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Perioperative chemotherapy for primary sarcoma of bone
INTERNATIONAL ABSTRACTS
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distinct advantage in survival if presentation was seen at less than 2 years of age (overall, 74%; synchronous, 89%; metachronous, 59%) as compared with greater than 2 years (overall, 52%; synchronous, 52%; metachronous, 50%). Prognosis seemed to correlate with the highest local stage but was unrelated to tumor size. Overall, the age of presentation of stage V disease was lower than that of other stages with metachronous being younger than synchronous. The presence of unilateral Wilms' tumor obligates close follow-up of the other kidney. All metachronous stage V disease was seen within 31/2 years of initial tumor presentation.--lan T. Cohen and Michael P. Hirsh Genitourinary Rhabdomyosarcoma
in Children. K.R. Loughlin,
A.B. Retik, H.J. Weinstein, et al. Cancer 63:1600-1606, (April 15), 1989. This report describes the management of 36 children with genitourinary tract rhabdomyosarcomas seen at the Childrens Hospital, Dana-Farber Cancer Institute and Joint Center for Radiation Therapy, Boston, from 1957 to 1985. Within the group, survival results in patients with paratesticular primary tumors were the most favorable, with 88% surviving event-free 2 to 27 years following radical orchidectomy. Of interest is one patient in this group who had recurrence in the groin following a negative retroperitoneal node dissection. This patient is a long-range survivor after secondary local excision of the recurrence and irradiation. Six other patients with paratesticular lesions and negative nodes, who did not receive postoperative irradiation, have remained relapse-free. Among patients with bladder-prostate rhabdomyosarcoma, 9 of 15 had initial conservative surgery and six of these are long-term survivors. Only three of these have retained a functional bladder. Among patients with bladder-prostate tumors treated early in the series with initial radical surgery, three of four are long-term survivors. Nine females had rhabdomyosarcomas primary in the reproductive tract, including two in the vagina, three in the cervix, and four in the corpus uteri. Disease-free survival among these nine patients has been 63% during 3 to 16 years of surveillance. All of these patients had hysterectomies, two had cystectomies, and two had vaginal resections. All except one received intensive irradiation which was in a dosage greater than 5,000 rad in four. The large proportion of the total series of patients with primary uterine rhabdomyosarcomas is unique to this series.--Daniel M. Hays Rhabdomyosarcoma Presenting as Disseminated Malignancy From an Unknown Primary Site: A Retrospective Study of Ten Pediatric Cases. E. Etcubanas, S. Peiper, S. Stass, et al. Med Pediatr Oncol
17:39-44, 1989. This study from St. Jude's Children Research Hospital is the first pediatric series reported of rhabdomyosarcoma presenting as metastatic cancer without a detectable primary. Ten patients (4% of all rhabdomyosarcomas treated at St. Jude's Children Research Hospital from 1962 to 1984) were seen with this entity, six of which could be identified as rhabdomyosarcomas at the time of presentation. The remaining four cases were not finally identified until subsequent biopsies were performed for recurrences. Although two patients survived, the median survival time for the others was only 7.5 months. The authors urge continued search for the primary and exact identification of tumor type. The authors feel that in children directed therapy could optimize survival results. However, failure to establish a diagnosis or primary site should not delay starting therapy. Michael P. Hirsh and Ian T. Cohen Perioperative Chemotherapy for Primary Sarcoma of Bone. K.W.
Chan, M. Knowling, and C.P. Beauchamp. Can J Surg 32:43-46, (January), 1989. Because animal studies showed impaired wound healing associated with perioperative chemotherapy, preoperative chemotherapy
for primary osteosarcoma has usually been delayed before operation. Clinical studies have not shown this to be correct, and this small series of eight patients with osteosarcoma and Ewing's sarcoma of the extremities corroborates this opinion. One to three cycles of chemotherapy (doxorubicin, cisplatin, and vincristine) were given preoperatively with the operation performed 1 to 4 days after the last dose. No wound healing or infectious complications occurred, and postoperative chemotherapy was not delayed by the surgery.-Sigmund H. Ein Therapy for Localized Ewing's Sarcoma of Bone. F.A. Hayes, E.l.
Thompson, W.H. Meyer, et al. J Clin Oncol 7:208-213, (February), 1989. Fifty-two patients at St. Jude's Children Research Hospital with localized Ewing's sarcoma of bone were treated with chemotherapy plus surgery or radiation in doses determined by tumor response to chemotherapy. For many years, the most significant factor in prognosis was felt to be the site of the Ewing's primary, regardless of the tumor size. The relapse of patients after initial treatment was also thought to be more commonly metastatic rather than was local recurrence. The St. Jude's data seem to overturn those previously held tenets. Fifty of the 52 patients had initial response to chemotherapy with and without radiation therapy. Seventeen patients relapsed, with 3 metastatic, 10 local, and 4 combined recurrences. It was the size of primary at first presentation (greater than 8 cm) that seemed to correlate best with likelihood of recurrence. Twelve of 14 local recurrences had tumors greater than 8 cm, while only 2 of 22 patients with tumors greater than 8 cm had local recurrence. The 3-year disease-free survival with lesions greater than 8 cm was 64%, compared with 82% for smaller tumors. The overall 5-year survival estimate is approximately 80%, a marked improvement over patients treated before this study.--Michael P. Hirsh and lan T. Cohen Chest Wall Tumors in Infancy and Childhood. R.C. Shamberger, E.G. Holcombe, H.J. Weinstein, et al. Cancer 63:774-785. (February), 1988.
Therapeutic management and results in 17 pediatric patients with chest wall tumors seen at the Children's Hospital, Dana-Farber Cancer Institute, and the Joint Center for Radiotherapy, Boston, from 1976 to 1978 are reviewed. The authors regard Askin's tumor and Ewing's sarcoma of the thoracic cage as a single entity, and 11 of the 17 patients are in this category. Other histologic types in the survey were undifferentiated spindle-cell sarcoma (2) and osteogenic sarcoma, large cell lymphoma, synovial sarcoma, and infantile congenital fibrosarcoma, 1 each. The paramount Askin's tumorEwing's sarcoma group were labeled "malignant small round cell tumors" (MSRCT) and were found in older pediatric patients, among whom 7 of 11 were older than 12 years of age and 4 of 11 were older than 18 years. Nine of 17 patients have survived with a median follow-up of 5 years. Seven patients with localized M S R C T were treated by multimodality therapy, including surgical resection (two after initial chemotherapy), radiotherapy, and intensive chemotherapy. Four of these are disease-free from 16 months to 10 years since diagnosis. Additional experience with a relatively successful primary chemotherapy approach to the largest group of tumors reported in this series is found in "Chest Wall Resection for Ewings' Sarcoma of Rib: An Unnecessary Procedure," by B.N. Rao, F.A. Hayes, and E.I. Thompson in the Annals o f Thoracic Surgery, volume 46, pages 40-44, 1988. A similar survival outcome is reported in J.L. Grosfeld, F.J. Rescorla, K.W. West, et al in "Chest Wall Resection and Reconstruction for Malignant Conditions of Childhood," in Journal of Pediatric Surgery, volume 23, pages 667-673, 1988.--Daniel M. Hays
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distinct advantage in survival if presentation was seen at less than 2 years of age (overall, 74%; synchronous, 89%; metachronous, 59%) as compared with greater than 2 years (overall, 52%; synchronous, 52%; metachronous, 50%). Prognosis seemed to correlate with the highest local stage but was unrelated to tumor size. Overall, the age of presentation of stage V disease was lower than that of other stages with metachronous being younger than synchronous. The presence of unilateral Wilms' tumor obligates close follow-up of the other kidney. All metachronous stage V disease was seen within 31/2 years of initial tumor presentation.--lan T. Cohen and Michael P. Hirsh Genitourinary Rhabdomyosarcoma
in Children. K.R. Loughlin,
A.B. Retik, H.J. Weinstein, et al. Cancer 63:1600-1606, (April 15), 1989. This report describes the management of 36 children with genitourinary tract rhabdomyosarcomas seen at the Childrens Hospital, Dana-Farber Cancer Institute and Joint Center for Radiation Therapy, Boston, from 1957 to 1985. Within the group, survival results in patients with paratesticular primary tumors were the most favorable, with 88% surviving event-free 2 to 27 years following radical orchidectomy. Of interest is one patient in this group who had recurrence in the groin following a negative retroperitoneal node dissection. This patient is a long-range survivor after secondary local excision of the recurrence and irradiation. Six other patients with paratesticular lesions and negative nodes, who did not receive postoperative irradiation, have remained relapse-free. Among patients with bladder-prostate rhabdomyosarcoma, 9 of 15 had initial conservative surgery and six of these are long-term survivors. Only three of these have retained a functional bladder. Among patients with bladder-prostate tumors treated early in the series with initial radical surgery, three of four are long-term survivors. Nine females had rhabdomyosarcomas primary in the reproductive tract, including two in the vagina, three in the cervix, and four in the corpus uteri. Disease-free survival among these nine patients has been 63% during 3 to 16 years of surveillance. All of these patients had hysterectomies, two had cystectomies, and two had vaginal resections. All except one received intensive irradiation which was in a dosage greater than 5,000 rad in four. The large proportion of the total series of patients with primary uterine rhabdomyosarcomas is unique to this series.--Daniel M. Hays Rhabdomyosarcoma Presenting as Disseminated Malignancy From an Unknown Primary Site: A Retrospective Study of Ten Pediatric Cases. E. Etcubanas, S. Peiper, S. Stass, et al. Med Pediatr Oncol
17:39-44, 1989. This study from St. Jude's Children Research Hospital is the first pediatric series reported of rhabdomyosarcoma presenting as metastatic cancer without a detectable primary. Ten patients (4% of all rhabdomyosarcomas treated at St. Jude's Children Research Hospital from 1962 to 1984) were seen with this entity, six of which could be identified as rhabdomyosarcomas at the time of presentation. The remaining four cases were not finally identified until subsequent biopsies were performed for recurrences. Although two patients survived, the median survival time for the others was only 7.5 months. The authors urge continued search for the primary and exact identification of tumor type. The authors feel that in children directed therapy could optimize survival results. However, failure to establish a diagnosis or primary site should not delay starting therapy. Michael P. Hirsh and Ian T. Cohen Perioperative Chemotherapy for Primary Sarcoma of Bone. K.W.
Chan, M. Knowling, and C.P. Beauchamp. Can J Surg 32:43-46, (January), 1989. Because animal studies showed impaired wound healing associated with perioperative chemotherapy, preoperative chemotherapy
for primary osteosarcoma has usually been delayed before operation. Clinical studies have not shown this to be correct, and this small series of eight patients with osteosarcoma and Ewing's sarcoma of the extremities corroborates this opinion. One to three cycles of chemotherapy (doxorubicin, cisplatin, and vincristine) were given preoperatively with the operation performed 1 to 4 days after the last dose. No wound healing or infectious complications occurred, and postoperative chemotherapy was not delayed by the surgery.-Sigmund H. Ein Therapy for Localized Ewing's Sarcoma of Bone. F.A. Hayes, E.l.
Thompson, W.H. Meyer, et al. J Clin Oncol 7:208-213, (February), 1989. Fifty-two patients at St. Jude's Children Research Hospital with localized Ewing's sarcoma of bone were treated with chemotherapy plus surgery or radiation in doses determined by tumor response to chemotherapy. For many years, the most significant factor in prognosis was felt to be the site of the Ewing's primary, regardless of the tumor size. The relapse of patients after initial treatment was also thought to be more commonly metastatic rather than was local recurrence. The St. Jude's data seem to overturn those previously held tenets. Fifty of the 52 patients had initial response to chemotherapy with and without radiation therapy. Seventeen patients relapsed, with 3 metastatic, 10 local, and 4 combined recurrences. It was the size of primary at first presentation (greater than 8 cm) that seemed to correlate best with likelihood of recurrence. Twelve of 14 local recurrences had tumors greater than 8 cm, while only 2 of 22 patients with tumors greater than 8 cm had local recurrence. The 3-year disease-free survival with lesions greater than 8 cm was 64%, compared with 82% for smaller tumors. The overall 5-year survival estimate is approximately 80%, a marked improvement over patients treated before this study.--Michael P. Hirsh and lan T. Cohen Chest Wall Tumors in Infancy and Childhood. R.C. Shamberger, E.G. Holcombe, H.J. Weinstein, et al. Cancer 63:774-785. (February), 1988.
Therapeutic management and results in 17 pediatric patients with chest wall tumors seen at the Children's Hospital, Dana-Farber Cancer Institute, and the Joint Center for Radiotherapy, Boston, from 1976 to 1978 are reviewed. The authors regard Askin's tumor and Ewing's sarcoma of the thoracic cage as a single entity, and 11 of the 17 patients are in this category. Other histologic types in the survey were undifferentiated spindle-cell sarcoma (2) and osteogenic sarcoma, large cell lymphoma, synovial sarcoma, and infantile congenital fibrosarcoma, 1 each. The paramount Askin's tumorEwing's sarcoma group were labeled "malignant small round cell tumors" (MSRCT) and were found in older pediatric patients, among whom 7 of 11 were older than 12 years of age and 4 of 11 were older than 18 years. Nine of 17 patients have survived with a median follow-up of 5 years. Seven patients with localized M S R C T were treated by multimodality therapy, including surgical resection (two after initial chemotherapy), radiotherapy, and intensive chemotherapy. Four of these are disease-free from 16 months to 10 years since diagnosis. Additional experience with a relatively successful primary chemotherapy approach to the largest group of tumors reported in this series is found in "Chest Wall Resection for Ewings' Sarcoma of Rib: An Unnecessary Procedure," by B.N. Rao, F.A. Hayes, and E.I. Thompson in the Annals o f Thoracic Surgery, volume 46, pages 40-44, 1988. A similar survival outcome is reported in J.L. Grosfeld, F.J. Rescorla, K.W. West, et al in "Chest Wall Resection and Reconstruction for Malignant Conditions of Childhood," in Journal of Pediatric Surgery, volume 23, pages 667-673, 1988.--Daniel M. Hays