 25%), pilocytic astrocytoma (n = 1, 6.3%), and pituicytoma (n = 1, 6.3%). Two patients developed new diabetes insipidus after the procedure, and 3 patients had new postoperative hypothyroidism. There were no postoperative neurological deficits in this series.
CONCLUSIONS
Neuroendoscopic biopsy via the transcortical intraventricular corridor was shown to be safe and to aid in a reliable histopathological diagnosis in the management of isolated pituitary stalk lesions in pediatric patients. It can be considered a minimally invasive alternative to open biopsy via transcranial or endonasal corridors.
PubMed: 34861646
DOI: 10.3171/2021.9.PEDS21254 -
Journal of Surgical Case Reports Jun 2020Pituicytoma is a rare tumor that has been recently recognized and described, where only few reported cases of pituicytoma associated with Cushing's disease. We describe...
Pituicytoma is a rare tumor that has been recently recognized and described, where only few reported cases of pituicytoma associated with Cushing's disease. We describe a case of a 47 years old female with a history of high cortisol levels and a diagnosis of Cushing's disease was made. Brain magnetic resonance imaging showed lesion in pituitary gland compatible with microadenoma and tumor resection was carried out. The histopathological findings were of a pituicytoma with positive thyroid transcription factor-1 immunostain.
PubMed: 32577204
DOI: 10.1093/jscr/rjaa104 -
Endocrine Regulations Oct 2019Pituicytomas are rare, solid, well-circumscribed, low grade (grade I), non-neuroendocrine, and noninfiltrative tumors of the neurohypophysis or infundibulum, which...
OBJECTIVES
Pituicytomas are rare, solid, well-circumscribed, low grade (grade I), non-neuroendocrine, and noninfiltrative tumors of the neurohypophysis or infundibulum, which appear in the sellar/suprasellar regions. Herein, we present a case with Cushing's disease (CD) caused by an ACTH-secreting pituitary adenoma in association with an infundibular pituicytoma. Subject and Results. A 37-year-old male patient presented to the hospital with a six-month history of blurry vision. Physical examination demonstrated plethora, excessive sweating, weight gain, moon facies, and acne. Basal serum cortisol and ACTH levels were 16 µg/dl and 32 pg/ml, respectively. The results of screening tests were suggestive of Cushing syndrome. It was also 1.97 µg/dl following 8 mg dexamethasone suppression test which was consistent with CD. Pituitary MR imaging revealed a single lesion measuring 6x6.5 mm on the pituitary stalk. Infundibular mass excision and pituitary exploration by extended endoscopic endonasal approach were applied. On immunohistochemistry, strong diffuse immunolabeling for both S100 and TTF-1 was noted for the cells of infundibular mass, diagnosed as pituicytoma. Because the developed panhypopituitarism postoperatively, patient was discharged with daily desmopressin, levothyroxine, hydrocortisone, and intramuscular testosterone, once a month.
CONCLUSIONS
Pituicytoma is an uncommon noninvasive tumor of the sellar and suprasellar regions. In this case report, we described a patient with Cushing's disease to whom MRI displayed only an infundibular well-circumscribed lesion, but not any pituitary adenoma. Despite the absence of any sellar lesion, awareness of other undetected possible lesion and exploring hypophysis during the transsphenoidal surgery is mandatory for the correct diagnosis.
Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Adrenocorticotropic Hormone; Adult; Humans; Male; Neoplasms, Multiple Primary; Pituitary ACTH Hypersecretion; Pituitary Gland; Pituitary Neoplasms
PubMed: 31734654
DOI: 10.2478/enr-2019-0026