Pancreatoblastoma: Case report and review of treatment in the literature

Nonductal neoplasms of the pancreas

“…Pediatric patients with localized tumors can be cured by complete surgical resection. Children with metastatic disease have generally faired poorly, although recent reports of favorable responses to chemotherapy and radiation [163][164][165][166][167] suggest that even some of these patients may experience long-term survival. In adults, pancreatoblastomas have been nearly universally fatal, with most cases progressing quickly, similar to acinar cell carcinomas.…”

Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature

“…The production of AFP by PB has been noted repeatedly in the literature, both in the form of serum elevation [3][4][5][6][7][8]11,12,27,33,34] and immunohistochemical detection [7,12,27]. Similar to AFP-secreting yolk sac tumors, it may be useful for diagnosis and tumor marker in the followup after surgery and during chemotherapy [3,6,7,12,34].…”

“…However, the relationship between tumor behavior and AFP is not fully understood. According to several authors, serum AFP levels do not necessarily correlate with disease activity, disease recurrence, or the presence of metastases [4,8,11,13]. Tsuchida et al [35] had developed a method that differentiates the origin of the AFP using lectin-affinity immunoelectrophoresis.…”

“…At present, the long-term outcome and prognosis of such cases are dismal. Preoperative chemotherapy is recommended to turn an unresectable tumor into a resectable one and has produced long-term survivors [3][4][5][6][7][8]. However, optimal polychemotherapeutic regimens are still under discussion because of the small number of patients treated as yet.…”

“…Complete surgical resection remains the most commonly associated treatment with long-term survival. In unresectable tumor at diagnosis, responses to preoperative chemotherapy (some complete) have been reported [3][4][5][6][7][8]. In patients with local recurrence, spillage, or metastatic tumor, adjuvant chemotherapy and/or radiotherapy should be considered [4,5,[7][8][9][10][11][12][13][14][15][16].…”