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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 -
Otolaryngologic Clinics of North America Apr 2022This article reviews the histopathology and classification of neoplasms that arise from the adenohypophysis (anterior pituitary), the neurohypophysis (posterior... (Review)
Review
This article reviews the histopathology and classification of neoplasms that arise from the adenohypophysis (anterior pituitary), the neurohypophysis (posterior pituitary) as well as other common miscellaneous lesions that arise within or secondarily involve the pituitary gland.
Topics: Adenoma; Craniopharyngioma; Humans; Pituitary Gland; Pituitary Neoplasms
PubMed: 35256168
DOI: 10.1016/j.otc.2021.12.003 -
Annals of Diagnostic Pathology Aug 2017Pituicytoma is a rare low-grade glial neoplasm that originates in the distribution of the neurohypophysis, including the posterior pituitary lobe and infundibular stalk.... (Review)
Review
Pituicytoma is a rare low-grade glial neoplasm that originates in the distribution of the neurohypophysis, including the posterior pituitary lobe and infundibular stalk. The tumor cells resemble pituicytes, which are specialized glial cells of the neurohypophysis. Pituicytoma can be misdiagnosed pre-operatively as a pituitary adenoma due to overlapping clinical and neuroimaging features between these two entities. Pituicytoma can also mimic other neoplasms of the sellar and parasellar regions microscopically - meningioma, schwannoma and pilocytic astrocytoma - and shares immunohistochemical expression of TTF-1 with spindle cell oncocytoma and granular cell tumor of the sellar region, suggesting a common histogenesis. In this short review, we present the key features of pituicytoma, discuss commonalities and distinguishing features from other neoplasms of the sellar and parasellar regions and highlight the importance of recognizing this tumor entity for clinical and surgical management.
Topics: Adenoma; Biomarkers, Tumor; Craniopharyngioma; Diagnosis, Differential; Humans; Meningeal Neoplasms; Pituitary Neoplasms
PubMed: 28807344
DOI: 10.1016/j.anndiagpath.2017.05.004 -
La Radiologia Medica Mar 2020Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis... (Review)
Review
Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients' clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considered the neuroradiological investigation of choice. The features suggestive for HP include an enlarged triangular- or dumbbell-shaped gland with a thickened and not obviously deviated stalk, further supported by the absence of posterior pituitary bright spot on T1weighted images, particularly in patients presenting with diabetes insipidus. Contrast enhancement pattern is quite variable; dural enhancement has been reported in some cases after intravenous contrast administration. The characterization of the unusual sellar mass is not straightforward and generally results in a wide differential. HP should be primarily differentiated from pituitary adenomas (including pituitary apoplexy), from pituitary metastases, and from other sellar and parasellar tumors, e.g., craniopharyngiomas, germinomas, gliomas, lymphomas, meningiomas, pituicytomas, chordomas, teratomas, dermoids and epidermoids, Rathke's cleft cysts, and abscesses. In patients suspected for secondary forms related to systemic pathology, additional imaging is helpful in identifying other involved sites. Neuroradiologists need to know MRI appearance of this rare disease, as well as its typical symptoms and serological markers. A strict collaboration with endocrinologists and neurosurgeons is mandatory in order to reach a definitive diagnosis, allowing to promptly initiating an appropriate treatment.
Topics: Adenoma; Autoimmune Hypophysitis; Contrast Media; Diagnosis, Differential; Gadolinium; Humans; Hypophysitis; Immunoglobulin G4-Related Disease; Magnetic Resonance Imaging; Neuroradiography; Pituitary Gland; Pituitary Neoplasms; Xanthomatosis
PubMed: 31863360
DOI: 10.1007/s11547-019-01120-x -
Reviews in Endocrine & Metabolic... Jun 2019In 2017, the World Health Organization established that pituicytoma, granular cell tumor (GCT), spindle cell oncocytoma (SCO) and sellar ependymomas (SE) are posterior... (Review)
Review
In 2017, the World Health Organization established that pituicytoma, granular cell tumor (GCT), spindle cell oncocytoma (SCO) and sellar ependymomas (SE) are posterior pituitary tumors (PPT). They probably arise from the pituicytes and may constitute a unique histopathological entity. We carried out a systematic review using PubMed's database. A total of 266 patients with pathological diagnosis of PPT (135 pituicytomas, 69 GCT, 47 SCO, 8 SE and 7 mixed histology tumors) were analyzed. Gender distribution was identical and median age at diagnosis was 48 ± 21.8 years. Main presentation symptoms were visual disorders (n = 142; 58.1%), headache (n = 99; 40.5%), hypopituitarism (n = 84; 34.4%), hypercortisolism (n = 10; 4.1%), polyuriapolydipsia (n = 6; 2.4%) and acromegaly features (n = 5; 2.0%). On MRI, 122 (47.6%) patients showed sellar with suprasellar extension masses, 67 (23.1%) were suprasellar and 63 (24.6%) exclusively sellar. Median tumor size was 22.0 ± 14.2 mm. Two hundred sixty four patients underwent surgery, transphenoidal access was selected in 132 (64.4%) and craniotomy in 58 (28.3%). Complications were hypopituitarism (n = 70; 42.1%), diabetes insipidus (n = 55; 33.1%) and hemorrhage (n = 50; 30.1%). Tumor persisted in 93 patients (45.6%) and recurred in 13 (6.4%). Regarding comparison between main types of PPT, SCO patients were diagnosed later (60.0 vs 47.0 vs 47.0 years, p = 0.023), the tumor was larger 25.0 mm [10.8] vs 20.0 mm [14.2] vs 2.0 mm [15.0] and they were frequently sellar with suprasellar extension tumors (71.7% vs 46.2% vs 32.8%, p = 0.003) compared to pituicytoma and GCT. In conclusion, PPT are rare tumors and have been misdiagnosed mainly as non-functioning pituitary adenomas. Different types of PPT share similar epidemiology, clinical manifestations and surgical outcomes. Surgery is the only curative option but complications and subtotal resection are common.
Topics: Animals; Granular Cell Tumor; Humans; Pituitary Neoplasms
PubMed: 30864049
DOI: 10.1007/s11154-019-09484-1 -
World Neurosurgery Apr 2020Pituicytoma is a rare neoplasm arising in the sellar region (World Health Organization grade I). Clinically, pituicytomas mimic nonfunctioning pituitary macroadenomas...
BACKGROUND
Pituicytoma is a rare neoplasm arising in the sellar region (World Health Organization grade I). Clinically, pituicytomas mimic nonfunctioning pituitary macroadenomas and are occasionally incidentally discovered at autopsy. Pituitary adenomas can occur with other sellar pathologies, and the term "collision sellar lesions" has been coined for this rare entity. There have only been a few reports of the coexistence of pituicytoma and pituitary adenoma. We present 2 cases of pituicytoma coexisting with acromegaly and Cushing disease.
CASE DESCRIPTION
Case 1: A 29-year-old woman had acromegaly. The macroadenoma was partially removed in her first surgery; thus an endonasal reoperation was required for debulking and posterior radiosurgery. Pituicytoma coexisting with somatotropinoma was diagnosed on pathologic examination. Case 2: A 33-year-old woman had adrenocorticotropic hormone-dependent Cushing disease. She underwent endonasal resection. Undetectable postoperative cortisol levels provided evidence that the underlying adrenocorticotropic hormone source was successfully removed. On the basis of morphologic features and the immunohistochemical profile, pituicytoma was diagnosed on pathologic examination. Pituitary adenoma was not confirmed histologically in this patient.
CONCLUSIONS
Only 117 cases of pituicytoma have been reported since it was first described in 1955. Before our report, only 5 cases of patients with pituicytoma coexisting with pituitary adenoma had been described. The coexistence of these 2 entities may not just be a mere coincidence but may be due to a yet unknown pathophysiologic link or common progenitor lineage of both lesions. Association between pituicytoma and pituitary adenoma is increasingly being reported.
Topics: Adult; Female; Growth Hormone-Secreting Pituitary Adenoma; Humans; Magnetic Resonance Imaging; Neoplasms, Multiple Primary; Pituitary ACTH Hypersecretion; Pituitary Gland, Posterior; Pituitary Neoplasms
PubMed: 31874293
DOI: 10.1016/j.wneu.2019.12.085 -
British Journal of Neurosurgery Oct 2018Pituicytoma is a rare low-grade glioma arising from the pituicytes of the posterior pituitary. To date, the clinical and pathological correlates of pituicytoma have not...
PURPOSE
Pituicytoma is a rare low-grade glioma arising from the pituicytes of the posterior pituitary. To date, the clinical and pathological correlates of pituicytoma have not been investigated. This study was thus designed to examine the correlation between pituicytoma and the normal pituitary gland.
METHODS
The records of patients who underwent pituitary surgery at Chang Gung Memorial Hospital in Linkou, Taiwan between 2000 and 2016 were reviewed. Patients who received a pathological diagnosis of pituicytoma were included; however, those with inadequate specimens for pathological study were excluded. Clinical information, including patients' presenting symptoms, serum hormone levels, neuroimages, and specimens, were collected. Hematoxylin and eosin stains and immunohistochemical (IHC) stains were performed for differential diagnosis.
RESULTS
Among the 1532 patients who underwent pituitary surgery, nine (0.59%) received a pathological diagnosis of pituicytoma. Two patients were excluded due to inadequate specimens. Among the seven remaining patients, six presented with hormone changes. The IHC stains revealed that pituicytoma has no secretory function; however, the resected pituitary glands showed positive results for hormone change. Coexisting pituicytoma and adrenocorticotropic hormone adenoma were identified in one patient with a diagnosis of Cushing disease.
CONCLUSIONS
Pituicytoma revealed a negative endocrine secretory function through IHC staining. Additionally, pituicytoma is associated with hypersecretion of the pituitary gland both clinically and pathologically. Diagnosing pituicytoma before pathological confirmation is difficult because the tumour may present with hormone dysfunction. Therefore, IHC staining of specimens is useful to exclude the possibility of coexisting pituicytoma and pituitary adenoma.
Topics: Adenoma; Adult; Craniopharyngioma; Diagnosis, Differential; Female; Glioma; Humans; Male; Middle Aged; Pituitary ACTH Hypersecretion; Pituitary Gland, Posterior; Pituitary Hormones; Pituitary Neoplasms
PubMed: 29749277
DOI: 10.1080/02688697.2018.1472212 -
Journal of Endocrinological... Apr 2019Pituicytomas (PTs) are extremely rare, low-grade glial tumors closely related to the neurohypophyseal axis. Definite conclusions concerning the optimal diagnostic and... (Review)
Review
BACKGROUND
Pituicytomas (PTs) are extremely rare, low-grade glial tumors closely related to the neurohypophyseal axis. Definite conclusions concerning the optimal diagnostic and therapeutic approach to these neoplasms are lacking to date, as most of this information has been presented as case reports.
METHODS
Retrospective review of case reports published in the scientific literature to date, including a new illustrative example treated in our department.
RESULTS
116 cases were collected. PTs had a higher prevalence in the fifth and sixth decades of life, with a slight male predominance. Main symptoms, which tended to be progressive, included visual field defects and pituitary-hypothalamic dysfunction. Radiologically, PTs were found anywhere along the hypothalamic-pituitary axis mimicking other, more frequent tumors growing in this anatomical region. Surgical treatment included both transcranial or transsphenoidal approaches, and resulted in gross total resection and morbidity rates of 46.8 and 59%, respectively; the latter essentially consisted in anterior and posterior pituitary dysfunction, with limited impact on daily quality of life.
CONCLUSIONS
Due to both low frequency and the absence of pathognomonic clinical and/or radiological features, formulating a suspicion diagnosis of PT represents a considerable challenge even for experienced professionals. The indication for treatment should be made on an individual basis, but it is inescapable in the presence of a visual field defect. The surgical approach has to be tailored according to the topography of the tumor and preoperative symptoms; the greatest challenges in accomplishing a gross total removal are represented by the degree of adherence and vascularization of the PT.
Topics: Glioma; Humans; Pituitary Neoplasms
PubMed: 30030746
DOI: 10.1007/s40618-018-0923-z -
Neurosurgery Apr 2021The pituitary gland is the site of numerous neoplastic and inflammatory processes. The overwhelmingly most frequent tumors arise from cells of the anterior lobe, the... (Review)
Review
The pituitary gland is the site of numerous neoplastic and inflammatory processes. The overwhelmingly most frequent tumors arise from cells of the anterior lobe, the pituitary neuroendocrine tumors (PitNETs). Immunohistochemistry assay staining for pituitary hormones is the core tool for classifying PitNETs, resulting in the diagnosis of somatotroph PitNETs, lactotroph PitNETs, and so on. For cases showing no hormonal expression, the updated WHO classification system now considers the assessment of several transcription factors: PIT-1 (pituitary-specific POU-class homeodomain transcription factor); T-PIT (T-box family member TBX19); and SF-1 (steroidogenic factor regulating gonadotroph cell differentiation) before rendering a diagnosis of null cell adenoma. Other tumors and disease processes of this site often mimic PitNETs radiographically and sometimes even clinically (ie, compression of the optic chiasm). These potpourri of processes include germ cell neoplasms (especially germinomas), tumors that originate from Rathke's pouch (craniopharyngiomas, Rathke's cleft cyst), tumors that originate from the posterior lobe of the pituitary (pituicytoma, spindle cell oncocytoma, granular cell tumor), and tumors that originate from the meninges (especially meningiomas). In addition to neoplasms, several described inflammatory and related conditions exist that need to be distinguished from PitNETs. These include lymphocytic hypophysitis and Langerhans cell histiocytosis, a neoplastic disorder of histiocytes. In this review, we aim to briefly describe the main pituitary and sellar lesions, with emphasis on the most common tumors, the PitNETs.
Topics: Adenoma; Adolescent; Adult; Child; Female; Humans; Male; Middle Aged; Pituitary Gland; Pituitary Neoplasms; Young Adult
PubMed: 33476394
DOI: 10.1093/neuros/nyaa548 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Apr 2022To investigate the clinicopathological features and treatment strategies of pituicytoma. Twenty-one cases of pituicytoma were collected at the First Affiliated...
To investigate the clinicopathological features and treatment strategies of pituicytoma. Twenty-one cases of pituicytoma were collected at the First Affiliated Hospital of Nanjing Medical University and Jinling Hospital, Nanjing, China from 2009 to 2020. The clinical data of 21 pituicytoma patients was retrospectively analyzed, and the relevant literature was reviewed. Twenty-one patients aged 4 to 68 years, including 8 males and 13 females. All patients underwent surgical treatment. Histologically, the tumor was consisted almost entirely of elongate, bipolar spindle cells arranged in a fascicular or storiform pattern. Mitotic figures were rare. Immunohistochemically, tumor cells were diffusely positive for S-100 protein (21/21), vimentin (15/15) and TTF1 (14/14), while they were weakly or focally positive for GFAP (13/16) and EMA (6/12). CKpan was negative in all cases and Ki-67 proliferation index was low (<5%). Among the 18 patients with follow-up, all survived and 2 relapsed after surgery. Pituicytoma is a rare low-grade glioma of the sellar area. It is easily confused with other sellar tumors. Preoperative diagnosis is difficult. It needs to be confirmed by histopathology and immunohistochemistry. Microsurgery is the main treatment method at present.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Craniopharyngioma; Female; Glioma; Humans; Immunohistochemistry; Male; Middle Aged; Pituitary Neoplasms; Retrospective Studies; Young Adult
PubMed: 35359042
DOI: 10.3760/cma.j.cn112151-20210818-00579