Published online Oct 27, 2026. doi: 10.4240/wjgs.123199
Revised: July 22, 2026
Accepted: August 20, 2026
Published online: October 27, 2026
Processing time: 161 Days and 5.8 Hours
Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare, low-grade malignant tumor. In children, it rarely exhibits aggressive malignant features or presents as a rapidly progressive acute abdomen.
A 16-year-old female patient was admitted with abdominal pain accompanied by nausea and vomiting for three hours. An abdominal ultrasound scan, Contrast-enhanced abdominal magnetic resonance imaging, and computed tomography identified a space-occupying lesion in the pancreatic tail, accompanied by multi
This case is remarkable for its rare aggressive biological behavior, manifesting as a rapidly progressive acute abdomen.
Core Tip: We present a case of solid pseudopapillary neoplasm of the pancreas (SPN) in a child with rare aggressive features, including local pancreatic invasion and multiple liver metastases. The patient presented with acute abdominal manifestations, characterized by progressively worsening abdominal pain and rapidly developing high fever. In addition, we review the literature on the clinical presentation, aggressive malignant features, and treatment of SPN.
- Citation: Zhou XY, Chen T, Liang J, Yue JQ, Wu LL, Li HM, Liu ZH, Peng CJ, Liu GW. Aggressive pediatric solid pseudopapillary neoplasms of the pancreas presented as acute abdomen: A case report and review of literature. World J Gastrointest Surg 2026; 18(10): 123199
- URL: https://www.wjgnet.com/1948-9366/full/v18/i10/123199.htm
- DOI: https://dx.doi.org/10.4240/wjgs.123199
Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare pancreatic tumor, accounting for approximately 0.9% to 2.7% of pancreatic exocrine neoplasms and predominantly affecting young women[1]. SPN is characterized by low malignant potential, with only a minority of cases demonstrating aggressive features such as distant metastasis or local invasion. Most SPNs are asymptomatic, although abdominal pain is the most common symptom; fever, jaundice, and weight loss are relatively rare[2]. The incidence of SPN in children (aged ≤ 18 years) is even lower, at only 0.1 to 0.2 per million[3]. In this report, we describe a case of SPN in a child that presented with both local pancreatic invasion and multiple liver metastases, along with rapidly progressive acute abdominal symptoms, including worsening abdominal pain and high fever over a short period. Subsequently, emergency laparoscopic distal pancreatectomy with splenectomy and lymph node dissection was performed. Furthermore, we reviewed the literature regarding the aggressive malignant features and treatment of SPN in children.
A 16-year-old female presented with abdominal pain accompanied by nausea and vomiting for three hours.
The patient presented with a three-hour history of persistent colicky pain localized to the left upper abdomen without an identifiable cause, accompanied by nausea and vomiting of gastric contents.
There was no significant past medical history.
She was unmarried and nulliparous, and there was no family history of malignant tumors.
Physical examination showed the following vital signs: Temperature, 36.2 °C, blood pressure, 16.8/10.2 kPa; heart rate, 71 beats/minute; and respiratory rate, 20 breaths/minute. Abdominal examination revealed a soft abdomen with tenderness localized to the left upper quadrant, without rebound tenderness or guarding.
On admission, complete blood count revealed a white blood cell count of 13.45 × 109/L. In contrast, hepatic and renal function tests, coagulation profiles, and serum tumor markers (alpha fetoprotein, carcinoembryonic antigen, car
On admission, an abdominal ultrasound examination identified a hypoechoic mass measuring approximately 114 mm × 82 mm × 92 mm situated in the body and tail of the pancreas, in addition to multiple nodules present within the liver parenchyma. Contrast-enhanced abdominal computed tomography (CT) revealed a lesion measuring approximately 125 mm × 107 mm × 88 mm in the pancreatic body and tail, with heterogeneous density and mild heterogeneous enhancement. The splenic vein was compressed, and multiple nodules suspicious for metastases were observed in the liver, suggesting pancreatic malignancy with liver metastases. Subsequent contrast-enhanced abdominal magnetic resonance imaging (MRI) showed a mixed-signal lesion measuring approximately 116 mm × 111 mm × 89 mm in the pancreatic body and tail, with patchy high signal intensity on T1-weighted images and heterogeneous enhancement. The tumor also showed elevated signal intensity on T1-weighted fat-suppressed images. Multiple suspicious nodules were again noted in the liver, and the distal splenic artery and mid splenic vein were poorly visualized, consistent with pancreatic malignancy with hemorrhagic necrosis and liver metastases (Figure 1). Examination of the remaining tissues and organs revealed no abnormalities.
The pancreatic tumor measured approximately 12 cm × 11 cm × 8 cm. Histologic examination revealed pseudopapillary structures characterized by fibrovascular cores, cholesterol deposits, and extensive areas of hemorrhagic necrosis. Tumor cell invasion into the pancreatic parenchyma was evident. Immunohistochemical analysis of the pancreatic tumor showed positivity for CD56, progesterone receptor (PR), and vimentin, with a Ki-67 proliferation index of approximately 1%. In contrast, the suspected metastatic liver nodule showed positivity for β-catenin, CD10, CD56, PR, and vimentin, whereas E-cadherin and chromogranin A were negative, with a Ki-67 proliferation index of approximately 2%. Based on the medical history, imaging features, histopathologic findings, and immunohistochemical results, the diagnosis was consistent with SPN with local pancreatic invasion and liver metastasis, without lymph node involvement (Figure 2).
Upon admission, the patient received analgesic and antibiotic therapy. Subsequent contrast-enhanced abdominal MRI and CT revealed a malignant pancreatic tumor with intratumoral hemorrhage and necrosis and possible liver metastases, whereas no abnormalities were identified in other tissues or organs. A multidisciplinary team consultation was convened to further clarify the diagnosis and formulate a treatment strategy.
However, the patient’s abdominal pain worsened the following evening. It was accompanied by high fever and tachycardia, with the heart rate increasing to 130 beats per minute. Hematologic analysis showed a white blood cell count of 20.72 × 109/L and a neutrophil percentage of 77.7%, both significantly elevated compared with admission values. Given the progression of hemorrhage and necrosis within the pancreatic tumor and the absence of infection in other organs, the infection was attributed to the pancreatic lesion. To prevent rupture and tumor dissemination caused by excessive pressure within the tumor cyst, emergency laparoscopic distal pancreatectomy with splenectomy and lymph node dissection (groups 7, 8, 9, 11, and 18) was performed, along with biopsy of the liver metastasis in segment IVb (Figure 3).
The patient had an uneventful postoperative recovery, with only a grade A pancreatic fistula (biochemical leak). Transarterial chemoembolization was planned to further manage the multiple liver metastases, but the guardian subsequently declined treatment in favor of regular follow-up. At the 17month followup, no evidence of pancreatic endocrine or exocrine insufficiency or tumor recurrence was observed. The liver metastases remained stable without evidence of enlargement (Figure 4).
We present a case of SPN in a child with rare aggressive features, including local pancreatic invasion and multiple liver metastases. The patient presented with acute abdominal manifestations, characterized by progressively worsening abdominal pain and rapidly developing high fever. These features are rarely observed in clinical practice.
Most SPNs exhibit benign behavior, with only a limited number of cases demonstrating aggressive malignant characteristics[4]. To date, only 12 cases of SPN in children with aggressive features have been reported in the literature. However, only 5 of these had complete medical information, and their clinical data are summarized in Table 1. All five patients presented with abdominal pain. The case reported by Nunes et al[5] presented with acute severe abdominal pain accompanied by high fever, with a peak temperature of 40 °C. In contrast, the case described by Gehlot and Sethna[6] had a prolonged disease course and manifested rare symptoms, including weight loss and occasional fever. Postoperative pathological examination of both cases revealed extensive necrosis within the pancreatic tumors. Three tumors were located in the pancreatic tail, with an average diameter of 10.48 cm. The case reported by Nunes et al[5] showed tumor capsule rupture and local pancreatic invasion, whereas the other cases demonstrated multiple liver metastases. Histologically, characteristic pseudopapillary structures and positive immunostaining for β-catenin and PR were observed, with the highest Ki-67 index being less than 10%. One study[7] found no significant differences in tumor location or size between pediatric and adult patients with SPN. Nevertheless, that study was limited by a small pediatric sample size and the lack of comparative analysis of clinical manifestations. Aggressive features were observed in only 9.2%[4] of cases at initial diagnosis and were primarily documented in isolated case reports, with no clinical studies comparing SPN with aggressive features at initial diagnosis between children and adults. Cheng et al[8] described a similar case involving a 55-year-old male patient who presented with a 4.5 cm tumor in the pancreatic tail and multiple liver metastases at initial diagnosis. Biopsy confirmed SPN with liver metastases and local pancreatic invasion.
| Ref. | Age (year-old) | Sex | Symptoms | Tumor size (cm) | Location | Liver metastasis | local pancreatic invasion | Histological features | IHC | Treatment | Prognosis alive |
| Nunes et al[5], 2026 | 11 | Female | Acute abdominal pain, fever, nausea, and vomiting | 6.8 | Tail | No | Yes | Extensive necrosis; cholesterol clefts | Ki-67 < 2%, β-catenin (+), CD56(+), CD10(+), Cyclin D1(+) | SPDP | No recurrence (follow-up duration unspecified); yes |
| Gehlot and Sethna[6], 2025 | Early adolescence | Female | Abdominal pain for 4 months, weight loss, occasional fever | 12.1 | Tail | VIII, IVa | No | Characteristic pseudopapillary structures; eosinophilic cytoplasm; extensive hemorrhagic necrosis; cholesterol clefts | Ki-67 < 2%, Syn(-), CgA(-) | Resection of pancreatic and liver tumors | No recurrence at 4-month follow-up; yes |
| Hu et al[23], 2024 | 17 | Female | Trauma-induced abdominal pain | 11 | Anterior pancreas | Multiple | No | NA | β-catenin(+), Vimentin(+), CD10(+), Ki-67 approximately 5%, Syn (partial+), CgA (-) | Initial pancreatic tumor resection + partial hepatectomy; recurrence at 3 months, RFA | No recurrence at 43-month follow-up; yes |
| Sumida et al[24], 2007 | 14 | Female | Abdominal pain for 4 days | 8.5 | Tail | Multiple | No | NA | PR(+) | Initial DPS; liver metastasis enlarged at 3 months, LT | No recurrence at 24-month follow-up; yes |
| Hassan et al[13], 2005 | 15 | Female | Abdominal pain | 14 | Head | II, IV, V, VI, VII | No | Gross: Cystic-solid components with partial hemorrhage; microscopic: Characteristic pseudopapillary structures, focal necrosis and hemorrhage, cholesterol deposition | Vimentin(+), ACT(+), AAT(+), NSE(+), PR(+), Ki-67 < 10%, CgA(-) | Initial PD + PVR + RH; liver metastasis enlarged at 12 months, liver metastasectomy | No recurrence at 6-month follow-up; yes |
The patient experienced progressively worsening abdominal discomfort over one month, without accompanying symptoms such as fever or weight loss. The Ki-67 index was approximately 30%, and genetic analysis identified a shared homozygous atypical mutation in the APC gene. Further research is needed to investigate differences in the clinical manifestations, tumor characteristics, immunohistochemical features, and genetic profiles between adult and pediatric patients exhibiting aggressive features.
According to published data, more than 40% of patients with SPN are asymptomatic at the time of diagnosis. The most prevalent symptom is abdominal pain, and other common symptoms include nausea and vomiting[2]. Abdominal pain usually presents as non-progressive chronic abdominal pain. However, both our case and that reported by Nunes et al[5] presented with rapidly progressive acute abdominal symptoms accompanied by high fever, in contrast to the case reported by Gehlot and Sethna[6], which was characterized by intermittent fever persisting for up to one month. Postoperative pathological examinations in three cases revealed extensive necrosis within the tumors, suggesting that the fever may be associated with large-scale tumor necrosis. SPN is known for its propensity for spontaneous hemorrhage, and the persistent, progressive abdominal pain that is difficult to relieve may be related to ongoing intratumoral bleeding and increased intratumoral pressure[9].
The aggressive malignant features of SPN lack a precise definition. Its biological behaviors include deep infiltration, perivascular invasion, and distant metastasis[10]. Pathological characteristics include a diffuse growth pattern, large tumor volume, extensive necrosis, an abnormally elevated mitotic rate, and the presence of undifferentiated components such as sarcomatoid carcinoma[11,12]. In this case, the tumor measured up to 12 cm in diameter and was accompanied by extensive hemorrhagic necrosis and perivascular invasion. Among the five cases reviewed in this study, the average tumor diameter was 10.48 cm. Significant necrosis of pancreatic tumors was documented in studies by Nunes et al[5] and Gehlot and Sethna[6], whereas Hassan et al[13] reported a case with focal necrosis of a pancreatic tumor; all of these cases demonstrated aggressive malignant features. Therefore, prompt intervention is warranted when SPN exhibits these features, given the risk of malignant transformation.
The Ki-67 index is typically regarded as positively correlated with the invasive malignant characteristics of SPN[10,11]. Nevertheless, a growing body of research has documented cases of invasive malignancy despite low Ki-67 expression levels. In a study by Comut et al[14], 28 patients exhibited metastatic or locally invasive features, with a median Ki-67 index of 5% (range, 1% to 15%). Similarly, Yang et al[4] reported that 11 patients displayed invasive characteristics, yet 7 of these patients had a Ki-67 index of only 1%. Furthermore, Chen et al[15] identified a Ki-67 index exceeding 1% as an independent risk factor for tumor recurrence. In our analysis of five pediatric cases, all presented with a low Ki-67 index. This apparent paradox between clinical invasiveness and low proliferation may be explained by the tumor microenvironment (TME). Yang et al[4] demonstrated that while the Ki-67 index was 1% in 7 out of 11 invasive cases, there was a significant increase in the infiltration of tumor-associated macrophages (TAM) both within and at the margins of the tumors in the invasive group. TAMs can secrete various proteolytic enzymes that degrade the extracellular matrix, thereby compromising this fibrous barrier and potentially enabling tumor cell invasion[16,17]. Furthermore, TAMs can upregulate vascular endothelial growth factor-A, which promotes tumor angiogenesis and contributes to metastasis[18,19]. Therapeutic strategies targeting TAMs may modulate the tumor microenvironment, potentially improving prognostic outcomes.
An additional noteworthy observation is that, despite the rapid clinical progression and malignant characteristics suggestive of tumor aggressiveness in our case, no subsequent antitumor therapies, including chemotherapy or immunotherapy, were pursued following the resection of the primary tumor; instead, the patient was subjected solely to follow-up monitoring. Remarkably, over the course of a 17-month follow-up period, there was no observed increase in either the size or number of multiple liver metastases. This atypical scenario may be attributable to alterations in the TME following resection of the primary tumor. Proteomic analysis conducted by Tanaka et al[20] has demonstrated that SPN can be classified as an immunologically cold tumor, characterized by low expression of major histocompatibility complex class I molecules. This classification is further supported by the sparse infiltration of T cells and B cells, in contrast to a substantial presence of TAMs, alongside a marked reduction in the antigen processing machinery. Such a composition of the tumor immune microenvironment facilitates the tumor’s evasion of immune surveillance. Following the resection of the primary tumor, this immune-mediated equilibrium may shift in favor of the host[21]. Moreover, Li and Liu[22] documented a case of SPN located in the pancreatic tail with multiple liver metastases, where no additional treatment was administered post-palliative debulking surgery. Remarkably, a follow-up conducted 20 months postoperatively revealed that the liver metastases had diminished and ultimately disappeared. Conversely, the analysis of the three cases revealed a recurrence or enlargement of liver metastases after the initial resection of the primary pancreatic lesion[13,23,24]. Consequently, a thorough evaluation encompassing various dimensions, including biological behavior (such as metastasis or local invasion), pathological characteristics (such as tumor size, extent of necrosis, and Ki-67 index), and the TME, may be necessary for future assessments of tumor malignancy and prognosis.
Surgical resection remains the primary treatment for SPN, regardless of synchronous or metachronous metastases[25]. In pediatric patients, preservation of pancreatic function should be prioritized whenever feasible. Radical resection procedures, such as distal pancreatectomy with splenectomy and pancreaticoduodenectomy, require careful consideration of potential postoperative complications, including pancreatic fistula, endocrine and exocrine dysfunction, effects on growth and development, and psychological stress[26-28]. Tumor recurrence is associated with risk factors such as larger tumor size, local invasion, and distant metastasis[29,30]. Such patients may be candidates for radical pancreatectomy[10]. Emergency surgical intervention for SPN is often necessitated by rupture and hemorrhage, which may occur secondary to trauma or spontaneous rupture[31]. Such rupture-induced hemorrhage can facilitate intraperitoneal dissemination of the tumor, potentially leading to postoperative recurrence and metastasis[32]. Repeated surgical procedures can have a profound impact on long-term quality of life in pediatric patients. Consequently, timely in
Preoperative imaging identified a tumor measuring approximately 12 cm in diameter, exerting compressive effects on the splenic artery and vein and showing significant hemorrhagic and necrotic changes. Despite symptomatic treatment, including anti-infective agents and analgesics, the patient’s abdominal pain progressively worsened and was accompanied by high fever, ultimately necessitating urgent surgical intervention. Because of the presence of diffuse multifocal liver metastases, radical resection was deemed unfeasible. Postoperative pathological analysis demonstrated extensive hemorrhagic necrosis within the tumor, along with positive progesterone receptor status. Although the postoperative recovery in this pediatric case was favorable, this report describes only a single case. For patients with rapidly worsening abdominal pain, high fever, large tumor size, and concurrent hemorrhage, further research is needed to determine the optimal timing of surgical intervention. In the case reported by Gehlot and Sethna[6], liver metastases were identified in segments VIII and IVa, prompting concurrent hepatectomy. Conversely, the case reported by Hu et al[23] involved multiple liver metastases, necessitating biopsy of the metastatic lesions. Similarly, the case reported by Sumida et al[24] also showed multiple liver metastases and underwent liver transplantation during a subsequent surgical intervention.
For patients with unresectable SPN and liver metastases, there is currently no standardized treatment protocol. Most SPNs exhibit mutations in the CTNNB1 gene, leading to the activation of the Wnt/β-catenin signaling pathway, which plays a crucial role in tumor proliferation. Furthermore, it has been consistently observed that, in addition to CTNNB1 mutations, the expression of other significant genes can interact with various signaling pathways to synergistically enhance tumor development. This process involves a complex network of molecular interactions. Huang and He[34] suggested that sex hormone receptors, including PR, estrogen receptors (ER), and androgen receptors, function as key regulatory nodes in SPN development, influencing pathways such as Wnt/β-catenin, notch, and hedgehog, as well as the epithelial-mesenchymal transition process. Kornietskaya et al[35] administered the antiestrogen drug tamoxifen to a patient with overexpression of ER and PR and liver metastasis, resulting in tumor stabilization after one year of treatment. Wang et al[36] identified mutations in the PTEN gene in tumors and proposed a potential interaction between the Wnt/β-catenin and mTOR signaling pathways. The use of the mTOR inhibitor everolimus led to partial tumor regression and maintained stability for more than 22 months. Cheng et al[8] documented a homozygous atypical mutation in the APC gene, thereby advancing the understanding of Wnt pathway abnormalities that occur independently of CTNNB1 mutations. After chemotherapy with a modified FOLFIRINOX regimen, liver metastases demonstrated a 15.9% reduction in size, and the tumor disease remained stable for 34 weeks. In addition to endocrine therapy, targeted therapy, and systemic chemotherapy informed by signaling pathways, other treatment modalities reported include radiofrequency ablation[37], locoregional interventional chemotherapy[38], and liver transplantation[24]. Nevertheless, therapies aimed at regulating the TME warrant further investigation.
This study presents a rare case of aggressive SPN in a child, characterized by multiple liver metastases and pancreatic invasion. The case showed an unusual rapid progression, presenting as an acute abdomen with severe abdominal pain and high fever. Future research should investigate the molecular mechanisms underlying its malignant progression and explore personalized treatment strategies targeting these highly aggressive features.
The authors express their sincere gratitude to the patient and her family for their support.
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