T-cell lymphoma and mature nephroblastoma after synchronous bilateral Wilms' tumor

Article Abstract:

The majority of Wilms' tumors, kidney tumors arising from tissues of the embryonic mesoderm, occur in children. While the majority of cases involve only one kidney, as many as 10 percent of all cases are bilateral. Bilateral Wilms' tumors are found in combination with urogenital defects at a rate significantly higher than chance. Wilms' tumor is one of the three features that characterize the 'Drash syndrome' along with pseudohermaphroditism and nephron disorder. The authors describe a case in which pseudohermaphroditism and bilateral Wilms' tumor occurred together, but nephron disorder has not been observed. At birth, a male infant had a short phallus and hypospadias, the exit of the urethra occurring in an abnormal spot, in this case, on the underside of the penis. The infant had a normal male karyotype, and the hypospadias was surgically corrected. At seven moths of age, symptoms of diarrhea, vomiting, and fever prompted a medical examination during which a palpable mass was discovered in the abdomen. This observation was compatible with bilateral Wilms' tumor; the right kidney was completely removed and the majority of the tumor mass on the left was removed. The residual left tumor was irradiated. Fifteen years later, the patient had an enlarged left tonsil removed after recurrent tonsillitis. Examination of the removed tonsil revealed a T-cell lymphoblastic lymphoma. At the same time, a firm mass was noticed in the left kidney. The mass was removed by partial nephrectomy and was found to be a fully differentiated Wilms' tumor. This simultaneous diagnosis of lymphoma and differentiated kidney tumor in a patient with bilateral Wilms' tumor is striking. While it cannot be ruled out that the second malignancy is a result of the treatment for the first, the authors suggest that bilateral Wilms' tumor is perhaps a unique case with a genetic contribution that may be lacking in unilateral cases. They also propose that as more becomes known about the genes of Wilms' tumor locus (located on the short arm of chromosome 11 at 11p13), different aspects of this tumor will be considered to be related. (Consumer Summary produced by Reliance Medical Information, Inc.)

Genetic aspects, Cancer in children, Childhood cancer, Nephroblastoma

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Primary lymphoma of the heart: prolonged survival with early systemic therapy in a patient

Article Abstract:

Lymphomas are tumors of the lymphatic tissues and may occur in many places within the body. Lymphoma of the heart is rare, and has a particularly poor prognosis; in many cases the patients die before the condition is diagnosed and in other cases within two or three weeks despite prompt treatment. In such cases, however, it might be imagined that the aggressiveness of this tumor is only part of the reason for the poor outcome; delay in diagnosis seems likely to have played an important part as well. A case has now been seen in which early diagnosis may have helped a patient with lymphoma of the heart to enjoy longer survival. The patient was a 64-year-old woman who developed chest pain, nausea, shortness of breath, and profuse sweating. Physical examination revealed nothing unusual, but electrocardiographic examination revealed heart abnormalities and a temporary pacemaker was required after the patient developed compete heart block. When ultrasound imaging was used to examine the patient's heart, signs of fluid collecting in the pericardium surrounding the heart were seen. This finding was confirmed with CT scan, which also showed a vague mass next to the heart. The pericardial fluid was sampled and malignant cells were discovered. Imaging techniques revealed no indications of lymphoma elsewhere in the body, and bone biopsies uncovered no signs of lymphoma. The patient was treated with the chemotherapeutic drugs cyclophosphamide, doxorubicin, vincristine, and prednisone, and CT scan revealed a reduction of about 75 percent in the mass by the heart. The patient remains alive more than 18 months after completion of therapy. The characteristics of the lymphoma in this patient were similar to those in other patients who died rapidly. The initiation of chemotherapy, which in this case began within three days of the patient's presentation, is most likely responsible for the long survival of this patient. At present, this woman seems to be the longest survivor of lymphoma of the heart. (Consumer Summary produced by Reliance Medical Information, Inc.)

Cardiovascular tumors, Vascular tissue tumors

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Adult T-cell lymphoma in Israeli patients of Iranian origin

Article Abstract:

Four patients with adult T-cell lymphoma were seen in the Tel Aviv area of Israel. Interestingly, all four had emigrated from the same region of Iran. A review of the cases was unremarkable, except that there was no skin involvement in the disease. Using the polymerase chain reaction, an extremely sensitive DNA detection method, three of the four patients were found to have evidence of infection with human T-cell lymphotropic leukemia virus 1 (HTLV-1). The prevalence of HTLV-1 infection among Iranians is not known, although a previous study of a small group of Israelis of Iraqi and Iranian origin did not reveal an unusually high rate. Nonetheless, HTLV-1 infection should be considered in Iranian patients with disease resembling adult T-cell lymphoma. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, HTLV-I (Virus), Adult T-cell leukemia-lymphoma

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