gms | German Medical Science

Case Report: We report the case of a 60-year-old female patient who presented in our department with a left-sided complete facial paralysis. Dizziness, ear ache or hearing loss werenot reported. Otoscopically, an obturating, smooth tumor was seen in the left ear canal, while the remaining clinical examination was unremarkable. The findings of an angio-CT and a cranial-MRI (Figure 1 [Fig. 1]) were compatible with a glomus tumor of the temporal bone. Further diagnostic imaging revealed, however, the presence of multiple metastases in the ribs and sternum. Laboratory testing showed a monoclonal gammopathy in serum and light chains of type kappa in urine. Taking the above findings into consideration, the diagnosis of multiple myeloma was probable and was confirmed through a biopsy of both the ear canal tumor and bone marrow. The patient went through 3 cycles of chemotherapy with bendamustin and prednisolon. After a brief remission, the tumor was progressive again. Locally it exceeded the limits of the temporal bone and spread into the soft tissues of the neck (Figure 2 [Fig. 2]). The progression of the tumor was controlled with local radiotherapy and 2 cycles of VAD combination chemotherapy.

Discussion: Multiple myeloma belongs to the Non-Hodgkin's lymphomas and consists of a malignant clone of plasma cells. The malignant cells grow diffusely in the bone marrow and stimulate the osteoclasts causing osteolysis. Predilection sites of the osteolytic activity are bones, which contain red bone marrow, such as the vertebrae, the ribs, the skull and the pelvis. A multiple myeloma infiltrating the temporal bone and thereby causing a facial paralysis is a very rare event with only 5 reported cases in the literature [1], [2].

The most common causes of non-idiopathic facial paralysis are traumata, viral or bacterial infections and malignancies such as the cholesteatoma, the glomus tumor, the neurinoma of the facial nerve, the parotid gland tumors and the tumors, which metastasize in the temporal bone (breast, lungs, kidney, stomach, larynx) [3]. Although trauma and infection are relatively easy to diagnose, the differential diagnosis between a tumor-related and an idiopathic facial paralysis may prove difficult. In that case the following findings are suggestive of a tumor [4]:

1.

progression of the paresis for more than 3 weeks

2.

absence of improvement of the facial nerve function after 6 months

3.

recurrence of paresis

4.

pain in the ear

5.

neuropathy of the caudal cranial nerves

6.

presence of a tumor in the ear canal

Conclusion:

When confronted with a facial paralysis with untypical history and findings a thorough diagnostic evaluation must follow to rule out a tumor. The differential diagnosis must include common tumors such as the cholesteatoma and the parotid tumors or rare entities such as the multiple myeloma.