1/7. Evidence by spectral karyotyping that 8q11.2 is nonrandomly involved in lipoblastoma.We report two cases of lipoblastoma with chromosome 8-related aberrations, ie, a 92,XXYY,t(7;8Xp22;q11.2)x2 [8]/46,XY[16] in Case 1 and a 46,XY,-8,-13,add(16) (q22), mar, r [cp13]/46,XY[7] in Case 2. Using spectral karyotyping and fluorescence in situ hybridization techniques, the karyotype of Case 2 was redesignated as 46,XY, r(8), del(13)(q12), der(16)ins(16;8)(q22; q24q11.2)[cp13]/46,XY[7]. This report delineates a new chromosome rearrangement, ie, der(16)ins(16;8)(q22; q24q11.2) in lipoblastoma, and also confirms the t(7; 8)(p22;q11.2), reported only once previously, as a recurrent translocation involved in such a tumor. These findings provide valuable information for clinical molecular cytogenetic diagnosis of lipoblastoma. Furthermore, this report highlights the value of cytogenetic and molecular cytogenetic analysis in differential diagnosis of childhood adipose tissue tumors and adds to the number of lipoblastomas reported with chromosomal abnormalities at 8q11.2.- - - - - - - - - - ranking = 1keywords = lipoblastoma (Clic here for more details about this article) |
2/7. Lipoblastomatosis of the neck causing hemiparesis: a case report and review of the literature.BACKGROUND: lipoblastoma and lipoblastomatosis are rare pediatric adipose tumors that sometimes affect the neck or spinal cord. This case is the third report of lipoblastoma extending into the spinal canal, the first report of intradural tumor extension, and the first report of hemiparesis resulting from lipoblastoma compressing the spinal cord. methods: A 13-month-old boy was seen by a pediatrician for a firm, supraclavicular neck mass on the left side. After being evaluated by CT and MRI scanning, the tumor was partially resected. RESULTS: Postoperative microscopic examination of the tumor showed adipose cells with mature nuclei and well-formed fat vacuoles interspersed with fibrovascular septa, a finding consistent with maturing lipoblastoma. CONCLUSIONS: Although lipoblastomatous tumors are treated with complete surgical resection when possible, location of these tumors in the neck may dictate partial resection to avoid intraoperative injury to the spinal cord.- - - - - - - - - - ranking = 0.625keywords = lipoblastoma (Clic here for more details about this article) |
3/7. lipoblastoma in a 23-year-old male: distinction from atypical lipomatous tumor using cytogenetic and fluorescence in-situ hybridization analysis.lipoblastoma is a rare benign tumor that occurs primarily in infancy and early childhood. We present the rare presentation of a 12 cm sized intramuscular lipoblastoma of the thigh in a 23-year-old patient. On histology, the tumor strongly resembled an atypical lipomatous tumor due to the presence of lipoblasts and atypical stromal nuclei. The very focal lobulation and myxoid change presented the only histological hint towards a lipoblastoma. Cytogenetic and subsequent FISH evaluation of the tumor cells showed a 46,XY, t(8;15) (q12;q25) as chromosomal change with rearrangement of the PLAG1 gene. The present case indicates that lipoblastoma should enter the differential diagnosis of an 'atypical' deep seated fatty tumor in adults. The diagnostic value of cytogenetic/molecular analysis in the differential diagnosis of lipomatous tumors is underscored as well.- - - - - - - - - - ranking = 0.375keywords = lipoblastoma (Clic here for more details about this article) |
4/7. lipoblastoma in an 11-month-old infant.lipoblastoma and lipoblastomatosis are rare benign mesenchymal tumors of embryonal white fat, which normally affect children under 3 years. We report an 11-month-old boy who was presented with a painless lump in the suprapubic region, which was diagnosed on ultrasound as herniated omental fat or lipoma.- - - - - - - - - - ranking = 0.125keywords = lipoblastoma (Clic here for more details about this article) |
5/7. Recurrent maturing perineal lipoblastoma.lipoblastoma is a rare benign neoplasm of fetal adipose tissue that we see mostly in infants and young children less than 3 years of age. Most lipoblastomas occur on the extremities, trunk, head and neck, and various other organs have been described. We report a case of a recurrent perineal lipoblastoma in a 2.5-year-old boy, which showed maturation of the lipoblasts as compared to the primary tumor.- - - - - - - - - - ranking = 0.75keywords = lipoblastoma (Clic here for more details about this article) |
6/7. Fibrous lipoblastoma with 8q11.2 abnormality.A 3-month-old African American female infant had a rapidly growing lipoblastoma with a prominent fibrous component in the soft tissue of the left lateral knee, which recurred at 10 months. cytogenetic analysis revealed deletion of 8(q11.2q13) with a 19(q12q13.3) insertion at that site, confirming that this is closely related to the conventional lipoblastoma. The presence of multivacuolated lipoblasts and the staining characteristics (no staining for CD99, CD34, or smooth muscle actin) distinguish this from the recently described lipofibromatosis.- - - - - - - - - - ranking = 0.75keywords = lipoblastoma (Clic here for more details about this article) |
7/7. lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature.OBJECTIVES: To review the experience at a children's hospital of lipoblastoma and liposarcoma and to identify any factors that would differentiate one type of tumour from the other. DESIGN: A retrospective case series. SETTING: british columbia's Children's Hospital a tertiary-care pediatric centre. patients: All patients with a pathological diagnosis of lipoblastoma and liposarcoma recorded over 12 years. MAIN OUTCOME MEASURES: The frequency of lipoblastoma and liposarcoma, identified from biopsy specimens of pediatric adipose tumours. The clinical, pathological and cytogenetic variables between lipoblastoma and liposarcoma. RESULTS: One hundred and forty-nine adipose tumours were recorded. Seven (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas. All tumours presented as asymptomatic, slow-growing, soft-tissue masses. The children with lipoblastoma tended to be younger, but 29% were over 3 years of age. The liposarcoma patients were aged 9 and 14 years. One liposarcoma was of myxoid type and the other was a round cell variant. Karyotypes were reported for 1 lipoblastoma and 1 liposarcoma. The myxoid liposarcoma karyotype was 46,XY,t(12;16)(q13;p11), and the lipoblastoma was reported as 46,XY,der(8)?t(8q;?), mar. CONCLUSIONS: lipoblastoma is an unusual childhood neoplasm and liposarcoma is very rare in children. Both tumours may present in a similar fashion, and differentiating them histologically can be difficult. Age cannot be relied upon to accurately predict their behaviour. The tumour karyotype is very helpful in differentiating these neoplasms.- - - - - - - - - - ranking = 1keywords = lipoblastoma (Clic here for more details about this article) |