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R J Andrassy

Publications and source records attributed to R J Andrassy.

At least 19 recordsLinked to original sources
PubMed1999

Progress in the surgical management of vaginal rhabdomyosarcoma: a 25-year review from the Intergroup Rhabdomyosarcoma Study Group.

BACKGROUND/PURPOSE: During its 25 years of experience, the Intergroup Rhabdomyosarcoma Study Group (IRSG) has completed four sequential prospective clinical trials to improve survival and decrease morbidity rates in childhood rhabdomyosarcoma (RMS). Surgical management of vaginal RMS has changed dramatically. METHODS: The records of 72 patients with localized vaginal RMS were reviewed to assess surgical therapy, chemotherapy, and radiotherapy and their relation to outcome. RESULTS: Each successive IRSG trial resulted in decreased need for surgical resection (IRS-I, 100%; IRS-II, 70%; IRS-III, 30%; IRS-IV, 13%) and excellent disease-free survival using increasingly effective multiagent chemotherapy. CONCLUSIONS: Primary chemotherapy after initial biopsy provides excellent tumor control. Local resection may be appropriate, but removal of organs (ie, complete vaginectomy/hysterectomy) has no role except in persistent or recurrent disease. Mature, residual rhabdomyoblasts may be evidence of a chemotherapy response, and further surveillance and biopsy without surgical resection is adequate treatment.

Algorithms↗
PubMed1999

Does debulking improve survival rate in advanced-stage retroperitoneal embryonal rhabdomyosarcoma?

UNLABELLED: BACKGROUND, METHODS, AND PURPOSE: The authors examined demographic and clinical features, therapy, and outcome of patients with advanced (group III or IV) rhabdomyosarcoma (RMS) of the retroperitoneum and nongenitourinary pelvis treated in the Intergroup Rhabdomyosarcoma Study Group (IRSG) III (1984 to 1991, n = 41) or IV pilot (1987 to 1991, n = 53) studies to assess the role of initial debulking surgery. RESULTS: Ninety-four patients with retroperitoneal primary tumors and gross locoregional residual tumor (group III, n = 53) or metastatic disease (group IV tumors, n = 41) were treated with combination chemotherapy (ie, vincristine, dactinomycin, and cyclophosphamide with or without other agents plus radiation therapy, RT) after biopsy only or subtotal resection. These retroperitoneal tumors usually were invasive (T2, 76%). Most patients were younger than 10 years of age (n = 69, 73%), the male to female ratio was 1.4, and tumors usually were embryonal (n = 64, 68%). Overall 4-year failure-free survival (FFS) was 50%; survival was 60%. Survival rate was better for girls (4-year survival rate, 75% v49% for boys; P = .05) and was not significantly different for patients treated in IRS-III (66%) or IRS-IV pilot (52%). However, it was better for patients with embryonal versus alveolar or undifferentiated tumors (4-year survival rate, 70% v 42%; P = .002). In adolescents, RMS is different from that seen in children less than 10 years old; most cases are alveolar or undifferentiated (16 of 29, 55%). Surgery for most (21 of 24) patients with alveolar tumors comprised biopsy only. By contrast, of 64 patients with embryonal tumors, 39 (61%) underwent biopsy only, whereas 25 (39%) had debulking surgery. Patients whose tumors were debulked fared better than those whose tumors underwent biopsy only (4-year FFS rate, 72% v48%; P = 0.03). Patients with group IV embryonal tumors fared unexpectedly better than those with group IV alveolar or undifferentiated tumors (70% versus 42% 4-year survival rate, P < .05), and patients less than 10 years of age with group IV embryonal tumors had 4-year survival rate of 77%, indicating the importance of the biology of these tumors. CONCLUSIONS: Multimodal therapy, including multiagent chemotherapy plus RT, appears to improve survival rate in patients with advanced embryonal RMS arising in the retroperitoneum. These data suggest that debulking tumors of embryonal histology improves outcome further. This approach will be assessed in IRSG V.

Adolescent↗
PubMed1998

Operative treatment of truncal vascular injuries in children and adolescents.

BACKGROUND/PURPOSE: Pediatric truncal vascular injuries are rare, but the reported mortality rate is high (35% to 55%), and similar to that in adults (50% to 65%). This report examines the demographics, mechanisms of injury, associated trauma, and results of treatment of pediatric patients with noniatrogenic truncal vascular injuries. METHODS: A retrospective review (1986 to 1996) of a pediatric (< or = 17 years old) trauma registry database was undertaken. Truncal vascular injuries included thoracic, abdominal, and neck wounds. RESULTS: Fifty-four truncal vascular injuries (28 abdominal, 15 thoracic, and 11 neck injuries) occurred in 37 patients (mean age, 14+/-3 years; range, 5 to 17 years); injury mechanism was penetrating in 65%. Concomitant injuries occurred with 100% of abdominal vascular injuries and multiple vascular injuries occurred in 47%. Except for aortic and one SMA injury requiring interposition grafts, these wounds were repaired primarily or by lateral venorrhaphy. Nonvascular complications occurred more frequently in patients with abdominal injuries who were hemodynamically unstable (systolic blood pressure [BPS] <90) on presentation (19 major complications in 11 patients versus one major complication in five patients). Thoracic injuries were primarily blunt rupture or penetrating injury to the thoracic aorta (nine patients). Thoracic aortic injuries were treated without bypass, using interposition grafts. In patients with thoracic aortic injuries, there were no instances of paraplegia related to spinal ischemia (clamp times, 24+/-4 min); paraplegia occurred in two patients with direct cord and aortic injuries. Concomitant injuries occurred with 83% of thoracic injuries and multiple vascular injuries occurred in 25%. All patients with thoracic vascular injuries presenting with BPS of less than 90 died (four patients), and all with BPS 90 or over survived (eight patients). There were 11 neck wounds in 9 patients requiring intervention, and 8 were penetrating. Overall survival was 81%; survival from abdominal vascular injuries was 94%, thoracic injuries 66%, and neck injuries 78%. CONCLUSIONS: Survival and subsequent complications are related primarily to hemodynamic status at the time of presentation, and not to body cavity or vessel injured. Primary anastomosis or repair is applicable to most nonaortic wounds. The mortality rate in pediatric abdominal vascular injuries may be lower than previously reported.

Abdomen↗
PubMed1998

Nutritional support of the pediatric oncology patient.

The child with a malignancy frequently will have associated cachexia with significant weight loss and malnutrition. The reasons for this are multifactorial and may be related directly to the tumor, such as increased metabolic rate, circulating peptides leading to anorexia, and decreased intake due to poor appetite or gut involvement. There appears to be other reasons involved, including increased whole body protein breakdown, increased lipolysis, and increased gluconeogenesis. Release of certain cytokines, such as tumor necrosis factor, interleukin-1, interleukin-6, and others may increase the cancer cachexia. Malnutrition in these children leads to intolerance of chemotherapy and radiotherapy as well as increased local and systemic infections. For many years, oncologists were hesitant to provide nutrition support to cancer patients for fear that tumor growth would be enhanced. Pediatric oncologists learned early that starvation plays no positive role in cancer therapy. Adjunctive nutritional support, either enterally or parenterally, supports the patient during therapy with surgery, chemotherapy, or radiation. Many studies have now shown that the nutritionally replete patient tolerates therapy better and in some pediatric malignancies may enhance survival.

Cachexia↗
PubMed1998

Thoracic sarcomas in children.

OBJECTIVE: This study reviews the experience of the Intergroup Rhabdomyosarcoma Studies (IRS) II and III with thoracic sarcomas. SUMMARY BACKGROUND DATA: Thoracic sarcomas in children present special problems with histologic diagnosis, total resection of tumor, and local control. Previous studies have shown high rates of local and distant relapse. METHODS: Data from evaluable patients enrolled in IRS II and IRS III were reviewed. Progression-free and overall survival were estimated by the Kaplan-Meier method and statistical differences were calculated by the log-rank test. The multivariate regression analysis of data was performed using the Cox proportional hazards model. RESULTS: A total of 84 patients presented with thoracic sarcomas during IRS II (51 patients) and IRS III (33 patients). Of these, 76 were chest wall tumors, 3 were lung tumors, 4 were pleural tumors, and 1 arose from the heart. The average age at diagnosis was 8.5 years (range, 1 month-20.5 years). Thirty-three patients had alveolar histology, 17 had embryonal histology, and 12 had extraosseous Ewing's sarcoma, with indeterminate, undifferentiated, and pleomorphic histologies in the remaining 22 patients. Thirteen patients presented as group I (localized disease completely resected), 18 as group II (microscopic residual or nodal disease), 31 as group III (gross residual disease or biopsy only), and 22 as group IV (distant metastatic disease). Sixty patients (71%) achieved a complete response. Thirty-nine patients had a local relapse, and 22 had a distant relapse. Thirty-five patients (42%) survived with a mean follow-up of 1.8 years (range 0.7-11.7 years); 49 (58%) have died with an average survival of 1.1 years (range 0.3-11.7 years). Four patients (5%) died of infection or treatment complications. Progression-free survival was not significantly associated with histology, site, clinical group, or IRS study. Overall survival was significantly associated with clinical group, size, and local or distant recurrence, but not with histology or IRS study by univariate analysis. In a multivariate analysis, only clinical group and local and distant recurrence showed statistical significance (p < 0.002, < 0.006, and < 0.001, respectively). CONCLUSIONS: As expected, outcome was better for patients with totally resected or microscopically residual tumor after resection. A higher rate of recurrence in group I versus group II,although not statistically significant, suggests that identification of margin status may be difficult, and these patients may require more intensive efforts for local control. These patients continued to have a high rate of local and distant relapse, and new strategies for preventing these are under development.

Adolescent↗
PubMed1997

Primary and metastatic rhabdomyosarcoma in the breast: neoplasms of adolescent females, a report from the Intergroup Rhabdomyosarcoma Study.

UNLABELLED: The occurrence of rhabdomyosarcoma (RMS) primary in or metastatic to breast has been regarded as an uncommon event, associated with an unfavorable outcome. Records of 26 patients with diagnoses of breast RMS, either primary or secondary, entered in the Intergroup Rhabdomyosarcoma Study (IRS) (1972-1992) were reviewed and compared with data regarding 47 similar patients in published reports. Of the 26 IRS cases, the histologic subtype was alveolar in 24, embryonal in 1, and not determined in 1. All were female with ages ranging from 11.5 to 20.2 years (median, 15.2 years; mode, 14-16 years). This compact age distribution of both primary (n = 7) and metastatic (n = 19) breast RMS was seen in previously reported series. Among the 19 cases of RMS with initial dissemination to breast, primary tumor sites, were extremity (n = 8), nasopharynx/paranasal sinuses (n = 7), and trunk (n = 4). IRS treatment was risk-based according to site and extent of disease. Four of 7 patients with primary RMS remain disease free 2.9 to 7 years post diagnosis. Among 19 patients with RMS initially metastatic to breast, including 7 in IRS clinical group IV at original diagnosis, three are disease free at 7.6, 15.7 and 17.0 years. CONCLUSIONS: primary or metastatic RMS in breast is almost confined to adolescent females having tumors with alveolar histology. Approximately one-half of the patients with primary breast disease and 15% of those with metastatic breast disease as an initial recurrence are long-term survivors.

Adolescent↗
PubMed1997

Second malignant neoplasms in children after treatment of soft tissue sarcoma.

Currently, approximately 67% of children diagnosed with cancer can be expected to survive more than 5 years. Among the most significant late effects of cancer therapy is the development of second malignant neoplasm (SMN). This study was performed to identify the factors associated with the development of second malignant neoplasms after treatment for soft tissue sarcomas in childhood. Retrospectively the charts of 20 children who developed second malignant neoplasms after treatment for primary childhood soft tissue sarcoma were reviewed. Presentation, age at diagnosis, tumor histology, extent of tumor, treatment, family histories (when available), and outcome were recorded. The mean age of the patients (10 boys, 10 girls) was 8.5 years of age (range, 1 to 20 years). Most primary tumors were rhabdomyosarcoma (14/20) and occurred in an extremity (10/20). Ninety percent of the patients (18/20) had a complete response to treatment of the primary cancer. Eleven out of 20 received combined chemotherapy and radiation therapy. The most common secondary malignancy was a bone sarcoma (6/20), followed by brain tumors (n = 3), leukemia (n = 2), and other sarcomas (n = 2). Four of the bone sarcomas developed in the field of radiation treatment. Median follow-up was 16 years (range, 1 to 26 years). The median time to development of a SMN was 11.4 years (range, 1.5 to 21 years). Survival after a second malignancy was only 30%. Two patients developed a third malignant neoplasm. The occurrence of a secondary malignancy represents a serious complication of childhood cancer. Certain tumors are related directly to treatment such as osteosarcoma within irradiated fields and secondary leukemias or lymphomas after certain chemotherapy regimens. Combined radiotherapy and chemotherapy may play an additive role in the development of second malignant neoplasms. Genetic factors may predispose affected patients to the development of both primary and secondary malignancies. Close surveillance of children previously treated for childhood cancers is warranted.

Adolescent↗
PubMed1997

Rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of children. Whereas adult sarcomas are mostly extremity tumors and rarely spread to regional nodes, RMS is ubiquitous, occurring almost everywhere in the body (most commonly in the head, neck, and genitourinary area), and frequently spreads to regional nodes (40% in extremity RMS). Survival has improved with each successive intragroup study, and surgical intervention, although integral for survival, is less mutilating.

Child↗
PubMed1997

Pulmonary contusion: are children different?

BACKGROUND: Pulmonary contusion (PC) is a common sequelae of blunt trauma in adults and children; previous reports suggest that children have more favorable outcomes because of differences in mechanisms of injury, associated injury, and physiologic response. Our objective was to determine whether children who sustain PC have different outcomes compared with similarly injured adults. STUDY DESIGN: Our Level I Trauma Registry was reviewed for a 4-year period and identified 251 consecutive patients who sustained PC. Their charts were reviewed retrospectively for demographics, injury mechanism, injury severity scores, associated injuries, and outcomes (measured by the need for intubation, ventilation days, pneumonia, acute respiratory distress syndrome, and death). Data are expressed as the mean +/- SEM. The Student's t-test was used to compare the groups. A p value less than 0.05 was considered significant. RESULTS: Of the study patients, 41 (16%) were children (ages 2-16, mean 10 years) and 210 (84%) were adults (ages 17-80, mean 34 years). The most common injury mechanisms in children were motor vehicle accidents (56%) and auto-pedestrian accidents (39%), but in adults, motor vehicle accidents (80%, p = 0.02) predominated. Injury severity score was not significantly different between groups (children, 26 +/- 2 and adults 25 +/- 1). Similarly, the incidence of associated injuries was not different between children and adults: head 78% versus 62%, abdomen 59% versus 43%, and skeletal fractures 41% versus 29%, respectively. Neither need for intubation, ventilator days, pneumonia, acute respiratory distress syndrome, or death differed significantly between groups. CONCLUSIONS: Although children and adults differ in regard to injury mechanism, their overall injury severity, associated injuries, and outcomes are quite similar. Thus, contrary to previous reports, children do not have a more favorable outcome after PC.

Accidents, Traffic↗
PubMed1996

Fibromyxoid sarcoma in a four-year-old child: case report and review of the literature.

We present a child with a rare and chemotherapy-resistant form of soft-tissue cancer, low-grade fibromyxoid sarcoma, first noted when he was 4 years old. He is the youngest patient reported to date. An 11-year-old white male presented to. The University of Texas M.D. Anderson Cancer Center's Department of Pediatrics with a 7-year history of right thigh mass and pulmonary nodules, confirmed on examination. He had undergone extensive prior chemotherapy and surgery. He received chemotherapy with high-dose cyclophosphamide (7 g/m2) and later etoposide (150 mg/m2/day x 5), with only slight shrinkage of the thigh mass and none in the lungs. Subsequently the tumor in his proximal thigh and his lung metastases were resected, and radiation therapy was administered to the thigh. His disease remained stable for 12 months, but he then developed a pleural-based metastasis on the left side and new bilateral lung metastases also. The tumors on the left side were removed; residual disease is stable after treatment for 6 months with subcutaneous alpha-interferon-2b. Low-grade fibromyxoid sarcoma is very uncommon in children. It grows slowly and metastasizes to distant organs, chiefly to the lungs. It is resistant to conventional chemotherapy, and thus far only surgery seems to have a life-prolonging effect. Newer chemotherapeutic and possibly biologic agents should be tried in future patients, in order to find an effective way to control the disease.

Child↗
PubMed1996

Melanoma as a second malignant neoplasm after childhood cancer.

BACKGROUND: As more children survive childhood cancers, the population at risk for second malignant tumors increases. The development of melanoma as a second malignant tumor is not well described. METHODS: The M.D. Anderson Cancer Center's 50-year experience with patients who developed melanoma after treatment of a childhood cancer was retrospectively reviewed. RESULTS: One hundred seventy-two patients with a second malignancy were identified; 11 patients had melanoma as a second malignancy. The most common first malignancies were Hodgkin's disease, brain tumors, and retinoblastomas. Melanoma developed in an irradiated field in 4 patients. Six patients had lymphatic or distant metastasis at diagnosis. Five of 11 patients died of melanoma. CONCLUSIONS: Factors contributing to melanoma as a second malignancy may include genetic factors and the effects of chemotherapy and radiation. Survivors of childhood malignancy should be considered at risk for developing melanoma, and suspicious pigmented lesions should be carefully evaluated.

Child↗
PubMed1996

Malignant fibrous histiocytoma in children.

Because malignant fibrous histiocytoma (MFH) rarely occurs in children, the natural history of this tumor and prognostic factors predictive of outcome have not been well described. The charts of all pediatric patients with MFH seen at M.D. Anderson Cancer Center were reviewed with respect presentation, treatment, and outcome, in an attempt to determine prognostic factors that are predictive of survival. Forty-four pediatric patients were identified. Extremities were the most common tumor site (31 of 44 patients). Five patients presented with angiomatoid histology subtype; all subsequently survived. The estimated 5-year survival rate was 85% for clinical group I patients, 87% for clinical group II, 53% for clinical group III, and 0% for clinical group IV. The estimated 5-year survival rate was 95% for patients with tumors of less than 5 cm in diameter and 45% for those with larger tumors. Overall, the estimated 5-year survival rate was 71%. Significant prognostic factors found to affect survival (by univariate analysis) were clinical group, tumor size, and recurrence. Gender and race were not significant predictors. The use of chemotherapy and radiation was not found to improve the chance of survival, but this most likely reflected the more frequent use of adjuvant therapy in patients with unresectable or high-grade tumors. Although adequate surgical resection continues to be the most effective treatment, investigation of adjuvant chemotherapy and radiation therapy on protocol is warranted.

Adolescent↗
PubMed1996

The argument for conservative, delayed surgery in the management of prostatic rhabdomyosarcoma.

Exenteration is no longer required for most patients who have rhabdomyosarcoma (RMS) of the prostate. This site comprised only about 5% of newly diagnosed cases in the IRS-III (1984-1991). The mean age at the time of diagnosis was 5.3 yrs (range, 0 to 19 years). Most tumors were relatively large, had embryonal histology, and were clinically localized but unresectable without major loss of organ function. The 44 patients with group III tumors (gross residual disease) were treated according to the IRS-III protocol. Forty-three of them underwent biopsy only, and one patient had subtotal resection as the initial procedure. The average number of surgical procedures per patient was two (range, one to five). Six of the 44 patients had no additional surgery. The second-look procedures performed in the other 38 patients included exenteration (14), prostatectomy (7), cystoscopic/perineal needle biopsy (8), laparotomy with biopsy (6), and subtotal excision with bladder salvage (3). Additional surgery was required for four patients, for evaluation of a residual mass, postoperative fistula, ureteral stricture, or small bowel obstruction. Six patients with relapse or residual disease underwent additional chemotherapy and late exenteration (3), prostatectomy (1), or biopsy (2). Four of the six have been cured, one is in treatment for a second malignancy, and the other has residual disease after exenteration. Thirty-six of the 44 patients with group III tumors have been cured (minimum follow-up period, 6 years; range, 6 to 11 years), compared with 23 of the 47 in IRS-II (1978-1984) (P = .001). Two of the six deaths in this group were caused by infection. The bladder salvage rate for those cured of their disease also was better (64% v 57% for IRS-II). The two patients with group IIA tumors were cured by gross primary excision, local radiotherapy, and vincristine and actinomycin therapy. By contrast, all patients with metastatic disease (group IV) died of the tumor. Conservative, delayed surgery, performed after intensive chemotherapy with or without radiotherapy, yields a better cure rate while maintaining a high rate of bladder salvage in children with group III prostatic RMS.

Adolescent↗
PubMed1996

Extremity sarcomas: an analysis of prognostic factors from the Intergroup Rhabdomyosarcoma Study III.

PURPOSE: Prognostic factors for extremity sarcomas have been reported previously, after analysis of Intergroup Rhabdomyosarcomas Studies (IRS) I and II. This report reviews the experience of IRS III (1984-1992), in light of these reported factors, and the pretreatment factors used in the staging system currently being evaluated in IRS IV. The results of treatment of extremity sarcomas in IRS III are reported. METHODS: The charts of all patients entered in IRS III with an extremity-site tumor were reviewed. This group included patients with shoulder girdle and buttock sites. All patients were treated according to IRS III protocols. Survival rates were estimated by the method of Kaplan and Meier, and comparisons among groups of patients were made using a log-rank test. A multivariate analysis was performed to analyze all pretreatment factors that were significant by univariate analysis. RESULTS: Of the 189 patients entered in IRS III with extremity sites, 88 (47%) had the most common alveolar histology. Fifty-nine patients were in group I at the time of presentation (completely resected disease), 48 in group II (microscopic residual disease), 36 in group III (gross residual disease), and 46 in group IV (metastatic disease). By univariate analysis, the significant prognostic factors affecting survival were clinical group, age at time of diagnosis, tumor size, distant metastases, nodal metastases, and local and distant recurrence. By multivariate analysis of pretreatment factors, age at time of diagnosis, nodal metastases, and distant metastases were significant prognostic factors affecting survival; tumor size approached significance. Both the clinical group system and pretreatment staging system (Lawrence/Gehan) predicted significant differences in survival between groups of patients. The lower survival rate among group II and III node-negative patients with a distal tumor (who, with more extensive surgery, could have been in group I) in comparison to group I patients with a distal lesion, approached significance. In patients without distant metastases, survival was significantly different in those patients that had negative nodes from those in whom nodes were not biopsied. CONCLUSION: This review confirms that both clinical grouping and the new pretreatment staging system used in IRS IV can predict the likelihood of survival of children with extremity sarcomas. By multivariate analysis of the elements included in the staging system, nodal metastases, distant metastases, and tumor size were useful in predicting survival. In addition, age was a significant predictor. This study confirms previous suggestions that complete excision with gross and microscopically negative margins is preferable in the treatment of children with extremity rhabdomyosarcomas.

Adolescent↗
PubMed1996

Surgical management of rhabdomyosarcoma in children.

This review of the past year's literature summarizes the most relevant advances in the surgical treatment of rhabdomyosarcomas. Improved responses to multimodality therapy and advances in surgical techniques have allowed for more aggressive surgical resection in some patients and less extensive resections in others. The Intergroup Rhabdomyosarcoma Study committee continues to examine prognostic factors in attempts to provide risk-adapted therapy. Development and evaluation of specific recommendations for surgical treatment by site of tumor continue to be a focus of the Intergroup Rhabdomyosarcoma Study. The overall trend is toward less radical surgical intervention with organ preservation.

Chemotherapy, Adjuvant↗
PubMed1995

Malignant solid tumors in neonates: a 40-year review.

To evaluate the outcome of neonatal malignant solid tumors, we reviewed the records of 222 infants under the age of 1 year with malignant disease who were treated at the University of Texas M.D. Anderson Cancer Center over a 40-year period. Forty-five cases of neonatal (< 30 days old at the time of presentation) malignancies were found. Thirty-two infants had solid tumors and form the basis of this report. Diagnoses included soft tissue sarcoma (13), brain tumor (5), neuroblastoma (6), retinoblastoma (3), malignant melanoma (2), hemangiopericytoma (2), and nephroblastoma (1). The mean age at which initial signs and symptoms were noted was 9 days of life. Fifty-nine percent (19) presented within the first week of life, and 47% (15) presented at birth. The mean age at histological diagnosis was 54 days. The head and neck region was the most common site (18), followed by trunk (9), and extremities (5). Thirty-one patients underwent surgical resection of the primary tumor. Thirteen of those neonates received no additional chemotherapy and/or radiation therapy, whereas 18 received some combination of surgery plus perioperative chemotherapy and/or radiation therapy. Overall survival was 78% (25 of 32) with an average follow-up of 8 years (range, 2 months to 29 years). There were no survivors among those patients with distant metastatic disease at the time of diagnosis. Despite delays, prognosis is excellent in the absence of distant metastatic disease, particularly for extracranial tumors.

Brain Neoplasms↗
PubMed1995

Conservative management of uterine pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III and IV pilot.

Previous studies have suggested that women with uterine rhabdomyosarcomas (RMS) represent a distinct group of patients who present at an older age, are less responsive to treatment, and have a poorer prognosis than patients with vaginal RMS. During the Intergroup Rhabdomyosarcoma Study (IRS) III and the IRS IV pilot study, 14 patients were registered with uterine primary RMS. Three patients presented with cervical tumors that were completely removed (group 1). Eight patients had initial biopsies with gross residual disease (group 3), and 3 had metastatic disease at presentation (group 4). Of the 5 patients treated with primary chemotherapy or chemotherapy and radiation, 2 had delayed hysterectomy and vaginectomy, 1 had no further surgery, and 2 had exploratory laparotomy with no evidence of disease. There were no relapses or deaths in this group. One patient underwent initial resection of a broad ligament mass, experienced an early (3-week) recurrence of the mass while on chemotherapy, and progressed to developing distant metastases and death. Four patients died of chemotherapy toxicity or sepsis, one after achieving a complete response from chemotherapy and hysterectomy. This primary chemotherapy or chemotherapy and radiotherapy regimen resulted in 8 of 9 (89%) patients (not including those who died of chemotoxicity) surviving between 1.5 and 6 years without evidence of disease. Of the surviving patients, 2 had hysterectomy and vaginectomy, but pathological specimens showed only localized microscopic residual tumor. This report suggests that less vigorous operative resection may be possible in combination with primary chemotherapy when treating uterine rhabdomyosarcomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗