World Journal of Surgery

, Volume 4, Issue 1, pp 29–36

Neuroblastoma: an analysis of 160 cases

  • Jay L. Grosfeld
  • Robert L. Baehner
Article

Abstract

Neuroblastoma was observed in 160 patients from 1948–1978. Ninety-seven patients were boys and 63 were girls. At diagnosis, 74 patients were less than 2 years of age, 28 between 2–3 years, and 58 over 3 years. Sixty-two (38%) patients had localized disease, while 98 (62%) had metastases. Patients were grouped by extent of disease according to the staging criteria of Evans et al.: stage I (5), stage II (31), stage III (26), stage IV (82), stage IV-S (16). Tumors occurred in the neck (3), mediastinum (16), abdomen (136), and pelvis (3). Clinical findings often included abdominal mass, weight loss, anemia, bone pain, and proptosis. Six patients had diarrhea and 3 had cerebellar ataxia and nystagmus. Lesions were often calcified (>50%), and bone marrow aspirate frequently demonstrated tumor clumps (rosettes). Urinary VMA was elevated in 85% of cases.

Therapy varied according to stage. Stage I patients received operative excision alone and stage II patients operative resection with radiation for residual tumor and/or positive lymph nodes. Stage III patients were managed aggressively with operative resection (when possible), irradiation, and combination chemotherapy (cyclophosphamide, vincristine, DTIC, Adriamycin®, VM-26). Patients with metastases (stage IV) were initially treated with multiagent chemotherapy with late “second-look” or delayed primary laparotomy for tumor resection done in clinical responders.

Two-year disease-free survival occurred in 57 of 160 patients or 35.6%. Survival rates were best for infants under age 1 year (74%) and for patients with stage I (100%), stage II (74%) and stage IV-S (75%) tumors. There was improved survival in patients with tumors that occurred in the neck (100%), pelvis (100%), and mediastinum (75%). Survival rates were poor in patients over 2 years of age (13–17%), with abdominal tumors (28%), and with stage III (34%) and stage IV (10%) tumors. While chemotherapy and irradiation have improved tumor response, survival rate has not been improved. Immunotherapy has been disappointing. Unfortunately, at the present time, there is no specific chemotherapeutic agent that has a curative effect on this tumor.

Résumé

Entre 1948 et 1978, nous avons observé 160 malades atteints de neuroblastomes, 97 garçons et 63 filles. Au moment du diagnostic, 74 malades avaient moins de 2 ans, 28 entre 2 et 3 ans et 58 plus de 3 ans. La tumeur était localisée dans 62 cas (38%) et avec métastases dans 98 cas (62%). Les malades ont été groupés selon l’étendue de la maladie et selon les critères de Evans en: 5 stades I, 31 stades II, 26 stades III, 82 stades IV et 16 stades IV-S. La tumeur était localisée au cou dans 3 cas, au médiastin dans 16, dans l’abdomen dans 136, au petit bassin dans 3 cas. Les principaux symptomes étaient une masse abdominale, une perte de poids, une anémie, des douleurs osseuses et une exophtalmie. Six malades présentaient de la diarrhée et 3 une ataxie cérébelleuse avec nystagmus. La lésion était souvent calcifiée (>50%) et la ponction médullaire a souvent mis en évidence des amas de cellules tumorales (rosettes). L’excrétion urinaire de VMA était élevée dans 85% des cas.

La thérapeutique a varié selon le stade de la maladie. Pour les malades au stade I, le seul traitement a été l’exérèse chirurgicale. Pour les stades II, l’exérèse a été complétée par une irradiation, soit du tissu tumoral résiduel, soit des aires ganglionnaires envahies. Le traitement des malades au stade III a été aggressif: exérèse lorsqu’elle est possible, radiothérapie et polychimiothérapie (cyclophosphamide, Vincristine, DTIC, Adriamycine, VM-26). Les malades présentant des métastases (stade IV) ont été traités au début par polychimiothérapie avec “second look” tardif ou laparotomie primitive retardée pour exérèse de la tumeur dans les cas répondant à la chimiothérapie.

La survie à deux ans sans récidive a été de 57/160 cas (35.6%). Les meilleures survies ont été obtenues chez les enfants endessous de 1 an (74%) et pour les tumeurs aux stades I (100%), II (74%) et IV-S (75%). La survie est également meilleure pour les tumeurs localisées au cou (100%), au petit bassin (100%) et dans le médiastin (75%). Les pourcentages de survie sont faibles pour les malades âgés de plus de 2 ans (13–17%), pour les tumeurs abdominales (28%), et pour les tumeurs aux stades III (34%) et IV (10%). Si la chimio- et la radiothérapie ont un effet thérapeutique sur les neuroblastomes, elles n’ont cependant pas augmenté les chances de survie. L’immunothérapie est inefficace. Il n’y a malheureusement, à l’heure actuelle, aucun agent chimiothérapique qui ait une réelle action curative sur ce type de tumeur.

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References

  1. 1.
    Evans, A.E., D’Angio, G.J., Randolph, J.G.: A proposed staging for children with neuroblastoma. Cancer27:374, 1971PubMedGoogle Scholar
  2. 2.
    Priebe, C.R., Clatworthy, H.W., Jr.: Neuroblastoma: an evaluation of 90 children. Arch. Surg.95:538, 1967PubMedGoogle Scholar
  3. 3.
    Evans, A.E., Gerson, J., Schnaufer, L.: Spontaneous regression of neuroblastoma. Natl. Cancer Inst. Monogr.44:49, 1976PubMedGoogle Scholar
  4. 4.
    Hellstrom, I., Hellstrom, K.E., Pierre, G.E., Bill, A.H.: Demonstration of cell bound humoral immunity against neuroblastoma cells. Proc. Natl. Acad. Sci.60:1231, 1968PubMedGoogle Scholar
  5. 5.
    Hellstrom, K.E., Hellstrom, I.: Lymphocyte-mediated cytotoxicity and blocking serum activity to tumor antigens. Adv. Immunol.18:209, 1974PubMedCrossRefGoogle Scholar
  6. 6.
    Jose, D.G., Skvaril, F.: Serum inhibitors of cellular immunity in human neuroblastoma IgG subclass of blocking activity. Int. J. Cancer13:173, 1974PubMedGoogle Scholar
  7. 7.
    Helson, L., Ghavimi, F., Wu, C.J., Fieisher, M., Schwartz, M.K.: Carcinoembryonic antigen in children with neuroblastoma. J. Natl. Cancer Inst.57:725, 1976PubMedGoogle Scholar
  8. 8.
    Necheles, T.F., Rausen, A.R., Kung, F.H., Pochedly, C.: Immunochemotherapy in advanced neuroblastoma. Cancer41:1282, 1978PubMedGoogle Scholar
  9. 9.
    Akeson, R., Seeger, R.C.: Interspecies neural membrane antigens on cultured human and murine neuroblastoma cells. J. Immunol.118:1995, 1977PubMedGoogle Scholar
  10. 10.
    Casper, J.T., Borella, L., Sen, L.: Reactivity of human brain antiserum with neuroblastoma cells and nonreactivity with thymocytes and lymphoblasts. Cancer Res.37:1750, 1977PubMedGoogle Scholar
  11. 11.
    Evans, A.E., Albo, V., D’Angio, G.J., Finklestein, J.Z., Leikin, S., Santulli, T., Weiner, J., Hammond, G.D.: Cyclophosphamide treatment of patients with localized and regional neuroblastoma: a randomized study. Cancer38:655, 1976PubMedGoogle Scholar
  12. 12.
    Koop, C.E., Johnson, D.G.: Neuroblastoma: assessment of therapy in reference to staging. J. Pediatr. Surg.6:595, 1971CrossRefPubMedGoogle Scholar
  13. 13.
    Grosfeld, J.L., Ballantine, T.V.N., Baehner, R.L.: Current management of childhood solid tumors. Surg. Clin. North Am.56:513, 1976PubMedGoogle Scholar
  14. 14.
    Grosfeld, J.L., Schatzlein, M.L., Ballantine, T.V.N., Weetman, R.M., Baehner, R.L.: Metastatic neuroblastoma: factors influencing survival. J. Pediatr. Surg.13:59, 1978PubMedGoogle Scholar
  15. 15.
    Grosfeld, J.L., Ballantine, T.V.N., Baehner, R.L.: Experience with “second-look” operations in pediatric solid tumors. J. Pediatr. Surg.13:275, 1978PubMedGoogle Scholar
  16. 16.
    Koop, C.E., Schnaufer, L.: The management of abdominal neuroblastoma. Cancer35:905, 1975PubMedGoogle Scholar
  17. 17.
    Grosfeld, J.L., Sitarz, A., Finklestein, J., Leikin, S.: The effect of primary tumor resection on survival in metastatic neuroblastoma. Proc. Am. Soc. Clin. Oncol.18:308, 1977Google Scholar
  18. 18.
    D’Angio, G.J., Evans, A.E., Koop, C.E.: Special pattern of widespread neuroblastoma with a favorable prognosis. Lancet1:1046, 1971CrossRefPubMedGoogle Scholar
  19. 19.
    Schnaufer, L., Koop, C.E.: Silastic abdominal pouch for temporary hepatomegaly in stage IV-S neuroblastoma. J. Pediatr. Surg.10:73, 1975PubMedGoogle Scholar
  20. 20.
    Weetman, R.M., Rider, P.S., Oei, T.O., Hempel, J.S., Baehner, R.L.: Effect of diet on urinary excretion of VMA, HVA, metanephrines and total free catecholamines in normal preschool children. J. Pediatr.88:46, 1976CrossRefPubMedGoogle Scholar
  21. 21.
    Laug, W.E., Siegel, S.E., Shaw, K.N.F., Landing, B., Baptista, J., Gutenstein, M.: Initial urinary catecholamine metabolite concentrations and prognosis in neuroblastoma. Pediatrics62:77, 1978PubMedGoogle Scholar
  22. 22.
    Hassenbusch, S., Kaizer, H., White, J.J.: Prognostic factors in neuroblastic tumors. J. Pediatr. Surg.11:287, 1976PubMedGoogle Scholar
  23. 23.
    Breslow, N., McCann, B.: Statistical estimation of prognosis for children with neuroblastoma. Cancer Res.31:2098, 1971PubMedGoogle Scholar
  24. 24.
    Altman, A., Baehner, R.L.: Favorable prognosis for survival in children with coincident opsomyoclonus and neuroblastoma. Cancer37:846, 1976PubMedGoogle Scholar
  25. 25.
    Bloom, S.R., Polak, J.M., Pearse, A.G.E.: Vasoactive intestinal peptide and watery diarrhea syndrome. Lancet2:14, 1973CrossRefPubMedGoogle Scholar
  26. 26.
    Jansen-Goemans, A., Engelhardt, J.: Intractable diarrhea in a boy with vasoactive intestinal peptide producing ganglioneuroblastoma. Pediatrics59:710, 1977PubMedGoogle Scholar
  27. 27.
    Helson, L., Helson, C., Peterson, R.F., Das, S.K.: A rationale for the treatment of metastatic neuroblastoma. J. Natl. Cancer Inst.57:727, 1976PubMedGoogle Scholar
  28. 29.
    Hayes, F.A., Green, A.A., Mauer, A.M.: Correlation of cell kinetic and clinical response to chemotherapy in disseminated neuroblastoma. Cancer Res.37:3766, 1977PubMedGoogle Scholar
  29. 30.
    Maurer, H.M.: Solid tumors in children. New Engl. J. Med.299:1345, 1978PubMedCrossRefGoogle Scholar

Copyright information

© Société Internationale de Chirurgie 1980

Authors and Affiliations

  • Jay L. Grosfeld
    • 1
    • 2
  • Robert L. Baehner
    • 1
    • 2
  1. 1.Section of Pediatric Surgery, Department of Surgery, and Section of Pediatric Hematology-Oncology, Department of PediatricsIndiana University School of MedicineUSA
  2. 2.J.W. Riley Hospital for ChildrenIndianapolisUSA

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