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复发性转移性甲状腺髓样癌:持续对生长抑素类似物治疗有反应的病例。

Recurrent Metastatic Medullary Thyroid Carcinoma: A Case of Sustained Response to Prolonged Treatment with Somatostatin Analogues.

机构信息

Department of Medical Oncology, Hospital General de Ciudad Real , Ciudad Real, Spain .

出版信息

Thyroid. 2017 Nov;27(11):1450-1455. doi: 10.1089/thy.2016.0540. Epub 2017 Oct 24.

DOI:10.1089/thy.2016.0540
PMID:28927353
Abstract

BACKGROUND

Medullary thyroid carcinoma (MTC) is a rare disease. Treatment options for recurrent disease are limited. Although somatostatin analogues might have a role as anticancer agents in MTC, the evidence is inconclusive.

PATIENT FINDINGS

A 64-year-old male was diagnosed with MTC in January 2010. Total thyroidectomy with neck dissection (stage IVA, pT2pN1bM0, R1) was performed, followed by adjuvant locoregional radiotherapy. Two years later, in January 2012, the patient developed recurrent metastatic disease, evidenced by elevated carcinoembryonic antigen (CEA) and calcitonin levels, and a positive uptake (Octreoscan) in the right adrenal gland and pancreatic head. A further computed tomography (CT) scan revealed metastases in the right adrenal gland, the duodenal bulb, and two pancreatic lesions, which were later confirmed as metastases by endoscopic ultrasound and cytology, and therefore salvage surgery was ruled out. Treatment with Somatuline Autogel (120 mg subcutaneously every 28 days) was initiated in September 2012, and 11 months later, calcitonin and CEA levels had both normalized, and a new CT scan showed that the metastatic lesions had disappeared or shrunk markedly. An Octreoscan performed in January 2014 and a repeat contrast-enhanced CT in February 2014 showed sustained tumor response. The patient remained in remission until February 2016, when a new Octreoscan revealed recurrent disease in the right adrenal gland, a nodule in the right upper pulmonary lobe, and nodal disease in the celiac trunk. CEA and calcitonin levels remained normal, although with a slight increase in calcitonin levels (47 pg/mL).

SUMMARY

The unusual case is described of a patient with metastatic MTC involving the adrenal gland, duodenum, and pancreas, who achieved a sustained response to somatostatin analogues after 11 months of treatment. The patient remained in remission for nearly 3.5 years from initiation of treatment with somatostatin analogues.

CONCLUSIONS

The case presented here is one of the few described in the literature in which long-term treatment with somatostatin analogues resulted in a sustained tumor response in a patient with metastatic recurrent MTC following curative-intent surgery. These findings suggest that prolonged treatment with somatostatin analogues may be beneficial in asymptomatic cases with a low tumor burden and a positive Octreoscan following recurrence. More data are needed to confirm these findings.

摘要

背景

甲状腺髓样癌(MTC)是一种罕见疾病。复发性疾病的治疗选择有限。虽然生长抑素类似物可能在 MTC 中具有抗癌作用,但证据尚不确定。

患者发现

一名 64 岁男性于 2010 年 1 月被诊断出患有 MTC。进行了全甲状腺切除术和颈部淋巴结清扫术(IV 期,pT2pN1bM0,R1),随后进行了辅助局部放疗。两年后,即 2012 年 1 月,患者出现复发性转移性疾病,表现为癌胚抗原(CEA)和降钙素水平升高,右侧肾上腺和胰头有阳性摄取(奥曲肽扫描)。进一步的计算机断层扫描(CT)显示右侧肾上腺、十二指肠球部和两个胰腺病变有转移,随后通过内镜超声和细胞学检查证实为转移,因此排除了挽救性手术。2012 年 9 月开始使用 Somatuline Autogel(每 28 天皮下注射 120mg)治疗,11 个月后,降钙素和 CEA 水平均恢复正常,新的 CT 扫描显示转移病灶已消失或明显缩小。2014 年 1 月进行奥曲肽扫描,2014 年 2 月进行增强对比 CT 扫描显示肿瘤持续缓解。患者一直处于缓解状态,直到 2016 年 2 月,新的奥曲肽扫描显示右侧肾上腺、右上肺叶结节和腹腔干淋巴结有复发疾病。CEA 和降钙素水平仍然正常,尽管降钙素水平略有升高(47pg/mL)。

总结

描述了一例罕见的转移性 MTC 病例,涉及肾上腺、十二指肠和胰腺,患者在接受生长抑素类似物治疗 11 个月后获得持续缓解。自开始使用生长抑素类似物治疗以来,患者已缓解近 3.5 年。

结论

本例是文献中描述的少数几例之一,长期使用生长抑素类似物治疗后,在根治性手术后复发性转移性 MTC 患者中获得持续肿瘤缓解。这些发现表明,对于复发性疾病且肿瘤负荷低、奥曲肽扫描阳性的无症状患者,延长生长抑素类似物治疗可能有益。需要更多的数据来证实这些发现。

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