Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 56
Filtrar
Mais filtros










Intervalo de ano de publicação
1.
Pediatr Blood Cancer ; 66(11): e27952, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31397065

RESUMO

PURPOSE/OBJECTIVE(S): Bladder and prostate are unfavorable sites for rhabdomyosarcoma (B/P-RMS), and represent a challenging location for radiotherapy. MATERIALS/METHODS: Nineteen patients with B/P-RMS were enrolled on a prospective registry protocol (2008-2017) and treated with chemotherapy, proton beam therapy (PBT), and surgical resection (n = 8; 42%). Emphasis was given to treatment technique, disease-related outcomes, and toxicity associated with PBT. RESULTS: The majority of patients had bladder RMS (74%) of embryonal histology (95%), Group III (68%), and intermediate-risk disease by Children's Oncology Group (COG) risk stratification (89%). Seven patients (37%) had primary tumors >5 cm in size. All patients were treated according to COG protocols. With a median follow-up of 66.2 months, 5-year overall survival (OS) and progression-free survival (PFS) were 76%. Four patients (21%) experienced disease relapse, all presenting with local failure. The 5-year local control (LC) rate was 76%. Tumor size predicted LC, with 5-year LC for patients with >5 cm tumors being 43% versus 100% for those with ≤5 cm tumors (P = .006). Univariate analysis demonstrated an effect of tumor size on OS (tumor >5 cm, hazard ratio [HR] 17.7, P = .049) and PFS (HR 17.7, P = .049). Acute grade 2 toxicity was observed in two patients (11%, transient proctitis). Late grade 2+ toxicity was observed in three patients (16%; n = 1 grade 2 skeletal deformity; n = 3 transient grade 2 urinary incontinence; one patient experienced both). CONCLUSIONS: PBT for B/P-RMS affords promising disease-related outcomes with an acceptable toxicity profile. Higher local failure rates were observed for larger tumors, supporting dose-escalation components of ongoing RMS clinical trials.


Assuntos
Neoplasias da Próstata/radioterapia , Terapia com Prótons , Rabdomiossarcoma Embrionário/radioterapia , Neoplasias da Bexiga Urinária/radioterapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Pré-Escolar , Terapia Combinada , Cistectomia , Feminino , Seguimentos , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Estadiamento de Neoplasias , Proctite/etiologia , Intervalo Livre de Progressão , Modelos de Riscos Proporcionais , Estudos Prospectivos , Prostatectomia , Neoplasias da Próstata/tratamento farmacológico , Neoplasias da Próstata/patologia , Neoplasias da Próstata/cirurgia , Terapia com Prótons/efeitos adversos , Terapia com Prótons/métodos , Lesões por Radiação/etiologia , Sistema de Registros , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Alveolar/patologia , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Embrionário/tratamento farmacológico , Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/cirurgia , Risco , Carga Tumoral , Neoplasias da Bexiga Urinária/tratamento farmacológico , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/cirurgia , Incontinência Urinária/etiologia
2.
Int J Radiat Oncol Biol Phys ; 105(5): 1043-1054, 2019 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-31419513

RESUMO

PURPOSE: The standard of care of childhood parameningeal rhabdomyosarcoma (pRMS) is chemotherapy and local radiation therapy. Protons are increasingly being used to decrease late effects. The aim of the present study is to analyze the pattern of relapse and the correlation with dosimetric factors in pRMS treated with proton therapy. METHODS AND MATERIALS: This retrospective evaluation includes children treated in our institution for pRMS. Information on demographics, treatment, tumor characteristics, and toxicities and outcome was prospectively collected within the in-house registry. For patients presenting with local relapse, a fusion of the dosimetry with magnetic resonance imaging displaying site and geometry of recurrence was performed. RESULTS: Median follow-up time was 2.9 years (0.5-4.7). Forty-six patients were identified in our institution between July 2013 and November 2017. Main characteristics of patients were as follows: 56.5% male, median age 5.1 years (1.3-17.5), 39.1% alveolar histology, 26.1%, 52.2%, 8.7%, and 13% patients with subgroup risk classification D, E/F/G, H, or metastatic, respectively, median total prescribed dose 55.8 Gy (50.4-56.4). Estimated 2-year local control, metastasis-free survival, event-free survival, and overall survival were 83.8%, 87.8%, 76.9%, and 88.9%, respectively. No acute or late local toxicity exceeding grade 3 was observed. Risk-group was identified as prognostic factor for metastasis-free survival in univariate analysis but not in multivariate analysis (trend: P = .09). In this cohort, dosimetric factors did not correlate with outcome. Isolated local failure happened in 5 of the 11 relapses. Local relapses were matched with dosimetry for 6 patients: 4 of them occurred in the high dose volume and 2 in the intermediate or low dose volume. CONCLUSIONS: Proton therapy was effective and well feasible even in a critical cohort. Still, local relapse within the target volume of the radiation therapy remains an important issue in pRMS and new treatment strategies are needed.


Assuntos
Neoplasias Meníngeas/radioterapia , Recidiva Local de Neoplasia , Terapia com Prótons , Rabdomiossarcoma/radioterapia , Adolescente , Análise de Variância , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Intervalo Livre de Doença , Estudos de Viabilidade , Feminino , Seguimentos , Humanos , Lactente , Imagem por Ressonância Magnética/métodos , Masculino , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/mortalidade , Neoplasias Meníngeas/cirurgia , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/mortalidade , Prognóstico , Terapia com Prótons/efeitos adversos , Lesões por Radiação/patologia , Dosagem Radioterapêutica , Planejamento da Radioterapia Assistida por Computador , Estudos Retrospectivos , Rabdomiossarcoma/diagnóstico por imagem , Rabdomiossarcoma/mortalidade , Rabdomiossarcoma/cirurgia , Rabdomiossarcoma Alveolar/diagnóstico por imagem , Rabdomiossarcoma Alveolar/mortalidade , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Alveolar/cirurgia , Fatores de Tempo , Resultado do Tratamento
3.
Int J Radiat Oncol Biol Phys ; 103(5): 1151-1157, 2019 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-30508617

RESUMO

PURPOSE: Recent trends, including the use of proton therapy and administration of reduced doses of cyclophosphamide, have been adapted in head and neck (HN) rhabdomyosarcoma (RMS) to reduce late morbidity. Our primary goal was to analyze local control and survival outcomes after photon versus proton irradiation in pediatric patients with HN-RMS, with the secondary goal of analyzing the effect of cyclophosphamide dose on disease outcomes. METHODS AND MATERIALS: This single-institution cohort study comprised 76 pediatric HN-RMS patients treated with definitive chemoradiation from 2000 to 2018. Fifty-one patients (67%) received intensity modulated photon radiation therapy, and 25 (33%) received proton therapy. RESULTS: Local failure (LF) at 2 years was 12.5% for parameningeal RMS and 0% for orbital RMS and other head and neck sites (P = .24). Patients treated with protons were more likely to have received reduced-dose cyclophosphamide (P < .0001). The 2-year LF was 7.9% in the intensity modulated photon radiation therapy cohort versus 14.6% in the proton cohort (P = .07), with no difference in survival outcomes. Cumulative cyclophosphamide dose was significantly associated with 2-year LF: 0% for cumulative dose of >20 g/m2 versus 15.3% for ≤20 g/m2 (P = .04). In parameningeal RMS patients (n = 59), both cumulative cyclophosphamide dose and dose intensity were associated with LF (P = .01). There was a trend toward worse event-free survival for parameningeal RMS patients who received reduced-dose-intensity cyclophosphamide (59.2% vs 70.6%, P = .11). CONCLUSIONS: Both dose-intensity and cumulative cyclophosphamide dose seem to play an important role in achieving local control for HN-RMS patients treated with either protons or photons. Longer follow-up is needed to further assess disease outcomes with proton therapy.


Assuntos
Antineoplásicos Alquilantes/administração & dosagem , Ciclofosfamida/administração & dosagem , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/radioterapia , Terapia com Prótons/métodos , Radioterapia de Intensidade Modulada/métodos , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Embrionário/tratamento farmacológico , Rabdomiossarcoma Embrionário/radioterapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada/métodos , Feminino , Neoplasias de Cabeça e Pescoço/mortalidade , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Masculino , Recidiva Local de Neoplasia , Terapia com Prótons/efeitos adversos , Terapia com Prótons/estatística & dados numéricos , Dosagem Radioterapêutica , Radioterapia Adjuvante/métodos , Radioterapia de Intensidade Modulada/efeitos adversos , Radioterapia de Intensidade Modulada/estatística & dados numéricos , Eficiência Biológica Relativa , Rabdomiossarcoma Alveolar/mortalidade , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Embrionário/mortalidade , Rabdomiossarcoma Embrionário/cirurgia , Resultado do Tratamento , Adulto Jovem
4.
Rom J Morphol Embryol ; 59(4): 1247-1252, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30845308

RESUMO

Alveolar rhabdomyosarcoma (RMS) is a common pediatric malignant mesenchymal tumor, representing half of soft tissue sarcomas and approximately 5% of all cancers. We present the case of an adolescent male patient treated in our Department for a tumoral mass located in the middle third of the forearm. Magnetic resonance imaging (MRI) and angiography-computed tomography (angio-CT) showed a large mass located in the muscles of the anterior compartment of the forearm. Surgical treatment consisted of tumor ablation including segmental resection of the radial and ulnar arteries and of the median nerve, followed by saphenous autograft vascular bypass. The treatment plan was based on tumor type, histological grading (high), age, tumor size greater than 5 cm, unfavorable location, postoperative tumor, node, metastasis (TNM) stage II, presence of microscopic tumoral tissue in the margins of the resected piece, lymph node metastases (N1) and bone metastases (M1) found on positron-emission tomography (PET)-CT according to the German soft tissue sarcoma study (CWS)-IV 2002 protocol. The chemotherapy used Carboplatin and Topotecan. Survival was less than two years after the initial presentation. Adolescent extremity masses should raise suspicion to exclude serious malignancy. Despite early diagnosis and use of multimodal therapies, alveolar RMS prognostic remains unpredictable.


Assuntos
Rabdomiossarcoma Alveolar/patologia , Adolescente , Humanos , Imagem por Ressonância Magnética , Masculino , Rabdomiossarcoma Alveolar/diagnóstico por imagem , Rabdomiossarcoma Alveolar/cirurgia , Tomografia Computadorizada por Raios X
5.
Indian J Pathol Microbiol ; 58(4): 521-3, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26549082

RESUMO

Rhabdomyosarcoma (RMS) is a high-grade sarcoma that predominantly affects children, and rarely, the adult population. RMS demonstrates three major histologic variants: Embryonal, alveolar, and pleomorphic. A limited number of documented pure RMS cases of the gynecologic organs in adult women are found in the literature. Of these reports, the fallopian tube (FT) is reported as the primary site in only three cases, those included one of embryonal and two of the pleomorphic histologic variants. Herein, we report the first case of alveolar RMS arising in the FT of an adult woman and presenting as a unilateral adnexal mass.


Assuntos
Neoplasias das Tubas Uterinas/patologia , Rabdomiossarcoma Alveolar/patologia , Adulto , Neoplasias das Tubas Uterinas/cirurgia , Tubas Uterinas/patologia , Evolução Fatal , Feminino , Humanos , Rabdomiossarcoma Alveolar/cirurgia
6.
Int J Clin Exp Pathol ; 8(11): 15316-21, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26823887

RESUMO

Alveolar rhabdomyosarcoma (ARMS) is a common soft tissue tumor in children which can rarely metastasize to the breast in adults. Here we report the rare case of a 42-year-old Asian woman, who was diagnosed with ARMS of the nasopharynx and paranasal sinuses, and got a complete remission (CR) after surgery and chemoradiotherapy. Then the patient relapsed in the unilateral breast seventeen months later. Histology and immunohistochemistry of the primary sites and the breast lesions, combined with FISH, have been performed to confirm the diagnosis of metastatic alveolar rhabdomyosarcoma. With a rational therapeutic regimen of surgery, chemotherapy and radiotherapy, the patient has got a complete remission again.


Assuntos
Neoplasias da Mama/secundário , Neoplasias Nasofaríngeas/patologia , Neoplasias dos Seios Paranasais/patologia , Rabdomiossarcoma Alveolar/secundário , Adulto , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , Biópsia , Neoplasias da Mama/química , Neoplasias da Mama/genética , Neoplasias da Mama/cirurgia , Quimiorradioterapia Adjuvante , Feminino , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Mamografia , Neoplasias Nasofaríngeas/química , Neoplasias Nasofaríngeas/genética , Neoplasias Nasofaríngeas/cirurgia , Neoplasias dos Seios Paranasais/química , Neoplasias dos Seios Paranasais/genética , Neoplasias dos Seios Paranasais/cirurgia , Tomografia por Emissão de Pósitrons , Indução de Remissão , Reoperação , Rabdomiossarcoma Alveolar/química , Rabdomiossarcoma Alveolar/genética , Rabdomiossarcoma Alveolar/cirurgia , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento
7.
Surg Endosc ; 29(5): 1105-14, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25149634

RESUMO

BACKGROUND: Surgery for rhabdomyosarcoma is challenging due to a lack of clear delineation between tumor and surrounding tissue. Mutilating surgery can be necessary in difficult tumor localizations. Therefore, novel diagnostic and therapeutic modalities are required. The aim of this study was to evaluate the in vivo tumor detection of RMS using fluorescence laparoscopy and to analyze the efficacy of hypericin-induced photodynamic therapy in a mouse model. METHODS: Seventeen NOD/LtSz-scid IL2Rγnull-mice were divided into four groups. In group 1, mCherry-expressing tumor cells and in group 2-4 non-transfected tumor cells were xenotransplanted. Three weeks later, one fluorochrome per group (ICG, ICG-cetuximab, hypericin) was injected. Fluorescence laparoscopy was carried out and tumors were resected using fluorescence guidance. In the hypericin group, photodynamic therapy was performed using blue light and apoptosis was evaluated by TUNEL test. RESULTS: A clear discrimination between healthy and tumor tissue was feasible by fluorescending properties with mCherry expressing tumor cells and after injection of hypericin. No fluorescence was detected in mice injected with ICG and ICG-labeled cetuximab. Hypericin photodynamic therapy induced apoptosis of tumor cells after exposure to blue light. CONCLUSIONS: Intraoperative photodynamic diagnosis was feasible using mCherry-transfected tumor cells or hypericin. Additionally, intraoperative photodynamic therapy was possible and effective.


Assuntos
Laparoscopia/métodos , Neoplasias Experimentais , Fotoquimioterapia/métodos , Rabdomiossarcoma Alveolar/patologia , Neoplasias de Tecidos Moles/patologia , Animais , Apoptose , Fluorescência , Marcação In Situ das Extremidades Cortadas , Camundongos , Camundongos Endogâmicos NOD , Perileno/análogos & derivados , Perileno/uso terapêutico , Proteína Quinase C/antagonistas & inibidores , Radiossensibilizantes/uso terapêutico , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Alveolar/cirurgia , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/cirurgia , Células Tumorais Cultivadas
8.
Magy Onkol ; 58(1): 59-64, 2014 Mar.
Artigo em Húngaro | MEDLINE | ID: mdl-24712008

RESUMO

Malignant tumors of mesenchymal origin are called sarcomas. Mesenchymal cells normally mature into skeletal muscle, smooth muscle, fat, fibrous tissue, bone and cartilage. Rhabdomyosarcoma (RMS) arises from immature mesenchymal cells that are committed to skeletal muscle lineage. However, it can also arise in tissues in which striated muscle is normally not found (such as the urinary tract). Undifferentiated sarcomas cannot be ascribed to any specific lineage. Treatment results improved significantly in the last decade by combined treatment (chemotherapy, surgery, irradiation, in some cases targeted therapy). Good treatment results can be achieved in pediatric oncology centers by early diagnosis and adequate treatment according to international treatment protocols.


Assuntos
Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/cirurgia , Criança , Terapia Combinada , Humanos , Estadiamento de Neoplasias , Rabdomiossarcoma/tratamento farmacológico , Rabdomiossarcoma/radioterapia , Rabdomiossarcoma Alveolar/diagnóstico , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Embrionário/diagnóstico , Rabdomiossarcoma Embrionário/cirurgia , Sarcoma/diagnóstico , Sarcoma/cirurgia , Resultado do Tratamento
9.
Ophthalmic Plast Reconstr Surg ; 30(1): e20-2, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-23552609

RESUMO

Orbital cavitary rhabdomyosarcoma has been previously reported in 2 cases. The case presented here was a 15-year-old boy who had proptosis, pain, periorbital hyperemia, and visual loss in his OD, which progressed in 6 weeks. Radiologic studies demonstrated a well-demarcated, multilobulated, large mass with cavities, extending from the anterior orbit to the apex, suggesting a diagnosis of venolymphatic malformation with an intrinsic hemorrhage. On surgery, a multilobular, hemorrhagic cystic mass was almost completely excised. Histologic examination revealed a diagnosis of embryonal rhabdomyosarcoma. After postoperative chemotherapy and radiotherapy, the tumor did not recur during a follow-up period of 45 months. This case, together with the previous ones, suggests that cavitary orbital rhabdomyosarcomas may have some distinct clinical, radiologic, and surgical characteristics.


Assuntos
Neoplasias Orbitárias/patologia , Rabdomiossarcoma Alveolar/patologia , Adolescente , Humanos , Imagem por Ressonância Magnética , Masculino , Músculos Oculomotores/patologia , Neoplasias Orbitárias/radioterapia , Neoplasias Orbitárias/cirurgia , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Alveolar/cirurgia , Tomografia Computadorizada por Raios X , Acuidade Visual
11.
J Prosthet Dent ; 107(4): 227-31, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22475465

RESUMO

It is challenging to treat and irradiate empty cavities with external-beam radiation therapy (EBRT) because body contour irregularities can result in dose heterogeneities. The use of compensator materials to fill the empty cavities can provide a more homogeneous radiation dose distribution. The purposes of this clinical report are to describe the use of 3 different materials (elastomeric material, water-filled balloon, and acrylic resin) in an orbital defect and compare the dosimetric parameters and photon-electron dose distribution during EBRT.


Assuntos
Materiais Biocompatíveis/química , Órbita/efeitos da radiação , Próteses e Implantes , Dosagem Radioterapêutica , Resinas Acrílicas/química , Cateterismo/instrumentação , Pré-Escolar , Elastômeros/química , Feminino , Humanos , Imagem Tridimensional/métodos , Terapia Neoadjuvante , Órbita/cirurgia , Neoplasias Orbitárias/radioterapia , Neoplasias Orbitárias/cirurgia , Polivinil/química , Desenho de Prótese , Lesões por Radiação/prevenção & controle , Proteção Radiológica/instrumentação , Planejamento da Radioterapia Assistida por Computador , Radioterapia Assistida por Computador , Radioterapia de Alta Energia/instrumentação , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Alveolar/cirurgia , Siloxanas/química , Água/química
13.
Zhonghua Zhong Liu Za Zhi ; 34(12): 910-6, 2012 Dec.
Artigo em Chinês | MEDLINE | ID: mdl-23336377

RESUMO

OBJECTIVE: Albeit rare, rhabdomyosarcoma (RMS) may occur in middle to old-aged adults. The purpose of this study was to investigate the clinicopathological characteristics and prognosis of RMS in patients ≥ 40 years of age. METHODS: The clinical, pathological and immunohistochemical profiles of the 76 cases were reviewed, and the outcomes were analyzed. RESULTS: There were 46 males and 30 females with a median age of 55 years. Sixty cases occurred in somatic soft tissues, including extremities (n = 25), head and neck (n = 19) and trunk (n = 16). Sixteen developed in the internal organs with predilection for the urogenital tract. Histologically, 38 cases (50.0%) were of embryonal RMS (ERMS) subtype, 29 cases (38.2%) of pleomorphic RMS (PRMS), 7 cases (9.2%) of poorly-differentiated RMS (PdRMS), and 2 cases (2.6%) of alveolar RMS (ARMS). Immunohistiochemically, the tumor cells showed diffuse staining of desmin (68/68, 100%), with a variable expression of myogenin (30/43, 69.8%), MyoD1 (33/51, 64.7%) and MSA (29/40, 72.5%). The therapy data available in 54 cases showed that three inoperable cases were treated with chemotherapy and radiotherapy, 51 cases underwent local excision, including 10 cases with chemotherapy, 9 cases with radiotherapy, 8 cases with chemotherapy and radiotherapy after surgery, and the remaining 24 cases were not administrered with postoperative adjuvant therapy. The follow-up data available in 54 cases showed that 22 patients were alive with unresectable or recurrent/disease and 32 patients were alive with no evidence of disease. The median disease-free and overall survivals were 6.0 months and 7.0 months, respectively. Twenty-seven patients exhibited progression of disease with recurrence in 8 cases and metastasis in 19 cases. The median interval to progression was 6.0 months. CONCLUSIONS: The most common subtypes of RMS in middle to old aged patients are ERMS and PRMS. In general, Rhabdomyosarcomas of middle to old aged patients shows a highly aggressive behavior. Patients may benefit from multi-disciplinary therapy.


Assuntos
Extremidades , Rabdomiossarcoma , Neoplasias de Tecidos Moles , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimioterapia Adjuvante , Desmina/metabolismo , Intervalo Livre de Doença , Feminino , Seguimentos , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/metabolismo , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/radioterapia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Proteína MyoD/metabolismo , Miogenina/metabolismo , Recidiva Local de Neoplasia , Radioterapia Adjuvante , Rabdomiossarcoma/tratamento farmacológico , Rabdomiossarcoma/metabolismo , Rabdomiossarcoma/patologia , Rabdomiossarcoma/radioterapia , Rabdomiossarcoma/cirurgia , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Alveolar/metabolismo , Rabdomiossarcoma Alveolar/patologia , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Embrionário/tratamento farmacológico , Rabdomiossarcoma Embrionário/metabolismo , Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/radioterapia , Rabdomiossarcoma Embrionário/cirurgia , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/metabolismo , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/cirurgia , Taxa de Sobrevida
14.
Pathol Int ; 61(6): 377-81, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21615615

RESUMO

Herein is presented a very rare case of alveolar rhabdomyosarcoma in the uterine corpus of a 72-year-old woman. The wall of the uterine corpus was replaced by multiple whitish-yellow, friable nodules, measuring up to 6 cm. Microscopically, the tumor was predominantly composed of round to polygonal cells arranged in an alveolar, papillary or nest pattern intermingled with multinuclear giant cells with abundant eosinophilic cytoplasm. Extensive sampling failed to show epithelial elements. Immunohistochemically, the tumor was positive for striated muscle markers such as myoglobin, myoD1 and myogenin. Metastatic lesions were found in the retroperitoneum and pelvic lymph nodes. The patient was treated by postoperative chemotherapy, but she died of systemic metastases 12 months after surgery.


Assuntos
Rabdomiossarcoma Alveolar/patologia , Neoplasias Uterinas/patologia , Idoso , Biópsia , Quimioterapia Adjuvante , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Histerectomia , Imuno-Histoquímica , Imagem por Ressonância Magnética , Rabdomiossarcoma Alveolar/secundário , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Alveolar/ultraestrutura , Tomografia Computadorizada por Raios X , Neoplasias Uterinas/cirurgia , Neoplasias Uterinas/ultraestrutura , Útero/patologia , Útero/cirurgia
15.
J Surg Res ; 170(2): e243-51, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21529833

RESUMO

OBJECTIVE: To examine incidence and outcomes for pediatric rhabdomyosarcoma (RMS). METHODS: The SEER registry was examined for patients with RMS < 20 y old. RESULTS: Overall, 1544 patients were identified for an incidence of 0.4414/100,000 per year. Males outnumbered females 3:2. Tumors were classified as embryonal (67%), alveolar (32%), and pleomorphic (1%). Alveolar and pleomorphic RMS were more common in adolescents, whereas embryonal type was more common in younger children (P = 0.0001). Pleomorphic (47%) and alveolar (39%) RMS commonly presented with distant disease, in contrast to embryonal (25%). Most patients had surgical resection (81%) and radiotherapy (63%). Overall, 5- and 10-y survival was 60% and 57%, respectively. Univariate analysis identified higher survival for age < 10 y, local stage, favorable site, embryonal type, <5 cm tumor size, and surgical resection. Multivariate analysis identified non-embryonal type (HR 1.451), non-favorable site (HR 1.570), no surgery (HR 1.726), age ≥ 10 y (HR 1.734), 1973-1978 diagnosis year (HR 1.730), and distant disease (HR 3.456) as independent predictors of mortality. CONCLUSIONS: Embryonal histology, the most common type of pediatric RMS, presents in young children and has better prognosis than alveolar or pleomorphic types. Patients with embryonal tumors, favorable tumor location, age < 10 y, localized disease, and surgical resection have improved survival.


Assuntos
Rabdomiossarcoma Alveolar/mortalidade , Rabdomiossarcoma Embrionário/mortalidade , Programa de SEER/estatística & dados numéricos , Neoplasias de Tecidos Moles/mortalidade , Adolescente , Distribuição por Idade , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Estimativa de Kaplan-Meier , Masculino , Análise Multivariada , Valor Preditivo dos Testes , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Alveolar/terapia , Rabdomiossarcoma Embrionário/cirurgia , Rabdomiossarcoma Embrionário/terapia , Distribuição por Sexo , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/terapia , Adulto Jovem
16.
Int J Radiat Oncol Biol Phys ; 80(2): 333-8, 2011 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-20646841

RESUMO

PURPOSE: Postoperative radiation therapy (RT) is recommended for patients with rhabdomyosarcoma having microscopic disease. Sometimes RT dose/volume is reduced or omitted in an attempt to avoid late effects, particularly in young children. We reviewed operative bed recurrences to determine if noncompliance with RT protocol guidelines influenced local-regional control. METHODS AND MATERIALS: All operative bed recurrences among 695 Group II rhabdomyosarcoma patients in Intergroup Rhabdomyosarcoma Study Group (IRS) I through IV were reviewed for deviation from RT protocol. Major/minor dose deviation was defined as >10% or 6-10% of the prescribed dose (40-60 Gy), respectively. Major/minor volume deviation was defined as tumor excluded from the RT field or treatment volume not covered by the specified margin (preoperative tumor volume and 2- to 5-cm margin), respectively. No RT was a major deviation. RESULTS: Forty-six of 83 (55%) patients with operative bed recurrences did not receive the intended RT (39 major and 7 minor deviations). RT omission was the most frequent RT protocol deviation (19/46, 41%), followed by dose (17/46, 37%), volume (9/46, 20%), and dose and volume deviation (1/46, 2%). Only 7 operative bed recurrences occurred in IRS IV (5% local-regional failure) with only 3 RT protocol deviations. Sixty-three (76%) patients with recurrence died of disease despite retrieval therapy, including 13 of 19 nonirradiated children. CONCLUSION: Over half of the operative bed recurrences were associated with noncompliance; omission of RT was the most common protocol deviation. Three fourths of children die when local-regional disease is not controlled, emphasizing the importance of RT in Group II rhabdomyosarcoma.


Assuntos
Fidelidade a Diretrizes , Recidiva Local de Neoplasia/radioterapia , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Embrionário/radioterapia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Protocolos Clínicos , Humanos , Lactente , Recidiva Local de Neoplasia/mortalidade , Neoplasia Residual , Dosagem Radioterapêutica , Rabdomiossarcoma Alveolar/mortalidade , Rabdomiossarcoma Alveolar/patologia , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Embrionário/mortalidade , Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/cirurgia , Carga Tumoral
17.
J Pediatr Hematol Oncol ; 33(1): e26-8, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21102351

RESUMO

A 7-year-old girl was diagnosed with alveolar rhabdomyosarcomas of the right crural region. The patient was initially treated with chemotherapy and surgery with wide surgical margins, with radiotherapy. The tumors relapsed in the popliteal region surrounding the popliteal vessels. The patient received sentinel lymph node biopsy for a recurrent tumor using isosulfan blue dye, with the "sentinel node" being malignant-negative, and a "nonsentinel node" positive. Sentinel lymph node biopsy with conventional blue dye alone might be insufficient to assess lymph node status in patients with recurrent rhabdomyosarcoma previously treated with surgery and/or radiotherapy.


Assuntos
Linfonodos/patologia , Linfonodos/fisiopatologia , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Alveolar/cirurgia , Biópsia de Linfonodo Sentinela/métodos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Feminino , Humanos , Recidiva , Rabdomiossarcoma Alveolar/diagnóstico
18.
Acta Gastroenterol Belg ; 74(4): 576-81, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22319971

RESUMO

Rhabdomyosarcomas are malignant tumors that display features of striated muscle differentiation. They are the most common soft-tissue sarcomas among children and young adults. In mature adults however there are very rare. The liver as a primary site in adults has only been described in 12 cases. We report a case of a primary alveolar rhabdomyosarcoma of the liver in a 59 year old female, confirmed by histological examination using immunohistochemical analysis (positive actin, desmin, vimentin and myogenin staining) and fluorescent in situ hybridization (FISH) analysis (positivity for PAX3/FOXO1A fusion). The patient underwent primary surgical resection, but presented a few weeks after surgery already with recurrent disease in the abdomen and bone metastasis. Despite initial good response to chemotherapy (doxorubicin/ifosfamide) and stable disease at 12 months after diagnosis, the patient died 31 months after the first presentation secondary to complicated abundant abdominal recurrent disease. We further present a review of the literature on published similar cases since 1979.


Assuntos
Neoplasias Hepáticas/patologia , Rabdomiossarcoma Alveolar/patologia , Evolução Fatal , Feminino , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/metabolismo , Neoplasias Hepáticas/cirurgia , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Alveolar/metabolismo , Rabdomiossarcoma Alveolar/cirurgia
19.
Nepal J Ophthalmol ; 2(1): 64-7, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-21141330

RESUMO

INTRODUCTION: Rhabdomyosarcoma is the most common primary orbital malignant tumor in children. Orbital lesions represent about 10 % of all the cases of rhabdomyosarcoma. Rhabdomyosarcoma is a rare cause of proptosis in adults. OBJECTIVE: To report a case of primary orbital rhabdomyosarcoma in a 45-year-old female. DESIGN: Interventional case report. The main outcome measures are a rare cause ofproptosis in an adult, discussion on treatment options and prognosis ofrhabdomyosarcoma. RESULT: The patient underwent total orbital exenteration and was referred for radiotherapy and chemotherapy. CONCLUSION: Rhabdomyosarcoma is a rare cause of proptosis in adults. It should be suspected in a case of rapidly-progressive proptosis in adults.


Assuntos
Exenteração Orbitária/métodos , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Neoplasias Orbitárias/diagnóstico , Neoplasias Orbitárias/cirurgia , Rabdomiossarcoma Alveolar/diagnóstico , Rabdomiossarcoma Alveolar/cirurgia , Tomografia Computadorizada por Raios X
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA