Clinical outcomes of rhabdomyosarcoma and Ewing's sarcoma of the head and neck in children

Objective

To review our experience and critically evaluate treatment strategy and results in children with head and neck rhabdomyosarcoma and Ewing's sarcoma.

Methods

Retrospective charts review of children affected by non-orbital rhabdomyosarcoma or Ewing's sarcoma of the head and neck who were treated at our institution from January 1996 to August 2009.

Results

Seven consecutive children with head and neck rhabdomyosarcoma or Ewing's sarcoma were identified. Four children had rhabdomyosarcoma, 3 children had Ewing's sarcoma. Regions involved were: cheek, ethmoid and maxillary sinuses, nasopharynx, middle ear/mastoid and frontal bone. In one case, surgery was performed as primary treatment modality; the other children were treated firstly with chemotherapy. Three patients underwent surgical resection after chemotherapy, while 4 patients received radiotherapy. Five children are disease free after a median of 7.7 years from initial diagnosis. Two patients relapsed after 10 and 29 months from initial diagnosis respectively; despite the administration of additional therapy both children died of disease.

Conclusion

Treatment for rhabdomyosarcoma and Ewing's sarcoma consists in a multimodal therapy involving chemotherapy, radiotherapy and surgery. The optimum use, timing and intensity of these three treatments are still matters of international debate. Chemotherapy in association with radiotherapy has proven capable to obtain local and distant control of disease. But when surgery is unfeasible or fails in radicality, local control is difficult without radiotherapy. Despite additional therapeutic efforts, prognosis of relapsing disease remains poor.     

Efficacy and morbidity of temporary 125I brachytherapy in pediatric rhabdomyosarcomas

PurposeRhabdomyosarcomas (RMSs) are the most common soft tissue tumors in the pediatric population. The American Brachytherapy Society provides recommendations for the use of brachytherapy (BRT) in the treatment of soft tissue sarcomas; yet, there are no clearly defined recommendations for the use of adjuvant BRT in treating RMSs in particular. Radiation therapy has an important role in maximizing local control, and BRT has the advantage over external beam radiation therapy of providing a high dose of radiation to the most susceptible area of recurrence, while delivering a lower dose to the surrounding normal tissue.Methods and MaterialsThis study examines a group of 8 pediatric patients with RMSs who were treated with temporary low-dose-rate ¹²⁵I BRT and investigates the efficacy and side effects of such treatment.ResultsThe results demonstrate a local recurrence rate of 12.5%, with minimal side effects occurring in the patients who had no prior radiation history. Each patient's side effects are discussed.ConclusionsThe high efficacy and ease of radiation protection for visitors establishes this as an effective treatment that is logistically convenient for patients and families. This is the first report of patients exclusively with RMSs being treated exclusively with ¹²⁵I BRT and demonstrates promising results.     

Embryonal rhabdomyosarcoma of the omentum: two cases occurring in children

Rhabdomyosarcoma is a soft-tissue malignancy that represents approximately 4–8% of all solid tumours in children and commonly arises from the head and neck and genitourinary system. Intraperitoneal rhabdomyosarcoma, in particular with omental involvement, has been rarely reported in the literature. Furthermore, reports of omental rhabdomyosarcoma of embryonal origin do not exist, to our knowledge. We report two cases of omental embryonal rhabdomyosarcoma affecting children and illustrate the imaging characteristics of this rare tumour.     

Differentiating between congenital rhabdomyosarcoma versus fibromatosis of the pediatric tongue

Congenital rhabdomyosarcoma of the tongue is exceedingly rare. Fibromatosis of the tongue is also rare, and very difficult to distinguish from the spindle cell variant of rhabdomyosarcoma. Both appear histologically as spindle neoplasms replacing normal striated musculature of the tongue. The treatment protocol for the former has been developed by the Intergroup Rhabdomyosarcoma Studies (IRS) I-IV and requires surgery, radiation, and chemotherapy. For fibromatosis, complete surgical excision is usually adequate without additional therapy, although some cases of aggressive fibromatosis also require chemotherapy. With significant differences in appropriate treatment and prognosis, each entity must not be mistaken for the other. We review the differences in radiologic, histologic, and immunohistochemical (IHC) features of both entities.     

儿童横纹肌肉瘤免疫组织化学诊断评估

目的：对目前常用的6种免疫组织化学标志物－波纹蛋白（vimentin,VIm),结蛋白（desmin,Des),肌红蛋白（myoglobin,MG),肌球蛋白（myosin,MS),平滑肌肌动蛋白（smooth-muscle actin,SMA),肌节肌动蛋白（sarcomeric actin,Sr-A)在横纹肌肉瘤（RMA）诊断中的应用价值进行探讨。方法：24例RMS及其他多种儿科肿瘤标本均来自手术切除组织,经10％中性甲醛固定,石 包埋,切片采用LSAB三步法进行免疫组织化学染色,另外用高温组织抗原修复法提高Des,MS,Sr-A3种标志物的阳性率和阳性强度。结果：6种标志物在RMS中的阳性率由高到低依次为Vim,MG,MS,Des,Sr-A;特异度由高到低为Sr-A,Des,SMA,MG,Vim(MS资料暂缺）,实验有效率由高到低是Des,MG,Sr-A,SMA,Vim,结论:Vim和MG对RMS的灵敏度最高,但二者的特异性均较差,而特异性最佳的Sr-A和Des在RMS中表达的阳性率却不高,因此,对分化较差的RMS必须联合采用多种抗体进行标记,结合对实验有效率的分析,建议优先联合使用Des,MG和Sr-A。     

Efficacy of topotecan plus vincristine and doxorubicin in children with recurrent/refractory rhabdomyosarcoma

Background This study investigates the efficacy and the feasibility of a chemotherapy regimen with topotecan plus vincristine and doxorubicin (TVD) given on an individually tailored basis to patients with refractory/recurrent rhabdomyosarcoma (RMS). Patients and methods Nine patients received TVD therapy at relapse, and six were assessable for response. Results All the six patients experienced objective response after two cycles of chemotherapy: one minor response, four partial response, and one complete response. Conclusions The value of our study is severely limited by the small number of cases, the single-institutional setting and the individually tailored treatment, but we nonetheless confirmed the feasibility and tolerability of topotecan-based chemotherapy in RMS.     

Rhabdomyosarcoma of nose, nasopharynx and paranasal sinuses

Rhabdomyosarcoma is an aggressive malignant soft tissue tumor that arises from primitive striated muscle cells called rhabdomyoblasts. It is the most common soft tissue malignant tumor in children and accounts for about 7–8% of childhood cancers. It can arise from almost anywhere in the body but it is most common in the head and neck region. This article presents six cases of rhabdomyosarcoma involving the nose, nasopharynx and the paranasal sinuses four of which were treated with a combination of radiotherapy and chemotherapy and two were treated with medial maxillectomy combined with chemotherapy and radiotherapy.     

Rhabdomyosarcoma Subtyping by Immunohistochemical Assessment of Myogenin: Tissue Array Study and Review of the Literature

Myogenin immunostaining has been described as a useful marker of the alveolar subtype of rhabdomyosarcoma and as a tool for distinguishing it from the more common embryonal subtype. To add to the growing body of literature describing this phenomenon we analysed myogenin immunohistochemical staining in 152 tumors using a rhabdomyosarcoma tissue array. Results were analysed blinded to histological type by two independent investigators. Samples were excluded if any samples failed to stain with desmin and/or myogenin. Mean percentage of myogenin positive cells was significantly greater for ARMS (n = 31; mean percentage positivity 59% (95% confidence intervals ± 7%) than ERMS (n = 41, mean percentage positivity 16%, 95% confidence intervals ± 4; P < 0.0001). This data is consistent with previously published studies identifying strong nuclear myogenin staining in a high proportion of cells as a marker of alveolar histology.     

肝未分化胚胎性肉瘤的CT及MRI表现

目的 探讨肝未分化胚胎性肉瘤(UESL)的螺旋CT及高场强MR的影像学表现,提高UESL诊断准确率.方法 回顾性分析14例经手术病理证实的UESL患者.螺旋CT检查5例,MR检查9例.结果 5例患者CT扫描均表现为平扫边界清晰的囊状低密度影,其内含有不规则的软组织影,增强后动脉期软组织强化,门脉期及延迟期继续强化.MRI 9例患者中6例T1WI表现为囊状高低信号混杂影,T2WI病灶以高信号为主.增强后动脉期病灶边缘强化,门脉期及延迟期继续强化;3例患者T1WI表现为囊状低信号影,T2WI呈高信号.增强后动脉期病灶边缘强化,门脉期及延迟期继续强化.结论 平扫和动态增强螺旋CT及高场强MRI能反应UESL的影像学特点,提高了UESL诊断准确率.     

Massive bone marrow involvement by clear cell variant of rhabdomyosarcoma

A 16-year-old boy with generalized myalgia and petechial hemorrhage was found to have a diffuse infiltrative disease in his bone marrow (BM). The BM aspirate contained a dense population of vacuolated blast-like cells. The BM biopsy displayed compact sheets of small round cells with clear cytoplasm, reminiscent of Ewing sarcoma. Immunostains were not diagnostically conclusive while transmission electron microscopy on the BM cells demonstrated a clear skeletal muscle differentiation. The morphologic findings led to a tentative designation of metastatic embryonal rhabdomyosarcoma (RMS). It was not until cytogenetic analysis revealed the specific translocation t(2;13)(q35;q14) did the alveolar RMS finally get confirmed. Despite an exhaustive search by imaging studies, a primary tumor was not detected. This case illustrates that the massive BM involvement by atypical alveolar RMS of unknown origin may pose serious diagnostic challenges. Multidisciplinary studies are required to reach a definitive diagnosis