Soft Tissue Sarcoma or Malignant Mesenchymal Tumors in the First Year of Life: Experience of the International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor Committee

2005 ◽  
Vol 23 (19) ◽  
pp. 4363-4371 ◽  
Author(s):  
D. Orbach ◽  
A. Rey ◽  
O. Oberlin ◽  
J. Sanchez de Toledo ◽  
M.J. Terrier-Lacombe ◽  
...  
Keyword(s):  
International Society ◽  
Life Experience ◽  
Local Treatment ◽  
Newborn Infants ◽  
Local Therapy ◽  
Mesenchymal Tumor ◽  
Mesenchymal Tumors ◽  
Total Study Population ◽  
Undifferentiated Sarcoma ◽  
Malignant Mesenchymal Tumor

Purpose To describe the outcome of infants with a histologically confirmed diagnosis of malignant mesenchymal tumor (MMT) included in the International Society of Paediatric Oncology studies MMT 84 and MMT 89. Patients and Methods One hundred two infants (≤ 12 months old) were included. Twenty-four children were less than 3 months old, and 16 were less than 1 month old. Sixty-four patients had rhabdomyosarcoma (RMS), 26 had undifferentiated sarcoma, and 12 had other histology. Clinical TNM stage was stage I (41%), II (39%), III (6%), and IV (14%). First-line treatment was ifosfamide, vincristine, dactinomycin, whereas the second-line combination consisted of either cisplatin and doxorubicin (in MMT 84) or vincristine, carboplatin, etoposide/teniposide (in MMT 89). Chemotherapy doses were adapted to age. Local therapy was conservative surgery as often as possible. Results After a median follow-up of 7.8 years (range, 0.1 to 13 years), 5-year overall survival (OS) and event-free survival rates were 66% and 55% for the total study population and 72% and 60% for nonmetastatic patients, respectively. Only two of 13 stage IV patients survived. Sixty-seven percent of newborn infants survived. Infants with alveolar subtype had a poorer survival than those with non-RMS MMT or nonalveolar RMS (5-year OS, 37% v 75% or 82%, respectively; P = .002). When compared with older children with MMT, young age does not seem to be an important prognostic factor. Conclusion OS was satisfactory even when local treatment was not aggressive, although the prognosis was poor for infants with alveolar RMS or metastatic tumors. Chemotherapy toxicity was manageable with appropriate dose modification.

Treatment of Nonmetastatic Rhabdomyosarcoma in Childhood and Adolescence: Third Study of the International Society of Paediatric Oncology—SIOP Malignant Mesenchymal Tumor 89

2005 ◽  
Vol 23 (12) ◽  
pp. 2618-2628 ◽  
Author(s):  
Michael C.G. Stevens ◽  
Annie Rey ◽  
Nathalie Bouvet ◽  
Caroline Ellershaw ◽  
Françoise Flamant ◽  
...  
Keyword(s):  
Alkylating Agents ◽  
Randomized Study ◽  
Local Treatment ◽  
Local Therapy ◽  
Paediatric Oncology ◽  
Mesenchymal Tumor ◽  
Childhood And Adolescence ◽  
Free Survival ◽  
Malignant Mesenchymal Tumor ◽  
Better Than

Purpose To improve outcome for children with nonmetastatic rhabdomyosarcoma and to reduce systematic use of local therapy. Patients and Methods Five hundred three previously untreated patients aged from birth to 18 years, recruited between 1989 and 1995, were allocated to one of six treatment schedules by site and stage. Results Five-year overall survival (OS) and event-free survival (EFS) were 71% and 57%, respectively. Primary site, T-stage, and pathologic subtype were independent factors in predicting OS by multivariate analysis. Differences between EFS and OS reflected local treatment strategy and successful re-treatment for some patients after relapse. Patients with genitourinary nonbladder prostate tumors had the most favorable outcome (5-year OS, 94%): the majority were boys with paratesticular tumors treated successfully without alkylating agents. Patients with stage III disease treated with a novel six-drug combination showed improved survival compared with the Malignant Mesenchymal Tumor 84 study (MMT 84; 5-year OS, 60% v 42%, respectively). OS was not significantly better than that achieved in the previous MMT 84 study, but 49% of survivors were cured without significant local therapy. Conclusion Selective avoidance of local therapy is justified in some patients, though further work is required to prospectively identify those for whom this is most applicable. Exclusion of alkylating agents is justified for the most favorable subset of patients. The value of the new six-drug chemotherapy combination is being evaluated further in a randomized study (MMT 95).

Randomized Comparison of Intensified Six-Drug Versus Standard Three-Drug Chemotherapy for High-Risk Nonmetastatic Rhabdomyosarcoma and Other Chemotherapy-Sensitive Childhood Soft Tissue Sarcomas: Long-Term Results From the International Society of Pediatric Oncology MMT95 Study

2012 ◽  
Vol 30 (20) ◽  
pp. 2457-2465 ◽  
Author(s):  
Odile Oberlin ◽  
Annie Rey ◽  
José Sanchez de Toledo ◽  
Hélène Martelli ◽  
Meriel E.M. Jenney ◽  
...  
Keyword(s):  
High Risk ◽  
Soft Tissue ◽  
International Society ◽  
Pediatric Oncology ◽  
Local Therapy ◽  
Survival Advantage ◽  
Long Term Results ◽  
Undifferentiated Sarcoma ◽  
Response To Chemotherapy ◽  
Significant Difference

Purpose MMT95 was the fourth of a series of International Society of Pediatric Oncology (SIOP) collaborations for children with high-risk nonmetastatic soft tissue sarcoma (STS). The principal objective was to explore survival advantage for an intensified chemotherapy strategy in a randomized trial. Patients and Methods From July 1995 to June 2003, 457 previously untreated patients with incompletely resected embryonal rhabdomyosarcoma (RMS), undifferentiated sarcoma, and soft tissue primitive neuroectodermal tumor at all sites except paratesticular, vagina, and uterus, or with alveolar RMS were randomly assigned to receive either ifosfamide, vincristine, and dactinomycin (IVA) or a six-drug combination (IVA plus carboplatin, epirubicin, and etoposide) both delivered over 27 weeks. Cumulative doses were as follows: ifosfamide 54 g/m2 (both arms), epirubicin 450 mg/m2, etoposide 1,350 mg/m2 (six-drug regimen). Poor responders after three courses of IVA were to be switched to the other arm. Delivery of radiotherapy was determined according to site and/or response to chemotherapy with or without surgery. Results Overall survival (OS) for all patients was 81% (95% CI, 77% to 84%) at 3 years. No significant difference in outcome in either OS or event-free survival was noted between the two arms (3-year OS: 82% [95% CI, 76% to 86%] for IVA and 80% [95% CI, 74% to 85%] for the six-drug arm). Toxicity was significantly greater (infection, myelosuppression, and mucositis) in the six-drug arm. Overall burden of local therapy was consistent with data from previous SIOP studies and showed no difference between the two chemotherapy regimens. Conclusion Intensification of chemotherapy for nonmetastatic RMS and other chemotherapy-sensitive STS provides no survival advantage or reduction in the intensity of local therapy and adds toxicity.

Conservative Treatment for Girls With Nonmetastatic Rhabdomyosarcoma of the Genital Tract: A Report From the Study Committee of the International Society of Pediatric Oncology

1999 ◽  
Vol 17 (7) ◽  
pp. 2117-2117 ◽  
Author(s):  
Hélène Martelli ◽  
Odile Oberlin ◽  
Annie Rey ◽  
Jan Godzinski ◽  
Richard D. Spicer ◽  
...  
Keyword(s):  
Survival Rate ◽  
International Society ◽  
Pediatric Oncology ◽  
Genital Tract ◽  
Radical Surgery ◽  
External Beam Radiotherapy ◽  
Local Treatment ◽  
Complete Response ◽  
Conservative Surgery ◽  
Mesenchymal Tumors

PURPOSE: To report the results of a conservative multimodal approach in girls with nonmetastatic rhabdomyosarcoma (RMS) of the genital tract, treated in International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors 84 and 89 protocols. PATIENTS AND METHODS: From 1984 to 1994, 38 girls with RMS of the genital tract (vulva, vagina, uterus) were treated in SIOP protocols. With the exception of patients with rare small tumors, which were resected at the start of the studies, all patients received initial chemotherapy (CHT) (ifosfamide, vincristine, and actinomycin D). Local treatment including surgery, brachytherapy (BT), and external-beam radiotherapy (ERT) was given only to girls who did not achieve complete remission (CR) with CHT or who subsequently relapsed. RESULTS: The primary tumor originated in the vulva or vagina in 27 girls and in the uterus in 11. The overall survival rate (± SE) was 91% ± 6% at 5 years, and the event-free survival rate was 78% ± 7%. At a median follow-up of 5 years, 30 girls were alive and in first CR and five were alive and in second CR. Four patients treated with complete resection of the tumor at diagnosis received less CHT. Thirteen patients were treated with CHT alone. In 17 patients, local treatment was necessary to achieve complete local control, for a residual mass after initial CHT (10 patients), for viable tumor on biopsy (three patients), or for local relapse (four patients). The local treatment used was radiotherapy (RT) (ERT in three patients, BT in seven), radical surgery with uterine ablation (three patients), RT and radical surgery (three patients), and conservative surgery with RT (one patient). CONCLUSION: Girls with nonmetastatic RMS of the genital tract have an excellent prognosis. We found no difference in outcome between uterine and vulvovaginal RMS. Local treatment does not seem necessary in patients who have a complete response to CHT. When a local treatment is needed, BT may be an alternative to radical surgery or ERT.

Long-term follow-up of ifosfamide renal toxicity in children treated for malignant mesenchymal tumors: an International Society of Pediatric Oncology report.

1991 ◽  
Vol 9 (12) ◽  
pp. 2177-2182 ◽  
Author(s):  
A Suarez ◽  
H McDowell ◽  
P Niaudet ◽  
E Comoy ◽  
F Flamant
Keyword(s):  
Renal Function ◽  
International Society ◽  
Pediatric Oncology ◽  
Severe Toxicity ◽  
Mesenchymal Tumors ◽  
Primary Tumor Site ◽  
Malignant Mesenchymal Tumor ◽  
Long Term Follow Up ◽  
Chemotherapy Protocol ◽  
Beta 2

The renal function of 74 children with malignant mesenchymal tumors in complete remission and who have received the same ifosfamide chemotherapy protocol (International Society of Pediatric Oncology Malignant Mesenchymal Tumor Study 84 [SIOP MMT 84]) were studied 1 year after the completion of treatment. Total cumulative doses were 36 or 60 g/m2 of ifosfamide (six or 10 cycles of ifosfamide, vincristine, and dactinomycin [IVA]). None of them had received cisplatin chemotherapy. Ages ranged from 4 months to 17 years; 58 patients were males and 42 females. The most common primary tumor site was the head and neck. Renal function was investigated by measuring plasma and urinary electrolytes, glucosuria, proteinuria, aminoaciduria, urinary pH, osmolarity, creatinine clearance, phosphate tubular reabsorption, beta 2 microglobulinuria, and lysozymuria. Fifty-eight patients (78%) had normal renal tests, whereas 16 patients (22%) had renal abnormalities. Two subsets of patients were identified from this latter group: the first included four patients (5% of the total population) who developed major toxicity resulting in Fanconi's syndrome (TDFS); and the second group included five patients with elevated beta 2 microglobulinuria and low phosphate reabsorption. The remaining seven patients had isolated beta 2 microglobulinuria. Severe toxicity was correlated with the higher cumulative dose of 60 g/m2 of ifosfamide, a younger age (less than 2 1/2 years old), and a predominance of vesicoprostatic tumor involvement. This low percentage (5%) of TDFS must be evaluated with respect to the efficacy of ifosfamide in the treatment of mesenchymal tumors in children.

scholarly journals SURG-35. INTRACRANIAL MYXOID MESENCHYMAL TUMOR WITH EWSR1-ATF1 GENE FUSION

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii210-ii211
Author(s):  
Benjamin Ward ◽  
Christopher Wang ◽  
Rob Macaulay ◽  
James Liu
Keyword(s):  
Radiation Therapy ◽  
Gene Fusion ◽  
Histological Analysis ◽  
Treatment Plan ◽  
Mesenchymal Tumor ◽  
Tumor Control ◽  
Adjuvant Radiation ◽  
Middle Aged ◽  
Mesenchymal Tumors ◽  
Middle Aged Adult

Abstract Intracranial myxoid mesenchymal tumors (IMMT) carrying an EWSR1-CREB gene family fusion are extremely rare and have only been identified in ten relatively recent reported cases. There is some question as to whether this is a novel entity, or a myxoid variant of angiomatoid fibrous histiocytoma (AFH), given certain histopathological similarities. Despite these similarities, the increasing number of cases reported appear to demonstrate clear histological differences that indicate IMMTs are a distinct and novel entity. Previous reports have focused on histological analysis but have lacked detailed long-term clinical follow-up and recommendations regarding treatment approach. In this case, we describe a 48-year-old female who presented with a left intraventricular mass that was identified histologically as an IMMT with an EWSR1-ATF1 gene fusion. Following initial resection, the tumor demonstrated local recurrence. Repeat resection was performed followed by immediate demonstration of local, as well as distant, tumor recurrence. Subsequent histological analysis of the tumor demonstrated a myxoid mesenchymal tumor clearly distinct from AFH. Fractionated stereotactic radiation therapy was administered following the second resection and tumor control was achieved at 1 year. This case is particularly remarkable as it is only the second reported IMMT case to occur in a middle-aged adult, with all other cases occurring in children or young adults. The two middle-aged adult cases share striking similarities in clinical presentation, including a history of breast cancer. Our findings indicate that an intracranial myxoid mesenchymal tumor is a novel and rare entity that may demonstrate rapid local and distant recurrence. Given the aggressive recurrence seen with the presented case, we recommend the treatment plan to be surgical resection followed by adjuvant radiation therapy to maintain tumor control.

scholarly journals Botryoid-type Embryonal Rhabdomyosarcoma of Liver in a Young Cat

1997 ◽  
Vol 34 (6) ◽  
pp. 618-621 ◽  
Author(s):  
G. Minkus ◽  
M. Hillemanns
Keyword(s):  
Tumor Heterogeneity ◽  
Neuron Specific Enolase ◽  
Cell Types ◽  
Embryonal Rhabdomyosarcoma ◽  
Age Group ◽  
Pediatric Age ◽  
Mesenchymal Tumors ◽  
Pediatric Age Group ◽  
S 100 ◽  
Malignant Mesenchymal Tumor

An unusual malignant mesenchymal tumor arising in the liver of a 2-year-old cat is described. Histologically, the tumor showed considerable variation in growth pattern, cellularity, and cell types. Phenotypical diversity was confirmed by immunohistochemistry, showing expression of desmin, vimentin, S-100, and neuron-specific enolase in various areas of the tumor. On the basis of histopathology, immunohistochemistry, electron microscopy, and gross morphology, the tumor was classified as botryoid-type embryonal rhabdomyosarcoma. Differential diagnosis included so-called undifferentiated (embryonal) sarcoma of the liver, a rare tumor of the pediatric age group in humans. Problems of tumor heterogeneity and differentiation in mesenchymal tumors are discussed.

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