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Opsoclonus and occult neuroblastoma. G. E.
INTERNATIONAL
ABSTRACTS
OF PEDIATRIC
173
SURGERY
in the prevention of recurrence of Wilms’ tumor was compared to that of multiple dose Dactinomycin therapy. The study was limited to children over 12 months of age with localized unilateral disease including those with extention to the adjoining parieties or lymph nodes, but excluding those with metastases in remote areas such as Radiolung, liver, bone, or pelvic peritoneum. therapy was used in both groups. Dactinomycin was started as soon as the diagnosis of Wilms’ tumor was made and was given over a 5 day period. In those receiving multiple courses, the drug was again given at 6 weeks, 3 months, and then every 3 months up to 15 months after the operation. Of the 23 acceptable subjects in group I. that is those receiving a single drug course, 11 have not had a recurrence to date. There have been no recurrences in 19 of the 22 patients in croup II (multiple drug courses). The difference in recurrence rate between the 2 groups is significant suggesting that multiple dose of therapy is superior to the single course of Dactinomycin in the prevention of metastases.-B. ,II. Henderson. ULTRASTRUCTURAL STUDIES IN WILMS’ TUMORS. A. M. Balsaver, C. W. Gibley, Jr., and C. F. Tessmer. Cancer 22:417427 (August) 1968. Sections taken from 3 human Wilms’ tumors (nephroblastomas) were studied in detail both with light and electron microscopy. The tumor cells showed several degrees of differentiation, at times forming well-formed tubules and glomeroloid structures. The glomeroloid structures contained aberrant basement membranes and epithelial cells with cytoplasmic processes but lacking blood cells. The authors suggest that these findings possibly point to the metanephrogenic blastema as the origin of Wilms’ tumors-B. M. Henderson. THE DEVELOPMENTALDEFECTS ASSOCIATEDWITH NEUHoBLASTOhlA-ETIOLOGIC IMPLICATIONS. w. M. Sy. and J. H. Edmondson. Cancer 22:234238 (July) 1968. The authors present the case of a B-month-old infant who was found to have neuroblastoma in association with syndactyly and rib anomalies. On reviewing the literature they found 26 other cases of neuroblastoma associated with congenital anomalies involving various systems. Familial aggregation is generally absent making it likely that most cases of neuroblastoma arise as a result of postzygotic disorders which may be teratogenic. -B. M. Henderson.
OPSOCLONUS AND OCCULT NEUROBLASTOMA. G. E. Solomon, and A. M. Med.
279:475477
Chutmian.
(August
29)
New
Eng.
J.
1968.
Two children, one 13 months old and the other 2% years of age, are described each presenting with opsoclonus (irregular, multidirectional, hyperkinetic spontaneous eye movements ), truncal ataxia, and abnormal movements of the head. Abnormal calcification was seen in the chest in the first case and in the abdomen in the second leading to exploration with the discovery of occult neuroblastoma. Urinary catecholamines and cystathionine were normal in the first child and not reported in the second. The mechanism of production and significance of opsoclonus and its possible relationship to neuroblastoma is discussed.-B. ICI. Henderson. MELANOTIC NEUROECTODEKMAL TuhfoA OF INFANCY. J. Koudstaal, 1. Oldhoff, A. K. Panders, und M. J. Hardmk. Cancer 22:151-161 (Jnly) 1968. Two cases of melanotic neuroectodermal tumor of infancy are presented in children aged 3?6 months and 6 weeks. The tumors were of bluish color and on histochemical and histologic examination appeared to be made up of neuroectodermal cells. In spite of their infiltrative appearance these lesions are benign. Enzyme studies demonstrated similarities between this tumor and malignant melanoblastoma, paragangliomas, and pheochromocytomas. The authors conclude that these tumors probably are derived from cells of the neural crest.-B. M. Henderson. MULTIPLE MUCOSAL NEUROMAS, PHEOCHROMOCYTOMA AND MEDULLARYCARCINOMA OF THE THYROI-A SYNDROME. R. J. Go&n, H. 0. Sedatw, R. A. Vickers, and J. Ceruenka. Cancer 22:293-299 (August) 1968. This is an analysis of 17 cases including 3 children ages 8, 9, and 12 years who presented with plexiform neuromas of the mucosal surfaces of the head and neck, medullary carcinoma of the thyroid and pheochromocytoma. They usually did not develop all the components of the syndrome at the same time. The relationship of this syndrome to other similar ones is discussed.-B. M. Henderson. EMBRYONAL RHABLI~MYOSARCOMA. A REPORT OF 24 CASES AND STUDY OF THE EFFECTIVENESS OF RADIATION THERAPY UPON THE PRIMARI.
ABSTRACTS
OF PEDIATRIC
173
SURGERY
in the prevention of recurrence of Wilms’ tumor was compared to that of multiple dose Dactinomycin therapy. The study was limited to children over 12 months of age with localized unilateral disease including those with extention to the adjoining parieties or lymph nodes, but excluding those with metastases in remote areas such as Radiolung, liver, bone, or pelvic peritoneum. therapy was used in both groups. Dactinomycin was started as soon as the diagnosis of Wilms’ tumor was made and was given over a 5 day period. In those receiving multiple courses, the drug was again given at 6 weeks, 3 months, and then every 3 months up to 15 months after the operation. Of the 23 acceptable subjects in group I. that is those receiving a single drug course, 11 have not had a recurrence to date. There have been no recurrences in 19 of the 22 patients in croup II (multiple drug courses). The difference in recurrence rate between the 2 groups is significant suggesting that multiple dose of therapy is superior to the single course of Dactinomycin in the prevention of metastases.-B. ,II. Henderson. ULTRASTRUCTURAL STUDIES IN WILMS’ TUMORS. A. M. Balsaver, C. W. Gibley, Jr., and C. F. Tessmer. Cancer 22:417427 (August) 1968. Sections taken from 3 human Wilms’ tumors (nephroblastomas) were studied in detail both with light and electron microscopy. The tumor cells showed several degrees of differentiation, at times forming well-formed tubules and glomeroloid structures. The glomeroloid structures contained aberrant basement membranes and epithelial cells with cytoplasmic processes but lacking blood cells. The authors suggest that these findings possibly point to the metanephrogenic blastema as the origin of Wilms’ tumors-B. M. Henderson. THE DEVELOPMENTALDEFECTS ASSOCIATEDWITH NEUHoBLASTOhlA-ETIOLOGIC IMPLICATIONS. w. M. Sy. and J. H. Edmondson. Cancer 22:234238 (July) 1968. The authors present the case of a B-month-old infant who was found to have neuroblastoma in association with syndactyly and rib anomalies. On reviewing the literature they found 26 other cases of neuroblastoma associated with congenital anomalies involving various systems. Familial aggregation is generally absent making it likely that most cases of neuroblastoma arise as a result of postzygotic disorders which may be teratogenic. -B. M. Henderson.
OPSOCLONUS AND OCCULT NEUROBLASTOMA. G. E. Solomon, and A. M. Med.
279:475477
Chutmian.
(August
29)
New
Eng.
J.
1968.
Two children, one 13 months old and the other 2% years of age, are described each presenting with opsoclonus (irregular, multidirectional, hyperkinetic spontaneous eye movements ), truncal ataxia, and abnormal movements of the head. Abnormal calcification was seen in the chest in the first case and in the abdomen in the second leading to exploration with the discovery of occult neuroblastoma. Urinary catecholamines and cystathionine were normal in the first child and not reported in the second. The mechanism of production and significance of opsoclonus and its possible relationship to neuroblastoma is discussed.-B. ICI. Henderson. MELANOTIC NEUROECTODEKMAL TuhfoA OF INFANCY. J. Koudstaal, 1. Oldhoff, A. K. Panders, und M. J. Hardmk. Cancer 22:151-161 (Jnly) 1968. Two cases of melanotic neuroectodermal tumor of infancy are presented in children aged 3?6 months and 6 weeks. The tumors were of bluish color and on histochemical and histologic examination appeared to be made up of neuroectodermal cells. In spite of their infiltrative appearance these lesions are benign. Enzyme studies demonstrated similarities between this tumor and malignant melanoblastoma, paragangliomas, and pheochromocytomas. The authors conclude that these tumors probably are derived from cells of the neural crest.-B. M. Henderson. MULTIPLE MUCOSAL NEUROMAS, PHEOCHROMOCYTOMA AND MEDULLARYCARCINOMA OF THE THYROI-A SYNDROME. R. J. Go&n, H. 0. Sedatw, R. A. Vickers, and J. Ceruenka. Cancer 22:293-299 (August) 1968. This is an analysis of 17 cases including 3 children ages 8, 9, and 12 years who presented with plexiform neuromas of the mucosal surfaces of the head and neck, medullary carcinoma of the thyroid and pheochromocytoma. They usually did not develop all the components of the syndrome at the same time. The relationship of this syndrome to other similar ones is discussed.-B. M. Henderson. EMBRYONAL RHABLI~MYOSARCOMA. A REPORT OF 24 CASES AND STUDY OF THE EFFECTIVENESS OF RADIATION THERAPY UPON THE PRIMARI.