Научная статья на тему 'SOLID NON BRAIN TUMOURS. Soft Tissue Sarcomas'

SOLID NON BRAIN TUMOURS. Soft Tissue Sarcomas Текст научной статьи по специальности «Клиническая медицина»

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Текст научной работы на тему «SOLID NON BRAIN TUMOURS. Soft Tissue Sarcomas»

May 2016, Moscow, Russia




Experience of high-dose chemotherapy and autologous hematopoietic stem cell transplantation of pediatric patients with high risk rhabdomyosarcoma

Seung Min Hahn, Won Kee Ahn, Ju Yeon Lim, Jung Woo Han, Moon Kyu Kim, Chuhl Joo Lyu

Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea

Key words: rhabdomyosarcoma, autologous transplantation

Introduction. The treatment of rhabdomyosarcoma is guided on the 'risk group' assessment. Intermediate risk group patients with embryonal rhabdomyosarcoma have shown more than 80 % of survival rates while alveolar less than 60 %. Parameningeal rhabdomyosarcoma is also known as poor risk factor. High risk patients with metastases have poorer prognosis (lower than 50 % of survival) with current chemotherapy and local control methods. Hence several new approaches are required for the treatment of intermediate to high risk group rhabdomyosarcoma. Although several studies have failed to improve the outcome with high dose chemotherapy (HDCTx) and autologous hematopoietic stem cell transplantation (HSCT) in rhabdomyosarcoma, there are controversial opinions of the treatment.

Aim.To find the possible role of HDCTx, this study reviewed the experience of HDCTx followed by autologous HSCT in pediatric patients of newly diagnosed, high risk rhabdomyosarcoma. Materials and methods. We reviewed 20 cases of high risk rhabdomyosarcoma patients treated with HDCTx followed by autologous HSCT between 2002 and 2014 in our hospital. All stage IV patients and intermediate group with parameningeal tumors or alveolar pathologic subtypes were classified as high risk group in the study. The primary end point was overall survival (OS), which was defined as the duration of diagnosis to time of death by any cause. The secondary end point was event free survival (EFS). EFS was defined as the time from diagnosis to disease progression, recurrence or death. Data were analyzed using the Kaplan-Meier method, and tested by log-rank test. O

Results. Median age of the patients at diagnosis was 6.13 years old (range, 0.56 to 15.1 years), median age at transplantation was 6.85 years old (range, 1.21 to 15.6 years). oc

Fifteen patients were stage IV, 3 patients were stage III with alveolar subtype, 1 patient was stage I with alveolar subtype and 1 patient was stage III with parameningeal tumor. ^

Twelve patients received 2 or 3 times of HDCTx following HSCT while 8 patients received one course of HDCTx. Carboplatin, etoposide, with melphalan (CEM) and busulfan with ^

melphalan (BM) chemotherapy were commonly used conditioning methods. Ten patients expired; seven patients died due to disease progression, 2 with unknown cause, and 1 was oo

transplantation related mortality. OS was 49.5 ± 11.3 %, and EFS was 40.0 ± 11.0 %. The patients with complete remission at the time of HDCTx and HSCT showed better survival rate than patients with residual disease at the time of transplantation (80.0 ± 12.6 % vs. 20.0 ± 12.6 %, P = 0.015). Age over 10 years old and lower than 1 years old group had poorer I—

outcome compared with the patients with age between 1 to 10 years old (30.0 ± 14.5 % vs. 70.0 ± 14.5 %, P = 0.029). Bone and bone marrow involvement are known as poor o

risk factor of rhabdomyosarcoma and similar results were shown in our report. Patients with bone and bone marrow involvement showed lower survival rate than patients without 00

metastasis in the sites (12.5 ± 11.7 % vs. 74.1 ± 12.9 %, P = 0.001).

Conclusion. Survival rate of high risk rhabdomyosarcoma patients with HDCTx with autologous HSCT was comparable with historical reports, and some patients resulted in even better outcome; patients who have shown remission before HDCTx (80 %), and patients with 1 to 10 years old (70 %). Although our study has limitation of small number of patients, these finding suggest potential benefit of HDCTx and autologous HSCT in rhabdomyosarcoma.

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Results of treatment of soft tissue sarcomas at children during 12-years period

V.B. Makhonin1, R.R. Bayramgulov1,2, A.A. Gumerov2

Republican Children Clinical Hospital, Ufa, Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: CWS, soft tissue sarcomas

Introduction. Soft tissue sarcomas (STS) at children - morphorogically different group of diseases that is also different on the biological behaviour and treatment approaches. It takes 5-7 % in the structure of oncological diseases. Diseases is diagnosed usually at III-IV stages, prognosis is negative in this case. The most popular method of treatment is usage of CWS group protocol.

Aim. Our aim was analysis of children with STS treated with program therapy with CWS-2002P protocol.

Materials and methods. During the period 2004 - 2015 in our center it were diagnosed 60 children with STS (males - 34, females - 26) at the age from 20 days to 17 years old. The majority of them were teated with the help of CWS-2002 (n = 45) protocol, 10 - received non-protocol treatment, 5 - rejected from the therapy. Among the children with STS, received treatment with CWS-2002, 22 were diagnosed with rhabdomyosarcoma (13 - embrional, 6 - alveolar), 7 - synovial sarcomas, 6 - PNET, 10 - other histological types. Tumour localization: body - 15 cases, pelvis and urogenital tract - 11, extremities - 11, head and neck - 8. IRS staging system was used: I stage - 7 patients, II - 6, III - 22, IV - 10. Only surgical treatment were performed for 5 patients, surgery and radiotherapy - 1 patient. Other patients received chemotherapy with or without radiotherapy. According the CWS-2002 criterions 1 patient received treatment with low risk (LR), 5 patients - standard risk (SR), 29 - high risk (HR) and 10 - very high risk (VHR). Results. Among the patients with LR and SR all children are alive, but late relapse was diagnosed at 1 patient with SR, now this patient in second remission. Among patients with HR the prolonged remission was diagnosed at 17 patients, 1 patient relapsed and treated, 1 patients alive with signs of tumour; 10 children died: 7 - from disease progression, 2 - from toxicity of treatment and 1 - from intercurrent disease (AIDS). Among the VHR group 5 patients died from progression, 1 - in the process of therapy, 4 - alive in remission. Relapses were more frequent in HR and VHR groups - 14 patients from 45 with STS, 10 patients in this group died, 4 alive. Overall survival in the protocol group was 28/45 (62.2 %), relapse free survival - 24/45 (53.3 %), mortality - 17/45 (37.7 %).

Conclusion. Our data suggests that treatment approaches of CWS-2002P protocol can be done in the conditions of regional center. Results of our study is the same with declared. The following improvement of STS treatment schemes required especially for patients with HR group.


Rapidly progressive Kaposi's Sarcoma in an Iraqi boy received valproic acid: a case report and review of literature

Likaa Fasih Y. Al-Kzayer1, Peter Keizer2, Farah T. Abdulraheem3, Kenji Sano4, Kenichi Koike5

1Shinshu University; 2Dr. G.B. Cross Memorial Hospital, Eastern Health; 3Al-Hamdaniya General Hospital in Mosul; 4Shinshu University Hospital; 5Shinshu University School of Medicine


< Key words: Kaposi's sarcoma (KS), Classic Kaposi Sarcoma (CKS), Valproic Acid (VPA), Human herpesvirus-8 (HHV-8)

q Introduction. Kaposi's sarcoma (KS), a low-grade vascular neoplasm, is associated with human herpes virus (HHV)-8 Infection. Immunosuppression is an important cofactor

U in KS process. Classic KS (CKS) is an indolent disease affects commonly the Mediterranean elderly men. However, CKS is exceedingly rare in children and when it occurs is much more

< disseminated than adults.

¡^ Aim. To our knowledge this is the first pediatric CKS case from Iraq. Our patient was showing an unusual aggressive course of the disease while receiving valproic acid (VPA)

3 of the potential immune-suppressive effect.

uo Materials and methods. Case presentation: A six-year-old Iraqi boy who had cerebral palsy (CP) and epilepsy since he was 9-month-old, had received VPA to control his seizures.

He developed skin discoloration followed by nodules that disseminated proximally from the lower extremities to groin, face, ears and oral cavity, and then he died from severe lj- respiratory distress after 110 days from the disease evolution.

uo Results. KS diagnosis was proved by skin biopsy. As the patient was of Arab-Asian ethnicity, and was HIV-seronegative, accordingly, his condition best fitted the classic form of KS.

Recent studies showed the link of VPA with the reactivation of HHV-8. Accumulated experimental and clinical data elucidated that VPA induces T-cell suppression. Conclusion. Our report demonstratesa rare rapidly progressing pediatric CKS caseand highlightsthe possible roleofthe 5-years administered VPA and its challenging effect on cellular immunity based on recent studies. Thus, VPA could have promoted the development of the CKS in our patient. This report also recalls the need of pediatricians to consider CKS especially when skin lesion appears on the child's foot even in countries outside the geographical map of the disease.

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May 2016, Moscow, Russia



Application of punctional gastrostomy at children with sarcomas of soft tissue

with localization on head and neck

D.Yu. Kachanov, I.V. Zakharov,T.V. Shamanskaya, T.V. Sergeeva, E.S. Vasilyeva, M.V. Teleshova, D.V. Shevtsov, R.A. Moiseenko, A.V. Nechesnyuk, N.N. Merkulov, S.R. Varfolomeeva

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: percutaneous endoscopic gastrostomy, soft tissue sarcomas of the head and neck, children

Introduction. A pediatric patients with soft tissue sarcomas (STS) of the head and neck might need treatment strategies that include multimodality treatment with surgery, radiotherapy, and chemotherapy. A local spread of the tumor process and the intensive therapy including concurrent chemoradiotherapy can be performed a number of complications associated with malnutrition.

Aim. The present study was performed to evaluate the possibilities use to percutaneous endoscopic gastrostomy (PEG) in pediatric patients with STS of the head and neck. We spent analysis indications for PEG, evaluate feasibility staging at different stages of treatment, incidence estimate risk of complications.

Materials and methods. The study included 15 patients of 50 pediatric patients with STS of the head and neck at Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev from 06.2012 and 12.2015 (42 month). All patients was performed the percutaneous puncture endoscopic gastrostomy (PEG) under sedation in order to provide nutritional rehabilitation.

Results. Median age at the time of PEG was 54 month (range, 4.8-128.4). Twelve patients had rhabdomyosarcoma (80 %), two rhabdoid tumor (13.3 %), and one undifferentiated pleomorphic sarcoma (7.7 %).Median time from diagnosis to staging PEG was 18 days (range, 6-143). Specific indications for PEG included radiotherapy on the primary tumor site, malnutrition due to problems oral feeding due to the anatomical location of the tumor. All patients was performed PEG at the stage of intensive care. 1/15 (6.7 %) received relapse treatment, 1/15 (6.7 %) - radiation therapy. One of 15 patients had pancytopenia (neutrophil levels of less than 1 thousand/ml). He had not complications in the postoperative period. 3/15 (20 %) at the time of PEG received parenteral nutrition. 1/15 (%) - enteral nutritional support through via nasogastric tube. PEG tubes were successfully inserted into all patients. A feeding by PEG was started two days later. Immediate complications occurred in four (27 %) patients: 2/15 (13.3 %) had local pain or low grade sepsis, which respond to analgesics and appropriate antibiotics. 1/15 (6.7 %) had respiratory depression wich required the next day of the puncture of the anterior abdominal. The gastrostomy was preserved and had a function. And one of 15 patients had the presence of signs of peritonitis which necessitated PEG tube removal. Median duration of PEG was 135 days (range, 58-230). Conclusion. One of the methods of the nutritional rehabilitation in patients with STS of the head and neck is application feeding via gastrostomy. PEG can be performed both on stages of intensive care in patients with malnutrition and with a preventive strategies, for example, before start of radiation therapy. PEG is an established route for providing long term enteral nutrition. This procedure is associated with frequent minor morbidity and with low risk of complications.


Interstitial brachytherapy for childhood soft tissue sarcomas: long term disease outcome & late effects

Siddhartha Laskar, Nehal Khanna, Ajay Puri, Sajid Qureshi, Ashish Gulia, Tushar Vora, Bharat Rekhi, Seema Media,

Girish Chinnaswamy, Shashikant Juvekar, Subhash Desai, Yogesh Ghadi <

Tata Memorial Hospital, Mumbai, India ^


Key words: soft tissue sarcoma, children, interstitial brachytherapy, outcomes

Introduction. Radiation therapy forms an important component in the multimodality management of soft tissue sarcomas in children. Interstitial Brachytherapy (BRT) can be very effective in improving disease control & reducing long term toxicity.

Aim. To evaluate the clinical outcome & long term adverse effects of interstitial brachytherapy (BRT) for children with soft tissue sarcomas (STS).

Materials and methods. From September 1984 to Dec 2013, 84 children (median age 15 years, range 1 to 18) with STS who received BRT as part of loco-regional treatment were included. There were 49 males and 35 females. Majority (74 %) had primary lesions. Synovial sarcoma (30 %) was the most common histological type, and 44 % had high-grade lesions. Treatment included wide local excision and BRT with or without external beam radiotherapy (EBRT). Forty children (66 %) received BRT alone. Results. After a median follow-up of 70 months, the local control (LC), disease-free survival (DFS), and overall survival (OS) were 85 %, 74 %, and 77 %, respectively. LC was superior in patients with tumor size < 5 cm versus > 5 cm (96 % vs. 78 %, P = 0.08), symptom duration 2 months (100 % vs. 81 %, P = 0.06), and Grade I versus Grade II versus Grade III tumors (100 % vs. 95 % vs. 70 %, P = 0.02). Children receiving a combination of BRT and EBRT had comparable LC to those receiving BRT alone (76% vs. 89%, p=0.28). There was no significant difference in LC for patients receiving LDR versus HDR BRT (84 % vs. 95 %, P=0.31, for BRT alone; and 73 % vs. 83 %, P=0.60, for BRT + EBRT). Surgical wound healing complications was seen in 8 % patients. Subcutaneous fibrosis (31 %) was the commonest late complication followed by distal limb edema (5 %), joint stiffness (3.3 %), & bone growth abnormality (1.6 %). There was no neuropathy, non-healing ulcer or second malignancy.

Conclusion. Interstitial BRT with or without EBRT result in excellent outcome in children with STS. Radical BRT alone, when used judiciously in select groups of children, results in excellent local control and functional outcome with reduced treatment-related morbidity. ^

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Childhood non rhabdomyosarcoma soft tissue sarcomas pattern and outcome

Key words: childhood, soft tissue sarcoma, outcome

Introduction. Non rhabdomyosarcoma soft tissue sarcomas represent a group of heterogenous malignancies with varying grade and outcome. Most of data and managment experience are derived from cummulative experience in adult patients. Paucity of information in pediatric age group calls for extensive studies.

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Aim. the present study aimed to describe the overall survival, event free survival, clinical features, and treatment response in Non-Rhabdomyosarcoma soft tissue sarcomas (NRSTS). Materials and methods. clinical features and tumor characteristics data of all 66 NRSTS patients younger than 18 years was reviewed. All patients were treated at the National Cancer Institute, Egypt between January 2008 and December 2013. Surgery was the mainstay of treatment, while chemotherapy was administered to 35 patients, and only 15 patients received radiotherapy

Results. Synovial sarcoma and fibromatosis were the most frequent histotypes with a median age of diagnosis was at 11 years. Tumors were greater than 5 cm in 52 patients (79 %), high grade in 34 (51.5 %), originated from extremity in 42 (63.6 %) and metastatic at presentation in 14 (21 %). Overall survival at 5-year was 59.3 % for whole series of patients, 67.8 % in patients who underwent complete resection versus 63.6 % in patients who had marginal resection, 31.4 % in un-resected patients, and 20.8 % in patients presented with distant metastases. The Event free survival was 17 % for the entire group of study patients. Outcome was unsatisfactory in patients with high-grade tumors, non-extremity location of primary tumor, high-risk stratification and un-resected primary tumor. The rate of objective response to neo-adjuvant chemotherapy was 28 %. Highest rates of recurrence was in tumors > 5 cm in diameter, high-grade with positive surgical margins and received no adjuvant radiotherapy

Conclusion. Wide surgical resection of primary tumor with negative margins continues to be the mainistry of therapy. Local control was improved by the addition of postoperative RT to high-grade tumors with positive margins. Both adjuvant chemo- and radio-therapy should be directed to high-grade tumors specially if inadequate surgical margins and in neoadjuvant settings when complete resection could be facilitated by these modalities.

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Mohamed Fawzy Hasan

NCI, Cairo University

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