1 Department of Pathology & Immunology, Washington University School of Medicine , St. Louis, Missouri.
Thyroid. 2014 Feb;24(2):319-26. doi: 10.1089/thy.2013.0117. Epub 2013 Aug 24.
Anaplastic thyroid carcinoma (ATC) is a rare, aggressive malignancy with a median survival of five months. Multimodality treatment is associated with some improvement in survival, but patients are only infrequently curable. Although β-hCG secretion has been reported in many neoplasms, it has never been described in ATC. The objectives of this study were to report a case of β-hCG-secreting ATC and to study the expression and significance of β-hCG and PAX8 in an institutional cohort of ATC.
The sentinel case was characterized and then immunohistochemistry was performed for β-hCG and PAX8 on 30 ATC patients. Clinical follow-up was obtained by chart review.
The sentinel patient with β-hCG-secreting ATC had a dramatic response to chemotherapy and radiation. After surgical excision of residual disease, the patient developed a regional recurrence of differentiated thyroid carcinoma at 18 months. However, she is now, 30 months after initial therapy, with no evidence of disease and no detectable serum β-hCG or thyroglobulin. Five of the 30 (17%) total ATCs were positive for β-hCG and 18 (60%) for PAX8. Outcomes for the β-hCG-positive cases were not significantly different from those for negative ones. However, none of the other four β-hCG-positive ATC patients received treatment with either chemotherapy or radiation. Interestingly, PAX8 positivity correlated with statistically significantly better overall survival (p=0.019).
β-hCG is expressed in a minority of ATCs. Although only a single case in the study had diffuse immunohistochemical expression, the response it showed to aggressive multimodality therapy and the resulting favorable outcome suggest that β-hCG-positive ATC may be a unique tumor subtype, or possibly even a unique entity. PAX8 is a useful marker of ATC and may be helpful in the differential diagnosis with other malignant neoplasms.
间变性甲状腺癌(ATC)是一种罕见的侵袭性恶性肿瘤,中位生存期为五个月。多模态治疗可改善生存,但患者很少能治愈。虽然β-HCG 在许多肿瘤中均有分泌,但在 ATC 中从未有过描述。本研究的目的是报告一例β-HCG 分泌型 ATC,并研究β-HCG 和 PAX8 在 ATC 机构队列中的表达及意义。
对一个β-HCG 分泌型 ATC 患者进行特征描述,然后对 30 例 ATC 患者进行β-HCG 和 PAX8 的免疫组化检测。通过病历回顾获取临床随访资料。
该β-HCG 分泌型 ATC 患者经化疗和放疗后出现明显缓解。残留疾病经手术切除后,患者在 18 个月时出现分化型甲状腺癌的区域性复发。然而,在初始治疗后 30 个月,患者仍无疾病证据,血清β-HCG 和甲状腺球蛋白均无法检测到。30 例 ATC 中,有 5 例(17%)β-HCG 阳性,18 例(60%)PAX8 阳性。β-HCG 阳性病例的结局与阴性病例无显著差异。然而,其他四个β-HCG 阳性 ATC 患者均未接受化疗或放疗。有趣的是,PAX8 阳性与总生存率的显著改善相关(p=0.019)。
β-HCG 在少数 ATC 中表达。虽然研究中仅有一个病例存在弥漫性免疫组化表达,但它对积极的多模态治疗的反应以及由此产生的良好结果表明,β-HCG 阳性 ATC 可能是一种独特的肿瘤亚型,甚至可能是一种独特的实体。PAX8 是 ATC 的有用标志物,有助于与其他恶性肿瘤进行鉴别诊断。