Abstract
Objectives
The aim of the study was to present a case of small cell carcinoma arising from the tonsil with metastasis to the external auditory canal (EAC) and associated facial nerve paralysis.
Methods
This study includes a case report and review of the literature.
Conclusions
Extrapulmonary small cell carcinoma from the head and neck is rarely described and carries a poor prognosis. It often presents with widely metastatic disease. To our knowledge, this is the first case report describing extrapulmonary small cell carcinoma from the tonsil with metastatic disease to the EAC. Irregular lesions in the EAC must be considered suspicious for metastasis in a patient with a history of cancer.
1
Case report
A 54-year-old male with a medical history of hepatitis C, diabetes, and hypertension presented to the Division of Otolaryngology at the VA Medical Center in Milwaukee (WI) with a several month history of a large left neck mass. He had 35-year history of smoking and heavy alcohol use. Computed tomography (CT) demonstrated left level II to V necrotic lymphadenopathy and left tonsillar enhancement concerning for malignancy ( Fig. 1 ). An excisional biopsy of a left level V lymph node was positive for metastatic small cell carcinoma.
Further workup was remarkable for periesophageal, gastrohepatic, and periceliac lymphadenopathy, 2 liver lesions consistent with metastasis, and no evidence of disease in the lungs. The patient was started on palliative chemotherapy for his metastatic extrapulmonary small cell carcinoma arising from his tonsil. For the next 9 months, the patient underwent multiple cycles of chemotherapy and radiation therapy to his left neck for local tumor control.
Ten months after his initial biopsy, an audiogram was performed for a 3-week history of decreased hearing in his left ear, demonstrating a 10 dB sensorineural loss on the left. Shortly thereafter he was seen in the VA emergency department for acute onset of left facial nerve paralysis. Evaluation included a CT scan of the head that was negative except for the presence of a new left mastoid effusion. He was treated with decadron and rocephin for a suspected facial nerve compression from acute mastoiditis. He failed to respond, and a magnetic resonance imaging (MRI) of the brain was performed. This was negative for intracranial metastasis but showed persistence of the left mastoid effusion.
The patient was then referred back to the otolaryngology clinic. On examination, the patient had complete left facial paralysis (House-Brackmann grade VI/VI). His left external auditory canal (EAC) had friable, granulation-like tissue anteriorly, just medial to the bony cartilaginous junction ( Fig. 2 ). His tympanic membrane appeared thickened and an air-bone gap was present on tuning fork examination. A repeat audiogram revealed a newly developed, left conductive hearing loss with an air-bone gap of 35 to 40 dB. A biopsy was obtained, and the histopathology was positive for small cell carcinoma. Imaging was reviewed with radiology, and there was no radiographic evidence of metastasis to the temporal bone.
The patient underwent additional palliative chemotherapy and died of his disease 3 months later.
2
Discussion
Extrapulmonary small cell carcinoma is rarely reported, comprising only 2.5% to 5% of all small cell carcinomas . Head and neck cases comprise an estimated 10% to 15% of extrapulmonary small cell carcinoma, with the larynx being the most commonly affected site, followed by the salivary glands and the sinonasal region . The tonsil has only been described as a site of origin in 11 cases in the literature; this represents the twelfth .
Small cell carcinoma of the head and neck has a poor prognosis, as it has a tendency to be locally aggressive and is prone to systemic metastasis. Treatment is generally systemically based at the time of diagnosis with a combination of chemotherapy and radiation therapy. Extensive workup is also recommended for these patients including CT or MRI of the head and neck, MRI of the brain, bronchoscopy with evaluation of sputum, positron emission tomographic scan, and possible bone marrow biopsy . Five-year survival rates for small cell carcinoma from head and neck and other extrapulmonary small cell carcinoma is reported to be less than 15% .
Metastatic disease to the temporal bone is rare with most common primaries arising from the breast, lung, and kidney . Metastases are most commonly found in the petrous portion (35%), the internal auditory canal (17%), the mastoid (8%), and EAC (8%) . Facial nerve paresis is the most common symptom followed by sudden and progressive hearing loss . It has been suggested that temporal bone metastases occur more commonly but are not diagnosed because symptoms and imaging mimic mastoiditis or chronic inflammation . Primary lung small cell carcinoma metastatic to the temporal bone has been described . This, however, is this is the first report of a small cell carcinoma arising from the tonsil with metastasis to the EAC/temporal bone.
Metastatic disease to the EAC is also rare with only with only 11 cases described in large case series . There have been case reports of isolated colonic adenocarcinoma, hepatocellular carcinoma, and rectal adenocarcinoma metastasizing to the EAC . To our knowledge, this is the first report of tonsil small cell carcinoma with metastasis to the EAC.
Our patient had a delayed diagnosis of his temporal bone metastasis. He presented with facial nerve paralysis and was treated for acute mastoiditis, as imaging was interpreted as negative for metastatic disease. Clinically, however, the patient had an abnormality of his EAC. Abnormalities of the EAC in patients with metastatic disease should be considered suspicious for metastasis.
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