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Pancreatic exocrine tumours in infants and children are rare.

Visit: Pancreatic Pathology Online

The major problem for the clinician is a lack of experience and of

accepted therapeutic strategies.

Malignant pancreatic tumours in children show a pattern different from

that in adults.

In infants, especially pancreatoblastomas, solid cystic tumours of females,

and endocrine carcinomas of the pancreas must be expected.

In addition  acinar cell carcinoma can also occur.  Ductal

adenocarcinoma is virtually absent.

Pancreatoblastomas and solid cystic tumors are mainly found in the

head of the pancreas. Fibrotic capsules with rare, late metastases

are characteristics of these tumors, indicating total resection to

be an important therapeutic procedure. Pancreatoblastomas

should additionally be treated with chemotherapy.

Endocrine carcinomas of the pancreas (malignant gastrinomas and

malignant insulinomas) should also primarily be treated with radical

surgery, including extensive lymph node dissection.

In case of distant metastases, local resection (liver) or somatostatin in

combination with chemotherapy (streptozocin in the case of

malignant insulinomas) may be used.

Click on the following links:

-Pancreatoblastoma

-Solid Pseudopapillary Tumour of the Pancreas

-Acinar cell carcinoma

-Pancreatic Insulinomas

-Pancreatic Gastrinoma

                 

Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics. 2006 Jul-Aug;26(4):1211-38.

Pancreatic neoplasms are rare in children and have a different histologic spectrum and prognosis than those in adults. In general, these tumors are well demarcated with expansile rather than infiltrating growth patterns. They may be quite large at diagnosis, and central cystic necrosis is common. They infrequently cause biliary duct obstruction. The imaging appearance of each neoplasm reflects its pathologic features. Pancreatoblastoma is the most common pancreatic neoplasm in young children. At imaging, pancreatoblastomas are heterogeneous and often multilocular with hyperechoic and enhancing septa. Solid-pseudopapillary tumor occurs in adolescent girls. It is heterogeneous in internal architecture, with a mixture of solid and cystic hemorrhagic and necrotic elements. This tumor is distinguished by its fibrous capsule and hemorrhagic nature, which are best shown at magnetic resonance imaging as a dark rim on T1- or T2-weighted images and hyper-intense foci on T1-weighted images, respectively. Islet cell tumors in children are insulinomas or gastrinomas. These tumors manifest early due to hormonal syndromes and are distinguished by their small size, homogeneous appearance, and intense enhancement with intravenous contrast material. All pancreatic neoplasms in children are capable of producing metastases, usually to the liver and lymph nodes; however, on the whole, these tumors have a better clinical outcome than most pancreatic tumors in adults. Knowledge of the differential diagnosis of pancreatic masses in children and their relatively good prognosis may promote correct preoperative diagnosis and appropriate treatment.

Laparoscopic distal pancreatectomy in children: case report and review of the literature. Ann Surg Oncol. 2007 Mar;14(3):1065-9.

BACKGROUND: Laparoscopic resection of benign tumors of the pancreas has been reported in adults, but only four cases of partial laparoscopic pancreatectomy in children have been described in the English-language literature. METHODS: We describe the case of an 11-year-old girl with a solid pseudopapillary tumor who was treated with a laparoscopic, spleen-preserving, distal pancreatectomy. The specimen was extracted in an endoscopic bag retrieval system through a Pfannenstiel incision. Operative time was 120 minutes, and minimal blood loss occurred. The literature is reviewed. RESULTS: The postoperative course was uneventful. Twenty-two months after the operation, clinical follow-up (including assessment of exocrine and endocrine pancreatic function) revealed nothing abnormal. The functional and aesthetic results were satisfactory. CONCLUSIONS: The technique used for our case is simple and reproducible, was completed safely within a reasonable operative time, and yielded a good result.

Pancreatic tumors in children: report of three cases.Acta Paediatr Taiwan. 1999 Sep-Oct;40(5):335-8.

From 1981 to 1996, we experienced 3 cases of pancreatic tumors in children--two pancreatoblastomas (PB) and one solid and cystic tumor (SCT). The ages were 1 month, 4 years, and 13 years of age respectively. The two cases of pancreatoblastoma initially presented as chronic diarrhea with failure to thrive, the other case presented with abdominal mass. All of them were studied by laboratory examination, ultrasonography, computed tomography and pathology. Increasing alpha- fetoprotein (AFP) levels were found in the 2 pancreatoblastoma cases, however, the level in the SCT case was normal. Abdominal sonography showed pancreatic masses with or without calcification, and the echogenicity may be solid and/or cystic. All patients underwent total excision of the tumors. We have reviewed the literature and find no pancreatoblastoma with chronic diarrhea was reported in young children, especially in neonate. Therefore, we suggest that young children presenting with an abdominal mass and/or weight loss should undergo imaging studies for the possibility of pancreatic tumors.

A 20-year review of pediatric pancreatic tumors.J Pediatr Surg. 1992 Oct;27(10):1315-7.

Pancreatic tumors are rare surgical problems in infants and children. A 20-year audit (1971 to 1991) of this institution showed six patients ranging in age from 3 weeks to 16 years who were operated on for pancreatic neoplasms. Five of these tumors were malignant, bringing the reported experience to 71 cases. This series of malignancies included three solid cystic tumors, one insulin-secreting tumor, and one pancreatoblastoma. The clinical presentations varied: three had abdominal pain, one developed hypoglycemia, and one had an abdominal mass with jaundice. In five of the six patients pancreatic pathology was suspected preoperatively. All were treated primarily with pancreatic resection including one pancreatoduodenectomy. No radiotherapy or chemotherapy was used. The perioperative mortality was 0% with a morbidity of 50%. The long-term results are encouraging, with all patients alive after a mean follow-up of 7.8 years. These data suggest that aggressive surgical therapy is warranted in the management of pediatric pancreatic tumors.

Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present.J Pediatr Surg. 2002 Jun;37(6):887-92.

BACKGROUND: Malignant tumors of the pancreas are uncommon in children and adolescents and only recently have the most common tumor types been well characterized. As a result, the treatment approach to these patients has yet to be standardized, and much of the information available in the literature, particularly with regard to the role of chemotherapy and radiation, is anecdotal. METHODS: A retrospective review was undertaken of all patients less than 21 years of age with malignant pancreatic tumors who were cared for at Memorial Sloan-Kettering since 1967. RESULTS: Seventeen patients were identified. The pathologic types were pancreatoblastoma, 5; solid pseudopapillary tumor, 7; acinar cell carcinoma, 1; nonfunctioning pancreatic endocrine neoplasm, 1; malignant VIPoma, 1; and PNET, 2. A complete resection of the primary tumor was achieved in 82%, and 12 of 15 are alive, 10 with no evidence of disease. Chemotherapy or radiation were used in selected cases. CONCLUSIONS: Unlike malignant pancreatic tumors in adults, tumors in children and adolescents usually are resectable, and long-term survival is likely. However, the risk of recurrence for pancreatoblastoma is high. The roles of chemotherapy and radiation remain undefined.

Pancreatic tumors in childhood: analysis of 13 cases.J Pediatr Surg. 1990 Oct;25(10):1057-62.

Pancreatic tumors are rare in children. Over a 20-year period we have treated 13 children with pancreatic neoplasms. There were eight boys and five girls (age range, 4 months to 12 years). Seven tumors were benign, including five insulinomas, and two cystadenomas. Six lesions were malignant (rhabdomyosarcoma, 2; pancreatic carcinoma, 4). Children with insulinoma presented with hypoglycemia and irrational behavior. Three had abnormal insulin:glucose ratios ( greater than 1.0). The tumor was detected by computed tomography scan in three cases, at the time of surgery in one, and with intraoperative ultrasound in one. Surgical treatment included tumor enucleation in four cases and 80% pancreatectomy in one. Mucinous cystadenomas were observed in two patients, ages 4 months and 10 months. Tha latter infant underwent cyst excision alone, resulting in malignant recurrence at 18 months of age and death. The 4-month-old child had a distal pancreatectomy and is alive at 6 years. Two of the four children with pancreatic cancer had unresectable tumors at diagnosis, and were treated by biopsy (ductal adenocarcinoma), irradiation, and chemotherapy. Length of survival was 6 months and 9 months. Two others (ages 4 and 12 years) underwent 85% distal pancreatic resection for pancreatoblastoma and a pancreatoduodenectomy for papillary carcinoma, respectively. The latter is alive and tumor-free at 20 years of follow-up. The former underwent hepatic lobectomy for a 3.0 x 3.0 cm solitary liver metastases and is alive at 6 years with no evidence of disease. One child with rhabdomyosarcoma died of progressive disease, the other is alive with residual disease despite resection and chemotherapy. Most insulinomas can be treated by enucleation.


October 2007

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INDEX: A-D ; INDEX: E-L ; INDEX: M-P INDEX: Q-Z ; FUNGAL DISEASE ; VIRAL DISEASE.

Anatomy of Normal Pancreas;

Normal Islets of Langerhans ;  

The Apud Concept ;

Developmental Defects of Pancreas;

Nesidioblastosis ;

Pancreas Divisum ;  

Aberrant (Ectopic) or Accessory Pancreas;

Annular Pancreas  ;  

Pancreatic Agenesis;

Non-Neoplastic Pancreatic Cysts;  

Pancreatitis ;  

Acute Pancreatitis ;

Chronic Pancreatitis;

Autoimmune Pancreatitis ;  

Herpes Simplex Pancreatitis ;  

Diabetes Mellitus ;

Neoplasms of the Endocrine Tumours ;

Islet Cell Tumours

Alpha Cell Tumours;

Beta Cell Tumours ;  

Somatostatinoma ;

VIPomas ;  

Pancreatic Polypeptide-Secreting Tumours ;  

Enterochromaffin Cell (Carcinoid) Tumours ;

Pancreatic Gastrinoma;

Corticotropinoma 

Multiple Endocrine Neoplasia (MEN) Syndrome

Carcinoma of the Pancreas;

Adenosquamous Carcinoma of the Pancreas;

Intraductal Papillary Mucinous Tumour of the Pancreas ;

Pancreatic carcinoma in childhood. Report of an autopsy case and a review of the literature.Acta Pathol Jpn. 1985 Nov;35(6):1543-54.

Clinical and histological findings of pancreatic carcinoma in a 6-year-old boy are reported. Gradual change of histological appearance of the tumor during his course of 3 years and elevation of serum alpha-1-fetoprotein (AFP) are documented. Two biopsy specimens showed immature histological appearance compatible with pancreatoblastoma, and autopsy material showed well-differentiated adenocarcinoma with distinct ductal and acinar differentiation. Electron microscopy demonstrated zymogen-like granules in the apical portion of the neoplastic cells. Immunoperoxidase method demonstrated AFP in the neoplastic cells in addition to alpha-1-antitrypsin. Literature of pancreatic tumor in the young was reviewed, and characteristics of this case were discussed.