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Modesto Carli

Publications and source records attributed to Modesto Carli.

At least 37 records · Page 2Linked to original sources
PubMed2005

SECONDARY LEIOMYOSARCOMAS: A Report of 4 Cases.

Survivors of childhood cancer risk developing second malignant neoplasms (SMN). a small proportion of SMNs is represented by soft tissue sarcoma (STS), including leiomyosarcoma. The files of the STS Italian Cooperative Group (ICG) were reviewed and 4 patients with secondary leiomyosarcoma were identified, only two of them still alive. In 3 cases, the leiomyosarcoma occurred in the radiation field. The authors found no clear differences between primary and secondary leiomyosarcomas, but leiomyosarcoma proved to be the most frequent SMN registered by the ICG, confirming the need for further studies to identify any distinctive features or syndromes.

Adolescent↗
PubMed2005

Early cerebral sinovenous thrombosis in a child with acute lymphoblastic leukemia carrying the prothrombin G20210A variant: a case report and review of the literature.

This paper describes the case of a male child with acute lymphoblastic leukemia who developed cerebral sinovenous thrombosis on day 6 of induction therapy with intrathecal methotrexate and methylprednisolone. A central venous catheter had been implanted the day before. The heterozygous prothrombin G20210A variant was found together with acquired activated protein C resistance and a reduced activated partial thromboplastin time. Clinical course and magnetic resonance imaging with magnetic resonance venography gradually improved over the following days after starting anticoagulant therapy (heparin and nadroparin). Cerebral sinovenous thrombosis is a serious disease in leukemic children, occurring in up to 6% of these patients. Data from the literature are in favor of anti-coagulant treatment, even though the efficacy and safety of thromboprophylaxis during chemotherapy in leukemic children with inherited thrombophilic conditions remain to be demonstrated.

Activated Protein C Resistance↗
PubMed2005

Expression and activity of vascular endothelial growth factor and metalloproteinases in alveolar and embryonal rhabdomyosarcoma cell lines.

Rhabdomyosarcoma (RMS) is a malignant tumour of skeletal muscle origin which includes two major histological subtypes: alveolar rhabdomyosarcoma (ARMS), the more aggressive, and embryonal rhabdomyosarcoma (ERMS). In order to establish whether the higher metastatic potential of ARMS cells may depend on differential expression of specific matrix metalloproteinases (MMPs) and angiogenesis-related factors, we studied the expression of MMP-2, MT1-MMP, TIMP-2, VEGF and VEGF receptors in four ARMS (RH30, RH4, RH18, RH28), three ERMS (RD, RH36, SMS-CTR) and one undifferentiated sarcoma cell line (A204). Semi-quantitative analysis of MMP-2 revealed high levels of expression in 3 out of 4 ARMS cell lines whereas, among ERMS, only RH36 showed comparable levels of the protease. TIMP-2 and MT1-MMP showed no significant differences among cell lines. in vitro invasiveness was also evaluated. The MMP-2-overexpressing RH30 cells were more invasive than RD cells, which expressed low levels of MMP-2. Exogenous expression of the ARMS specific PAX3-FKHR chimeric protein in RD cells increased MMP-2 activity and invasiveness. Of the three main VEGF isoforms only VEGF165 and VEGF121 were detected in RMS lines: ARMS expressed both isoforms, whereas the ERMS cell line SMS-CTR and the undifferentiated sarcoma cell line A204 showed the VEGF121 isoform only. All RMS cell lines expressed VEGFR-1 at mRNA as well as at protein level. The VEGFR-2, on the contrary, was undetectable with the sole exception of the RH28 cell line. Overall, our data suggest that a high level of MMP-2 protein and VEGF/VEGFR expression may contribute to the metastatic phenotype of ARMS cells and that exogenously induced PAX3-FKHR expression increases MMP-2 secretion and invasive capability of RMS cells.

Blotting, Western↗
PubMed2005

A prospective survey on incidence, risk factors and therapy of hepatic veno-occlusive disease in children after hematopoietic stem cell transplantation.

BACKGROUND AND OBJECTIVES: Veno-occlusive disease (VOD) is one of the most frequent complications after stem cell transplantation. We conducted a prospective survey of 244 hematopoietic stem cell transplants in children to determine the incidence of VOD, its main risk factors, treatment and effect on the transplant. DESIGN AND METHODS: Two hundred and forty-four hematopoietic stem cell transplants (HSCT) performed in 220 pediatric patients from 1993 to 2003 were evaluated. The series included 127 males and 93 females with a median age of 6.7 years at the time of transplantation. RESULTS: VOD was diagnosed following 26 of the 244 transplants (cumulative incidence 11%), but a higher incidence was found in patients with at least one known risk factor for VOD (cumulative incidence 20%). In multivariate analysis, risk factors for VOD were age < 6.7 years; type of VOD prophylaxis, and busulphan-containing conditioning regimens. Routine treatment of VOD was based on supportive care and, starting from 1999, defibrotide was used. All patients were monitored with daily Doppler ultrasound-(US) for early diagnosis of inversion of portal blood flow. Twelve patients developed inversion of portal flow (9 had severe VOD; 3 had moderate VOD) and were promptly started on fibrinolytic and anticoagulant therapy with heparin and recombinant tissue plasminogen activator (rt-PA). Hepatic flow reverted to normal in all 12 patients; only 4 patients ultimately developed multiorgan failure and died. The transplant-related-mortality (TRM) rate in patients with or without inversion of portal flow was 33% vs 7%, (p=0.1). The TRM in patients with or without VOD was 19% vs 8% (p=0.001). INTERPRETATION AND CONCLUSIONS: This study showed that younger age, type of VOD prophylaxis, and busulphan-based conditioning regimens are independent risk factors for VOD. Inversion of portal flow was found in 9 of 10 patients with severe VOD. Doppler US monitoring may be helpful in early identification of the patients with VOD-induced inversion of portal flow who might benefit from therapy with heparin and rt-PA.

Adolescent↗
PubMed2004

Vinorelbine and low-dose cyclophosphamide in the treatment of pediatric sarcomas: pilot study for the upcoming European Rhabdomyosarcoma Protocol.

BACKGROUND: Following their previous report on the activity of vinorelbine in the treatment of rhabdomyosarcoma, the authors report the results of a pilot study aimed at defining the optimal dose of vinorelbine when this agent is used in conjunction with continuous, orally administered low-dose cyclophosphamide to treat patients with refractory or recurrent sarcoma. It is hoped that the combination of vinorelbine and low-dose cyclophosphamide can be used as a maintenance regimen in an upcoming European trial involving high-risk patients with rhabdomyosarcoma. METHODS: In the current pilot study, the cyclophosphamide dose was fixed at 25 mg/m2 per day for 28 days. Vinorelbine was administered intravenously on Days 1, 8, and 15, with trial doses escalated from an initial level of 15 mg/m2 in steps of 5 mg/m2; intrapatient dose escalation was not allowed. RESULTS: Between April 2002 and November 2003, 18 patients ages 2-23 years were treated with the study regimen after having received 1-4 (median, 2) other regimens previously. Ninety cycles were administered in total (median, 5 cycles per patient; range, 1-10 cycles per patient). Two cases of dose-limiting toxicity (Grade 4 neutropenia in both cases) were observed among the 5 patients who received vinorelbine at a dose of 30 mg/m2. Of the 41 cycles in which vinorelbine was administered at a dose of 25 mg/m2, Grade > or = 3 neutropenia was observed in 15 (37%); no other major toxicity was documented in association with these cycles. One complete remission and 6 partial remissions were noted among the 17 patients who had measurable disease. Three of the eight assessable patients with rhabdomyosarcoma (which was embryonal in two cases and alveolar in one) had responses to treatment. CONCLUSIONS: Combination therapy involving vinorelbine and low-dose cyclophosphamide was found to be feasible and to possess activity against recurrent sarcomas. The maintenance therapy doses recommended for use in the upcoming European trial are cyclophosphamide 25 mg/m2 per day for 28 days and vinorelbine 25 mg/m2 on Days 1, 8, and 15.

Adolescent↗
PubMed2004

Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group.

BACKGROUND: Survivors of childhood malignancies have an increased risk of developing second malignant neoplasms (SMN) due to their prior treatment and/or genetic susceptibility. A small proportion of SMNs are soft tissue sarcomas (STS), whose prognosis is generally thought to be poor, though publications on such patients' treatment and outcome is limited. METHODS: The authors analyzed 25 patients who were registered for the Italian Cooperative Group protocols for pediatric STS from 1979 to 2000. The primary tumor was STS in five patients; Hodgkin disease in five patients; leukemia in four patients; retinoblastoma, neuroblastoma, and Wilms tumor in two patients each; and other tumor types in five patients. SMNs occurred after a median of 8 years (range, 1.9-15.0 years) and included rhabdomyosarcoma (RMS) in 4 patients, malignant peripheral nerve sheath tumor in 4 patients, extraosseous Ewing family tumor (EFT) in 4 patients, leiomyosarcoma in 3 patients, fibrosarcoma in 2 patients, synovial sarcoma in 2 patients, and other tumor types in 6 patients. Treatment generally was administered according to the guidelines for primary STS. RESULTS: Seven non-RMS patients with STS underwent surgery alone, whereas 18 patients received chemotherapy and 8 patients received radiotherapy. Retreatment was feasible with acceptable toxicity. Fifteen patients were alive in complete remission of their SMN at the time of last follow-up. Responses to chemotherapy and survival were satisfactory for patients with tumors such as RMS and EFT. Complete tumor resection was correlated with a favorable prognosis in patients with other types of STS and in patients with postirradiation sarcoma. Two patients developed a third malignancy. CONCLUSIONS: Although prior treatment may hinder the management of these patients, pediatric STS second malignancies can be cured using the same strategies used for de novo pediatric sarcomas. Long-term follow-up is mandatory given the risks of further malignancies and more severe, treatment-related side effects.

Adolescent↗
PubMed2003

Involvement of p53 in specific anti-neuroectodermal tumor activity of aloe-emodin.

Previously, we have identified aloe-emodin (AE) as a new type of anticancer agent, with activity that is based on apoptotic cell death promoted by a neuroectodermal tumor-specific drug uptake. We attempt to clarify the intracellular target of AE and the apoptosis-signaling pathway activated by AE in neuroblastoma cell lines. Two-photon excitation microscopy and spectroscopic titrations documented that AE is highly concentrated in susceptible cells and binds to DNA. One of the most important mediators of apoptotic response to genotoxic stimuli, such as anticancer agents, is the p53 tumor suppressor gene. To evaluate the role played by p53 in AE-induced apoptosis a p53 mutant cell line, which lacks transcriptional activity of p53 targeted genes, was tested. AE displayed a reduced growth inhibitory and pro-apoptotic activity in p53 mutant cells (SK-N-BE(2c)) with respect to the p53 wild-type line (SJ-N-KP). This effect was not caused by a reduced drug uptake in the mutant neuroblastoma cell line but was related to a different apoptotic cell phenotype. Whereas SJ-N-KP cells were susceptible to a p53 transcription-dependent pathway of apoptosis, SK-N-BE(2c) cells underwent apoptosis with up-regulation of p53 expression but not of p53-target genes. After AE treatment p53 translocates to the mitochondria inter-membrane space in both neuroblastoma cell lines. Due to its high accumulation in neuroectodermal tumor cells AE could also kill tumor cells harboring p53 mutant genes. This property would further contribute to AE specific anti-tumor activity and might be exploitable in the clinic.

Anthraquinones↗
PubMed2003

Rhabdomyosarcoma in infants younger than one year old: a report from the Italian Cooperative Group.

BACKGROUND: The management of rhabdomyosarcoma (RMS) in patients age < 1 year is particularly problematic and requires a tailored therapeutic approach. We report on the Italian Cooperative Group's 20-year study of 50 children with RMS who were age < 1 year at diagnosis. METHODS: Patients were treated using multimodality therapeutic approaches that were based on three consecutive protocols. Chemotherapy was administered to all patients, with dosages calculated according to body weight; calculated doses subsequently were reduced by 33-50%. Radiotherapy was administered to 10 patients. RESULTS: With a median follow-up of 76 months, 5-year event-free survival and overall survival rates were 42.3% and 61.7%, respectively. Local recurrence was the major reason for treatment failure. In particular, the local recurrence rate was high in patients who warranted radiotherapy but received none due to their age. Completeness of surgery and nodal involvement were the most significant prognostic factors. After a suitable reduction in dosage was made, acute toxicity was no different from what has been observed in older children. The most relevant toxic event was cardiotoxic death in a newborn (n = 1). CONCLUSIONS: The current study confirmed that the outcome for infants with RMS is less satisfactory than for older children and that infants with RMS require more careful monitoring and specific treatment guidelines. The absence of local control is the major cause of treatment failure; aggressive conservative surgery should be encouraged, but more radiotherapy may be advisable in selected cases. Intensive chemotherapy is essential; a 33% dose reduction may ensure adequate tolerance. In addition, patients age < 3 months should not receive anthracyclines.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Primary transcrotal excision for paratesticular rhabdomyosarcoma: is hemiscrotectomy really mandatory?

BACKGROUND: To evaluate the role of primary reexcision (PRE) with scrotal resection in patients with paratesticular rhabdomyosarcoma enrolled in the German-Italian Cooperative Studies. The authors compared patients who underwent this procedure, according to the protocol guidelines, with those who did not. METHODS: In 32 of 198 patients with localized disease, the primary surgery was performed through a noncorrect scrotal approach. Twenty-four patients underwent PRE as recommended by the protocol guidelines (Group A) and 8 did not receive this treatment (Group B). The Group B patients were treated with the same chemotherapeutic regimens as the Group A patients and no radiotherapy was given to either group. RESULTS: After PRE, residual tumor was not detected in 21 of the 24 Group A patients. Twenty patients are alive in first complete remission 26-250 months after diagnosis (median, 40 months), 2 are alive in second complete remission at 3 and 9 months from diagnosis of lymph node and lung recurrence, and 2 died of disease after lymph node and distant metastases at 16 and 13 months from diagnosis. Three-fourths of these patients were older than 10 years old and the tumor was larger than 5 cm. The eight Group B patients are all alive in first complete remission 24-250 months since diagnosis. CONCLUSIONS: The data on the eight patients who obtained local control without PRE or radiotherapy warrant further investigation. Because of the supposed high risk of contamination with subsequent microscopic residual tumor after a transcrotal approach, we emphasize the utility of PRE with hemiscrotectomy.

Child↗
PubMed2003

Role of surgery for nonmetastatic abdominal rhabdomyosarcomas: a report from the Italian and German Soft Tissue Cooperative Groups Studies.

BACKGROUND: In the current study, the authors aim to evaluate clinical features and treatment results observed in patients from the German and Italian studies who had nonmetastatic abdominal rhabdomyosarcomas (RMS). METHODS: One hundred sixty-one patients were observed; 78 registered in the German studies between October 1980 and August 1995, and 83 registered in the Italian studies between April 1975 and December 1995. The age range of the patients was 0-18 years (median, 4 yrs). The distribution of tumor sites was as follows: 32 intraperitoneal, 42 retroperitoneal, 75 pelvic, and 12 not otherwise specified (NOS). Most patients had a large and invasive primary mass (26 T1b, 114 T2b). The breakdown in histology was as follows: 116 embryonal, 34 alveolar, and 11 other (leiomyomatous, pleomorphic, and NOS); all cases were staged according to the Intergroup Rhabdomyosarcoma Studies (IRS) system. Nine Group I patients were treated after surgery with chemotherapy (CT) (radiotherapy [RT] was delivered to treat alveolar RMS in the 1991 German and 1988 Italian studies); 19 Group II patients received CT + RT (40-44 Gy); 133 Group III patients underwent neoadjuvant CT +/- surgery and/or RT (54 Gy) + CT. Different CT regimens (based primarily on the administration of vincristine, dactinomycin, doxorubicin, and cyclophosphamide or ifosfamide) were adopted. RT was not recommended for patients age < 3 years. RESULTS: The 10-year overall survival (OS) and progression-free survival (PFS) were 47.2% and 43.9%, respectively. The OS was related significantly to the following variables: histology (alveolar, 29.4% vs. nonalveolar, 52.1% [P = 0.0156]), tumor size (> 5 cm, 42.1% vs. < 5 cm, 81% [P = 0.005]), age (< 10 yrs, 51.4% vs. >or= 10 yrs, 27.8% [P = 0.02]), complete surgery at diagnosis or after CT (+/-RT) (70.4% vs. 34.4% without it [P = 0.0015]). Most patients who achieved the delayed local control had responded well to neoadjuvant CT. CONCLUSIONS: Tumor size, histology, age, and initial or delayed achievement of local control were important prognostic factors. Most relapsed patients had unfavorable outcomes.

Abdominal Neoplasms↗
PubMed2003

Disseminated neuroblastoma in children older than one year at diagnosis: comparable results with three consecutive high-dose protocols adopted by the Italian Co-Operative Group for Neuroblastoma.

PURPOSE: To compare the outcomes associated with modifications in three consecutive protocols employed by the Italian Co-Operative Group for Neuroblastoma (ICGNB) in disseminated neuroblastoma. PATIENTS AND METHODS: Between January 1985 and November 1997, a total of 359 children aged 1 to 15 years with newly diagnosed stage 4 neuroblastoma were enrolled in three consecutive protocols. Compared with ICGNB-85, the ICGNB-89 protocol contained two more chemotherapy cycles, and some drugs were given at greater doses, whereas in the ICGNB-92 protocol, the induction phase included a chelating agent, and individual cycles contained four drugs instead of two. RESULTS: A total of 330 of 359 evaluable children were included in this analysis; 106 children were treated with ICGNB-85, 65 children were treated with ICGNB-89, and 159 children were treated with ICGNB-92 protocols. Radical resection of primary tumor was carried out in 59.4%, 50.8%, and 57.9% of the patients, respectively. Major tumor response after induction therapy was achieved in 66.7%, 69.2%, and 68.6% of the patients, respectively. A total of 218 of 232 patients received consolidation therapy consisting of conventional chemotherapy in 65 patients and of high-dose chemotherapy in 153 patients. Disease recurrence or progression occurred in 82.1%, 69.2%, and 74.8% of the patients, respectively. Therapy-related deaths occurred in 1.9%, 12.3%, and 6.9% of the patients, respectively. Five-year overall survival (OS) for the three studies was 26%, 23%, and 28%, and event-free survival (EFS) was 19%, 17%, and 17%, respectively. CONCLUSION: The therapeutic modifications adopted in the ICGNB-89 and ICGNB-92 protocols were not associated with a significant improvement in response rate or in the 5-year OS and EFS as compared with the ICGNB-85 protocol. Attempts at intensifying chemotherapy were associated with greater toxicity.

Adolescent↗
PubMed2003

Renal cell carcinoma in children: a clinicopathologic study.

PURPOSE: To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC). PATIENTS AND METHODS: The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts. RESULTS: Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients. CONCLUSION: RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.

Adolescent↗
PubMed2002

Primary intracranial fibrosarcoma.

INTRODUCTION: Primary fibrosarcomas of the brain are very rare tumors, so that information regarding the treatment is scarce. We report the contributions that different therapeutic options made to the treatment of a child with one of these aggressive tumors. CASE REPORT: A 13-year-old boy underwent a complete resection of a left temporo-parietal mass that had been diagnosed as a fibrosarcoma by two independent pathologists. Adjuvant chemotherapy with vincristine, actinomycin-D, ifosfamide and Adriamycin was started, but after 3 months tumor relapse was evident. The boy subsequently received radiation therapy during which there was evidence of progressive tumor shrinkage. A second surgery was performed 6 months after radiotherapy and a small enhancing lesion, revealed to be gliosis, was resected. The child remains alive and well 44 months after diagnosis. CONCLUSION: Our experience supports the importance of total resection followed by radiation therapy, and radiotherapy should be started as soon as possible after surgical resection, rather than administering chemotherapy first.

Adolescent↗
PubMed2002

Clear cell sarcoma of tendons and aponeuroses in pediatric patients: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group.

BACKGROUND: Clear cell sarcoma (CCS) of tendons and aponeuroses is extremely rare in childhood and little information is available on its clinical management. Originally believed to be a type of melanoma of soft tissue origin, CCS is now considered a distinct clinicopathologic entity that behaves like a high-grade soft tissue sarcoma. We report on a series of 28 pediatric patients treated from 1980 to 2000 by the Soft Tissue Sarcoma Italian Cooperative Group and the German Cooperative Group. METHODS: Patients were treated with a multimodality therapeutic approach. Surgical resection was complete in 17 patients (mutilating in 3), radiotherapy was administered to 8 patients, and 20 patients received chemotherapy. RESULTS: After a median follow-up of 102 months (range, 19-238 months), the 5-year and event-free survival rates were 66.4% and 63.3%, respectively. Seventeen patients were alive in first remission, two were alive in second remission, and nine had died of disease. The response to chemotherapy in the 7 evaluable patients included one partial remission, one minor response, and five no responses. Radiotherapy contributed to achieving local control in four of six Intergroup Rhabdomyosarcoma Study (IRS) Group II patients. Statistically significant differences in outcome were evident according to IRS group, tumor size, and site. CONCLUSIONS: Our study confirms the aggressive behavior of CCS. Complete surgical resection represents the mainstay of treatment, and even the only treatment for patients with small tumors. Radiotherapy may control microscopic residual disease after surgery. Chemotherapy is ineffective and the prognosis is unfavorable for patients with unresectable and large tumors.

Adolescent↗
PubMed2002

Undifferentiated sarcoma of the liver in childhood: a curable disease.

BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis. METHODS: The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995. They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second-look operation in cases of residual disease. Radiotherapy was occasionally used (2 of 17 patients). RESULTS: Twelve patients are alive with follow-up ranging from 2.4 to 20 years. Eight underwent complete tumor resection either at diagnosis or after preoperative chemotherapy, and all are currently alive. After initial chemotherapy tumor reduction was evident in six of nine evaluable cases. Overall three patients died of disease and one of a surgical complication. One child died in second complete remission for a non-disease-related cause. CONCLUSIONS: The current prognosis of UESL no longer should be regarded as poor. Modern multimodal treatment and supportive therapy have improved survival.

Adolescent↗
PubMed2002

Malignant vascular tumors in children and adolescents: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group.

BACKGROUND: Malignant vascular tumors are extremely rare in childhood and few data on their clinical management are available. We report on a series of 18 children who had malignant vascular tumors, treated from 1980 to 2000 by the Italian and German Soft Tissue Sarcoma Cooperative Group. PROCEDURE: Twelve patients had angiosarcoma, four had malignant hemangioendothelioma, and two had Kaposi's sarcoma. Surgical resection was completed in six cases; radiotherapy was administered to 6 children, and chemotherapy to 14. RESULTS: After a median follow-up of 208 months, the 5-year survival and event-free survival rates were 30.9 and 20.8%. Six patients were alive, four in first remission (three had tumor < 5 cm, grossly completely resected), and two in second remission. Response to chemotherapy was evaluable in nine cases and was: six no response, two partial remission, one complete remission. CONCLUSIONS: Angiosarcoma and related malignant vascular tumors are aggressive neoplasms with a poor prognosis; their behavior in children seems no different from their adult counterparts. Complete surgical resection remains the mainstay of treatment, but is probably sufficient in only a minority of cases. Postoperative radiotherapy may have a role and could be added to improve local control. The role of chemotherapy is uncertain, but the high rate of metastatic spread prompts investigation into new chemotherapeutic approaches.

Adolescent↗