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PubMed1981

Surgery of soft tissue sarcomas in children.

Surgery of soft tissue sarcomas in children has been modified by the introduction of multidisciplinary treatment so that major amputation and exenterations are now rare in the management of these tumors in children. Surgery must still be well planned for total removal of the tumor with preservation of limbs and, in most instances, the function of pelvic organs. The commonest soft tissue sarcomas in children are embryonal rhabdomyosarcoma (RMS), fibrosarcoma, and synovial sarcoma. Treatment of embryonal RMS can now achieve a 2-year survival of 80% at all sites. Surgery and irradiation are used to control the primary tumor and multidrug chemotherapy to control metastases or prevent dissemination of localized tumor. Fibrosarcoma in children, usually a low-grade, extra-abdominal desmoid lesion, does not respond to radiation treatment or chemotherapy, and management is by surgery alone. It shows a marked tendency to local recurrence, and multiple local resections may be necessary for cure. Ninety percent of the children with this tumor can be salvaged by surgery and careful follow-up. The management of synovial sarcoma is surgical and similar to that of RMS. Although not radiosensitive as is RMS, this tumor has responded well to multidisciplinary treatment. Adequate resection is now followed by a chemotherapy protocol similar to that used in osteogenic sarcoma. The smaller numbers of these tumors and their varied natural history make evaluation of treatment difficult. Other soft tissue sarcomas seen with extreme rarity in children are liposarcoma, angiosarcoma, and neurosarcoma. These tumors are treated with the same protocol as that of RMS.

Child↗
PubMed2002

Molecular alterations of h-warts/LATS1 tumor suppressor in human soft tissue sarcoma.

h-warts/LATS1 is a human homolog of the Drosophila warts tumor suppressor gene, which functions as a component of the mitotic apparatus. LATS1-deficient (Lats1(-/-)) mice have been reported to develop ovarian stromal tumors and soft tissue sarcomas. We investigated the status of the h-warts/LATS1 in 50 human soft tissue sarcomas to address its potential function as a tumor suppressor in human sarcoma development. In our RT-PCR assay, 7 (14%) (3 of 4 myxoid liposarcomas, 3 of 7 leiomyosarcomas, 1 of 9 malignant fibrous histiocytomas) of 50 sarcomas examined had no or a reduced expression of the h-warts/LATS1, indicating down-regulated gene expression. We further analyzed alterations of the h-warts/LATS1 in these seven sarcomas. Using microsatellite markers for chromosome 6q23-25.1, to which the h-warts/LATS1 is localized, an allelic loss of this locus was detected in one leiomyosarcoma, in which a missense point mutation of the h-warts/LATS1 was detected by PCR single-strand conformation polymorphism. Clusters of CpG dinucleotides in a 5' putative promoter region were hypermethylated in the other six sarcomas. Our data suggest that the molecular alterations of the h-warts/LATS1 could be of pathologic importance in human sarcomagenesis.

Base Sequence↗
PubMedDate not supplied

[Liposarcoma of the ischiorectal fossa, an unusual tumoral site].

The main locations of the liposarcoma, the most common of the soft tissue sarcomas, are the lower limbs and the retroperitoneal space. We report the case of a 58 year-old male patient presenting with a huge and painless mass of the left fossa ischiorectalis. Preoperative tests and CT-scan of the pelvis evoked the diagnosis of liposarcoma, laminating and lifting the rectum and bladder. Visceral walls were respected. The patient underwent a en-bloc excision of the tumour by a combined perineal and abdominal route. Pathological examination of the mass (1.7 kg) confirmed the diagnosis of well differentiated liposarcoma. No further treatment was initiated, but, because of the high risk of local recurrence, the patient has been placed under a close follow-up schedule.

Humans↗
PubMed2003

Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma.

Forty-four samples from 25 cases of retroperitoneal sarcoma initially diagnosed as malignant fibrous histiocytoma were histologically reviewed. Immunohistochemistry for mdm2 and cdk4 was performed on 20 cases. Comparative genomic hybridization was performed on 18 samples from 13 patients. Seventeen cases were reclassified as dedifferentiated liposarcoma. Twenty-one of 32 samples from these patients showed areas of well-differentiated liposarcoma, allowing the diagnosis of dedifferentiated liposarcoma. Immunohistochemistry performed in 15 of these cases showed positivity for mdm2 and cdk4. Comparative genomic hybridization analysis performed on 15 samples from 11 of these patients showed an amplification of the 12q13-15 region. Eight cases were reclassified as poorly differentiated sarcoma. Twelve samples from these patients showed no area of well-differentiated liposarcoma. Immunohistochemistry showed positivity for mdm2 and cdk4 in one of six of these patients and showed positivity for CD34 in another one. Comparative genomic hybridization analysis performed on three samples from two of these patients showed no amplification of the 12q13-15 region but showed complex profiles. This study shows that most so-called malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcoma and that a poorly differentiated sarcoma in this area should prompt extensive sampling to demonstrate a well-differentiated liposarcoma component, immunohistochemistry for mdm2 and cdk4, and if possible, a cytogenetic or a molecular biology analysis.

Adult↗
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↗
PubMed2003

Atypical lipoma: well-differentiated liposarcoma of the orbit with dedifferentiated areas.

Primary orbital liposarcoma is a very rare orbital tumor and dedifferentiation in the liposarcomas occurs rarely. Dedifferentiation is defined by development, in a well-differentiated tumor, of poorly differentiated, high-grade areas. The dedifferentiated liposarcomas (DDLS) have a better prognosis than pleomorphic sarcomas as a whole. The treatment of DDLS patients proves to be complicated due to the fact that the histopathological diagnosis usually becomes evident only after the surgery. In the present paper, the clinical presentation, histopathological features, radiological characteristics and clinical treatment of one case of well-differentiated liposarcoma with dedifferentiated areas, abruptly delimited from the well-differentiated adipocytic component, are reported.

Combined Modality Therapy↗
PubMed2005

Differential gene expression in liposarcoma, lipoma, and adipose tissue.

Malignant transformation is thought to be associated with changes in the expression of a number of genes, and this alteration in gene expression is felt to be critical to the development of the malignant phenotype. Sarcomas represent a diverse group of tumors derived from cells of mesenchymal origin. Marked heterogeneity exists in the biological behavior of sarcomas, even within histologic subtypes of sarcomas. In an effort to better understand the biology of liposarcomas, gene expression in normal adipose tissue, lipomas, and liposarcomas was examined using the Affymetrix microarray technology. Differences in gene expression were quantified as the fold change in gene expression among the sample sets. Differences in gene expression among normal adipose tissue, lipomas, and liposarcomas were observed. In addition, genes expressed uniquely in liposarcoma among these and 18 other tissue sample sets were identified. Gene sets were devised that allowed the separation of liposarcomas from other samples, and most normal adipose tissue from most lipomas using the Eisen clustering software "Cluster." We conclude that differences in gene expression can be identified among different tumors derived from the adipocyte series. Such differences in gene expression may help differentiate among subtypes of sarcomas, and may also yield clues to the pathophysiology of this heterogeneous group of tumors.

Adipose Tissue↗
PubMed1981

[Clinical and x-ray morphological parallels in soft tissue liposarcomas].

The clinicoroentgenological data in 45 cases (20 males and 25 females, average age 50.3 years) of soft tissue liposarcomas, histologically subdivided into 28 myxoid, 12 polymorphic and 5 round-cell tumors were compared. The means case history was 3.2 years (3.8 years--for myxoid and 2.2 years for polymorphic and roundcell liposarcomas). In 95.6% of cases, tumor sites were in the musculare of the extremities, more frequently, legs. Tumors were rather large in size (average 16.5 cm). Myxoid tumors developed slowly, causing no pain. The radiograms of these tumors showed multiple tumor nodes, reduced shadow density, structural heterogeneity, distinct contours, infrequent cutaneous infiltration and secondary changes in bones. Signs of malignancy were indistinct in angiograms. Clinically and roentgenologically, round-cell liposarcomas resembled most other types of soft tissue sarcomas. Polymorphic tumors had many features in common both with myxoid and round-cell neoplasms.

Adolescent↗
PubMed1996

The management of spermatic cord sarcoma.

BACKGROUND: Between April 1963 and July 1991, 18 patients were treated for spermatic cord sarcoma. The histologic subtype distribution was: 7 leiomyosarcoma, 7 liposarcoma, 2 malignant fibrous histiocytoma, and 1 mesothelioma. METHODS: All patients underwent surgical resection: 16 radical orchiectomy and local excision. Nine were treated with orchiectomy alone, and 9 received adjuvant radiation. The radiation fields encompassed the ipsilateral iliac and inguinal lymph nodes, vas deferens, and hemiscrotum in 7 patients, and iliac and inguinal lymph nodes in 2 patients. RESULTS: The actuarial 5 and 8-year disease free survivals for the 18 patients were 77% and 58%, with an overall survival of 78% and 70%, respectively. The 5 and 8-year locoregional control rates were 82% and 61%. Five of 9 patients treated with surgery alone developed locoregional recurrence while none of the nine who had adjuvant radiation relapsed. The median follow-up for the irradiated group, however, was shorter (123 vs 63 months) and staging studies more complete. These potential biases are discussed. CONCLUSION: In this series, relapse was common after orchiectomy alone. Adjuvant radiation therapy may reduce the incidence of locoregional failure.

Actuarial Analysis↗
PubMed1986

Soft-tissue and bone sarcoma histopathology peer review: the frequency of disagreement in diagnosis and the need for second pathology opinions. The Southeastern Cancer Study Group experience.

Histopathologic peer review of specimens from 216 consecutive patients with soft-tissue or bone sarcomas on Southeastern Cancer Study Group protocols was performed by a panel of three pathologists. Subtype of sarcoma, degree of confidence in diagnosis, and grade were compared with agreement or disagreement in pathologic opinion from the primary member institution v the pathology review panel. The most common soft-tissue sarcoma was leiomyosarcoma, followed by malignant fibrous histiocytoma, fibrosarcoma, liposarcoma, malignant schwannoma, and rhabdomyosarcoma. There was total agreement between the primary pathologist and reviewer in 66% of cases. However, 12 cases (6%) were considered on review to not be sarcomas. In 27% of cases the subtype of sarcoma was felt to be incorrect by reviewers. Discordant diagnoses were more common when the confidence of the pathologist was less and when the tumor was of higher grade. There was no improvement in frequency of agreement in the second half of the study, despite educational workshops. We conclude that histologic peer review is important in sarcoma studies and is essential in many patients with presumed sarcomas.

Bone Neoplasms↗
PubMed1992

Retroperitoneal sarcoma in a series of 51 adults.

Fifty-one patients with retroperitoneal sarcoma underwent surgery at the University Clinics of Hamburg from 1970 to 1988. Malignant schwannoma, liposarcoma and malignant fibrous histiocytoma were the most common histological types. High grade sarcomas (G3) predominated (42%), while 26% were graded G2 and 33% G1. Complete resection was possible in 59% of cases. Reoperation for local recurrence and debulking in case of not completely resectable disease was common. Regional lymph node metastasis was found in 20% of cases. The peritoneum was the preferred location of metastatic spread. All adjuvant chemotherapies were followed by tumour recurrences. After adjuvant radiotherapy three of six patients remained free of disease. In cases of residual tumour we observed few partial responses on chemo- and radiotherapy, and all patients died because of the disease. The mean survival time was 60 months, the 5- and 10-year survival rates were 35% and 15%, respectively. Factors influencing prognosis in univariate analysis included grade, size, resectability, presence of metastatic spread, histological type, microscopic local growth (infiltrative vs intact pseudocapsule of fibrous tissue) and local recurrence. With multivariate analysis grade, size and lymph node metastasis retained their prognostic significance.

Adolescent↗
PubMed1987

Aspiration cytology of lipomatous tumors: a 10-year experience at an orthopedic oncology center.

The cytodiagnoses of preoperative fine-needle aspirate smears from 72 histologically proven lipomatous tumors (52 lipomas and 20 liposarcomas of varying type) collected during the 10-yr period 1972-1981 were correlated to the corresponding histopathologic diagnoses. Three out of 52 lipomas were erroneously diagnosed as low-grade malignant liposarcomas, and one out of 20 liposarcomas was falsely diagnosed as a benign soft-tissue tumor. Of the 20 liposarcomas, 15 were cytologically diagnosed as such, and the majority of the myxoid and the pleomorphic liposarcomas were correctly diagnosed as to type. Important differential diagnostic problems were, on one hand, the separation of lipoma with regressive changes and histiocytic reaction and lipoma with chondroid metaplasia from low-grade malignant liposarcoma and, on the other hand, liposarcoma from intramuscular myxoma, myxofibrosarcoma, and malignant fibrous histiocytoma. The differential diagnosis of liposarcoma as opposed to other soft-tissue sarcomas is discussed, and it is concluded that preoperative fine-needle aspiration of lipomatous tumors is of value in the management of these tumors.

Biopsy, Needle↗
PubMed2006

[Retroperitoneal sarcomas: diagnostic and therapy].

BACKGROUND: Due to the fact that there are no distinct anatomical compartments, retroperitoneal sarcomas are moreover diagnosed with evidence of large tumors and infiltration of adjacent organs. In spite of improvement of the diagnostic facilities and surgical techniques, quite frequently local recurrences with unfavourable prognosis turn up even after complete removal. It was the aim of this study to analyze diagnosis, therapy and long-term prognosis in patients with retroperitoneal sarcomas over a period of 10 years. PATIENTS AND METHODS: Between January 1995 and January 2005, 379 patients underwent surgery for a primary retroperitoneal tumor at our clinic. Among the 67 (17.1 %) malignant lesions, a sarcoma was found in 35 patients. The present study is focused on the long-term prognosis of those 21 patients with a primary resected retroperitoneal sarcoma, recurrent sarcomas and exploratory laparotomies excluded. RESULTS: The median patient age at the time of surgery was 61 (25-86) years, 57.1 % were males. The duration of symptoms was 3 (1-36) months. A pR0-resection was achieved in 20/21 patients. Among the histopathological tumor types, liposarcomas (n = 10) and leiomyosarcomas (n = 6) were found most frequently. Local recurrence developed in 12 out of 21 patients at 13.5 (5-42) months after the first operation, and in 4 patients distant metastases were observed. The long-term survival of all 21 patients with primary resected retroperitoneal sarcoma was 24 (1-101) months. A more favourable prognosis was seen in patients with leiomyosarcoma as compared with liposarcoma. CONCLUSION: Retroperitoneal sarcomas are a heterogeneous entity, and they were found among our own patients in 6.1 % of all primary operatively treated retroperitoneal tumors. The strategy of treatment is complex and dependent on the stage, localization and histopathological characteristics. Long-term survival is limited as a result of the high rate of local recurrence.

Adult↗
PubMed1986

An unusual case of malignant cystosarcoma phyllodes of the breast.

A unusually large tumor of the left breast diagnosed as a cystosarcoma phyllodes with multiple malignant sarcomatous changes of the stroma, consisting of liposarcoma, myxoid fibrosarcoma, anaplastic, and giant cell sarcoma is described. The weight of 6200 g (13.5 lb) seems to be the largest so far presented in the literature reviewed by the authors.

Breast Neoplasms↗
PubMed1992

Aggressive angiomyxoma of the vulva.

A case of aggressive angiomyxoma (AAM) of the vulva is presented. The tumor presented as a slowly growing polypoid mass in the right labium minus near the clitoris. The tumor was treated by wide local excision. The microscopic appearance was that of spindle-shaped neoplastic cells widely separated by a loose myxoid stroma rich in collagen fibers, hyaluronic acid, and prominent irregular-shaped blood vessels. Aggressive angiomyxoma of the vulva must be distinguished from the more common benign and malignant myxoid tumors including myxoma, myxoid liposarcoma, myxoid variant of malignant fibrous histiocytoma, sarcoma botryoides, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. Local recurrence of AAM may be avoided by wide local excision.

Adult↗
PubMed2026

Driver genomic lesions in MDM2, CDK4, and JUN co-opt targetable super-enhancer networks to impose liposarcomagenic core regulatory circuitry.

INTRODUCTION: Amplification of chromosome 12q13-15 spanning MDM2 and CDK4 genes serves as a molecular diagnostic hallmark of dedifferentiated liposarcoma (DDLPS), an aggressive soft-tissue sarcoma. Epigenetic activation of master transcription factors (RUNX proteins, FOSL2, and MYC) establishes a self-reinforcing oncogenic transcriptional circuitry in DDLPS. Nevertheless, the collaborative interplay between genomic alterations and epigenetic dysregulation in defining DDLPS cell identity remains elusive. OBJECTIVES: This work aimed to elucidate the primary genetic drivers and mechanistic basis of DDLPS-specific core transcriptional regulatory circuitry. METHODS: We performed integrative chromatin profiling analysis of DDLPS clinical specimens and cell lines to map cis-regulatory landscapes. Cistromes of MDM2, JUN, and E2F1 were delineated through chromatin immunoprecipitation sequencing in two DDLPS models. Essential driver functions and transcriptional regulatory effects of key regulators were assessed via various genetic manipulation approaches. Synergistic interactions between BET-targeting agents and MDM2/p53 or CDK4 inhibitors were quantified by cell viability assays. In vivo xenograft assays evaluated the oncogenic potential of key regulators and the therapeutic efficacy of novel strategies. RESULTS: Co-amplification of MDM2, CDK4, and JUN during sarcomagenesis converges with BET protein-dependent chromatin remodeling to fuel feed-forward transcriptional circuits among master transcription factors. Mechanistically, excessively expressed MDM2 stabilizes the core regulatory circuitry by forming chromatin-bound complexes with JUN/FOSL2 at cis-regulatory elements, especially super-enhancers across DDLPS genome. Concurrently, CDK4 maintains expression of E2F1 which further fosters transcriptional output of master transcription factors in DDLPS cells. Leveraging DDLPS-selective overexpression of MDM2 and its E3 ligase activity, targeted degradation of BET proteins by MDM2-recruiting proteolysis targeting chimera selectively disrupted the core regulatory circuitry, suppressing DDLPS growth and exhibiting strong synergy with CDK4 inhibitor. CONCLUSION: DDLPS-associated genomic lesions collaborate with BET-dependent chromatin regulation to establish disease-sustaining transcriptional circuitry. Our findings also provide a mechanistic rationale for harnessing MDM2's E3 ligase activity to therapeutically degrade oncoproteins in MDM2-amplified malignancies.

Core transcriptional regulatory circuitry↗
PubMed2006

Sirolimus therapy after early cyclosporine withdrawal reduces the risk for cancer in adult renal transplantation.

Sirolimus (SRL) is a mammalian target of rapamycin inhibitor that, in contrast to cyclosporine (CsA), has been shown to inhibit rather than promote cancers in experimental models. At 3 mo +/- 2 wk after renal transplantation, 430 of 525 enrolled patients were randomly assigned to remain on SRL-CsA-steroids (ST) or to have CsA withdrawn and SRL troughs increased two-fold (SRL-ST). Median times to first skin and nonskin malignancies were compared between treatments using a survival analysis. Mean annualized rates of skin malignancy were calculated, and the relative risk was determined using a Poisson model. Malignancy-free survival rates for nonskin malignancies were compared using Kaplan-Meier estimates and the log-rank test. At 5 yr, the median time to a first skin carcinoma was delayed (491 versus 1126 d; log-rank test, P = 0.007), and the risk for an event was significantly lower with SRL-ST therapy (relative risk SRL-ST to SRL-CsA-ST 0.346; 95% confidence interval 0.227 to 0.526; P < 0.001, intention-to-treat analysis). The relative risks for both basal and squamous cell carcinomas were significantly reduced. Kaplan-Meier estimates of nonskin cancer were 9.6 versus 4.0% (SRL-CsA-ST versus SRL-ST; P = 0.032, intention-to-treat analysis). Nonskin cancers included those of the lung, larynx, oropharynx, kidney, gastrointestinal tract, prostate, breast, thyroid, and cervix as well as glioma, liposarcoma, astrocytoma, leukemia, lymphoma, and Kaposi's sarcoma. Patients who received SRL-based, calcineurin inhibitor-free therapy after CsA withdrawal at month 3 had a reduced incidence of both skin and nonskin malignancies at 5 yr after renal transplantation compared with those who received SRL therapy combined with CsA. Longer follow-up and additional trials are needed to confirm these promising results.

Adult↗