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Two unusual congenital tumours with long-term follow-up after local surgical excision
INTERNATIONAL
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surgical excision was performed and both patients grouped as stage III favorable histology. Postoperative treatment included chemotherapy and radiotherapy. The patients are clinically well at follow-up I years and 20 months, respectively.-ThomasA. Angerpointner Stage III Neuroblastoma Over 1 Year of Age at Diagnosis: Improved Survival With Intensive Multimodality Therapy Including Multiple Alkylating Agents. D.C. West, R.C. Shamberger, R. Mucklis, et al. J Clin Oncol 1184-90, (January), 1993.
This Dana Farber Cancer Institute study looked at a new chemotherapy regimen (MADDOC) consisting of mechlorethamine, doxorubicin, cisplatin, dacarbazine, vincristine, and cyclophosphamide. When combined with surgery and radiation, this treatment provided a 72% event free survival after 85 months of follow-up in a group of children, stage III neuroblastoma patients > 1 year old, with notoriously poor prognoses. Patients with better histology did have a better prognosis, while patients in which cytogenetic tumor analysis showed N-myc amplification had a poorer prognosis. This approach is aggressive but provides new hope for success in these patients. Further study will be necessary.-Michael P. Hirsh Cisplatin, Vincristine, and Fluorouracil Therapy for Hepatoblastoma: A Pediatric Oncology Group Study. E.C. Douglass, M. Rqvnolds, M. Finegold, et al. J Clin Oncol 11:96-99, (January),
1993. The Pediatric Oncology Group evaluated a new form of comhination chemotherapy in pediatric patients with all stages of hepatoblastoma. This combination had apparently similar results to protocols containing the drug doxorubicin without some of the severe side effects such as mucositis and cardiac toxicity. Sixty patients were studied. Nineteen of 21 (90%) with stage I or II disease survived. Twenty-four of 31 (67%) with stage III disease (incomplete resection) had a disease-free survival of 4 years. Only 1 of 4 patients with stage IV attained remission and survived. This protocol will be studied further but lends some optimism to the treatment of this pediatric tumor.-Michael P. Hirsh
ABSTRACTS
Second Malignant Neoplasms in Children Treated for Rhabdomyosarcoma. R. Heyn, V. Haeberlen, W.A. Newton, et al, for the Intergroup Rhabdomyosarcoma Study Committee (IRS). J Clin On-
co1 11:262-270, (February), 1993. This retrospective review of 1770 patients with primary rhabdomyosarcoma (RMS) entered into IRS I or IRS II, found 22 patients who developed secondary malignant neoplasms (SMN) postsurviva1of treatment of RMS. The most common type of RMS was bone sarcoma, followed by acute nonlymphoblastic leukemia. These SMN’s appeared approximately 7 years after the original RMS with a cumulative incidence density of 1.7%. There was a disproportionately high number of SMN patients with strong family histories of neurofibromatosis (NF) or with NF themselves, with Li-Fraumeni syndrome (LFS), cancer family history syndrome, or with combination radiation therapy and alkylating agent containing chemotherapy. This review will help give practitioners some clues as to who is likely to develop SMNs after successful survival from therapy of RMS.-Michael
P. Hirsh
Two Unusual Congenital Tumours With Long-Term Follow-Up After Local Surgical Excision. C.L. James, N.H. Bramwell, R.B. Davey, et al. Pediatr Surg Int 8:270-273, (April), 1993.
A case of congenital fibrosarcoma overlying the left scapula and a case of hemangiopericytoma on the right side of the face, who were treated with local excision and followed-up for 18 and 15 years, respectively, are reported. Both these patients had focal histological tumor involvement of the surgical margins. Neither received radiotherapy or chemotherapy. The authors note that the metastatic rate drops in children under 10 years with fibrosarcoma in contrast to adult patients. Subsequent DNA ploidy analysis showed predominantly diploid cell population in both the tumors and may he an indicator of a benign course. However, further investigations are necessary to predict the prognostic role of DNA ploidy analysis. The authors emphasize the reasonable cosmetic and functional results with local surgical resection in these congenital tumors.-R. Surana
1516
surgical excision was performed and both patients grouped as stage III favorable histology. Postoperative treatment included chemotherapy and radiotherapy. The patients are clinically well at follow-up I years and 20 months, respectively.-ThomasA. Angerpointner Stage III Neuroblastoma Over 1 Year of Age at Diagnosis: Improved Survival With Intensive Multimodality Therapy Including Multiple Alkylating Agents. D.C. West, R.C. Shamberger, R. Mucklis, et al. J Clin Oncol 1184-90, (January), 1993.
This Dana Farber Cancer Institute study looked at a new chemotherapy regimen (MADDOC) consisting of mechlorethamine, doxorubicin, cisplatin, dacarbazine, vincristine, and cyclophosphamide. When combined with surgery and radiation, this treatment provided a 72% event free survival after 85 months of follow-up in a group of children, stage III neuroblastoma patients > 1 year old, with notoriously poor prognoses. Patients with better histology did have a better prognosis, while patients in which cytogenetic tumor analysis showed N-myc amplification had a poorer prognosis. This approach is aggressive but provides new hope for success in these patients. Further study will be necessary.-Michael P. Hirsh Cisplatin, Vincristine, and Fluorouracil Therapy for Hepatoblastoma: A Pediatric Oncology Group Study. E.C. Douglass, M. Rqvnolds, M. Finegold, et al. J Clin Oncol 11:96-99, (January),
1993. The Pediatric Oncology Group evaluated a new form of comhination chemotherapy in pediatric patients with all stages of hepatoblastoma. This combination had apparently similar results to protocols containing the drug doxorubicin without some of the severe side effects such as mucositis and cardiac toxicity. Sixty patients were studied. Nineteen of 21 (90%) with stage I or II disease survived. Twenty-four of 31 (67%) with stage III disease (incomplete resection) had a disease-free survival of 4 years. Only 1 of 4 patients with stage IV attained remission and survived. This protocol will be studied further but lends some optimism to the treatment of this pediatric tumor.-Michael P. Hirsh
ABSTRACTS
Second Malignant Neoplasms in Children Treated for Rhabdomyosarcoma. R. Heyn, V. Haeberlen, W.A. Newton, et al, for the Intergroup Rhabdomyosarcoma Study Committee (IRS). J Clin On-
co1 11:262-270, (February), 1993. This retrospective review of 1770 patients with primary rhabdomyosarcoma (RMS) entered into IRS I or IRS II, found 22 patients who developed secondary malignant neoplasms (SMN) postsurviva1of treatment of RMS. The most common type of RMS was bone sarcoma, followed by acute nonlymphoblastic leukemia. These SMN’s appeared approximately 7 years after the original RMS with a cumulative incidence density of 1.7%. There was a disproportionately high number of SMN patients with strong family histories of neurofibromatosis (NF) or with NF themselves, with Li-Fraumeni syndrome (LFS), cancer family history syndrome, or with combination radiation therapy and alkylating agent containing chemotherapy. This review will help give practitioners some clues as to who is likely to develop SMNs after successful survival from therapy of RMS.-Michael
P. Hirsh
Two Unusual Congenital Tumours With Long-Term Follow-Up After Local Surgical Excision. C.L. James, N.H. Bramwell, R.B. Davey, et al. Pediatr Surg Int 8:270-273, (April), 1993.
A case of congenital fibrosarcoma overlying the left scapula and a case of hemangiopericytoma on the right side of the face, who were treated with local excision and followed-up for 18 and 15 years, respectively, are reported. Both these patients had focal histological tumor involvement of the surgical margins. Neither received radiotherapy or chemotherapy. The authors note that the metastatic rate drops in children under 10 years with fibrosarcoma in contrast to adult patients. Subsequent DNA ploidy analysis showed predominantly diploid cell population in both the tumors and may he an indicator of a benign course. However, further investigations are necessary to predict the prognostic role of DNA ploidy analysis. The authors emphasize the reasonable cosmetic and functional results with local surgical resection in these congenital tumors.-R. Surana