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Current treatment of Wilms' tumor
ABSTRACTS
588 ages ranged from 10 months to 14 years. In most, the melanoma arose from the general body skin. In one, there was transplacental transmission of malignant melanoma. The buccal mucosa was the site of origin in another. Others arose from blue nevi, retrobulbar soft tissues or from areas within the head and neck region. Eight of the twelve children died within 18 months of metastatic disease. Of the six cases that arose from the general body skin, other than from giant nevi, four have survived for more than five years. Surgical excision with lymph node dissection, if clinically positive, was the treatment of choice. Malignant melanomas in childhood are extremely rare, there being only 43 well documented cases up to 1966. Because of the difficulty in differentiating malignant melanoma of childhood from juvenile melanoma, the diagnosis can only be made in the presence of metastasis. The five-year survival in the reported group of patients was 33 per cent which was comparable to that seen in adults. Any suspicious pigmented lesion should be excised and if found to contain malignant melanoma a radical wide excision should then be performed. If lymph nodes are palpable then these should be removed in continunity, if possible. It would appear that the patients with the larger lesions, but without palpable lymph nodes, might be benefited by prophylactic lymph node dissection. Out of the 41 cases reported in the literature, 10 developed malignant melawithin giant hypertrophic nevi. nomas Therefore, it would appear that these lesions should be removed as soon as possible, not only for cosmetic reasons but also to prevent malignant complications.-Bruce H. Henderson
CONGENITAL NEUROBLASTOMA.SYMPTOMSIN THE MOTHER DURING PREGNANCY. P. A. Joute,S. K. Wadman and W. J. Van Putten. Clin. Ped. 9:206 (April), 1970. This is a short review of 6 infants with diagnosis of neuroblastoma. All these infants had elevated urinary vanilmandelic acid (VMA), homovanillic acid (HVA), and vanil glycol (VG). Of interest was the symptoms which were displayed in the eighth or ninth month of pregnancy by the mothers of these six children. All these mothers had symptoms similar to those seen in patients with a pheochromocytoma:
paroxymal or persistant hypertension, headache, tachycardia, nervousness, vomiting, dyspnea, excessive sweating, tingling of feet and hands. The authors postulate that these symptoms were caused by fetal catecholamines entering the maternal circulation. Unfortunately urinary excretion of catecholamine metabolites were done only in the infants, and not in the mothers. Three of these six infants survived, the authors conclude that infants born of eclamptic or preeclamptic mothers, should be studied intensively for neuroblastoma.-Michel Gilbert
CURRENT TREATMENT OF WILMS' TUMOR. A. T. Uson, J. A. Wolff and P. Tretter. J. Ural. 103:217-221 (February), 1970. The key to success in the management of a Wilms’ tumor is early diagnosis. Nephrectomy is the treatment of choice, with removal of any other affected tissues such as the tail of the pancreas, spleen, diaphragm or the vena cava. Postoperative radiotherapy is generally used and on rare occasions, with very large tumors, preoperative radiation has been helpful. Actinomycin D is used in all cases as soon as the tumor hqs been excised. Proohylactic courses are given at six weeks. three mo-tths, six months, nine months, one year and at fifteen months postoperatively. Vincristine is also effective. When present in a newborn, operation should be delayed until patient can withstand anesthesia which is usually at 48 hours. In the premature the operation might have to be delayed for several weeks. In children under the age of 12 months, radical nephrectomy should be performed as soon as possible and this should be followed by radiotherauy to the renal and tumor beds only if gross or microscopic evidence of local spread or contamination is present. Those older, should undergo nephrectomy with Actinomycin D or Vincristine chemotherapy. Radiotherapy is added if there is evidence of local or regional spread. In children with clinically unresectable tumors, chemotherapy and radiotherapy are started, initially, possibly using Vincristine, and is then followed by operation when the tumor has become resectable. As a result of these modes of treatment the current survival rate is 50-60 per cent overall, and can be as high as 90 per cent in selected groupsBruce
M. Henderson
588 ages ranged from 10 months to 14 years. In most, the melanoma arose from the general body skin. In one, there was transplacental transmission of malignant melanoma. The buccal mucosa was the site of origin in another. Others arose from blue nevi, retrobulbar soft tissues or from areas within the head and neck region. Eight of the twelve children died within 18 months of metastatic disease. Of the six cases that arose from the general body skin, other than from giant nevi, four have survived for more than five years. Surgical excision with lymph node dissection, if clinically positive, was the treatment of choice. Malignant melanomas in childhood are extremely rare, there being only 43 well documented cases up to 1966. Because of the difficulty in differentiating malignant melanoma of childhood from juvenile melanoma, the diagnosis can only be made in the presence of metastasis. The five-year survival in the reported group of patients was 33 per cent which was comparable to that seen in adults. Any suspicious pigmented lesion should be excised and if found to contain malignant melanoma a radical wide excision should then be performed. If lymph nodes are palpable then these should be removed in continunity, if possible. It would appear that the patients with the larger lesions, but without palpable lymph nodes, might be benefited by prophylactic lymph node dissection. Out of the 41 cases reported in the literature, 10 developed malignant melawithin giant hypertrophic nevi. nomas Therefore, it would appear that these lesions should be removed as soon as possible, not only for cosmetic reasons but also to prevent malignant complications.-Bruce H. Henderson
CONGENITAL NEUROBLASTOMA.SYMPTOMSIN THE MOTHER DURING PREGNANCY. P. A. Joute,S. K. Wadman and W. J. Van Putten. Clin. Ped. 9:206 (April), 1970. This is a short review of 6 infants with diagnosis of neuroblastoma. All these infants had elevated urinary vanilmandelic acid (VMA), homovanillic acid (HVA), and vanil glycol (VG). Of interest was the symptoms which were displayed in the eighth or ninth month of pregnancy by the mothers of these six children. All these mothers had symptoms similar to those seen in patients with a pheochromocytoma:
paroxymal or persistant hypertension, headache, tachycardia, nervousness, vomiting, dyspnea, excessive sweating, tingling of feet and hands. The authors postulate that these symptoms were caused by fetal catecholamines entering the maternal circulation. Unfortunately urinary excretion of catecholamine metabolites were done only in the infants, and not in the mothers. Three of these six infants survived, the authors conclude that infants born of eclamptic or preeclamptic mothers, should be studied intensively for neuroblastoma.-Michel Gilbert
CURRENT TREATMENT OF WILMS' TUMOR. A. T. Uson, J. A. Wolff and P. Tretter. J. Ural. 103:217-221 (February), 1970. The key to success in the management of a Wilms’ tumor is early diagnosis. Nephrectomy is the treatment of choice, with removal of any other affected tissues such as the tail of the pancreas, spleen, diaphragm or the vena cava. Postoperative radiotherapy is generally used and on rare occasions, with very large tumors, preoperative radiation has been helpful. Actinomycin D is used in all cases as soon as the tumor hqs been excised. Proohylactic courses are given at six weeks. three mo-tths, six months, nine months, one year and at fifteen months postoperatively. Vincristine is also effective. When present in a newborn, operation should be delayed until patient can withstand anesthesia which is usually at 48 hours. In the premature the operation might have to be delayed for several weeks. In children under the age of 12 months, radical nephrectomy should be performed as soon as possible and this should be followed by radiotherauy to the renal and tumor beds only if gross or microscopic evidence of local spread or contamination is present. Those older, should undergo nephrectomy with Actinomycin D or Vincristine chemotherapy. Radiotherapy is added if there is evidence of local or regional spread. In children with clinically unresectable tumors, chemotherapy and radiotherapy are started, initially, possibly using Vincristine, and is then followed by operation when the tumor has become resectable. As a result of these modes of treatment the current survival rate is 50-60 per cent overall, and can be as high as 90 per cent in selected groupsBruce
M. Henderson