Ruptured solid pseudopapillary tumor of pancreas presented as an acute abdomen: a case report

Abstract

Background

A solid pseudopapillary tumor of the pancreas is one of the rare pancreatic tumors. It usually occurs in young women. The common clinical presentation of this tumor is nonspecific abdominal pain and an abdominal mass. Since it has low malignancy potential, with complete surgical resection, the outcome is excellent.

Case presentation

We report the case of a 12-year-old Black African female who presented with hemorrhagic shock secondary to bleeding from a ruptured pancreatic head mass after she sustained blunt abdominal trauma. The patient underwent surgery, and a mass excision was done. She had a smooth postoperative course and was discharged on postoperative day 6. The biopsy revealed a solid pseudopapillary tumor.

Conclusion

Although a solid pseudopapillary tumor is a rare occurrence in the pediatric age group, it should be considered in female adolescents presenting with a pancreatic mass. Complete surgical resection suffices for the treatment.

Introduction

Solid pseudopapillary tumor (SPPT) is a rare tumor arising from the pancreas [1, 2]. It usually occurs in young female patients. Its presentation usually has nonspecific symptoms, including abdominal pain and a gradually increasing abdominal mass. Some patients can present with nausea, vomiting, and jaundice. Very rarely, spontaneous tumor rupture can be the initial patient presentation. The diagnosis of SPPT depends on clinical presentation, imaging, and pathologic evaluation. Imaging and histopathology play a great role. It is known for its benign nature with excellent outcomes after surgical intervention, but about 10% of these tumors are malignant, with possible local invasion and distal metastasis [3, 4].

Herein, we report a pediatric patient who presented with hemorrhagic shock from a bleeding solid pseudopapillary tumor after sustaining blunt abdominal trauma and was managed in a resource-limited area.

Case presentation

A 12-year-old Black African female presented to a primary hospital with a complaint of abdominal pain after she sustained blunt abdominal trauma 5 hours before presentation. Upon arrival at the hospital, she was in shock. After assessing her, it was determined that she was experiencing hemorrhagic shock due to intrabdominal bleeding caused by a solid organ injury. She was resuscitated with crystalloids, and an exploratory laparotomy was performed. The intraoperative finding was about 2 L of intraperitoneal hemorrhage along with a bleeding mass located between the duodenum (first and second part) and the pancreatic head. Omental buttressing was done to control the bleeding. However, due to a lack of blood products for transfusion and the difficulty of dealing with the mass, she was referred to our hospital. Before referral to our hospital, she stayed in the same hospital for about 24 hours because there were difficulties in getting intensive care unit (ICU) beds in the referral hospitals for possible postoperative ICU admission.

At the presentation to our hospital, about 36 hours after the trauma, she was tachycardic (pulse rate of 140), tachypneic (respiratory rate of 28), and febrile (temperature of 38.6 °C), but her blood pressure (blood pressure of 100/60 mmHg) was within the normal limit and she had adequate urine output. She was fully conscious. She had pale conjunctivae and palmar pallor. There was diffuse tenderness all over the abdomen with positive signs of fluid collection. Complete blood count was determined, and hemoglobin was 3 g/dL with a hematocrit of 9%. She was transfused with 2 units of packed red blood cells and taken to the operating room within 4 hours of admission to our hospital. Since the patient had a history of generalized peritonitis with positive signs of fluid collection on physical examination she did not undergo any imaging modalities. The intraoperative finding was about 300 mL of hemorrhagic fluid in the general peritoneum. There was a mass that measured about 6 cm × 8 cm with a firm and smooth surface, which was located between the duodenum and the pancreas. It was attached to the pancreatic head and adherent to the duodenal wall (first and second part) (Fig. 1A). The mass was enucleated (Fig. 1B). Since it was not this difficult to remove the mass, and the histology was also not known, it was decided to do just the enucleation. But during removal, there was an iatrogenic duodenal perforation, which was primarily repaired. Since the tissue was not fragile, it was repaired without patching. There was no injury to the pancreatic ducts. There was no local invasion, no lymphadenopathy, and no liver metastasis seen. The mass was sent for histopathologic evaluation, which revealed a solid pseudopancreatic tumor (Fig. 2). The patient was discharged on postoperative day 6 and had a smooth postoperative course at 6-month postoperative follow-up.

Fig. 1.

Intraoperative image showing the mass. (A) Arrow pointing to the pancreas: the mass is marked with a star and the duodenum is marked with a blue dot. (B) The mass after it was excised

Fig. 2.

Histopathology slide: sections show capsulated tissue consisting of solid sheets and pseudopapillary composed of mildly pleomorphic cells with round nuclei and fine chromatin and eosinophilic-to-clear cytoplasm (A). Hyaline globules (B). Rare mitotic activity (C)

Discussion

Solid pseudopapillary tumors of the pancreas are one of the rare tumors arising from the pancreas [1, 2]. It commonly arises from the tail and body of the pancreas, but in the pediatric age group, the pancreatic head is the most common site, as in our patient [5–7]. This tumor is predominantly seen in young females, with a peak age of 20–30 years, but can affect any age [7–10].

It is characterized by a benign nature. However, about 10% of solid pseudopapillary tumors are malignant, and there may be local invasion and distal metastasis to the liver and the lung at the time of diagnosis. Tumors with larger sizes and occurring at extreme ages, less than 10 years and above 50 years, are associated with a higher malignancy potential [4, 11–16].

Patients with solid pseudopapillary tumors usually present with nonspecific abdominal symptoms. The most common presentation is a gradually progressive abdominal mass and abdominal pain, but nausea, vomiting, and jaundice can be the initial presentation, rarely with spontaneous tumor rupture [7, 17–21]. Imaging plays a role in the diagnosis of SPPT. On a multiphasic CT scan, the tumor is characterized by cystic and solid components showing weak early arterial enhancement compared with normal parenchyma, and the solid component shows a gradual increase in enhancement in the portal-venous phase, exceeding the normal parenchyma [22, 23]. The correct diagnosis of SPPT depends on careful analysis of clinical features, imaging findings, and histopathologic evaluation [24–27].

Complete resection of the tumor is the main goal of the treatment of SPPT. Even patients with malignant SPPT have an excellent prognosis with complete tumor resection [12, 28–31]. The type of surgery performed will depend on the anatomic location of the tumor and the extent of the tumor. The possible procedures performed are tumor enucleation, partial pancreatectomy, or pancreaticoduodenectomy, depending on the site, extent, and nature of the tumor [7, 24, 26, 29, 32].

Conclusion

We report a rare case of a solid pseudopapillary tumor of the pancreas in a 12-year-old female child who presented with hemorrhagic shock from a ruptured solid pseudopapillary tumor following blunt abdominal trauma. Even though pancreatic tumors are uncommon in pediatrics, the possibility of SPPT should be kept in mind for patients presenting with bleeding pancreatic masses.

Author contributions

All authors contributed in different aspects. MG and HA operated on the patient and also followed the patient. HD reviewed the histopathologic slide. GK wrote the case presentation and wrote the draft of the case report. TG wrote the final case report and is also following the patient. All authors read and approved the final manuscript.

Funding

Not applicable.

Availability of supporting data

All data and materials are available upon request to the Editor-in-Chief.

Declarations

Ethics approval and consent to participate

Ethical approval is held to be unnecessary by St. Paul’s Hospital Millennium Medical College Institutional Review Board, as this is a single, rare case encountered during clinical practice.

Consent for publication

Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

No authors have disclosed any conflicts of interest.