AHBPS
Annals of Hepato-Biliary-Pancreatic Surgeryhttps://doi.org/10.14701/ahbps.2021.25.3.436
Adult pancreatoblastoma:
A rare malignant tumor of the pancreas
German Esteban Sánchez Morales
1, Hillary Lizarraga Payan
1, Roberto Andre Moguel Valladares
1, Ismael Domínguez Rosado
2, Carlos Chan
21
Department of General Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico,
2
Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Case Report
Pancreatoblastoma (PB) is a rare malignant tumor in adults. It has an overall incidence of 0.004 per 100,000 inhabitants. Its diagnosis with fine-needle aspiration (FNA) is difficult due to multiple differentiation lines present on PB that overlap with other tumors. A 76-year-old male patient presented with jaundice, weight loss of 10 kg in 6 months, and appetite loss. Abdominal computed tomog- raphy scan showed a tumor in the pancreas head. Transendoscopic ultrasound with FNA biopsy revealed a malignant epithelial neo- plasia compatible with PB with immunohistochemistry CK19 (+), P63(+), synaptophysin (–), and Ki67 50%. The patient underwent surgical resection and adjuvant chemotherapy. Currently, the patient is in follow-up with the latest imaging showing no evidence of disease at 32 months after his initial diagnosis. PB is an uncommon malignant neoplasia with an aggressive behavior. Its diagnostic and therapeutic protocols are unclear. Its preoperative diagnosis may be difficult since its imaging features and serological markers are non-specific. However, FNA may be useful in some situations. Surgical resection is currently the recommended treatment that is asso- ciated with the best long-term survival.
Key Words: Pancreatoblastoma; Pancreas surgery; Whipple; Pancreatoduodenectomy
pISSN: 2508-5778ㆍeISSN: 2508-5859
Ann Hepatobiliary Pancreat Surg 2021;25:436-439 https://doi.org/10.14701/ahbps.2021.25.3.436
INTRODUCTION
Pancreatoblastoma (PB) is a neoplasm of the pancreas that histologically appears similar to fetal pancreatic tissue. In the pediatric population, it accounts for 25% of childhood pancre- atic neoplasms. However, adult PB is an infrequent lesion with an incidence of 0.004 per 100,000 inhabitants. It represents less than 1% of all pancreatic neoplasms. To the best of our knowl- edge, there are less than thirty cases reported in the literature
[1]. PB has a preference for the head of the pancreas in almost 50% of cases. Its size ranges from 1.5 to 20 cm. Mass lesions can cause compressive symptoms, mainly abdominal pain [2].
CASE
A 76-year-old male presents with jaundice and weight loss of 10 kg in 6 months. His past medical history was significant for systemic arterial hypertension. Initial evaluation outside our institute showed alterations in liver function tests character- ized by cholestasis. An abdominal ultrasound revealed dilation of the extrahepatic bile duct up to 15 mm secondary to a 31 mm × 20 mm sized solid lesion in the pancreas head. The pa- tient underwent a transendoscopic ultrasound with fine-needle aspiration (FNA) biopsy that revealed a malignant epithelial neoplasia compatible with PB and immunohistochemistry CK19 (+), P63(+), chromogranin A (–), synaptophysin (–), ACE (–), and Ki67 50%. The patient was referred to our institute to continue workup and treatment. Laboratory results revealed a total bilirubin level of 40.22 mg/dL, direct bilirubin level of 25.30 mg/dL, indirect bilirubin level of 14.92 mg/dL, alanine transaminase level of 104 U/L, aspartate transaminase level of
Received: December 24, 2020, Revised: February 15, 2021, Accepted: February 15, 2021
Corresponding author: Carlos Chan
Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Cien- cias Médicas y Nutrición Salvador Zubirán, Avenida Vasco de Quiroga No.15, Colonia Belisario Domínguez Sección XVI, Delegación Tlalpan C.P.14080, Mexico City, Mexico
Tel: +52-5487-0900 (ext. 2133), Fax: +52-55-5487-0900 (ext. 2133), E-mail: chancarlos@me.com
ORCID: https://orcid.org/0000-0002-9082-9722
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