Gastrointestinal autonomic nerve tumors in the pediatric population

A report of four cases and a review of the literature

Jody Z. Kerr, M. John Hicks, Jed G. Nuchtern, Victor Saldivar, Josefine Heim-hall, Shafqat Shah, David R. Kelly, Walter S. Cain, Murali M. Chintagumpala

Research output: Contribution to journalArticle

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Abstract

BACKGROUND. Gastrointestinal autonomic nerve tumors (GANTs) are a subpopulation of gastrointestinal stromal tumors (GISTs) that are characterized by ultrastructural features resembling enteric autonomic nerve cells, without epithelial, Schwannian, or smooth muscle differentiation. Delineation of the clinicopathologic features of GANT in the pediatric population is lacking. METHODS. The clinicopathologic and outcome data for four pediatric patients with GANT are presented. The data from these patients and four previously reported pediatric patients are summarized and compared with data for GANT in adults. RESULTS. All four cases occurred in females at a mean age of 12.5 years. The primary tumor site was the stomach in all cases, and the mean tumor size was 6.3 cm. Immunocytochemical and ultrastructural examination were essential in distinguishing GANT from GIST in all cases by identifying features of neural origin (neuron specific enolase in all four cases, NFP in three cases, S-100 in two cases, dense core neurosecretory granules in all four cases, and neuritelike processes in all four cases), while failing to identify features of myogenic origin (no desmin, smooth muscle actin, myofilaments, or dense bodies were found in any of the cases). Primary treatment was surgical, with chemotherapy administered to 1 patient at the time of recurrence. All patients are alive after a mean follow-up of 60 months (range, 8 months to 9 years). CONCLUSIONS. Similarities of pediatric GANT to GANT in adults include the need for a high index of suspicion for diagnosis; comparable histopathologic, immunohistochemical, and ultrastructural features; and surgery as the primary therapy. Distinguishing features in children may be a prevalence among females in the second decade, a predominance of smaller gastric tumors, and a positive prognostic value of younger age.

Original languageEnglish (US)
Pages (from-to)220-230
Number of pages11
JournalCancer
Volume85
Issue number1
DOIs
StatePublished - Jan 1 1999

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Pediatrics
Population
Gastrointestinal Stromal Tumors
Smooth Muscle
Stomach
Autonomic Pathways
Neoplasms
Desmin
Phosphopyruvate Hydratase
Myofibrils
Plexosarcoma
Actins
Neurons
Recurrence
Drug Therapy
Therapeutics

Keywords

  • Gastrointestinal autonomic nerve tumor
  • Gastrointestinal stromal tumor
  • Immunohistochemistry
  • Pediatric
  • Ultrastructural

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Gastrointestinal autonomic nerve tumors in the pediatric population : A report of four cases and a review of the literature. / Kerr, Jody Z.; Hicks, M. John; Nuchtern, Jed G.; Saldivar, Victor; Heim-hall, Josefine; Shah, Shafqat; Kelly, David R.; Cain, Walter S.; Chintagumpala, Murali M.

In: Cancer, Vol. 85, No. 1, 01.01.1999, p. 220-230.

Research output: Contribution to journalArticle

Kerr, JZ, Hicks, MJ, Nuchtern, JG, Saldivar, V, Heim-hall, J, Shah, S, Kelly, DR, Cain, WS & Chintagumpala, MM 1999, 'Gastrointestinal autonomic nerve tumors in the pediatric population: A report of four cases and a review of the literature', Cancer, vol. 85, no. 1, pp. 220-230. https://doi.org/10.1002/(SICI)1097-0142(19990101)85:1<220::AID-CNCR30>3.0.CO;2-A
Kerr, Jody Z. ; Hicks, M. John ; Nuchtern, Jed G. ; Saldivar, Victor ; Heim-hall, Josefine ; Shah, Shafqat ; Kelly, David R. ; Cain, Walter S. ; Chintagumpala, Murali M. / Gastrointestinal autonomic nerve tumors in the pediatric population : A report of four cases and a review of the literature. In: Cancer. 1999 ; Vol. 85, No. 1. pp. 220-230.
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abstract = "BACKGROUND. Gastrointestinal autonomic nerve tumors (GANTs) are a subpopulation of gastrointestinal stromal tumors (GISTs) that are characterized by ultrastructural features resembling enteric autonomic nerve cells, without epithelial, Schwannian, or smooth muscle differentiation. Delineation of the clinicopathologic features of GANT in the pediatric population is lacking. METHODS. The clinicopathologic and outcome data for four pediatric patients with GANT are presented. The data from these patients and four previously reported pediatric patients are summarized and compared with data for GANT in adults. RESULTS. All four cases occurred in females at a mean age of 12.5 years. The primary tumor site was the stomach in all cases, and the mean tumor size was 6.3 cm. Immunocytochemical and ultrastructural examination were essential in distinguishing GANT from GIST in all cases by identifying features of neural origin (neuron specific enolase in all four cases, NFP in three cases, S-100 in two cases, dense core neurosecretory granules in all four cases, and neuritelike processes in all four cases), while failing to identify features of myogenic origin (no desmin, smooth muscle actin, myofilaments, or dense bodies were found in any of the cases). Primary treatment was surgical, with chemotherapy administered to 1 patient at the time of recurrence. All patients are alive after a mean follow-up of 60 months (range, 8 months to 9 years). CONCLUSIONS. Similarities of pediatric GANT to GANT in adults include the need for a high index of suspicion for diagnosis; comparable histopathologic, immunohistochemical, and ultrastructural features; and surgery as the primary therapy. Distinguishing features in children may be a prevalence among females in the second decade, a predominance of smaller gastric tumors, and a positive prognostic value of younger age.",
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AU - Saldivar, Victor

AU - Heim-hall, Josefine

AU - Shah, Shafqat

AU - Kelly, David R.

AU - Cain, Walter S.

AU - Chintagumpala, Murali M.

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N2 - BACKGROUND. Gastrointestinal autonomic nerve tumors (GANTs) are a subpopulation of gastrointestinal stromal tumors (GISTs) that are characterized by ultrastructural features resembling enteric autonomic nerve cells, without epithelial, Schwannian, or smooth muscle differentiation. Delineation of the clinicopathologic features of GANT in the pediatric population is lacking. METHODS. The clinicopathologic and outcome data for four pediatric patients with GANT are presented. The data from these patients and four previously reported pediatric patients are summarized and compared with data for GANT in adults. RESULTS. All four cases occurred in females at a mean age of 12.5 years. The primary tumor site was the stomach in all cases, and the mean tumor size was 6.3 cm. Immunocytochemical and ultrastructural examination were essential in distinguishing GANT from GIST in all cases by identifying features of neural origin (neuron specific enolase in all four cases, NFP in three cases, S-100 in two cases, dense core neurosecretory granules in all four cases, and neuritelike processes in all four cases), while failing to identify features of myogenic origin (no desmin, smooth muscle actin, myofilaments, or dense bodies were found in any of the cases). Primary treatment was surgical, with chemotherapy administered to 1 patient at the time of recurrence. All patients are alive after a mean follow-up of 60 months (range, 8 months to 9 years). CONCLUSIONS. Similarities of pediatric GANT to GANT in adults include the need for a high index of suspicion for diagnosis; comparable histopathologic, immunohistochemical, and ultrastructural features; and surgery as the primary therapy. Distinguishing features in children may be a prevalence among females in the second decade, a predominance of smaller gastric tumors, and a positive prognostic value of younger age.

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