Cases reported "Retroperitoneal Neoplasms"

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1/8. Retroperitoneal and deep-seated lipoblastoma: diagnosis by CT scan and fine-needle aspiration biopsy.

    Lipoblastomas are most commonly superficially-located tumors. Our experience with three examples of deep-seated lipoblastomas is reported. The CT scans of all cases showed low-attenuation densities (less than 0 Hansfield units), strongly suggesting fatty composition. Fine-needle aspiration biopsy (FNAB) smears contained uni- and multivacuolated lipoblasts, myxoid areas, and a plexiform capillary network. The combination of CT scan and FNAB findings seems to be enough for definitive preoperative diagnosis of lipoblastoma, allowing differential diagnosis with other deep-seated tumors.
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2/8. leptin and leptin receptor expression in a lipoblastoma in an 8-year-old girl.

    leptin is a hormone that is produced by adipocytes. leptin acts on specific receptors in the hypothalamus. rna was isolated from a lipoblastoma of an 8-year-old girl and the expression of leptin and leptin receptor mRNA was analyzed by RT-PCR. The lipoblastoma tumor, a rare form of childhood tumors, expressed leptin and leptin receptors in a fashion similar to normal adipose tissue. We hypothesize that the peripheral action of leptin via its receptors could play a role in the development and/or progression of lipoblastoma. Whether or not leptin and leptin receptor expression play a role in the development and/or progression of lipoblastoma and other tumors is not clear to date. Copyrightz1999S.KargerAG, Basel
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3/8. Rearrangement involving chromosomes 1 and 8 in a retroperitoneal lipoma.

    Superficial lipomas are very common benign adipose tissue tumors. In contrast, deep-seated lipomas such as retroperitoneal lipomas, are extremely rare and have to be carefully distinguished from well-differentiated liposarcomas for appropriate treatment and follow-up. We report to, our knowledge, the first cytogenetic analysis of a retroperitoneal lipoma occurring in an adult, which showed a complex rearrangement interpreted as t(1;8)(q32;q22-q23) followed by a pericentric inversion of der(8). There was no detectable rearrangement of chromosome 12, and in particular no 12q14-q15 amplification. Because rearrangements of the 8q11-q13 region involving the PLAG1 gene have been described in lipoblastoma-another kind of benign adipose tumor--we used fluorescence in situ hybridization analysis to determine in the present case the chromosomal breakpoint on 8q was located between the ETO (8q22) and COX6C (8q22-q23) genes at a great distance from PLAG1. Karyotypic analysis of additional cases of retroperitoneal lipomas will be required to assess the significance of chromosome 1 and 8 rearrangements in a continuous effort to attain a better classification of adipose tissue tumors. Of great importance is the determination of such genetic markers as additional tools for the differential diagnosis between benign and malignant forms of adipose tumors, and to avoid erroneous diagnoses.
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keywords = lipoblastoma
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4/8. Retroperitoneal lipoblastoma. Report of a case and review of the literature.

    We present the first case of retroperitoneal lipoblastoma at the University Hospital of the west indies. This is one of the few reported cases in the general literature.
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keywords = lipoblastoma
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5/8. Retroperitoneal lipoblastoma involving the right common iliac artery and vein.

    A case of benign lipoblastoma of the retroperitoneum in a 12-month-old boy, presenting as a huge abdominal mass and right lower extremity swelling, was reported. Surgery revealed involvement of the right common iliac artery and vein by lipoblastoma, requiring the sacrifice of both vessels for complete removal of the tumour. Vascular continuity of both vessels was re-established by a saphenous vein graft interposition. Involvement of a great artery by benign lipoblastoma has not been reported before.
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keywords = lipoblastoma
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6/8. lipoblastoma in infancy and childhood.

    lipoblastoma is a rare benign neoplasm of fetal-embryonal fat tissue with a 14% tendency to recur. It occurs almost exclusively in infants and children. To my knowledge, less than 100 cases, including children and adults, have been reported in the English literature. Two retroperitoneal and one case of mesenteric lipoblastoma, all presenting as a large palpable abdominal mass, are described. Our patients, aged 12 years, 7 months, and 11 months, were treated by total surgical excision of the tumors. They are alive and well 5, 4, and 3 years postoperative, respectively, with no evidence of recurrence and no indication for any adjunctive therapy. Pathologic evaluation was decisive for the best treatment and prognosis.
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keywords = lipoblastoma
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7/8. Pediatric lipoblastomatosis: two unusual cases.

    Lipoblastomatosis is a rare, fatty tumor of childhood that is usually located in the limbs. Two pediatric patients were seen with tumors that originated in the mediastinum and retroperitoneal area. The mediastinal tumor was the infiltrating form of lipoblastomatosis while the retroperitoneal tumor was the noninfiltrating type. Neither form is malignant but local recurrences are common. Both were surgically excised without sacrifice of important contiguous structures that were surrounded by tumor. There has been no recurrence 21/2 and 6 years after operation. Conservative excision of this malignant-appearing but histologically benign tumor is emphasized.
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keywords = lipoblastoma
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8/8. Retroperitoneal lipoblastoma: report of one case.

    A one-year-old boy presented with progressive distension of the abdomen occurring over a few months. Computed tomography (CT) scan demonstrated a large, well-defined retroperitoneal mass with heterogeneous attenuation appearance and areas of low density showing as fatty substance. Slight enhancement of the soft tissue components was also noted. Reviewing of the literature indicates an accurate preoperative diagnosis of such fatty tumor is allowed by analysis of patient's age, tumor location, and characteristic CT pictures.
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