Pituitary stalk hemangioblastoma: The fourth case report and review of the literature

doi:10.1016/j.clineuro.2006.11.007

Clinical Neurology and Neurosurgery

Volume 109, Issue 3, April 2007, Pages 292-298

https://doi.org/10.1016/j.clineuro.2006.11.007 Get rights and content

Abstract

Supratentorial hemangioblastomas (HBL) have been rarely described in the literature. Herein we report the fourth case of pituitary stalk HBL diagnosed concurrently with cerebellar HBLs in a 51-year-old woman with von Hippel-Lindau disease. Complete resection of the lesion was achieved using left frontopterional craniotomy and no recurrence was observed after 8 years of follow-up. The clinical presentation, radiological features, pathological findings, and the management of this particular case are discussed in the light of the available literature. To our knowledge, this case is the only pituitary stalk HBL with total resection, and a long follow-up fully documented.

Introduction

Hemangioblastomas (HBLs) represent about 1.5–2.5% of all primary intracranial tumors and 7–12% of posterior fossa tumors [1], [2]. The most common site of occurrence is the cerebellum; however, they may also occur in the medulla oblongata and spinal cord [3], [4]. In 20% of cases, they are associated with von Hippel-Lindau (VHL) disease which is an autosomal dominant syndrome resulting from a germline mutation of the VHL tumor suppressor gene on the short arm of chromosome 3. Patients affected can develop various benign or malignant tumors of the central nervous system, kidneys, adrenal glands, pancreas, and reproductive organs. In a series of 231 cases of VHL disease, Wanebo found 4% of supratentorial HBLs [5]. Pituitary stalk HBLs have been reported exceptionally; to date, only three cases have been described [2], [6], [7]. Herein we report the fourth case of pituitary stalk HBL with a literature review focusing upon sellar and suprasellar HBLs. In addition, the radiosurgical management of these tumors is discussed.

Section snippets

Case report

A 51-year-old woman was admitted to our hospital in 1998 with a 4-month history of progressive impairment of the vision in her right eye. Her medical history revealed that she had already lost her left eye's vision at the age of 18 (1965), following cobalt therapy for a retinal HBL. Five years later, she developed a progressive paraparesis accompanied by back pain and impaired sensation in both lower limbs. An intramedullar hypervascular mass was demonstrated at the T₄ level and was removed

Discussion

Supratentorial HBLs are rare with less than 100 published cases [1], [8], [9], [10], [11], [12], [13], [14] located in the cerebral hemispheres, generally in young adults as sporadic tumors. Sellar and suprasellar HBLs are exceptional with only 16 cases reported in the literature [2], [4], [6], [7], [11], [12], [14], [15], [16], [17], [18], [19], [20], [21]. Of the described cases, only three originated from the pituitary stalk [2], [6], [7]. Table 1 summarizes the features of these cases as

Conclusions

We report the fourth case of pituitary stalk HBL. The co-existence of VHL disease and a suprasellar highly enhancing mass likely suggested this diagnosis. A total tumor resection was achieved without recurrence 8 years later. Due to the apparent unsuccessful control of the posterior fossa HBLs with SRS in the present patient, we suggest to keep SRS as an alternative therapeutic strategy for multiple and surgically challenging HBLs.

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Cited by (21)

Supratentorial Hemangioblastoma in Adults: A Systematic Review and Comparison of Infratentorial and Spinal Cord Locations
2023, World Neurosurgery
Supratentorial hemangioblastomas are benign and highly vascularized neoplasms that appear most often in the spine and posterior cranial fossa. They can also be located in the supratentorial compartment of the brain. We conducted a systematic review of the literature to better understand the clinical insights of supratentorial hemangioblastoma in adults.
Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, the authors reviewed the English-language literature in the PubMed/MEDLINE database on supratentorial hemangioblastoma in adults, published in the past 40 years. We analyzed the differences between sporadic hemangioblastomas and hemangioblastomas associated with von Hippel-Lindau disease. In addition, we compared the characteristics of supratentorial hemangioblastomas with infratentorial and spinal cord locations.
We reviewed 92 articles, describing 157 supratentorial hemangioblastomas in a total cohort of 148 adult patients. Most articles reporting supratentorial hemangioblastomas were published in the United States.
Supratentorial hemangioblastomas occur more frequently in women than men. The median age at the time of diagnosis was 44.48 years. The sellar/parasellar region was the most commonly tumor location, while the most common morphological type was the solid type. Almost 80% of hemangioblastomas were completely resected; the outcome was favorable in more than 3 fourth of patients.
Given their rarity, the literature on supratentorial hemangioblastomas is limited and based mostly on case reports. Complete surgical resection is the first choice of treatment and is associated with a good treatment outcome.
Suprasellar Hemangioblastoma with Reversible Edema-Like Change Along the Optic Tract: A Case Report and Literature Review
2018, World Neurosurgery
Citation Excerpt :
Hemangioblastomas originating from the pituitary region are rare. To date, with the inclusion of autopsy cases, only 8 sellar and 33 suprasellar hemangioblastoma cases have been reported (Table 1).1,4-32 Regarding suprasellar hemangioblastomas, many previous reports noted the pituitary stalk as the origin site, whereas other reports did not clarify the origin due to huge tumor size; the origin of these cases would presumably be the pituitary stalk.
Hemangioblastomas usually occur in the cerebellum and are often a component of von Hippel-Lindau disease. Edema-like changes along the optic tract are commonly observed in association with tumors in the sellar and suprasellar regions, such as craniopharyngiomas and pituitary adenomas. Here, we describe a case of sporadic suprasellar hemangioblastoma with edema-like changes along the optic tract that completely resolved after surgery.
The patient was a 28-year-old woman with failing vision and a bitemporal hemianopia. Magnetic resonance imaging revealed a tumor accompanied by a 10-mm cyst. Fluid-attenuated inversion recovery axial magnetic resonance imaging revealed a hyperintense region along the optic tract. Other regions of the central nervous system were normal. Endoscopic transsphenoidal surgery was performed, and the histopathologic findings confirmed hemangioblastoma. Six months after surgery, the edema-like change along the optic tract had disappeared. The patient is doing well without pituitary insufficiency, pituitary hormone medications, or signs of tumor recurrence. In addition, the genetic test was negative for von Hippel-Lindau mutations.
When examining a patient with suprasellar tumor, clinicians must consider the possibility of hemangioblastoma. Adequate treatment can improve the edematous change and visual disturbance.
Surgical approach for suprasellar hemangioblastomas preserving the pituitary stalk: Review of the literature and report of a further case
2018, Clinical Neurology and Neurosurgery
Citation Excerpt :
Preoperative arteriography with embolization of feeding vessels could be useful to reduce the risk of suprasellar HBL bleeding during tumour dissection. Fomekong et al. reported the first detailed case of complete resection of a suprasellar HBL through a left frontotemporal craniotomy in a 51-year-old woman with VHL disease [19]. The procedure was performed through a left frontopterional craniotomy.
Hemangioblastomas (HBLs) are challenging vascular tumours with rare suprasellar location Surgery is recommended in patients with visual impairment, endocrine disorders or hydrocephalus. Surgical removal of pituitary stalk HBLs is challenging due to their location and high vascularity.
Our narrative review was guided by the question: "what is the more suitable surgical approach to excise a suprasellar HBL?". Pertinent English literature was scrutinized from database inception to October 2016.
Eighteen articles matched our selection criteria. Among the surgically treated patients, 4 were treated through a trans-sphenoidal (TS) approach, 13 through a transcranial approach (2 of them after TS failure). Five other cases were treated with radiotherapy (adjuvant in 3 cases) and 9 patients were managed with simple observation and/or medical treatment. We add a case of complete suprasellar HBL resection through an orbito-zygomatic (OZ) craniotomy with extradural anterior clinoidectomy (EAC).
To achieve satisfactory oncologic results with acceptable morbidity for symptomatic suprasellar HBLs, complete tumour removal with pituitary stalk sparing should be attempted through an OZ craniotomy with EAC that provides adequate exposure of the tumour, its vascular supply and the adjacent neural structures.
Infundibular Hemangioblastoma in a Patient with Neurofibromatosis Type 1: Case Report and Review of the Literature
2016, World Neurosurgery
Citation Excerpt :
In our literature review, 15 patients were treated with surgical resection. One patient had monocular blindness caused by sectioning of the optic nerve,17 2 patients developed new permanent pan-hypopituitarism,8,11 and 1 experienced additional endocrinopathies.21 Two groups reported brisk bleeding while attempting surgical resection, which necessitated aborting the procedure.3,20
Supratentorial hemangioblastomas are rare tumors, most commonly occurring in the sellar/suprasellar region, cerebrum, and ventricle. They are generally found in patients with von Hippel Lindau disease but have infrequently been reported in patients without this syndrome.
A 35-year-old woman with a history of neurofibromatosis type 1 presented to our care with visual loss and headaches. Magnetic resonance imaging of the brain demonstrated an 8-mm cystic, contrast-enhancing lesion abutting the optic chiasm and optic tracts. The patient's endocrine profile was unremarkable. The tumor was resected using an endoscopic expanded transsphenoidal approach. Pathologic evaluation was consistent with hemangioblastoma. Postoperatively, the patient experienced an improvement in her visual symptoms.
Hemangioblastoma should be included in the differential of sellar/suprasellar mass lesions, particularly in patients with von Hippel Lindau disease. Small suprasellar lesions may be safely and effectively removed using an expanded transsphenoidal approach.
Hemangioblastomas in the elderly: Epidemiology and clinical characteristics
2014, Journal of Clinical Neuroscience
Intracranial hemangioblastomas are benign vascular tumors. The peak age of incidence is between 20 to 50 years. Hemangioblastomas rarely occur in patients over the age of 65. To our knowledge there is no review of the prevalence and clinical features in an elderly population. We reviewed our 12 year experience with intracranial hemangioblastomas, and characterized the clinical features of hemangioblastomas in patients over the age of 65. We present a 72-year-old man with a cerebellar mass initially thought to be a metastasis as an illustrative case. We reviewed our pathology database and identified all patients with a histopathologically confirmed diagnosis of hemangioblastoma over the last 12 years in a large tertiary adult hospital; all patients were over the age of 18. Of all cases of hemangioblastoma in the last 12 years, six of 77 (7.7%) occurred in patients over the age of 65. All were cerebellar in location, and none were associated with von-Hippel Lindau disease. Hemangioblastomas are uncommon, but not rare, in patients over the age of 65, and should be included in the differential diagnosis of patients presenting with gait ataxia and a cerebellar lesion in this age group.
Preoperative Diagnosis of Suprasellar Hemangioblastoma with Four-Dimensional Computed Tomography Angiography: Case Report and Literature Review
2021, Indian Journal of Radiology and Imaging

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Case reportPituitary stalk hemangioblastoma: The fourth case report and review of the literature

Abstract

Introduction

Section snippets

Case report

Discussion

Conclusions

Surg Neurol

Clin Imaging

Surg Neurol

Surg Neurol

Clin Radiol

J Clin Neurosci

Int J Radiat Oncol Biol Phys

Surg Neurol

Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome

J Neurosurg

Surgical management of hemangioblastoma of the spinal cord: a report of 18 cases

Neurosurgery

The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease

J Neurosurg

Supratentorial haemangioblastoma. Case report

Acta Neurochir (Wien)

von Hippel-Lindau disease manifesting as a chiasmal syndrome

Surv Ophthalmol

Cerebral hemangioblastoma occurring in a patient with von Hippel-Lindau disease. Case report

J Neurosurg

Sellar-sphenoid sinus hemangioblastoma: case report

Surg Neurol

Gamma knife radiosurgery in 11 hemangioblastomas

J Neurosurg

Case report
Pituitary stalk hemangioblastoma: The fourth case report and review of the literature