RESUMO
An increased incidence of acute myeloid leukemia (AML) has recently been documented in patients post-solid organ transplantation but the incidence and types of myelodysplastic syndromes (MDS) occurring in this patient population are not known. We identified 5 patients (3M, 2F, age 48-64 years) who developed MDS ranging from 1.8 to 25 years (median 4.2 years) post-solid organ transplantation, only 2 patients had received azathioprine. The cumulative incidence of MDS in heart and lung transplant recipients at 15 years was 0.5% and 1.8%, respectively, which is markedly higher compared to the general population. Low-risk types of MDS predominated, 3 of 5 patients are alive (median 3.9 years) since diagnosis. Deletions of chromosome 20q, which have not been previously reported in post-transplant MDS/AML, were identified in 3 cases. Our findings expand the morphologic and cytogenetic spectrum of MDS occurring post-solid organ transplantation and suggest that mechanisms beside azathioprine toxicity might be important in disease pathogenesis.
Assuntos
Síndromes Mielodisplásicas/classificação , Síndromes Mielodisplásicas/epidemiologia , Transplante de Órgãos/efeitos adversos , Biópsia , Medula Óssea/patologia , Feminino , Humanos , Incidência , Leucemia Mieloide/epidemiologia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/patologia , Transplante de Órgãos/classificação , Complicações Pós-Operatórias/classificação , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/patologia , Estudos RetrospectivosRESUMO
This study reports the first well-documented case of sporadic Burkitt lymphoma arising in and confined to the uterine corpus in a 40-year-old woman who presented with vaginal bleeding. Endometrial curettings showed a diffuse infiltrate of medium sized lymphocytes with the characteristic morphologic and immunophenotypic features of Burkitt lymphoma. Fluorescence in-situ hybridization demonstrated the t(8;14)(q24;q32) translocation. There was no evidence of extra-uterine disease and the patient is alive without disease 10 months after hysterectomy and chemotherapy. This report demonstrates that Burkitt lymphoma can present as isolated, organ confined disease at unusual sites and with protean symptoms.
Assuntos
Linfoma de Burkitt/patologia , Neoplasias Uterinas/patologia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Burkitt/genética , Linfoma de Burkitt/terapia , Cromossomos Humanos Par 14/genética , Cromossomos Humanos Par 8/genética , Feminino , Humanos , Histerectomia , Hibridização in Situ Fluorescente/métodos , Sensibilidade e Especificidade , Translocação Genética , Resultado do Tratamento , Neoplasias Uterinas/genética , Neoplasias Uterinas/terapiaRESUMO
We report a case of congenital pulmonary myofibroblastic tumor, and review prior reports of this rare neoplasm to demonstrate its clinically benign behavior despite histologic features previously interpreted as sarcoma. The patient, a female neonate, presented with severe respiratory distress after cesarean section delivery. A large radio-opaque mass was detected in the right hemithorax and resected by right bilobectomy. The tumor mass, confined to the lung, was composed of interlacing fascicles of plump spindle cells showing myofibroblastic differentiation and complex cytogenetic abnormalities. Though sarcomatous in appearance, with highly cellular areas and numerous mitoses, there has been neither tumor recurrence nor metastases. The patient remains alive and well 1 year after surgery. Review of the few other reported cases confirms the uniformly benign behavior of this tumor.
Assuntos
Fibroblastos/patologia , Fibrossarcoma/congênito , Fibrossarcoma/patologia , Neoplasias Pulmonares/congênito , Neoplasias Pulmonares/patologia , Músculo Liso/patologia , Feminino , Fibrossarcoma/diagnóstico por imagem , Humanos , Imuno-Histoquímica , Recém-Nascido , Neoplasias Pulmonares/diagnóstico por imagem , Radiografia TorácicaRESUMO
BACKGROUND: Villin, a 95-kd cytoskeletal protein associated with axial microfilament bundles of brush border microvilli, is mostly restricted to intestinal glandular tumors. Villin immunoexpression was recently observed in a small number of carcinoids of the intestinal tract and lung, but its significance in a broad category of neuroendocrine tumors has not been evaluated. DESIGN: A total of 114 neuroendocrine tumors of different origins were tested for villin expression. They included gastrointestinal carcinoids (n = 30), lung carcinoids (n = 15), small cell carcinomas of the lung (n = 24), small cell carcinomas of other sites (n = 15), islet cell tumors (n = 8), Merkel cell carcinomas (n = 6), paragangliomas (n = 6), and others (n = 10). Nine round cell sarcomas were tested as well. RESULTS: Villin immunoreactivity was present in 85% of gastrointestinal carcinoids and small cell carcinomas, but was found in only 40% of lung carcinoids. Other tumors tested were virtually negative for villin. In general, while cytoplasmic reactivity was most common, a characteristic apical membranous pattern simulating brush border was seen in 76% of the gastrointestinal carcinoids and in 50% of the lung carcinoids. CONCLUSIONS: We found that villin was predominantly restricted to gastrointestinal neuroendocrine tumors (excluding islet cell tumors), although a small number of bronchial carcinoids may be positive as well. These results suggest a role for villin in the differential diagnosis of neuroendocrine tumors.