The sarcomatous guise of cervical ectopic hamartomatous thymoma.

Abstract

Ectopic hamartomatous thymoma is a rare benign neoplasm occurring in the deep soft tissues adjacent to the sternoclavicular joint. Although clinical presentation and diagnostic imaging can be consistent with a malignant lesion such as a sarcoma, recognition of pathologic features can readily exclude such a diagnosis. However, this remains a challenge caused by their unusual histologic features and diverse composition. Recognition of this tumor is, however, important, because it follows a benign clinical course, and conservative surgical excision is the therapy of choice. A literature review of all reports of ectopic hamartomatous thymoma was undertaken and compared with our indexed case. From this review, we identified 26 previous reports. Most patients were men, with a 4.5:1 predominance. There was a wide range of ages at presentation, from 26 to 79 years. All tumors were located in the lower neck, with the exception of one arising presternally. Treatment predominantly consisted of tumor resection, with no reported recurrences on follow-up. We conclude that ectopic hamartomatous thymoma is an extremely rare neoplasm usually presenting in the lower neck. Correct identification of this tumor is important, because it follows a benign course, and surgical excision is adequate therapy.