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PubMed2002

Prognostic significance of grading and staging systems using MIB-1 score in adult patients with soft tissue sarcoma of the extremities and trunk.

BACKGROUND: The predictive value of histologic grading and staging systems for overall survival in different types of adult soft tissue sarcoma of the extremities and trunk is unclear. METHODS: Histologic slides from 193 patients with primary tumors were reviewed for diagnosis, and Ki-67 (MIB-1) immunostaining was performed for grading in all patients. Univariate and multivariate analyses were conducted to analyze the results from patients with soft tissue sarcomas as a group and among the six main histologic categories: malignant fibrous histiocytoma (n = 49 patients), liposarcoma (n = 48 patients), synovial sarcoma (n = 30 patients), spindle cell sarcoma (n = 24 patients), small round cell sarcoma (n = 15 patients), and others (n = 27 patients). The median follow-up was 50 months. RESULTS: Univariate analysis of soft tissue sarcomas showed that tumor size and depth, histologic type, MIB-1 score, grades based on three criteria (tumor differentiation/histologic type, necrosis, and either mitosis or MIB-1 score) and disease stage, as assessed by tumor size, depth, and grade, were associated with overall survival. Among these variables, grading and staging systems using the MIB-1 score had better predictive value compared with the MIB-1 score and standard grading and staging models in the main histologic categories. Because survival curves for the different tumor grades and stages showed similar trends between the different histologic types, multivariate analysis was conducted adjusting for age, gender, disease site, surgical margin, tumor size and depth, grade, stage, and histologic type. Consequently, Grade 3 emerged as the most significant adverse prognostic factor. Additional adverse prognostic factors were Stage III, Grade 2, a histologic type of spindle cell sarcoma, and patient age > 50 years at the time of presentation. The histologic type liposarcoma was identified as a favorable prognostic factor. CONCLUSIONS: The current results indicate that grading and staging systems using the MIB-1 score are very strong prognostic factors in patients with the main histologic types of soft tissue sarcoma. Specific assessment of histologic type should be carried out before deciding on treatment strategies.

Adolescent↗
PubMedDate not supplied

[Leiomyosarcoma and liposarcoma: rare tumors of the kidney].

Sarcoma accounts for 2.8% of all renal tumours. We report a case of renal leiomyosarcoma (T2 N0 M0) and a case of renal liposarcoma (T3 N0 M0) submitted to surgery and chemo- and radiotherapy. Follow-up at nine months and 4 years, respectively, revealed the patients are tumor-free. The literature is reviewed highlighting the beneficial effect of chemo- and radiotherapy combined with surgery in the treatment of these tumors with a poor prognosis per se.

Adolescent↗
PubMed1995

[Paratesticular neoplasms of mesenchymatous origin. Clinico-pathologic study].

There is a group of malignant intrascrotal tumours which represent a very small number when compared to testicular neoplasias and which are known as paratesticular neoplasias. More than 90% belong to the sarcomatous species. From a clinical point of view and based on the patient's age, two groups can be distinguished: sarcomas affecting children and adolescents (primarily rabdomiosarcomas) and those occurring in adulthood, the more frequent histopathological variants being fibrosarcoma, leiomiosarcoma and liposarcoma. This work contributes five paratesticular sarcomas (two rabdomiosarcomas, one liposarcoma, one leiomiosarcoma and one fibrosarcoma). The features related to histogenesis, diagnosis (revealing signs and clinical examination), natural history (dissemination routes based on histopathology) and treatment are reported. With regard to treatment, both the local surgery (total orchiectomy by inguinoscrotal approach with or without hemiscrotectomy), and indications for lymphadenectomy and radiotherapy in relation to staging are discussed though insisting on the chemotherapy approach.

Adolescent↗
PubMed2002

Pleomorphic liposarcoma: clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases: a study from the French Federation of Cancer Centers Sarcoma Group.

The clinicopathologic and immunohistochemical features of 63 pleomorphic liposarcomas are presented. There were 35 men and 28 women (median age 63 years; range 18-93 years). Tumor size ranged from 2 to 23 cm (median 10 cm). Tumor locations included lower extremity (36.5%), especially the thigh (28.5%), limb girdles (17.5%), upper extremity (16%), thoracoabdominal wall (9.5%), and internal trunk (20.5%). A total of 75% were deep seated and/or extracompartmental. Histologically, lesions show a varying combination of lipogenic and nonlipogenic areas characterized by malignant fibrous histiocytoma-like, round cell liposarcoma-like, and/or epithelioid/carcinoma-like features. A pericytic pattern was focally present in 15 (24%) tumors. Eighteen (29%) lesions were grade 2, and 45 (71%) were grade 3 sarcomas. Tumor necrosis was observed in 51 (81%) cases, vascular invasion in three, and mitotic counts ranged from 3 to 124 per 10 high power fields (median 25). Lipogenic areas were S-100 protein immunoreactive, at least focally, in 20 of 42 (48%) cases. Nonlipogenic areas showed focal reactivity for smooth muscle actin (24 of 49; 49%), desmin (9 of 48; 19%), CD34 (18 of 45; 40%), S-100 protein (5 of 49, 10%), CD68 (6 of 46, 13%), and epithelial membrane antigen (13 of 49, 26.5%). Epithelioid areas showed epithelial membrane antigen (4 of 11; 36%) but not cytokeratin (0 of 11) reactivity. Treatment procedures in 51 patients consisted of simple tumorectomy (16) and wide excision (33). Five and 31 patients received neoadjuvant and adjuvant chemotherapy and/or radiation therapy, respectively. Follow-up (48 patients, range 7-276 months; median 38 months) showed a 45% local recurrence rate and a 42.5% metastasis rate, metastases occurring mostly in lungs and pleura. Seventeen patients (35%) died of disease, of whom none was metastatic at diagnosis. Five-year overall, metastasis-free, and local recurrence-free survivals were 57%, 50%, and 48%, respectively. Patient age > or =60 years, truncal tumor location, deep situation, tumor size >5 cm, vascular invasion, and incomplete tumor excision were significant adverse prognostic factors. Tumor grade and histology did not affect patient outcome. In conclusion, pleomorphic liposarcoma is a rare, often deep-seated and limb-based aggressive and metastasizing neoplasm of late adulthood. It shows a wide range of morphologic appearances, but tumor grade and histology have no effect on patient outcome.

Adolescent↗
PubMed1994

Atypical and malignant lipomatous lesions of the head and neck.

Although liposarcoma is the second most common soft-tissue sarcoma in adults, the incidence of liposarcoma of the head and neck is low. There are only 83 reported cases of head and neck liposarcoma. We report four cases of atypical and malignant lipomatous lesions of the head and neck and discuss their histologic classification and treatment implications. The histologic nature of liposarcoma is correlated clinically with treatment outcome. Recently, many authors have adopted the term atypical lipoma to describe well-differentiated lipomatous lesions in superficial extremity locations because of their tendency toward local recurrence only, without metastases or patient mortality. Although we accept the classification atypical lipoma for superficial, well-differentiated lesions, we believe that histologically similar lesions in nonsuperficial locations in the head and neck are best designated "well-differentiated liposarcoma."

Adult↗
PubMed1987

Limb sparing operations for sarcomas of the extremities involving critical arterial circulation.

Seven patients underwent resection of a sarcoma of the extremity requiring excision of a vital artery and revascularization. These included osteosarcoma of the pubis, osteosarcoma of the distal femur, undifferentiated sarcoma of the thigh, liposarcoma of the thigh, liposarcoma of the popliteal space, chondrosarcoma of the proximal tibia and rhabdomyosarcoma of the thumb. Preoperative evaluation included computed tomographic scan, magnetic resonance imaging and angiography. Operation involved excision of the iliac artery in one instance, femoral artery in three, popliteal artery in two instances and radial artery in one. Wide local resection including revascularization was undertaken when the tumor could be resected with the artery but separated from the nerve and it was a low grade malignant disease or the patient refused amputation for a high grade malignant disease. In five patients, the tumor margins were adequate. One patient with an osteosarcoma of the pubis with distant disease had a palliative resection and one patient had positive margins but refused amputation. Coverage of the soft tissue and vascular grafts was achieved using a distant pedicle flap in two patients. Amputation was avoided and each patient remained ambulatory. Five patients remained free of disease with patent grafts at six months to six years of follow-up study. One patient died of late myocardial infarction and one who underwent palliative resection died eight months later of metastatic disease. Involvement of the major arterial circulation does not preclude adequate resection of sarcomas of the extremity with limb salvage.

Adult↗
PubMed1988

Histological changes in recurrent soft tissue sarcomas: analysis of 56 patients.

Histological changes in 56 patients with recurrent soft tissue sarcomas (STS) were analysed. The original tumors included 23 malignant fibrous histiocytomas, five liposarcomas, four leiomyosarcomas, four synovial sarcomas, four neurogenic sarcomas, four dermatofibrosarcomas and 12 other assorted tumor types. The histological features of the recurrent tumors which underwent changes included increased cellularity, an increased number of mitotic figures, extension of sclerotic areas and the appearance of a storiform pattern; these were found in 30%, 43%, 41% and 29% of cases, respectively. No case showed a histological change from one subtype to another. A follow-up study revealed increased cellularity and mitotic counts in the recurrent tumors to be signs of an unfavorable prognosis.

Adolescent↗
PubMed1983

Pleomorphic lipoma: a tumour simulating liposarcoma.

Pleomorphic lipoma or giant-cell lipoma is a recently recognized entity affecting predominantly elderly and middle-aged men. The neck, shoulder region and back are the sites of predilection. The clinical setting is similar to that seen with spindle-cell lipoma. The lesion is characterized by an intricate mixture of mature fibrous tissue, adipose tissue and myxoid tissue interspersed with cellular foci. Most characteristic of the latter are a variety of giant cells and especially the 'floret' giant-cells, so named because of the arrangement of their nuclei which is reminiscent of the petals of a flower. The pleomorphism of the lesion frequently leads to misdiagnosis as liposarcoma. Criteria for the differentiation from the various types of liposarcoma are discussed. The possible relationship of pleomorphic lipoma to some of the 'atypical lipomas' described in the recent literature is analysed. The pleomorphic lipoma is a benign lesion of subcutaneous tissue which must be sharply differentiated from sarcomas. Some of the liposarcomas alleged in the literature to have originated within a pre-existing lipoma possibly represented pleomorphic lipomas. Pleomorphic lipoma is an entity which must be added to the growing number of pseudosarcomatous lesions of soft tissue.

Adipose Tissue↗
PubMed1998

Immunohistochemical analysis of Fas ligand expression in sarcomas. Sarcomas express high level of FasL in vivo.

Fas ligand (FasL) and its receptor, Fas, play a key role in the regulation of apoptosis within the immune system. Several prior experimental studies of Fas ligand expression in tumors have suggested a mechanism that enables tumors to evade immune destruction by inducing apoptosis in activated lymphocytes near the tumor cells. Many types of carcinomas have been shown to express FasL, but at present nothing is known about the range of sarcomas capable of expressing FasL in vivo. The aim of this study was to determine the in vivo patterns of FasL expression in human sarcomas. Archival paraffin-embedded tissues of 57 sarcomas and 30 carcinomas were analyzed by immunohistochemistry for the expression of FasL. FasL immunoreactivity was seen in 39 of 57 (68%) sarcomas, including 10 of 10 rhabdomyosarcomas, 5 of 5 malignant schwannomas, 2 of 2 Ewing's sarcomas, 8 of 11 malignant fibrous histiocytomas, 4 of 5 angiosarcomas, 2 of 5 synovial sarcomas, 2 of 5 liposarcomas, 3 of 5 leiomyosarcomas, 2 of 6 osteosarcomas, and 1 of 3 chondrosarcomas. All carcinomas tested (10 gastric adenocarcinomas, 10 hepatocellular carcinomas, and 10 renal cell carcinomas) were positive for FasL. These results demonstrate that FasL expression in sarcomas, although less frequent than in carcinomas, is widespread among the sarcoma types, and suggest that FasL might contribute to the immune escape of sarcomas through killing Fas-bearing lymphocytes.

Apoptosis↗
PubMed1994

Cytogenetic intratumor heterogeneity in soft tissue tumors.

Multiple (two to seven) samples, obtained from the same surgical specimen or at different occasions, were analyzed in 54 benign and malignant soft tissue tumors, to investigate cytogenetic clonal evolution. In 28 tumors only normal karyotypes were found. Ten tumors had abnormal karyotypes, but were noninformative, most often due to a high level of karyotypic complexity or great cell-to-cell variation. Sixteen tumors were informative: four (leiomyosarcoma, liposarcoma, malignant Schwannoma, and a benign mesenchymal tumor, probably leiomyoma) had identical karyotypes in different samples, whereas the remaining 12 tumors (seven malignant fibrous histiocytomas [MFH], two leiomyosarcomas, two liposarcomas, and one synovial sarcoma) displayed intersample heterogeneity. Also, intrasample heterogeneity was detected; more than one clone was found in 21 of 73 samples with aberrations from 26 tumors. The different clones were related in all cases except two. In seven cases, samples from different occasions were studied, and clonal evolution could be evidenced in five of them, whereas in two cases the karyotypes remained unchanged. The results indicate that the acquisition of ring chromosomes is an early event in the development of MFH and possibly also pleomorphic liposarcoma. The findings, together with previous data, also indicate that rearrangements of 19p13 are late events in the progression of pleomorphic sarcomas. The overall conclusion from this study is that cytogenetic heterogeneity is common in soft tissue tumors, and that this might influence the evaluation of cytogenetic and molecular genetic findings.

Adult↗
PubMed1997

Soft tissue tumours: the impact of cytogenetics and molecular genetics.

Cytogenetic and molecular analysis of soft tissue tumours has yielded a wealth of new information over the past 10-15 years. Many soft tissue neoplasms show specific karyotypic aberrations which have proved to be diagnostically valuable, and have also assisted in the understanding of pathogenetic mechanisms and rationalisation of classification systems (e.g. lipomatous tumours and Ewing's sarcoma/PNET). In certain clinical subsets, especially round cell sarcomas and fatty neoplasms, determination of karyotype (whether by conventional analysis, FISH or RT-PCR) has proved often to be useful in the diagnostic setting. Additionally the recognition of clonal abnormalities in both benign neoplasms as well as lesions formerly thought to be non-neoplastic (e.g. inflammatory "pseudotumour") has prompted reassessment of biologic concepts with regard to growth control. Inherited molecular genetic defects which predispose to soft tissue neoplasia (e.g. NF-1, Li-Fraumeni syndrome) have been characterised, leading to a greater understanding of tumour suppressor genes. Mesenchymal differentiation genes, the modes of action of which may help to expunge concepts of histogenesis, are being characterised. It is becoming clear that there exist growth control genes (such as the HMGI family) which, irrespective of differentiation, play an important role in a wide range of different mesenchymal tumours. Additionally it is evident that different histologic types of sarcoma (e.g. variants of liposarcoma) show quite different abnormalities of cell cycle control (notably at the G1-S checkpoint) and it seems increasingly likely that certain genetic aberrations, identifiable either at the chromosomal or individual gene level, may prove to be of prognostic relevance in sarcomas and may also open novel therapeutic avenues. While the validity of all molecular genetic data depends totally on skilled histological diagnosis and grading, there has never been a better time for close collaboration between pathologists and basic scientists in the study of soft tissue neoplasia.

Cell Cycle↗
PubMed1994

Immunohistochemical detection of bone morphogenetic proteins in bone and soft-tissue sarcomas.

BACKGROUND: Bone morphogenetic proteins (BMPs) are potent inducers of bone formation. Functional and immunohistochemical studies have identified BMPs in a subset of osteosarcomas. In the present study, the authors extend the analysis of BMP expression to other bone and soft tissue sarcomas. METHODS: Monoclonal antibody AbH3b2/17 against human BMP-2 and BMP-4 was used in avidin-biotin-immunoperoxidase assays with frozen sections of bone tumors (71 specimens), soft tissue sarcomas (69 specimens), and normal tissues. RESULTS: Among bone tumors, BMP was detected in osteosarcomas (17 of 29 samples), malignant fibrous histiocytomas (MFHs) (6 of 6), and the spindle cell sarcomatous components of spindle cell (dedifferentiated) chondrosarcomas (4 of 4), but not in conventional chondrosarcomas (0 of 10) or Ewing's sarcomas (0 of 14). Histologic subtypes of osteosarcoma differed for BMP expression, with 8 of 9 fibrohistiocytic, 9 of 13 osteoblastic, and 0 of 5 chondroblastic lesions showing immunostaining. In all BMP-positive bone tumors, immunostaining was localized in the cytoplasm of primitive mesenchymal cells, with little or no staining in tumor matrix and more mature osteoblastic/chondrocytic cells. Among soft tissue sarcomas, MFHs (11 of 12), liposarcomas (5 of 11), leiomyosarcomas (3 of 9), and malignant schwannomas (3 of 8) showed cytoplasmic BMP immunostaining. Synovial sarcomas (0 of 9), rhabdomyosarcomas (0 of 8), and fibrosarcomas (0 of 7) were BMP-negative. All normal human tissues tested, including the tissues of a 16-week-old fetus, lacked BMP immunoreactivity. CONCLUSIONS: Bone morphogenetic protein is expressed in a subset of osteosarcomas, a high proportion of MFHs of bone and soft tissue, and in spindle cell chondrosarcomas. In these tumors, BMP is localized predominantly to the cytoplasm of malignant cells with primitive mesenchymal features; no or little BMP is detected in the more differentiated elements of bone and soft tissue sarcomas. Different histologic types of bone and soft tissue sarcomas may mimic discrete stages of mesenchymal differentiation as defined by BMP expression and histologic criteria.

Antibodies, Monoclonal↗
PubMed1970

Microcytotoxicity test: detection in sarcoma patients of antibody cytotoxic to human sarcoma cells.

A microcytotoxicity test has been used to detect a factor cytotoxic for human sarcoma cells; the factor was found in serums from 70 percent of sarcoma patients, 58 percent of their family members, and 8 percent of serums from normal blood donors. This cytotoxin is an antibody against a common cell surface sarcoma antigen since it is specific for sarcoma cells, is complement dependent, and is extractable with the globulin fraction of serum.

Antibodies↗
PubMed1998

Cytologic features of sarcomas in fluids.

BACKGROUND: Sarcomas account for < 6% of malignant effusions and their diagnosis usually is made in the setting of a known primary tumor. However, these tumors often exhibit a variety of features that can differ from those of the original neoplasm and may preclude the correct diagnosis. This article evaluates the cytomorphology of sarcomas in fluids and determines characteristic features for identification and classification. METHODS: The study included 24 Papanicolaou-stained fluid samples (15 pleural, 8 peritoneal, and 1 pericardial) from patients with a diagnosis of sarcoma. The following features were evaluated: cellular arrangement, cellularity, nuclear/cytoplasmic ratio, background, quality and quantity of cytoplasm, cell borders, and nuclear details. RESULTS: All cytology specimens were diagnosed accurately as malignant. The histopathologic diagnoses were eight malignant fibrous histiocytomas, five leiomyosarcomas, three rhabdomyosarcomas, three liposarcomas, two high grade sarcomas, one osteogenic sarcoma, one synovial sarcoma, and one chondrosarcoma. Cytomorphologic features shared by sarcomas in metastatic sites included single cell arrangement (23 of 24; 95.8%), indistinct cell borders (18 of 24; 75.0%), nuclear pleomorphism (18 of 24; 75.0%) multinucleation (13 of 24; 54.2%), and proteinaceous background with lysed blood (17 of 24; 70.8%). CONCLUSIONS: Sarcomas in effusions share morphologic features that allow their correct diagnosis. Further subclassification can be attempted in the proper clinical setting and by comparison with the primary lesion.

Adolescent↗
PubMed2005

[Soft tissue sarcomas: current data in the field of pathology].

Over the last fifteen years, pathology underwent significant changes in the field of soft tissue tumours. They were related to considerable advances in molecular biology and genetics. New data led to the revision of the WHO classification. Malignant fibrous histiocytoma is no longer considered as an entity. It has split up into several subgroups belonging to liposarcomas, leiomyosarcomas or undifferentiated sarcomas. Haemangiopericytoma underwent reappraisal and was put in the same category as solitary fibrous tumour. Many tools have improved. Immunohistochemistry performed with new antibodies had its specificity increased, and became appropriate for the prediction of therapeutic response in some cases, e.g. CD117 detecting mutations of the c-kit proto-oncogen in gastro-intestinal stromal tumours. Refinement of the techniques allows accurate diagnoses from core needle biopsies. Surgical specimens are collegially examined by surgeons and pathologists with special attention paid to resection margins. Although bound by some limitations, the grading system of the French Federation of Cancer Centers has currently remained the best predictor of metastasis-free survival and overall survival of patients. It is based on an assessment of three parameters: differentiation, amount of necrosis, and mitotic count of tumours. The pathologist sets up a diagnosis, and actively takes part in the prediction of the prognosis and therapeutic response. He is one of the major participants in decision making for multimodal treatment of sarcomas.

Humans↗
PubMed1986

Fast neutron radiotherapy for soft tissue sarcomas. University of Washington experience and review of the world's literature.

Sixteen patients with inoperable soft tissue sarcomas were treated definitively with fast neutrons at the University of Washington between August, 1970 and May, 1982. Eleven of these 16 patients were treated with curative intent and form the basis of this report. Actuarial plots are shown for local tumor control and survival. This work is placed in the context of worldwide experience in using fast neutrons to treat unresectable soft tissue sarcomas.

Fast Neutrons↗
PubMed1999

Inhalation chemotherapy for macroscopic primary or metastatic lung tumors: proof of principle using dogs with spontaneously occurring tumors as a model.

This study represents part of an effort to determine the safety and efficacy of inhaled antineoplastic drugs, using pet dogs with spontaneously arising primary and metastatic lung cancers (including sarcoma, carcinoma, and malignant melanoma) as a model. Dogs received new formulations of either paclitaxel (PTX) or doxorubicin (DOX) by the inhalation route every 2 weeks using a specially designed aerosol device. Response was assessed radiographically using the indices of tumor nodule number and volume measurement of discrete pulmonary nodules. Dogs experiencing progressive disease after two consecutive treatments were crossed over to receive the alternate compound. In 24 dogs, 6 (25%) responses were noted including 5 partial responses (PR) and 1 complete response. These include 4 (22.2%) of 18 responses to DOX and 2 (13.3%) of 15 responses to PTX. Responses were noted with osteosarcoma (including three dogs with metastatic osteosarcoma that had failed prior systemic chemotherapy), liposarcoma, hemangiosarcoma, and undifferentiated sarcoma. One dog with mammary carcinoma experienced a 47% reduction in volume after PTX inhalation, just shy of PR criteria. One dog with liposarcoma is experiencing a long-term (>12 months) stabilization of disease on PTX. To date, no systemic toxicities have been observed with either PTX or DOX inhalations. Local (pulmonary) toxicity was not observed with PTX; however, changes consistent with pneumonitis/fibrosis were observed in some dogs receiving DOX. Only one of these dogs showed clinical signs, which were responsive to steroid and antitussive therapy. These data represent "proof of principle" for the avoidance of systemic toxicity while delivering efficacious local drug levels by the inhalation route.

Administration, Inhalation↗