Sellar-Suprasellar Paraganglioma: Report of 2 Cases and Review of Literature

Vasoya, Pavan; Aryan, Saritha; Thakar, Sumit; Sivaraju, Laxminadh; Ghosal, Nandita; Hegde, Alangar S.

doi:10.1016/j.wneu.2020.04.157

Cited by 5 publications

(7 citation statements)

References 22 publications

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“…Common parasympathetic PGL locations include the jugular bulb, the tympanic branch of the glossopharyngeal nerve, and the auricular branch of the vagus nerve 12 13. The sellar and parasellar regions are extremely rare cranial sites, with only 35 reported cases so far including this case 3. None of these cases have reported an associated RCH or VHL, although one case had only a family history of VHL 4.…”

Section: Discussionmentioning

confidence: 87%

“…Histopathology of the suprasellar lesion depicted a zellballen pattern of tumour cells with large cell size, abundant cytoplasm and small round nuclei, which also showed cytoplasmic positivity for chromogranin and diffuse positivity for synaptophysin suggestive of a PGL (figure 2). Considering this rare location of PGL, this case was published in the World Neurosurgery journal 3…”

Section: Case Presentationmentioning

confidence: 99%

“…Sympathetic PGLs are typically located in the chest and abdomen 1. Parasympathetic lesions have been described in the head and neck region, and location in the sella is rare, with only 35 cases reported so far in literature 2 3. Though most PGLs are sporadic, associations with familial syndromes, particularly von Hippel-Lindau (VHL) syndrome, have been described.…”

Section: Introductionmentioning

confidence: 99%

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Suprasellar paraganglioma in a clinical setting of von Hippel-Lindau syndrome

et al. 2022

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A man in his 20s presented to the neurosurgery department 2 years ago with headache and blurred vision. He was diagnosed to have a suprasellar mass on neuroimaging. Best-corrected visual acuity in the right eye was 6/36 and that in the left eye was 6/60. Automated visual fields showed a temporal hemianopia in the right eye and an advanced field defect in the left eye. His hormonal profile was normal, and he underwent partial excision of suprasellar tumour, which was a histopathologically proven paraganglioma (PGL). Subsequently, the patient underwent radiotherapy and his vision and visual fields showed improvement. Follow-up examination 3 years later showed a left retinal capillary hemangioblastoma (RCH), which was treated with green laser photocoagulation, resulting in complete sclerosis. This case is unique because of the extremely rare coexistence of a sellar PGL and RCH, which to our knowledge has not been reported so far.

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Section: Discussionmentioning

confidence: 87%

Section: Case Presentationmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Suprasellar paraganglioma in a clinical setting of von Hippel-Lindau syndrome

et al. 2022

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“…Although the exact origin of the SP is unknown, in the current case, the border between the tumor and the pituitary gland was clear, and the tumor extended into the cavernous sinus, suggesting that it may have originated from within the cavernous sinus. MRI of SP shows heterogeneous intensity on T2 with gadolinium enhancement, indicating high vascularity [ 12 , 32 , 33 ]. However, the findings were atypical for the present case, with a weak contrast effect and minimal enhancement in dynamic studies.…”

Section: Discussionmentioning

confidence: 99%

Gamma Knife Radiosurgery: An Adjuvant Therapy for Primary Sellar Paraganglioma

Bajagain,

Fujio,

Kirishima

et al. 2024

Cureus

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Sellar paraganglioma (SP) is a rare benign tumor, usually treated by surgery. SPs are lobulated, firm, adherent, and highly vascular, allowing mostly partial resection. We present the case of a 52-year-old man diagnosed with primary SP, treated with a transcranial-transsphenoidal (TC-TS) surgical approach, followed by adjuvant Gamma Knife stereotactic radiosurgery (GKSR). The tumor has an extra-pituitary origin, with a sellar-suprasellar, right cavernous sinus extension that encroached the bilateral optic nerve and anterior cerebral artery. Histopathology confirmed SP with a Zellballen pattern. Despite postoperative tumor growth observed at four and 10 months, a stable residual tumor was noted at a follow-up two years after GKSR. SP is diagnosed mainly in middle age or in adolescent males. The TC-TS approach offers a bidirectional view that allows greater resection by minimizing blind spots, thus reducing complications. Similar to the paragangliomas of other sites, the efficacy of GKSR was observed for primary SP. SP is a rare differential diagnosis of pituitary diseases; however, it should be considered. After surgical resection of primary SP, GKSR is observed as an effective adjuvant therapy.

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“…Besides epinephrine and norepinephrine, the dopamine level may also be increased in up to one-third of patients with head and neck PGL (3). PGLs in the sellar and parasellar region are extremely rare with 31 cases reported so far (4)(5)(6)(7)(8), none of them being secreting (4,6,9). Their origin has not been fully elucidated; however, it was suggested that they arise from residual aggregates of paraganglionic cells present during fetal and neonatal period along the tympanic or ciliary nerves or branches of the glossopharyngeal nerve within or close to the cavernous sinus (6,9).…”

Section: Introductionmentioning

confidence: 99%

Case Report: Malignant Primary Sellar Paraganglioma With Unusual Genetic and Imaging Features

et al. 2021

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BackgroundParaganglioma occurs rarely in the sellar/parasellar region. Here, we report a patient with malignant paraganglioma with primary sellar location with unusual genetic and imaging features.Case PresentationA 31-year-old male presented with mild hypertension, headache, nausea, and vomiting. A sellar/parasellar tumor mass was revealed by magnetic resonance imaging (MRI), while an endocrine work-up found partial hypopituitarism, suggesting that it was a non-functioning pituitary tumor. Antihypertensive therapy and hormone replacement were initiated. Tumor reduction was achieved with transsphenoidal neurosurgery. However, histological diagnosis was not possible due to extensive tissue necrosis. After 4 years of stable disease, the residual tumor showed re-growth requiring gamma knife radiosurgery. Four years after the radiosurgery, MRI showed a significant tumor progression leading to a second neurosurgery. This time, pathological and immunohistochemical findings revealed paraganglioma. Plasma levels of metanephrine and normetanephrine were normal. A gene sequencing panel performed on DNA extracted from blood excluded germline mutations in 17 susceptibility genes. The patient developed new tumor masses in the neck, and the third surgery was performed. Immunohistochemistry demonstrated lack of ATRX (alpha thalassemia/mental retardation syndrome X-linked) protein in tumor cells, indicating an ATRX gene mutation. Molecular genetic analysis performed on tumor DNA revealed a combination of ATRX and TP53 gene abnormalities; this was not previously reported in paraganglioma. MRI and 68Ga-DOTANOC PET/CT revealed the full extent of the disease. Therapy with somatostatin LAR and 177Lu-DOTATATE Peptide Receptor Radionuclide Therapy (PRRT) was initiated.ConclusionAlthough rare, paraganglioma should be considered in the differential diagnosis of sellar/parasellar tumor lesions, even in the absence of typical imaging features. ATRX gene mutation in paraganglioma is an early predictor of malignant behavior and a potential novel therapeutic marker when pharmacological therapy targeting mutated ATRX becomes available.

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Sellar-Suprasellar Paraganglioma: Report of 2 Cases and Review of Literature

Cited by 5 publications

References 22 publications

Suprasellar paraganglioma in a clinical setting of von Hippel-Lindau syndrome

Suprasellar paraganglioma in a clinical setting of von Hippel-Lindau syndrome

Gamma Knife Radiosurgery: An Adjuvant Therapy for Primary Sellar Paraganglioma

Case Report: Malignant Primary Sellar Paraganglioma With Unusual Genetic and Imaging Features

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