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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Grosfeld, J.L., Baehner, R.L. Neuroblastoma: an analysis of 160 cases. World J. Surg. 4, 29–36 (1980). https://doi.org/10.1007/BF02393089
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DOI: https://doi.org/10.1007/BF02393089