Solid Pseudopapillary Tumor of the Pancreas
Incidence
Solid pseudopapillary tumor of the pancreas, also known as Frantz tumor, is the most common pediatric pancreatic tumor, accounting for up to 70% of cases in most institutional series.[1,2] This tumor has low malignant potential and most commonly affects females of reproductive age (median age, 21 years), Black people, and East Asian people.[2,3,4] There is no known genetic or hormonal factor to explain the strong female predilection, although it has been noted that all tumors express progesterone receptors.[5]
Histology and Genomic Alterations
Histologically, solid pseudopapillary tumor of the pancreas is characterized by a combination of solid, pseudopapillary, and cystic changes. The fragility of the vascular supply leads to secondary degenerative changes and cystic areas of hemorrhage and necrosis.[2,3,4] The cells surrounding the hyalinized fibrovascular stalks form the pseudopapillae.[3]
A highly specific, paranuclear, dot-like immunoreactivity pattern for CD99 has been described.[6]
Variants in the CTNNB1 gene have been identified in more than 90% of these tumors.[7]
Diagnosis
Solid pseudopapillary tumor of the pancreas is a very friable tumor, and tumor rupture and hemoperitoneum have been reported.[2,3,4] Tumors can occur throughout the pancreas and are often exophytic. On imaging, the mass shows typical cystic and solid components, with intratumoral hemorrhage and a fibrous capsule.[3]
A retrospective review of the National Cancer Database identified 21 pediatric patients (younger than 18 years) and 348 adult patients with solid pseudopapillary neoplasm of the pancreas.[8] When compared with their adult counterparts, the children had similar disease severity at presentation, received similar treatments, and experienced equivalent postoperative outcomes.
Treatment of Solid Pseudopapillary Tumor of the Pancreas
The outcomes of patients with solid pseudopapillary tumors of the pancreas are excellent, with 10-year survival rates exceeding 95%.[5]
Treatment options for solid pseudopapillary tumor of the pancreas include the following:
- Surgery.
- Chemotherapy.
Surgery
Treatment of solid pseudopapillary tumor of the pancreas is primarily surgical. However, preoperative and operative spillage is not unusual.[9] Whipple procedures (pancreaticoduodenectomy) are often necessary, but non-Whipple, pancreatic-sparing resections may be possible with a pancreatico-jejunostomy procedure. Surgery is usually curative, although local recurrences occur in 5% to 15% of cases.[4]
A retrospective review of the Italian Pediatric Rare Tumor Registry identified 43 pediatric patients diagnosed with solid pseudopapillary tumor of the pancreas between 2000 and 2018.[10][Level of evidence C1] The median age at diagnosis was 13.2 years (range, 7–18 years). Only one patient presented with metastatic disease.
- At follow-up (median, 8.4 years; range, 0–17 years), one recurrence occurred in a patient who had intraoperative rupture, and all patients were alive.
A study identified 38 children aged 0 to 18 years who were diagnosed with pseudopapillary solid tumor of the pancreas between 2008 and 2022 in a German registry.[11] Patients were a median age of 14.5 years at diagnosis (range, 8–18 years), with a female preponderance (81.6%). The pancreatic tail was the most frequent location of the tumor. All patients underwent surgical resection.
- No recurrences occurred during follow-up, although two patients underwent resection with microscopic residual disease.
Chemotherapy
Single-agent gemcitabine is reportedly effective in cases of unresectable or metastatic disease.[12] Metastatic disease, usually in the liver, may occur in up to 15% of cases.[2,3,4,5,6]
References:
- Rojas Y, Warneke CL, Dhamne CA, et al.: Primary malignant pancreatic neoplasms in children and adolescents: a 20 year experience. J Pediatr Surg 47 (12): 2199-204, 2012.
- Dall'igna P, Cecchetto G, Bisogno G, et al.: Pancreatic tumors in children and adolescents: the Italian TREP project experience. Pediatr Blood Cancer 54 (5): 675-80, 2010.
- Chung EM, Travis MD, Conran RM: Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 26 (4): 1211-38, 2006 Jul-Aug.
- Papavramidis T, Papavramidis S: Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg 200 (6): 965-72, 2005.
- Estrella JS, Li L, Rashid A, et al.: Solid pseudopapillary neoplasm of the pancreas: clinicopathologic and survival analyses of 64 cases from a single institution. Am J Surg Pathol 38 (2): 147-57, 2014.
- Laje P, Bhatti TR, Adzick NS: Solid pseudopapillary neoplasm of the pancreas in children: a 15-year experience and the identification of a unique immunohistochemical marker. J Pediatr Surg 48 (10): 2054-60, 2013.
- Rodriguez-Matta E, Hemmerich A, Starr J, et al.: Molecular genetic changes in solid pseudopapillary neoplasms (SPN) of the pancreas. Acta Oncol 59 (9): 1024-1027, 2020.
- Leraas HJ, Kim J, Sun Z, et al.: Solid Pseudopapillary Neoplasm of the Pancreas in Children and Adults: A National Study of 369 Patients. J Pediatr Hematol Oncol 40 (4): e233-e236, 2018.
- Vasudevan SA, Ha TN, Zhu H, et al.: Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study. Pediatr Blood Cancer 67 (9): e28425, 2020.
- Crocoli A, Grimaldi C, Virgone C, et al.: Outcome after surgery for solid pseudopapillary pancreatic tumors in children: Report from the TREP project-Italian Rare Tumors Study Group. Pediatr Blood Cancer 66 (3): e27519, 2019.
- Jentzsch C, Fuchs J, Agaimy A, et al.: Solid pseudopapillary neoplasms of the pancreas in childhood and adolescence-an analysis of the German Registry for Rare Pediatric Tumors (STEP). Eur J Pediatr 182 (12): 5341-5352, 2023.
- Maffuz A, Bustamante Fde T, Silva JA, et al.: Preoperative gemcitabine for unresectable, solid pseudopapillary tumour of the pancreas. Lancet Oncol 6 (3): 185-6, 2005.