Solid Pseudopapillary Neoplasm of the Pancreatic Head in an 18-Year-Old Presenting with Chronic Intermittent Abdominal Pain: A Case Report

Abstract

Introduction

Solid pseudopapillary neoplasm (SPN) is an uncommon pancreatic epithelial tumor of low malignant potential, seen predominantly in adolescent and young adult females. Imaging typically shows a well-circumscribed heterogeneous mass; complete resection is usually curative.

Case Presentation

An 18-year-old woman presented with ∼1 year of intermittent colicky epigastric pain, vomiting, and poor oral intake, without jaundice, fever, or weight loss. Examination and routine laboratories, including liver enzymes and bilirubin, were unremarkable. Ultrasonography revealed a 4.3 × 4.2 × 6.0 cm heterogeneous lesion inferior to the pancreatic head, abutting the third part of the duodenum and contacting the superior mesenteric vein. Contrast-enhanced CT demonstrated a 4.3 × 5.1 × 4.9 cm low-attenuation, heterogeneously enhancing mass impressing the pancreatic head, compressing the third part of the duodenum, and closely related to the superior mesenteric vein; small para-aortic nodes (≤6 mm) were present. Ultrasound-guided biopsy suggested SPN. She underwent standard pancreatoduodenectomy. Gross pathology showed an encapsulated 5.5-cm tumor within the pancreatic head. Histology confirmed SPN with negative margins, no vascular or perineural invasion, and no nodal metastasis (0/1).

Discussion

The patient’s age, sex, and imaging features were characteristic of SPN. Head-site disease may produce duodenal compression without cholestasis. R0 resection is definitive; adverse features were absent, supporting an excellent prognosis, though structured surveillance is advisable.

Conclusion

SPN should be considered in young females with a heterogeneous pancreatic mass and nonspecific upper abdominal symptoms. Multimodal imaging, selective biopsy, and complete resection optimize outcomes.

Introduction

Pancreatic solid pseudopapillary tumor (SPT) is a rare neoplasm with low malignant potential, originally described by Virginia Frantz in 1959 as a papillary cystic tumor in a 2-year-old boy [1]. SPT accounts for 2–3% of pancreatic neoplasms and 0.9–2.7% of exocrine pancreatic tumors, occurring almost exclusively in young women (>82% of cases) with a mean age of 30 years [2, 3]. Despite its rarity, SPT is the most common pancreatic neoplasm in the pediatric population and is frequently located in the pancreatic body-tail region [4]. The clinical presentation is heterogeneous. Children may present with a palpable mass and/or abdominal pain, or be entirely asymptomatic with incidental radiological evidence of pancreatic expansion on imaging performed for unrelated indications; acute abdominal tumor rupture is rare [5]. These neoplasms are identifiable on multiple imaging modalities including ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) which can facilitate their differentiation from other pancreatic lesions [6]. For this tumor type, surgery remains the indicated treatment, and the prognosis is considered excellent [7]. Here we present an 18-year-old woman with a 1-year history of intermittent epigastric pain and vomiting; US and contrast-enhanced CT revealed a 4.3 × 5.1 × 4.9 cm heterogeneously enhancing, low-attenuation pancreatic head mass. She underwent pancreatoduodenectomy, and histopathology confirmed an SPT. The work has been reported in line with the CARE checklist criteria [8] (the CARE checklist is attached as online suppl. material, see https://doi.org/10.1159/000551633).

Case Presentation

An 18-year-old female school student, unmarried, nonsmoker, and non-alcohol user presented with a ∼1-year history of colicky epigastric pain occurring in recurrent attacks, unrelated to meals and accompanied by vomiting and intolerance of oral intake. There were no fever or chills, no change in urine or stool color, no weight loss, and no change in bowel habits. She had been treated conservatively by gastroenterology for approximately 1 year without improvement. Past history included allergic asthma managed with anti-asthma medications. There were no prior surgeries and no known drug allergies.

On examination, she was in good general condition without pallor or jaundice and with no palpable lymph nodes. The abdomen was symmetric with respiration, soft, and without generalized or focal guarding; Murphy’s sign was negative. No organomegaly was palpable. Percussion was tympanic, and bowel sounds were normal. Cardiovascular examination showed a regular sinus pulse with normal heart sounds and no murmurs. Neurological examination was within normal limits.

Initial laboratory investigations showed: WBC 11.5 × 10⁹/L, hemoglobin 13 g/dL, platelets 248 × 10⁹/L, sodium 137 mmol/L, potassium 3.3 mmol/L, urea 16 mg/dL, creatinine 0.6 mg/dL, ALT/AST 17/20 U/L, total bilirubin 0.3 mg/dL (direct bilirubin recorded in the chart), alkaline phosphatase 67 U/L, serum amylase 53 U/L, glucose 87 mg/dL, prothrombin activity 100%, and total protein 6.7 g/dL. Abdominal US demonstrated a heterogeneous isoechoic lesion with mild vascularity situated in the mesenteric fat inferior to the pancreatic head, abutting the third portion of the duodenum. The margins were well defined and mostly smooth with posterior-medial lobulation. The lesion contacted lateral aspects of the superior mesenteric vein and extended between it and the abdominal aorta. Its dimensions were 4.3 × 4.2 × 6.0 cm; a gastrointestinal stromal tumor was considered among the possibilities (other differentials not excluded).

Subsequent contrast-enhanced CT of the neck, chest, abdomen, and pelvis revealed spindle-shaped lymph nodes in the anterior cervical triangles, more prominent on the right along the jugular chains and behind the mandibular angle (short axis up to 11 mm), interpreted as reactive on imaging; the chest was unremarkable. In the abdomen, a low-attenuation retroperitoneal mass showing heterogeneous enhancement, more evident in the portal venous phase, was identified immediately inferior to the pancreatic head, impressing upon it with elevation of adjacent pancreatic parenchyma. The mass compressed the third portion of the duodenum, was contiguous with the superior mesenteric vein causing impression (infiltration not excluded), and had posterior-medial lobulation. The lesion measured approximately 4.3 × 5.1 × 4.9 cm and was favored to arise from the pancreatic head. Small para-aortic lymph nodes were noted near the lesion, the largest measuring 6 mm in short axis. Representative axial CT images are shown (Fig. 1a–c).

Fig. 1.

a–c Contrast-enhanced axial CT images of the abdomen demonstrating a low-attenuation, heterogeneously enhancing mass contiguous with the pancreatic head, compressing the third portion of the duodenum, and abutting the superior mesenteric vein.

An ultrasound-guided needle biopsy of the pancreatic head mass demonstrated a proliferation of small uniform cells arranged focally in papillary formations with degenerative stroma, findings suggestive of a solid pseudopapillary neoplasm; immunohistochemical studies were recommended for further evaluation. The patient underwent exploratory laparotomy through a midline incision above and around the umbilicus. Exploration revealed no ascites, no peritoneal implants, and no metastases. A mass was identified in the pancreatic head (Fig. 2). A standard pancreaticoduodenectomy (Whipple procedure) was performed with pancreaticojejunostomy, hepaticojejunostomy, and gastrojejunostomy. A Nélaton tube stent was placed, a gastrostomy was created for postoperative gastric decompression and to facilitate enteral access, and a cholecystectomy was performed. Two tubular drains were left in situ: one posterior to the pancreatic anastomosis and the second adjacent to the biliary anastomosis (Fig. 3).

Fig. 2.

Intraoperative photograph showing the pancreatic head mass during exploration.

Fig. 3.

Resected pancreaticoduodenectomy specimen with the encapsulated pancreatic head mass.

Gross pathology of the resection demonstrated an encapsulated, well-defined mass within the pancreatic head, greatest dimension 5.5 cm, located 2 cm from the pancreatic transection margin (Fig. 4a–c). The specimen included a segment of intestine; the duodenal mucosa was congested with a small polypoid area measuring 0.2 cm. Final histopathology confirmed a solid pseudopapillary epithelial neoplasm of the pancreas involving the pancreatic head. There was no vascular or perineural invasion. The pancreatic margin and peripheral surgical margins were free of tumor, the duodenal wall was uninvolved, and one examined lymph node was negative for metastasis (0/1).

Fig. 4.

a–c Solid pseudopapillary neoplasm of the pancreatic head (H&E). Low-intermediate power shows a well-circumscribed, encapsulated tumor with alternating solid sheets and pseudopapillary fronds around delicate fibrovascular cores, uniform cells with pale eosinophilic cytoplasm and oval nuclei, and foci of hemorrhagic/degenerative change. No lymphovascular or perineural invasion identified; resection margins negative (not shown).

Postoperatively, the patient had an uneventful recovery and was discharged on postoperative day 10. At 3-month follow-up, she remains asymptomatic with no radiological evidence of recurrence.

Discussion

SPNs are low-grade malignant tumors of poorly cohesive, uniform epithelial cells forming solid and pseudopapillary structures [9]. They are an unusual pancreatic carcinoma of unknown cause, comprising ∼0.17–2.7% of all pancreatic tumors [9]. SPNs predominantly affect young women (∼25 years) with a marked female predominance [10], and there are no established risk factors or associations with functional endocrine syndromes [10–15]. SPNs arise throughout the pancreas but slightly favor the tail; rare extra-pancreatic sites include the omentum, mesentery, retroperitoneum, ovary, stomach, and duodenum [11, 14]. Slow growth and frequent paucity of symptoms mean that many are large at diagnosis; exocrine and endocrine insufficiency has not been described. The mean size is ∼ 6.5 cm (range 0.5–30 cm) [15].

Malignant SPN (solid pseudopapillary carcinoma [SPC]) occurs in ∼15% of adults, and the WHO classifies these tumors as borderline malignant SPN or SPC [11]. Distinguishing SPN from other solid cellular pancreatic neoplasms can be challenging [16]. The differential spans serous microcystic adenoma, cystadenocarcinoma, mucinous cystic neoplasms, solid/cystic neuroendocrine tumors, cystic acinar cell carcinoma, teratoma, pancreatoblastoma, and congenital, inflammatory, or infectious cysts [17]. Features favoring SPC include angioinvasion, perineural invasion, and deep parenchymal infiltration; extensive necrosis, nuclear atypia, high mitotic activity, elevated Ki-67, and sarcomatoid change may also correlate with aggressive behavior [18, 19]. Distant metastases occur in ∼7.7%, with lymph node metastases in ∼1.6% [13]. In this case, there was no vascular or perineural invasion, and the single examined node was negative (0/1).

Clinical manifestations are nonspecific; 38.1% are asymptomatic [13]. Other presentations include abdominal mass, weight loss, jaundice, anorexia, fever, fatigue, abdominal discomfort, nausea, and vomiting; rarely, spontaneous or traumatic rupture causes hemoperitoneum [12, 13, 20–23]. Here, intermittent epigastric pain and vomiting over 1 year suggested duodenal compression rather than the cholestatic picture often anticipated for head-site lesions.

Endoscopic ultrasound-guided fine needle aspiration is an operator-dependent but well-tolerated, minimally invasive test for solid and cystic pancreatic neoplasms, with ∼85% sensitivity for malignant cytology and ∼98% specificity [24]. Macroscopically, lesions are well circumscribed with a thin fibrous capsule; microscopically, cystic zones are bordered by solid tumor with pseudopapillary architecture and evolving necrotic/hemorrhagic change [25]. Immunohistochemically, common markers include vimentin, α1-antitrypsin, neuron-specific enolase, CD56, and CD10, while chromogranin is consistently negative [16]. Genetically, SPN is characterized by activating β-catenin mutations that disrupt protein phosphorylation [24]. In our patient, gross and microscopic examination confirmed SPN with negative margins and no adverse histology.

Multiple imaging modalities support diagnosis US, contrast-enhanced CT, MRI, and EUS. On CT, SPNs are typically well-demarcated, heterogeneous masses with mixed solid-cystic components; enhancing solid areas are usually peripheral with centrally located cystic regions, and peripheral or central stippled calcifications may be present [26, 27]. MRI shows a well-defined mass with heterogeneous T1/T2 signal reflecting mixed composition; high T1 signal corresponds to hemorrhagic necrosis/debris, with variable T2 from hemoglobin breakdown products. The solid component may be iso- to hypointense on T1 and slightly hyperintense on T2 [5, 27]. Surgical resection is the treatment of choice for SPN. Operation depends on site and size; options include distal pancreatectomy (with/without splenectomy), spleen-preserving distal pancreatectomy, central pancreatectomy, total pancreatectomy, pancreaticoduodenectomy, and pylorus-preserving pancreaticoduodenectomy [12, 13]. Head-site lesions are typically managed with pancreaticoduodenectomy (often pylorus-preserving), whereas body/tail lesions are treated with distal pancreatectomy [28, 29]. Incomplete excision should be avoided due to risks of dissemination, pancreatic fistula, and higher recurrence [30]. Large series have consistently shown an excellent long-term prognosis for SPN after complete resection. Papavramidis et al. [12] reported 2- and 5-year overall survival rates of approximately 97% and 95%, with more than 95% of patients with disease confined to the pancreas being cured by complete surgical excision. Similarly, single-center series and recent systematic reviews document disease-free survival rates around 95–96% and recurrence rates of only 4–7% after radical/R0 resection [13, 30]. Reflecting this, the head-located tumor here underwent standard pancreaticoduodenectomy with pancreaticojejunostomy, hepaticojejunostomy, and gastrojejunostomy, achieving an R0 resection and favorable pathology.

Conclusion

We report an SPN of the pancreatic head in an 18-year-old woman characterized by a heterogeneous, low-attenuation mass compressing the third portion of the duodenum and histologically confirmed after pancreatoduodenectomy with negative margins and no adverse features. This case underscores the importance of considering SPN in young females with pancreatic masses lacking cholestasis, leveraging multimodal imaging and selective biopsy to guide definitive surgery. R0 resection remains the cornerstone of management and portends an excellent prognosis; nevertheless, a structured, long-term surveillance plan is advisable to identify uncommon recurrences or metastases.

Statement of Ethics

The Ethics Committee at Damascus University does not require ethical approval for reporting individual cases or case series. Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

This study was not supported by any sponsor or funder.

Author Contributions

Ahmad Bishr Nasra collected the patient’s clinical and imaging data, contributed to data analysis, and participated in drafting the initial version of the manuscript. Mohammad Alaa Aldakak assisted in data collection, performed the literature review, and contributed to writing and organizing the case presentation section. Nawwar Fallouh participated in the interpretation of diagnostic findings and contributed to preparing figures and figure legends. Nizar Alabdullah assisted in the histopathological analysis and contributed to the discussion of immunohistochemical findings. Manar Darwish contributed to reviewing radiologic data, assisted in data validation, and helped revise the manuscript for important intellectual content. MHD-Fadi Alshurbaji participated in clinical data verification and helped in reviewing the final version of the manuscript. Yahia Alabed Alkhamis supervised the overall case management and ensured accuracy of diagnostic and treatment details. Abdulrahman Hammadieh provided senior supervision throughout the study, reviewed the manuscript for scientific integrity, and performed the final critical revision prior to submission. All authors have read and approved the final version of the manuscript.

Funding Statement

This study was not supported by any sponsor or funder.

Data Availability Statement

All data generated or analyzed during this study are included in this article. Further inquiries can be directed to the corresponding author.