Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lipoblastoma and lipoblastomatosis are rare examples of benign mesenchymal tumors that exhibit a tendency to invade locally but not to metastasize. The tumors are found primarily in children less than 5 years of age and can arise almost anywhere within the soft tissues. The rate of growth is variable and has no bearing on prognosis, which is excellent in the majority of cases. The experience at Childrens Hospital of Los Angeles with six children (3 males, 3 females) having these tumors is described. There was no instance of recurrence after surgical removal of the tumors, with follow-up periods ranging from 3 months to 5 years. Histologic appearance and outcome were similar in all patients, and there was no relationship between histologic appearance and prognosis. Complete local excision without radical mutilating resection is the treatment of choice.
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PMID:Lipoblastoma and lipoblastomatosis--a report of six cases. 341 88

Lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat with a tendency to local invasion but not to metastasize. To date, there have been 60 cases described in the international literature. There has never been a report in a child older than 8 yr. The main clinical characteristics are the presentation, usually below 3 yr of age, the rapid growth of the mass and the peripheral location, mainly in the extremities. Although CT scan may show a fatty tumor, there is no single test to make the differential diagnosis (which includes benign lipoma, liposarcoma and myxoliposarcoma) and the treatment should be based on clinical findings. We have treated four patients with this condition. Two were intrathoracic, one was intraperitoneal, and one was a tumor of the upper arm. All presented as rapidly-growing tumors and were clinically assumed to be malignant. We recommend complete but conservative excision of the tumor; there are reported recurrences after inadequate excision. This is a benign tumor and radical cancer surgery should be avoided.
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PMID:Lipoblastoma in infants and children. 710 15

Lipoblastoma and lipoblastomatosis are rare benign tumours of embryonal fat that exhibit a tendency to invade locally but not to metastasize. This condition most often presents before the age of 3 years, affects males more than females, and is typified by a slowly-growing, usually subcutaneous mass arising in a limb. Cases involving the head and neck are extremely rare, with only nine cases in the neck having been described to date. This tenth case arose in a 13-month-old male presenting with a rapidly enlarging lump in the right side of the neck, clinically mimicking a cystic hygroma. Diagnosis is by histology, and treatment involves complete surgical resection. We report this tenth known case of lipoblastoma in the neck and review the literature.
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PMID:Benign lipoblastoma arising in the neck. 956 56

Lipoblastoma is a rare adipose tumor occurring exclusively in childhood. There have been no reports of metastases, making the designation "blastoma" confusing, since this term is usually reserved for malignant tumors. Two recent cases treated at our institution confirm its benign nature. In addition, a review of the literature supports the idea that the tumor may more accurately be described as an "infantile lipoma". Infantile lipoma better reflects many of the tumor's characteristics such as, its early occurrence, it's ability to mature into a simple lipoma, it's cellular composition of mainly mature adipocytes, and its benign course. Although lipoblastoma is an uncommonly encountered tumor, making an effort to change its name to infantile lipoma will result in a more a accurate term that will facilitate treatment.
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PMID:Lipoblastoma: better termed infantile lipoma? 1095 94

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.
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PMID:A thoracic wall lipoblastoma in a 3-month-old infant: A case report and review of the literature. 1700 66

Lipoblastoma-like tumor of the vulva (LLTV) is an exceptionally rare adipocytic mesenchymal tumor with only 4 cases reported previously. The aim of this study is to help characterize this tumor type. Eight cases of LLTV were identified in the consult files of one of the authors (C.D.M.F.). Clinical data and follow-up information were obtained from the referring pathologists. Detailed clinical information is available in all 8 cases. Patient age ranged from 17 to 46 years (median 27 y). Lesions presented as a vulvar mass with a variable growth rate, sometimes being painful. The most common preoperative clinical diagnosis was a Bartholin gland abnormality (4). The size of the mass ranged from 3.5 to 15 cm (median 5.6 cm). The lesions were described as grossly myxoid, mucoid, or gelatinous (4), well defined (4), and lobulated (3). None of the lesions exhibited necrosis. Histologically, LLTVs were lobulated and composed of variable proportions of mature adipocytes, bland univacuolated and bivacuolated lipoblasts, and spindle cells with short stubby nuclei in a diffusely myxoid background with prominent branching vessels. Nuclear atypia was minimal. No necrosis and only rare mitotic activity was identified. Only 1 of 7 tumors (in a 26-y-old patient) was positive for PLAG1 and retinoblastoma (Rb). Rb was lost, and PLAG1 was not expressed in all other tumors. HMGA2 was negative in 6 of 6 cases tested. Tumors were negative for S100 (5/5), MDM2, and CDK4 (5/6; 1 with just scattered cells positive for both antibodies). Two of 5 cases were positive for CD34. Fluorescence in situ hybridization for DDIT3 gene rearrangement was negative in 5 cases tested. The follow-up interval ranged from 4 months to 11 years (median 2.75 y). Three patients developed 1 local recurrence, 7 months, 2 years, and 8 years, respectively, after excision of the primary tumor. None of the patients has developed metastatic disease. LLTVs are indolent adipocytic mesenchymal tumors arising in adults. Lack of PLAG1 and HMGA2 expression in the majority of LLTV suggests that these lesions are distinct from "true" lipoblastoma. The loss of Rb in the majority of cases suggests a possible role of 13q chromosomal alterations and a possible relationship with the spindle cell lipoma tumor family.
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PMID:Lipoblastoma-like tumor of the vulva: further characterization in 8 new cases. 2592 53

Lipoblastoma is a rare mesenchymal tumor, occuring in less than three-year-old infants in 90% of the cases and in 40% of the cases in chidren aged less than one year. It can be present at birth. It is a benign tumor, with defined margins, that can be locally aggressive, without metastases. We report the case of retroperitoneal lipoblastomatosis, with a neonatal diagnosis made by ultrasonographic and CT features.
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PMID:Rare retro peritoneal tumor in infants. 3043 Apr 70