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PubMed1999

Prognostic relevance of p53 alterations and Mib-1 proliferation index in subgroups of primary liposarcomas.

For prognostic analyses of p53 alterations (p53 gene mutations + p53 immunopositivity) and Mib-1 proliferation index, we investigated 42 primary malignant lipomatous tumors for which complete clinical data and a long follow-up were available. p53 gene mutations were investigated by PCR-single strand conformation polymorphism-sequencing analysis, and immunohistochemistry was used to determine p53 protein expression and Mib-1 proliferation index. We found a mutation frequency of 14.3%. Nine liposarcomas (21%) were p53 immunopositive, and 11 (26.2%) had at least one p53 alteration. In myxoid liposarcomas, p53 alterations are not relevant to the presence or absence of round cell components. Pleomorphic liposarcomas showed a significantly higher proliferation index and more p53 alterations than myxoid or well-differentiated variants (P<0.001). When the Cox's regression analysis tumors of grade III histology (P = 0.005) was performed, the pleomorphic subtype (P = 0.016) and liposarcomas of retroperitoneal localization (P = 0.015) showed a significantly poorer prognosis. Moreover, we found that p53 alterations and high proliferation index correlated significantly with reduced overall survival. Their prognostic value seemed to be higher in myxoid than in pleomorphic liposarcomas. The metastasis-free survival was reduced in patients who had liposarcomas with p53 alterations (P = 0.171) or elevated proliferation index (P<0.016), reflecting a more aggressive behavior. In conclusion, the determination of p53 alterations and/or Mib-1 proliferation index is useful for assessing the prognosis of patients with liposarcomas and may especially be helpful in dividing different prognostic groups for patients with myxoid variants.

Adolescent↗
PubMed1975

Liposarcoma a clinicopathologic, radiographic and prognostic study.

The present investigation is a clinicopathologic, radiologic and prognostic study of 122 liposarcomas registered in the Swedish Cancer Registry between 1958 and 1966. The incidence of liposarcoma in Sweden was estimated to be 2.5 cases per million inhabitants per year. The liposarcomas were divided into 4 main subtypes according to WHO's recommended classification (1969): well-differentiated (33 cases), myxoid (22 cases), rundcell (14 cases) and pleomorphic types (21 cases). When predominantly well-differentiated or myxoid liposarcomas contained areas of round-cell or pleomorphic types (24 and 8 cases, respectively), they were classified separately, thus adding 2 further subtypes to our classification. The validity of this histologic classification was emphasized by the prognostic study, based on a 10 year follow-up. The differential diagnosis for each of the 4 main subtypes is discussed. Three well-differentiated, lipoma-like liposarcomas which contained highly vascular hibernoma-like areas are described. Two well-differentiated liposarcomas situated in the neck and upper part of the back, respectively, of elderly patients included area similar to "spindle-cell lipoma" (Enzinger). An intracytoplasmic network of birefringent fibers with the staining reaction of collagen was found in 2 well-differentiated fibrosing liposarcomas...

Adolescent↗
PubMed2004

Intussusception secondary to metastasis from a low-grade retroperitoneal liposarcoma.

Liposarcomas are the most prevalent soft tissue sarcomas in adults. Low-grade liposarcomas are the most frequent and least aggressive and are noted as having a low risk of metastasis. We present a case of low-grade myxoid liposarcoma that metastasized to the small bowel resulting in intussusception. This case involves a 44-year-old woman with a recurrent retroperitoneal liposarcoma. Approximately 30 months after initial diagnosis, the patient was found to have a solitary metastasis to the small bowel that presented as a jejunal intussusception. Interestingly, there was no indication of a round cell component in either the metastasis or recurrent tumor, although the original tumor did contain a small round cell component. This is the first report in the English literature of liposarcoma metastasizing to the small bowel. We suggest that atypical presentations of liposarcoma metastases should always be considered in the management of patients with a history of liposarcoma.

Adult↗
PubMed2005

[Correlation between pathology and image characteristics of retroperitoneal liposarcoma].

OBJECTIVE: To investigate the correlation between the pathological and image features of different subtypes of retroperitoneal liposarcoma. METHODS: Pathological and image characteristics of 21 retroperitoneal liposarcomas were retrospectively analyzed comparing their different histopathological subtypes. RESULTS: This series consisted of 11 well-differentiated, 4 myxoid, 3 round-cell, 2 pleomorphic and 1 dedifferentiated liposarcoma. Well-differentiated liposarcomas were predominantly composed of lipoma-like and sclerosing components. On CT and MRI image, the attenuation and signal intensity of lipoma-like components resembled those of fat, whereas the signal intensity of sclerosing components was similar to those of muscle. Unenhanced CT image of the myxoid subtype showed density resembling that of water. On contrast-enhanced CT image, it showed gradual reticular or sheet-like enhancement. The CT and MRI image appearance of the round cell and pleomorphic subtypes resembled those of nonfatty soft tissue masses with foci of necrosis. The dedifferentiated liposarcoma showed the image feature of a well-differentiated component clearing enhanced soft tissue mass. CONCLUSION: Different subtypes of retroperitoneal liposarcoma show different CT and MRI features relating to their major histologic components. A good understanding of the relation between their radiological and pathological features is helpful to arrive at a correct diagnosis for retroperitoneal liposarcoma.

Adult↗
PubMed2006

[A case of retroperitoneal dedifferentiated liposarcoma initially diagnosed as malignant fibrous histiocytoma: a case report].

We report a case of retroperitoneal tumor which turned out to be liposarcoma by the histological evaluation of its recurrent tumor, although the initial tumor was diagnosed as malignant fibrous histiocytoma (MFH). A retroperitoneal tumor in a 62-year-old man was removed and pathologically diagnosed as MFH. Five years after the initial surgery, computed tomography (CT) demonstrated a recurrent tumor near the spleen. The tumor was resected together with the spleen, tail of pancreas, and connective tissue due to adhesion and diagnosed as well-differentiated liposarcoma with sclerosing component. Generally dedifferentiated liposarcoma is difficult to distinguish from MFH and the presence of a well-differentiated liposarcoma component in the adjacent adipose tissue leads to the diagnosis of dedifferentiated liposarcoma. The clinical course of the present case indicated that the initial tumor was dedifferentiated liposarcoma and the recurrent tumor developed from the surrounding well-differentiated liposarcoma.

Diagnosis, Differential↗
PubMed1997

Liposarcoma of soft tissue: MRI findings with pathologic correlation.

OBJECTIVE: To evaluate the MRI findings of liposarcomas of different histologic types and correlate these with the histopathologic features. DESIGN: The MR images of seven liposarcomas were reviewed retrospectively to assess the tumor size, location, margination, signal characteristics and enhancement patterns in different histologic types. PATIENTS: Seven liposarcomas comprising three well-differentiated, two myxoid and two pleomorphic types were evaluated. RESULTS AND CONCLUSION: All tumors showed well-defined and mostly lobulated margins. The well-differentiated liposarcomas were composed mainly of fat with septations or nodules, were hyperintense on T2-weighted images, and demonstrated faint enhancement or no enhancement following intravenous contrast. Myxoid liposarcomas were homogeneous or mildly heterogeneous and a pseudocapsule was present in one case. Pleomorphic types showed a markedly heterogeneous internal structure. Both myxoid and pleomorphic lesions-showed moderate or marked heterogeneous enhancement after contrast administration. Well-differentiated liposarcomas may be differentiated from other types of the tumor by their largely lipomatous appearance. The malignancy grade increases in parallel with tumor heterogeneity and contrast enhancement.

Adult↗
PubMedDate not supplied

Unusual spread of liposarcoma.

BACKGROUND: The aim of our study was to investigate the unusual pattern of metastatic spread of liposarcoma. MATERIALS AND METHODS: We retrospectively studied thirty-two patients with liposarcoma (in seventeen located in the retroperitoneum and in fifteen in the extremities) who were managed at our institution over a ten-year period. Six patients with extremity liposarcoma developed local recurrence and or distal metastases and in three the site of the distal spread was other than the lungs. In only one patient with retroperitoneal liposarcoma we observed extra-pulmonary spread. The histologic type of all four cases with unusual spread was myxoid, with small areas of round cell differentiation in two cases. RESULTS: The survival of the patients after the initial recurrence was relatively prolonged (6, 1.5, 8 and 3 years with the last patient alive and well). CONCLUSION: Physicians should maintain a low threshold for initiating evaluation of relatively minor symptoms in patients with liposarcoma, as these symptoms may reflect the first manifestation of an extrapulmonary metastasis. Such an approach should result in earlier detection and hopefully improve survival for patients with liposarcoma.

Abdominal Neoplasms↗
PubMed1995

Chimeric TLS/FUS-CHOP gene expression and the heterogeneity of its junction in human myxoid and round cell liposarcoma.

Myxoid liposarcomas have a unique and specific t(12;16)q13;p11) chromosomal translocation. The breakpoint has recently been identified and shown to involve the TLS/FUS gene on chromosome 16 and the CHOP gene on chromosome 12. This translocation causes fusion of these genes resulting in the expression of a novel chimeric TLS/FUS-CHOP message. Using the polymerase chain reaction with primer sets derived from sequences of TLS/FUS and CHOP cDNAs, we could amplify three types of the fusion transcripts from seven of seven samples of myxoid and round cell liposarcomas. In six of the seven positive samples, two kinds of chimeric messenger RNAs were found that have been reported previously. However, the last sample had a novel chimeric message that had an extra sequence of 33 bp derived from the TLS/FUS gene. Thus, it was shown that these fusion transcripts had a varying extent of the sequence of TLS/FUS gene incorporated at the site of the fusion. However, the TLS/FUS-CHOP fusion transcripts were not detected in two pleomorphic liposarcomas or in three myxoid variants of malignant fibrous histiocytomas. Our findings indicate that in liposarcomas TLS/FUS-CHOP fusion transcripts have variations at the junction of chimeric messages, which was the case for Ewing's sarcoma. Detection of the chimeric message by reverse transcription polymerase chain reaction was also suggested to be a useful approach for the diagnosis of myxoid and round cell liposarcomas that have (12;16) translocation, and for distinguishing them from pleomorphic liposarcoma and myxoid variant of malignant fibrous histiocytomas.

Adult↗
PubMed1999

Fine-needle aspiration cytology of well-differentiated inflammatory liposarcoma: a case report with histologic follow-up.

Inflammatory liposarcoma is an unusual variant of well-differentiated liposarcoma. We report on the fine-needle aspiration (FNA) cytology findings of a retroperitoneal well-differentiated inflammatory liposarcoma from a 63-yr-old white female. The smears showed numerous dispersed inflammatory cells, with the majority being reactive lymphoid cells and plasma cells. There were scattered, large, atypical cells containing multiple or hyperlobated nuclei with coarse chromatin and abundant ill-defined cytoplasm. The large atypical cells, as well as the inflammatory cells, were also found within the fibrous tissue fragments. The follow-up surgical resection of the tumor demonstrated a well-differentiated inflammatory liposarcoma with coexistent dedifferentiated areas and lipoma-like, well-differentiated liposarcoma. With the appropriate anatomic and radiographic settings, the FNA cytology findings of abundant, reactive inflammatory cells and scattered, large, atypical tumor cells that are CD15-, CD30-, and cytokeratin-negative are highly suggestive of a well-differentiated inflammatory liposarcoma.

Biopsy, Needle↗
PubMed1992

Cytogenetic findings in liposarcoma correlate with histopathologic subtypes.

The cytogenetic findings in 31 liposarcomas from 26 patients are reported. Four other tumors did not grow. Three histologic types are represented in this analysis. The well-differentiated liposarcomas were characterized by telomeric associations, large marker chromosomes and ring chromosomes, and in some cases, double minutes. The pleomorphic liposarcomas contained very high clonal chromosomal numbers with near-tetraploid modes and numerous variable, often unidentifiable, chromosomal abnormalities. The myxoid liposarcomas were characterized primarily by a t(12;16)(q13;p11) as the sole abnormality or additional changes. These results indicate that cytogenetic findings may provide a new criterion, not only for establishing the diagnosis of liposarcoma, but also for differentiating confusing histologic types of liposarcoma and these lesions from other types of sarcomas.

Aged↗
PubMed1995

Head and neck liposarcoma.

BACKGROUND: Liposarcoma of the head and neck region represents approximately 1% of head and neck sarcomas. Therefore, there are few data on the natural history, presentation, treatment, and prognosis of this neoplasm. METHODS: This study is a report of data from 76 patients with head and neck liposarcoma of whom 4 were treated at The Royal Marsden Hospital during the past 50 years. RESULTS: The median age of patient presentation was the seventh decade (range, 6 months-86 years), and 65% of the patients were male. The commonest site of presentation was the neck (28%), followed by the larynx (20%) and pharynx (18%). Sixty-two percent of tumors were low grade (well differentiated and myxoid), and 38% were high grade (pleomorphic and round cell). The principal determinant of outcome was histologic grade. Five-year survival by life-table analysis was 67% overall and varied with tumor type as follows: well differentiated 100%, myxoid 73%, pleomorphic 42%, and round cell 0%. Site appears to have had some influence on prognosis. Oral liposarcoma had a poor prognosis with a 5-year survival of 50%, despite the low grade of all tumors; however, the 5-year survival for laryngeal (89%) and head (83%) liposarcoma was considerably better. Tumor size did not affect prognosis. The mainstay of treatment was surgical excision, used alone in 70% of the cases. Radiotherapy was used with other treatments in 25% of the cases. Prognosis was best for patients treated with surgery only (5-year survival, 83%), compared with those receiving surgery plus radiotherapy (5-year survival, 63%), chemotherapy (5-year survival, 33%), and radiotherapy alone (5-year survival, 0%). CONCLUSIONS: Liposarcoma rarely involves the head and neck region. The prognosis for patients with this disease appears to be better than for those with liposarcoma arising elsewhere, particularly in the retroperitoneum. Prognosis is principally dependent on histologic grade. Complete surgical excision provides the most effective treatment.

Adolescent↗
PubMed1990

Myxoid liposarcoma: magnetic resonance imaging appearances with clinical and histological correlation.

Myxoid liposarcoma is the most common type of liposarcoma. The magnetic resonance imaging (MRI) features of this tumor were evaluated and correlated with its clinical and histological features in seven patients to determine under what circumstances the tumor should be considered in differential diagnosis and why its signal intensity differs from those of lipoma and lipoma-like (lipoblastic) liposarcoma. In all patients the tumor presented in a lower extremity (5 thigh, 2 calf) as a painless, slowly growing mass which had been present for several months to several years. MRI examination revealed the tumors to be encapsulated, noninfiltrating, and usually septated. On T1-weighted sequences five of seven lesions (71%) showed lacy, amorphous, or linear foci of high signal within a low signal mass. These foci are believed to represent fat within the tumor and distinguish it from several other benign and malignant masses. If an indolent mass in a lower extremity demonstrates a predominantly low signal with a few amorphous or linear high signal foci on T1-weighted sequences, one should consider the possibility of myxoid liposarcoma even if it appears benign by all other criteria. Histologic evaluation showed that myxoid liposarcomas contain less than 10% mature fat, which accounts for their low signal on T1-weighted sequences in contrast to the high signal of lipomas and lipoblastic liposarcomas.

Connective Tissue↗
PubMed2005

Primary liposarcoma of the thoracic spine: case report.

Liposarcoma is a malignant tumor of soft tissue. The thoracic spine is an unusual location, even for metastasis, and to our knowledge, no case of primary pleomorphic liposarcoma of the vertebral body has been reported until now. A female patient presented with paraplegia. She had a previous medical history of mental depression, and complained of dorsal pain for three months following a road accident. Magnetic Resonance Imaging (MRI) revealed a collapse of T7-T8, and the diagnosis of plasmocytoma was made. She was treated with decompressive laminectomy and posterior instrumentation. Histological examination revealed a pleomorphic liposarcoma. She received a course of radiotherapy. At 13 months follow-up she developed pulmonary metastases and rib involvement. The spine is an unusual location for pleomorphic liposarcoma, even as metastasis. The differential diagnoses of this rare entity are discussed, as well as the criteria for diagnosing primary spinal liposarcoma. Although rare, our case demonstrates that liposarcoma should be considered in the differential diagnosis of spinal tumors.

Diagnosis, Differential↗
PubMed2006

Overexpression of a member of the pentraxin family (PTX3) in human soft tissue liposarcoma.

A unique feature of human soft tissue liposarcoma is a stable (12;16)(q13;p11) translocation observed mainly in myxoid and roundcell liposarcomas. This translocation results in FUS/CHOP fusion transcripts with a corresponding oncogenic protein. We hypothesised that genes downstream of FUS/CHOP might serve as attractive candidates for novel tumour associated antigens. Among a panel of analysed genes, only pentraxin related gene (PTX3) demonstrated high expression in liposarcomas as compared to normal tissues. The analysis of RNA and protein expression demonstrated concordant results. However, the level of RNA and protein overexpression did not correlate in all cases. Finally, PTX3 expression was not related to presence of a FUS/CHOP fusion transcript within the liposarcoma tissues. PTX3 has been associated with adipocyte differentiation and now, additionally, is characterised by a markedly increased expression in human soft tissue liposarcoma. This finding mandates further research efforts to clarify the exact role of PTX3 in liposarcoma oncogenesis.

Adult↗
PubMed1986

Liposarcoma of the breast: a clinicopathologic study of 20 cases.

The clinical and pathologic features of 13 patients with pure liposarcomas of the breast and seven patients with liposarcomas that had arisen in cystosarcomas are reviewed. Metastases occurred in three of the 13 patients with pure mammary liposarcomas, and two women died of tumor. One of the patients with a liposarcoma that had arisen in a cystosarcoma had a local recurrence one year after diagnosis but was subsequently lost to follow-up study. Fourteen of the 20 patients for whom follow-up information was available for as long as 14 years had no evidence of recurrence. Features associated with the development of recurrence, based on the cases studied here and previous reports, included the pleomorphic liposarcoma pattern and an infiltrative margin. Features associated with tumor-free survival included the well-differentiated liposarcoma pattern, male gender (two patients), and a circumscribed microscopic tumor margin. There were no axillary lymph node metastases. Follow-up data indicate that complete surgical excision of tumor with tumor-free margins is necessary, but total mastectomy and removal of the axillary tail are not required unless these procedures are needed for complete excision.

Adult↗
PubMed2002

Expression of the FUS domain restores liposarcoma development in CHOP transgenic mice.

Fusion proteins created by chromosomal abnormalities are key components of mesenchymal cancer development. The most common chromosomal translocation in liposarcomas, t(12;16)(q13;p11), creates the FUS-CHOP fusion gene. In the past, we generated FUS-CHOP and CHOP transgenic mice and have shown that while FUS-CHOP transgenic develop liposarcomas, mice expressing CHOP, which lacks the FUS domain, display essentially normal white adipose tissue (WAT) development, suggesting that the FUS domain of FUS-CHOP plays a specific and critical role in the pathogenesis of liposarcoma. To test the significance of FUS and CHOP domain interactions within a living mouse, we generated mice expressing the FUS domain and crossed them with CHOP-transgenic mice to generate double-transgenic FUSxCHOP animals. Here we report that expression of the FUS domain restores liposarcoma development in CHOP-transgenic mice. Our results provide genetic evidence that FUS and CHOP domains function in trans for the mutual restoration of liposarcoma. These results identify a new mechanism of tumor-associated fusion genes and might have impact beyond myxoid liposarcoma.

Adipocytes↗
PubMed2004

[Liposarcoma of the spermatic cord--report of one new case and review of the literature].

Liposarcoma of the spermatic cord is a rare entity. Although most liposarcomas of the spermatic cord are well-differentiated, the propensity for local recurrence is high. Preferential treatment of spermatic cord liposarcoma is radical orchiectomy with high ligation of the cord. Radiation therapy is recommended in addition to surgery in cases with evidence of more aggressive tumour behavior (i.e., high-grade tumour, lymphatic invasion, inadequate margin, or recurrence). A 39-year-old-male presented with a 4-year history of a mass in the left scrotum. Radical orchiectomy was performed. Pathological analysis demonstrated a well-differentiated liposarcoma with tumour detection in the surgical margin. In view of the incomplete surgical removal of the tumour a retroperitoneal reoperation of the testicular vessels and vas deferent with R0-resection was conducted. Without any postoperative adjuvant therapy in evidence of recurrence or metastasis was noted during the 12-month follow-up period. The current literature on management of malignant tumours of the spermatic cord is reviewed. Paratesticular liposarcomas are most commonly well-differentiated and lipoma-like and have a prolonged clinical course. Radical orchiectomy with wide local excision of the mass is the recommended therapy, while adjuvant radiotherapy may be considered in high-grade tumours and in recurrent liposarcomas. Retroperitoneal lymphadenectomy does not offer any additional therapeutic benefit, and the role of chemotherapy is not well defined. Regardless of initial therapy, the risk of local recurrence always necessitates long-term followup.

Adult↗
PubMed2003

Different mRNA expression profile during tumor progression in a well-differentiated liposarcoma--A microdissection approach.

Like malignant fibrous histiocytoma (MFH), dedifferentiated liposarcoma represents a distinct subtype of liposarcoma and is characterized by an abrupt transition from well-differentiated liposarcoma (WDL) to highgrade dedifferentiated liposarcoma (DDL) . In addition, specific cytogenetic aberrations support the close biological relationship between WDL and DDL. Recent observations indicated the significance of cell cycle aberrations in tumor progression from the low-malignant, well differentiated to its dedifferentiated form, the prognosis of which is poor. Thus, alterations of mdm2 and p53 genes belong to the most frequently reported alterations in these two subtypes of liposarcoma. In previous investigations, we reported that loss of heterozygosity at the Rb gene locus, telomerase activity, hTERT, and c-Myc expression were associated with tumor progression in liposarcomas. In this study, we report on a case of a WD/DDL, in which both tumor components were separated using laser microdissection (P.A.L.M.) for the investigation of hTERT mRNA expression on a LightCycler. Macroscopically selected and histologically proven cryosections of low malignant and highly malignant tumor areas were cytogenetically investigated to confirm the diagnosis and to find additional chromosomal alterations with tumor progression.

Aged↗