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21/56. Tumor in the horseshoe kidney: clinical implications and review of embryogenesis.

    We report on 3 patients with tumor in a horseshoe kidney, 1 of whom had bilateral tumor (renal cell cancer on the right side and urothelial cancer on the left side). Tumors that arise predominantly in the bridge of a horseshoe kidney can mimic the symptoms of an intra-abdominal disease process. Besides routine diagnostic procedures, angiography is essential to plan the surgical approach, which in principle should be organ-sparing. The literature of the embryology of the horseshoe kidney was reviewed for a relationship between the abnormal renal development and the site of tumorigenesis, and for development of a key for the wide variation of blood supply. Recently reported data suggest that the theory of a mechanical fusion is valid only for horseshoe kidneys with a fibrous isthmus but that an abnormal migration of the posterior nephrogenic area causes the majority of horseshoe kidneys in which the isthmus consists of parenchyma. Development of the isthmus through abnormal migration could predispose this location for renal cell cancer and would explain the varying forms of blood supply. Additionally, this hypothesis supports the previously raised assumption that horseshoe kidneys may be the result of teratogenic factors, which also may be responsible for the known increased incidence of related congenital anomalies and of nephroblastoma.
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ranking = 1
keywords = tumorigenesis
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22/56. Mediastinal germ cell tumor associated with histiocytic sarcoma of spleen: case report of an unusual association.

    We present an unusual association of mediastinal germ cell tumor containing seminoma and angiosarcoma components and splenic histiocytic sarcoma. A 15-year-old boy presented with chest pain. Histopathologically, an anterior mediastinal mass contained typical seminoma, immature teratoma, embryonal carcinoma, angiosarcoma, yolk sac tumor, and polyembryoma. An abdominal ultrasonogram revealed a huge splenomegaly with multiple ill-defined low echogenic nodules, 1 month after the second cycle of chemotherapy. Histopathologically, large, round-to-oval tumor cells with abundant eosinophilic cytoplasm often contained eccentrically placed nuclei with vesicular chromatin and an irregular nuclear membrane. The tumor cells were immunoreactive for CD68, CD31, and CD4. The cytogenetic results showed deletion of the long arm of chromosome 5 and trisomy 8. This lesion might have been on the pathway of multistep tumorigenesis toward a final leukemia.
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ranking = 1
keywords = tumorigenesis
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23/56. Gliomatosis cerebri and pituitary adenoma: case report and literature review.

    The diagnosis of Gliomatosis cerebri (GC) is known to be difficult and is still a matter of debate. We describe an in vivo case of GC associated with a pituitary tumor. A 47-year-old woman presented with short-term memory loss. A MRI revealed the presence of a pituitary enhancing tumor and a diffuse lesion involving the brain. A left pterional craniotomy with partial temporal lobectomy and removal of the pituitary lesion were performed in order to obtain diagnosis. The histological analyses showed a pituitary non-functioning tumor and a GC consisting of neoplastic oligodendrocytes and astrocytes. Both lesions showed nuclear immunoreactivity for progesterone receptors (PGr) and estrogen receptors (EGr). This result could suggest there is a common receptor substrate in these tumors. In this case hormones could constitute a common step in tumorigenesis of both lesions.
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ranking = 1
keywords = tumorigenesis
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24/56. Simultaneous occurrence of acute myelogenous leukemia and seminoma of the testis.

    Simultaneous tumors are rarely encountered during the course of acute leukemias. We report on a case of seminoma of the testis that occurred during the evolution of acute myelogenous leukemia. To our knowledge, this simultaneous association has not previously been described, but a causal relationship was not apparent in the present case. The likelihood of a common carcinogenesis existed, but direct exposure to carcinogens could not be established. Although the results of a physical examination and echography were normal at the time of diagnosis, we cannot exclude the presence of microscopic cancer of the testis. Since the dissemination pattern of seminoma is usually slower than that observed in this case and the disease remains limited to the lymph nodes for long periods following dissemination, the rapid development of the present case might have been attributable to the immunosuppression and the scrotal sepsis that occurred during the induction therapy. immunosuppression might have stimulated the progression of a primary microscopic seminoma and the development of metastasis, whereas the scrotal sepsis and inflammation might have favored the occurrence of metastasis through bypass of the lymphatic barrier.
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ranking = 0.6349615253955
keywords = carcinogenesis
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25/56. Simultaneous endometrioid ovarian and uterine carcinoma diagnosed after an in vitro fertilization procedure--case report and review of the literature.

    BACKGROUND: The presence of simultaneous carcinomas involving both the ovary and uterus is relatively uncommon, while the possible link between fertility drugs and carcinogenesis still remains controversial. CASE: The case of a 40-year-old patient with simultaneous aggressive endometrioid carcinoma of the ovary and uterus a few months after the sixth attempt of in vitro fertilization is presented. The patient had de novo lung disease at surgery and diffuse metastatic spread to adjacent bone, subcutaneous tissue and the central nervous system (CNS) soon after a spectacular response to the primary paclitaxel/carboplatinum chemotherapy and while on maintenance and second-line chemotherapy, respectively. CONCLUSION: The fulminating course of our patient might in part be attributed to the existence of advanced disease at presentation. Definite conclusions about the possible association with the previously performed assisted reproduction cannot be drawn but close clinical surveillance of such patients before, during and after infertility treatment is strongly warranted.
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ranking = 0.6349615253955
keywords = carcinogenesis
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26/56. Multiple bronchioloalveolar carcinomas in acromegaly: a potential role of insulin-like growth factor i in carcinogenesis.

    The molecular pathogenesis of lung cancer, especially multiple and synchronous bronchioloalveolar carcinomas (BACs), is still unknown. Here, we report two cases of multiple BACs associated with acromegaly, and discuss about the possible relationship between these two pathological condition. The first patient was a 52-year-old female with a history of Hardy's surgery for pituitary growth hormone cell adenoma 2 years earlier. The second patient was a 57-year-old female with acromegaly and obstructive sleep apnea syndrome. Both patients were non-smokers and showed a high serum level of insulin-like growth factor i (IGF-I) at the time of admission, even though the level of growth hormone had decreased. High-resolution computed tomography (HRCT) revealed multiple small nodules with pure ground-glass opacity (GGO) in both lungs of the first patient and a small nodule with pure GGO in the right lung of the second one. Partial resection for these tumors were performed under video-assisted thoracoscopic surgery. Resected lung specimens of the first case revealed one papillary adenocarcinoma, seven BACs, and 11 atypical adenomatous hyperplasias (AAHs). The second case showed two foci of BACs. Immunohistochemically, all BACs were strongly positive for IGF-IR which is a specific receptor for IGF-I, and all AAHs were also weakly positive for IGF-IR. Since IGF-I is known as a potent growth factor for normal as well as cancerous cells, it might play an important role for tumorigenesis and/or tumor progression of BACs through its interaction with and/or upregulation of IGF-IR. In addition, much attention should be paid to detect lung lesions in acromegaly with high serum level of IGF-I.
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ranking = 3.539846101582
keywords = tumorigenesis, carcinogenesis
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27/56. Developmental genetics of neuroblastoma.

    case reports of neuroblastoma revealed that some individuals are genetically predisposed and that this genetic predisposition may have other consequences. According to a mutation model, two classes of individuals could acquire neuroblastoma. One (prezygotic) was a rare class that carried a dominant gene imparting high risk of the tumor. The other (postzygotic) comprised all other individuals, each at low risk. The model related tumor incidence to germinal and somatic mutation rates and thereby carried implications for environmental modification of tumorigenesis and demographic variation in incidence. case reports also revealed associations of neuroblastoma with congenital defects and a susceptibility to second tumors. Analogy with retinoblastoma and Wilms' tumor of the kidney suggested that these associations could result from action of a neuroblastoma gene or from chromosomal aberration. One known dominantly inherited condition, von Recklinghausen's disease, could dispose to neuroblastoma and create some associations. According to the two-mutation model, neuroblastoma may have been a single recessive gene disorder at the level of the cell. The phenomena of aganglionosis, neuroblastoma in situ, maturation of neuroblastoma to ganglioneuroma, and spontaneous regression suggested that such a neuroblastoma gene interfered with normal developmental processes. The specificities of this gene and of those for von Recklinghausen's disease and pheochromocytoma suggested that the functiof a membrane macromolecule.
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ranking = 1
keywords = tumorigenesis
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28/56. A case of pheochromocytoma associated with meningioma.

    We have recently observed a rare case of pheochromocytoma associated with meningioma. To determine the mechanism of such a coexistence in this particular case, we compared the constitutional and pheochromocytoma genotypes of the patient using polymorphic four dna markers, and we found a loss of heterozygosity at the D1S7 locus. A loss of heterozygosity at this locus has previously been reported by Mathew et al. in pheochromocytoma patients, and our results were in agreement with that study. Therefore the allelic deletion at the D1S7 locus may be an important step in the tumorigenesis of pheochromocytoma in this case.
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ranking = 1
keywords = tumorigenesis
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29/56. Intrasellar primitive neuroectodermal tumor (PNET) in familial retinoblastoma: a variant of "trilateral retinoblastoma".

    An infant with bilateral familial retinoblastomas was found at age 6 months to have a large mass within the region of the sella turcica. The histology, immunoreactivity and ultrastructure of the tumor showed primitive neuroectodermal cells. A small number of cells showed positive immunostaining to retinal S antigen. This represents a rare form of so-called trilateral retinoblastoma. In most cases of trilateral retinoblastoma, the intracranial non-metastatic tumor is within the pineal gland. The occurrence of such a tumor in the region of sella turcica is very unusual and its possible significance is discussed in regard to our current understanding of the susceptibility to carcinogenesis conferred by inheritance of the mutant retinoblastoma gene, Rb-1.
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ranking = 0.6349615253955
keywords = carcinogenesis
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30/56. Multifocal tumorigenesis in the upper female genital tract--implications for staging and management.

    A review of 128 cases of "primary" ovarian mullerian carcinoma treated at the King George V Memorial Hospital was undertaken to determine the relative frequency with which such tumors were associated with evidence of multifocal primary neoplasia. Of the 128 cases studied, 115 were invasive carcinomas and 13 were noninvasive or borderline ovarian tumors ("tumors of low malignant potential"). Eight of 10 borderline serous ovarian tumors (80%) and 37 of 75 invasive serous carcinomas (49%) exhibited evidence of independent primary neoplasia at more than one anatomical site in the biopsy material available for review. Many of these cases represented bilateral primary ovarian tumors, but autochthonous extraovarian neoplasia was also commonly encountered. A single borderline endometrioid ovarian tumor and six of 15 endometrioid carcinomas (40%) were associated with biopsy-proven multifocal primary tumorigenesis. These were predominantly neoplasms in one or both ovaries plus adenocarcinoma in the uterine corpus. Other histological types of malignant common epithelial tumors of the ovaries did not demonstrate any such tendency, highlighting major differences in pathogenesis between members of this loosely associated group of ovarian cancers. Our study suggests that gynecological endometrioid and serous malignancies are commonly multifocal and we feel this has significant implications for the way these neoplasms are staged and therefore treated.
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ranking = 5
keywords = tumorigenesis
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