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胰腺实性假乳头状瘤伴妊娠期远处转移:诊断和治疗挑战。

Solid pseudopapillary neoplasm of pancreas with distant metastasis during pregnancy: a diagnostic and treatment challenge.

机构信息

Obstetrics and Gynaecology Department, Hospital Doutor Nélio Mendonça, Funchal, Portugal

Obstetrics and Gynaecology Department, Hospital Doutor Nélio Mendonça, Funchal, Portugal.

出版信息

BMJ Case Rep. 2020 Dec 9;13(12):e237309. doi: 10.1136/bcr-2020-237309.

Abstract

Solid pseudopapillary neoplasms (SPNs) are rare pancreatic cystic neoplasms occurring predominantly in young women and diagnosis is often a challenge. This report describes the case of a 23-year-old primigravida who presented with abnormally elevated liver blood tests at 24 weeks of gestation. Imaging studies were suggestive of SPN with metastatic liver disease. A multidisciplinary team approach decided on a preterm caesarean delivery of a healthy female child at 36 weeks of gestation. Subsequently, a CT-guided biopsy was performed, with confirmation of SPN in the anatomopathological study. Subpartial pancreatectomy, partial gastrectomy, cholecystectomy, total splenectomy and partial hepatectomy were successfully performed. A 3-month control CT scan and positron emission tomography-CT studies revealed disease recurrence with pulmonary and liver metastatic disease. The patient was started on a palliative chemotherapy protocol with good tolerance. To our knowledge this is the first case of a SPN described in pregnancy with distant metastasis and disseminated recurrence after surgical treatment.

摘要

实性假乳头状肿瘤(SPN)是一种罕见的胰腺囊性肿瘤,主要发生在年轻女性中,诊断通常具有挑战性。本报告描述了一例 23 岁初产妇的病例,她在妊娠 24 周时出现异常升高的肝血液检查。影像学研究提示存在 SPN 合并肝转移。多学科团队决定在妊娠 36 周行早产剖宫产术娩出一名健康女婴。随后,进行了 CT 引导下活检,解剖病理学研究证实为 SPN。成功进行了部分胰腺切除术、部分胃切除术、胆囊切除术、全脾切除术和部分肝切除术。3 个月后的 CT 扫描和正电子发射断层扫描-CT 研究显示疾病复发,伴有肺和肝转移。患者开始接受姑息性化疗方案,耐受性良好。据我们所知,这是首例妊娠合并 SPN 伴远处转移和手术治疗后播散复发的病例。

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Nat Rev Gastroenterol Hepatol. 2019 Nov;16(11):676-689. doi: 10.1038/s41575-019-0195-x. Epub 2019 Sep 16.
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Clinical update on the management of pseudopapillary tumor of pancreas.胰腺假乳头状瘤治疗的临床进展
World J Gastrointest Endosc. 2018 Sep 16;10(9):145-155. doi: 10.4253/wjge.v10.i9.145.
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Nonobstetric Surgery During Pregnancy.孕期非产科手术。
Obstet Gynecol. 2018 Aug;132(2):395-403. doi: 10.1097/AOG.0000000000002748.
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Management of a Pregnancy with a Solid Pseudopapillary Neoplasm of the Pancreas.胰腺实性假乳头状瘤合并妊娠的管理
Case Rep Obstet Gynecol. 2018 Apr 16;2018:5832341. doi: 10.1155/2018/5832341. eCollection 2018.
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Solid pseudopapillary tumor: a rare neoplasm of the pancreas.实性假乳头状瘤:一种罕见的胰腺肿瘤。
Gastroenterol Rep (Oxf). 2014 May;2(2):145-9. doi: 10.1093/gastro/gou006. Epub 2014 Feb 28.

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