Cytogenetic findings in pediatric adipose tumors: Consistent rearrangement of chromosome 8 in lipoblastoma

ArticleinGenes Chromosomes and Cancer 6(1):24-9 · January 1993with8 Reads
DOI: 10.1002/gcc.2870060106 · Source: PubMed
Characteristic cytogenetic aberrations have been reported in adult lipomas and liposarcomas, but few karyotypes have been reported for pediatric adipose neoplasms. In this report we describe a consistent rearrangement, der(8)(pter-->q13::q24.1-->qter), in 2 of 3 lipoblastomas. A similar der(8) was present in the only other published lipoblastoma karyotype, but this der(8) has not been reported in lipomas, liposarcomas, or nonadipose solid tumors. We investigated the potential specificity of der(8)(pter-->q13::q24.1-->qter) by karyotyping an unselected series of nonlipoblastoma adipose tumors in children and young adults. The series included 14 lipomas, 2 atypical lipomas ("well-differentiated liposarcomas"), and 2 angiomyolipomas; der(8) was not found in any tumor from this series. Three lipomas, however, contained rearrangements in the region of chromosome band 12q14, as has been described frequently in adult lipomas. Because clinical features in lipoblastoma can mimic those in liposarcoma, recognition of der(8)(pter-->q13::q24.1--qter) is of potential diagnostic relevance.
    • "der the age of 10 years. Principal clues for the diagnosis of lipoblastoma are striking lobulation, maturation toward the center of the lobules, and absence of nuclear atypia or atypical mitoses. [4] Lipoblastoma often exhibits chromosome abnormalities at 8q11‑13, whereas liposarcoma typically exhibits chromosome rearrangements in the region 12q14. [8,9] Lipoblastomas are benign tumors and no malignant degeneration has been documented. Recurrence has been reported in 14%‑25% of cases, [1] usually due to incomplete resection or diffuse disease. Complete resection of lipoblastomas yields excellent prognosis. [1,2]"
    Full-text · Article · Jan 2013
    • "The tumor arises in many different anatomic locations and can have morphologic features that could be confused with liposarcoma [2], lipoma [3], fibrolipoma [4], lipofibromatosis [5] , lowgrade fibromyxoid sarcoma [6], or even neurothekeoma [7] . Recently, a nonrandom distinct cytogenetic abnormality characterized by 8q11-q24 chromosomal aberration has been demonstrated in lipoblastomas891011. We report a rare case of mediastinal lipoblastomatosis with complex cytogenetic karyotype, and we review the medical literature. "
    [Show abstract] [Hide abstract] ABSTRACT: Lipoblastoma or lipoblastomatosis is an uncommon fatty tumor seen in children up to the age of 5 years, with very rare exceptions above that age. It usually occurs in the extremities, but it can arise in other anatomical sites, including the head and neck area, trunk, mediastinum, mesentry, and retroperitoneum. We report a 9-month-old boy with mediastinal lipoblastomatosis showing unusually complex chromosomal aberrations. Five years following surgical excision that was incomplete as a result of the tumor's proximity to major blood vessels, the patient developed a recurrence of the tumor in the mediastinum with extension to the neck and spinal dura. We present a review of the literature related to mediastinal lipoblastoma and lipoblastomatosis and the chromosomal abnormalities of these tumors.
    Full-text · Article · Mar 2009
    • "The biological significance of this finding is unclear [3]. Cytogenetic investigations have so far been reported on 4 solitary angiomyolipomas, and two of them had clonal chromosomal abnormalities456. "
    [Show abstract] [Hide abstract] ABSTRACT: Cytogenetic data on angiomyolipomas are scarce. We report a series of 10 cases from nine patients without clinical history of tuberous sclerosis. We found chromosome abnormalities in two, and in both cases the chromosomal changes were only numerical ones, i.e., trisomy 7 and trisomy 8.
    Full-text · Article · Jan 1998
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