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Presented by William Westra, M.D. and prepared by Anil Parwani, M.D.,Ph.D
Case 1: 59 year-old man with long history of goiter and a recent history of expanding left neck mass.
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1. Question
Week 109: Case 1
59 year-old man with long history of goiter and a recent history of expanding left neck mass./images/10702case1fig1.jpg
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/images/10702case1fig5.jpgCorrect
Answer: Sclerosing mucoepidermoid carcinoma with eosinophilia
Histology: The background thyroid shows prominent chronic inflammation with germinal center formation, Hurthle cell change of the follicular epithelium, follicle atrophy, and fibrosis. Indeed, most of the thyroid has been entirely replaced by dense fibrohyaline stroma. In some areas the fibrotic stroma shows a conspicuous infiltration by eosinophils. Within this dense stromal fibrosis are cords and and individual cells that exhibit large and vesicular nuclei with prominent nucleoli. Focally, nests of cells demonstrate distinct squamoid differentiation, and rare individual cells have vacuolated cytoplasm suggestive of intracytoplasmic mucin. The lesion is non-encapsulated and circumscribed, but it extends beyond the thyroid. Moreover, perineural invasion is identified.
Discussion: Sclerosing mucoepidermoid carcinoma with eosinophilia is an obscure low-grade carcinoma of the thyroid gland. The tumor cells probably originate from a follicular epithelium that has undergone squamous metaplasia. Thus, it is not surprising that most of these tumors arise in a background of sclerosing Hashimoto’s thyroiditis. Histologically, these tumors are characterized by: 1) a background of dense fibrohyaline stroma, 2) a background inflammatory infiltrate comprised predominantly of eosinophiles, 3) irregular cords and nests of cells exhibiting vesicular nuclei and prominent nucleoli, and 4) focal areas of squamoid differentiation. Despite the name “mucoepidermoid” carcinoma, the mucinous component is often poorly developed or entirely absent. Importantly, the tumor does not demonstrate the high degree of cellular anaplasia and aggressive extension into the structures of the neck that characterize anaplastic thyroid carcinoma.
Although the clinical experience with these tumors is very limited, it is clear that they are well-behaved tumors. Occasionally, however, they may metastasize to regional lymph nodes, and there have been a few reports of distant metastatic spread to the lungs.
Incorrect
Answer: Sclerosing mucoepidermoid carcinoma with eosinophilia
Histology: The background thyroid shows prominent chronic inflammation with germinal center formation, Hurthle cell change of the follicular epithelium, follicle atrophy, and fibrosis. Indeed, most of the thyroid has been entirely replaced by dense fibrohyaline stroma. In some areas the fibrotic stroma shows a conspicuous infiltration by eosinophils. Within this dense stromal fibrosis are cords and and individual cells that exhibit large and vesicular nuclei with prominent nucleoli. Focally, nests of cells demonstrate distinct squamoid differentiation, and rare individual cells have vacuolated cytoplasm suggestive of intracytoplasmic mucin. The lesion is non-encapsulated and circumscribed, but it extends beyond the thyroid. Moreover, perineural invasion is identified.
Discussion: Sclerosing mucoepidermoid carcinoma with eosinophilia is an obscure low-grade carcinoma of the thyroid gland. The tumor cells probably originate from a follicular epithelium that has undergone squamous metaplasia. Thus, it is not surprising that most of these tumors arise in a background of sclerosing Hashimoto’s thyroiditis. Histologically, these tumors are characterized by: 1) a background of dense fibrohyaline stroma, 2) a background inflammatory infiltrate comprised predominantly of eosinophiles, 3) irregular cords and nests of cells exhibiting vesicular nuclei and prominent nucleoli, and 4) focal areas of squamoid differentiation. Despite the name “mucoepidermoid” carcinoma, the mucinous component is often poorly developed or entirely absent. Importantly, the tumor does not demonstrate the high degree of cellular anaplasia and aggressive extension into the structures of the neck that characterize anaplastic thyroid carcinoma.
Although the clinical experience with these tumors is very limited, it is clear that they are well-behaved tumors. Occasionally, however, they may metastasize to regional lymph nodes, and there have been a few reports of distant metastatic spread to the lungs.
