Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Lipoblastoma”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources
PubMed2001

Case of lipoblastoma with two derivative chromosomes 8 containing homogeneously staining-like regions and a review of the literature: lipoblastoma and chromosome 8.

We report a case of a lipoblastoma in a 10-month-old girl in which the cytogenetic aberration showed a homogeneously staining-like region (hsr) within two derivative chromosomes 8. There was a loss of one normal copy of chromosome 8 and gain of two identical derivative chromosomes 8 with the karyotype designation 47,XX,psu idic(8)(pter-->q12 approximately 13::hsr::q12 approximately 13-->pter),+psu idic (8)(pter-->q12 approximately 13::hsr::q12 approximately 13-->pter). This is the first report of a chromosomal aberration of this type seen in lipoblastoma.

Chromosome Banding↗
PubMed2001

PLAG1 alterations in lipoblastoma: involvement in varied mesenchymal cell types and evidence for alternative oncogenic mechanisms.

Lipoblastomas are rare soft tissue tumors that occur primarily in young children. They typically contain variably differentiated adipocytes, primitive mesenchymal cells, myxoid matrix, and fibrous trabeculae. Abnormalities in chromosome 8, leading to rearrangements of the PLAG1 gene, were demonstrated recently in four lipoblastomas. In the present report, we determine the frequency of PLAG1 alterations in 16 lipoblastomas from children aged 13 years or younger, and we also evaluate the stages of lipoblastoma differentiation at which PLAG1 genomic alterations are found. Eleven lipoblastomas (69%), including those with either classic or lipoma-like histology, had rearrangements of the 8q12 PLAG1 region. Another three lipoblastomas had polysomy for chromosome 8 in the absence of PLAG1 rearrangement. Only two cases (13%) lacked a chromosome 8 abnormality. Notably, the lipoblastomas with chromosome 8 polysomy had up to five copies of chromosome 8 as an isolated cytogenetic finding in an otherwise diploid cell. We also demonstrate that PLAG1 alterations are found in a spectrum of mesenchymal cell types in lipoblastomas, including lipoblasts, mature adipocytes, primitive mesenchymal cells, and fibroblast-like cells. This finding is consistent with neoplastic origin in a primitive mesenchymal precursor and with variable differentiation to a mature adipocyte end-point. Hence, our studies provide biological validation for the clinical observation that lipoblastomas can evolve into mature, lipoma-like, lesions. They also suggest that PLAG1 dosage alterations caused by polysomy 8 might represent an alternative oncogenic mechanism in lipoblastoma.

Child↗
PubMed1998

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.

Adolescent↗
PubMedDate not supplied

Lipoblastoma of infancy mimicking hemangioma of infancy.

Lipoblastomas are rare benign tumors of infancy that usually affect children younger than 3 years. Most lipoblastomas (70%) occur on the extremities. Lipoblastomas may mimic other infantile tumors, including hemangiomas, hibernomas, lipomas, and liposarcomas, and correct diagnosis is necessary to ensure appropriate treatment. Lipoblastomas fall under 2 discrete subtypes: well-circumscribed lipoblastomas and diffuse lipoblastomatosis. Both types present with firm, nontender masses of lobulated, well-circumscribed soft tissue. Histologically they can be highly vascularized with plexiform capillaries, often with an individual feeder artery to each lobule. Complete surgical removal is the recommended treatment. Only 2 cases of lipoblastomas of the cheek have been reported in the English-language literature. We present the case of a young child with a cheek lipoblastoma, emphasizing the importance of correct diagnosis and highlighting techniques used to provide suitable treatment.

Biopsy, Needle↗
PubMed2005

Cytological features of lipoblastoma: a report of three cases.

Lipoblastoma is a rare benign neoplasm occurring mostly in children under the age of three. Accurate preoperative diagnosis is mandatory for planning the treatment. The main aim of this study is to establish the cytological features of lipoblastoma and to answer the question: "Is cytological diagnosis of lipoblastoma reliable?"Preoperative fine-needle aspiration biopsy (FNAB) and tissue sections of three children treated for lipoblastoma at the Division of Pediatrics, University Medical Center, Ljubljana, Slovenia, in the period from 1997 to 2004 were reexamined. The Giemsa- and Papanicolaou-stained FNAB smears were moderately or poorly cellular and contained lipocytes, lipoblasts, and spindle cells in various proportions. The tumor cells were in clusters and tissue fragments or as single cells. Thin branching capillaries were observed in most of the clusters and tissue fragments. In the background, abundant myxoid extracellular material and naked oval nuclei were present. In the first case, the cytological diagnosis was benign soft-tissue tumor, in the second the diagnosis was not conclusive and the last case was correctly diagnosed as lipoblastoma. In the differential diagnosis of the second case, both lipoblastoma and liposarcoma were considered cytologically as well as histologically. At 7-yr follow-up, there is no evidence of the disease.Thus, we conclude that lipoblastoma with typical cytological features could be accurately diagnosed by FNAB. However, tumors containing numerous lipoblasts could pose a diagnostic problem.

Biopsy, Fine-Needle↗
PubMed2004

Chest wall lipoblastoma in a seven-month-old girl: a case report.

Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infanthood and early childhood. It is typically located in the extremities and the trunk and less frequently in the head-neck region, mediastinum, and retroperitoneum. However, cases of pleural, thoracic wall, and pulmonary lipoblastoma have also been reported in the literature. Lipoblastoma is a tumor with good prognosis despite its potential for local invasion and rapid growth. Our patient is a 7-month-old girl who was brought to our clinic for a rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the mass was removed by thoracic wall resection and the resultant defect covered with an absorbable polyglactine mesh. The excised mass was diagnosed pathologically as a benign lipoblastoma. In the postoperative period, the thorax wall was stable, and after 24 months of follow-up no recurrence was observed. In the literature, 4 types of thoracic wall lipoblastomas have been reported, but thoracic wall resection has not been applied in any benign lipoblastoma case. To the best of our knowledge, the only benign lipoblastoma case in which a thoracic wall resection was carried out, with its clinical and histopathologic specifications alongside that in the literature, is presented here for the first time.

Female↗
PubMed2001

Lipoblastoma: a rare cervical mass in childhood.

A 4-year-old girl had a left side cervical swelling for three months. The mass was free from surrounding tissues and excised completely. Histopathologic diagnosis of the specimen was lipoblastoma. Cervical lipoblastoma is a rapidly growing neoplasm. Only two cases of cervical lipoblastomas caused airway obstruction have been reported. Recurrence is rare in cases of lipoblastoma. Complete excision of the tumor is the treatment of choice. Seventeen cases of cervical lipoblastoma have been reported until now in the literature, but we think that lipoblastoma is more frequent than reported. Lipoblastoma must be remembered as a rare cause of cervical masses in childhood.

Child, Preschool↗
PubMed2006

A thoracic wall lipoblastoma in a 3-month-old infant: A case report and review of the literature.

Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infancy and early childhood. Lipoblastoma is present in 2 forms which are pathologically identical: circumscribed and diffuse. It is typically located in the extremities, and less frequently in head-neck region, trunk, and various organs. Lipoblastoma is a tumor with good prognosis with no reported metastases, despite its potential for local invasion and rapid growth. Our patient was a 3-month-old boy who was brought to our clinic for rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the lipomatous mass was removed by local resection. In addition to the patient's age, histologic and cytogenetic analyses assisted the diagnosis of diffuse lipoblastoma. In the postoperative period, the thorax wall was unaffected, and after 6 years of follow-up no recurrence was observed. In the English literature, 8 cases of thoracic wall lipoblastoma have been previously reported, and only 3 of diffuse form. Here, we report, at our knowledge, the fourth case of thoracic diffuse lipoblastoma, in which cytogenetic analysis showed a previously undescribed karyotype aberration involving chromosomes 8, 13, and 16.

Chromosome Aberrations↗
PubMed2006

Radiological-pathological correlation in lipoblastoma and lipoblastomatosis.

BACKGROUND: Lipoblastoma and lipoblastomatosis are uncommon benign mesenchymal lesions that predominantly occur in infancy and early childhood. OBJECTIVE: To evaluate the imaging and histological features of lipoblastoma and lipoblastomatosis. MATERIALS AND METHODS: Retrospective review of the radiological and pathological findings in children with lipoblastoma and lipoblastomatosis treated at a single centre between 1997 and 2004. RESULTS: Eight children (median age 18 months) had undergone imaging and surgery at our institution. An infiltrative growth pattern was identified at imaging in two children with lipoblastomatosis, and a well-defined mass in six children with lipoblastoma. In all patients, imaging showed a lesion composed mostly, but not entirely, of fat. There were no recurrences at follow-up of between 1 and 91 months. CONCLUSION: In infancy and early childhood, the identification of a tumour composed mostly of fat should suggest the diagnosis of lipoblastoma or lipoblastomatosis.

Arm↗
PubMed2002

Atypical lipomatous tumor in a 14-year-old patient: distinction from lipoblastoma using FISH analysis.

Liposarcomas are rare in young age. We present the rare case of an atypical lipomatous tumor (synonym: well-differentiated lipoma-like liposarcoma) in a 14-year-old girl with the differential diagnosis of lipoblastoma which was excluded by fluorescence in situ hybridization (FISH) analysis. The tumor presented as a soft tissue mass at the dorsal part of the right thigh measuring up to 18 cm. Microscopically the lesion consisted of atypical adipocytes with hyperchromatic nuclei and additional multivacuolated lipoblasts. Interphase dual-color FISH performed with chromosome 8 centromeric and YAC164H5 (mapping to exons 2-5 of the PLAG1 gene) probes revealed no rearrangement of PLAG1 oncogene or polysomy of chromosome 8. Additional FISH using an MDM2 gene probe and an BAC534N15 probe (containing sequences specific for the CDK4 gene) showed amplification of the CDK4 gene. These findings indicate that this tumor was no lipoblastoma but an atypical lipomatous tumor, which is of clinical relevance. In young individuals the distinction between lipoblastoma and liposarcoma is often impossible by light microscopy alone. This case shows that FISH can serve as a decisive tool in the differential diagnosis of lipoblastoma and lipoma-like liposarcoma apart from its role in distinction between lipoblastoma and myxoid/round cell liposarcoma.

Adolescent↗
PubMed2005

Lipoblastoma of the neck: a rare cause of respiratory problems in children.

Lipoblastomatous tumours are rare, and they occur primarily in children younger than 3 years of age. They are benign and may be divided in lipoblastomas and lipoblastomatosis. A case with cervical lipoblastoma causing respiratory difficulty is reported, and a clinical characterisation of patients with lipoblastomatous tumours in the neck is presented. A 6-year-old boy with complains of stridorous respiration and significant reduction in physical capacity was referred to the ENT Department, Odense University Hospital, Denmark. He was treated with total surgical resection of a soft and slowly growing tumour in the left side of the neck, extending from the base of the skull to the upper part of the mediastinum. The histological examination showed a lipoblastoma. After surgery all symptoms disappeared, and the patient was without any operative sequelae. Including the actual case, a review of English literature resulted in the identification of 37 patients with cervical lipoblastoma or lipoblastomatosis. However, in most cases the information was sparse, and only 13 patients were eligible for an analysis of basic clinical and demographic data. The median age was 25 months (range: 7-75 months), and the median tumour size 9 cm (range: 3-18 cm). No difference in sex distribution was found. Lipoblastomas (85%) seemed more frequent than lipoblastomatosis (15%). Stridorous respiration was present in 31%. It is concluded that a considerable part of lipoblastomatous tumours in the neck are combined with respiratory difficulties (31%), and that complete but gentle surgical treatment in most cases will restore normal physiological conditions.

Child↗
PubMed2001

Lipoblastoma.

BACKGROUND/PURPOSE: Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat that occurs almost exclusively in infants and children. This study was done to determine the clinical and pathologic characteristics of lipoblastoma. METHODS: Nine cases of pathologically proven lipoblastoma from 1979 to 1997 were reviewed. There were 6 boys and 3 girls ranging in age from 3 months to 29 months. RESULTS: A soft tissue mass was the chief complaint in 7 patients, abdominal distension in 1 patient with a retroperitoneal mass, and defecation difficulty in 1 patient with a perirectal mass. In 4 patients, tumors occurred on the back. Other tumor location includes the neck, scrotum, retroperitoneum, perirectal area, and buttock in 1 patient each. Lesions measured 2.3 to 19.5 cm. Complete excision was done in 8 patients. One perirectal tumor was removed by both the posterior sagittal approach and the intraabdominal approach but incompletely resected. Two tumors located on the back recurred with intraspinal extension 12 months and 18 months after resection. Second resection and second resection with laminectomy were done. Leg pain and urinary incontinence developed in 1 patient but improved on conservative treatment. CONCLUSIONS: Lipoblastoma is a benign neoplasm but can frequently recur (25%) in spite of complete excision. Lipoblastomas occurring on the back had a high recurrence rate (50%) and associated with intraspinal extension. J Pediatr Surg 36:905-907.

Child, Preschool↗
PubMed2004

[Imaging characteristics of childhood lipoblastoma].

PURPOSE: Lipoblastoma and lipoblastomatosis are very rare benign neoplasms, which almost exclusively occur in infants and young children. Despite their potential to local invasion and rapid growth, these tumors have an excellent prognosis, particularly if resected completely. Usually, the diagnosis is not taken into consideration preoperatively, and depends on histopathological evaluation. This study was done to determine imaging characteristics of lipoblastoma. MATERIALS AND METHODS: Eight cases of histopathologically proven lipoblastoma treated from 1988 to 2003 were reviewed, comprising four girls and four boys ranging in age from 17 months to nine years. The localization was chest wall (four times), abdominal wall (once), gluteal region (once), lower leg (once) and forearm (once). Ultrasound, MRI and CT scans were evaluated and correlated to clinical data of the patients. RESULTS: Ultrasound showed lipomatous echogenicity and echotexture. The tumors appeared signal intensive on T1-weighted MR images and had a mean intensive signal on T2-weighted MR images with fat suppression. They were markedly hypodense on CT. The growth pattern was invasive in all imaging methods, with extension into preformed spaces, such as intercostal spaces and neural foramina, but without infiltration into surrounding structures or metastases. CONCLUSION: Ultrasound and MRI are the methods of choice to diagnose lipoblastoma by revealing structures and local growth pattern that appear specific of this rare tumor entity. The imaging characteristics of the lipoblastoma have to be correlated with the age of patients to exclude other conditions in the differential diagnosis.

Child↗
PubMedDate not supplied

Lipoblastoma and lipoblastomatosis in infancy and childhood: histopathologic, ultrastructural, and cytogenetic features.

Lipoblastoma is a relatively rare tumor that occurs in infancy and early childhood and arises from embryonic white fat. Although a benign tumor, lipoblastomas tend to recur and may resemble myxoid liposarcoma. The authors report 26 cases over a 15-year period at Texas Children's Hospital. There was a slight female predilection (14F:12M). The most common symptom was a painless mass with or without increasing size. The trunk, extremities, head and neck, retroperitoneum, inguinal canal, peritoneal cavity, and lung were the tumor sites. Most tumors were circumscribed lipoblastomas and the minority were diffuse infiltrative lipoblastomatosis. Reexcision for residual or recurrent tumor was necessary more frequently in patients with lipoblastomatosis. Histopathologic examination and ultrastructural examination revealed cellular neoplasms composed of immature adipocytes with relatively well-defined septa, frequent lipoblasts, a fine vascular network, and often a myxoid appearance resembling myxoid liposarcoma. Cytogenetics was performed in 4 cases with chromosome 8q abnormality being most common. The major concern with lipoblastoma in children is to completely excise the tumor to avoid leaving residual tumor and to prevent recurrences. Confusion with myxoid liposarcoma, well-differentiated liposarcoma, and typical lipomas may occur. Although asymptomatic, lipoblastomas may cause dysfunction of other organ systems due to mass effect. Complete surgical excision with at least 2 years of follow-up is the preferred therapy.

Age Factors↗
PubMed1988

Benign lipoblastoma: first case report of a mesenteric origin.

A case of benign lipoblastoma of the mesentery, surgically resected in a 4-year-old boy, is reported, with data on its gross and histologic features. Both diffuse lipoblastomatosis and single benign lipoblastoma have been observed in different anatomical sites, mostly involving the subcutaneous tissue, and only sporadic cases of mediastinal or retroperitoneal lipoblastomas have been reported. The present case therefore appears to be the first description of a lipoblastoma arising from mesentery. The main tumoral lesions with which lipoblastoma can be confused are briefly discussed.

Child, Preschool↗
PubMed2004

Intrascrotal lipoblastoma with a complex karyotype: a case report and review of the literature.

Lipoblastoma is a tumor of adipose tissue that usually occurs in young children. Most lipoblastomas occur on the extremities, trunk, and head and neck, and most have rearrangements of the 8q region. We describe a lipoblastoma in a 12-month-old boy who presented with a rapidly enlarging scrotal mass. Electron microscopy revealed features consistent with immature adipocytes, and cytogenetic analysis revealed the following karyotype: 57,XY,+4,+6,+7,der(8)t(8;12) (q22;q13), +der(8)t(8;12) (q22;q13), +9,+10,+12,-16,+17,+der(18)t(8;18)(q22;q23),+19,+20. Interestingly, the breakpoint on chromosome 12 (q13) is the same as that seen in lipoblastomas. To our knowledge, this is the first reported case of such a complex karyotype in lipoblastoma and adds to the expanding list of karyotypic abnormalities seen in such tumors.

Aneuploidy↗
PubMed2000

PLAG1 fusion oncogenes in lipoblastoma.

Lipoblastomas are pediatric neoplasms resulting from transformation of adipocytes. These benign tumors are typically composed of adipose cells in different stages of maturation within a variably myxoid matrix, and they contain clonal rearrangements of chromosome band 8q12. Because lipoblastomas resemble embryonic adipose tissue, characterization of their transforming mechanisms might reveal biological pathways in physiological adipogenesis. Herein, we demonstrate that lipoblastoma chromosome 8q12 rearrangements bring about promoter-swapping events in the PLAG1 oncqgene. We show that the hyaluronic acid synthase 2 (HAS2) or collagen 1 alpha 2 (COL1A2) gene promoter regions are fused to the entire PLAG1 coding sequence in each of four lipoblastomas. PLAG1 is a developmentally regulated zinc finger gene whose tumorigenic function has been shown previously only in epithelial salivary gland cells. Our findings reveal that PLAG1 activation, presumably resulting from transcriptional up-regulation, is a central oncogenic event in lipoblastoma.

Adipose Tissue↗