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Neuro Endocrinology Letters Nov 2021Pituicytomas (PTs) are rare and benign neoplasms. The variable imaging and clinical features of PTs, which overlap with other sellar pathologies, can make preoperative... (Review)
Review
OBJECTIVE
Pituicytomas (PTs) are rare and benign neoplasms. The variable imaging and clinical features of PTs, which overlap with other sellar pathologies, can make preoperative diagnosis challenging. In the interest of a more comprehensive understanding of the diagnostic aspects of PTs, it is necessary to report and synthesize the variable imaging and clinical features of PTs.
METHODS
We retrospectively included and analysed four pathologically proven PTs with unusual imaging and/or clinical features. Additionally, we reviewed the literature on PT between 2007 and 2019 in the PubMed database to provide context for the individual patient data described herein.
RESULTS
Our series included three female and one male adult patient (mean age: 44.75, age range: 20-56 y). Based on clinical symptoms, we noticed that case 1 had Cushing's syndrome, case 2 had increased prolactin, case 3 had extremity enlargement but with a normal level of human growth factor, and case 4 presented with tinnitus and dizziness. On radiograph, inconsistent with the main imaging findings of PTs in the literature, there was one case in the pituitary anterior lobe, three cases with hypointensity on T2-weighted images, two patients with reduced homogeneous contrast enhancement, and one case demonstrating invasion potential. In addition, one of our patients underwent PET-CT examination, and the lesion had a slight increase in glucose uptake and no significant decrease in ammonia uptake. Postoperative follow-up monitoring revealed no tumour recurrence.
CONCLUSION
Our cases highlight the unusual imaging manifestations of PTs. Recognizing these imaging features plays an important role in the preoperative diagnosis, treatment, and postsurgery monitoring of PTs.
Topics: Adult; Craniopharyngioma; Female; Glioma; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Recurrence, Local; Pituitary Neoplasms; Positron Emission Tomography Computed Tomography; Retrospective Studies; Young Adult
PubMed: 34847317
DOI: No ID Found -
Applied Immunohistochemistry &...
PubMed: 34757345
DOI: 10.1097/PAI.0000000000000990 -
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 -
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 -
Acta Endocrinologica (Bucharest,... 2021
PubMed: 34539922
DOI: 10.4183/aeb.2021.137 -
Hormones (Athens, Greece) Dec 2021Pituicytoma is a rare tumor of the pituitary gland derived from neurohypophyseal pituicytes. CASE 1: A 58-year-old female presented with decreased vision; she was... (Review)
Review
INTRODUCTION
Pituicytoma is a rare tumor of the pituitary gland derived from neurohypophyseal pituicytes. CASE 1: A 58-year-old female presented with decreased vision; she was admitted to the neurosurgery department of Ege University after the detection of a pituitary macroadenoma. Magnetic resonance imaging (MRI) showed a 28 * 18 * 17-mm suprasellar mass, and laboratory tests revealed hypopituitarism. Hydrocortisone and L-thyroxine treatment were initiated, and the patient underwent resection through the endoscopic endonasal approach (EEA). The histopathological examination revealed a pituicytoma. The recurrence of tumor was detected during the 1-year follow-up, and the patient is awaiting surgery. CASE 2: A 70-year-old woman presented with visual changes; she had a past medical history of hypophyseal macroadenoma and pituicytoma resected through an EEA in 2012 and 2017, respectively. During follow-up, 2 years after the second surgery, MRI showed progression of the pituicytoma then measuring 38 × 23 × 22 mm; it had invaded the cavernous sinus and was causing hydrocephaly and panhypopituitarism. The patient underwent the third resection through the transcranial approach in order to minimize bleeding. After this surgery, the patient developed diabetes insipidus and underwent treatment with desmopressin. Histopathological examination revealed a pituicytoma. At 6-month follow-up, imaging showed a sellar suprasellar mass 37 × 22 × 24 mm invading the cavernous sinus, indicative of recurrence. In the postoperative period, the patient applied to the department of radiation oncology to have fractionated radiotherapy.
DISCUSSION
Pituicytomas are known to be low-grade tumors; because of their rarity, they are a real challenge. These patients should be followed up closely.
Topics: Aged; Female; Glioma; Humans; Magnetic Resonance Imaging; Middle Aged; Pituitary Gland; Pituitary Neoplasms
PubMed: 34390481
DOI: 10.1007/s42000-021-00301-6 -
Military Medical Research Jul 2021Pituicytoma is an extremely rare low-grade glial tumor that is closely related to the neurohypophysis axis. Most studies of pituicytomas include only several cases. To...
Clinical features, radiological profiles, pathological features and surgical outcomes of pituicytomas: a report of 11 cases and a pooled analysis of individual patient data.
BACKGROUND
Pituicytoma is an extremely rare low-grade glial tumor that is closely related to the neurohypophysis axis. Most studies of pituicytomas include only several cases. To better understand this disease, we reviewed a series of cases of pituicytomas. The diagnosis and treatment of pituicytoma must be further elucidated.
METHODS
Eleven patients with pituicytoma admitted to Beijing Tiantan Hospital from 2012 to 2019 were selected. The clinical features, including radiological and histological examination, surgical records and prognosis were reviewed. Sixty-eight other previously published cases of pituicytoma also were used to analyze the predictive factors for the results. The Cox regression model was used for univariate and multivariate analyses.
RESULTS
Our patients included 5 males (45.5%) and 6 females (54.5%), with a mean age of 49.3 years. The tumor was located in the suprasellar region in 5 patients (45.5%), intrasellar region in 4 patients (36.4%), and intrasellar-suprasellar region in 2 patients (18.2%). All patients were misdiagnosed with other common tumors in the sellar region before the operation. During the operation, gross total resection (GTR) of the tumor was achieved in 6 patients (54.5%), and subtotal resection (STR) was achieved in 5 patients (45.5%). The mean progression-free survival (PFS) time was 29.82 months. Tumor progression after surgical resection occurred in 4 patients (36.4%). Among them, 60.0% of the patients (cases 4, 5, 7) with STR experienced progression, while 16.7% of the patients (case 2) with GTR experienced progression. Combined with the 68 cases in the literature, GTR was an independent risk factor for PFS time (P < 0.05).
CONCLUSIONS
Pituicytomas are more common in middle-aged people and the sellar region. The clinical manifestations of pituicytomas are different, but no diagnostic clinical features have been identified other than an abnormally abundant blood supply. Currently, GTR is the best approach for the treatment of pituicytomas. More patients and longer follow-up periods were needed to further elucidate the biological features of pituicytomas.
Topics: Adult; Beijing; Female; Glioma; Humans; Male; Middle Aged; Outcome Assessment, Health Care; Pituitary Gland; Proportional Hazards Models; Radiography; Retrospective Studies; Treatment Outcome
PubMed: 34210357
DOI: 10.1186/s40779-021-00332-5 -
Neurosurgery Mar 2022Tumors that arise in and around the skull base comprise a wide range of common and rare entities. Recent studies have advanced our understanding of their pathogenesis,... (Review)
Review
Tumors that arise in and around the skull base comprise a wide range of common and rare entities. Recent studies have advanced our understanding of their pathogenesis, which in some cases, have significantly influenced clinical practice. The genotype of meningiomas is strongly associated with their phenotype, including histologic subtype and tumor location, and clinical outcome. A single molecular alteration, NAB2-STAT6 fusion, has redefined the category of solitary fibrous tumors to include the previous entity hemangiopericytomas. Schwannomas, both sporadic and familial, are characterized by near ubiquitous alterations in NF2 , with additional mutations in SMARCB1 or LZTR1 in schwannomatosis. In pituitary adenohypophyseal tumors, cell lineage transcription factors such as SF-1, T-PIT, and PIT-1 are now essential for classification, providing a more rigorous taxonomy for tumors that were previously considered null cell adenomas. The pituicyte lineage transcription factor TTF-1 defines neurohypophyseal tumors, which may represent a single nosological entity with a spectrum of morphologic manifestations (ie, granular cell tumor, pituicytoma, and spindle cell oncocytoma). Likewise, the notochord cell lineage transcription factor brachyury defines chordoma, discriminating them from chondrosarcomas. The identification of nonoverlapping genetic drivers of adamantinomatous craniopharyngiomas and papillary craniopharyngiomas indicates that these are distinct tumor entities and has led to successful targeted treatment of papillary craniopharyngiomas using BRAF and/or mitogen-activated protein kinase inhibitors. Similarly, dramatic therapeutic responses have been achieved in patients with Langerhans cell histiocytosis, both with BRAF -mutant and BRAF -wildtype tumors. Familiarity with the pathology of skull base tumors, their natural history, and molecular features is essential for optimizing patient care.
Topics: Craniopharyngioma; Humans; Meningeal Neoplasms; Nervous System Diseases; Pituitary Neoplasms; Proto-Oncogene Proteins B-raf; Skull Base Neoplasms; Transcription Factors
PubMed: 34164689
DOI: 10.1093/neuros/nyab209 -
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