RESUMO
BACKGROUND: The optimal approach to patients with gastric lymphoma of extranodal mucosa-associated lymphoid tissue (MALT) that resist to anti-Helicobacter pylori (HP) eradication therapy is still to be defined. PATIENTS AND METHODS: From January 1997 to December 2004, we observed 24 patients affected with newly diagnosed early-stage and HP-positive gastric lymphoma of the MALT type. Five of them resisted to oral anti-HP antibiotic regimens and to subsequent one (two patients) or two (three patients) chemotherapy regimens. Age ranged between 51 and 77 years (median 70); three were females. Translocation (11;18) was ascertained in one subject. They were admitted to local radiation therapy with a total dose of 30 Gy. RESULTS: All such resistant patients achieved complete remission after radiotherapy. No relapses were observed after 21, 45, 48, 52, and 67 months of uninterrupted follow-up. Early toxicity was very low and consisted of mild nausea. Late toxicity or secondary malignancy was not recorded so far. CONCLUSIONS: Radiotherapy proved to be effective and safe for early-stage HP-positive gastric extranodal lymphoma of MALT type that is resistant to anti-HP eradication antibiotics and to following chemotherapy. Radiotherapy might be suggested as principal salvage therapy after resistance to HP eradication, instead of chemotherapy.
Assuntos
Antibacterianos/uso terapêutico , Antineoplásicos/uso terapêutico , Helicobacter pylori/efeitos dos fármacos , Linfoma de Zona Marginal Tipo Células B/radioterapia , Neoplasias Gástricas/radioterapia , Idoso , Terapia Combinada , Resistencia a Medicamentos Antineoplásicos , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Indução de Remissão , Neoplasias Gástricas/tratamento farmacológicoRESUMO
AIMS AND BACKGROUND: The role of radiotherapy in the treatment of stage III non-small cell lung cancer is controversial. The aim of this survey was to investigate the use of this modality in current clinical practice in Lombardy, a highly industrialized region of northern Italy. METHODS: A questionnaire was sent to all 13 radiotherapy centers in Lombardy, covering statistical, clinical, technical and strategical aspects, and the responses were analyzed. RESULTS: A wide range of attitudes was observed among participating radiation oncologists; the percentage of cases treated with curative intent varied largely between centers (4-100%), as did the proportion of patients given to radiation only rather than combined modality treatment (5-100% vs 0-90%). CONCLUSIONS: An urgent need exists for better cooperation between all clinicians involved in lung cancer treatment, pursuing the goals of a more uniform clinical practice and a more aggressive clinical research attitude.
Assuntos
Carcinoma Pulmonar de Células não Pequenas/radioterapia , Neoplasias Pulmonares/radioterapia , Carcinoma Pulmonar de Células não Pequenas/patologia , Humanos , Itália , Neoplasias Pulmonares/patologia , Estadiamento de Neoplasias , Estudos Retrospectivos , Inquéritos e Questionários , Resultado do TratamentoRESUMO
PURPOSE: With the aim of establishing clinical and technical criteria to homogenize radiotherapy practice, a working group of AIRO-Lombardia (Associazione Italiana di Radioterapia Oncologica--Gruppo regionale della Lombardia) has tried to define minimal requirements for radical and postoperative irradiation in prostate cancer. The document has been structured in such a way as to be also of interest to the urological and medical oncology communities. METHODS: The working group, composed of representatives of most of the regional radiotherapy departments in the Lombardy region, had monthly meetings during 1996 and 1997. The document on minimal requirements has been derived from the participants' combined experience and knowledge, from review of the literature, and from a 1995 regional survey on current practice of prostate irradiation. RESULTS: Minimal requirements for radical and postoperative irradiation of prostate cancer have been defined with respect to treatment strategies, pre-treatment diagnostic evaluation and staging, treatment prescription, preparation and execution, and quality assurance procedures. CONCLUSION: Standards of reference for minimal requirements in prostate cancer irradiation adapted to the regional structures and resources have been defined.
Assuntos
Neoplasias da Próstata/radioterapia , Humanos , Itália , Masculino , Estadiamento de Neoplasias , Neoplasias da Próstata/patologia , Radioterapia/normas , Dosagem RadioterapêuticaRESUMO
AIMS AND BACKGROUND: We report the results of a survey performed in 1994 by the AIRO-Lombardia Cooperative Group, on the clinical patterns of radiation treatment for prostatic carcinoma in Lombardy, Italy, involving all radiotherapy centers serving an overall local population of about 8,800,000 people. METHODS: A questionnaire was sent to all 13 radiotherapy centers throughout Lombardy, asking for demographic and treatment details concerning the local population of patients with a localized (T1-4, N0-1, M0) carcinoma of the prostate treated with radiotherapy; 12 centers responded, making the basis for the present report. RESULTS: Analysis of collected data showed that in Lombardy: a) approximately 400 patients per year are irradiated for a localized carcinoma of the prostate, accounting for less than 30% of the total expected number of patients with this disease presentation; b) a complete staging (with PSA, transrectal ultrasonography, abdomino-pelvic CT or MRI scan and total-body bone scan) is performed in over 95% of patients before initiating radiotherapy; c) significant differences exist between radiotherapy centers as regards treatment planning and delivery. CONCLUSIONS: An urgent need exists for implementing procedures aimed at standardizing radiotherapy procedures within Lombardy.
Assuntos
Carcinoma/radioterapia , Neoplasias da Próstata/radioterapia , Carcinoma/diagnóstico , Humanos , Itália , Masculino , Estadiamento de Neoplasias , Neoplasias da Próstata/diagnóstico , Dosagem Radioterapêutica , Inquéritos e Questionários , Resultado do TratamentoRESUMO
Thirty-two long-term survivors after treatment with surgery, radiotherapy and chemotherapy for neuroblastoma (NRB) and Wilms' tumour (WT) were re-evaluated after a period of more than 10 years (mean 16 yr 1 mo, range 27 yr 2 mo/10 yr 5 mo). In addition to routine clinical and laboratory studies, all patients underwent auxometry, echocardiography, spinal X-ray, abdominal US and kidney radionuclide scans. Neither obvious physical signs nor cutaneous toxicity were noted at inspection. No dysmenorrhoea or sexual disorders were mentioned. Haematological data turned out to be all in the normal range, except for TIBC/UIBC ratio (out of range in 70 % of cases); gammaGt (62.5 %); A/G ratio (12.5 %); ALT (37.5 %). Auxometrical data were all in the normal range; in particular, results of mean weight and height were just slightly over the 50 degrees centile. Spinal X-rays revealed mild kyphotic deformities (68.9 %), pelvic obliquity and tilting (62.5 %), rotation of the vertebral bodies (34.3 %) and compensatory curves of the spine (21.8 %). No impairment of cardiac functions was noted at echocardiography. No alteration of the hepatic structure was detectable at US scans. Marked impairment of the renal function (20 % and 1.5 % residual function) was detected at radionuclide imaging in 2 NRB patients who had not undergone nephrectomy, with the kidney having almost disappeared in one. In conclusion, the study emphasises the need for careful and prolonged follow-up for the detection of sequelae than can appear even many years after the initial treatment, and the wisdom and the need for a continued search for less aggressive protocols; as long as the cure rate is not compromised, less aggressive protocols should aim for a satisfactory overall survival with a reduced incidence of sequelae and hence a better quality of life.
Assuntos
Neuroblastoma/terapia , Neoplasias Retroperitoneais/terapia , Procedimentos Cirúrgicos Operatórios/métodos , Tumor de Wilms/terapia , Adolescente , Criança , Pré-Escolar , Terapia Combinada/métodos , Feminino , Seguimentos , Humanos , Lactente , Masculino , Nefrectomia/métodos , Estudos Retrospectivos , Fatores de TempoRESUMO
Advances in vascular radiology techniques for superselective transfemoral arterial infusion prompted us to evaluate the effects of high-dose rapid regional carboplatin infusion for patients with advanced head and neck squamous cell carcinomas. Twenty untreated patients received three infusions of carboplatin (300-350 mg/m2) every 2 weeks with this method. All the infusions were performed without any complication. Treatment was well tolerated, with moderate (Grade 1-3 WHO) local toxicity (stomatitis, dermatitis and alopecia) and minimal (Grade 1-2 WHO) myelosuppression. The total response index (complete response plus partial response) was 94% for primary tumors and 50% for neck metastases. Neoadjuvant chemotherapy employing superselective rapid infusion of high-dose carboplatin is a feasible, relatively nontoxic, effective technique and may have important applications in multimodality therapy of untreated patients with advanced head and neck cancer.
Assuntos
Antineoplásicos/uso terapêutico , Carboplatina/uso terapêutico , Quimioterapia Adjuvante , Neoplasias Bucais/tratamento farmacológico , Boca/patologia , Neoplasias Nasofaríngeas/tratamento farmacológico , Nasofaringe/patologia , Neoplasias Orofaríngeas/tratamento farmacológico , Orofaringe/patologia , Adulto , Idoso , Antineoplásicos/administração & dosagem , Carboplatina/administração & dosagem , Relação Dose-Resposta a Droga , Feminino , Humanos , Injeções Intravenosas , Masculino , Pessoa de Meia-Idade , Neoplasias Bucais/patologia , Neoplasias Nasofaríngeas/patologia , Neoplasias Orofaríngeas/patologia , Estudos RetrospectivosRESUMO
The results of larynx carcinoma radiotherapy with electron beams are reported: 67 cases of glottic carcinoma (vocal cords) Tis, T1a, T1b, T2, treated between 1965 and 1978 are studied. Survival cumulative and not evidence disease rates are reported 84.7% at three years, 80.2% at five years, 77.1% at ten years, 77.1% at fourteen years. To conclude we underline the importance of radiotherapy in the treatment of carcinoma of the vocal cords.
Assuntos
Neoplasias Laríngeas/radioterapia , Prega Vocal/efeitos da radiação , Adulto , Idoso , Elétrons , Feminino , Humanos , Neoplasias Laríngeas/mortalidade , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Dosagem Radioterapêutica , Radioterapia de Alta EnergiaRESUMO
Infantile hepatic hemangioendothelioma is a rare tumor of infancy, sometimes associated with cutaneous hemangiomatosis. It is clinically evident within the first six months after birth and can be life threatening because of heart failure, intraperitoneal hemorrhage or thrombocytopenia. In less severe forms spontaneous regression has been described. Current treatment may be surgical ligation of the hepatic artery, or pharmacological therapy with corticosteroids or radiotherapy. A 4-month infant is described, admitted with acute heart failure and huge hepatomegaly. Since a surgical approach was not possible and corticosteroid therapy failed to achieve the expected effect, radiotherapy was given with excellent results.
Assuntos
Hemangioendotelioma/radioterapia , Neoplasias Hepáticas/radioterapia , Hemangioendotelioma/patologia , Humanos , Lactente , Neoplasias Hepáticas/patologia , MasculinoRESUMO
A case of malignant small cell tumor of the thoracopulmonary region in a 3-year-old boy is presented. The rarity of this tumor in children justifies the presentation of a new case. Differential diagnosis with other childhood neoplasms is discussed.
Assuntos
Neoplasias Pulmonares , Neoplasias Torácicas , Biópsia , Pré-Escolar , Diagnóstico Diferencial , Humanos , Imunossupressores/uso terapêutico , Pulmão/diagnóstico por imagem , Pulmão/ultraestrutura , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/ultraestrutura , Masculino , Prognóstico , Radiografia , Rabdomiossarcoma/diagnóstico , Sarcoma de Ewing/diagnóstico , Neoplasias Torácicas/diagnóstico , Neoplasias Torácicas/tratamento farmacológico , Neoplasias Torácicas/cirurgia , Neoplasias Torácicas/ultraestruturaRESUMO
Thyroid volume reduction was observed, among 25 subjects off-therapy after Hodgkin's disease. The volume reduction was related to dose (p = 0.014) and time from radiotherapy (p = 0.01). The correlation was very specific since all patients with reduced volume had hypothyroidism, but not very sensitive since 25% of subjects with thyroid dysfunction had normal gland volume.
Assuntos
Doença de Hodgkin/radioterapia , Lesões por Radiação/patologia , Radioterapia/efeitos adversos , Glândula Tireoide/patologia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Atrofia , Criança , Pré-Escolar , Terapia Combinada , Feminino , Doença de Hodgkin/tratamento farmacológico , Humanos , Hipotireoidismo/etiologia , Lactente , Masculino , Pescoço/efeitos da radiação , Testes de Função Tireóidea , Glândula Tireoide/efeitos da radiaçãoRESUMO
BACKGROUND: Pulmonary sequelae have been reported in patients treated for Hodgkin's disease (HD). Few data are available about patients treated for childhood HD followed over several years. PATIENTS AND METHODS: In a cross-sectional study carried out for 76 months (median time) after treatment completion, we evaluated the lung function abnormalities and respiratory symptoms in 27 patients (16 males and 11 females) with HD diagnosed between 1983 and 1994 (median age at diagnosis 11 years, range 2-16 years). They had been treated with chemotherapy and radiotherapy according to current protocol AIEOP-MH 83 (n = 14) or AIEOP-MH 89 (n = 13). At the time of the study, 26 patients were in first complete remission and one in second remission. Of the 27 patients, 19 had had mediastinal irradiation at a dose of 20 Gy (n = 5) or 20.8-44 Gy (n = 14). Forced vital capacity (FVC), functional residual capacity (FRC), forced expiratory volume in one second (FEV1), FEV1/FVC ratio, and maximal expiratory flow at 25% of FVC were registered; diffusion capacity for carbon monoxide (DLCO) was determined. Data were expressed as standard deviation (SD) score. Four patterns of respiratory function abnormalities were defined: restrictive, obstructive, isolated bronchiolar impairment, isolated diffusing impairment. RESULTS: Twelve patients (44%) were asymptomatic and showed completely normal pulmonary function tests. Three patients reported dyspnea on exertion, and one of them also cough and phelgm: out of these symptomatic subjects, only 1 had functional abnormality (isolated DLCO impairment). A restrictive pattern was found in 5 patients (18%), including 2 who also had a pathological DLCO SD score. Eight additional patients (30%) had isolated diffusing impairment. Oxygen saturation was normal in all patients. Forty-seven percent of patients with normal DLCO had had lower dose irradiation (20 Gy) compared to 10% of patients with impaired DLCO (P = 0.054). Similarly, patients with normal DLCO had had significantly less chemotherapy as compared to patients with abnormal DLCO (P = 0.003). Occurrence of lung abnormalities was not significantly associated with sex, age at treatment, mediastinal irradiation, and time elapsed from treatment completion. CONCLUSION: Adolescents and young adults treated for childhood HD are at risk for lung function abnormalities, significantly more frequent in patients who received more intense treatment, as mediastinal irradiation at a higher dose (> 20 Gy) and more chemotherapy blocks. Long-term follow-up should be offered to these patients because of their possible limited potential for pulmonary function and possible lesser resistance to adverse agents such as smoke, pollution, infections and aging.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/radioterapia , Pneumopatias/etiologia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Bleomicina/administração & dosagem , Bleomicina/efeitos adversos , Criança , Pré-Escolar , Terapia Combinada , Estudos Transversais , Dacarbazina/administração & dosagem , Dacarbazina/efeitos adversos , Doxorrubicina/administração & dosagem , Doxorrubicina/efeitos adversos , Feminino , Seguimentos , Doença de Hodgkin/complicações , Humanos , Pulmão/fisiologia , Pneumopatias/induzido quimicamente , Masculino , Mecloretamina/administração & dosagem , Mecloretamina/efeitos adversos , Prednisona/administração & dosagem , Prednisona/efeitos adversos , Procarbazina/administração & dosagem , Procarbazina/efeitos adversos , Radioterapia/efeitos adversos , Testes de Função Respiratória , Vimblastina/administração & dosagem , Vimblastina/efeitos adversos , Vincristina/administração & dosagem , Vincristina/efeitos adversosRESUMO
Choroid plexus carcinoma (CPC) is a rare tumor with usually severe prognosis, whose optimal treatment has not yet been established. The exact role of complete surgical resection, chemotherapy, and radiotherapy has been debated but not clarified. We report one girl with CPC diagnosed at age 3 months and apparently cured with minimal surgical resection, chemotherapy, and delayed irradiation. At the age of 8 years, she is well, with minor psychomotor retardation and growth hormone deficiency as the only sequelae.
Assuntos
Carcinoma Papilar/terapia , Neoplasias do Plexo Corióideo/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma Papilar/diagnóstico , Carcinoma Papilar/patologia , Neoplasias do Plexo Corióideo/diagnóstico , Neoplasias do Plexo Corióideo/patologia , Terapia Combinada , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Prognóstico , Tomografia Computadorizada por Raios XRESUMO
PURPOSE: We addressed the issue of the relationship between the general practitioner (GP) and the radiotherapist to improve the quality of care of cancer patients. MATERIAL AND METHODS: The study consisted in evaluating medical requests and phone interviews, with a questionnaire with yes/no and multiple choice answers to the following 5 questions: 1) Do you think a cancer diagnosis is always a hopeless death sentence? 2) Is it professionally rewarding to cure a cancer patient? 3) Are you satisfied with your relationship, as a general practitioner, with oncologic reference centers? 4) Is it more wearing for a general practitioner to manage a cancer than a noncancer patient? 5) Would you answer a questionnaire about the relationship between the general practitioner, the cancer patient and the oncologist? We evaluated 1590 medical requests and made 401 phone interviews; 255 colleagues (70%) answered the questionnaire. RESULTS: Medical requests were correctly and completely formulated by GPs in 45% of cases. A cancer diagnosis was not considered a hopeless death sentence in 90.9% of cases and 76% of GPs considered it professionally rewarding to cure a cancer patient. 75.6% of GPs considered it more wearing to manage a cancer than a noncancer patient, and female GPs felt this more strongly than their male counterparts. Irrespective of gender, GPs over 50 years of age tend to consider cancer a hopeless and fatal disease. The relationship with oncologic centers was considered satisfactory in 86.2% of cases. However, since cancer patients need greater medical care, GPs would like a closer cooperation with oncologists. DISCUSSION AND CONCLUSIONS: The great interest GPs took in this study encourages further investigation through a more in depth questionnaire designed with the help of GPs themselves and interested statisticians.
Assuntos
Relações Interprofissionais , Médicos de Família , Radioterapia (Especialidade) , Adulto , Feminino , Humanos , Entrevistas como Assunto/métodos , Itália , Masculino , Pessoa de Meia-Idade , Médicos de Família/estatística & dados numéricos , Médicas/estatística & dados numéricos , Qualidade da Assistência à Saúde/estatística & dados numéricos , Radioterapia (Especialidade)/estatística & dados numéricos , Inquéritos e Questionários , TelefoneRESUMO
Seventy-six patients with primary gastric non-Hodgkin's lymphomas (PGL) were diagnosed, and 75 were treated between 1975 and 1985. According to the Working Formulation 22 patients had low-grade malignant histologic subtypes, 27 intermediate-grade, and 27 high-grade. Twenty-four cases were diagnosed by endoscopic biopsies, 52 through laparotomy biopsies. Forty-five underwent subtotal or total gastric resection; seven were considered unresectable at laparotomy; 23 did not undergo surgery because of the high operative risk, mainly due to advanced age and coexisting diseases; and one died of myocardial infarction a few days after admission, before starting therapy. All patients who did not undergo laparotomy were staged with bipedal lymphangiography or abdominal ultrasonography and/or computed tomography. Stage, evaluated according to the criteria of Musshoff, was I or II1 in 16 cases, II2 in five, and IV in the remaining 55. Treatment modalities included surgery (S), chemotherapy (CT), radiotherapy (RT), and combinations thereof in the following proportions: only S in ten cases, S + CT in 32 cases, S + RT in one case, S + CT + RT in two cases, CT only in 25 cases, CT + RT in five cases. No substantial differences in response to therapy and in survival were found in relation to the different treatments. Ten-year survival was 43% in Stage I or II and 20% in Stage IV. Of the 45 resected patients, five postoperative deaths were recorded (11%). No bleeding or perforations were observed in the 30 unresected patients, and survival of such cases compared with that of the resected ones. These findings, together with data from the literature, suggest that some of the advantages claimed for surgery in PGL (debulking and abatement of the risk of perforation or hemorrhage during CT or RT) have been overestimated in relation to the intrinsic surgical risk and to the possibility of anticancer therapy. Gastric resection may still be unavoidable as a diagnostic procedure in a minority of cases and may represent the primary therapeutic procedure in clinically assessed early-stage and low-risk patients, but it cannot be considered mandatory whenever possible merely for debulking purposes or to obviate possible perforation or hemorrhage. The CT and/or RT can be effective in unresected and even bulky cases, providing minimal risk of severe hemorrhage or perforation.
Assuntos
Linfoma não Hodgkin/cirurgia , Neoplasias Gástricas/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Terapia Combinada , Endoscopia , Feminino , Humanos , Linfoma não Hodgkin/mortalidade , Linfoma não Hodgkin/terapia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Complicações Pós-Operatórias , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/terapia , Taxa de SobrevidaRESUMO
PURPOSE: To test the adequacy of the CVPP four-drug regimen as ancillary chemotherapy associated with extended-field radiotherapy in the treatment of early, unfavorable, clinically staged Hodgkin's disease. PATIENTS AND METHODS: The population of this prospective, multicenter study consisted of 49 patients with stage I-II disease, associated with bulky involvement or unfavorable histology (lymphocyte-depleted nodular sclerosis or lymphocyte depletion), systemic symptoms or extranodal involvement, or presenting with stage III A favorable-histology disease, with or without extranodal involvement. RESULTS: Complete remission was achieved in 39 patients, partial remission in 2, while 8 patients did not respond. Four patients have relapsed so far (median follow-up: 43 months), all of whom were subsequently rescued with different salvage treatments. Dose intensity (mean +/- SD: 0.83 +/- 0.12) and hematological toxicity (including 2 deaths from infection) were higher when RT followed CT than when it was interposed in the middle of the 6 cycles. No growth factors were used. Nonhematological toxicity was very low and fully tolerable. CONCLUSIONS: Results confirmed the mild neurological and gastroenteric side effects of CVPP that make it an interesting MOPP-variant regimen. This combination seems most indicated when a regimen devoid of cardiac and pulmonary toxicity is required for association with full-dosage mediastinal radiotherapy, as is often the case in early, unfavorable Hodgkin's disease. The optimal sequence consists of radiotherapy administered after completion of the chemotherapy program. The use of growth factors for correction (or prevention) of marked leukopenia seems appropriate.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/radioterapia , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Terapia Combinada , Feminino , Doença de Hodgkin/patologia , Humanos , Lomustina/administração & dosagem , Lomustina/efeitos adversos , Masculino , Mecloretamina/administração & dosagem , Mecloretamina/efeitos adversos , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Prednisona/efeitos adversos , Procarbazina/administração & dosagem , Procarbazina/efeitos adversos , Prognóstico , Estudos Prospectivos , Indução de Remissão , Vimblastina/administração & dosagem , Vimblastina/efeitos adversos , Vincristina/administração & dosagem , Vincristina/efeitos adversosRESUMO
La distrofia muscular tipo Becker se diferencia de la tipo Duchenne por ser de instalación más tardía, progresión lenta y pronóstico más favorable. Se presenta un paciente de 21 años, varón, que comenzó después de la pubertad con debilidad muscular, que evolucionó con lentitud sin llegar a comprometer sus tareas habituales. La sintomatología cardíaca fue la dominante del cuadro, falleciendo por insuficiencia cardíaca y tromboembolismo pulmonar. Los hallazgos clínicos, de laboratorio, electromiográficos, electrocardiográficos y anatomopatológicos confirmaron el diagnóstico de distrofia muscular tipo Becker con afectación cardíaca. Llama la atención el mayor compromiso del músculo cardíaco respecto del esquelético, hecho no referido en la literatura. Nos planteamos la posibilidad de estar frente a una forma de distrofia muscular diferente de la descripta por Becker