RESUMO
Colorectal primary signet ring cell carcinoma (SRCC) is a rare entity accounting for nearly 1% of all colorectal carcinomas. It is an independent prognostic factor associated with less favorable outcome. This aggressiveness is mainly due to the intrinsic biology of these tumors. Here is an overview of the literature related to clinicopathological features, molecular biology, and management of SRCC of the colon and the rectum.
Assuntos
Carcinoma de Células em Anel de Sinete , Neoplasias do Colo , Doenças Raras , Neoplasias Retais , Carcinoma de Células em Anel de Sinete/epidemiologia , Carcinoma de Células em Anel de Sinete/genética , Carcinoma de Células em Anel de Sinete/patologia , Carcinoma de Células em Anel de Sinete/terapia , Neoplasias do Colo/epidemiologia , Neoplasias do Colo/genética , Neoplasias do Colo/patologia , Neoplasias do Colo/terapia , Genes ras , Humanos , Incidência , Perda de Heterozigosidade , Instabilidade de Microssatélites , Mucina-5AC/metabolismo , Mucina-2/metabolismo , Mutação , Proteínas de Neoplasias/metabolismo , Prognóstico , Doenças Raras/epidemiologia , Doenças Raras/genética , Doenças Raras/patologia , Doenças Raras/terapia , Neoplasias Retais/epidemiologia , Neoplasias Retais/genética , Neoplasias Retais/patologia , Neoplasias Retais/terapia , Quinases raf/genéticaRESUMO
BACKGROUND: Malignant mediastinal germ cell tumors are a rare disease and represent only 1% to 4% of all mediastinal tumors. Gonadal germ cell tumors are generally the most common type and constitute 90% of germ cell tumors. The mediastinum is the second most frequently affected area ahead of other extragonadal areas, which include the retroperitoneum, the sacrococcygeal area, and the central nervous system. We report on the case of a mediastinal yolk sac tumor with a complete histological response to chemotherapy. CASE PRESENTATION: A 26-year-old Moroccan man, without a medical or surgical history, presented with a four-month history of chest distress, dyspnea, and a frequent dry cough for the previous month. A computed tomographic scan of the chest revealed a bulky mediastinal mass, which was biopsied. Histologically, the tumoral mass proved to be a yolk sac tumor. The serum level of alpha-fetoprotein of this patient was elevated to 19052 ng/ml.After 4 courses of preoperative chemotherapy, the patient underwent a surgical resection of the tumor, with a complete pathologic response.At the time of writing, the patient is alive with complete remission without any evidence of recurrence. CONCLUSION: Primary mediastinal Yolk sac neoplasm represent a unique entity, and as such require specialized management. The diagnosis should be made not only by morphological studies but the patient's age and the elevation of serum alpha-fetoprotein should also be considered. The utilization of cisplatin-based chemotherapy is associated with the best chance of a cure for this disease. This should be followed by surgical resection of the residual tumor in the nonseminomatous germ cell tumor.
Assuntos
Tumor do Seio Endodérmico/tratamento farmacológico , Tumor do Seio Endodérmico/patologia , Neoplasias do Mediastino/tratamento farmacológico , Neoplasias do Mediastino/patologia , Adulto , Tumor do Seio Endodérmico/diagnóstico por imagem , Humanos , Masculino , Neoplasias do Mediastino/diagnóstico por imagem , Radiografia Torácica , Tomografia Computadorizada por Raios X , Resultado do TratamentoAssuntos
Antineoplásicos Hormonais/efeitos adversos , Hipertrigliceridemia/induzido quimicamente , Tamoxifeno/efeitos adversos , Adulto , Antineoplásicos Hormonais/uso terapêutico , Neoplasias da Mama/tratamento farmacológico , Feminino , Humanos , Hipertrigliceridemia/fisiopatologia , Índice de Gravidade de Doença , Tamoxifeno/uso terapêuticoRESUMO
INTRODUCTION: Primary lymphoma of the prostate is very rare. In this paper we present a case of early stage non-Hodgkin lymphoma of the prostate managed with six cycles of rituximab-based chemotherapy, and review the related literature. CASE PRESENTATION: An 84-year-old man was admitted to our hospital having signs and symptoms suggestive of prostatic disease for 3 years. Histological and immunocytochemical studies of trans-urethral biopsy of the prostate showed diffuse large B-cell lymphoma. Radiological assessment of disease confirmed the diagnosis of early stage lymphoma of the prostate. The patient was managed by 6 of rituximab 375 mg/m2 on day 1, cyclophosphamide 750 mg/m2 on day 1, doxorubicin 50 mg/m2 on day 1, vincristine 1.4 mg/m2 on day 1, and prednisone 50 mg/m2 on days 1 to 5 with complete clinical and radiological response. He remained disease free, until now, 30 months after the end of chemotherapy. CONCLUSION: According to the literature, the treatment and prognosis of primary lymphoma of the prostate is the same as that of other nodal lymphomas. Rituximab-based regimen should be considered in the management of prostatic diffuse large B-cell lymphoma.