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Journal of Radiology Case Reports

Sporadic Hemangioblastoma Arising from the Infundibulum

Case Report

Michael N Pakdaman1*, Matthew J Austin1, Serguei Bannykh2, Barry D Pressman1

Radiology Case. 2017 May; 11(5):1-6 :: DOI: 10.3941/jrcr.v11i5.2981

Cite this paper

1. Department of Imaging, Cedars Sinai S. Mark Taper Foundation Imaging Center, Los Angeles, USA
2. Department of Pathology and Laboratory Medicine, Cedars Sinai Medical Center, Los Angeles, USA

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  ABSTRACT
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Hemangioblastomas are rare vascular tumors most often found in the posterior fossa and cervical spinal cord and commonly associated with von Hippel-Lindau Disease. We report a case of sporadic hemangioblastoma in a patient without von Hippel-Lindau Disease. Imaging characteristics included a solid, suprasellar mass that was homogeneously enhancing. These findings most resembled a pituicytoma or choroid glioma because of the close association with the infundibulum and the homogeneous avid enhancement. Microscopically, the neoplasm was seen to be composed of vascular channels associated with foamy stromal cells, containing clear cytoplasmic vacuoles. Microscopic and immunohistochemical findings were consistent with hemangioblastoma. Hemangioblastomas are a rare form of vascular tumor most commonly associated with von-Hippel Lindau disease. Our finding of non-cystic hemangioblastoma arising from the infundibulum demonstrates that, while rare, hemangioblastomas should be considered on the differential diagnosis for an avidly enhancing suprasellar mass.

  CASE REPORT
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A 38-year-old female presented with a 9-month history of headaches and amenorrhea. Laboratory workup revealed low luteinizing hormone LH and follicle stimulating hormone FSH. Her past medical history includes irregular menses, remote history of melanoma excision from her thigh, and surgical removal of an ovarian cyst as a child. MRI of the pituitary was performed.

Magnetic resonance imaging [MRI] exam of the pituitary found the pituitary gland to be within normal limits in appearance but relatively small in size with a maximum height of approximately 3 mm. For a woman in the childbearing years this pituitary size was considered small. Additionally, a 13 x 13 x 13.2 mm homogeneously enhancing spherical mass was seen in the upper half of the infundibulum, which appeared to arise from the infundibulum itself and impinge upon the undersurface of the optic chiasm and third ventricle. The mass was isointense relative to the cortex on T1- and T2-weighted images, and enhanced avidly and homogeneously after contrast administration (Fig. 1 Preview this figure

Figure 1: Magnetic Resonance Imaging
38-year-old female with sporadic hemangioblastoma of infundibulum. Technique: Gadolinium enhanced 3T MRI of the brain, pituitary protocol. Sagittal and coronal post-contrast T1 weighted images and axial T2 weighted image through the pituitary fossa. Findings: (a)Homogeneously enhancing 13x13mm spherical mass seen in upper half of infundibulum (long arrow), (b) and impinging on the undersurface of the optic chiasm (short arrow). (c) The mass was isointense relative to cortex on both t1-weighted images (white arrowhead) (d) t2-weighted images (black arrowhead), and t1-weighted precontrast sagittal images.
). The lesion was not well appreciated on DWI or on ADC map (Fig. 2 Preview this figure
Figure 2: Magnetic Resonance Imaging
38-year-old female with sporadic hemangioblastoma of infundibulum. Technique: 3T MRI of the brain, pituitary protocol. Diffusion weighted images with ADC map. Findings: No diffusion restriction is noted on the b1000 images (a) and there is no dark spot on the ADC map (b).
).

The patient underwent endoscopic transsphenoidal resection of the mass using stereotactic navigation. Intraoperatively, a soft, fleshy, yellowish tumor with a vascular capsule was identified in the suprasellar space arising directly from the pituitary stalk, grossly reminiscent of a pituicytoma. Gross total resection of the primary lesion was obtained, and the pituitary stalk was sectioned as a component of tumor removal. Partial resection of pituitary gland was also performed for better exposure to the lesion.

Gross inspection of the mass revealed a 6 x 3 x 3 mm fragment of red-tan soft tissue. Histopathologic analysis of hematoxylin and eosin [H & E] stained sections showed a neoplasm composed of vascular channels associated with foamy stromal cells, containing clear cytoplasmic vacuoles (Fig. 3 Preview this figure
Figure 3: Microscopic pathology
38-year-old female with sporadic hemangioblastoma of infundibulum. Technique: (a) H&E stained section 200x magnification, (b) s100 stained section 200x magnification, (c) NSE stained section 200x magnification, (d) galectin 3 stained section 100x magnification. Findings: (a) H&E stained section disclose vascularized tumor with abundance of vacuolated foamy cells (*). (b) Positive red chromagen staining for s100 (**). (c) Positive red chromagen on NSE stain (***). (d) Positive red chromagen on galectin 3 stain (****).
A). No necrosis or mitotic activity was seen. Immunohistochemical analysis revealed positive staining for S100 (Fig. 3 Preview this figure
Figure 3: Microscopic pathology
38-year-old female with sporadic hemangioblastoma of infundibulum. Technique: (a) H&E stained section 200x magnification, (b) s100 stained section 200x magnification, (c) NSE stained section 200x magnification, (d) galectin 3 stained section 100x magnification. Findings: (a) H&E stained section disclose vascularized tumor with abundance of vacuolated foamy cells (*). (b) Positive red chromagen staining for s100 (**). (c) Positive red chromagen on NSE stain (***). (d) Positive red chromagen on galectin 3 stain (****).
B), neuron-specific enolase [NSE] (Fig. 3 Preview this figure
Figure 3: Microscopic pathology
38-year-old female with sporadic hemangioblastoma of infundibulum. Technique: (a) H&E stained section 200x magnification, (b) s100 stained section 200x magnification, (c) NSE stained section 200x magnification, (d) galectin 3 stained section 100x magnification. Findings: (a) H&E stained section disclose vascularized tumor with abundance of vacuolated foamy cells (*). (b) Positive red chromagen staining for s100 (**). (c) Positive red chromagen on NSE stain (***). (d) Positive red chromagen on galectin 3 stain (****).
C) and galectin-3 (Fig. 3 Preview this figure
Figure 3: Microscopic pathology
38-year-old female with sporadic hemangioblastoma of infundibulum. Technique: (a) H&E stained section 200x magnification, (b) s100 stained section 200x magnification, (c) NSE stained section 200x magnification, (d) galectin 3 stained section 100x magnification. Findings: (a) H&E stained section disclose vascularized tumor with abundance of vacuolated foamy cells (*). (b) Positive red chromagen staining for s100 (**). (c) Positive red chromagen on NSE stain (***). (d) Positive red chromagen on galectin 3 stain (****).
D), with some weak staining for inhibin. The tumor was negative for meningioma and spindle cell oncocytoma marker epithelial membrane antigen [EMA], as well as chordoid glioma of the third ventricle and pituicytoma marker glial fibrillary acidic protein [GFAP]. Based on the microscopic and immunohistochemical findings, a diagnosis of hemangioblastoma was established.

  DISCUSSION
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Etiology & Demographics:

Hemangioblastomas are highly vascular tumors found in the central nervous system [CNS], most commonly detected in the posterior fossa and cervical spinal cord, but also seen in retina [1]. Hemangioblastomas are uncommon, accounting for approximately 3% of CNS tumors [2]. They are less commonly sporadic, and rarely occur outside of the posterior fossa. Only a small handful of cases have been reported of supratentorial hemangioblastoma without von Hippel-Lindau Disease [VHL] [3]. Of these, only four cases of hemangioblastoma arising from the pituitary infundibulum have been reported [4].


Clinical & Imaging Findings:

We report a case of suprasellar hemangioblastoma in a patient without VHL, which, based on close association with the infundibulum and avid enhancement, strongly resembled a pituicytoma.

While up to 83% of VHL patients may have cerebellar hemangioblastomas, solitary hemangioblastomas are associated with VHL disease in only 10-20% of cases [2, 3, 5-8]. VHL-associated hemangioblastomas are typically multiple, often recur after surgical resection, and present at age 20-30 years, whereas the sporadic form is commonly solitary, occurs at age 40-50 years, and does not recur after definitive treatment.

Hemangioblastomas of the CNS are best visualized on gadolinium-enhanced MRI. While MRI findings are not pathognomonic, hemangioblastomas are typically seen on MRI as a cystic mass with an enhancing mural nodule, most commonly in the posterior fossa. The nodule is dark on T1-weighted images, bright on T2-weighted images, and homogeneously enhancing [3, 7, 9]. Infrequently hemangioblastomas may be solid, with peripheral enhancement, or cystic with an enhancing wall. Neuroangiography reveals a highly vascular tumor within an avascular cyst, with feeding vessels from dural arteries [3].

The clinical presentation of hemangioblastomas depends on the site of the lesion. As hemangioblastomas are most commonly found in the posterior fossa, symptoms are typically related to cerebellar dysfunction and include ataxia and gait disturbance [10]. Headaches are also common and related to mass effect from the tumor resulting in increased intracranial pressure [10].

Hemangioblastomas can be divided into sporadic and familial syndromic forms. Familial forms are associated with von Hippel-Lindau Disease, an autosomal dominant condition characterized by a large variety of predominantly low-grade lesions (endolymphatic sac tumor, cystadenoma of epididymis and broad ligament, renal and pancreatic cysts), but also more aggressive (pheochromocytomas and neuroendocrine tumors of pancreas) and malignant (renal cell carcinoma) tumors. VHL is caused by mutations in tumor suppressing VHL gene resulting in altered signaling pathways that cause upregulation of vascular endothelial growth factor [VEGF] [9].

Histopathologically, hemangioblastomas are highly vascular tumors consisting of numerous capillaries and stromal cells [10]. They are seen as an extensive vascular network with neoplastic stromal cells featuring nuclear hyperchromasia and degenerative atypia [10]. They are commonly positive for CD34, NSE [7], S100 protein [11, 12], and galectin-3 [13].


Treatment & Prognosis:

Definitive management of CNS hemangioblastoma is by surgical resection of the enhancing nodule. If the location of the mass is not amenable to excision, then stereotactic surgery and radiation therapy are non-definitive alternatives. Additionally, as hemangioblastomas in VHL can often recur, surgery is reserved for symptomatic lesions.


Differential Diagnoses:

The differential diagnosis for a solid suprasellar enhancing mass includes meningioma, pituicytoma, chordoid glioma, germ cell tumor, aneurysm, sarcoidosis, Langerhans cell histiocytosis, metastases, and rarely hemangioblastomas. Meningiomas are typically isointense on T1 and T2 and less likely hypointense on T1 and hyperintense on T2 to grey matter, and contain calcifications. Pituicytomas are hypointense to gray matter on T2 weighted images. Chordoid gliomas are isointense to gray matter on T1 and T2-weighted images. Germ cell tumors are variable depending on the subtype, but are generally hypointense or isointense to gray matter on both T1 and T2 weighted images, and may demonstrate susceptibility artifact from small hemorrhages. Aneurysms are best seen on CT or MR angiography studies, where a vessel diameter greater than 3mm is highly suggestive. Unlike hemangioblastomas that demonstrate a T2 hyperintense cystic component, aneurysms will be dark on T2 due to flow voids. Neurosarcoid typically causes hydrocephalus from ventricular outflow compression and is multifocal, whereas hemangioblastoma is typically solitary, particularly in the sporadic form. Although Langerhans cell histiocytosis may present as a slightly hyperintense mass compared to gray matter on T2-weighted images, it is often accompanied by multiple lytic bony lesions seen on CT, plain film, or MRI. Metastases are also typically in the differential diagnosis for any intracranial mass, however the infundibulum is an uncommon location for metastatic disease.

  TEACHING POINT
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We report a sporadic case of solitary, non-cystic hemangioblastoma arising from the infundibulum and appearing quite similar to a pituicytoma and chordoid glioma. This demonstrates that, while rare, hemangioblastomas should be considered on the differential diagnosis for an avidly enhancing suprasellar mass.








  FIGURES
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Display figure 1 in original size

Figure 1: 38-year-old female with sporadic hemangioblastoma of infundibulum.
Technique: Gadolinium enhanced 3T MRI of the brain, pituitary protocol. Sagittal and coronal post-contrast T1 weighted images and axial T2 weighted image through the pituitary fossa.
Findings: (a)Homogeneously enhancing 13x13mm spherical mass seen in upper half of infundibulum (long arrow), (b) and impinging on the undersurface of the optic chiasm (short arrow). (c) The mass was isointense relative to cortex on both t1-weighted images (white arrowhead) (d) t2-weighted images (black arrowhead), and t1-weighted precontrast sagittal images.


Figure 1: Magnetic Resonance Imaging (Open in original size)
38-year-old female with sporadic hemangioblastoma of infundibulum.
Technique: Gadolinium enhanced 3T MRI of the brain, pituitary protocol. Sagittal and coronal post-contrast T1 weighted images and axial T2 weighted image through the pituitary fossa.
Findings: (a)Homogeneously enhancing 13x13mm spherical mass seen in upper half of infundibulum (long arrow), (b) and impinging on the undersurface of the optic chiasm (short arrow). (c) The mass was isointense relative to cortex on both t1-weighted images (white arrowhead) (d) t2-weighted images (black arrowhead), and t1-weighted precontrast sagittal images.


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Display figure 2 in original size
Figure 2: 38-year-old female with sporadic hemangioblastoma of infundibulum.
Technique: 3T MRI of the brain, pituitary protocol. Diffusion weighted images with ADC map.
Findings: No diffusion restriction is noted on the b1000 images (a) and there is no dark spot on the ADC map (b).


Figure 2: Magnetic Resonance Imaging (Open in original size)
38-year-old female with sporadic hemangioblastoma of infundibulum.
Technique: 3T MRI of the brain, pituitary protocol. Diffusion weighted images with ADC map.
Findings: No diffusion restriction is noted on the b1000 images (a) and there is no dark spot on the ADC map (b).


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Display figure 3 in original size
Figure 3: 38-year-old female with sporadic hemangioblastoma of infundibulum.
Technique: (a) H&E stained section 200x magnification, (b) s100 stained section 200x magnification, (c) NSE stained section 200x magnification, (d) galectin 3 stained section 100x magnification.
Findings: (a) H&E stained section disclose vascularized tumor with abundance of vacuolated foamy cells (*). (b) Positive red chromagen staining for s100 (**). (c) Positive red chromagen on NSE stain (***). (d) Positive red chromagen on galectin 3 stain (****).


Figure 3: Microscopic pathology (Open in original size)
38-year-old female with sporadic hemangioblastoma of infundibulum.
Technique: (a) H&E stained section 200x magnification, (b) s100 stained section 200x magnification, (c) NSE stained section 200x magnification, (d) galectin 3 stained section 100x magnification.
Findings: (a) H&E stained section disclose vascularized tumor with abundance of vacuolated foamy cells (*). (b) Positive red chromagen staining for s100 (**). (c) Positive red chromagen on NSE stain (***). (d) Positive red chromagen on galectin 3 stain (****).


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Display figure 4 in original size
Figure 4: Summary table for Hemangioblastoma.

Figure 4: Table (Open in original size)
Summary table for Hemangioblastoma.

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Display figure 5 in original size
Figure 5: Differential diagnosis table for Hemangioblastoma.

Figure 5: Table (Open in original size)
Differential diagnosis table for Hemangioblastoma.

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  REFERENCES
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1. Kassardjian CD, Macdonald RL, Munoz DG. Hemangioblastomas in the elderly: epidemiology and clinical characteristics. J Clin Neurosci 2014;21(7):1205-8 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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2. Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome J Neurosurg Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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3. Pandey S, Sharma V, Pandey D, Kumar V, Kumar M. Supratentorial haemangioblastoma without von Hippel-Lindau syndrome in an adult: A rare tumor with review of literature. Asian journal of neurosurgery 2016;11(1):8-14 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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4. Schar RT, Vajtai I, Sahli R, Seiler RW. Manifestation of a sellar hemangioblastoma due to pituitary apoplexy: a case report. Journal of medical case reports 2011;5:496 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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5. Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau disease: genetic, clinical, and imaging features. Radiology 1995;194(3):629-42 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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6. Feletti A, Anglani M, Scarpa B, Schiavi F, Boaretto F, Zovato S, Taschin E, Gardi M, Zanoletti E, Piermarocchi S, Murgia A, Pavesi G, Opocher G. Von Hippel-Lindau disease: an evaluation of natural history and functional disability. Neuro Oncol 2016 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

Back Back

7. Li Z, Feng T, Teng H, Hu Y, Yao Y, Liu Y. Suprasellar hemangioblastoma without von Hippel-Lindau disease: a case report and literature review. International journal of clinical and experimental pathology 2015;8(6):7553-8 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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9. Bamps S, Calenbergh FV, Vleeschouwer SD, Loon JV, Sciot R, Legius E, Goffin J. What the neurosurgeon should know about hemangioblastoma, both sporadic and in Von Hippel-Lindau disease: A literature review. Surg Neurol Int 2013;4:145 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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10. Innus C, Patterson J. Hemangioblastoma without von Hippel-Lindau disease. JAAPA : official journal of the American Academy of Physician Assistants 2007;20(11):28, 31-2 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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11. Cheng HX, Chu SG, Xu QW, Wang Y. A spinal tumor showing mixed features of ependymoma and hemangioblastoma: a case report and literature review. Brain tumor pathology 2015;32(2):112-8 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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12. Epari S, Bhatkar R, Moyaidi A, Shetty P, Gupta T, Kane S, Jalali R. Histomorphological spectrum and immunohistochemical characterization of hemangioblastomas: an entity of unclear histogenesis. Indian journal of pathology & microbiology 2014;57(4):542-8 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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13. Al-Salam S, Al-Salam M, Al Ashari M. Galectin-3: a novel protein in cerebellar hemangioblastoma. International journal of clinical and experimental pathology 2013;6(5):853-61 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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  ABBREVIATIONS
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CAN = central nervous system
EMA = Epithelial membrane antigen
GFAP = glial fibrillary acidic protein
H & E = hematoxylin and eosin
MRI = magnetic resonance imaging
NSE = neuron-specific enolase
VEGF = vascular endothelial growth factor
VHL = von Hippel-Lindau Disease









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