Intrathoracic desmoid tumor: brief report and review of literature.

INTRODUCTION: The chest wall is the most common extraabdominal site for desmoid tumors. However, true intrathoracic desmoid tumors are exceedingly rare with most cases actually representing intrathoracic extension of chest wall tumors. A comprehensive review of the literature was undertaken to identify the prevalence and characteristics of true intrathoracic desmoid tumors.
METHODS: A case of surgical treatment of a true intrapleural desmoid tumor in a 42-year-old woman is reported. A comprehensive MEDLINE search was performed to identify all previously reported cases of intrathoracic desmoid tumor.
RESULTS: Twenty-two reported cases of true intrathoracic desmoid tumor were identified including the case presented in this report. Of these, 12 were intrapleural in origin. Patient age ranged from 5 to 66 years. Average tumor size was 9.2 cm. All patients underwent surgical resection with negative margins in two-thirds. Twenty-five percent of those patients developed local recurrence.
CONCLUSIONS: Unlike superficial chest wall desmoid tumors which will create a palpable mass, intrapleural tumors will not cause symptoms until they grow large enough to locally invade chest wall or surrounding structures or compress pulmonary parenchyma. Wide local excision (as is generally recommended for most desmoid tumors) is often impossible because of surrounding vascular and neural structures. Therefore, it may be advisable to consider adjuvant therapy, either radiation directed at known positive margins or antiestrogen therapy.