The value of postoperative radiotherapy in childhood nonrhabdomyosarcoma soft tissue sarcoma

OBJECTIVE: To determine the value of postoperative radiotherapy (RT) in the management of nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) of childhood. PROCEDURE: From 1964 to 2000, 62 children with a median age of 14 years were seen at the University of Iowa and underwent a wide local excision for non-metastatic NRSTS. Tumors were high grade in 36 (58%) and >5 cm in 24 (39%). Margins of resection were negative (Group I) in 37 (60%) and positive (Group II) in 25 (40%). Postoperative RT was delivered to 20 patients (32%); eight of 37 (22%) Group I and 12 of 25 (48%) Group II children received postoperative RT. Chemotherapy was employed in 19 patients (31%). Median follow-up was 9.6 years. RESULTS: The 5- and 10-year overall survival rates for Group I were 69 and 63% and for Group II were 66 and 60%. The 5- and 10-year local control rate was 66%. On multivariate analysis, size of tumor (P < 0.001) and postoperative RT (P = 0.017) were prognostic factors for local control. All 13 Group I children with low grade, 相似文献   

Adjuvant chemotherapy for the treatment of advanced pediatric nonrhabdomyosarcoma soft tissue sarcoma: the National Cancer Institute experience

BACKGROUND: The survival of children and adolescents with advanced (unresectable or metastatic) nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) is poor. In order to clarify the role of combining chemotherapy with aggressive local control using surgery and/or radiation, we reviewed our institutional experience with the treatment of advanced pediatric NRSTS. PROCEDURE: We reviewed the charts of all patients less than 21 years treated for an advanced NRSTS at the National Cancer Institute (NCI) between 1983 and 2003. Tumor pathology was confirmed and demographic, disease, and treatment data were abstracted. Survival was calculated using standard methods. RESULTS: Of the 25 patients who were treated over the study period, 15 had metastatic disease and 10 had unresectable or incompletely resected disease at presentation. Twenty-one patients received chemotherapy consisting of the combination of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide, and the remaining 4 received regimens that included doxorubicin. Twenty patients (80%) had a complete (5/25) or partial (15/25) response after chemotherapy alone. After the combination of chemotherapy and local control, 14 patients (56%) had a complete response (CR). The estimated 5-year overall and event-free survival (EFS) for all patients was 0.50 (standard error = 0.11) and 0.34 (standard error = 0.10), respectively. CONCLUSIONS: The combination of chemotherapy with aggressive local control in this cohort of pediatric patients with advanced NRSTS yielded results comparable to those observed in patients with advanced sarcomas that are chemotherapy responsive. Prospective randomized trials are needed to quantify the contribution of chemotherapy and to determine the ideal regimen.     

Current management of pediatric soft tissue sarcomas

Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.     

Favorable response to soft tissue sarcoma therapy in an adolescent with embryonal renal sarcoma

Embryonal renal sarcomas were first identified in 1995 among banked tumor samples originally classified as adult Wilms tumor. Few long-term remissions were observed when these rare tumors were treated with chemotherapy usually used for childhood Wilms. Data were collected from the medical record of an adolescent female with embryonal renal sarcoma and treated with sarcoma-directed chemotherapy and radiation. At 66 months following diagnosis, the patient has no evidence of tumor but has experienced severe renal dysfunction and ovarian failure. We believe there is a subset of patients with disseminated embryonal renal sarcoma that respond to intense sarcoma-directed therapy.     

High-dose-rate brachytherapy in childhood sarcomas: A local control strategy preserving bone growth and function

The administration of external beam radiation therapy (EBRT) has been an integral part of the successful treatment of childhood sarcomas. However, EBRT has severe late morbidity in the developing child. In an attempt to deliver adequate tumoricidal radiation while preserving bone growth and organ function, 13 children with diverse sarcomas were treated with high dose rate brachytherapy (HDR). Seven patients had rhabdomyosarcoma and six patients had other soft tissue sarcoma variants. All patients were treated with disease-appropriate chemotherapy, usually according to the Intergroup Rhabdomyosarcoma Study. Eleven patients received fractionated 36 Gy HDR alone at a mean of 3.5 months from diagnosis. Two patients received 10–12.5 Gy intraoperative HDR brachytherapy and additional 27 Gy EBRT. Nine of 11 patients in first remission have had no recurrences. One died of recurrent pulmonary metastases. The other patient that did recur is disease-free 21 months post-recurrence. Two additional patients were treated with HDR after tumor recurrence. One patient with recurrent Ewing's sarcoma, relapsed and died. The second is disease free 3 months after autologous bone marrow transplant. Grade 1 morbidity occurred in 46%, Grade 2 in 15%, and Grade 3 in 8% of the children, while relatively good bone and organ growth was maintained. The combination of conservative surgery, chemotherapy, and HDR offers the potential for disease control in young children while preserving bone growth and organ function. © 1995 Wiley-Liss, Inc.     

Sentinel node biopsy in pediatric soft tissue sarcomas of extremities

Background

Sentinel Node Biopsy is an established staging technique in many adult malignancies. However, only few reports describe this procedure for the evaluation of regional lymph nodes in childhood and adolescents. Our experience with sentinel node biopsy in soft tissue sarcomas of extremities in children is reported.

Methods

Seventeen children were evaluated with sentinel node biopsy between 2002 and 2007: 11 at initial surgery, 5 at primary re‐excision, 1 at local relapse. The diagnosis was rhabdomyosarcoma in 5 and other soft tissue sarcomas in 12: Ewing/PNET sarcoma 6, epithelioid sarcoma 1, malignant peripheral‐nerve‐sheath tumor 1, undifferentiated sarcoma 1, myxoid liposarcoma 2, adult‐type fibrosarcoma 1. Primary sites included lower limbs (8), upper limbs (9). Mapping of nodes was performed with lymphoscintigraphy plus intra‐operative injections with blue‐dye in 14 cases, with lymphoscintigraphy and intra‐operative injections alone in 2 and 1, respectively.

Results

Of the 17 lymphatic regions (9 axilla, 8 inguinal), 16 were identified with lymphoscintigraphy, 15 by intra‐operative injections. Thirty‐five lymph nodes were removed. Nodes were positive for metastasis in two patients with alveolar rhabdomyosarcoma and undifferentiated sarcoma. There were no complications. No further lymph node metastases were recognized either at diagnosis or during the follow‐up (6–78 months).

Conclusions

Sentinel node biopsy was technically feasible, reliable, and free of complications. It could be an alternative to aggressive or random biopsies for extremity rhabdomyosarcoma and it can contribute to define those non‐rhabdomyosarcoma soft tissue sarcomas that spread to regional nodes. Pediatr Blood Cancer 2009;52:51–54. © 2008 Wiley‐Liss, Inc.     

儿童非横纹肌软组织肉瘤临床预后因素及治疗对策

目的 分析18岁以下儿童和青少年的非横纹肌肉瘤的生存率,并探讨常见肿瘤的治疗策略。方法 对1989年1月到2002年12月治疗并随访的67例非横纹肌肉瘤病例的资料进行分析,其中滑膜肉瘤16例,恶性纤维组织细胞瘤9例,纤维肉瘤9例,脂肪肉瘤7例,骨外尤文氏肉瘤/原始神经外胚叶瘤7例,腺泡状软组织肉瘤6例,平滑肌肉瘤4例,恶性外周神经鞘瘤、透明细胞软组织肉瘤、血管肉瘤各2例,上皮性软组织肉瘤、促纤维增生性小圆细胞肿瘤、恶性肾外横纹肌样肿瘤各1例。引入年龄、性别、肿瘤大小、外科病理分期、是否接受化疗和放疗等因素,应用SPSS10.0统计软件,采用COX回归和χ^2检验进行统计学分析。结果 影响非横纹肌肉瘤生存率的唯一临床因素是外科病理分期。手术完全切除比肿瘤残留或转移者的生存率更高,Ⅰ、Ⅱ期和Ⅲ、Ⅳ期的2年EFS分别为89.74%和17.86%,差异有统计学意义。无肿瘤残留者生存率高于肿瘤残留或不能切除者。结论 儿童和青少年非横纹肌软组织肉瘤罕见,各肿瘤的临床特征不同,尚无统一治疗方法。治疗原则仍以达到无肿瘤残留为目标的外科手术治疗为主;除尤文氏瘤/原始神经外胚叶瘤和滑膜肉瘤已经证实化疗有效外,其他肿瘤的术后辅助化疗和放疗尚存在争议。新辅助化疗对于部分不能切除的肿瘤可以提高手术切除率。成立全国性的协作组可以尽快积累病例,增加治疗经验,制定规范的治疗方案,是改善儿童非横纹肌软组织肉瘤疗效的有效途径。     

Local failure in childhood rhabdomyosarcoma and undifferentiated sarcoma: Prognostic factors and implications for curative therapy

In this retrospective review, the risk factors for local failure in childhood rhabdomy-osarcoma and undifferentiated sarcoma were assessed in 49 patients managed by a multi-disciplinary team at the Prince of Wales Children′s Hospital, Sydney, between 1970 and 1988. Average follow-up time was 6.1 years. Sixteen of 49 patients experienced local failure defined as local recurrence after complete excision (5/20) or progressive local disease following incomplete resection (11/29). Fourteen of 16 patients who experienced local failure have died. Using logrank analysis of time to local failure, we found significantly increased risk with “non-embryonal” histology (P = 0.032), residual tumour (P = 0.052, higher IRS group (P = 0.088), “inadequate radiotherapy” for residual tumour (P = 0.001), delay in definitive local treatment (P = 0.038) and Adriamycin-containing chemotherapy (P = 0.017). When these factors were examined by multivariate analysis (Cox regression), only the presence of residual tumour after resection, “inadequate radiotherapy” for residual tumour (P < 0.001), and delay in definitive local therapy (P = 0.037) were shown to have independent significant association with local failure. We conclude that local failure may be avoided by prompt local treatment by either complete surgical resection or adequate radiotherapy. © 1993 Wiley-Liss, Inc.     

Orbital sarcoma with metastases at diagnosis: A report from the soft tissue sarcoma committee of the Children's Oncology Group

We reviewed clinicopathologic features and treatment outcomes in seven patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I–III. Three patients had embryonal rhabdomyosarcoma (RMS), and two patients each had alveolar RMS or unclassified sarcoma. Median age at diagnosis was 1.8 years (range 0.2–6.9 years). All patients had bone marrow involvement, including six with normal complete blood count at diagnosis. Cerebrospinal fluid was normal in six patients. Three patients survived >5 years, including one with local recurrence. In conclusion, further study is needed to determine necessity of bone marrow and CSF examination in orbital sarcoma patients. Pediatr Blood Cancer 2010;54:1045–1047 © 2010 Wiley‐Liss, Inc.     

Refractory pediatric nonrhabdomyosarcoma soft tissue sarcoma associated with ectopic production of beta hCG and hypercalcemia induced by PTHrP

A 3-month-old male with a mass on the right side of his back was admitted to our hospital. The tumor was a pathologically high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Treatment included subtotal tumor resection followed by chemotherapy. Elevation of serum beta hCG and hypercalcemia with detection of PTHrP was associated with tumor progression. The tumor was refractory to multiagent chemotherapy, and the patient died of the disease at 22 months of age. This case is novel in demonstrating a beta hCG secreting refractory NRSTS.