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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 -
International Journal of Surgery Case... Sep 2022Pituicytomas are low-grade glial tumors in the sellar and suprasellar region. They may be easily confused with pituitary lesions. We review the literature in order to...
INTRODUCTION
Pituicytomas are low-grade glial tumors in the sellar and suprasellar region. They may be easily confused with pituitary lesions. We review the literature in order to better understand and categorize the natural history, clinical presentations, and treatments.
PRESENTATION OF CASE
A 45-year-old female patient who complained of left eye blurred vision for 2 months. The imaging study revealed a solid sellar tumor with marked homogeneous enhancement following intravenous administration of gadolinium, and compression of the optic chiasm. Thus, under the preoperative diagnosis of pituitary macroadenoma, the patient underwent endoscope-assisted surgery via the transsphenoidal approach. The patient recovered well after surgery. The histopathological diagnosis was pituicytoma, WHO grade I.
CLINICAL DISCUSSION
Pituicytomas are defined as a circumscribed low-grade glial tumor arising from the neurohypophysis or infundibulum with bipolar spindle cells arranged in a fascicular or storiform pattern (a cartwheel). The clinical symptoms are variable depending on the tumor size and location. They usually present due to mass effect. The radiographic characteristics are not nonspecific. The diagnosis of pituicytoma is based on histopathological evidence. Pituicytomas consist of a solid proliferation of elongated spindle cells arranged in interlacing fascicles and/or in a "storiform" pattern. In immunohistochemical studies, pituicytomas was strongly expressed in TTF-1.
CONCLUSION
Pituicytomas are benign, slow-growing glial tumors. It is difficult to diagnosed before operation as its clinical presentations and imaging studies resemble those of non-functional pituitary adenomas. The best chance of successful treatment is gross total resection by the endoscopic approach or transcranial approach.
PubMed: 36057248
DOI: 10.1016/j.ijscr.2022.107553 -
Oncology Letters Jan 2011This report describes two cases of pituicytoma. The two patients were female and presented with visual complaints, diabetes insipidus, headaches and menstrual disorders....
This report describes two cases of pituicytoma. The two patients were female and presented with visual complaints, diabetes insipidus, headaches and menstrual disorders. The imaging characteristic was an intrasellar or suprasellar mass, and one mass originated in the pituitary stalk. The mass showed homogeneous enhancement with contrast administration. A hematoxylin and eosin stain showed a compact structure consisting of elongated, bipolar spindle cells arranged in interlacing fascicles or assuming a storiform pattern. Immunohistochemically, the tumor showed diffuse strong expression of S-100 protein, vimentin and epithelial membrane antigen, and glial fibrillary acidic protein was focally positive. The pituicytoma may have originated in the pituitary stalk and presented with diabetes insipidus, and the differential diagnosis should be compared with the pituitary stalk mass. Bleeding during resection was moderate, and surgery was regarded as the first choice of treatment for pituicytomas.
PubMed: 22870125
DOI: 10.3892/ol.2010.209 -
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 -
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 -
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 -
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 -
Brain Tumor Research and Treatment Oct 2017A 19-year-old man presented with bitemporal hemianopsia and was found to have a large sellar and suprasellar tumor, resembling a pituitary macroadenoma. Emergency...
A 19-year-old man presented with bitemporal hemianopsia and was found to have a large sellar and suprasellar tumor, resembling a pituitary macroadenoma. Emergency transsphenoidal approach was attempted because of rapid visual deterioration with headache. However, the approach was complicated and stopped by uncontrolled hemorrhage from the tumor. After conventional cerebral angiography and recognition of an unusual pathology, transcranial approach was achieved to prevent permanent visual loss. The final pathological diagnosis was pituicytoma with epithelioid features. Pituicytoma is a rare low-grade tumor (WHO Grade I) of pituicytes involving the sellar and suprasellar region, and originating from special glial cells of the neurohypophysis. Because of the high vascularity, the firm consistency, and invasion to surrounding neurovascular structures, a pituicytoma should be included in the differential diagnosis of a mass in the sellar and suprasellar area if the tumor shows high enhancement with vascular components. We report a case of rare pituicytoma mimicking a pituitary macroadenoma with massive hemorrhage to disturb surgery.
PubMed: 29188213
DOI: 10.14791/btrt.2017.5.2.110 -
Neurology India 2010
Review
Topics: Adult; Glial Fibrillary Acidic Protein; Glioma; Humans; Magnetic Resonance Imaging; Male; Pituitary Neoplasms; S100 Proteins; Visual Fields
PubMed: 21045523
DOI: 10.4103/0028-3886.72187 -
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