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胰腺母细胞瘤,一种罕见的儿童肿瘤:病例报告

Pancreatoblastoma, a Rare Childhood Tumor: A Case Report.

作者信息

Argon Asuman, Çelik Ahmet, Öniz Haldun, Özok Geylani, Barbet Funda Yilmaz

机构信息

Department of Pathology, İzmir Bozyaka Training and Research Hospital, İZMİR, TURKEY.

出版信息

Turk Patoloji Derg. 2017;33(2):164-167. doi: 10.5146/tjpath.2014.01268.

Abstract

Pancreatoblastoma, rarely encountered in the literature, is a malignant exocrine tumor seen in the pancreas. A 5-year-old boy suffering from abdominal pain was sent to our institute for further examination and treatment. Clinical examination was normal but for a palpable abdominal tumor mass. Abdominal Doppler ultrasonography showed a mass with well-defined margins within the body of the pancreas. Laboratory tests, including lactic dehydrogenase, alpha-fetoprotein and cancer antigen 125 were abnormal. The tumor invading the splenic vein and transverse colon was removed totally. We observed a hypercellular tumor in histopathological examination. The tumor had epithelial acinar cells and squamoid morules (corpuscles) separated by stromal bands. Adjuvant chemotherapy was used after surgery. However, the patient died 14 months later. All data about pancreatoblastoma have to be collected in order to choose the treatment to elucidate the molecular pathogenesis of the tumor, to diagnose it early and to develop target-specific treatments.

摘要

胰腺母细胞瘤在文献中很少见,是一种发生于胰腺的恶性外分泌肿瘤。一名5岁腹痛男孩被送至我院进一步检查和治疗。临床检查除可触及腹部肿瘤肿块外均正常。腹部多普勒超声显示胰腺体内有一边界清晰的肿块。包括乳酸脱氢酶、甲胎蛋白和癌抗原125在内的实验室检查均异常。侵犯脾静脉和横结肠的肿瘤被完全切除。我们在组织病理学检查中观察到一个细胞增多的肿瘤。肿瘤有上皮腺泡细胞和被间质带分隔的鳞状小体(小球)。术后采用辅助化疗。然而,患者在14个月后死亡。必须收集所有关于胰腺母细胞瘤的数据,以便选择治疗方法,阐明肿瘤的分子发病机制,早期诊断并开发针对靶点的治疗方法。

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