1/14. Klinefelter's syndrome and bladder cancer.A patient with Klinefelter's syndrome is described who also had transitional cell carcinoma of the bladder. His mother and maternal grandfather died of neoplasms. It is suggested that neoplasm and aneuploidy in the same family could have been caused by an inherited chromosomal instability rather than coincidence.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/14. Mediastinal choriocarcinoma in a chromatin-positive boy.A case of primary mediastinal choriocarcinoma in a chromatin-positive boy is reported. The incidence of neoplasms in patients with so-called Klinefelter's syndrome is discussed as well as embryogenesis and diagnostic evaluation in patients presenting with this tumor.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
3/14. A case of klinefelter syndrome with retroperitoneal teratoma.klinefelter syndrome (KS) is often associated with various neoplasms, especially germ cell tumors. mediastinum is the most favored site of extragonadal germ cell tumors with KS, which is somewhat different from those without KS. The retroperitoneal germ cell tumor in KS is very rare. A five-month-old boy with an abdominal mass was found to have a retroperitoneal tumor. After surgical removal, he was diagnosed to have mature cystic teratoma. Cytogenetic study of his peripheral lymphocytes revealed that his karyotype was consistent with KS. This case suggests that patients with KS might be at risk of having germ cell tumors in sites other than mediastinum. It also suggests that all cases with these tumors should be screened for the presence of karyotypic abnormalities, and it might help to assess the exact correlation between germ cell tumors and KS, and to treat them accordingly.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
4/14. Agnogenic myeloid metaplasia associated with klinefelter syndrome: a case report.klinefelter syndrome is the most commonly diagnosed sex chromosome disorder among males. It is usually associated with 47 chromosomes, including two Xs and one Y. The formal cytogenetic designation for klinefelter syndrome is 47, XXY; the extra sex chromosome is due to meiotic chromosomal nondisjunction. Increased risk of various malignant diseases has been recognized among patients with different congenital chromosomal abnormalities. Since the early 1960s, numerous reports have appeared of an increased risk of malignant neoplasms among patients with klinefelter syndrome. Evidence suggests a correlation with increased incidences of germ cell tumors and breast cancers. Whether these patients are at an increased risk of hematologic malignant disease, especially acute leukemia, is still uncertain. This report describes a patient with agnogenic myeloid metaplasia and klinefelter syndrome, an association not previously reported.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
5/14. adenocarcinoma of the prostate in a 41-year-old man with XXY karyotype and chronic lymphocytic leukemia: report of a case.We report a case of adenocarcinoma of the prostate in a 41-year-old man with 47XXY karyotype (Klinefelter's syndrome) and chronic lymphocytic leukemia. The increased incidence of malignancy in individuals with Klinefelter's syndrome has been well documented for certain neoplasms. adenocarcinoma of the prostate has not been reported previously in a patient with Klinefelter's syndrome and a 47XXY karyotype. Absence of mosaicism was confirmed by peripheral lymphocyte, skin fibroblast, bone marrow cell and spleen stroma fibroblast cultures. Chronic lymphocyte leukemia, especially the T-cell cytotoxic/suppressive variant, may additionally add to an immunological deficit. Since carcinoma of the prostate, Klinefelter's syndrome and chronic lymphocytic leukemia are common, the lack of a previous report is interesting. Etiological aspects are discussed.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
6/14. Isolated primary aldosteronism in a patient with adrenal carcinoma and XY/XXY mosaic Klinefelter's syndrome.Although breast cancer, germ cell tumors and other neoplasms are known to occur in patients with Klinefelter's syndrome, adrenal carcinoma has not yet been reported in such patients. We describe a rare case of severe primary aldosteronism as the unique manifestation of a large adrenocortical carcinoma in a patient with Klinefelter's syndrome. Complete biological and hormonal evaluation was performed. Surgical treatment was successful and the patient remained asymptomatic with normal biological and hormonal values after 1 year of followup.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
7/14. Klinefelter's syndrome and extragonadal germ cell tumors.A 25-year-old man presented with diffuse metastatic pure choriocarcinoma, thyrotoxicosis, and cardiac tamponade. No discernable testicular primary tumor was found. The patient's peripheral blood karyotype was 47, XXY and phenotypic features of Klinefelter's syndrome were present. The patient was treated with aggressive combination chemotherapy followed by salvage surgery and remains in complete remission 3 years after diagnosis. Pure choriocarcinoma, although rare as a primary testicular neoplasm, accounts for 15% of extragonadal germ cell tumors in general and 30% of germ cell tumors in patients with Klinefelter's syndrome. Historically, the diagnosis of pure choriocarcinoma has been thought to convey a very poor prognosis. The occurrence of hyperthyroidism is unique to tumors containing choriocarcinomatous elements and the management of this disorder is discussed. Treatment of extragonadal germ cell tumors is also discussed with special reference to the roles of combination chemotherapy and salvage surgery.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
8/14. Acute leukemia following a malignant teratoma in a child with Klinefelter's syndrome: case report and review of secondary leukemias in children following treatment of a primary neoplasm.A case of Klinefelter's syndrome with the development of a mediastinal teratocarcinoma is reported suggesting that the association of a gonadotropin-secreting tumor with the XXY chromosomal abnormality may be more than coincidental. Whereas this child appeared to survive the effects of the teratocarcinoma, he succumbed to acute leukemia two years later. This prompted a review of secondary leukemias in children following chemotherapy/radiotherapy for another primary malignancy. These patients responded poorly to treatment of the secondary leukemia with a median survival of about four months. The incidence of secondary leukemias might be expected to be on the rise as increasing numbers of pediatric cancer patients are surviving longer after treatment with agents that are potentially leukemogenic or carcinogenic themselves. Children who have survived cancer and its therapy present special problems and it will be necessary for the pediatrician and practitioner to monitor these children.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
9/14. Klinefelter's syndrome and mediastinal germ cell neoplasms.We describe 2 patients with Klinefelter's syndrome in whom mediastinal germ cell neoplasms developed. This association also has been noted in the literature in 6 other cases. The pathogenesis of these otherwise rare neoplasms probably is related to the genetic abnormality and the abnormal hormonal status in Klinefelter's syndrome. patients with Klinefelter's syndrome appear to have an increased risk of development of extragonadal mediastinal germ cell neoplasms and should be followed closely, while patients with mediastinal germ cell neoplasms should have karyotypes performed.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
10/14. Mediastinal teratoma and precocious puberty in a boy with mosaic klinefelter syndrome.We describe a boy who developed precocious puberty resulting from chorionic gonadotropin produced by a mediastinal germ cell tumor. Following tumor removal he began spontaneous precocious sexual development which was treated and then arrested spontaneously. Investigation of this arrested puberty established that he had klinefelter syndrome (KS) mosaicism. He represents the first instance of KS mosaicism reported with a mediastinal germ cell tumor, a neoplasm commonly reported in males with a 47,XXY karyotype. We recommend that all males with KS and early sexual development or with "normal" testicular growth be screened with measurement of germ cell tumor markers including beta-subunit of human chorionic gonadotropin and alpha-fetoprotein.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
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