Solid Pseudopapillary Neoplasm of the Pancreas

Two cases

Historical note

This article updates a report of two pancreatic tumors diagnosed and treated at the University Hospital in Jamaica. The original report used the term “papillary cystic neoplasm of the pancreas.” The preferred modern term is solid pseudopapillary neoplasm of the pancreas (SPN), also historically called papillary cystic tumor, solid and cystic tumor, Frantz tumor, or Hamoudi tumor (Omiyale 2021, StatPearls 2024).

SPN is a rare low-grade malignant pancreatic neoplasm with excellent prognosis after complete surgical resection. Contemporary systematic reviews include large number of cases: a 2024 systematic review included 1,384 surgically treated patients, and a 2025 systematic review included 1,888 patients from 54 studies (Mazzarella et al. 2024, Pontrelli et al. 2025).

Abstract

Two cases of solid pseudopapillary neoplasm of the pancreas, historically termed papillary cystic neoplasm of the pancreas, diagnosed and treated at the University Hospital are presented. Both patients were young women with large solid and cystic tumors in the head of the pancreas and underwent proximal pancreaticoduodenectomy. Complete resection is the primary treatment and is associated with excellent long-term survival. Recurrent or metastatic disease is uncommon, and repeat resection or metastasectomy is considered when feasible; non-surgical therapies are individualized because evidence for routine radiation, chemotherapy, targeted therapy, or endocrine therapy remains limited (Pontrelli et al. 2025, Li et al. 2024, NCI PDQ).

Introduction

Solid pseudopapillary neoplasm is uncommon, accounting for approximately 0.9% to 2.7% of exocrine pancreatic neoplasms and about 5% of cystic pancreatic neoplasms (Omiyale 2021, Pontrelli et al. 2025). It predominantly affects young women, although it can occur across a wide age range and in men. Contemporary reviews report mean age around 29 to 31 years, with women representing about 85% of surgically treated patients in large reviews (Mazzarella et al. 2024, Pontrelli et al. 2025).

The two cases below were managed at the University Hospital of the West Indies, Jamaica. Their diagnostic pathway and terminology are consistent with the time of treatment. Modern evaluation would usually include contrast-enhanced CT and/or MRI, with endoscopic ultrasound-guided biopsy and immunohistochemistry when tissue confirmation is needed before treatment (Djokic Kovac et al. 2023, StatPearls 2024).

Case I

S.A., a thirty-year-old woman, gave a two-year history of a painful abdominal mass. On physical examination she was anicteric and had a right upper quadrant abdominal mass. Her hematological and biochemical investigations were normal. Barium meal examination demonstrated a widened duodenal “C” loop. Ultrasonography and computed tomography showed an 11 cm solid and cystic mass in the head of the pancreas. Fine needle aspiration under sonographic guidance was not diagnostic.

At laparotomy, no metastases were identified, and a proximal pancreaticoduodenectomy was performed. Three years and seven months after surgery, there was no sonographic evidence of tumor recurrence or metastases.

Histopathological examination revealed features consistent with solid pseudopapillary neoplasm. The tumor was composed of cuboidal cells with eosinophilic cytoplasm, rounded clear nuclei, and small nucleoli. There was central necrosis, while peripheral areas showed hemorrhagic cyst-like spaces with papillary protrusions. Other areas were solid, with small interspersed vascular channels producing a pseudorosette pattern. Elsewhere, there were cystic spaces containing mucinous material. The tumor was limited by a fibrous capsule with focal invasion but no complete capsular penetration.

Case II

M.D., a thirty-year-old woman, was referred from a neighboring Caribbean island with a one-year history of epigastric pain. Physical examination revealed no abnormality. Hematological and biochemical investigations were normal. Ultrasonography identified a 7 cm mass with solid and cystic areas in the head of the pancreas. Computed tomography showed the cyst to be thick-walled, with no evidence of peripancreatic infiltration or intra-abdominal metastases. Ultrasound-guided fine needle aspiration was not diagnostic.

At laparotomy, a proximal pancreaticoduodenectomy was performed. Convalescence was uncomplicated, allowing the patient to return early to her island home.

Histopathological examination of the pancreatic mass showed a thick-walled cystic lesion. The cyst content was necrotic debris, and the cyst wall had insular nests of round to ovoid cells with clear cytoplasm. The cells were arranged in sheets and papillary configurations with fibrovascular stalks. Some stalks contained foam cells, while others showed mucinous degeneration. These features were consistent with solid pseudopapillary neoplasm.

Discussion

Terminology and epidemiology

SPN is a rare low-grade malignant neoplasm of the pancreas. The older terms “papillary cystic tumor” and “papillary cystic neoplasm” are now best treated as historical synonyms. Increased recognition, improved imaging, and wider availability of immunohistochemistry have led to many more reported cases than were available in the early literature (Omiyale 2021, Mazzarella et al. 2024).

The tumor strongly predominates in women, but the previous explanation that female predominance is caused by estrogen and progesterone receptors should be avoided as a current causal statement. Modern reviews emphasize the molecular association with CTNNB1 mutation and abnormal nuclear beta-catenin/Wnt pathway activation, while the precise cell of origin and the reason for female predominance remain incompletely explained (Omiyale 2021, Meliti and Al-Maghrabi 2023).

SPN may occur in the head, neck, body, or tail of the pancreas. Contemporary series often report body and tail predominance, although head-of-pancreas tumors such as the two cases above are well recognized. A 2024 retrospective study of 195 cases reported 64.1% in the body or tail and 34.9% in the head or neck (Fu et al. 2024).

Pathology and biology

SPN is typically well circumscribed and may be encapsulated. Grossly, it often contains mixed solid, cystic, hemorrhagic, and necrotic areas. Microscopically, it shows poorly cohesive cells arranged in solid sheets, nests, pseudopapillary structures, and pseudorosettes around delicate fibrovascular cores. Foamy macrophages, cholesterol clefts, hemorrhage, and cystic degeneration may be present.

Current diagnostic workup relies on morphology plus immunohistochemistry. Typical immunophenotypic findings include nuclear beta-catenin, CD10, CD56, vimentin, cyclin D1, progesterone receptor, alpha-1-antitrypsin or alpha-1-antichymotrypsin in many cases, loss of membranous E-cadherin, and usually chromogranin negativity. This profile helps distinguish SPN from pancreatic neuroendocrine tumor, acinar cell carcinoma, pancreatoblastoma, mucinous cystic neoplasm, and other pancreatic lesions (Omiyale 2021, Meliti and Al-Maghrabi 2023).

SPN is considered a low-grade malignant neoplasm because local invasion, recurrence, and metastasis can occur. However, most patients do very well after resection. Risk prediction remains imperfect, but contemporary reviews identify possible adverse factors including male sex, positive margin, lymph node involvement, perineural or vascular invasion, adjacent-organ infiltration, high proliferation index, undifferentiated carcinoma, large size, and tumor rupture (Pontrelli et al. 2025).

Clinical presentation

Many SPNs are discovered because of vague abdominal symptoms, abdominal pain, abdominal fullness, a palpable mass, or imaging performed for another reason. A 2025 systematic review reported that about 34.7% of patients were asymptomatic and that common symptoms included abdominal pain, lower back pain, abdominal mass, nausea, vomiting, abdominal discomfort, and weight loss (Pontrelli et al. 2025).

Because the tumors often grow slowly and have low-grade biology, they may become large before diagnosis. The 11 cm and 7 cm tumors in the two cases above are consistent with the typical presentation of a large, well-defined, solid and cystic pancreatic mass.

Imaging and diagnosis

Contrast-enhanced CT and MRI are central to modern evaluation. Typical imaging shows a well-defined or encapsulated pancreatic mass with variable solid and cystic components, hemorrhagic degeneration, necrosis, and sometimes calcification. MRI is particularly useful for characterizing hemorrhage, capsule, internal architecture, and differential diagnosis (Djokic Kovac et al. 2023).

Endoscopic ultrasound-guided biopsy or fine needle aspiration may help establish the diagnosis before surgery, especially when imaging is atypical or when the differential diagnosis includes pancreatic neuroendocrine tumor or other lesions. Tissue diagnosis is strengthened by cell-block preparation, immunohistochemistry, and, when available, molecular confirmation of CTNNB1-related beta-catenin pathway alteration (StatPearls 2024, Meliti and Al-Maghrabi 2023).

Older tests such as barium meal examination, angiography, ERCP, and exploratory laparotomy with frozen section are no longer routine diagnostic steps for a typical suspected SPN, although the exact approach depends on local resources, imaging availability, and surgical planning.

Treatment

Complete surgical resection is the primary treatment. The operative approach depends on tumor location, size, relation to the pancreatic duct and vessels, patient factors, and local expertise. Head-of-pancreas tumors may require pancreaticoduodenectomy, as in these two cases. Tumors in the body or tail may be treated by distal pancreatectomy, sometimes with spleen preservation. Parenchyma-sparing operations may be considered in selected cases to preserve endocrine and exocrine function, but they must be balanced against fistula risk and oncologic adequacy (Pontrelli et al. 2025).

Routine lymphadenectomy is generally not emphasized because lymph node metastasis is uncommon, but suspicious nodes or locally invasive disease should be managed according to operative findings and multidisciplinary judgment. Minimally invasive surgery is increasingly used in selected patients and centers, but open surgery remains appropriate when anatomy, tumor size, vascular involvement, or local expertise makes it safer.

Advanced, recurrent, or metastatic disease

Recurrence and metastasis are uncommon but clinically important. In a 2025 systematic review, recurrence was reported in 2% to 10% of cases, and overall survival was approximately 98% (Pontrelli et al. 2025). Liver is the most frequent metastatic site. A 2024 study of SPN with hepatic metastases reported that 8 of 287 patients had liver metastases and that long-term survival appeared better when metastatic tumors were treated surgically when feasible (Li et al. 2024).

For recurrent, residual, locally advanced, or metastatic SPN, repeat resection or metastasectomy should be considered when technically feasible and clinically appropriate. The evidence base for chemotherapy, radiation therapy, targeted therapy, and endocrine therapy remains limited. These options should be individualized in multidisciplinary discussion rather than presented as standard routine treatment. The NCI PDQ summary lists surgery and chemotherapy as treatment options for pediatric solid pseudopapillary tumor and notes excellent outcomes, with 10-year survival exceeding 95%, but also reflects the limited evidence base for systemic treatment in rare situations (NCI PDQ).

Follow-up

Long-term follow-up is recommended because recurrence can occur years after initial resection, although optimal surveillance intervals are not standardized. Follow-up commonly includes clinical assessment and cross-sectional imaging, with intensity tailored to tumor features, margin status, rupture, invasion, metastatic disease, and institutional practice.

Conclusion

The two cases reported illustrate the classic presentation of SPN in young women with large solid and cystic pancreatic head tumors treated successfully by pancreaticoduodenectomy. Complete excision is usually curative. This review includes modern terminology, epidemiology, pathogenesis, diagnostic testing, and advanced-disease treatment.

SPN is now recognized as a rare low-grade malignant pancreatic neoplasm with characteristic morphology, CTNNB1/beta-catenin pathway biology. SPN has excellent prognosis after complete resection. Recurrent or metastatic disease should be evaluated for repeat resection or metastasectomy when feasible. Non-surgical treatment is usually not advised because evidence for routine adjuvant therapy, radiation, hormonal therapy, or chemotherapy, remains limited.

Educational disclaimer

This article is for educational purposes and does not substitute for individualized medical advice. Patients with pancreatic tumors should be evaluated and managed by qualified clinicians in an appropriate multidisciplinary setting.

References

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