Nunes Gonçalo, Coelho Hélder, Patita Marta, Barosa Rita, Pinto Marques Pedro, Roque Ramos Lídia, Brito Maria José, Tomaz Aurora, Fonseca Jorge
Gastroenterology Department, Hospital Garcia de Orta, Almada, Portugal.
Pathology Department, Hospital Garcia de Orta, Almada, Portugal.
Clin J Gastroenterol. 2018 Apr;11(2):161-166. doi: 10.1007/s12328-017-0812-6. Epub 2017 Dec 28.
The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin 11.7 g/dl), abnormal liver tests (total bilirubin 1.4 g/dl, aspartate aminotransferase 70 U/l, alanine aminotransferase 37 U/l and alkaline phosphatase 520 U/l). Abdominal ultrasound (US) displayed a large heterogeneous liver with a segment IV 25 mm nodule. Magnetic resonance revealed a 4 cm pancreatic tail mass and several liver nodules consistent with metastasis. The patient underwent an endoscopic ultrasound (EUS) with fine needle aspiration (FNA) from the pancreatic mass and liver metastasis with cytological evaluation consistent with a pancreatoblastoma, later confirmed through a percutaneous US-guided liver biopsy. During the inpatient period, liver function deteriorated and acute kidney injury developed. Severe progressive cachexia was observed. The patient was discharged on renal replacement therapy and palliative care. Death occurred 3 months after diagnosis. Pancreatoblastoma is an uncommon pancreatic malignant epithelial cancer of the pancreas, typically occurring in the paediatric population. Adult pancreatoblastoma is extremely rare, with about 40 cases reported in the literature and generally presenting a more aggressive biologic and clinical behaviour. Surgical resection is the treatment of choice, but most cases are detected in advanced stages. This case underlines the ability to establish a pancreatoblastoma cytology-based diagnosis with EUS-FNA, and confirms the associated poor outcome.
作者描述了一名31岁男性,因进行性体重减轻、腹泻和肝脏肿大入院。实验室检查显示贫血(血红蛋白11.7 g/dl),肝功能异常(总胆红素1.4 g/dl,天冬氨酸转氨酶70 U/l,丙氨酸转氨酶37 U/l,碱性磷酸酶520 U/l)。腹部超声显示肝脏巨大且不均匀,IV段有一个25 mm的结节。磁共振成像显示胰尾有一个4 cm的肿块以及几个符合转移表现的肝脏结节。患者接受了内镜超声检查(EUS),并对胰腺肿块和肝脏转移灶进行了细针穿刺抽吸(FNA),细胞学评估结果符合胰母细胞瘤,随后经皮超声引导下肝脏活检得以证实。住院期间,肝功能恶化并出现急性肾损伤。观察到严重的进行性恶病质。患者出院时接受肾脏替代治疗和姑息治疗。诊断后3个月死亡。胰母细胞瘤是一种罕见的胰腺恶性上皮性肿瘤,通常发生于儿童人群。成人胰母细胞瘤极为罕见,文献报道约40例,一般具有更具侵袭性的生物学和临床行为。手术切除是首选治疗方法,但大多数病例在晚期才被发现。本病例强调了通过EUS-FNA建立基于细胞学的胰母细胞瘤诊断的能力,并证实了其相关的不良预后。