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Surgical Neurology International 2018Pituicytomas are considered World Health Organization Grade I malignancies. Until September 2017, a total of 81 cases of pituicytomas were diagnosed and described in...
BACKGROUND
Pituicytomas are considered World Health Organization Grade I malignancies. Until September 2017, a total of 81 cases of pituicytomas were diagnosed and described in literature. We present such a case in which histopathology shows an epithelioid pattern, a rare variant of pituicytoma. As far as we know, this is only the second such case described in the literature.
CASE DESCRIPTION
A 61-year-old male patient presented with complaints of progressive decrease in visual acuity for about 7 months, worse on the left side. Laboratory and endocrinological investigation returned normal values. Magnetic resonance imaging revealed a mixed solid-cystic lesion, measuring about 3.1 × 2.2 × 2.9 cm. The lesion presented with intermediate signal intensity in T1 and T2 sequences and showed avid postcontrast enhancement. The patient underwent resection through a left pterional approach. Pathology revealed a glial neoplasm with an epithelioid pattern and moderate cellularity with rounded-elongated cell nuclei and with a broad eosinophilic cytoplasm. Absence of cellular pleomorphism, any mitotic figures, or areas of necrosis was noted.
CONCLUSION
The epithelioid variant of pituicytomas differs from the commonly encountered forms of this tumor which typically present in a fascicular pattern. Microsurgical resection is the treatment of choice. However, in many cases, subtotal resection was performed because of a considerable risk for neurovascular injuries.
PubMed: 30105139
DOI: 10.4103/sni.sni_319_17 -
Surgical Pathology Clinics Jun 2020Sellar region lesions include a broad range of benign and malignant neoplastic as well as non-neoplastic entities, many of which are newly described or have recently... (Review)
Review
Sellar region lesions include a broad range of benign and malignant neoplastic as well as non-neoplastic entities, many of which are newly described or have recently revised nomenclature. In contrast to other intracranial sites, imaging features are relatively less specific, and the need for histopathological diagnosis is of paramount importance. This review will describe pituitary adenomas, inflammatory lesions, and tumors unique to the region (craniopharyngioma) as well as tumors which may occur in but are not exclusively localized to the sellar location (schwannoma, metastasis, etc.).
Topics: Adenoma; Central Nervous System Cysts; Craniopharyngioma; Diagnosis, Differential; Humans; Hypophysitis; Immunoglobulin G4-Related Disease; Pituitary Diseases; Pituitary Gland; Pituitary Neoplasms
PubMed: 32389269
DOI: 10.1016/j.path.2020.02.006 -
Neuropathology : Official Journal of... Oct 2016Pituicytoma is rare and difficult to diagnose. This study explored the clinicopathological features, immunophenotype and differential diagnosis of pituicytoma. We...
Pituicytoma is rare and difficult to diagnose. This study explored the clinicopathological features, immunophenotype and differential diagnosis of pituicytoma. We compared 11 cases of pituicytoma and 26 cases of sellar glioma (16 pilocytic astrocytomas, four diffuse astrocytomas, three pilomyxoid astrocytomas, and three third ventricle chordoid gliomas). The 11 pituicytoma cases involved six men and five women (age: 33-65 years). Three of the 11 patients experienced recurrence due to a residual tumor, and one patient underwent three surgeries during a 6-month period. Imaging findings revealed tumors were in the intrasellar region (four cases), suprasellar region (four cases) and intra-suprasellar regions (three cases). The tumor diameters were 1.3-3.8 cm, and the preoperative diagnoses were pituitary adenoma, craniopharyngioma and meningioma. The tumors were solid and contained spindle or slightly chubby cells that were densely arranged with visible cleft-like or expanded sinusoid structures. The cells had vague boundaries, circular nuclei, fine chromatin, and a small nucleolus. Immunohistochemical staining of the pituicytomas revealed positive expression of thyroid transcription factor-1 (TTF-1) and S-100 protein (S-100), positive focal expression of glial GFAP;(five of 11 cases), and negative oligodendrocyte transcription factor 2 (Olig2), CD34 and neurofilament expression. The Ki67 index was 6% in one case and 1-2% in the other cases. Unlike pituicytoma, most sellar glioma cases exhibited GFAP and Olig2 expression, and negative TTF-1 expression. Third ventricle chordoid gliomas expressed TTF-1, GFAP and CD34, and were negative for Olig2. Our results indicate that pituicytoma typically involves dense arrangements of spindle or slightly chubby cells. The morphology is occasionally atypical, with ependymoma-like or meningioma-like structures, and occasionally exhibits pilomyxoid degeneration. Abundant sinusoids are characteristic of hemorrhagic tumors. The dense spindle cell arrangement is a relatively specific morphology, and staining for GFAP, TTF-1, Olig2 and CD4 may help differentiate pituicytoma from sellar glioma.
Topics: Adult; Aged; Biomarkers, Tumor; Craniopharyngioma; Diagnosis, Differential; Female; Glioma; Humans; Male; Meningeal Neoplasms; Middle Aged; Pituitary Neoplasms; Sella Turcica
PubMed: 26919073
DOI: 10.1111/neup.12291 -
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 -
Pathology International Oct 2011Pituicytoma is a rare low-grade glial neoplasm that originates in the neurohypophysis or infundibulum. Because of its rare occurrence, the morphology and differentiation...
Pituicytoma is a rare low-grade glial neoplasm that originates in the neurohypophysis or infundibulum. Because of its rare occurrence, the morphology and differentiation of pituicytoma have not been fully clarified. Here, we report a case of pituicytoma with unusual histological features mimicking ependymoma, but exhibiting the diverse morphology and differentiation of pituicytoma. The 1.4 cm-sized suprasellar mass was incidentally found in the magnetic resonance image of a 42-year-old Korean woman who had had a traffic accident. Four years later, she presented with symptoms of hypopituitarism and the follow-up images revealed slight enlargement of the mass. After gross total resection, microscopic examination revealed oval to elongated cells with abundant eosinophilic cytoplasm arranged in a perivascular pseudorosette pattern and short interlacing fascicles. Pleomorphic tumor cells and Herring bodies were diffusely distributed within the tumor. Neither Rosenthal fibers nor eosinophilic granular bodies were identified. The tumor cells were immunohistochemically positive for glial fibrillary acidic protein, vimentin and S-100 protein, but negative for synaptophysin and adenohypophyseal hormones. The epithelial membrane antigen and CD99 were expressed with a paranuclear dot-like or membranous pattern in some tumor cells. Ultrastructural examination revealed that the tumor cells with intermediate filaments were closely apposed with intercellular junctions and frequent basal lamina production.
Topics: Adult; Biomarkers, Tumor; Diagnosis, Differential; Ependymoma; Female; Glial Fibrillary Acidic Protein; Glioma; Humans; Magnetic Resonance Imaging; Pituitary Gland, Posterior; Pituitary Neoplasms; S100 Proteins; Treatment Outcome; Vimentin
PubMed: 21951670
DOI: 10.1111/j.1440-1827.2011.02708.x -
The Journal of Endocrinology Jun 2023'Pituitary tumours' is an umbrella term for various tumours originating from different regions of the hypothalamic-pituitary system. The vast majority of pituitary... (Review)
Review
'Pituitary tumours' is an umbrella term for various tumours originating from different regions of the hypothalamic-pituitary system. The vast majority of pituitary tumours are pituitary adenomas, also recently referred to as pituitary neuroendocrine tumours. The prevalence of clinically relevant pituitary adenomas is approximately 1 in 1000; other pituitary tumours such as craniopharyngioma and pituicytoma are comparatively very rare. This review addresses the molecular and genetic aspects of pituitary adenomas. We first discuss the germline genetic variants underlying familial pituitary tumours, which account for approximately 5% of all pituitary adenoma cases. This includes variants in established pituitary adenoma/hyperplasia predisposition genes (MEN1, PRKAR1A, AIP, CDKN1B, GPR101, SDHA, SDHB, SDHC, SDHD, SDHAF2) as well as emerging genetic associations. In addition, we discuss McCune-Albright syndrome which lies between the germline and somatic pituitary tumour genes as the causative GNAS mutations are postzygotic rather than being inherited, and the condition is associated with multiglandular features due to the involvement of different cell lines rather than being limited to the pituitary. By contrast, somatic GNAS mutations contribute to sporadic acromegaly. USP8 is the only other gene where somatic driver mutations have been established in sporadic pituitary tumorigenesis. However, there are now known to be a variety of other somatic genetic and molecular changes underpinning sporadic pituitary adenomas which we review here, namely: copy number variation, molecular changes in signalling and hypoxia pathways, epithelial-mesenchymal transition, DNA repair, senescence, the immune microenvironment and epigenetics.
Topics: Humans; Pituitary Neoplasms; DNA Copy Number Variations; Adenoma; Pituitary Gland; Mutation; Transcription Factors; Tumor Microenvironment
PubMed: 36951812
DOI: 10.1530/JOE-22-0291 -
Toxicologic Pathology 1997
Topics: Animals; Astrocytoma; Female; Mice; Mice, Inbred ICR; Pituitary Gland, Posterior; Pituitary Neoplasms
PubMed: 9323844
DOI: 10.1177/019262339702500515 -
Endocrine Pathology Sep 2013Pituicytoma is a rare low-grade (WHO grade I) sellar region glioma. Among sellar tumors, pituitary adenomas, mainly prolactinomas, may show amyloid deposits. Gelsolin is...
Pituicytoma is a rare low-grade (WHO grade I) sellar region glioma. Among sellar tumors, pituitary adenomas, mainly prolactinomas, may show amyloid deposits. Gelsolin is a ubiquitous calcium-dependent protein that regulates actin filament dynamics. Two known gene point mutations result in gelsolin amyloid deposition, a characteristic feature of a rare type of familial amyloid polyneuropathy (FAP), the Finnish-type FAP, or hereditary gelsolin amyloidosis (HGA). HGA is an autosomal-dominant systemic amyloidosis, characterized by slowly progressive neurological deterioration with corneal lattice dystrophy, cranial neuropathy, and cutis laxa. A unique case of pituicytoma with marked gelsolin amyloid deposition in a 67-year-old Chinese woman is described. MRI revealed a 2.6-cm well-circumscribed, uniformly contrast-enhancing solid sellar mass with suprasellar extension. Histologically, the lesion was characterized by solid sheets and fascicles of spindle cells with slightly fibrillary cytoplasm and oval nuclei with pinpoint nucleoli. Surrounding brain parenchyma showed marked reactive piloid gliosis. Remarkably, conspicuous amyloid deposits were identified as pink homogeneous spherules on light microscopy that showed apple-green birefringence on Congo red with polarization. Mass spectrometric-based proteomic analysis identified the amyloid as gelsolin type. Immunohistochemically, diffuse reactivity to S100 protein and TTF1, focal reactivity for GFAP, and no reactivity to EMA, synaptophysin, and chromogranin were observed. HGA-related mutations were not identified in the tumor. No recurrence was noted 14 months after surgery. To the knowledge of the authors, amyloid deposition in pituicytoma or tumor-associated gelsolin amyloidosis has not been previously described. This novel finding expands the spectrum of sellar tumors that may be associated with amyloid deposition.
Topics: Aged; Amyloidosis, Familial; Female; Gelsolin; Glioma; Humans; Pituitary Neoplasms
PubMed: 23817895
DOI: 10.1007/s12022-013-9254-y -
Human Pathology Sep 2016
Topics: Adenoma; Glioma; Humans; Magnetic Resonance Imaging; Pituitary Neoplasms
PubMed: 27184480
DOI: 10.1016/j.humpath.2016.03.024 -
Journal of Neurosurgery Nov 1987Tumors of the posterior pituitary are rare and the previous nomenclature has been confusing. A 40-year-old man presented with visual failure and disconnection...
Tumors of the posterior pituitary are rare and the previous nomenclature has been confusing. A 40-year-old man presented with visual failure and disconnection hyperprolactinemia (830 mU/liter) due to a large invasive pituitary tumor shown to be a pituicytoma (pilocytic astrocytoma). Its astrocytic nature was confirmed by positive immunostaining for glial fibrillary acidic protein and the finding of cytoplasmic filaments on electron microscopy. This case report contains the pathological description of a pituicytoma and illustrates that a non-adenomatous pituitary lesion may masquerade as a pituitary adenoma. Large pituitary lesions associated with serum prolactin concentrations of less than 3000 mU/liter require early surgical biopsy.
Topics: Adult; Astrocytoma; Humans; Immunohistochemistry; Male; Microscopy, Electron; Pituitary Neoplasms; Tomography, X-Ray Computed
PubMed: 3668648
DOI: 10.3171/jns.1987.67.5.0768