Remaining Problems and Controversies in the Management of Childhood Head and Neck Soft Tissue Sarcomas: Retrospective (National) Multicenter Study of the Polish Pediatric Solid Tumors Group

Soft tissue sarcomas in children are a heterogeneous group of malignant diseases. Among these, tumors localized in the head and neck region are especially difficult to treat. While multidisciplinary care has dramatically improved the prognosis of sarcoma patients, their treatment remains uncertain because of demand on radical surgical resection of the tumor. Achieving cure without deforming or mutilating the child remains the primary goal of treatment. This study is the multicenter (nationwide, 11 Polish centers) retrospective analysis of the treatment results in children having soft tissue sarcoma in the head and neck region during the previous decade (from 1991 to 2001). Late effects of the treatment are documented in long-term survivals. Eighty-five children from 1 to 212 months of age were included. Different multimodal treatment protocols were utilized (CWS-91, SIOP-MMT-91, and CWS-96). The median observation time was 25 months. Data on long-term effects were collected in 34 long-term survivals. Complete remission was achieved in 68 (80%) patients. Primary treatment failure occurred in 13 (15.3%) patients, all of whom succumbed in disease progression. Relapse occurred in 21 (30.9%) patients primarily achieving complete remission. Second primary neoplasm occurred in 3 children. The estimated 5-year event-free survival and the 5-year total survival rates for the whole group are 0.38 and 0.55, respectively. The main late effect documented in long-term survivals were cosmetic defects in 12 (35.3%) and visual field deficit or blindness in 8 (26.5%). Despite substantial improvement of the prognosis of pediatric soft tissue sarcomas, the multimodal treatment of head and neck region tumors remains controversial. Improved long-term outcome and focusing on psychosocial difficulties raise the important and difficult problem of functional results and cosmesis. Tumors localized in the orbit carry an excellent prognosis. However, the main late sequela is vision impairment and cosmetic defect due to the therapy given many years earlier. Two other tumor localizations—the parameningeal and nonparameningeal ones—still have bad prognosis. The observations made in this study confirm that main prognostic factors are the size of the primary tumor and the tumor stage. The worst prognosis remains invasive tumor (T2-stage) with a size over 5 cm. Individually adjusted multimodal therapy, which imperatively needs to be radical, though not mutilating, might minimize the late effects. Psychosocial problems in long-term survivors need to be focused on at the national level and better support must be provided in the future, involving a team of different medical and paramedical profiles.     

Results of the treatment of non-rhabdomyosarcomatous soft tissue neoplasms within the scope of the CWS 81 study

347 children with all types of soft tissue sarcoma (STS) have been entered between 1981 and April 1986 into the CWS-81 study. Excluding 229 patients with rhabdomyosarcoma, 118 patients with other STS were analysed in the histological subgroups, separately. 33 synovial sarcomas (SyS), 15 undifferentiated sarcomas (US) and 13 extrasceletal Ewing's sarcoma (EES) were treated with a multimodale trial including chemotherapy and radiotherapy after initial not-mutilating resection or biopsy only, Relapse-free survival rate by Kaplan-Meier was 66% in SyS, 48% in EES and 38% in US, respectively. Patients treated according to the guidelines of the CWS-protocol showed better prognosis than patients with major protocol violations or patients with recurrence when entered into the trial. In SyS only 2 of the 7 patients with recurrence developed metastases. The tumor response rate within 7-9 weeks chemotherapy exclusively given was 71% in patients with stage III and IV. Non-responding tumors to chemotherapy had bad prognosis, irrespective of histological subtype or further treatment. EES and US developed earlier recurrence and more often metastases than SyS or rhabdomyosarcomas. The administered VACA-chemotherapy seems not to be very effective in EES and US. In all other varieties of STS recurrence at the primary site assumed relatively greater importance. Chemotherapy and radiation were not administered in all patients. Fibrosarcoma (FS) was diagnosed in 13 patients. Six of these were entered in the trial at the time of local recurrence and 4 of these 6 relapsed again locally, one with metastasis concurrently.(ABSTRACT TRUNCATED AT 250 WORDS)     

Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and - III. IRS Group of the Children's Cancer Group and the Pediatric Oncology Group.

BACKGROUND: This review of children and adolescents with nonorbital soft-tissue sarcoma of the head and neck was undertaken to describe late sequelae of treatment, as manifested primarily by problems with statural growth, facial and nuchal symmetry, dentition, vision and hearing, and school performance. PROCEDURE: Four hundred sixty-nine patients entered the IRS-II and -III protocols with localized, nonorbital soft-tissue sarcomas of the head and neck from 1978 through 1987. Their overall survival rate was 53% (250/469) at 5 years. Two hundred thirteen patients were surviving relapse-free 5 or more years after diagnosis, for whom there were serial height measurements at 2 or more years after initiation of therapy. Their median age at diagnosis was 5 years; the median length of follow-up was 7 years. All received multiple-agent chemotherapy, and all but 3 received irradiation to the primary tumor volume. Sixty-eight percent of the tumors arose in cranial parameningeal sites, 22% in nonparameningeal sites, and 10% in the neck. We reviewed flow sheets submitted to the IRS Group Statistical Office to ascertain which late sequelae were recorded. RESULTS: One hundred sixty-four patients (77%) had one or more problems recorded. One hundred ninety of the two hundred thirteen patients (89%) were under 15 years of age at study entry, and at follow-up 92 (48%) had failed to maintain their initial height velocity, which had decreased by more than 25 percentile points from the original value. Thirty-six of the one hundred ninety patients (19%) were receiving growth hormone injections. Hypoplasia or asymmetry of tissues in the primary tumor site was reported in 74 patients, and 13 underwent reconstructive surgery. Poor dentition or malformed teeth were noted in 61 patients. Impaired vision developed in 37 patients, owing primarily to cataracts, corneal changes, and optic atrophy. Thirty-six patients had decreased hearing acuity, and 9 were fitted with hearing aids; 5 of these 9 had received cisplatin. Thirty-five patients were noted to have problems learning in school. Four patients developed a second malignancy (two sarcomas, one carcinoma, one leukemia). CONCLUSIONS: Late sequelae affected the majority of these patients treated for soft-tissue sarcoma of the head and neck on IRS-II and -III. The potential impact of certain sequelae could be reduced by specific measures, such as surgical reconstruction and hormonal therapy. Late sequelae must be taken into account in designing future curative treatments.     

Epithelioid Sarcoma in Childhood: An Immunohistochemical, Electron Microscopic, and Clinicopathologic Study of 11 Cases Under 15 Years of Age and Review of the Literature

Epithelioid sarcoma in a rare tumor and most of the cases occur in young adults. It is rare in childhood. We have been able to obtain data and histologic material for 11 patients with this disease. The primary sites were head and neck in three patients, inguinal region in one, and extremities in seven. The age range of the patients was 12 weeks to 13 years. There was a preponderance of males over females with a ratio of 1.75. The tumors presented with a typical nodular necrotizing pattern. In three cases giant osteoclast-like cells were present. The immunohistochemistry and electron microscopy showed features consistent with previous observations on epithelioid sarcomas. In one case islands of small dark cells noted on light microscopy were surrounded by basal lamina on electron microscopy. The cells inside the nests were undifferentiated. Six tumors studied by flow cytometry were in DNA diploid range. On follow-up, five children are alive and well 2 to 7 years after treatment. Three children died of tumor progression with metastases to lymph nodes and lungs. One child had been diagnosed only recently, and in one the disease has run a protractive course with multiple recurrences. The behavior of these epithelioid sarcomas in children is similar to that seen in adults, the prognosis being dependent on radical tumor surgery preventing recurrent disease. Long-term follow-up is necessary because the tumor may recur many years after the primary tumor was removed.     

Role of surgery in children with rhabdomyosarcoma

BACKGROUND: Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma of childhood. Historically, surgery has played a central role in the management of children with this tumor, though with surgery alone survival rates were poor. With current multimodal (surgery, radiotherapy and chemotherapy) treatment of these patients, survival has dramatically improved and, with this improvement, there has been an evolution of the role of surgery in the management of this condition. MATERIAL AND METHOD: The contemporary published literature (English) regarding surgical aspects of pediatric rhabdomyosarcoma was reviewed and evaluated. RESULTS: Multimodal therapy has improved the survival of children with RMS from 25% in 1970 to greater than 70% today. Surgical procedures for childhood RMS today are less apt to be exenterative or mutilating than those employed thirty years ago. CONCLUSIONS: Surgery plays a vital role in the diagnosis and treatment of children with RMS. This role has evolved in the context of multimodal therapy and improved survival to an emphasis on less radical procedures with decreased morbidity.     

Results of treatment of rhabdomyosarcomas (RMS) in children. A report of the Cooperative Soft Tissue Sarcoma Study (CWS-81) of the Society of Pediatric Oncology

This analysis refers to 129 children with RMS who were treated between 1981 and 1985 according to the protocol guidelines of the CWS-81 study. The duration of chemotherapy depended on the initial post-operative stage. Patients with stage I and IIA were not to receive any radiotherapy, and patients with primary stage III were treated according to the results of a 16-week chemotherapy treatment: either without radiation (stage Ipc), or with radiotherapy using 40 Gy (stage (IIpc) or 50 Gy (stage IIIpc). The median time of observation was 27 months (Juni 1985). The essential results of the study are as follow: RMS of the extremities in stages I and IIA need radiotherapy, contrary to all other localisations with the same stages. Patients with RMS stage III who are tumor-free after initial chemotherapy (histologically checked) do not need radiotherapy. Patients with microscopic residue (IIpc) or macroscopic residue (IIIpc) after pretreatment showed no difference in their local relapse rate, whereas metastases were found only in the group having macroscopic tumor residue up to week 16. An additional examination of this study observed tumor response during initial chemotherapy and its relationship to prognosis. This analysis showed that the degree of tumor regression per unit of time permits the most favorable prognostic statement. Patients with clinical complete remission after 7/9 weeks showed a 100% relapse-free chance of survival, independent of localisation, tumor size or histological subtype. Those with a tumor reduction of greater than 2/3 but no complete remission showed 67% chance of survival, and those with greater than 1/3-2/3 tumor reduction had 25%. Tumor response kinetics under initial chemotherapy allows better individual therapy in the future. The overall result of the study concerning localisation and stage corresponds to that of the IRS I and II studies. Patients with undifferentiated sarcomas, extraossary Ewing's sarcoma and synovial sarcomas can be treated according to the same principles as RMS, since no significant differences in prognosis could be found.     

Delayed resection in the management of non-orbital rhabdomyosarcoma of the head and neck in childhood

This retrospective study was undertaken to evaluate the effect of delayed resection on outcome of head and neck rms in a single institution which has experience in cranial base surgery. Since 1988, patients with primary non-orbital rms of the head and neck following treatment at the Children's Hospital of Pittsburgh, were evaluated by the Department of Otolaryngology, Eye and Ear Hospital at the University of Pittsburgh Medical Center either at the time of presentation or when response to chemotherapy and/or radiation therapy was thought to have been optimized for the possibility of definitive surgery. Medical records of patients who did or did not have delayed surgery were reviewed and compared with respect to demographics, tumor stage, response to therapy, survival, and cosmetic results. Of 16 children diagnosed with non-orbital head and neck rms from 1988–1994 and treated with chemotherapy according to IRS II–IV, 3 had group I or II disease following extensive surgery at diagnosis. Thirteen had group III or IV disease. Of these, 6 patients had delayed resection and 7 did not. Delayed resection was undertaken 3–12 months (median, 4 months) from diagnosis in 4 children who had a partial response (PR) and 2 children who had stable disease (SD) with chemotherapy and/or radiation. Delayed resection converted all children to complete responses (CR), including one child with clinical SD and one with PR who were found to have no viable tumor at surgery. The overall percentages of CRs for patients with group II or IV disease (documented any time post-diagnosis) were at least as good for patients who had undergone delayed surgery as for those who had not (100% vs. 71%, p = .465). Median survivals for patients with advanced disease were 3 ½ years and 2 years, respectively (p = .2801). Cosmetic and functional problems attributable to surgery were not severe but included facial asymmetry (n = 4), trismus (n = 1), cranial nerve deficits (n = 1), and abnormal dentition (n = 1). In locally extensive head and neck rms, cranial base surgery should be considered after initial cytoreductive therapy, since it may contribute to achievement of CR and to survival with acceptable morbidity. Med. Pediatr. Oncol. 28:294–298. © 1997 Wiley-Liss, Inc.     

Pathology of soft tissue sarcomas: 238 cases of the childhood tumor registry

Until April 1981 malignant soft tissue sarcomas were registered from 238 patients. Rhabdomyosarcoma was the most common tumor (115/238 = 48.3%). The embryonal subtype was predominantly seen among the rhabdomyosarcomas (83/115 = 72.2%). Rhabdomyosarcomas were localized most frequently in the head and neck area (40/115 = 34.8%), followed by genitourinary system (15/115), pelvis soft tissue (12), abdomen (10) and extremities (10). Non-rhabdomyosarcomatous soft tissue sarcomas (123/238 = 51.7%) were synovial sarcomas (20 = 8.4%), fibrosarcomas including spindle cell sarcoma (17 = 7.4%), leiomyosarcomas (12 = 5.0%), malignant tumors of the vascular system (11 = 4.6%) and neurofibrosarcomas (9 = 3.8%). Other types of sarcoma were extremely rare. 42 (17.6%) of all soft tissue sarcomas could not be classified histogenetically. Rhabdomyosarcomas could be diagnosed much more accurately (105/115 = 91.3%), compared to all other soft tissue sarcomas (99/121 = 81.8%). At present, the most difficult diagnostic problems remain with the tumors of connective tissue, in particular with fibrosarcomas and with the differential diagnosis of juvenile fibrosarcomas versus juvenile fibromatoses.     

Long-term morbidity in children treated with fractionated high-dose-rate brachytherapy for soft tissue sarcomas

BACKGROUND: The purpose of this study was to determine the long-term local control, disease-free survival, and morbidity of fractionated high-dose-rate brachytherapy (F-HDR) in infants and children with soft tissue sarcomas. PATIENTS AND METHODS: Fifteen children (13 girls and 2 boys, ages 5-101 months) with soft tissue sarcomas were treated with chemotherapy, organ-preserving surgery, and F-HDR (36 Gy in 12 fractions) to post-chemotherapy volumes. External beam radiotherapy was not part of the primary treatment, although four patients (27%) subsequently received salvage external beam radiotherapy after treatment failure. Chemotherapy was administered to all patients based on their tumor histology and stage. RESULTS: After a median follow-up of 10 years (range 32-154 months), 12 patients (80%) were alive without evidence of disease. Ten-year overall survival and local control rates were both 80% (12/15 children). The overall survival was better (91%) for children with microscopic residual versus gross residual disease (75%). With longer follow-up, grade 3 to 4 brachytherapy-related late morbidities increased from 8% (1/12) to 20% (3/15) and included trismus/osteonecrosis, vaginal stenosis, and periurethral fibrosis. There were two late complications associated with puberty that occurred 8 to 10 years after the initial treatments. Acute toxicity occurred in five patients (38%) and consisted primarily of grade 1 to 3 skin and mucosal reactions. CONCLUSIONS: As the sole radiation modality, F-HDR achieved excellent local control and disease-free survival in properly selected children with soft tissue sarcomas while preserving normal bone and organ development. A significant percentage of patients experience adverse late sequelae as a result of this treatment.     

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.     

Dental abnormalities in long-term survivors of head and neck rhabdomyosarcoma

To define the long-term dental sequelae of therapy for childhood rhabdomyosarcoma of the head and neck, and to identify factors in their development, we retrospectively reviewed the serial panoramic radiographs and clinical records of 22 survivors of head or neck rhabdomyosarcoma who had been followed for at least 5 years. Patients were divided into four groups based upon age at the time of therapy and three groups based upon radiation doses. All patients had received similar multiagent chemotherapy. Dental sequelae of oncotherapy occurred in over half of the long-term survivors of head and neck rhabdomyosarcoma. The abnormalities comprised root stunting in 54%, microdontia in 23%, and hypodontia in 50% of patients; 36% had multiple abnormalities. Microdontia and multiple abnormalities were more prevalent in patients treated at the earliest age, and abnormalities tended to be more prevalent with increasing doses of radiation. Five patients (23%) developed severe cosmetic and/or functional sequelae necessitating surgical and/or orthodontic intervention. The high frequency of dental sequelae we observed suggests that meticulous long-term dental and radiographic follow-up are needed. Early detection and treatment of the complications of therapy will expedite their correction and minimize morbidity. © 1995 Wiley-Liss, Inc.     

Synovial sarcoma in the neck of an eleven month old girl

Synovial sarcomas are rare primary tumors of the head and neckin any age group. We present a case of primary synovial sarcoma arising in the neck of an 11-month-old girl.     

儿童头面部胚胎性横纹肌肉瘤的临床病理特点及预后分析

摘要 目的 探讨儿童头面部胚胎性横纹肌肉瘤的临床病理特征及其与预后的关系。方法 按2002年国际病理学会的诊断标准收集复旦大学附属眼耳鼻喉科医院病理诊断确诊的头面部胚胎性横纹肌肉瘤病例，并将葡萄状型归入胚胎性的亚型。收集病理及免疫组化切片及临床资料，并请复旦大学附属肿瘤医院病理科软组织专家重新阅读病理片，对发生于儿童头面部的胚胎性横纹肌肉瘤进行临床病理学分析。结果 2004～2007年共有18例患儿纳入分析，其中男12例，女6例；发病年龄5个月至18岁，＜10岁者12例。临床表现为口、鼻或耳道肿物及鼻塞、声嘶、面瘫等肿瘤占位引起的相应症状和体征。依据美国横纹肌肉瘤研究组的分期标准，临床Ⅰ~Ⅱ期7例，Ⅲ~Ⅳ期11例。CT或MRI显示相应部位膨胀性生长的软组织占位，多伴有骨吸收破坏。术前有5例临床诊断为良性病变。组织病理学上，以出现不同分化阶段的横纹肌母细胞为特征，其中葡萄状亚型4例。行肿瘤完整或大部切除5例，部分切除2例，11例仅行手术探查或鼻内镜活检。随访到10例患儿，其中4例患儿病死，均为临床Ⅲ期，存活的6例均采用了手术结合化疗或放疗的综合治疗；余8例失访。结论 儿童头面部胚胎性横纹肌肉瘤为高度恶性的软组织肿瘤，生长迅速，临床较易漏诊或误诊，早期正确诊断和综合治疗是提高生存率的关键。     

Inflammatory Myofibroblastic Tumors in Childhood

Inflammatory myofibroblastic tumor (IMT) is a rare tumor of intermediate malignant potential that can occur anywhere in the body. Surgical resection is the principal treatment. We report on nine children diagnosed with IMT at our institution over a 10-year period. Presenting symptoms were reflective of tumor location. Complete surgical resection was curative. Local recurrence occurred in the presence of involved surgical margins. One patient with metastatic disease achieved long-term remission with chemotherapy alone. Severe inflammatory response and death occurred in one patient. The 3-year event free and overall survivals (OS) were 58 ± 20% and 89 ± 10% respectively.     

Treatment of soft tissue sarcomas in childhood and adolescence: results of the CWS-81 multicenter therapy study

344 previously untreated patients, under 19 years of age, with soft tissue sarcoma (STS) entered the first German STS Study, CWS-81. 218 of them with chemosensitive STS (Group A: rhabdomyosarcoma [RMS], synovial sarcoma, extraosseous Ewing's sarcoma, undifferentiated sarcoma and malignant peripheral neuroectodermal tumor) were evaluable for this analysis after a minimum potential follow-up of 6 years. A staging system based on the extent of disease, defined post-surgically, was used. The chemotherapy for stages I-III (VACA cycle) consisted of vincristine, dactinomycin, cyclophosphamide and doxorubicin. Patients with metastatic disease as well as stage III patients who failed to respond to VACA, were given ifosfamide instead of cyclophosphamide. The definitive local tumor control procedure for patients in stages II-III depended upon the tumor status at second-look surgery after 16 weeks of chemotherapy (no irradiation, 40Gy or 50Gy). The DFS rate after 5 years for group A was 57 +/- 4% and for patients with non-metastatic tumors (Stages I-III), 69 +/- 4%. There was no difference in prognosis between stages I and II (DFS rate 88 +/- 5% and 88 +/- 6% respectively). The DFS rate for stage III was 54 +/- 5% and for stage IV, 11 +/- 5%. Lack of local tumor control was the main cause of therapy failure: 10% of patients with localized disease never achieved CR, 18% relapsed locally. The most important prognostic factors were tumor size (p = .0002) and the degree of tumor regression after primary chemotherapy (p = .02).(ABSTRACT TRUNCATED AT 250 WORDS)     

Soft tissue sarcomas in infants younger than 1 year of age: a report of the German Soft Tissue Sarcoma Study Group (CWS-81)

Of 357 patients with soft tissue sarcomas (STSs) who were registered between 1981 and 1986 in the CWS-81 study, 21 (5.8%) were younger than 1 year of age. Six cases were probably of congenital origin since they were diagnosed in the 1st month of life. There was no difference in clinical grouping (staging and primary site) between infants and older patients. The major histological subsets comprised 12 patients with embryonal rhabdomyosarcoma (57% vs. 44% in older patients) and five patients with fibrosarcoma (23% vs. 2% in older patients). Of 21 patients, 17 received chemotherapy. The response rate (tumor reduction more than 1/3) to exclusive cytostatics therapy within 7-9 weeks, of ten evaluable patients, was 100% (vs. 86% in older patients). Eight of the responders achieved complete remission after 16 weeks of therapy and are in continuous complete remission (CCR). Most patients received 20-50% of the recommended chemotherapy dose for older children, which was calculated using body surface area. No fatal or life-threatening complications were observed. The event-free survival rate for children younger than 1 year of age with rhabdomyosarcoma was 70% vs. 47% in older children (no significant difference). Now, 2-5 years after therapy all five fibrosarcoma patients are alive and free of disease. Our data indicate that children under 1 year of age with rhabdomyosarcoma have a slightly better prognosis than older children, when therapy modalities are adapted to age.     

Malignant fibrous histiocytoma in a child. A case report and review of the literature

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma of late adult file. It is extremely rare in children, though, and its existence in the pediatric population remains controversial. Although the most common site of tumor in children is the extremities, which is similar to findings of adults' series, different sites have been reported in children. Because of the rarity of this tumor in childhood, the approach to treatment of MFH is based primarily on the experience with adults. We present the clinical and pathologic features of an eight-year-old boy with MFH located on his left retroperitoneum and also review the literature.     

Undifferentiated sarcoma of the liver: a single institution experience using a uniform treatment approach

Undifferentiated (embryonal) sarcoma of the liver is a rare malignant tumor, most commonly seen in children aged 6 to 10 years. Previously believed to carry a poor prognosis, more recent reports indicate that treatment regimens combining surgical resection and adjuvant chemotherapy can yield long-term, disease-free survival. In this study, we review 5 pediatric patients with undifferentiated sarcoma of the liver treated with a uniform approach of resection followed by adjuvant chemotherapy and radiation when indicated. All 5 patients are disease free in their first remission at a median of 53 months.     

Rhabdomyosarcoma

Rhabdomyosarcoma, the most common soft tissue sarcoma of children, carried a 10–15% survival rate in the late 1960s. Since then, better understanding of biology and pathology of the tumor and the judicious use of chemotherapy and radiation has improved the prognosis drastically. In addition instead of extirpative surgery, organ salvage is now feasible in many of these children. In this article the authors review current information regarding pathology, diagnosis and treatment of rhabdomyosarcoma     

Epithelioid sarcoma arising on the forearm of a 6-year-old boy: case report and review of the literature

Epithelioid sarcoma is a rare malignant tumor that occurs mainly in young adults and most frequently involves the distal portion of the upper extremities. The tumor is particularly rare in children and more frequently involves the head and neck; only one case involving the forearm has been reported in a child under 10 years of age, and he was treated with amputation. We report the case of a 6-year-old boy with an epithelioid sarcoma of the forearm whose initial management had been inappropriate. The patient was ultimately treated with limb sparing surgery. Two years later, no local recurrence was evident but pleural metastases were detected.