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23
RARE TUMOURS
ABSTRACT NO.: 0P-078
Malignant melanoma in children: etiology, treatment, and prognosis
Ali Varan, Ozay Gokoz, Figen Ozgur, Burca Aydin, Bilgehan Yalcin, Nilgun Kurucu, Tezer Kutluk, Canan Akyuz
Hacettepe University Cancer Institute, Ankara, Turkey
Key words: malignant melanoma, prognosis, treatment
Introduction. Primary malignant skin tumors are very rare in children. Most common skin tumor in children is malignant melanoma as in adult population. SEER reported the incidence of malignant melanoma in children to be 1.3/1 million. Several underlying pathological conditions like giant cell nevi, xeroderma pigmentosum, radiotherapy, some immunodeficiency disorders have been described in cutaneous malignant tumors. The survival rate is over 80 % while this ratio is lower when there is regional and distant metastasis. Overall survival rates in regional and distant metastasis were 60 and 25 %, respectively. Surgery, chemotherapy, and targeted therapy were used for the treatment of the patients. Aim. The aim of this study is to evaluate the etiology, treatment and prognosis of the malignant melanoma in children.
Materials and methods. Twenty-one patients who had been diagnosed with malignant melanoma between 1972 and 2015 were retrospectively analyzed. Staging was done according to AJCC. High dose interferon, or CDDP based regimens have been used. Mean and median values were used for demographic characteristics. Kaplan-Meier survival curves were used for survival analysis. The patient groups were compared in terms of survival duration using a log-rank test.
Results. We could define the etiologic factors in only 5 (23.8 %) patients. In three patients malignant melanoma occurred within the area of Giant hairy cell nevus, one melanoma patient previously had bone marrow transplantation due to Gricelli syndrome, one patient also had Xeroderma pigmentosum. Tumors were located in head and neck (7), trunk (5), ocular (2), upper extremity (2), lower extremity (2), and intracranial (1). Tumor location was not detected in two patients. Stage distributions were 0 (1), I (1), II (5), III (6), and IV (6). The stage was not defined in two patients. Six patients did not require treatment due to low stages. High dose interferon was given to 9 patients. Three patients were treated with cisplatin based protocols, and three with other chemotherapeutic regimens. Five patients died with disease progression. Overall survival was 75 %, whereas event-free survival rate oo
was 70 %. Prognostic factors on survival were treatment with interferon (P=0.001) and metastatic disease (P=0.01). 3
Conclusion. Malignant melanoma is rare tumor in childhood. Metastasis is the worst prognostic factor. Melanoma patients were treated successfully with high dose interferon in O
our series.
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SOCltTt INTERNATIONALE D'ONCOLOGIE PEHATRIQUE
SIOP ASIA CONGRESS
ABSTRACT NO.: P-117
Own experience of treatment of aggressive fibromatosis at children
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: desmoid, fibromatosis
Introduction. Aggressive fibromatosis (AF) (or desmoid fibroma) is a special variant of mesenchymal tumours of soft tissues, taking the place between non-malignant and malignant tumours. AF in contrast of malignant tumours doesn't have the possibility for metastasis forming but can infiltrate and relapse multiple times after surgical removing. Because of relapses and presence of non-resectable forms of AF, this disease requires complex approach of treatment. Despite of messages on possibility of usage of different schemes of chemotherapy, radiation and hormonal treatment, the real possibility of estimation of effectiveness of these methods is hard because of small numbers of observations. Aim. To perform the analysis of own results of treatment of patients with AF during 14 years.
Materials and methods. During the period of 2002-2015 it were treated 17 patients with AF at age 4 months to 16 years old in our center. The most frequent affected area were nates (9 patients). In all cases the first line of therapy was surgical treatment; macroscopical full resection was registered in 12 of 17 cases, biopsy only was done in 1 case. Five patients (older 1 year, total resection) in remission now with the follow-up from 6 to 11 years. Remission in these cases was reached only after surgical treatment and in 12 cases, adjuvant therapy was used. Applied scheme of chemotherapy: vinblastine and methotrexate 10 weeks - at 9 patients, and at 5 patients relapse was diagnosed. This situation requires repeated surgery and usage of the other scheme of therapy (VAC and etc.); radiation therapy was used at 3 children (total lesion dose - 44 Gy). Five children received prolonged therapy with vinblastine and methotrexate in combination with tamoxifen - 3 of them. Prolonged therapy duration was 2 years.
Results. As a result of treatment, remission was reached at 3 children (during 7 years), remission - 6 children (in terms from 3 to 9 years), late relapse - 2 children (in terms 3 and 5 years from treatment end).
Conclusion. 1. In case of possibility of radical surgery, the first line of therapy is resection of tumour. 2. In case of non-full resection, non-resectable tumours, in case of relapses (even in case of total resection of relapsed tumour) the adjuvant therapy is indicated. 3. Schemes with prolonged (from 6 months) usage of vinblastine and methotrexate (in several cases, especially at boys, tamoxifen can be used) is preferred. Usage of other medications and radiation therapy must be considered as second-line treatment.
ABSTRACT NO.: OP-176
Subcutaneous Ewing sarcoma: an indolent course of disease
E.S. Tyutikova, D.V. Litvinov, N.P. Makarova, V.Yu. Roshchin, A.P. Shcherbakov, N.V. Zhukov
Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia
Key words: a rare case, subcutaneous Ewing sarcoma, soft tissue sarcoma, oncology
Introduction. Ewing sarcoma is a highly malignant neuroectodermal tumour characterized by the presence of translocation with the involvement of EWS gene. Radical resection of tumour and modern aggressive chemotherapy programs make it possible to cure most of the patients, however without treatment all the patients die of disease progression within a short time. Most frequently in case of Ewing sarcoma, the primary site is located in bones, less frequently it occurs in the depth of soft tissue, extremely rarely - in the subcutaneous tissue and skin. To date, treatment approaches for Ewing sarcoma have not varied according to the localization of primary site, however available data suggest a fundamentally another prognosis for patients with superficially located primary site of the tumour. Yet a number of described cases of subcutaneous Ewing sarcoma is extremely limited. None of the cases illustrated the natural history of the disease (without specific treatment). Aim. Presentation of rare clinical setting.
oc Materials and methods. A fifteen-year-old patient with unremarkable development, negative familial history, without concomitant diseases. In July 2012 in the inguinal fold
^ area on the right a painless mobile tumour-like mass up to 2 cm in diameter appeared. The total biopsy performed by a local pathologist showed no signs of malignant growth.
^ About 1.5 years later (11.2013) the mass growth up to 1.5 cm in diameter was detected again in the same area. After the excision biopsy performed by the local pathologist the patient
3 was diagnosed with dermal cylindroma (adenocyctic canser). The material was sent to the reference laboratory of our clinic, where on the revision of all biopsy materials the following
diagnosis was established: ES with high proliferative activity (Ki-67 > 80 %) and EWSR1 gene rearrangement in t(11;22). However, the patient discontinued surveillance and was referred to our clinic only in September 2014 (after more than 2 years after initial biopsy). A detailed examination that included computer tomography, magnetic resonance imaging and positron emission tomography revealed no abnormal foci except for 3 palpable mobile foci of 0.5-1.5 cm in size in the area of postoperative cicatrix. A total biopsy was performed on these lesions and the presence of Ewing sarcoma in the lesions was confirmed.
Results. Thus, in spite of high proliferative activity and the presence of proven genetic rearrangement typical for ES, the subcutaneously located tumor had clearly distinct clinical course - the self-limitation of tumor mass volume (no more than 2 cm in size), the absence of distant metastases for more than 2 years. Despite this, the treatment according to CWS-2009 protocol was initiated, however after 3 blocks of therapy, the patient refused further treatment. To date, the patient is alive without any signs of recurrence. Conclusion. The given case is indicative of specific clinical course of subcutaneous Ewing sarcomas that probably require less aggressive treatment.
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ABSTRACT NO.: P-205
Congenital atypical teratoid/rhabdoid tumor of the brain, rhabdoid tumor of the kidney with heart metastases in a 2-months-old girl. A case report
K. Rastoltsev1, R. Burenkov1, D. Lantsov2, N. Kishchenko2, S. Mamtchenko3, E. Tashirova3
1Kaluga Regional Children's Hospital, Russia; 2Kaluga Regional Oncology Dispensary, Russia; 3Morozov Children's City Clinical Hospital, Moscow, Russia
Key words: atypical teratoid/rhabdoid tumor, AT/RT, rhabdoid tumor
Introduction. Atypical teratoid/rhabdoid tumor (AT/RT) and rhabdoid tumor of the kidney (RTK) are representatives of a malignant rhabdoid tumor family. AT/RT is a very rare, malignant, embrional tumor of the central nervous system. According to WHO Classification 2007 of tumours of the Central Nervous System, AT/RT belongs to embrional brain tumors, grade IV. RTK according to WHO Classification 2004 of Renal Tumors mainly belongs to mesenchymal tumors of the childhood. The part of AT/RT, according to various authors, consists of 1-3 % of all CNS tumors of the childhood; with the frequency of diagnostic tests three of one million children. Synchronous primary AT/RT with RTK are extremely rare and occur in 2-15 % of all cases AT/RT. A development of AT/RT connected with deletion and/or mutation in 22q11.2, where INI1 gene is located. Protein, encoded by this gene provides more efficient access to transcription unit and it is a biological, epigenetic supressor of the tumor's growth with a complex mechanism of the biological activity. Aim. We report a case of congenital AT/RT of the brain and rhabdoid tumor of the kidney with heart metastases.
Materials and methods. Patient G., hypotrophic girl at the age of two months and ten days. RT of the brain and the right kidney were diagnosed at her birth. Recommended chemotherapy protocol"EUROHAB"was not started because of her poor condition. Death from multiple organ dysfunction syndrome at two moths and ten days. On autopsy a median tumor was found, located supra- and infratentorial, measured 10 cm in its greatest dimension, with spreading to cerebellum lobes, superior vermis, brain stem, aqueduct, third and fourth ventricles (with their full obliteration). In the right kidney a dense, white, encapsuled tumor was found, measuring 14 cm, taking up to 90 % of entire organ volume. In lateral wall of left cardiac ventricle two dense, white, exofit tumor nodules were found, 0.8 cm in size, spreading into miocardium by 0.3 cm. In liver, left kidney, both adrenal glands, lymph nodes multiple metastases were found up to 5.5 cm in size.
Results. Hystopatologically, brain tumor was hypercellular, mainly presented by primitive basophilic neuroectodermal cells, which form primitive pseudorosettes and "glandular" structures, solitary rhabdoid cells. Right kidney tumor along with metastases were presented by vast fields of rhabdoid cells with solitary multinuclear forms. Immunohistochemically, tumor expressed Vim, CD56, however INI1 was absent. In kidney tumor and metastases additional expression of EMA, Syn was found. Conclusion. AT/R, RTK with heart, liver, contralateral kidney and lymph nodes metastases, "atipical" expression primitive neuroendicrine cell antigenes (CD56, Syn).
ABSTRACT NO.: OP-209
Colorectal carcinoma in children and adolescents. Single center experience
Asmaa Mohammed Hamoda, Ahmed Ibrahim Elhemaly, Wael Zekri Khalid
National Cancer Institute Egypt, Cairo, Egypt
Key words: colorectal carcinoma
Introduction. Colorectal carcinoma is extremely rare in children and presents with a poor prognosis. Surgical management and long-term follow-up of this entity are still obscure because of lack of data.
Aim. To evaluate the clinical characteristics of childhood colorectal carcinoma and to determine the predictors of poor outcome.
Materials and methods. Records of children who had colorectal carcinoma and were treated at our institute were reviewed retrospectively between 2008-2014. Information
recorded for each patient included age, sex, predisposing factors, positive family history, clinical characteristics, diagnostic procedures, extent of disease, treatment methods, ^
histological types, and outcome. ^
Results. There were 7 males and 8 females who were treated for colorectal carcinoma, their ages ranged from 13-17 years. Predisposing conditions were found in 2 patients, one with 3
familial adenomatous polyposis and another with neurofibromatosis type 1.4 cases had positive family history with cancer colon. Predominant symptoms were picture of intestinal ^
obstruction in 9 cases, weight loss in all cases, rectal bleeding in 5 cases. Colon was the site for the primary tumor in 10 patients, while rectum was the primary site in 5 patients. ^
Histological type in all patients was mucinous adenocarcinoma (100 %). Surgical intervention was just endoscopic biopsy in 7 patients, complete resection was done in 8 patients. oc
All patients except one- who did complete surgical excision with absence of lymph node involvement - received adjuvant chemotherapy, and 1/5 of rectal adenocarcinoma received
rectal radiotherapy. 9 patients died of disease in a period ranging from 1 month to 1 year after initial surgery. 7/9 patients who died did just biopsy without complete resection
and the other 2 patients, 1 had NF1 and developed irresectable HGG and died out of progressive brain tumor and the other one had history of familial adenomatous polyposis
and died out of disease progression. O
Conclusion. Delayed diagnosis, advanced stages of disease at presentation, delayed surgery and, most importantly, mucinous type of histology are the major determinants of poor
outcome in childhood colorectal carcinoma. We emphasize that possibility of a malignant colorectal tumor should be considered for any childhood case with signs and symptoms
of intestinal obstruction, intractable abdominal pain, alteration in bowel habits and gastrointestinal bleeding.
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SOCIÉTÉ INTERNATIONALE
D'ONCOLOGIE PÉMATRIQUE _
GpS^inth SIOP ASIA eLSiT IU CONGRESS
INTERNATIONAL SOCIETY OF PAEDIATRIC ONCOLOGY
ABSTRACT NO.: PP-229
Pediatric inflammatory myofibroblastic tumor: Experience of multicenter cooperation
D.Yu. Kachanov1, T.V. Shamanskaya1, A.M. Suleimanova1, S.R. Talypov1, N.N. Merkulov1, R.S. Oganesyan1, N.G. Uskova1, M.V. Teleshova1, Yu.V. Olshanskaya1, A.N. Kazakova1, V.Yu. Roshchin1, D.M. Konovalov1, D.S. Burmistrov2, A.V. Filin2, A.F. Matytsyn3, S.V. Kaplunov4, S.R. Varfolomeeva1
1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Russian Scientific Centre of Surgery named after Academician B.V. Petrovskiy, Moscow, Russia; 3Tambov Regional Children Clinical Hospital, Russia; 4Volgograd Regional Clinical Oncology Dispensary, Russia
Key words: Inflammatory myofibroblastic tumor, children, rare tumor, ALK
Introduction. Inflammatory myofibroblastic tumor (IMT) is a rare type of childhood malignancies with intermediate biologic behavior.
Aim. The aim of the study was to analyze clinical data and treatment results in a cohort of patients with IMT treated in four Russian children's hospitals.
Materials and methods. 9 patients with IMT for the period of 01.2012-12.2015 (48 months) were included in the analyses. Central review of histology was obligatory. Anaplastic
lymphoma kinase (ALK) immunohistochemistry and FISH for ALK gene rearrangements were performed in all cases. Surgery was the first treatment option.
Results. Male: female ratio was - 0.8:1. The median age at the diagnosis was 61.9 months (range 5.9-102.5). Tumor detection was an incidental finding in 3 (33.3 %) patients.
Most frequent symptom was fever - 4 (44.4 %). Tumor located in lungs - 4 (44.4 %), abdomen - 4 (44.4 %) and the liver - 1 (11.2 %). Regional lymph node involvement was observed
in 1 (11.1 %) case. Laboratory findings included leukocytosis (4/9), anemia (5/9) and thrombocytosis (7/9). ALK expression was revealed in 4/9 (44.4 %) cases. ALK rearrangements
were not detected in 4 studied cases. All patients were operated. Extent of the surgery included gross total excision in 8/9 (88.9 %), biopsy - 1/9 (11.1 %). Microscopic positive margins
were confirmed in 2/8 (25 %) cases. Surgery was the only therapy in 6/9 (66.7 %), 2/9 (22.2 %) patients with positive margins received celecoxib, 1/9 (11.1 %) with unresectable
tumor was treated with chemotherapy without response. All patients are alive. Median follow-up time was 10.5 months (range 1.3-27). 2/9 (22.2 %) relapses were observed.
Time to relapse was 5.9 and 9 months. Both patients received surgical procedure.
Conclusion. Our data confirm lungs and abdomen as the most frequent sites of the disease. Surgery is the mainstay of therapy. More data are needed regarding the role of adjuvant therapy in IMT.
ABSTRACT NO.: PP-243
Clinical experiences of infantile hepatic hemangioendothelioma
Jun Eun Park1, Leehuck Gil1, 2
Ajou University School of Medicine Suwon, Korea; 2Ajou University Hospital, Suwon, Korea
Key words: hemangioendothelioma, infant, hepatic
Introduction. Infantile hepatic hemangioendothelioma is a rare but most common hepatic vascular neoplasm in less than six months old infants. Aim. We tried to review the outcomes and treatment methods in infantile hepatic hemangioendothelioma.
Materials and methods. A retrospective review of 6 patients (2 males, 4 females) with infantile hepatic hemangioendothelioma at the Ajou University Hospital between 2001 and 2015 was performed.
Results. Six patients with infantile hepatic hemangioendothelioma were identified. Median diagnosis with infantile hepatic hemangioendothelioma was 18 days (range, 1 to 91 days). Characteristics at the time of diagnosis were varied. Two patients were diagnosed with infantile hepatic hemangioendothelioma by prenatal ultrasonography, cd 2 had hepatomegaly, 1 had congestive heart failure, and 1 was diagnosed incidentally in the absence of symptoms. All patients were diagnosed by imaging study, and 2 patients were
q confirmed by pathologic diagnosis. Treatment options were selected by considering the patient's symptoms and the characteristics of the tumor. Three patients were observed without
S treatment, 1 patient with congestive heart failure and multilobar tumors was treated by hepatic artery embolization with medical therapy. One patient received interferon alpha
^ and subsequent surgical resection, 1 patient with possibility of hepatoblastoma received only surgical resection. Treatment outcome showed benign disease course of infantile hepatic
w hemangioendothelioma. Overall, two patients without treatment revealed > 60 % decrease in tumor size and 1 patient without treatment showed complete tumor disappearance.
< One patient who underwent surgical resection because of possibility of hepatoblastoma was in stable condition without recurrence. One patient who received interferon alpha during
6 months and subsequent surgical resection due to intermittent congestive heart failure symptoms showed > 50 % decrease in tumor size after medical treatment with complete resolution of tumor after liver lobectomy. One patient who received initial hepatic artery embolization due to the unstable condition of congestive heart failure maintained 12 months of steroid and 9 months of interferon alpha and showed > 50 % decrease in the size and number of lesions with degenerative change.
Conclusion. Infantile hepatic hemangioendothelioma is rare but can be cured with surgical or/and medical approaches. Clinical symptoms are key point in selecting treatment options.
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ABSTRACT NO.: P-323
A case of neuroglial dystopia of nasopharynx, pterygomaxillary fossa, submandibular area
V. Alexeeva, L. Privalova, K. Isaev
Nizhny Novgorod Regional Children Clinical Hospital, Russia
Key words: neuroglial heterotopia, dystopia
Introduction. Heterotopy (dystopia, ectopia) is traditionally considered to be a congenital abnormality. During embryogenesis, due to the cells migrating from one primitive tissue layer to another, the anlage place of an organ or tissue is changed, there is the replacement of cells within a germ layer, or secondary replacement of organs and/or tissues. The tumours resulted from embryonic replacements are called heterotopic. Neuroglial heterotopias (NHs) are rare congenital abnormalities characterized by the presence of differentiated tissues of neuroectodermal genesis in the places, where, as a rule, there are no neuron and glial cells. The places usually affected are head and neck areas, and the lesions have been described more frequently in such extracranial loci as the nose, nasopharynx, oral cavity, oropharynx, palate, tongue, lips, scalp and orbits. Aim. The purpose of the study was to demonstrate a case of neuroglial dystopia recorded in Nizhny Novgorod region.
Materials and methods. A 1-month-old girl, Arina K., was admitted to Nizhny Novgorod Regional Children Clinical Hospital on October, 23, 2015. The child was from the first
pregnancy, the mother had a threatened miscarriage during the 7-8 weeks of gestation period. The delivery was first, at term. The weight at birth was 3600.
Results. Since birth the newborn was noticed to have"snoring"breathing. The patient was found to have the right submandibular lesion, and 13 days after birth she was admitted to Nizhny
Novgorod Regional Children Clinical Hospital. On admission: full blood count dated 24.10.2015: Hb 165 g/l, leucocytes 15.4 x 109/l, platelets 523 x 109/l, ESR 1 mm/h. biochemical blood
assay: LDH 5.3 pkat/l-h, ALT 0.16, AST 0.31 (norm), urea 1.9 mmol/l, total bilirubin 41 pmol/l, total protein 56.6 g/l, electrolytes - norm, HCE 20 ng/ml, ferritin 227 ng/ml.
Brain neurosonography dated 24.10.2015 showed no signs of altered brain structures. Chest X-ray dated 24.10.2015 showed the mediastinum in the upper and middle respiratory
tract to be dilated to the right due to thymus, the trachea being tortuous.
Abdominal ultrasound dated 24.10.2015 revealed no pathology. Ultrasound of soft tissues dated 24.10.2015 showed the signs of the right cervical soft tissue lesion. Neck soft tissue MRI (27.10.2015): the presentation of tumour of the cervical soft tissues on the right. On October, 28, 2015 the patient underwent the surgery for particle removal (biopsy) of the right cervico-submandibular tumour. Histological and immunohistochemical studies revealed congenital neuroglial heterotopy. The patient was readmitted to the Oncology Department for a follow-up. Cervical soft tissue ultrasound dated 15.12.2015: the signs of solid tumour on the right lateral neck side, 21.9 x 26 x 34.1 mm in size. Neurosonography dated 18.12.2015 revealed no pathology. Parotid MRI dated 18.12.2015 showed the signs of the lesion of the soft tissues of the right parotid area, irregularly shaped, with clear contours, up to 23 x 37 x 37 mm in size, extending to the infra-temporal region and pterygomaxillary fossa, the nasopharynx soft tissues, pushing aside and deforming the right parotid salivary gland. The laryngopharynx lumen is significantly narrowed and deformed. Compared to the previous studies, no significant changes were found. Conclusion. Thus, the follow-up showed no negative changes. An oncologic follow-up is recommended and scheduled.
ABSTRACT NO.: OP-325
Rare types of lung tumors in children. Experience of Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev
D.Yu. Kachanov, M.V. Teleshova, T.V. Shamanskaya, A.M. Suleimanova, S.R. Talypov, N.N. Merkulov, R.S. Oganesyan, N.G. Uskova, D.M. Konovalov, V.Yu. Roshchin, Yu.V. Olshanskaya, A.N. Kazakova, S.R. Varfolomeeva
Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia
Key words: inflammatory myofibroblasts tumor, pleuropulmonary blastoma, fetal lung interstitial tumor, rare tumors, children
Introduction. Tumors of the lung in children are a heterogeneous group according to the degree of the tumor malignancy, extremely rare in a cohort of children up to 14 years. Aim. To show and analyze the rare cases of primary lung tumors in children treated at the Federal scientific clinical center of Hematology, Oncology and Immunology in the period of 12.2012-12.2015 years.
Materials and methods. in the FRCC in the period of 12.2012-12.2015 years (36 months) were treated 9 patients with primary malignant neoplasms of the lungs. We analyzed the nosology, age at diagnosis, clinical and laboratory presentation of disease, stage. The diagnosis was established on the basis of histological examination in the laboratory of the pathology FRCC. Reference of the part of histological samples were made in international registry of pleuropulmonary blastoma (USA). Patients received therapy according to the diagnoses. <
Results. from 9 patients, 4 pts (44.4 %) had inflammatory myofibroblasts tumor (IMT), 2 pts had pleuropulmonary blastoma (PPB), (22.2 %, 1pt - type I, transformation in type II; 1pt - type III), in 1 case (11.1 %) fetal lung interstitial tumor (FLIT) was confirmed, 1pt (11.1 %) had mucoepidermal carcinoma (MEC) and 1pt (11.1 %) had leiomyosarcoma (LMS). The median age at diagnosis was 51 months (4 months - 16 years). The patient with FLIT diagnosis was made in 4 months. Diagnosis of patient with LMS was verified postmortem. M/F racio was 1/1.25. In most cases, the disease was presented by initial pulmonary and intoxication symptoms (7 pts, 77.7 %), 2 pts (22.3 %) - the tumor was accidentally detected during the examination. The tumor was localized in the right lung in 6 cases (66.6 %), in the left lung in 3 cases (33.4 %). Initial metastatic lesion was detected in 1 case (12.5 %, PPB). All patients underwent surgical treatment: R0-resection - 5 cases (55.5 %, 4 pt. - IMT, 1pt. - MEC); R2-resection - 1 case (11.1 %, PPB), resection degree was unknown in 2 cases (22.2 %, PPB, FLIT), autopsy was performed in 1 case (11.1 %, LMS). Chemotherapy in patients with PPB was performed according to the recommendations of the PPB registry. In the case of a patient with FLIT chemotherapy was performed according to the recommendations for patients with PPB. After the revision of histological specimens in the PPB registry, was recommended dynamic observation. IMT was subjected only to surgical interference. The median follow-up time was 19 months. (0.5 to 32 months). Outcome: 6 patients alive, NED (66.6 %), 2 patients alive with relapse (22.2 %, PPB) alive with recurrence, 1 patient died before treatment begun of disease progression (11.2 %, IMT). Conclusion. Malignant tumors with initial lung involvement in children are rare with a predominance of IMT. Outcome of the therapy directly depends on the of surgical intervention radicality. The chemotherapy decision in patients with FLIT requires further discussion because of the extreme rarity of the disease and the short median follow-up.
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SOCltTt INTERNATIONALE D'ONCOLOGIE PEHATRIQUE
SIOP ASIA CONGRESS
ABSTRACT NO.: OP-334
Intratissue infrared lazer thermotherapy in the treatment of kaposiform hemangioendothelioma
I.A. Abushkin1, A.G. Denis2
1South Ural State Medical University, Chelyabinsk, Russia; 2Children's Regional Hospital, Tver, Russia
Key words: intratissue infrared lazer thermotherapy, kaposiform hemangioendothelioma, minimally invasive method, diagnosis
Introduction. Kaposiform hemangioendothelioma (KHG) is a rare vascular tumor characterized by local aggressive growth. KHG is often accompanied by coagulopathy and thus, is associated with Kasabach—Merritt phenomenon. KHG is typically registered in newborns and infants, in equal number of males and females. KHG in adults is extremely rare. The prevalence of this disease is unknown. Published clinical trial data (S.E. Croteau et al., 2012) show that on average 0.071 cases of KHG per 100 000 children are diagnosed. A cause factor or genetic predisposition to this type of tumors are unknown. The most common complication of the tumor is hemorrhage that in 10-40 % of cases results in death. For the first time this tumor was described as a self-consistent neoplasm in 1993 (L.R. Zukerberg et al.). Diagnosis: symptoms, complete blood count with platelets, findings of Doppler ultrasound (US), contrast magnetic resonance imaging/computed tomography. If the results of the above investigations are not informative, a histopathological examination is performed. However, it should be taken into account the complexity of histopathology in the presence of concomitant thrombocytopenia and high risk of hemorrhage. There are many methods of treatment: 1) surgical resection of tumor, that is not always possible owing to the risks stated above; 2) drug treatment with hormonotherapy, cytostatic drugs, Vincristine and others, interferon-alpha; last year data on administration of the new medication Sirolimus were received; 3) X-ray treatment and embolization. Due to the fact that this disease is rare, there is still no uniform adequate treatment approach. Aim. The improvement of treatment outcomes of KHG.
Materials and methods. We used the method of intratissue infrared lazer thermotherapy in the treatment of KHG for the first time ever. Semiconductor and fiber-optic lasers with the radiation wavelength of 970 and 1560 nm respectively were applied. Four children (an equal amount of boys and girls) aged 5-7 months old with various KHG localization underwent treatment. KHG localization: in 1 child - in the region of superior surface of the lower leg (3 % of body surface area (BSA) involvement), in 1 child - in the lumbar region (2 % of BSA), in 1 child - an extensive KHG in the thoracic region with the spread to the lateral surface of the trunk and to the back (7 % of BSA) and in 1 patient - an extensive KHG in the abdominal region with the spread to the chest and lateral surface of the trunk (9 % of BSA). In children with extensive KHGs a concomitant Kasabach-Merritt phenomenon was observed (platelets up to 20 x 109/l). For KHG treatment we applied the method of intratissue thermotherapy under ultrasound control with anaesthesia. Semiconductor laser with the radiation wavelength of 970 nm and a fiber-optic laser with the radiation wavelength of 1560 nm were used. All 4 patients were found to have false initial diagnosis: lymphangioma, hemangioma, phlegmon of the trunk.
Results. In patients with extensive KHG, after the first procedure of intratissue lazer thermotherapy tumors have decreased in size significantly, platelet normalization was achieved. Full recovery after 1 procedure was registered in the child with the disease localization in the lumbar region. Full recovery after 2 procedures was detected in the child with the disease localization in the region of lower leg. After the 1st procedure of intratissue lazer thermotherapy, the significant improvement with normalization of general condition was registered in 2 children with extensive KHG.
Conclusion. Intratissue infrared lazer thermotherapy is a minimally invasive method of KHG treatment and may be recommended to wide use in cases of this rare and severe disorder.
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ABSTRACT NO.: PP-382 Desmoplastic small round cell tumor in children and adolescents: single institution study
Jung Yoon Choi, Hyoung Jin Lee, Che Ry Hong, Kyung Taek Hong, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin
Cancer Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea
Key words: desmoplastic small round cell tumor, pediatrics, Korea
Introduction. Desmoplastic small round cell tumor (DSRCT) is an aggressive malignancy with a poor prognosis. DSRCT is a rare disease, and therefore a standard treatment regimen has not been established.
Aim. In this study, we reviewed the clinical characteristics and treatment outcomes of pediatric DSRCT patients.
Materials and methods. We retrospectively reviewed the medical records of 5 DSRCT patients (2 boys, 3 girls) that were diagnosed and treated with DSRCT at Seoul National University Children's Hospital from January 1999 to January 2015.
Results. The median age at diagnosis was 11 years 5months (range 4 years 10 months - 17 years 2 months). The most frequent symptoms were abdominal pain (60 %) and palpable mass (40 %). The primary sites were gastrointestinal tract, bladder, and omentum, and the involved sites were the liver, gastrointestinal tract, bladder and bone. Three patients had multiple metastases at diagnosis. Two patients underwent upfront surgical excision of primary tumor, and the remaining 3 patients received neo-adjuvant chemotherapy after biopsy confirmation of the diagnosis. Combination chemotherapy was administered to all patients in addition to radiotherapy (median dose 45 Gy, range 17.5-54 Gy). Four patients showed disease progression or relapse, resulting in a 20 % overall survival rate. At the time of analysis, one patient is alive. She had localized disease at the time of diagnosis and was treated with upfront surgery, chemotherapy, and high-dose chemotherapy with autologous stem cell transplantation and radiotherapy.
Conclusion. Patients with DSRCT have a poor prognosis, even after multimodal treatment. Further studies are needed to determine the prognostic factors of DSRCT.
