Co-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease – A Case Report

Abstract Introduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel-Lindau (VHL) disease. Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuron-specific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration. Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.

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Interhemispheric transcallosal intervenous approach to a pineal region tumor

Neurosurgical Focus: Video ◽

10.3171/2021.4.focvid2120 ◽

2021 ◽

Vol 5 (1) ◽

pp. V11

Author(s):

Daniel A. Donoho ◽

Guillermo Aldave

Keyword(s):

Extent Of Resection ◽

Pineal Region ◽

Cerebral Veins ◽

Surgical Morbidity ◽

Pineal Tumors ◽

Pineal Region Tumor ◽

Pineal Region Tumors ◽

Transcallosal Approach ◽

Formidable Challenge ◽

The Right

Pineal region tumors represent a formidable challenge to the neurosurgeon. Choosing the right approach is key to optimizing the extent of resection and minimizing surgical morbidity. In this video, the authors show an interhemispheric transcallosal approach to a pineal region tumor in a 15-year-old boy. The advantage of this corridor over posterior approaches is that it provides a nice view of the tumor plane with the venous complex, especially while dissecting tumor from the anterior aspect of the internal cerebral veins on their vertical path. Thus, this approach represents a safe and effective alternative for selected pineal tumors. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2120.

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Papillary cystadenoma of right testis: Case report and literature review

Case Reports in Clinical Pathology ◽

10.5430/crcp.v4n2p8 ◽

2017 ◽

Vol 4 (2) ◽

pp. 8

Author(s):

Otobo O. Fidelis ◽

Ikpi Edet ◽

Enakirerhi Glen ◽

Isiwele M. Edoise ◽

Omotosho Ayodele ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Case Report ◽

Malignant Tumors ◽

Benign Neoplasm ◽

Adenomatoid Tumor ◽

Von Hippel Lindau ◽

Male Undergraduate ◽

History Of ◽

Papillary Cystadenoma ◽

Vhl Disease

Testicular cystadenoma is ranked the second commonest benign neoplasm. Other benign epididymal neoplasms include adenomatoid tumor (most common), leiomyoma, serous (nonpapillary) cystadenoma, cavernous hemangioma, and melanotic neuroectodermal tumor. Adenocarcinoma, mesothelioma, and metastatic renal cell carcinoma are malignant tumors that can affect the epididymis. A 24-year-old male undergraduate with a 3-month history of mildly tender right testicular swelling histologically diagnosed as papillary cystadenoma is presented. This case is presented from our locality as the first of its’ kind; and because it can be a possible manifestation of other diseases like von Hippel- Lindau (VHL) disease.

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Retinal Hemangioblastoma with Extraocular Extension: Report of Three Cases

Ocular Oncology and Pathology ◽

10.1159/000512173 ◽

2021 ◽

pp. 1-5

Author(s):

Namita Kumari ◽

Sima Das ◽

Anirban Bhaduri ◽

Arpan Gandhi

Keyword(s):

Retinal Detachment ◽

Vascular Lesion ◽

Exudative Retinal Detachment ◽

Von Hippel Lindau ◽

Young Males ◽

Unilateral Involvement ◽

Systemic Manifestations ◽

Vhl Disease ◽

Retinal Hemangioblastoma

Retinal hemangioblastoma (RH) is the earliest and most common clinical manifestation in Von Hippel-Lindau (VHL) disease. RH can also present in isolation without any evidence of VHL. Clinical course of RH can be stationary or progress to exudation and chronic retinal detachment requiring surgical intervention. We report 3 cases of aggressive RH with extraocular extension in young males causing painful blind eye requiring enucleation. Two of our cases were bilateral involvement and had systemic manifestations of VHL. The third patient had unilateral involvement with no systemic evidence of VHL. This manifestation of RH is rarely reported. Two of our patients with VHL had early manifestations of RH and had undergone multiple cryotherapy sessions as well as retinal detachment surgery for exudative retinal detachment. This differential should be considered in vascular lesion arising from intraocular structures especially in diagnosed patients of VHL. The cases also highlight the aggressive behaviour and long-term progression of RH in some patients despite early treatment.

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Pituitary stalk hemangioblastomas in von Hippel–Lindau disease

Journal of Neurosurgery ◽

10.3171/2008.4.17532 ◽

2009 ◽

Vol 110 (2) ◽

pp. 350-353 ◽

Cited By ~ 33

Author(s):

Russell R. Lonser ◽

John A. Butman ◽

Ruwan Kiringoda ◽

Debbie Song ◽

Edward H. Oldfield

Keyword(s):

Case Reports ◽

Pituitary Stalk ◽

Anatomical Location ◽

Clinical Effects ◽

Laboratory Findings ◽

Natural History Study ◽

Von Hippel Lindau ◽

Von Hippel Lindau Disease ◽

Vhl Disease

Object Pituitary stalk hemangioblastomas are rare, and information on them is limited to a small number of case reports. To gain insight into the incidence, clinical effects, and management of pituitary stalk hemangioblastomas, the authors analyzed a series of patients with von Hippel–Lindau (VHL) disease. Methods Patients with VHL disease who were enrolled in a prospective National Institutes of Health natural history study were included. Clinical, imaging, and laboratory findings were analyzed. Results Two hundred fifty patients were included in the study (120 male and 130 female patients). In 8 patients (3%), 8 pituitary stalk hemangioblastomas were identified on MR imaging. This anatomical location was the most common supratentorial site for these lesions; 29% of all supratentorial hemangioblastomas were found there. The mean (± standard deviation) pituitary stalk hemangioblastoma volume was 0.5 ± 0.9 cm3 (range 0.08–2.8 cm3). Results of endocrine laboratory profiles were normal in all patients. All patients remained asymptomatic and none required treatment during the follow-up period (mean duration 41.4 ± 14.4 months). Conclusions The pituitary stalk is the most common site for the development of supratentorial hemangioblastomas in patients with VHL disease. Pituitary stalk hemangioblastomas often remain asymptomatic and do not require treatment. These findings indicate that pituitary stalk hemangioblastomas in patients with VHL disease may be managed with observation and that surgery for them can be reserved until associated signs or symptoms occur.

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Preoperative Diagnosis of Suprasellar Hemangioblastoma with Four-Dimensional Computed Tomography Angiography: Case Report and Literature Review

Indian Journal of Radiology and Imaging ◽

10.1055/s-0041-1734335 ◽

2021 ◽

Vol 31 (02) ◽

pp. 499-509

Author(s):

Yi Tong ◽

Denis Sirhan ◽

Maria Cortes

Keyword(s):

Computed Tomography ◽

Case Report ◽

Literature Review ◽

Surgical Management ◽

Computed Tomography Angiography ◽

Diabetes Mellitus Type Ii ◽

Von Hippel Lindau ◽

First Case ◽

Imaging Characteristics ◽

Vhl Disease

Purpose Our case report presents the first case of suprasellar hemangioblastoma diagnosed preoperatively with dynamic computed tomography angiography (four-dimensional [4D] CTA) in a patient without Von Hippel-Lindau (VHL) disease. We illustrate the imaging characteristics of these exceedingly rare tumors and discuss the role of 4D CTA in confirming this diagnosis and guiding surgical management. Finally, we present a literature review of imaging findings, differential diagnosis, management, and prognosis. Case A 39-year-old woman known for diabetes mellitus type II and dyslipidemia presented with headache, bitemporal hemianopsia, and mild hyperprolactinemia. Initial diagnosis of suprasellar meningioma separate from pituitary gland was revised to definitive diagnosis of suprasellar hemangioblastoma after 4D CTA. Conclusion Suprasellar hemangioblastomas are extremely rare, often associated to VHL disease. They present as enhancing as suprasellar mass with prominent intra- and peritumoral vascular flow-voids on magnetic resonance imaging. 4D CTA confirms their vascular nature, demonstrates characteristic rapid shunting with feeding arteries, and enlarged draining veins, and is important in guiding surgical management.

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Sorafenib treatement for recurrent stage T1 bilateral renal cell carcinoma in patients with Von Hippel- Lindau disease: A case report and literature review

Canadian Urological Association Journal ◽

10.5489/cuaj.2863 ◽

2015 ◽

Vol 9 (9-10) ◽

pp. 651 ◽

Cited By ~ 4

Author(s):

Kyung Hwa Choi ◽

Young Dong Yu ◽

Moon Hyung Kang ◽

Dong Soo Park

Keyword(s):

Renal Cell Carcinoma ◽

Case Report ◽

Cell Carcinoma ◽

Surgical Resection ◽

Renal Cell ◽

Sorafenib Treatment ◽

Von Hippel Lindau ◽

First Case ◽

Vhl Disease ◽

Stage T1

Renal cell carcinoma (RCC) with Von Hippel-Lindau (VHL) syndrome is associated with multiple recurrences and a young age at diagnosis. Therefore the primary goal of treatment is to stabilize the disease, minimizing the surgical resection and preserving the renal function in the patients with VHL who have developing RCC nodules after initial treatment. This is the first case report of VHL disease, with long-term stable disease, treated with a half dose of sorafenib after surgical resection and radiofrequency ablation for multiple recurrent stage T1 masses. We discuss the efficacy and safety of low-dose sorafenib treatment and review RCC in a patient with VHL disease.

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Human Midbrain Auditory Evoked Potentials Do Not Differ Between Bursts and Suppressions of Cortex Activity in Propofol Anesthesia: Case Report

International Journal of Innovative Research in Medical Science ◽

10.23958/ijirms/vol05-i02/842 ◽

2020 ◽

Vol 5 (02) ◽

Author(s):

Anna O. Kantserova ◽

Lyubov B. Oknina ◽

Eugeny L. Masherov ◽

Vitaly V. Podlepich ◽

Maria I. Kamenetskaya ◽

...

Keyword(s):

Evoked Potentials ◽

General Anesthesia ◽

Auditory Evoked Potentials ◽

Obstructive Hydrocephalus ◽

Pineal Region ◽

Cerebral Aqueduct ◽

Pineal Region Tumor ◽

Transcallosal Approach ◽

Total Length ◽

Significant Difference

Propofol, the most widely administered anesthetic agent, is used for sedation and general anesthesia. During general anesthesia it can induce bursts and suppressions of cortex activity, which exact mechanism of generation has not been identified yet. The aim of study was to investigate the difference between midbrain auditory evoked potentials recorded during bursts and suppressions of cortex activity. These potentials were registered from the drainage-electrode implanted in the cerebral aqueduct of an adult patient with an obstructive hydrocephalus who had undergone pineal region tumor removal through anterior interhemispheric transcallosal approach. The cortex activity was divided into rare bursts of alpha activity (total length of 9 seconds) and prolonged suppressions (total length of 104 seconds). Midbrain auditory evoked potentials included long latency peaks with no statistically significant difference in their amplitudes and latencies between bursts and suppressions of cortex activity. The results suggest that human midbrain auditory evoked potentials do not differ between bursts and suppressions of cortex activity in propofol anesthesia. Therefore, for clear midbrain auditory evoked potentials cognitive but not the total electrical activity of the cortex should be suppressed.

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Cerebellar hemangioblastomas: A study of the immunoprofile of neoplastic stromal component

Vojnosanitetski pregled ◽

10.2298/vsp0403273t ◽

2004 ◽

Vol 61 (3) ◽

pp. 273-282

Author(s):

Desanka Tasic ◽

Dragan Dimov ◽

Milos Kostov ◽

Srbislav Ilic ◽

Dojcin Dojcinov ◽

...

Keyword(s):

Mast Cells ◽

Stromal Cells ◽

Protein Function ◽

Neuron Specific Enolase ◽

Factor Xiiia ◽

Von Hippel Lindau ◽

Stromal Component ◽

S 100 ◽

Vhl Protein ◽

Vhl Disease

Background. Central nervous system hemangioblastomas (HBs) are uncommon highly vascularized tumors that are predominantly found in the cerebellum. They occur sporadically or in association with von Hippel-Lindau (VHL) disease. HBs are of unknown histogenesis, and the origin of stromal cells is still a subject of debate. The aim of this study was to investigate the immunoprofile of neoplastic stromal component, and to determine whether the profile of the expression of immunomarkers used can contribute to the elucidation of the histogenesis of HBs. Methods. A series of eight cerebellar HBs were histochemically examined for the detection of mast cells and immunohistochemically for the expression of factor VIII-related antigen (FVIII-RAg), CD34, vimentin, factor XIIIa (FXIIIa), S-100 protein, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE) neurofilaments (NF), synaptophysin, chromogranin, and somatostatin. Results. Mast cells were present in all hemangioblastomas, and were particularly abundant in one tumor. Immunohistochemically, intense reactivity for vimentin and NSE in the stromal cells was constantly seen. Immunoreactivity with S-100 protein and FXIIIa was variable, but generally many HBs stromal cells were negative for these markers. However, stromal cells were uniformly negative for FVIII-RAg in all HBs investigated. They were negative for CD34 GFAP, NF, synaptophysin, chromogranin, as well as somatostatin. GFAP-positivity of the occasional stromal type cells, located only peripherally, was interpreted as "pseudopositivity". Conclusion. The immunoprofile of neoplastic stromal component in this study suggested a possible origin from undifferentiated multipotential mesenchymal cells. High expression of NSE (glycolytic and hypoxia-inducible enzyme) in the HBs stromal cells might be related to the loss of the VHL protein function.

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