Tumors of the neck showing thymic or related branchial pouch differentiation: a unifying concept

A number of rare tumors occurring in the soft tissues of the neck and the thyroid gland, reported in the literature under a variety of designations, show complete to partial histologic resemblance to the fetal, mature, or involuted thymus and mediastinal thymomas. This family of tumors spans a range of histologic appearances and behaviors from completely benign lesions to metastasizing malignant tumors. After reviewing the previously reported and new cases, we have been able to delineate four reasonably well-defined clinicopathologic entities within this spectrum. On the benign end is "ectopic hamartomatous thymoma," which occurs in the soft tissues of the lower neck. It is characterized by spindle epithelial cells, solid or cystic epithelial islands, and adipose cells which intermingle haphazardly to impart a hamartomatous quality. In the middle of the spectrum are the ectopic cervical thymomas which are usually benign, but can sometimes be locally invasive and can exceptionally metastasize. They are histologically identical to mediastinal thymomas, and residual ectopic thymus is not uncommonly identifiable in the periphery of the tumor. On the malignant end are tumors we have designated as "spindle epithelial tumor with thymus-like differentiation" (SETTLE) and "carcinoma showing thymus-like differentiation" (CASTLE). Tumors of the SETTLE type occur in the thyroid gland of young patients, and are highly cellular tumors comprised of compact bundles of long spindle epithelial cells which merge with tubulopapillary structures and/or mucinous glands. Tumors of the CASTLE type are histologically similar to thymic carcinoma of the lymphoepithelioma or squamous cell variety. We postulate that this family of tumors arises either from ectopic thymus or remnants of branchial pouches which retain the potential to differentiate along the thymic line.