Background
Solid pseudopapillary neoplasia (SPN) of the pancreas is an extremely rare epithelial tumor of low malignant potential. SPN accounts for less than 1% to 2% of exocrine pancreatic tumors. The aim of this study is to report our experience with SPN of the pancreas. It includes a summary of the current literature to provide a reference for the management of this rare clinical entity.

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Diagnosis and treatment of solid pseudopapillary tumor of the pancreas: experience of one single institution from Turkey.

Diagnosis and treatment of solid-pseudopapillary tumor of the pancreas.

Pancreatoblastoma is a very rare childhood tumor originating from the epithelial exocrine cells of the pancreas. It is the most common malignant pancreatic tumor in young children and has a mean age of diagnosis of 5 years. It is slow growing and its presentation is varied and often non-specific. Tumors tend to be quite large and appropriate cross sectional imaging is very important to assess for extent, metastatic disease, and resectability. Biopsy for tissue diagnosis is essential. Complete surgical resection is the goal of therapy although many patients are unresectable at initial diagnosis and require neoadjuvant chemotherapy. Adjuvant chemotherapy is also recommended and chemotherapeutic regimens involve cisplatin and doxorubicin. Even with curative resections, these lesions have a high recurrence rate and patients must be followed closely. Knowledge of this rare tumor is important for the clinician confronted with a large retroperitoneal mass in a young child.

Management of pancreatoblastoma in children and young adults.

BACKGROUND

The aim of the current study was to clarify the actual clinicopathologic status of extremely rare pancreatic carcinoids. To date, statistical evaluation of such carcinoids has been hampered because an insufficient number of cases has prevented any reliable statistical analyses.



METHODS

The Niigata Registry for Gut–Pancreatic Endocrinomas contains a total number of 156 cases of pancreatic carcinoids among 165 endocrinocarcinomas registered worldwide. This figure of 156 cases comprises 144 typical and 12 atypical carcinoids, which were compared statistically with carcinoids in other representative sites, according to various clinicopathologic criteria.



RESULTS

Pancreatic carcinoids made up 1.4% of the total number of registered cases (n = 11,343) and were characteristic in the following five ways. 1) They exhibited a high metastatic rate (66.7%), somewhat lower than that for the ileocecum (76.1%), identical to that for the ileum (66.9%), and far higher than that for the total average of 35.7%. 2) They displayed the largest average tumor size (68.6 mm), followed by that for the ovary (68.2 mm), and ileocecum (46.5 mm) against a total average of 30.2 mm. 3) They revealed a relatively high incidence of the carcinoid syndrome (23.3%), almost equal to that for the ileocecum (24.1%), and exceeded by that for the small intestine (35.8%), when compared with that for the total average of 11.0%. 4) They showed a high rate of immunohistochemical detection for serotonin (92.9%), lower than that for the testicle (100.0%), but higher than the total average of 54.9%. 5) Five-year survival rate was extremely low (28.9% ± 16.7%) compared with those for the appendix (89.7% ± 2.0%) and the small intestine (82.1% ± 3.3%). It was noteworthy that silver impregnations in the pancreatic carcinoid series indicated a result identical to that for the total average: Grimelius argyrophilia, 84.8% versus 85.4%; argyrophil cell type, 59.1% versus 58.5%; and argentaffin cell type, 22.7% versus 22.3%.



CONCLUSION

It may be said that in the treatment of patients with pancreatic carcinoids, appropriate procedures should be carried out with these distinguishing characteristics always kept in mind. Cancer 2005. © 2005 American Cancer Society.

Carcinoids of the pancreas: an analysis of 156 cases.

Pancreatic carcinoma accounts for the most dismal survival among all malignancies with 5-year survival rates approaching 5%. The reason for this, besides the inherent biologic nature of the disease, is the fact that the patients tend to present late in the disease. We present a review of the current published data on cystic neoplasms of the pancreas, which though rare, constitute an important subgroup of pancreatic neoplasms that have a better prognosis and are potentially curable lesions.

Cystic tumours of the pancreas

Papillary-cystic and solid neoplasm (PCSN) are rare tumors. Two personal observations and a review of the literature are presented with a total of 44 pediatric patients in addition to a total of 67 published cases in the review of Cohen (Pediatr. Surg. Int. 6 (1991) 128) and Snadjauf (Eur. J. Pediatr. Surg. 9 (1999) 416). Overall, PCSN shows a clear predominance in females and only occasionally occurs in males. Typically they grow to a large tumor mass with minimal symptoms. Their histologic and immunocytologic characteristics cause diagnostic difficulties, especially on frozen sections of small biopsies. The tumors are assumed to origin from pluripotent stem cells and present as tumors of low malignancy with a favorable prognosis. Nevertheless 10 children have been reported to develop metastases, 5 have demonstrated an invasive growth pattern and 4 local recurrence. But only two of the 111 pediatric cases have died from their tumor burden. Treatment of choice is a complete surgical resection, which is true for the primary tumor and for metastases as well as local recurrences. In our 2 patients one had spleen-conserving left pancreatic resection and one mesopancreatectomy with roux-en-y-reconstruction leading to long-term cure. Adjuvant therapy in curative resected patients is unnecessary and does not appear to improve prognosis.

Therapeutic strategy of papillary cystic and solid neoplasm (PCSN): a rare non-endocrine tumor of the pancreas in children

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Therapeutic strategy of papillary cystic and solid neoplasm (PCSN): a rare non-endocrine tumor of the pancreas in children