Normalization of systemic arterial hypertension following removal of posterior fossa hemangioblastoma: a case report
© Saberi et al; licensee Cases Network Ltd. licensee BioMed Central Ltd. 2009
Received: 15 December 2008
Accepted: 19 February 2009
Published: 29 April 2009
The concept of compression of the rostral ventrolateral medulla as a cause for hypertension is gaining more and more interest. This report is about a 36-year-old male with a three years history of hypertension who presented with a posterior fossa mass suggestive of a hemangioblastoma. Laboratory and imaging studies ruled out the presence of von Hippel-Lindau disease and/or concomitant pheochromocytoma. Post-surgical blood pressure monitoring revealed a 40 mmHg decline in blood pressure. It could be hypothesized that alleviation the compressive effect of the tumour on the rostral ventrolateral medulla as proposed by previous studies could be a contributing factor.
Hemangioblastomas (Grade I in World Health Organization classification of brain tumors) constitute around 2-3% of all primary nervous system tumors and although they can occur at all sites from the cerebral cortex to peripheral nerves, are most commonly found in the cerebellar hemispheres [1, 2]. Presentations are related to mass effects on the neural structures and raised intracranial pressure. Various abnormalities such as retinal hemangioblastomas, renal and pancreatic cysts, renal carcinoma, pheochromocytoma, pancreatic islet cell tumors, endolymphatic sac tumors, and papillary cystadenoma of the epididymis are reported in 25% of hemangioblastomas as a part of von Hippel-Lindau disease (VHL) [1, 3]. However, systemic hypertension not associated with pheochromocytomas in sporadic cases of hemangioblastoma is a rarity. The intriguing association between arterial hypertension and posterior fossa tumors and its amelioration after total surgical removal has been touched in this case.
Secondary hypertension may be caused by a myriad of secretory disorders, including some tumors such as pheochromocytoma and aldostrone-secreting masses. However, not all these hypertensive states could be attributed to humoral factors. In 1979, Janneta and Gendell introduced the concept of neurogenic hypertension caused by pulsatile vascular compression of the left lateral medulla . Animal studies using various models have confirmed the presence of a subpial neuronal group (C-1) in the rostral ventrolateral medulla (RVLM) producing a transient pressor response when stimulated chemically, electrically or mechanically -. Likewise, there has been an abundance of evidence based upon experimental studies, postmortem anatomic dissections, and radiographic findings to portend that compression of the medulla elicits sympathetically mediated hypertension, even non-pulsatile and regardless of laterality . As a therapeutic intervention, many reports have been published in which the microvascular decompression of the RVLM is associated with a decline in blood pressure of hypertensive patients [4, 10]. Also, there have been two reports of resolution of hypertension after posterior fossa decompression for Chiari malformation type I in which the compressive effect of the herniated cerebellar tonsils on the cervicomedullary junction seemed to be the core pathophysiologic process [11, 12]. To date, only few cases of arterial hypertension have been published, in which the associated hypertension (HTN) resolved following tumor resection . In the first of such reports, Cameron and Doig reported two cases of cerebellar tumors (hemangioblastoma) with brainstem compression presenting with malignant hypertension . Hedderwick et al. reported a case of hemangioblastoma of the cervicomedullary junction, containing neuropeptide Y in which the associated hypertension abated after tumor resection . However, this marker was not positive in our patient. Approximately 25% of hemangioblastomas are associated with VHL . Our patient did not manifest with retinal and adrenal lesions neither had he high urinary cathecholamine excretion precluding the diagnosis of VHL and possible secreting function of the tumor. The older age, and the solitary tumor of cerebellum, along with negative family history for autosomal dominant VHL, all were in favor of the diagnosis of sporadic posterior fossa hemangioblastoma.
Mitigation of arterial hypertension after resection of the tumor in our patient, as well as negative laboratory and imaging studies for secondary causes for HTN supports the proposed mechanism of RVLM compression, as the pathogenesis of HTN.
There had been reports of posterior fossa tumors associated with arterial hypertension. The plausible mechanism of rostral ventrolateral medullary compression is gaining interest as a cause for hypertension. The present case is another clue advocating the proposed mechanism.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
List of abbreviations
von Hippel-Lindau disease
Magnetic resonance imaging
Posterior inferior cerebellar artery
Epithelial membrane antigen
Neuron Specific Enolase
Rostral ventrolateral medulla
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