Multidisciplinary treatment of advanced or recurrent solid pseudopapillary neoplasm of the pancreas: three case reports

Kiyonori Tanoue,Yuko Mataki,Yoichi Yamasaki,Hiroshi Kurahara,Yoshiaki Kita,Yuto Hozaka,Hideyuki Oi,Tetsuya Idichi,Takaaki Arigami,Takao Ohtsuka,Kosei Maemura,Yota Kawasaki,Akihiro Nakajo

doi:10.1186/s40792-022-01358-0

Kiyonori Tanoue, Yuko Mataki + Show 11 more

Open Access

https://doi.org/10.1186/s40792-022-01358-0

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Abstract

BackgroundSolid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor that predominantly affects young females. Prognosis is excellent; however, 10–15% of patients show metastasis at the time of surgery or develop tumor recurrence after pancreatectomy.Case presentationWe reviewed the clinical course of three patients with advanced or recurrent SPN and subsequently underwent multidisciplinary treatment at our institution between 2002 and 2019. The primary tumor was resected in all three patients, and metastases were also resected if indicated. Intensive combined therapy, including re-resection, chemotherapy, ablation, arterial chemoembolization, and radiation therapy, allowed all patients to survive for a long time. The literature review showed that resection seems to be more effective than other treatments for metastatic SPN.ConclusionsMultidisciplinary treatment, including resection, may improve the prognosis of patients with SPN with recurrence or metastasis.

Highlights

Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor that predominantly affects young females
Case presentation Patient No 1 was a 49-year-old female who underwent computed tomography (CT) scan for a pancreatic tumor detected by abdominal ultrasound during her annual check-up
The CT scan highlighted a pancreatic tumor in the pancreatic tail, which was 7 cm in diameter, and multiple liver metastases in subsegments 2, 4, and 7 (Fig. 1a)

Summary

Background

Solid pseudopapillary neoplasm (SPN) of the pancreas, known as Frantz’s tumor, is rare and usually carries a favorable prognosis, with a 5-year survival rate of up to 97% [1,2,3]. After 2 months of chemotherapy using GEM plus S-1, which led to a slight increase in the size of the metastases, we performed multiple partial resections of the liver to achieve R0 resection. This patient had survived for 50 months without any sign of re-recurrence to date (a total of 79 months after the initial diagnosis). The CT scan revealed a pancreatic tumor, measuring 10 cm in diameter with para-aortic lymph node (#16b1 latero) and abdominal wall metastases (Fig. 1e–g). Because of the increasing accumulation in the para-aortic lymph node (#16b1 latero) on fluorodeoxyglucose position emission tomography without any other lesion, we performed resection of the metastatic lymph nodes, and thereafter, she has survived for an additional 14 months without any sign of recurrence to date (total 183 months after initial diagnosis)

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