RESUMO
Primary cardiac alveolar rhabdomyosarcoma (ARMS) is an extremely rare malignant tumor with dismal prognosis. We herein report a case of right atrial ARMS in a 63-year-old female with intermittent dizziness. Complete surgical resection of the mass was performed and the postoperative histopathologic examination confirmed ARMS. The patient received adjuvant therapy after surgery and was doing well at 1-year follow-up.
Assuntos
Neoplasias Cardíacas , Neoplasias do Mediastino , Rabdomiossarcoma Alveolar , Rabdomiossarcoma , Neoplasias do Timo , Feminino , Átrios do Coração/patologia , Átrios do Coração/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Humanos , Pessoa de Meia-Idade , Prognóstico , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/cirurgia , Rabdomiossarcoma Alveolar/diagnóstico , Rabdomiossarcoma Alveolar/patologia , Rabdomiossarcoma Alveolar/cirurgiaRESUMO
CASE: We describe a case of a 9-year-old boy who presented with a left calf mass consistent with alveolar rhabdomyosarcoma involving the Achilles tendon. The patient underwent radical resection of the Achilles tendon and Achilles tendon allograft reconstruction. At 2.5-year follow-up, the child had full ankle range of motion and strength and no signs of disease. CONCLUSIONS: Radical resection of Achilles tendon in the setting of malignancy and reconstruction with allograft is a rare procedure that has not been previously described in the pediatric population. Orthopaedic oncologists can consider this option for the rare malignancies involving the Achilles tendon.
Assuntos
Tendão do Calcâneo , Procedimentos de Cirurgia Plástica , Rabdomiossarcoma Alveolar , Tendão do Calcâneo/transplante , Criança , Humanos , Masculino , Amplitude de Movimento Articular , Procedimentos de Cirurgia Plástica/métodos , Rabdomiossarcoma Alveolar/cirurgia , Transplante HomólogoRESUMO
OBJECTIVES: Rhabdomyosarcoma (RMS) involving the spine is rare. The aim of the present study is to explore the clinicopathological features, surgical treatments and outcomes of this rare disease. PATIENTS AND METHODS: Eleven patients with spinal RMS who received surgery in our institution between 2012 and 2018 were retrospectively investigated. The literature on spinal RMS was also reviewed. RESULTS: Our study consisted of 7 cases of primary RMS and 4 cases of metastatic RMS. Seven primary and one metastatic spinal RMS received radical resection, the remaining three metastatic patients received palliative resection. Eight patients died with a median survival time of 8 months. The mean value of Ki-67 positivity was 48.2 %. Literature review revealed a total of 22 previously reported cases. 54.5 % of the pooled cases of the 33 patients were under the age of 18. Of the 20 patients with primary spinal RMS, 9 cases were diagnosed as embryonal, while 6 of the 13 metastatic patients were diagnosed as alveolar. Multiple modalities, including surgery and concurrent adjuvant therapy were performed in 19 patients. The median overall survival (OS) for 28 patients with detailed follow-up information was 10 months. Radical resection offered a significant longer median OS than non-radical resection (18 vs. 6 months, p = 0.027). CONCLUSION: Spinal RMS mainly affects young patients. The embryonal form and alveolar form is the most frequent subtype for primary and metastatic spinal RMS respectively. Spinal RMS is highly aggressive with dismal prognosis. Multimodality therapies are the mainstay of treatment. Radical resection is strongly recommended in eligible patients.
Assuntos
Procedimentos Neurocirúrgicos , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Embrionário/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Nádegas , Quimiorradioterapia Adjuvante , Quimioterapia Adjuvante , Pré-Escolar , Estudos de Coortes , Ciclofosfamida/uso terapêutico , Dactinomicina/uso terapêutico , Feminino , Humanos , Avaliação de Estado de Karnofsky , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Cuidados Paliativos , Neoplasias da Próstata/patologia , Neoplasias Retroperitoneais/patologia , Rabdomiossarcoma/diagnóstico por imagem , Rabdomiossarcoma/patologia , Rabdomiossarcoma/secundário , Rabdomiossarcoma/cirurgia , Rabdomiossarcoma Alveolar/diagnóstico por imagem , Rabdomiossarcoma Alveolar/patologia , Rabdomiossarcoma Alveolar/secundário , Rabdomiossarcoma Embrionário/diagnóstico por imagem , Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/secundário , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/secundário , Taxa de Sobrevida , Vincristina/uso terapêutico , Neoplasias Vulvares/patologia , Adulto JovemRESUMO
The authors present a rare case of anaplastic alveolar rhabdomyosarcoma (ARMS) occurring after Mandibular Distraction Osteogenesis. A 16-month-old male presented with a rapidly enlarging left lower jaw mass after removal of mandibular distractors placed shortly after birth for Pierre Robin Sequence and severe obstructive sleep apnea. Incisional biopsy of the mass revealed ARMS with anaplastic features. Although ARMS is an extremely rare entity, craniofacial surgeons should have a low threshold for seeking further diagnostic modalities of a growing mass postmandibular distraction.
Assuntos
Rabdomiossarcoma Alveolar/cirurgia , Biópsia , Humanos , Lactente , Masculino , Mandíbula/cirurgia , Osteogênese por Distração , Síndrome de Pierre Robin/complicações , Síndrome de Pierre Robin/cirurgia , Rabdomiossarcoma Alveolar/complicações , Rabdomiossarcoma Alveolar/diagnóstico por imagem , Apneia Obstrutiva do Sono/etiologia , Resultado do TratamentoRESUMO
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/etiologiaRESUMO
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 , Imageamento 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 TratamentoAssuntos
Neoplasias Duodenais/secundário , Neoplasias Pancreáticas/secundário , Rabdomiossarcoma Alveolar/patologia , Neoplasias Gástricas/secundário , Adolescente , Neoplasias Duodenais/cirurgia , Humanos , Masculino , Neoplasias Pancreáticas/cirurgia , Prognóstico , Rabdomiossarcoma Alveolar/cirurgia , Neoplasias Gástricas/cirurgiaRESUMO
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 JovemRESUMO
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 , Imageamento por Ressonância Magnética , Masculino , Rabdomiossarcoma Alveolar/diagnóstico por imagem , Rabdomiossarcoma Alveolar/cirurgia , Tomografia Computadorizada por Raios XRESUMO
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/cirurgiaRESUMO
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 TratamentoRESUMO
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 , Antracenos , 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 CultivadasRESUMO
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 TratamentoRESUMO
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 , Imageamento 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 VisualRESUMO
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 , Imageamento 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/químicaAssuntos
Rabdomiossarcoma Alveolar/patologia , Neoplasias Testiculares/patologia , Testículo/patologia , Adenocarcinoma/metabolismo , Adenocarcinoma/patologia , Adulto , Desmina/metabolismo , Diagnóstico Diferencial , Humanos , Neoplasias Pulmonares/secundário , Metástase Linfática , Masculino , Melanoma/metabolismo , Melanoma/patologia , Rabdomiossarcoma Alveolar/metabolismo , Rabdomiossarcoma Alveolar/cirurgia , Sarcoma Alveolar de Partes Moles/metabolismo , Sarcoma Alveolar de Partes Moles/patologia , Neoplasias Testiculares/metabolismo , Neoplasias Testiculares/cirurgia , Testículo/metabolismoRESUMO
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.