Pancreatic tumors in childhood

Analysis of 13 cases

Jay L. Grosfeld, Dennis W. Vane, Frederick Rescorla, Warren McGuire, Karen W. West

Research output: Contribution to journalArticle

81 Citations (Scopus)

Abstract

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 (> 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. The 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 × 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. However, non-endocrine tumors require pancreatic resection to effect a cure. Mucinous cystadenoma is a premalignant condition and must be managed by pancreatic resection. Although survival with carcinoma of the pancreas in adults has a dismal outlook (1% to 4% survival), pancreatic cancer in children has a relatively improved prognosis according to tumor histology (papillary, pancreatoblastoma). These observations emphasize the importance of extirpative tumor surgery in infants and children with pancreatic malignancy.

Original languageEnglish
Pages (from-to)1057-1062
Number of pages6
JournalJournal of Pediatric Surgery
Volume25
Issue number10
DOIs
StatePublished - 1990

Fingerprint

Neoplasms
Insulinoma
Pancreatic Neoplasms
Mucinous Cystadenoma
Pancreatectomy
Rhabdomyosarcoma
Survival
Cystadenoma
Drug Therapy
Pancreaticoduodenectomy
Papillary Carcinoma
Liver
Hypoglycemia
Cysts
Pancreas
Histology
Adenocarcinoma
Tomography
Insulin
Neoplasm Metastasis

Keywords

  • Frantz' tumor
  • insulinoma
  • Pancreatic cancer, childhood
  • pancreato-blastoma
  • papillary tumors of pancreas

ASJC Scopus subject areas

  • Surgery

Cite this

Pancreatic tumors in childhood : Analysis of 13 cases. / Grosfeld, Jay L.; Vane, Dennis W.; Rescorla, Frederick; McGuire, Warren; West, Karen W.

In: Journal of Pediatric Surgery, Vol. 25, No. 10, 1990, p. 1057-1062.

Research output: Contribution to journalArticle

Grosfeld, Jay L. ; Vane, Dennis W. ; Rescorla, Frederick ; McGuire, Warren ; West, Karen W. / Pancreatic tumors in childhood : Analysis of 13 cases. In: Journal of Pediatric Surgery. 1990 ; Vol. 25, No. 10. pp. 1057-1062.
@article{8e2691d129994b4ea8e057dab3b36696,
title = "Pancreatic tumors in childhood: Analysis of 13 cases",
abstract = "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 (> 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. The 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 × 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. However, non-endocrine tumors require pancreatic resection to effect a cure. Mucinous cystadenoma is a premalignant condition and must be managed by pancreatic resection. Although survival with carcinoma of the pancreas in adults has a dismal outlook (1{\%} to 4{\%} survival), pancreatic cancer in children has a relatively improved prognosis according to tumor histology (papillary, pancreatoblastoma). These observations emphasize the importance of extirpative tumor surgery in infants and children with pancreatic malignancy.",
keywords = "Frantz' tumor, insulinoma, Pancreatic cancer, childhood, pancreato-blastoma, papillary tumors of pancreas",
author = "Grosfeld, {Jay L.} and Vane, {Dennis W.} and Frederick Rescorla and Warren McGuire and West, {Karen W.}",
year = "1990",
doi = "10.1016/0022-3468(90)90218-X",
language = "English",
volume = "25",
pages = "1057--1062",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
number = "10",

}

TY - JOUR

T1 - Pancreatic tumors in childhood

T2 - Analysis of 13 cases

AU - Grosfeld, Jay L.

AU - Vane, Dennis W.

AU - Rescorla, Frederick

AU - McGuire, Warren

AU - West, Karen W.

PY - 1990

Y1 - 1990

N2 - 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 (> 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. The 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 × 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. However, non-endocrine tumors require pancreatic resection to effect a cure. Mucinous cystadenoma is a premalignant condition and must be managed by pancreatic resection. Although survival with carcinoma of the pancreas in adults has a dismal outlook (1% to 4% survival), pancreatic cancer in children has a relatively improved prognosis according to tumor histology (papillary, pancreatoblastoma). These observations emphasize the importance of extirpative tumor surgery in infants and children with pancreatic malignancy.

AB - 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 (> 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. The 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 × 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. However, non-endocrine tumors require pancreatic resection to effect a cure. Mucinous cystadenoma is a premalignant condition and must be managed by pancreatic resection. Although survival with carcinoma of the pancreas in adults has a dismal outlook (1% to 4% survival), pancreatic cancer in children has a relatively improved prognosis according to tumor histology (papillary, pancreatoblastoma). These observations emphasize the importance of extirpative tumor surgery in infants and children with pancreatic malignancy.

KW - Frantz' tumor

KW - insulinoma

KW - Pancreatic cancer, childhood

KW - pancreato-blastoma

KW - papillary tumors of pancreas

UR - http://www.scopus.com/inward/record.url?scp=0025038830&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0025038830&partnerID=8YFLogxK

U2 - 10.1016/0022-3468(90)90218-X

DO - 10.1016/0022-3468(90)90218-X

M3 - Article

VL - 25

SP - 1057

EP - 1062

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

SN - 0022-3468

IS - 10

ER -