Albuquerque Gisela Pereira Xavier, Ramos Aline Maria Pereira Cruz, Anaissi Ana Karyssa Mendes, Demachki Samia, Barra Williams Fernandes, Soares Helena Cecilia Branches, Costa Marta Solange Camarinha Ramos, Pantoja Amanda Carolina Rozario, Ishak Geraldo, Assumpção Paulo Pimentel
University Hospital João de Barros Barreto/Ebserh - Brazilian Company of Hospital Services, Mundurucus Street, N.4487, Guamá, Belém - PA, 66073-000, Belém, PA, Brazil.
Nursing School, Federal University of Pará, Augusto Corrêa Street, Belém, PA, CEP: Cep: 66075-110, Brazil.
Int J Surg Case Rep. 2020;71:66-69. doi: 10.1016/j.ijscr.2020.04.037. Epub 2020 May 11.
Frantz's Tumor or Solid Pseudopapillary Neoplasm (SPN) is rare with a solid-cystic pattern, and most common in young women.
This study based on guidelines for case reports (SCARE) reports a case of SPN in a teenager aged 13 years at the diagnosis time, attended at a teaching public hospital in Brazil, which evolved into liver metastases. Clinical, laboratory, therapeutic and imaging data were collected from the physical chart and analyzed in light of current publications on the topic. The first consultation occurred in January 2012, where weight loss, fever, vomiting, left-sided hypochondrium and epigastric pain were reported. Imaging exams evidenced an expansive heterogeneous process in the pancreatic tail; however, laboratory exams and tumor markers did not present alteration in relation to reference values. In March of 2012, she underwent caudal body pancreatectomy, splenectomy, segmental colectomy and colo-coloanastomosis as a function of the intraoperative findings. After 63 months, right-sided hepatectomy was performed to resect metastases. Currently, she is undergoing outpatient monitoring, without complaint or alterations in imaging and laboratory exams, totaling 100 months of global survival.
This is an interesting case report of a rare tumor, in so far as without any adjuvant chemotherapies, an 8-year long survival time could be achieved in this particular type of tumor despite initially large tumor expansion and later liver metastases.
Additional epidemiological studies, molecular and clinical trials are required to increase knowledge on SPN.
弗兰茨氏肿瘤或实性假乳头状瘤（SPN）罕见，呈实性囊性结构，多见于年轻女性。
本研究依据病例报告指南（SCARE）报告了一例13岁青少年的SPN病例，该病例在巴西一家公立教学医院确诊，后发生肝转移。从病历中收集了临床、实验室、治疗及影像学数据，并参照该主题的当前出版物进行分析。首次会诊于2012年1月进行，患者报告有体重减轻、发热、呕吐、左季肋部及上腹部疼痛。影像学检查显示胰尾有一个边界不清的异质性病变；然而，实验室检查及肿瘤标志物与参考值相比无异常。2012年3月，根据术中所见，患者接受了胰体尾切除术、脾切除术、节段性结肠切除术及结肠结肠吻合术。63个月后，为切除转移灶进行了右半肝切除术。目前，她正在门诊接受监测，无任何不适，影像学及实验室检查也无异常，总生存期达100个月。
这是一例罕见肿瘤的有趣病例报告，就该特殊类型肿瘤而言，尽管最初肿瘤广泛扩展且后来发生肝转移，但在未进行任何辅助化疗的情况下，仍实现了8年的生存期。
需要开展更多的流行病学研究、分子及临床试验，以增进对SPN的了解。
