Objectives: Adenomas/carcinoid tumors of the middle ear are very rare tumors of young to middle aged patients. The terms adenoma and carcinoid tumor of the middle ear can be used as synonyms, with the WHO favouring the term middle ear adenoma (MEA). These tumors usually present with unspecific clinical symptoms and a long case history. They are classified as benign tumors with only very few reported cases of regional metastasis after years of disease. According to recent literature, the clinical course is usually uncomplicated with complete surgical excision being adequate therapy.
Methods and patients: This study describes the clinical course and the diagnostic challenges in four cases of this rare tumor entity.
Results: The selected patients (2 males, 2 females, 25-38 years old) showed very similar clinical findings with decreased hearing acuity, tinnitus and sometimes pain. After the primary surgical excision up to 10 further operations were necessary, this being in contrast to the usual clinical course as described in the literature. In two cases a tumor recurrence was documented with one case recurring six times. In this case adjuvant radiotherapy (70 Gy) was performed. The histological differential diagnosis can also be problematic; in one case with a highly atypical morphology it was impossible to arrive at a definite diagnosis during the analysis of a frozen section.
Conclusion: Adenomas of the middle ear can have a much more complicated clinical course than is suggested by the recent literature. The presented cases in this study and the analysis of previously published cases shows that the typical progression described by the current WHO-classification with unproblematic surgical management of the tumor and an uncomplicated further clinical course does not always correspond to reality. The main reason for this is the difficulty in obtaining a complete surgical excision. Therefore, a well-planned and comprehensive surgical management with a high frequency of follow-up examinations should be chosen. In complicated individual cases adjuvant radiotherapy can be helpful.