A Pain in the Neck FREE

A 68-year-old female smoker recently diagnosed with small cell lungcancer presents with a bulging area on her neck, behind her right ear(Figure 1). The patient denies headache or vomiting. Physical examination is remarkable for ataxia with chorea, particularly of her right upper extremity, which has remained relatively unchanged for the last few years. The patient has a history of hemangioblastoma resected 12 years earlier, which recurred after 3 years, at which time she had a second resection and radiation therapy, with poor follow-up since then.

Cerebellar hemangioblastoma

B. Perform MRI of the head and neck.

The key clinical points to remember in this case are that it is possible to have 2 primary tumors and that a cerebellar hemangioblastoma is a slow-growing tumor that may insidiously recur.

William of Occam, a 14th-century philosopher, emphasized the principle that “plurality must not be posited without necessity,” subsequently named Occam's razor.¹ A natural assumption is that an intracranial lesion identified in a patient with lung neoplasia must be a metastatic process derived from a primary pulmonary lesion. Nevertheless, there should be a clinical suspicion for “plurality,” especially in patients with a familial predisposition toward malignancies.

Cerebellar hemangioblastomas are rare benign tumors, although they have a tendency to recur in an insidious fashion several years after initial presentation, as in this case when the patient is facing a second malignancy. Hemangioblastomas can be solid, cystic, or both and can occur in association with von Hippel-Lindau disease or sporadically.² Hemangioblastomas associated with von Hippel-Lindau disease are usually multiple and progressive, whereas sporadic hemangioblastomas are generally solitary lesions.³ This patient did not have a mutation in the von Hippel-Lindau gene and her retinal examination findings were normal.

Small cell lung cancer is a systemic disease that usually presents with bulky involvement in the lung and mediastinum with subclinical (limited stage) or detectable (extensive stage) metastases. When compared with non–small cell lung cancer (the most common type of lung malignancy), small cell lung cancer is more aggressive with a faster doubling time and a higher growth fraction. Current guidelines recommend that staging should not delay therapy for more than 1 week after diagnosis. Treatment-naive small cell lung cancer is highly sensitive to chemotherapy and radiotherapy; nevertheless, most patients eventually succumb to recurrent disease. Staging includes brain imaging, which identifies central nervous system metastases in approximately 15% of patients with small cell lung cancer at diagnosis, even though as many as 30% of such patients remain asymptomatic.⁴ Local modalities alone almost always result in relapse. Subsequent prophylactic cranial radiation does not deliver an ideal outcome for small cell lung cancer, although it may be a better alternative than observation with subsequent palliative radiation at the time of relapse.⁵ Compared with other imaging methods, contrast-enhanced MRI of the head and neck would be most appropriate both for staging of newly diagnosed small cell lung cancer and assessing a cerebellar hemangioblastoma.

In this patient, MRI showed a large heterogeneously enhancing mass occupying most of the right cerebellar region (Figure 2A) with areas of internal necrosis, similar to previous imaging that the patient had when she was first diagnosed with cerebellar hemangioblastoma. The right portion of the occipital bone appears to have been resected, and the tumor extends outward beyond the confines of the skull (Figure 2B).

Of these 2 malignancies, it is paramount to recognize that small cell lung cancer should be treated first because it is most aggressive. The most appropriate approach would be to continue chemotherapy for small cell lung cancer and reevaluate the need for palliative surgical resection of cerebellar hemangioblastoma with serial imaging studies. This is one case in which Occam's razor does not apply because the simplest, and hence most probable, diagnosis was incorrect.

At 3 months after presentation, the patient had received 4 cycles of platinum-based chemotherapy for her lung cancer with partial response. Her course was complicated by venous thromboembolic disease and a marked decline in her performance status; hence, she opted for hospice care.