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Hemangioblastoma without von Hippel-Lindau disease

Tumors account for only 1% of cases in which the patient presents with dizziness. This patient turned out to be a member of that rare group.

Cara Innus, PA-C, MPAS; Joel Patterson, PA-C, MPAS

Cara Innus works in family practice at the Highgate Medical Group and is an assistant professor at Daemen College’s PA program, Amherst, New York. Joel Patterson works in cardiology at the Buffalo Medical Group and is an assistant professor at Daemen College’s PA program. The authors have indicated no relationships to disclose relating to the content of this article.

CASE

The patient is a 44-year-old man who presented to a primary care office for follow-up after a previously diagnosed middle ear infection with benign positional vertigo. Four weeks earlier, he had experienced occasional episodes of dizziness during which he felt like the room was spinning when he moved his head from side to side or extended his neck. At the same time, he had right frontal headaches, mild nasal congestion, and an earache in the right ear. A physical examination revealed an erythematous right tympanic membrane with a significant effusion, nasal turbinate edema, and an erythematous pharynx. The results of a neurologic examination were normal. Otitis media and sinusitis were diagnosed, a 10-day course of amoxicillin was prescribed, and the patient was advised to follow up if his symptoms did not improve. No additional medications were prescribed for the vertigo or headaches, as they were attributed to the infection.

At the follow-up visit, the patient reported that the right ear pain had resolved but that he had begun to experience constant, dull, generalized headaches, with intermittent sharp headaches in the occipital region that persisted for about 30 seconds at a time. These episodes were triggered by urinating or defecating. Three separate episodes of severe dizziness had occurred in the past 2 weeks, all of which were triggered by rapid head movement while seated. These episodes were associated with tunnel vision and a feeling of complete weakness in all four extremities, which caused him to slump over in his seat. The episodes were brief, lasting less than a minute each time. No loss of consciousness, incontinence, tonic-clonic activities, or sensory deficits occurred with these episodes. None of the episodes was witnessed.

The patient denied speech or cognitive changes, hearing loss, memory impairment, facial numbness, gait changes, fatigue, night sweats, mood changes, or weight loss. He denied any previous history of headaches or vertigo, structural heart disease, arrhythmia, or significant medical or surgical history. He did not smoke or use alcohol. He was married and worked as a carpenter. He denied any recent trauma or travel. The family history was significant for diabetes and stroke in the patient’s 65-year-old father. The patient did not recollect a family history of any neurologic diseases or brain tumors.

The patient was 6 ft tall and weighed 232 lb. Temperature was 97.5°F. BP and pulse remained constant when measured both supine and seated and were 154/106 mm Hg and 78 beats per minute, respectively. Respiratory rate was 14 breaths per minute and regular. The patient appeared well-developed, well-nourished, and in no acute distress. The head was normocephalic with no evidence of trauma. Tympanic membranes were pearly gray without effusions, showing resolution of the previously diagnosed infection. The nasal mucosa and pharynx were pink. Pupils were equal, round, and reactive to light and accommodation. Extraocular movements were intact without nystagmus. Fundoscopic examination revealed no abnormal vascular findings consistent with hypertension, diabetes, or papilledema. The neck was supple without lymphadenopathy or carotid bruits. The lungs were clear, and the cardiac examination revealed a regular rate and rhythm without murmurs, gallops, or rubs. The extremities were without clubbing or edema. Peripheral pulses were 2+. Neurologic examination showed cranial nerves 2 to 12 intact without focal deficits, and the results of the following tests were normal: Romberg, pronator drift, finger to nose, rapid alternating movements, sensation (both sharp and dull), bilateral Babinski, and Mini-Mental State Examination. Reflexes were 2+ throughout. The sensation of vertigo could not be reproduced by head or neck repositioning at the time of examination.

An ECG demonstrated normal sinus rhythm at 78 beats per minute with no acute changes. A random, nonfasting fingerstick test revealed a glucose level of 82 mg/dL and a hemoglobin level of 13.4 g/dL. The differential diagnosis for this patient included benign positional vertigo, new-onset seizure disorder, a brain lesion, labyrinthitis, and transient ischemic attack.

DISCUSSION

Because of the progression of symptoms over the past 4 weeks—including attacks of vertigo that resulted in weakness and a new pattern of headaches that were aggravated with urination or defecation—a same day neurologic consultation and MRI of the brain were arranged. The biggest concern at this visit was to rule out an intracranial lesion that could be increasing intracranial pressure and causing the symptoms. Urgent evaluation was warranted because if a rapidly growing tumor was present, it needed to be identified and treated quickly to prevent worsening symptoms and potential disability. The patient’s new-onset hypertension was also a concern, and although therapy was not initiated at this visit, monitoring was advised. Physical examination findings were unchanged at the neurologist’s office, with the exception of a lower BP of 148/79 mm Hg.

MRI with and without contrast revealed a 4.5-cm right cerebellar mass with edema and some developing mass effect (see Figure 1). The mass appeared to be benign and cystic in nature, and it could be easily identified on the MRI in multiple different views and on both T1 and T2 weighted images. Because the tumor appeared to be low grade, surgical resection was an option and the patient was promptly referred to a neurosurgeon at a local cancer institute. He underwent a right suboccipital craniectomy for resection of the lesion. Pathology reports identified the tumor as a benign hemangioblastoma. The patient made a complete recovery without surgical complications or lingering neurologic symptoms and was cleared to return to work 6 weeks later.

Hemangioblastomas are highly vascular tumors composed primarily of capillaries and stromal cells. The vast majority occur in the cerebellum, although a small percentage develop in the spinal cord.¹ Most hemangioblastomas are slow growing and located in the posterior fossa; they account for 10% of posterior fossa tumors.² Patients may present with a variety of symptoms, including ataxia, gait changes, and minor neurologic complaints, but persistent headache due to increased intracranial pressure is the hallmark of the disease. The median age at presentation is 42 years.

Hemangioblastomas can occur sporadically or as a feature of von Hippel-Lindau disease, an autosomal dominant disorder in which cerebellar and retinal hemangioblastomas, pancreatic cysts and carcinomas, renal masses, and pheochromocytomas develop. These masses cause focal symptoms by compressing neural structures, and they can produce acute hemorrhage, slow bleeding, or neoplastic complications. Although hemangioblastomas are fairly unusual, they have a 25% association with von Hippel-Lindau disease. Sporadic hemangioblastomas are typically solitary, and when multiple tumors are found, the clinician should suspect von Hippel-Lindau disease.¹ Patients with hemangioblastomas of the retina and the CNS commonly have associated renal cell carcinomas.³

The diagnostic workup in patients with hemangioblastoma should include a careful evaluation of family history, an eye examination to identify any retinal tumors, abdominal imaging, and urinalysis.¹ Patients such as the one in this case may need yearly CT of the abdomen to screen for renal cell tumors. If suspicion is high for von Hippel-Lindau disease, genetic counseling and testing are available.⁴

Comment Vertigo is precipitated by an acute asymmetry in the vestibular system. Nearly all patients can recall this sensation as the transient spinning dizziness which may give the illusion of motion, often interpreted as self-motion or as motion of the environment, noted immediately after turning around rapidly several times. In the primary care setting, 93% of patients presenting with dizziness will receive a diagnosis of benign paroxysmal positional vertigo, acute vestibular neuronitis, or Meniere’s disease.⁵

Pathologic vertigo arises secondary to dysfunction of the labyrinth, vestibular nerve, or central vestibular structures in the brainstem resulting in asymmetry within the vestibular system. Tumors, predominantly acoustic neuromas, account for only 1% of cases in which patients present with dizziness.⁶ Tumors are less likely to produce vertigo because they typically cause gradual rather than acute asymmetries in vestibular function.

If standard treatments such as antihistamines fail to resolve vertigo, tests such as audiometry, brain stem evoked audiometry, and electronystagmography can be utilized, although specific indications for these tests are somewhat unclear.⁷ Most experts agree that routine brain imaging of patients with vertigo is unnecessary unless a tumor or stroke is being considered as a possible diagnosis.⁸

Patients with and without symptoms of dizziness have been compared in large studies to see if the results of brain imaging differ between the two groups; routine MRI in the investigation of dizziness is not warranted because structural brain abnormalities occur in both groups at an equal rate.⁹ The entire clinical picture needs to be assessed thoroughly so that significant pathology is not missed. If dizziness is associated with neurologic signs such as cranial nerve palsies, nystagmus, instability of gait, or sensorineural hearing loss, 40% of those patients may have abnormalities seen on brain MRI.⁵ Brain MRI is far better than CT at detecting an etiology for vertigo with neurologic symptoms because a large portion of the pathology is located in the posterior fossa. The value of CT can be limited because of the artifact produced by the surrounding thick bone in the region.¹⁰

The patient in this case had not only vertigo but progressive headaches that worsened with Valsalva maneuver and episodes of acute weakness precipitated by position change. These additional symptoms were key indicators that rapid further evaluation was needed. Other significant signs and symptoms to remember when evaluating a patient with vertigo or headaches include seizures, vomiting, confusion, headaches that are worse in the morning, a new or different pattern of headache, headaches in those older than 40 years, headaches in someone with a risk of metastatic disease, or headaches resistant to pharmacologic treatment.¹¹ If patients with headaches or vertigo are evaluated for these warning symptoms at initial presentation, pathology is more likely to be detected earlier, and prompt and appropriate care is more likely to ensue.

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