A single‐center experience with undifferentiated embryonal sarcoma of the liver

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare aggressive mesenchymal pediatric tumor. Previously, reported outcomes have been very poor. Here, we report a single‐center experience of five patients with UESL treated with upfront gross total resection and adjuvant chemotherapy. We have a median follow‐up of 8 years with a range from 5 to 19 years with 100% event‐free survival.     

儿童肝脏未分化胚胎性肉瘤8例临床分析及文献复习

目的分析儿童肝脏未分化胚胎性肉瘤(UESL)的临床特征、治疗方案及预后。方法回顾分析2012年11月至2019年6月收治的8例UESL患儿的临床资料,采用Kaplan-Meier生存分析法计算生存率。结果 8例患儿中,男性5例、女性3例,中位发病年龄7岁(8个月～10岁);8例患儿的肿瘤均位于肝右叶,中位最大径为13.5 cm(10～20 cm),超声检查均显示为混合回声为主的肝内囊实性肿块。所有患儿手术完整切除肿块,3例术前肿瘤破裂。术后均接受顺铂/长春新碱/阿霉素/环磷酰胺(AVCP)方案与异环磷酰胺/依托泊苷/长春新碱(IEV)交替化疗6个周期。患儿中位随访时间47(24～90)月,7例完全缓解,1例死于复发;5年无事件生存率(EFS)为(79.25±9.31)%。结论 UESL患儿术前可出现肿瘤破裂,完整切除手术联合化疗能获得长期无病生存,少数复发患儿预后不佳。     

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.     

A child with undifferentiated sarcoma of the liver complicated with bronchobiliary fistula and detected by hepatobiliary scintigraphy

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant mesenchymal tumor principally affecting patients of pediatric age. Bronchobiliary fistula is a very rare complication in patients with liver tumor. To the authors' knowledge, this is the first report of a bronchobiliary fistula resulting from tumor invasion in a child with liver sarcoma. A 12-year-old boy was diagnosed to have USL of the right liver lobe, invading the diaphragm. An extended right hepatectomy and total resection of the mass was performed, leaving patchy tumoral invasion of the anterior diaphragmatic surface followed by combined chemotherapy regimen. Six months after the operation, the presence of bilious sputum suggested a bronchobiliary fistula, which was confirmed by hepatobiliary scintigraphy. The patient underwent a right thoracotomy and fistula division. Although bronchobiliary fistula is a very rare complication in patients with hepatic tumors, suspicion in the appropriate clinical setting is necessary to recognize this problem. Hepatobiliary scintigraphy is the useful diagnostic procedure to define bronchobiliary fistula in children with liver tumor and clinical suspicion of bronchobiliary fistula.     

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   

Undifferentiated embryonal sarcoma of the liver in a child complicated by haemorrhage

Undifferentiated embryonal sarcoma (UES) of the liver is a rare malignant neoplasm that mostly affects children younger than 15 years of age. We report a patient with UES in the right lobe that was complicated by haemorrhage after needle biopsy. The tumour was managed by embolization of the right hepatic artery and treated successfully with chemotherapy and surgical resection. Prophylactic embolization of the feeding artery should be undertaken before a biopsy procedure if there is the possibility of tumour rupture, in the presence of signs of intratumoral or peritumoral bleeding, or in the presence of a vascular liver mass.     

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.     

An analysis of primary site control and late effects according to local control modality in non-metastatic Ewing sarcoma

PURPOSE: To examine prognostic factors for primary site control and analyze late effects according to local treatment modality in non-metastatic Ewing sarcoma (ES). MATERIALS AND METHODS: From 1976 to 2001, 76 patients with localized ES and a median age of 14.5 years were seen and treated at one institution. Tumors were located in the extremity in 38, pelvis in 13, spine in 11, trunk in 8, and head and neck in 6. Tumor size was < or = 8 cm in 44 and >8 cm in 32. Local therapy included radiotherapy (RT) alone in 40, surgery (S) alone in 27, and surgery followed by postoperative radiotherapy (S + RT) in 9. Chemotherapy (CT) was delivered to 65 patients (86%). Median follow-up for surviving patients was 9.7 years. RESULTS: The 5- and 10-year overall survival rates were 57.5% and 52.1% while the 5- and 10-year local control rate was 79.2%. The 5- and 10-year local control rates were 78.2% for RT, 77.6% for surgery, and 88.9% for S + RT (P = 0.68). Multivariate analysis showed that only the use of CT was found to be a prognostic factor for local control (P = 0.014). The 5- and 10-year local control rates were 83.7% for those receiving CT and 51.1% for those not receiving CT. For patients followed at least 5 years from diagnosis, late effects were seen in 10 of 19 (52.6%) receiving RT, 2 of 5 (40%) receiving S + RT, and 4 of 16 (25%) receiving surgery alone. The most common late effects with RT were muscular atrophy, limb length growth delay, and development of second malignancy. Scoliosis and decrease range of motion of an extremity were seen regardless of local treatment modality. Three patients who had surgery alone had an amputation whereas two who had RT had an amputation secondary to relapse or development of osteosarcoma. CONCLUSION: In this single institution study, the use of CT was the only factor found to impact on local control. Late effects were common regardless of local control strategy.     

Successful treatment of a child having generalized Kaposi's sarcoma after living donor liver transplantation with conversion to sirolimus

Abstract:  SRL is a new and potent immunosuppressive agent that has been successfully introduced in organ transplantation. In contrast to other immunosuppressive agents, SRL has a potent antitumor activity both in vitro and in vivo . Herein, we report a child with Kaposi's sarcoma that was diagnosed 30 months after LDLT and treated successfully with only conversion to SRL monotherapy. KS regressed completely at the end of the first month and remained in remission during 28 months follow-up.     

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.     

Long-term outcome of patients with monostotic Ewing's sarcoma treated with combined modality

One hundred and twenty-one consecutive patients with monostotic Ewing's sarcoma (ES) were treated according to three consecutive combined modality programs from 1974 to 1986. Their 3-year progression free survival (PFS) rate from diagnosis of 59% was identical to the event free survival (EFS) rate, since all the 50 events occurring within 3 years from diagnosis were tumor recurrences. Primary tumor was treated with radiotherapy in 75 cases, surgical resection plus radiotherapy in 38, and radical surgery in 8. Chemotherapy was given to all patients and each program included adriamycin, vincristine, and cyclophosphamide ± dactinomycin. Median follow-up was 12 years, ranging from 6 to 19 years. The PFS rate decreased to 49% at 6 years and plateaued at 46% after the 7th year from diagnosis, even though some relapses were observed as late as 14 years from diagnosis. Second malignancies developed in 7 patients free from progressive ES and were represented by osteogenic sarcoma in previously irradiated bone in 4 cases and by breast carcinoma in 3. No other event but tumor relapse or second malignancy occurred in this series. EFS rate was 47% at 6 years and 39% at 12 years, further decreasing in the following years because of a number of late events. A continuous PFS longer than 7 years may be consistent with cure in the majority of patients with monostotic ES. However, these patients should be followed indefinitely because of risk of second malignancies. © 1994 Wiley-Liss, Inc.