Solid pseudopapillary tumor of the pancreas (Frantz’s tumor): two case reports and a review of the literature

Abstract

IntroductionSolid pseudopapillary tumor of the pancreas is extremely rare in children; it usually occurs in young women between 18 and 35 years of age. It comprises less than 3 % of pancreatic tumors. It is of low malignancy; however, it may be locally aggressive. Surgical resection is the treatment of choice and its prognosis is excellent.Case presentationTwo Caucasian girls, 15 and 12 years of age were diagnosed with tumor of the pancreas. The first patient had severe abdominal pain. In the second case the tumor was asymptomatic, detected incidentally during ultrasound. Computed tomography confirmed pancreatic mass. In the first case, apart from the tumor located in the head and the body of her pancreas, focal change in her right kidney was found, which was an indication to biopsy that confirmed solid pseudopapillary tumor. In the second patient the tumor was located in the body of her pancreas, with portal vein occlusion and well-developed collateral circulation. In the first patient a pancreatoduodenectomy (Traverso-Longmire) was performed; there was no mass in her right kidney. In the second case, distal pancreatectomy and splenectomy were performed. In both cases histopathology revealed solid pseudopapillary tumor resected radically. Our first patient’s postoperative course was uneventful. In the second case, her postoperative course was complicated by necrosis of the remaining pancreatic head that needed pancreatoduodenectomy. Follow-up at 28 and 26 months revealed no evidence of tumor recurrence or metastases on magnetic resonance imaging.ConclusionsTypical radiological appearance of solid pseudopapillary tumor is an indication for surgery. The treatment of choice is tumor resection with sparing of pancreatic tissue. In one of our two cases we performed a preoperative biopsy because of an uncharacteristic mass in her right kidney. In our second patient, necrosis of her spared pancreatic head meant that we could not preserve pancreatic tissue. Our whole diagnostic process, treatment and possible complications analysis should be of interest and noteworthy not only to surgeons as the treatment of choice is radical resection, but also to pediatric oncologists because of differentiation from other pancreatic tumors in children.

Tumor de Frantz: desafios diagnósticos e terapêuticos: relato de caso / Frantz’s tumor: diagnostic and therapeutic challenges: case report

“…O tumor de Franz localiza-se mais tipicamente em corpo e cauda do pâncreas, podendo atingir também cabeça (6) . Além disso, não é comum que os pacientes apresentem alterações de função pancreática endócrina ou exócrina (3) . Por fim, a ecoedoscopia além de confirmar esses aspectos anatômicos pode fornecer fragmentos para análise anatomopatológica e confirmação do diagnóstico.…”

“…Trata-se de uma doença rara, com baixo potencial de malignidade, que acomete preferencialmente mulheres jovens e apresenta baixa prevalência de registros na literatura (2) . Até 2014 apenas 900 casos bem detalhados dessa malignidade tinham sido descritos (3) e, portanto, o presente artigo relata um caso de Tumor de Frantz diagnosticado na Santa Casa de São Paulo, sua evolução e complicações pós cirúrgicas.…”

Frantz tumor – a rare cause of abdominal pain in children – case report

“…A solid pseudopapillary neoplasm is a rare tumor of unclear etiology and of 1-2% of exocrine pancreatic neoplasms. It was first described in 1959 by the pathologist Virginia Kneeland Frantz and is often called "Frantz tumor" [1,2,6,7]. Sometimes SPN is also called Hamoudi tumor in honor of the pathologist who in 1970 described the histology of the mentioned tumor.…”

“…SPN is generally benign, slow-growing and less aggressive than that of many other pancreatic tumors, and its prognosis is better [12], although very rare forms of malignancy have been reported [13,14]. Then these tumors were more frequently observed in men [6]. Differential diagnosis includes a variety of pancreatic tumors including the non-functioning islet tumour, pancreatoblastoma, acinar cell cancer, mucinous cystic neoplasm, serous cystadenomas, lymphoma pseudocyst, and non-pancreatic tumor, such as pelvic origin lesions extending into the abdomen, including ovarian cysts and teratoma.…”

Solid pseudopapillary neoplasm–Case report of a rare pancreatic tumor

“…Most patients are female within the second or third decade of life with only a small minority concerning children [1], [5], [6].…”