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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↗

PubMed2000

High telomerase activity and high HTRT mRNA expression differentiate pure myxoid and myxoid/round-cell liposarcomas.

Molecular markers characterizing the transition of a myxoid to a more round-cell liposarcoma have not been described. To examine whether telomerase activity, hTRT and hTR mRNA expression were associated with tumor progression in myxoid liposarcoma, we investigated a total of 28 myxoid liposarcomas (13 pure myxoid tumors, 14 mixed-type tumors, and 1 pure round-cell variant) from 19 patients. Telomerase activity was detected by using the fluorescent PCR-based TRAP-assay. Expression of hTRT and hTR mRNAs was determined by the semi-quantitative RT-PCR. On the basis of only one tumor sample per patient, telomerase activity was found in 9 of 9 myxoid/round-cell liposarcomas and in 3 of 10 pure myxoid tumors. Elevated hTRT expression was found in 13 of 17 liposarcomas. All telomerase-positive tumors showed hTRT expression, whereas there were 3 cases showing hTRT expression without telomerase activity. HTR mRNA expression was elevated in all 19 liposarcomas. Thus, only the levels of telomerase activity and of hTRT mRNA expression differentiated pure myxoid liposarcoma and myxoid/round-cell liposarcoma (p < 0.003 and p = 0.029, respectively). We believe that high levels of telomerase activity and of hTRT expression are associated with tumor progression from low-grade pure myxoid to higher-grade malignant round-cell liposarcoma, and may consequently represent a useful prognostic marker for this histological sub-type of soft-tissue tumors.

Biomarkers↗

PubMed2003

A study of inter-alpha-trypsin inhibitor chains expression in liposarcomas.

AIM: Liposarcoma is common soft tissue sarcoma that is sometimes difficult to treat, besides its good prognosis. The inter-alpha-trypsin inhibitor heavy chains (HCs) has been reported to be linked to hyaluronan, which play important roles in tumour progression and metastasis. In this study, clinical significance of HCs in patients with liposarcoma was investigated. METHODS: HC expression was studied by immunohistochemistry on resected specimens of 33 liposarcoma patients and 10 lipoma patients. The expression of HC mRNA was analyzed by reverse transcription polymerase chain reaction (RT-PCR). Serum concentration of HC was determined with enzyme-linked immunosorbent assay (ELISA). RESULTS: Prominent positive staining of HC was observed in extracellular matrix of pleomorphic and myxoid liposarcoma. In well-differentiated liposarcoma and lipoma, faint staining was seen with HC. No products of HC could be detected by RT-PCR. Serum concentration of HC was not up-regulated in any subtypes of liposarcoma. HC expression was not significantly correlated with tumour subtypes and prognosis. CONCLUSION: HC was strongly accumulated in pleomorphic and myxoid liposarcoma, however, was not locally synthesized in liposarcoma. HC might play roles in stabilizing extracellular matrix, such as hyaluronan (HA), in liposarcoma.

Alpha-Globulins↗

PubMed1995

FUS/TLS-CHOP chimeric transcripts in liposarcoma tissues.

Myxoid liposarcoma and malignant fibrous histiocytoma (MFH) are common soft tissue sarcomas of adulthood. Histopathologically they often show intratumor heterogeneity. In some cases, differential diagnosis of liposarcoma and MFH is difficult. It has been reported that myxoid liposarcomas are characterized by chromosomal translocation t (12; 16) (q13; p11), and that this results in two types (type I and type II) of FUS/TLS-CHOP fusion transcripts. In this study, the FUS/TLS-CHOP chimeric transcripts in seven malignant soft tissue tumors of Asian patients were analyzed by reverse transcription-polymerase chain reaction, DNA blot hybridization and nucleotide sequencing. One myxoid liposarcoma and two round cell liposarcomas possessed a chimeric transcript whose fusion point was the same as that of the type I fusion transcript reported previously for myxoid liposarcoma. We were thus able to detect the type I FUS/TLS-CHOP fusion transcript in clinical specimens of liposarcoma from Asian patients, including the first examples of round cell liposarcoma. These results suggest that the detection of FUS/TLS-CHOP chimeric transcripts or chimeric genes can be used as a diagnostic tool for the pathological diagnosis of liposarcomas.

Adult↗

PubMed1983

Liposarcoma occurring in children. An analysis of 17 cases and review of the literature.

Liposarcoma, one of the most common malignant mesenchymal neoplasms affecting adults, has rarely been documented to arise in children. Therefore, in this retrospective study, the clinicopathologic features of 17 liposarcomas retrieved from the files of the Armed Forces Institute of Pathology (AFIP) which occurred in patients between the ages of eight months and 15 years were analyzed. In contrast to the slight male prevalence verified in most studies of adult liposarcoma, this group of 17 cases disclosed a distinct female majority (65%). The tumor had a predilection for the lower extremity (41%), particularly the thigh; the remaining examples, which included cases from the neck, back, axilla, buttock, and forearm, reflected a seemingly random anatomic distribution. The retroperitoneum, a very common primary location for adult liposarcoma, accounted for only one example in the group. The histologic variants of liposarcoma coincided with the types delineated in most generally accepted classifications employed for liposarcoma in adults, but their distribution varied considerably from that found in most extensive series of adult liposarcomas. Specifically, there were 13 myxoid liposarcomas, two well-differentiated liposarcomas, and one each of the round cell and mixed variants. Follow-up information, obtained for 15 cases, revealed local recurrence in three patients, one of whom eventually died as a result of direct tumor extension into the pleural cavity.

Adolescent↗

PubMed2004

[liposarcoma. Aspects of pathomorphology--an analysis of 209 tumos].

BACKGROUND: Among the heterogeneous group of adult soft tissue sarcomas, liposarcomas represent the largest entity along with malignant fibrous histiocytomas (MFH). PATIENTS AND METHODS: This article summarizes the results of pathomorphological data on 209 liposarcomas resected over a 10-year period. RESULTS: The most common tumor site was the thigh, and the peak age incidence was in the 5th and 6th decades. In general, three major subtypes of liposarcoma can be distinguished in terms of pathomorphology: well-differentiated/dedifferentiated liposarcoma, myxoid/round cell liposarcoma, and pleomorphic liposarcoma. Well-differentiated liposarcomas represent malignancy grade 1 tumors without biological potential to metastasize, but which are able to relapse locally in cases of incomplete resection. When a local relapse has occurred, the liposarcoma may show dedifferentiation and may metastasize. CONCLUSIONS: In the pathologic-anatomical diagnosis of liposarcomas, conventional light-microscopic findings are decisive. Additional methods of molecular pathology may help in single cases to gain further insights.

Adult↗

PubMed1997

Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation.

We studied 155 cases of dedifferentiated liposarcoma to define its clinicopathologic features and behavior, in particular how the extent and grade of dedifferentiation affected outcome. Tumors occurred in late adult life (median, 61.5 years: range, 21-92 years), most commonly in the retroperitoneum (106 cases), extremities and trunk (32 cases), and scrotum/spermatic cord (13 cases). The majority of dedifferentiated liposarcomas presented as de novo lesions, whereas the remainder developed as a late complication of a preexisting well-differentiated liposarcoma after an average interval of 7.7 years. At the time of presentation, most of the dedifferentiated liposarcomas displayed extensive areas of high-grade dedifferentiation resembling malignant fibrous histiocytoma or high-grade fibrosarcoma, whereas a minority contained only areas of low-grade dedifferentiation resembling fibromatosis or well-differentiated fibrosarcoma. Divergent myosarcomatous or osteosarcomatous differentiation was observed focally in six cases. The behavior of dedifferentiated liposarcomas was that of a high-grade sarcoma with a local recurrence rate of 41%, a metastatic rate of 17%, and disease-related mortality of 28%. The most important prognostic factor was location in that retroperitoneal tumors had significantly worse survival than those in other sites. Tumors were divided into those having less than or those with more than 25% dedifferentiation, and dedifferentiated zones were classified into low grade or high grade. Neither low-grade dedifferentiation nor a low percentage of dedifferentiation was associated with an improved outcome for the tumors examined in this study; however, in no cases was the absolute size of the dedifferentiated focus <2 cm. Therefore, this study did not determine a minimum, or threshold, amount of dedifferentiation below which outcome was more favorable. The behavior of liposarcomas in which the dedifferentiated component was entirely low grade was more similar to that of traditional dedifferentiated liposarcoma than to that of well-dedifferentiated liposarcoma. Our study supports the expansion of the definition of dedifferentiated liposarcoma to include tumors with low-grade dedifferentiation and also suggests that low-grade dedifferentiation represents a precursor lesion of high-grade dedifferentiation.

Adult↗

PubMed1999

Retroperitoneal liposarcoma with combined well-differentiated and myxoid malignant fibrous histiocytoma-like myxoid areas.

To broaden the knowledge of myxoid morphology in liposarcoma, eight cases of unusual liposarcoma with combined well-differentiated and myxoid malignant fibrous histiocytoma (MFH)-like myxoid areas are reported. The tumors arose as huge retroperitoneal masses in elderly patients, except for one that occurred in the spermatic cord. Three cases had local recurrences, and one of the seven patients who were followed up had died of the tumor. Grossly, the tumors were mostly confluent and multinodular and showed a glistening myxoid appearance in variable proportions, which merged gradually into or were juxtaposed to yellow fatty or sclerotic whitish areas. Microscopically, in addition to areas of well-differentiated lipoma-like or sclerosing liposarcoma, all the tumors contained myxoid portions characterized by scattered multinucleated or bizarre giant cells and a prominent plexiform vascular pattern that resembled myxoid MFH or myxofibrosarcoma. The myxoid areas were associated with discernible lipogenesis. High-grade dedifferentiation was present in one tumor. Cytogenetically, in one case, the myxoid lesion had nonrandom chromosomal aberrations, such as ring and marker chromosomes, characteristic of a well-differentiated variant of liposarcoma. In a nested reverse transcription-polymerase chain reaction analysis using archival paraffin-embedded tissue, it was seen that none of the eight tumors with myxoid MFH-like features had TLS/FUS-CHOP fusion transcripts characteristic of myxoid and round cell liposarcomas. These clinicopathologic and molecular features suggest that the current myxoid tumors are more closely related to well-differentiated liposarcoma rather than to ordinary myxoid liposarcoma despite their unequivocal myxoid morphology. Missense point mutations of the p53 gene were detected in two (25%) cases by single-strand conformation polymorphism and sequence analyses. Immunohistochemical expressions of p53 and mdm2 were observed in 75% of the cases, in which immunoreactive tumor cells were seen more often in the myxoid MFH-like areas. Thus, altered p53 pathways, such as p53 gene mutation and mdm2-mediated inactivation of p53, may play a pathogenetic role in this form of tumor progression showing myxoid MFH-like morphology in liposarcoma, as has been suggested in dedifferentiated liposarcoma.

Aged↗

PubMed2003

Paratesticular liposarcoma: a clinicopathologic study.

Paratesticular liposarcomas are rare and typically reported as isolated cases or as components of larger studies of liposarcomas. We studied a series of these tumors. All cases of paratesticular liposarcomas were retrieved from the archives of the Royal Marsden Hospital and the Johns Hopkins Hospital. Slides were reviewed and clinical information obtained. There were 30 paratesticular liposarcomas from men aged 41-87 years (mean 63 years; median 65 years) that involved the spermatic cord (23, 76%), testicular tunics (6, 20%), and epididymis (1, 4%). Tumors ranged from 3 to 30 cm (mean 11.7 cm; median 10 cm). Nineteen were well-differentiated liposarcomas (WDLs) and 10 were dedifferentiated liposarcomas (DDLs, five with high-grade and five with low-grade dedifferentiation). One was a myxoid/round cell liposarcoma with 70% round cell areas. All patients were treated by radical orchiectomy. One patient with WDL received radiation after his second recurrence and the myxoid/round cell liposarcoma received radiation and chemotherapy. Follow-up information was available for 16 of the patients, including 10 WDLs (range 24-216 months, mean 106 months), 5 DDLs (14-30 months, median 24 months), and for the myxoid/round cell liposarcoma (14 months) (range for all cases 14 months to 22 years; mean 87 months, median 36 months). Six of the WDLs (60%) recurred at 2, 4, 6, 10, 18, and 21 years (median 8 years). The lesion that recurred at 18 years (case no. 6) displayed foci of high-grade dedifferentiation in the recurrence, although the patient was disease free at 19 years. One patient with WDL had two recurrences at 4 and 7 years, and another had six recurrences over a 17-year period. Only one example of DDL recurred, at 30 months; another patient, who refused therapy for 15 years, had a primary tumor 30 cm in diameter, displayed pulmonary metastases 1 month after excision, and died after 14 months. The patient with MRCL had abdominal metastases after 1 year and was alive at 14 months. In summary, paratesticular WDL had a prolonged course with recurrences in more than half the cases, sometimes late. There were no metastases and the overall prognosis was good. One DDL recurred and only one of five (20%) developed metastases, but the mean follow-up for DDL was only 24 months.

Adult↗

PubMed2003

Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma.

OBJECTIVE: The aim of this study was to determine the pattern of recurrence and prognostic significance of histologic subtype in a large series of patients with primary retroperitoneal liposarcoma. SUMMARY BACKGROUND DATA: Classification of liposarcoma into subtypes, based on morphologic features and cytogenetic aberrations, is now widely accepted. Previous studies have shown that high histologic grade and incomplete gross resection are the most important prognostic factors for survival in patients with retroperitoneal sarcoma and suggest that patients with liposarcoma have a 3-fold higher risk of local recurrence compared with other histologies. METHODS: A prospective database was used to identify 177 patients with primary retroperitoneal liposarcoma treated between July 1982 and June 2002. Histology at primary presentation was reviewed by a sarcoma pathologist and subtyped into 4 distinct groups according to strict criteria. The influence of clinicopathological factors on local recurrence, distant recurrence, and disease-specific survival was analyzed. RESULTS: Of 177 patients with primary retroperitoneal liposarcoma operated on for curative intent, 99 (56%) presented with well-differentiated, 65 (37%) with dedifferentiated, 9 (5%) with myxoid, and 4 (2%) with round cell morphology. The tumor burden was determined by the sum of the maximum tumor diameters. The median tumor burden was 26 cm (5-139). Median follow-up time for 92 (52%) surviving patients was 37 (mean, 0.5-192) months. Multivariate analysis showed that dedifferentiated liposarcoma subtype was associated with a 6-fold increased risk of death compared with well-differentiated histology (P < 0.0001). In addition to histologic subtype, incomplete resection (P < 0.0001), contiguous organ resection (excluding nephrectomy; P = 0.05), and age (P = 0.03) were important independent prognostic factors for survival in retroperitoneal liposarcoma. Retroperitoneal dedifferentiated liposarcoma was associated with an 83% local recurrence rate and 30% distant recurrence rate at 3 years. CONCLUSIONS: The histologic subtype and margin of resection are prognostic for survival in primary retroperitoneal liposarcoma. Dedifferentiated histologic subtype and the need for contiguous organ resection (excluding nephrectomy) was associated with an increase risk of local and distant recurrence. Nephrectomy may be needed to achieve complete resection, but has no measurable influence on disease specific survival.

Databases, Factual↗

PubMed2001

H-ras oncogene mutation in dedifferentiated liposarcoma. Polymerase chain reaction-restriction fragment length polymorphism analysis.

Point mutations of the ras gene family (K-ras, H-ras, and N-ras) are thought to be involved in the development of a variety of human tumors. Dedifferentiated liposarcoma is characterized by the coexistence of well-differentiated (WD) and high-grade anaplastic (HG) components. The presence of point mutations at codons 12 and 13 of the H-ras gene was studied in 34 liposarcomas, comprising 15 well-differentiated liposarcomas and 19 dedifferentiated liposarcomas, and in 8 storiform-pleomorphic type malignant fibrous histiocytomas (MFHs) using polymerase chain reaction-restriction fragment length polymorphism and direct sequencing analysis. The 2 components of dedifferentiated liposarcoma were analyzed independently. H-ras mutations were seen only in dedifferentiated liposarcomas (4/19 [21%]), 1 in WD components and 3 in HG components. The mutation was not seen in any of 15 cases of well-differentiated liposarcoma. MFHs showed an H-ras mutation in 1 (12%) of 8 cases. Our results seem to suggest that the H-ras mutation is a relatively uncommon event in dedifferentiated liposarcoma, which may demonstrate an epiphenomenon of dedifferentiation in dedifferentiated liposarcoma.

Adult↗

PubMed1996

Liposarcoma: outcome and prognostic factors following conservation surgery and radiation therapy.

PURPOSE: Liposarcoma is among the more common types of soft-tissue sarcoma. This retrospective review was performed to determine the outcome and delineate prognostic factors for this disease when treated by conservation surgery and radiation. METHODS AND MATERIALS: Between 1964 and 1992, 112 consecutive patients with liposarcoma were treated with conservation surgery and radiotherapy. Univariate and multivariate statistics were used to evaluate the outcome with local control, metastatic relapse, and survival as end points, and to delineate independently significant prognostic factors. RESULTS: Preoperative radiation to a mean dose of 50 Gy was used in 36 patients and postoperative radiation to a mean dose of 61 Gy was used in 76 patients. At a median follow-up of 9 years (range 2-25), 34 patients (30%) developed disease progression at some site. Local recurrence developed in 14 patients (13%), and the actuarial local relapse rate was 13% at 10 years. Metastases developed in 23 patients (21%) with an actuarial metastatic rate of 23% at 10 years. Five-, 10-, and 15-year survival rates were 79%, 69%, and 61%, respectively. The most important factor influencing outcome--whether local control, metastases, or survival--was tumor histologic subtype. Well-differentiated (15 patients) and myxoid (including round cell) (71 patients) subtypes had a local control rate exceeding 90% at 10 years. No patient with well-differentiated liposarcoma developed metastases. The actuarial metastatic rate for myxoid tumors was 22% at 10 years and was higher for large than small tumors. The pattern of metastases for this variant was atypical with a preponderance of extrapulmonary sites. Pleomorphic liposarcoma (26 patients) was a highly malignant tumor with a high local recurrence rate of 37% and a metastatic rate of 41% at 10 years. Larger pleomorphic tumors had a significantly higher metastatic rate than smaller ones. These biologic factors were reflected in progressively decreasing 10-year survival rates from 87% for well-differentiated lesions, through 76% for myxoid tumors, to 39% for pleomorphic tumors. Although other factors such as positive resection margins and prior local recurrence were also independently significant determinants of outcome, histology was the overriding factor. CONCLUSION: Liposarcoma is a highly pleomorphic disease whose outcome is greatly determined by its histologic subtype: so greatly that results reported without histologic stratification are likely meaningless. Conservation surgery and radiation achieve excellent local results and with relatively little metastatic risk for patients with well-differentiated and myxoid liposarcomas. The same treatment strategy appears appropriate for pleomorphic liposarcoma, but with a much smaller expectation of a satisfactory outcome. The development of effective systemic therapy is an important goal for this variant of liposarcoma.

Adolescent↗

PubMed2000

Liposarcoma of the pleural cavity: clinical and pathologic features of 4 cases with a review of the literature.

BACKGROUND: Primary liposarcomas of the pleura are extremely rare malignancies, and relatively few reports appear in the world literature. DESIGN: We compiled a small series of 4 cases of primary pleural liposarcoma from the files of the Armed Forces Institute of Pathology (Washington, DC) and compared the histopathologic and clinical features of these 4 cases with those of 9 previously published cases. RESULTS: Our investigation included the case studies of 9 men and 4 women, aged 19 to 80 years (average, 49 years). Histologic subtypes in the 9 cases with available information included 5 myxoid liposarcomas, 1 well-differentiated liposarcoma, and 3 liposarcomas with mixtures of histologic types. Surgical resection with or without chemotherapy appeared to be the most common form of treatment, although radiation therapy was used in some cases and seemed beneficial. Survival information was available for 11 cases; 4 patients died of disease at 7, 9, 12, and 19 months; 1 died of heart failure 2 days after presentation; 1 died of unknown causes 16 months after presentation; and 3 patients were alive without tumor at 5, 16, and 66 months after diagnosis. One patient had local recurrence at 2 years. A second surgical resection in this patient failed, and he died of disease 9 years after initial presentation. A second patient experienced recurrence at 4 years and was free of disease 4 years after the second surgical resection. CONCLUSIONS: Primary pleural liposarcomas occur predominantly in older men, and the myxoid histologic subtype is the most common. Radiographic or surgical evaluation is important to distinguish primary pleural liposarcoma from chest wall or mediastinal sarcomas, as well as metastases from other sites. Although further investigation is needed, evidence from the cases reviewed here indicates that surgical resection with adjuvant radiation therapy may benefit patients with primary pleural liposarcoma.

Aged↗

PubMed1999

Patterns of recurrence in extremity liposarcoma: implications for staging and follow-up.

BACKGROUND: Liposarcoma is one of the most common histologic types of soft tissue sarcoma and presents a wide spectrum of clinical behavior. The authors examined the correlation among histologic subtypes, outcomes, and patterns of recurrence among patients with extremity liposarcomas. METHODS. A retrospective review of all patients with intermediate and high grade extremity liposarcoma referred to the University of Texas M. D. Anderson Cancer Center from January 1, 1980, to December 31, 1992, was performed. Data on clinical presentation, treatment, patterns of treatment failure, and outcome were evaluated. RESULTS: During the 13-year study period, 122 patients with intermediate or high grade extremity liposarcoma were identified: 102 patients (84%) with myxoid subtype, 18 patients (15%) with pleomorphic subtype, and 2 patients (2%) with mixed histology. There were no differences between the myxoid and pleomorphic subtype groups in tumor size (T1 vs. T2), depth in relation to the muscular fascia, or anatomic site. The median follow-up was 70 months. The 5-year overall survival rate for all intermediate and high grade extremity liposarcoma patients presenting with primary disease (n=85) was 74%; the 5-year local recurrence free survival, distant recurrence free survival, and disease free survival rates were 93%, 78%, and 73%, respectively. Among the 102 patients with myxoid tumors, 33 had distant recurrences; 31 of these were to extrapulmonary soft tissue sites (e.g., the retroperitoneum, chest wall, pleura, pericardium, pelvic sidewall, and soft tissue of the back), and 2 were to the lung only. Among the 18 patients with pleomorphic tumors, 10 had distant recurrences; 3 occurred at extrapulmonary sites, and 7 occurred in the lung only (P < 0.05 for myxoid vs. pleomorphic subtypes). CONCLUSIONS: Myxoid liposarcomas often metastasized to extrapulmonary sites and did so significantly more frequently than pleomorphic tumors. Imaging of the abdomen, retroperitoneum, and extrapleural chest should be performed for accurate staging and posttreatment follow-up of patients with myxoid liposarcoma. Patients presenting with "primary" myxoid liposarcoma of the trunk should be carefully evaluated for an occult primary tumor in an extremity.

Adolescent↗

PubMed1999

Cytomorphologic features of fine-needle aspiration of liposarcoma.

The cytomorphologic features of fine-needle aspirates (FNA) of liposarcomas have not been fully characterized. In the current study, we attempted to identify cytological attributes of liposarcoma in FNA. Twelve FNAs of histologically proven liposarcomas were used for this study. They included 10 myxoid and two well-differentiated liposarcomas. Four cytological criteria were chosen for evaluation. These included a complex capillary network, increased cellularity, the presence of a metachromatic stroma for myxoid liposarcoma, and finally the identification of lipoblasts. Eighteen FNAs of lipomas and 17 FNAs of nonlipomatous soft-tissue sarcomas were used as controls for evaluation of the same features. The diagnosis of liposarcoma had been rendered on FNA in 7 cases (58%). The diagnosis was suggested in 3 cases (25%), and a suggestion of sarcoma was rendered in 2 cases. Complex capillary networks were identified in 9 of 12 cases (75%); this was seen in 8 of the 10 myxoid liposarcomas and 1 of the 2 well-differentiated neoplasms. Increased cellularity was seen in 7 of 12 cases (58%), metachromatic stroma was seen in 3 of 12 cases (25%), and lipoblasts were identified in 4 of 12 cases (33%). None of the lipomas or sarcomas exhibited a complex capillary network. When capillaries were identified in lipomas, they were straight, with minimal branching. The lipomas did not show evidence of increased cellularity in any cases. Metachromatic stroma was more frequently seen in the nonlipomatous sarcomas than in the liposarcomas. No lipoblasts were identified in any of the control FNAs.

Adult↗

PubMed1989

The prognostic value of histologic subtypes in primary extremity liposarcoma.

Patients with a localized primary liposarcoma of the extremity who were treated in Memorial Sloan-Kettering Cancer Center between 1968 and 1978 were studied. The prognosis of patients with extremity liposarcoma was analyzed according to histopathologic subtypes. A system of five subtypes was used; well-differentiated, myxoid, fibroblastic, lipoblastic, and pleomorphic liposarcomas. The 5-year treatment failure rates and survival rates were, respectively, 30% and 100% for well-differentiated, 25% and 88% for myxoid, 60% and 58% for fibroblastic, 100% and 40% for lipoblastic, and 64% and 56% for pleomorphic liposarcomas. Although local recurrence was present in all five subtypes, a significant increment in local failure was seen in patients with high-grade liposarcomas. Distant metastasis after the initial operation was not found in patients with well-differentiated liposarcoma and rare in the patients with myxoid liposarcoma. In contrast, 50% of the patients with fibroblastic, lipoblastic, and pleomorphic liposarcoma had a distant relapse within 5 years.

Adolescent↗

PubMed2001

MDM2+/CDK4+/p53+ oral liposarcoma: case report and review of the literature.

Although liposarcoma is one of the most common soft tissue sarcomas, its location in the oral cavity is very rare. To our knowledge, only 43 cases of liposarcoma originating in the oral tissues have been reported in the English-language literature. In this article, we report a case of well-differentiated liposarcoma affecting the cheek of a 28-year-old man and review the oral liposarcoma literature. Immunohistochemical analysis of the tumor revealed an MDM2+/CDK4+/p53+ immunophenotype that is consistent with the immunohistochemical profile of well-differentiated liposarcoma originating in other areas of the body. Quantitative polymerase chain reaction analysis of the DNA levels of the MDM2 (human homologue of the murine double-minute type 2), CDK4 (cyclin-dependent kinase 4), and SAS (sarcoma amplified sequence), genes was performed, revealing only SAS gene amplification. The possibility of misdiagnosis of oral liposarcoma because of its sometimes inconspicuous clinical and microscopic features is emphasized. Careful pathologic examination of liposarcoma is essential for discrimination from benign adipose tissue neoplasms and for precise histologic classification, both of major prognostic significance. Possible implications of molecular and cytogenetic analysis for unraveling the pathogenesis and determining the prognosis of liposarcoma are discussed.

Adult↗

PubMed1993

Liposarcoma of the extremities: MR and CT findings in the histologic subtypes.

The findings on images of liposarcomas of the extremities in 48 patients (26 men and 22 women aged 20-85 years) were reviewed to correlate the histologic subtypes with radiologic appearance. Computed tomographic scans were obtained in 36 patients; magnetic resonance (MR) images, in 27 patients. The study group had 19 myxoid, 12 well-differentiated, nine round cell, and eight pleomorphic liposarcomas. Well-differentiated liposarcomas were predominantly composed of fat, typically with thick septa, which were hyperintense on T2-weighted spin-echo MR images. The heterogeneity of tumor helped differentiate tumor subtypes. Eleven of 19 myxoid tumors were mildly heterogeneous. Round cell and pleomorphic subtypes, which tend to be aggressive, were generally heterogeneous. Well-differentiated liposarcomas may be confidently differentiated from other subtypes of liposarcoma. Except for the well-differentiated subtypes, liposarcomas often contain no fat. Moderate to marked heterogeneity is common in high-grade liposarcomas; myxoid liposarcomas tend to be homogeneous and may mimic cysts.

Adult↗