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At least 235 records · Page 13Linked to original sources
PubMed1995

Deletion 6q in three cases of mixed-type liposarcoma in addition to t(12;16)(q13;p11)

We report the cytogenetic findings in three mixed liposarcoma following short-term cultures. During the course of cytogenetic investigation of various types of liposarcomas, we observed an interstitial deletion of the long arm of chromosome 6 together with the translocation (12;16)(q13;p11) in three tumors. Translocation (12;16) is associated with myxoid and mixed (myxoid/round cell) liposarcomas, although deletion of chromosome 6 has been observed in only a few of these tumors. Our findings suggest that del(6), as an additional change in myxoid liposarcoma, is probably related to tumor progression.

Adult↗
PubMed2006

Detection of TLS/FUS-CHOP fusion transcripts in a case of oral liposarcoma.

OBJECTIVE: The aim of this study was to detect the chromosomal translocation t(12;16)(q13;p11) that leads to a gene fusion encoding a FUS-CHOP chimeric protein and has been shown to be highly characteristic of myxoid and round cell subtypes of liposarcoma, in a case of oral myxoid liposarcoma. METHOD AND MATERIALS: Nested reverse transcriptase-polymerase chain reaction to detect the TLS/FUS-CHOP fusion gene transcript was performed. A case of inflammatory fibrous hyperplasia and a case of oral lipoma were included as negative controls. RESULTS: Only the myxoid oral liposarcoma showed a 103-base pair product, specific of TLS/FUS-CHOP fusion type II transcript. CONCLUSION: The identification of FUS-CHOP transcript is potentially useful in the diagnosis and research of oral liposarcomas.

Chromosomes, Human, Pair 12↗
PubMed2006

Conservative surgery for well-differentiated liposarcomas of the extremities adjacent to major neurovascular structures.

BACKGROUND: There is no clear consensus as to the appropriate nomenclature and the best surgical strategy for well-differentiated liposarcomas. A wide surgical excision is recommended over marginal resection for local control of well-differentiated liposarcomas. However, this surgical procedure should be reconsidered for lesions, which come into contact with major neurovascular structures. METHODS: Between 1998 and 2004, 12 well-differentiated liposarcomas of the extremities were treated at our institute. Among them, nine lesions (75%) adjacent to major nerves or blood vessels were marginally resected with careful dissection of these critical structures. The clinical outcomes were reviewed for a mean follow-up time of 4 years. RESULTS: Seven patients continued to be disease-free without any loss of limb function. One patient developed a local recurrence 30 months postoperatively. The other patient with a foot lesion involving the fourth metatarsal bone has experienced slight difficulty in gait-balancing due to the fourth toe amputation, but remains free of disease. The local control rate was 88.9%, even though seven lesions were resected with positive surgical margins. CONCLUSIONS: Our findings suggested that well-differentiated liposarcomas frequently arise in close proximity to major nerves or blood vessels. Marginal resection alone seems to be adequate for these lesions to preserve critical structures.

Adult↗
PubMed1997

Metastatic liposarcoma to the orbit.

PURPOSE: To report a woman with blurred vision and proptosis who harbored an orbital liposarcoma that had metastasized from her abdomen. METHODS: The patient underwent an orbital biopsy, followed by postoperative intravenous corticosteroids and radiation. RESULTS: Pathology showed a dedifferentiated liposarcoma. The patient's visual acuity improved from 20/200 to 20/50 after treatment. CONCLUSIONS: Physicians should suspect the presence of this rare orbital tumor in a person with a history of liposarcoma who has proptosis. Liposarcomas usually exhibit a higher grade of malignancy with recurrence, as shown in this patient.

Abdominal Neoplasms↗
PubMed2005

[Liposarcomas of the mediastinum in atypical locations: report of two cases].

Liposarcomas of the mediastinum are unusual tumors characterized by compression of neighboring structures and frequent recurrence. Computed tomography and magnetic resonance imaging provide useful data for diagnosis. According to a review of the literature, surgical resection is the most effective treatment. We report two cases of mediastinal liposarcoma: the first was a liposarcoma that was a recurrence of an atypical lipoma of the neck, and the second was a giant mediastinal liposarcoma involving both hemithoraces.

Humans↗
PubMed2003

Liposarcoma presenting as an intraluminal ureteral mass.

An 86-year-old woman with a previous excision of a large retroperitoneal low-grade liposarcoma presented with a 4-month history of persistent left flank discomfort. Workup revealed left hydronephrosis secondary to obstruction, which was caused by a recurrent liposarcoma in the intraluminal ureter. She underwent a left nephroureterectomy for symptomatic palliation. Pathologic study revealed high-grade liposarcoma. Despite negative surgical margins, the tumor rapidly recurred, leading to death only 4 months after surgery. This case highlights the aggressive nature of liposarcoma.

Aged↗
PubMed2003

Primary retroperitoneal liposarcoma.

A retroperitoneal liposarcoma that marginally involved the right kidney and ovary was presented clinically as an adnexial mass in a 61-year-old woman. At laparatomy a large retroperitoneal mass in addition to the right kidney and ovary was removed totally. A well-differentiated retroperitoneal liposarcoma was detected in histological speciment. Adjuvant irradiation was given. The 5-year survival rate of retroperitoneal liposarcoma is low. Curative resection remains the main treatment for primary and recurrent liposarcomas.

Adnexal Diseases↗
PubMed1996

Retroperitoneal liposarcoma in two siblings.

We report here on two cases of retroperitoneal liposarcoma which heterochronously occurred in two siblings. A huge myxoid liposarcoma, 20 x 14 cm size, was noticed in a 33-year-old female, who died with multiple liver metastasis in about half a year. Two years later after the death of the younger sister, pleomorphic liposarcoma, 8 x 8 cm in size, was noticed in a 39-year-old male who died with local recurrence and multiple metastasis one year after the operation. Their mother (69 years old) had had an operation for malignant fibrous histiocytoma of the right thigh at age 66. There was no evidence of multiple lipomatosis, an autosomal dominant trait, in the siblings or their family. Diverse soft-tissue sarcomas, but not liposarcoma, occur excessively in siblings with the syndrome of Li-Fraumeni. To our knowledge this is the first such report of its sibship occurrence.

Adult↗
PubMed2002

RNA splicing mediated by YB-1 is inhibited by TLS/CHOP in human myxoid liposarcoma cells.

Human myxoid liposarcoma contains a characteristic t(12;16) chromosomal translocation that results in fusion of the N-terminal domain of the translocated in liposarcoma (TLS) protein to the C/EBP homologous protein (CHOP). TLS possesses structural motifs that suggest it may participate in RNA processing. We demonstrate that in human myxoid liposarcoma cells, wild-type TLS binds to RNA polymerase II (Pol II) via its N-terminal domain and to the transcription and translation factor Y-box binding protein-1 (YB-1) through its C-terminal domain. The liposarcoma fusion protein TLS/CHOP retains the ability to bind RNA Pol II but lacks the ability to recruit YB-1 due to replacement of the C-terminal domain of TLS by CHOP. In an in vivo splicing assay, YB-1 promotes splicing of adenovirus EIA pre-mRNA predominantly to the 13S isoform. The oncogenic TLS/CHOP fusion protein inhibits this splicing function of YB-1 in a dominant negative manner. When considered in conjunction with studies on other sarcoma fusion proteins, these data suggest that aberrant RNA splicing may be a common feature of human sarcomas.

Adenovirus E1A Proteins↗
PubMed2000

Medical management of unresectable, recurrent low-grade retroperitoneal liposarcoma: integration of cytotoxic and non-cytotoxic therapies into multimodality care.

Liposarcomas of the retroperitoneum are rare tumors, and best managed by an expert multidisciplinary team consisting of a surgical oncologist with appropriate medical oncology and radiation oncology collaboration. For large tumors, surgical excision with wide margins is difficult to achieve, and even wide margins cannot ensure that microscopic remnants of residual disease will not grow back in the future. For these reasons, even following expert resection, local recurrence is common. For patients with recurrent and unresectable liposarcoma, treating the sarcoma while maintaining quality of life becomes the major therapeutic goal. Importantly, patients with advanced recurrent disease demonstrate the need for multidisciplinary team involvement, with timely consideration of palliative surgical, radiation therapy, and chemotherapy options. Such patients also represent ideal candidates for investigational approaches aimed at identifying new agents with which to treat this disease. In addition to the development of new cytotoxic agents, patients may be candidates for novel strategies such as differentiation therapies or anti-angiogenic approaches. The recent explosion of knowledge regarding the cytogenetics, molecular, and cellular biology of liposarcomas allows us to remain positive that new translational therapies will be developed to improve the clinical outcomes of patients with these diseases. Current strategies, such as the use of PPARgamma ligands to differentiate liposarcomas, will soon be tested in major national collaborative trials, and the cooperation of surgeons and medical oncologists at all levels of community and academic practice will be crucial to obtain answers in this field. This review will summarize an illustrative case in the process of describing the natural history and potential interventions which should be considered for patients with this disease.

Chromans↗
PubMed1995

Pleomorphic liposarcoma of the pterygomandibular space involving the maxilla.

Liposarcoma of the head and neck is rare. Because of its rarity, we report this case of pleomorphic liposarcoma of the left pterygomandibular space, involving the maxilla, maxillary sinus, left lateral maxillary wall, left maxillary gingiva with extension into the buccal space, retro-orbital region, left mandibular ramus, and left zygomatic process. To our knowledge, there are only two other reported cases of liposarcoma involving the maxilla, one of which was a pleomorphic liposarcoma. The pertinent literature is reviewed with an emphasis on the importance of histopathologic classification.

Adolescent↗
PubMed2006

Myxoid liposarcoma metastatic to the thyroid gland: a case report and literature review.

We present the second reported case of a myxoid liposarcoma metastatic to the thyroid gland in a 51-year-old gentleman with previous liposarcoma of the right thigh. Myxoid liposarcoma has a relatively good prognosis but tends to recur locally. Metastases affecting the thyroid gland are a rare entity and most commonly arise from the kidney, lung or breast. Clinical presentation, patterns of recurrence and prognosis of myxoid liposarcoma and metastases to the thyroid gland are discussed.

Antineoplastic Agents↗
PubMed2001

[Monstrous, retroperitoneal liposarcoma--a case report].

Liposarcomas comprise about 20% of soft tissue sarcomas and occur in 14% in the retroperitoneal space originating in one third from the perirenal fat. The case of a patient with an extraordinary huge, resectable, well-differentiated retroperitoneal liposarcoma is reported. The presenting symptoms were abdominal pain and distension as well as weight gain. Magneticresonance imaging revealed a huge retroperitoneal tumor suspected of well-differentiated liposarcoma. Curative resection of the tumor could be obtained. Only 50% of all tumors are excised without residual tumor and recurrence, occurring in 90% after 10 years, is the main therapeutic challenge. Since neoadjuvant and adjuvant therapy as well as intraoperative radiotherapy failed to prove prognostic value, curative resection remains the main treatment for primary and recurrent liposarcomas.

Adipose Tissue↗
PubMed2005

Alterations of the RB1 gene in dedifferentiated liposarcoma.

Dedifferentiated liposarcoma is a malignant adipocytic neoplasm containing a non-lipogenic sarcoma of variable histological grade that arises against the background of a pre-existing well-differentiated liposarcoma. The phenomenon of dedifferentiation is considered to be time-dependent, but the mechanism is not well known. The retinoblastoma protein, encoded by the RB1 gene located at 13q14, is a key regulator of proliferation, development, and differentiation of certain cell types, including adipocytes. In the current study, we investigated the genetic alterations of the RB1 gene, such as mutation (the essential promoter region and the protein-binding pocket domain; exons 20-24) and methylation of the promoter region, in addition to pRB expression and loss of heterozygosity (LOH) status, in two morphologically distinct areas (non-lipogenic dedifferentiated and well-differentiated components) in 27 patients. As a control, 11 undifferentiated high-grade pleomorphic sarcoma/pleomorphic malignant fibrous histiocytoma samples and 11 well-differentiated liposarcoma samples were also evaluated. Dedifferentiated components showed LOH (15/25; 60%) and abnormal retinoblastoma protein expression (18/27; 66.7%) more frequently than noted in the well-differentiated components (3/24; 12.5% and 9/27; 33.3%, respectively). Five and four out of the 27 dedifferentiated components harbored mutations and promoter methylation, respectively, whereas none of these alterations were seen in the well-differentiated components. These results suggest that retinoblastoma protein has a major role to play in dedifferentiation and that a 'two-hit' mechanism is involved in the altered retinoblastoma protein expression in dedifferentiated liposarcoma.

Adult↗
PubMed2001

Pleomorphic liposarcoma: a clinicopathologic analysis of 19 cases.

Pleomorphic liposarcoma is a variant of liposarcoma defined morphologically by the presence of pleomorphic lipoblasts. Because of its rarity, there are limited studies with long-term follow-up information. Nineteen pleomorphic liposarcomas were studied. Unequivocal pleomorphic lipoblasts were required for inclusion. In each case, the following features were noted: tumor site; tumor size; tumor depth; predominant histologic pattern (epithelioid or malignant fibrous histiocytoma [MFH]-like); extent of necrosis (absent, less than 15%, or at least 15%); mitotic counts; treatment and clinical follow-up. Patients were 11 females and 8 males, aged 33-87 years (mean, 64.5 y; median, 70 y). Tumors involved the extremities (13 patients: intramuscular in 10, subcutaneous in 2, depth unknown in 1), retroperitoneum (4 patients), mediastinum (1 patient), and paratesticular region (1 patient). Size ranged from 4.5--31 cm (mean, 11.9 cm; median, 12.0 cm). Predominant pattern was epithelioid in 7 and MFH-like in 12. Necrosis was present in 15 (79%) and was extensive (36 15%) in 14 patients. Mitotic counts ranged from 0.2--3.4/10 high-power fields (mean, 1.4; median, 1.4) by the average-count method and from 1--6/10 high power fields by the highest count method (mean, 2.9; median, 3.0). All patients were treated surgically; 10 patients received adjuvant chemotherapy and/or radiation therapy. On follow-up of 18 patients (range, 2--129 mo; mean, 35.4 mo; median, 23 mo) nine (50%) were dead of disease (range, 2--48 mo; mean, 20.1 mo; median, 12 mo), one died of other causes 2 months after diagnosis, two were alive with disease, five were disease free, and one was alive at 129 months (tumor status unknown). Five had recurrences (range, 3--28 mo; mean, 14.4 mo; median, 8 mo), and four of five (80%) with recurrences were dead of disease. Metastases developed in eight patients (range, 4--48 mo; mean, 19.5 mo; median, 11.5 mo), most commonly to the lungs. In conclusion, pleomorphic liposarcoma is a rare tumor of adulthood that occurs most commonly in the deep, soft tissues of the extremities. It behaves as a high-grade sarcoma that frequently metastasizes, most commonly to the lungs. Although this tumor has a wide range of histologic appearances, no clinical or pathologic feature is predictive of a more aggressive clinical course.

Adult↗
PubMedDate not supplied

Mixed myxoid/round cell liposarcoma of the scalp.

Liposarcoma of the head and neck is rare. Only 12 cases of scalp liposarcoma have been previously reported. In this report, we describe a case of myxoid/round cell liposarcoma in the scalp of a 28-year-old woman. This case report highlights a histologic pattern rarely reported in the head and neck but consistent with the evolving classification of liposarcomas.

Adult↗
PubMed2000

Dedifferentiated liposarcoma of retroperitoneum and mesentery: varied growth patterns and histological grades--a clinicopathologic study of 32 cases.

The purpose of this study was to obtain further information regarding cellular differentiation and proliferative characteristics of dedifferentiated liposarcoma (DDL) arising in the retroperitoneum and mesentery for accurate diagnosis and prognostic criteria. The patients included 20 men and 12 women, mean age, 60 years (range, 33 to 80 years). Twenty-seven tumors were located in the retroperitoneum and 5 in the mesentery. Tumor size ranged from 9 to 51 cm (mean, 24 cm). Follow-up was available on all patients and ranged from 4 to 243 months (mean, 64 months). Twenty-four (75%) patients developed local recurrences, 3 (9%) had distant metastasis, and 16 (50%) died of the disease. The predominant histology of dedifferentiation (DD) included fibrosarcoma or malignant fibrous histiocytoma (MFH) in 15 (47%), myxofibrosarcoma (myxoid MFH) in 5 (16%), mixed type in 10 (31%), and a whorling pattern in 2 (6%). Divergent differentiation, such as osseous, osteosarcomatous, chondrosarcomatous, and leiomyosarcomatous, was observed in 9 (28%). Immunoreactivity for vimentin, desmin, CD34, neurofilament, alpha-smooth muscle actin, p53, and MDM2 was observed in 32 (100%), 14 (44%), 8 (25%), 13 (41%), 14 (44%), 19 (59%), and 18 (56%) of DD areas, respectively. On the basis of a histological grading using MIB-1 (MIB-1 index range, 3% to 80%; mean, 27%) to replace mitosis counts (1 to 35/10 high-power fields [HPF]; mean, 13/10 HPF), 16 tumors each were classified as low-grade (grade 2) and high-grade (grade 3). The mixed type with poorly differentiated areas including scattered lipoblasts could be mistaken for myxoid liposarcoma but lacked the C/EBP-homologous protein-translocated in liposarcomat (CHOP-TLS) fusion genes specific for myxoid liposarcoma. Period to the first recurrence and histological grade using the MIB-1 index were associated with overall survival. Identification of DD areas, especially a poorly recognized variant of the mixed type, careful follow-up to detect early recurrence, and histological malignancy grading combined with proliferation indices are important in providing an accurate prognosis for all patients with retroperitoneal and mesenteric liposarcoma.

Adult↗
PubMed2000

[Lipoma-like liposarcoma of the tongue].

BACKGROUND: Liposarcomas of the tongue are rare. To date only 7 cases have been reported. PATIENT: We report a new case of a well differentiated lipoma-like liposarcoma of the body of the tongue in a 71-year old woman. Complete uneventful resection was performed. Histologically the tumor consisted of fat cells with occasional lipoblasts and spindle cell areas. There is no sign of recurrence of the tumor 2 years later. RESULTS AND CONCLUSIONS: Liposarcomas of the tongue are rare neoplasms. All cases reported were histologically well differentiated. Liposarcomas have to be considered in the differential diagnosis of solid masses of the tongue.

Aged↗