[Ectopic hamartomatous thymoma: a clinicopathological and immunohistochemical study of two cases].
OBJECTIVE
To study the clinicopathological and immunohistochemical features of ectopic hamartomatous thymoma (EHT), and to discuss its histogenesis.


METHODS
The clinical and pathologic features of two EHT cases of were evaluated. Immunohistochemical study was performed by LSAB method using a panel of antibodies including AE1/AE3, CK5, CK7, CK8, CK20, EMA, vimentin, CD5, CD10, alpha-SMA, calponin, desmin, CD34, S-100 protein, CD57, GFAP, TTF-1 and CD99.


RESULTS
Both cases occurred in males aged 20 years and 40 years respectively. Each patient presented with a solitary mass, one located in the suprasternal fossa and the other in the left supraclavicular region for a period of 6 months and 2 months respectively. Grossly, the masses were well-circumscribed with spherical and ovoid appearance, measuring 5 cm and 3 cm in maximum diameter respectively. On cut section, they were gray-white in color and of soft consistency. Histologically, both tumors were composed of a mixture of spindle cells, epithelial cells and mature adipose tissue. The spindle cells element accounted 85% and 70% each in the two cases. They resembled fibroblasts in morphology and were arranged frequently in fascicular, woven or storiform patterns. Epithelial cells element represented nearly 10% in both cases. Most of the epithelial cells had a non-keratinization squamous appearance. They formed small solid islands and adamantinoma-like "nastomosing cords", or appeared as lining cells in large cystic spaces. In focal areas, glandular differentiation presented as small glands. A transition between the spindle cell and epithelium components could be also identified in some areas. Mature adipose tissue was irregularly distributed in the two tumors, about < 5% and 20% respectively. Immunohistochemically, the epithelial element expressed AE1/AE3, CK5, CK7, CK8 and EMA, whereas the spindle component expressed AE1/AE3, CK5, CK7, CK8, vimentin, CD10, CD34, alpha-SMA, MSA, and calponin. Both elements were negative for CK20, TTF-1, desmin, S-100 protein, CD57, GFAP and CD99.


CONCLUSIONS
EHT is a benign tumor that occurs predominantly in the lower neck region of young to middle-aged males. Immunohistochemical study revealed myoepithelial differentiation of the spindle cells, suggesting EHT is a mixed tumor composed of epithelial and myoepithelial cells. EHT possibly originates from the remnants of cervical sinus of His, and therefore, may be renamed as branchial anlage mixed tumor.
Wang
Jian
J
Department of Pathology, Cancer Hospital, Fudan University, Shanghai 200032, China.
Zhang
Ren-yuan
RY
D000328:Adult; D001457:Anion Exchange Protein 1, Erythrocyte / Q000378:metabolism; D002828:Choristoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D006222:Hamartoma; D006801:Humans; D053531:Keratins, Type II / Q000378:metabolism; D008206:Lymphatic Diseases / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D008297:Male; D018396:Mucin-1 / Q000378:metabolism; D013945:Thymoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D013953:Thymus Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D014746:Vimentin / Q000378:metabolism
D001457:Anion Exchange Protein 1, Erythrocyte; D053531:Keratins, Type II; D018396:Mucin-1; D014746:Vimentin