Management of pancreatoblastoma in children and young adults Journal Article
| Authors: | Glick, R. D.; Pashankar, F. D.; Pappo, A.; LaQuaglia, M. P. |
| Article Title: | Management of pancreatoblastoma in children and young adults |
| Abstract: | 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. © 2012 by Lippincott Williams & Wilkins. |
| Keywords: | adult; cancer chemotherapy; child; cisplatin; doxorubicin; pancreas resection; treatment; cancer staging; nuclear magnetic resonance imaging; follow up; anorexia; pancreaticoduodenectomy; cancer prevention; computer assisted tomography; multiple cycle treatment; tumor volume; etoposide; vomiting; differential diagnosis; weight reduction; cancer screening; cyclophosphamide; continuous infusion; ifosfamide; childhood cancer; cancer invasion; liver metastasis; tumor recurrence; pancreas tumor; lactate dehydrogenase; age distribution; childhood; abdominal mass; pancreas biopsy; chromogranin; alpha 1 antitrypsin; alpha fetoprotein; abdominal distension; neuron specific enolase; synaptophysin; pancreatic tumor; pirarubicin; pancreatoblastoma; upper abdominal pain; failure to thrive; long term survival; glucose 6 phosphatase; beckwith wiedemann syndrome |
| Journal Title: | Journal of Pediatric Hematology/Oncology |
| Volume: | 34 |
| Issue: | Suppl. 2 |
| ISSN: | 1077-4114 |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2012-05-01 |
| Start Page: | S47 |
| End Page: | S50 |
| Language: | English |
| DOI: | 10.1097/MPH.0b013e31824e3839 |
| PROVIDER: | scopus |
| PUBMED: | 22525406 |
| DOI/URL: | |
| Notes: | --- - "Export Date: 4 June 2012" - "CODEN: JPHOF" - "Source: Scopus" |
