Multidisciplinary treatment of advanced or recurrent solid pseudopapillary neoplasm of the pancreas: three case reports

Background Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor that predominantly affects young females. Prognosis is excellent; however, 10–15% of patients show metastasis at the time of surgery or develop tumor recurrence after pancreatectomy. Case presentation We reviewed the clinical course of three patients with advanced or recurrent SPN and subsequently underwent multidisciplinary treatment at our institution between 2002 and 2019. The primary tumor was resected in all three patients, and metastases were also resected if indicated. Intensive combined therapy, including re-resection, chemotherapy, ablation, arterial chemoembolization, and radiation therapy, allowed all patients to survive for a long time. The literature review showed that resection seems to be more effective than other treatments for metastatic SPN. Conclusions Multidisciplinary treatment, including resection, may improve the prognosis of patients with SPN with recurrence or metastasis.

Prognostic Enigma of Pancreatic Solid Pseudopapillary Neoplasm: A Single-Center Experience of 63 Patients

Background: Studies investigating prognostic factors of solid pseudopapillary neoplasm (SPN) have been published with conflicting findings.Methods: Retrospective analysis of 63 consecutive cases of SPN in our institution from January 2010 to December 2019 was carried out. The clinicopathological features, treatment practices along with survival associations were collected and analyzed.Results: Fifteen patients (23.8%) were male, and 48 (76.2%) were female, with a median age of 34.0 ± 14.5 years. The larger tumor size was correlated with the more mixed components (p = 0.000) and the higher Ki-67 index (p = 0.042). No recurrence was found in the nine patients whose tumors fulfilled the WHO criteria for malignancy due to the presence of at least perineural invasion (6.4%), angiovascular invasion (2.3%), and/or adjacent organ invasion (6.4%). Microscopic infiltrative growth was detected in 9 (14.3%) tumors, which was correlated significantly with the WHO criteria (p = 0.002), capsule invasion (p = 0.005), and pancreatic parenchyma invasion (p = 0.001), but not with disease-free survival (p = 0.13). CD99 was found to be positively expressed in 88.9% (40/45) of tumors and more likely to have depressed Ki-67 index (p = 0.016). After a median follow-up of 58 months, only two patients (3.2%) had a recurrence after their first operation outside of our hospital. No patient died due to tumor progression.Conclusions: Although survival is favorable with aggressive surgery, it is actually difficult to assess the prognostic factors of resected SPNs. Future investigations into the role of clinicopathological evaluation will unveil the prognostic enigma of pancreatic SPN after resection.

Clinicopathological factors associated with recurrence in patients undergoing resection of pancreatic solid pseudopapillary neoplasm

Purpose Solid pseudopapillary neoplasm (SPN) is an uncommon pathology with a low-grade malignancy. Surgery is the milestone treatment. Nevertheless, despite appropriate management, some patients present recurrence. Risk factors associated with recurrence are unclear. The objective was to identify the clinicopathological factors associated with recurrence in patients with SPN treated with pancreatic resection. Methods Medical records of patients treated with pancreatic resection during 2006–2020 were evaluated. Patients with histological diagnosis of SPN were included. Survival analysis was performed to identify the clinicopathological factors related to recurrence. Results Seventy-four patients were diagnosed with SPN; 70 (94.6%) patients were female, and the median age was 20 years old. The median tumor diameter was 7.9 cm. Multivisceral resection was performed in 9 (12.2%) patients. Four (5.4%) patients presented lymph node metastasis.R0 resection was achieved in all cases. Six (8%) patients presented recurrence and the liver was the most frequent recurrence site (n = 5).After a median follow-up of 40.2 months, 9 (12%) patients died. Five (6.8%) patients died of disease progression. The 1–3- and 5-year overall survival (OS) was 97.1%, 90.2% and 79.9%, respectively. The 1–3-and-5-year recurrence-free survival (RFS) was 98.4%, 89.9% and 87%, respectively. In the univariate Cox-regression analysis, age ≥ 28 years(HR = 8.61, 95% CI 1.1–73.8),tumor diameter ≥ 10 cm(HR = 9.3, 95% CI 1.12–79.6),invasion of adjacent organs (HR = 7.45, 95% CI 1.5–36.9), lymph node metastasis (pN +) (HR = 16.8, 95% CI 2.96–94.9) and, AJCC Stage III (HR = 10.1, 95% CI 1.2–90.9) were identified as predictors for recurrence. Conclusions SPN is more frequently diagnosed in young women with a good overall prognosis after an R0 surgical resection even with disease recurrence. Age ≥ 28 years, larger tumors ≥ 10 cm, invasion of adjacent organs, lymph node metastasis(pN +) and, AJCC Stage III were predictors factors of recurrence in resected SPN.

Solid Pseudopapillary Neoplasm of the Pancreas: Unfolding an Intriguing Condition

Pancreatic cancer is one of the most lethal malignant neoplasms, with a 1-year survival rate after diagnosis of 24%, and a 5-year survival rate of only 9%. While this illustrates the behavior of its main histologic type – ductal adenocarcinoma, there are other histologic subtypes of pancreatic cancer that can harbor excellent prognosis. Solid pseudopapillary neoplasm, described as a rare low-grade malignant neoplasm by the World Health Organization, is the best example of that, having an overall 5-year survival rate of about 97%. Not only the prognosis, but everything about this entity is unique: its histogenesis, epidemiology, presentation, imaging characteristics, cytology features, immunohistochemical profile, and treatment. This explains the urge to improve our understanding about this entity and thus our ability to accurately recognize and manage it. Having this in mind, this article aims to summarize the most relevant topics regarding this entity.

Solid Pseudopapillary Neoplasm (SPN) of the Pancreas: A 7-year Study in A Tertiary Level Teaching Hospital in Bangladesh

Solid pseudopapillary neoplasm (SPN) of the pancreas, also referred to as Frantz's tumour, is a rare tumour and represents 1-3% of all pancreatic tumours, which typically affects young women without significant clinical symptoms. This cross-sectional study was done in the Department of Pathology, Bangabandhu Sheikh Mujib Medical Un iversity (BSMMU) between January 2009 and December 2015. A total of nineteen cases of solid pseudopapillary neoplasm (SPN) of pancreas were detected in patients and included in this study. In all the patients the diagnosis was confirmed by histopathology after the surgery. Tumours were nodular, cystic or solid-cystic, often encapsulated. Some of them were received as irregular fragmented pieces, as intact removal was not possible. Cut surfaces were partly grey-white and partly tan-brown with cystic degeneration. Areas of necrosis and hemorrhage were present in all the specimens.The age range was of 14-45 years (mean age 26.9±3.7 years) (Table-I). Among the tumours, 18 were found in female patients, while only 1 was from male patient. In all the cases, preoperative imaging contributed to diagnosis of an abdominal mass in the patients. 11 of these were reported as pancreatic mass, while 3 as pancreatic cystic mass, 2 as retroperitoneal mass, 2 as mesenteric mass and only 1 as adrenal mass. Tumour-size ranged from 6 to 19 cm (mean size 9.3±1.5 cm). 7 tumours were solid and cystic in nature, while 8 were solid and 4 were only cystic. Complete capsule was found in 17 specimens. Calcifications were found in only 5 specimens. Surgical resection was found generally curative. In our study, absence of metastasis suggests that these tumours hardly show aggressive clinical behaviour. However, follow-up is important to observe potential local recurrence and metastasis. CBMJ 2020 July: Vol. 09 No. 02 P: 34-38