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首页> 美国卫生研究院文献>Journal of the Endocrine Society >Calcitonin Non-Secreting Mixed Medullary-Papillary Thyroid Carcinoma: Report of a Rare Entity and Therapeutic Implications
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Calcitonin Non-Secreting Mixed Medullary-Papillary Thyroid Carcinoma: Report of a Rare Entity and Therapeutic Implications

机译：降钙素非分泌混合髓质 - 乳头状甲状腺癌：罕见实体和治疗含义的报告

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Background: Medullary thyroid carcinoma (MTC) is a relatively rare and aggressive form of thyroid malignancy arising from parafollicular cells. The biochemical hallmark of MTC is elevated serum calcitonin (CTN), which aids initial diagnosis and disease surveillance. An atypical form of calcitonin non-secreting MTC has been previously described, with just over 50 cases published in the literature. This case report of a true mixed medullary-papillary thyroid carcinoma with a non-secretory medullary component is exceptionally rare. Clinical Case: A 67-year-old male presented with new-onset hoarseness and was found to have a 4 cm thyroid nodule replacing the right lobe with associated ipsilateral cord paralysis and ipsilateral cervical lymphadenopathy. The patient had no family history of endocrinopathies. A diagnosis of MTC was rendered at core-needle biopsy, based on the characteristic morphological findings and positive immunohistochemistry (IHC) for calcitonin (CTN) and CEA. Cervico-thoracic CT demonstrated tracheal and esophageal involvement by the tumor. The serum CEA was 8.7 pg/ml [0-5]; however, serum CTN was undetectable < 2 pg/ml [2-8.2]. The patient subsequently underwent a total thyroidectomy with bilateral central neck dissection and right lateral neck dissection. Excision of the tumor necessitated the dissection of involved esophageal muscle from mucosa and removal of four tracheal rings with part of the cricoid. The surgical specimen demonstrated a single 3.6 cm tumor in the right thyroid lobe composed of medullary and papillary intermixed neoplastic cells (mixed MTC-PTC) with predominant medullary component and gross extrathyroidal extension. Positive IHC for calcitonin, CEA, and thyroglobulin confirmed the diagnosis. Out of ninety resected lymph nodes in total, five nodes were involved with medullary carcinoma (with extranodal extension), and three had papillary carcinoma micrometastases. Postoperatively, the patient underwent adjuvant external beam radiation to the surgical bed. At four-month follow-up, CEA levels were 1.4 pg/ml [0-5], TSH 0.04 µIU/ml [0.35-4.9] and thyroglobulin (Tg) with Tg Ab levels were undetectable. The patient continues close surveillance. Conclusion: Calcitonin non-secreting MTC management is challenging due to the lack of a reliable biomarker that aids initial diagnosis, surveillance, and prognostic stratification. Whether a rare form of non-secretory MTC represents a more aggressive variant is yet to be determined. The prognosis of patients with mixed MTC-PTC tumors appears to be driven by the medullary component that prioritizes the optimal surgical approach and further management.
机译：背景：髓质甲状腺癌（MTC）是由Parafolular细胞产生的甲状腺恶性肿瘤相对罕见和激进的形式。 MTC的生化标志是血清降钙素（CTN）的升高，有助于初步诊断和疾病监测。先前已经描述了一种非典型的降钙素非分泌MTC，在文献中发表了超过50例。这种情况下具有非分泌髓质组分的真正混合髓质 - 乳头状甲状腺癌的病例报告特别罕见。临床案例：一个67岁的男性呈现出新的发病嘶嘶声，发现有4厘米的甲状腺结节，用相关的同侧脐带瘫和同侧颈椎淋巴结病。患者没有内分泌术的家族史。基于对Calcitonin（CTN）和CEA的特征形态学发现和阳性免疫组织化学和阳性免疫组织化学（IHC），在核心针活检中诊断MTC。 Cervico-thoracic CT展示了肿瘤的气管和食管受益。血清CEA为8.7 pg / ml [0-5];然而，血清CTN未检测到<2 pg / ml [2-8.2]。患者随后经历了双侧中央颈部解剖和右侧颈部分析的总甲状腺切除术。肿瘤的切除需要从粘膜中解剖涉及食管肌肉的解剖，并用一部分的红外移除四个气管环。外科标本在右甲状腺叶中展示了由髓质和乳头状混合的肿瘤细胞（混合MTC-PTC）组成的单个3.6cm肿瘤，其具有主要髓质组分和总脱滴虫延伸。 Calcitonin，CEA和甲状腺蛋白的阳性IHC证实了诊断。在总共九十切除淋巴结中，五个节点参与髓质癌（带外延伸），三个具有乳头状癌微转移酶。术后，患者接受佐剂外部光束辐射到手术床。在四个月的随访中，CEA水平为1.4 pg / ml [0-5]，TS0.04μIU/ mL [0.35-4.9]和甲状腺球蛋白（TG）未检测到。患者继续紧密监测。结论：降钙素非分泌MTC管理是挑战，因为缺乏可靠的生物标志物，有助于初步诊断，监测和预后分层。稀有形式的非分泌MTC代表更具侵略性的变体尚未确定。混合MTC-PTC肿瘤患者的预后似乎由髓质组分驱动，优先考虑最佳手术方法和进一步的管理。

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