Solid pseudopapillary neoplasm (SPN) is a rare indolent pancreatic neoplasm that mainly occurs in female patients. It was first described by Frantz as a distinct disease in 1959. Generally, surgical resection is the first choice for SPN treatment, and post-surgical prognosis is excellent. However, reports have shown that in some male patients, SPN presents aggressive behavior and leads to inferior prognoses. In this case, we present a male patient with SPN who experienced local progressive disease after two surgical treatments. Then, apatinib plus S-1 chemotherapy was administered, which achieved satisfactory tumor control. This is the first report of a patient with aggressive SPN who was treated with apatinib plus S-1 chemotherapy.

Case report

Fig. 1 (a) Pancreatic tumor CT scan image before first surgery in 2013; (b) MRI image of recurrent pancreatic SPN before second surgery in 2017;(c) CT scan image for disease re-evaluation after second surgery in April 2017

Fig. 2 (a) Histologic study of tumor tissue demonstrated typical SPN pathological pattern; (b) Pathological histochemistry profiling of tumor

Fig. 3 (a) Pelvic MRI image of metastatic SPN before apatinib +getafur treatment in July 2017; (b) Pelvic MRI image of metastatic SPN after 1 month of apatinib + getafur regimen in December 2017; (c) Pelvic MRI image of metastatic SPN after 3 month of apatinib + getafur regimen in February 2018; (d)Pelvic MRI image of metastatic SPN after 5 month of apatinib + getafur regimen in April 2018

The patient was a 54-year-old male who incidentally found a solid mass located in the epigastric region during a routine health examination. He received his first surgery on November 24th, 2013. The surgical inspection showed that the solid mass was 15 cm in diameter and surrounded the tail of pancreas, pushing spleen posteriorly and the left colic flexure downward. No signs of spleen vasculature were seen. However, the tumor had severe adhesion with the retroperitoneum (Fig. 1a). Subsequent pathological inspections confirmed the diagnosis of solid pseudo papillary neoplasm (Fig. 2a). Histochemistry profiling demonstrated that the tumor possessed CD56+, CD10+,beta-catenin nuclear/ plasma+, PR +, Syn + partial, CgA–,PCK–, CK8/18–, EMA–, Ki-67LI 3% (Fig. 2b). The patient was discharged after surgery. In February 2017, the patient revisited our hospital after three days of abdominal pain.His MR scanning report showed multiple solid masses in his peritoneal cavity and retroperitoneum. Multiple circular long T2 signals were identified on the right lobe of his liver (Fig. 1b). For further treatment, the patient received secondary surgical resection of the pancreas body, spleen, omentum majus, left half transverse colon,left colic flexure, part of escend colon, and tumor mass.The surgical inspection indicated that the tumor was mainly located in the body of pancreas, closely attached to posterior wall of the stomach. The tumor was also found at the antrum of the spleen, sulcus of sigmoid colon, proximity of gall bladder, surface of right renal fascia, and omentum majus. The subsequent pathological diagnosis confirmed that the tumor was also a solid pseudo papillary neoplasm. Histochemistry indicated that the mass was PCK–, VIM+, CD10+, CYCLIN D1+, PR+,SYN+, BETA CATENIN+, KI67 5%–10%. After surgery,the disease was reevaluated by CT scan in April 2017,and no evidence of disease progression was found (Fig.1c). Four months later, another CT/MR scan detected a tumor at the bladder rectal space, right groin, and rectus abdominus (Fig. 3a). Considering the local progressive disease status, the patient was given two cycles of apatinib plus S-1 chemotherapy on November 5th, 2017.The MR scan on December 15th, 2017 showed significant reduction of the tumor in the proximity of the bladder rectal space (Fig. 3b). The patient’s status was considered PR afterwards, and the patient continued receiving six cycles of chemotherapy. A serial MR scanning reevaluation was performed every two months during the third-sixth cycles of chemotherapy (February and April 2018), and the reports demonstrated continuous size reduction of the metastatic tumor (Fig. 3c and Fig.3d). The tumor was well controlled; and throughout chemotherapy, there was no significant adverse patient is currently under his seventh and eights cycles of apatinib plus S-1 chemotherapy.

Discussion

Solid pseudo-papillary neoplasm (SPN) is a rare pancreatic tumor that accounts for only 1%–2% of pancreatic neoplasms[1]. Generally, clinical reports worldwide indicate that SPN is an indolent tumor with limited malignant potential. However, several aggressive SPN cases have been reported to present local recurrence and metastasis[2]. Current investigations on SPN pathogenesis have shown obscure findings, since SPN tends to occur in female patients (＞ 85% of total cases)[3], researchers have speculated that SPN could originate from genital ridges during organogenesis[4]. SPNs are usually large, soft solitary ovoid tumors that are well circumscribed by surround tissues[5]. Histologically, SPNs commonly consist of solid sheets of neoplastic cells that surround blood vessels and presents a pseudo-papillary appearance[5]. Through microscopic inspection, SPN cells always extend into the surrounding normal pancreatic parenchyma and sometimes exhibit calcification formation. However, vascular and perineural invasion are quite rare. Histochemical staining of SPN often show positivity for vimentin, CD10, neuron-specific enolase,progesterone receptors, CD56, alpha-1-antitrypsin, and alpha-1-antichymotrypsin, with the latter two stains showing patchy but intense positivity in areas of hyaline globules. A CT/MR scan is the main imaging diagnostic tool for patients with SPN. SPNs are typically hypoattenuated on unenhanced images and remain hypoattenuated during pancreatic and portal venous is commonly seen in the tumor with CT, MR scanning is quite suitable for identifying intra-tumor hemorrhages, which are a feature of SPN. For SPN treatment, surgical resection is the standard treatment for SPT and is usually associated with good prognosis, while a small portion of SPNs with aggressive behavior are associated with inferior prognosis. For this aggressive type of SPN, no suitable chemotherapeutic strategies have been proposed.

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