Abstract：
BACKGROUND: While primary tumor desmoplasia is a powerful biomarker of node metastases in sporadic medullary thyroid cancer (MTC), information for hereditary MTC is sparse.
METHODS: This proof-of-concept study, comprising 3 consecutive children with multiple endocrine neoplasia 2B, evaluated simultaneously the metastatic behavior of multiple primary thyroid tumors of disparate size and extent of desmoplasia within patients.
RESULTS: Altogether, MTC typically involved the ipsilateral central neck before spreading to the ipsilateral lateral and the contralateral neck. Medullary thyroid cancer in the upper thyroid lobe leaped the ipsilateral central neck to invade the ipsilateral lateral neck. Unlike the desmoplasia-positive 6-mm high-grade and 7-mm low-grade primary thyroid tumors, the desmoplasia-negative 8-, 11-, and 16-mm low-grade primary thyroid tumors did not spread to ipsilateral neck nodes. With extranodal growth, the extent of nodal desmoplasia was greater than with intranodal growth.
CONCLUSIONS: This proof-of-concept study suggests that primary tumor desmoplasia is an equally powerful biomarker of node metastasis in hereditary MTC.
METHODS: This proof-of-concept study, comprising 3 consecutive children with multiple endocrine neoplasia 2B, evaluated simultaneously the metastatic behavior of multiple primary thyroid tumors of disparate size and extent of desmoplasia within patients.
RESULTS: Altogether, MTC typically involved the ipsilateral central neck before spreading to the ipsilateral lateral and the contralateral neck. Medullary thyroid cancer in the upper thyroid lobe leaped the ipsilateral central neck to invade the ipsilateral lateral neck. Unlike the desmoplasia-positive 6-mm high-grade and 7-mm low-grade primary thyroid tumors, the desmoplasia-negative 8-, 11-, and 16-mm low-grade primary thyroid tumors did not spread to ipsilateral neck nodes. With extranodal growth, the extent of nodal desmoplasia was greater than with intranodal growth.
CONCLUSIONS: This proof-of-concept study suggests that primary tumor desmoplasia is an equally powerful biomarker of node metastasis in hereditary MTC.
摘要：
虽然原发性肿瘤组织增生是散发性甲状腺髓样癌（MTC）淋巴结转移的强大生物标志物，遗传性MTC的信息很少。这个概念验证研究,包括三个连续患有多发性内分泌肿瘤2B的儿童，同时评估患者体内不同大小和程度的多原发甲状腺肿瘤的转移行为。总之,MTC通常在扩散到同侧外侧和对侧颈部之前涉及同侧中央颈部。上叶的MTC跃过同侧中央颈，侵入同侧外侧颈。与组织增生阳性的6毫米高级别和7毫米低级别原发性甲状腺肿瘤不同，结缔组织增生阴性的8毫米，11毫米和16毫米低级别原发性甲状腺肿瘤没有扩散到同侧颈淋巴结。随着结外生长，结节增生的程度大于结节内生长。这项概念验证研究表明，原发性肿瘤组织增生是遗传性MTC淋巴结转移的同样强大的生物标志物。
