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PubMed1987

Primary retroperitoneal tumours, in the material of our department.

After giving a brief survey of the literature, the authors discuss the symptomatology, diagnostics and management of retroperitoneal tumours. Four of their own cases treated between 1979 and 1985 summarized in tables are presented. All of them were operated and three of them received irradiation therapy. The fate of the first patient is unknown. The second patient survived for 24 months following the first operation, with additional three operations and irradiation on two occasions for recurrence of tumour. The third patient survived for 5 months. In the fourth, it could be revealed by ultrasound and angiography that the retroperitoneal tumour was independent of the kidney. Thus, the intact left kidney was preserved during operation. The patient died three months postoperatively at the department of pulmonology. The histological finding of the tumours of all patients was a malignant tumour of mesenchymal origin, a type of sarcoma. Based on their experiences, the authors stress the importance of the early recognition and removal of the tumour, since the patients have a poor chance of surviving these extremely malignant sarcomas in spite of the surgical and irradiation therapies.

Adult↗
PubMed1978

Malignant fibrous histiocytoma: an analysis of 200 cases.

The clinicopathologic findings in 200 cases of malignant fibrous histiocytoma (MFH) with follow-up information are presented. This tumor occurred principally as a mass on an extremity (lower extremity 49%, upper extremity 19%) or in the abdominal cavity or retroperitoneum (16%) of adults (peak incidence 61-70 years of age). It typically involved deep fascia (19%) or skeletal muscle (59%) and only rarely was confined to the subcutis without fascial involvement (7%). The MFH had variable morphologic features and frequently showed transitions from areas having a highly ordered storiform pattern to less differentiated areas having a pleomorphic appearance. The rate of local recurrence of the tumor was 44%, and of metastasis, 42%. Metastasis was most frequently to the lung (82%) and lymph nodes (32%). Factors that influenced the rate of metastasis included depth, size, and inflammatory component of the tumor. Tumors that were small, superficially located, or had a prominent inflammatory component metastasized less frequently than larger, more deeply located tumors. In our experience the MFH is the most common soft tissue sarcoma of late adult life, and many tumors previously diagnosed as pleomorphic variants of liposarcoma, fibrosarcoma, or rhabdomyosarcoma are probably examples of MFH. Although the histogenesis of this neoplasm remains controversial, we feel it is best regarded as a primitive and pleomorphic sarcoma showing partial fibroblastic and histiocytic differentiation, as reflected by collagen production and occasional phagocytosis.

Adolescent↗
PubMed1998

Cytogenetic analysis of 46 pleomorphic soft tissue sarcomas and correlation with morphologic and clinical features: a report of the CHAMP Study Group. Chromosomes and MorPhology.

With the aim of identifying objective cytogenetic-morphologic correlations, we evaluated 46 pleomorphic soft tissue sarcomas (mainly diagnosed originally as malignant fibrous histiocytomas) with clonal chromosome aberrations both cytogenetically and morphologically as part of an international collaborative study. By detailed histopathologic examination, most cases could be categorized into specific tumor types. Eight sarcomas were diagnosed as lipogenic (4 pleomorphic, 1 combined pleomorphic and myxoid/round cell, and 3 dedifferentiated liposarcomas), 19 as myogenic [11 leiomyosarcomas, 1 rhabdomyosarcoma, 4 myosarcomas not otherwise specified (NOS), and 3 probable myosarcomas NOS], 8 as myxofibrosarcomas, 1 as a malignant peripheral nerve sheath tumor, 1 as malignant mesenchymoma, 1 as extraskeletal osteosarcoma, I as sarcoma resembling proliferative fasciitis, and 7 as pleomorphic sarcomas NOS. In a three-grade system, 10 tumors were grade 2 and 36 were grade 3. The majority had highly complex karyotypes. A total of 24 recurrent abnormalities (defined by their presence in at least five cases) were detected: ring chromosomes, homogeneously staining regions (hsr) and/or double minute chromosomes (dmin), and structural rearrangement of 22 different chromosome bands or regions. The frequency and distribution of the recurrent karyotypic features were uneven. Grade 3 tumors displayed, on average, more aberrations per case than did grade 2 tumors. Nine of the selected abnormalities, including hsr/dmin and rearrangements of 19p13 and 19q13, were found only among the high-grade tumors. When the tumors were subdivided according to lineage of differentiation, the highest frequency of aberrations was seen in pleomorphic sarcomas NOS, followed by myxofibrosarcomas, myogenic sarcomas, and lipogenic sarcomas. None of the selected rearrangements was, however, specific for any of these subgroups. The sole consistent cytogenetic-morphologic association was that all three dedifferentiated liposarcomas had multiple abnormal clones, at least one of which contained supernumerary ring chromosomes. Due mainly to karyotype complexity, it therefore seems unlikely that cytogenetic analysis can assist in the differential diagnostic subclassification of pleomorphic sarcomas, nor was there any clear-cut indication that the karyotypic picture could be used to predict clinical outcome. Although the mean number of recurrent chromosome aberrations was almost twice as high in sarcomas that gave rise to metastases as among those that did not, no particular aberration was restricted to either of the two subgroups.

Adult↗
PubMed1996

Isolated limb perfusion with tumor necrosis factor and melphalan for limb salvage in 186 patients with locally advanced soft tissue extremity sarcomas. The cumulative multicenter European experience.

OBJECTIVE: The objective of the study was to achieve limb salvage in patients with locally advanced soft tissue sarcomas that can only be treated by amputation or functionally mutilating surgery by performing an isolated limb perfusion (ILP) with tumor necrosis factor (TNF) + melphalan (M) as induction biochemotherapy to obtain local control and make limb-sparing surgery possible. SUMMARY BACKGROUND DATA: To increase the number of limb-sparing resections in the treatment of locally advanced extremity soft tissue sarcoma, preoperative radiation therapy or chemotherapy or a combination of the two often are applied. The ILP with cytostatic agents alone is another option but rarely is used because of rather poor results. The efficacy of the application of TNF in ILP markedly has changed this situation. METHODS: In 8 cancer centers, 186 patients were treated over a period of almost 4.5 years. There were 107 (57%) primary and 79 (43%) recurrent sarcomas, mostly high grade (110 grade III; 51 grade II; and 25 very large, recurrent, or multiple grade I sarcomas). The composition of this series of patients is unusual: 42 patients (23%) had multifocal primary or multiple recurrent tumors; median tumor size was very large (16 cm); 25 patients (13%) had known systemic metastases at the time of the ILP. Patients underwent a 90-minute ILP at 39 to 40 C with TNF + melphalan. The first 55 patients also received interferon-tau. A delayed marginal resection of the tumor remnant was done 2 to 4 months after ILP. RESULTS: A major tumor response was seen in 82% of the patients rendering these large sarcomas resectable in most cases. Clinical response rates were: 33 complete response (CR) (18%), 106 partial response (PR) (57%), 42 no change (NC) (22%), and 5 progressive disease (PD) (3%). Final outcome was defined by clinical and pathologic response: 54 CR (29%), 99 PR (53%), 29 NC (16%), and 4 PD (2%). At a median follow-up of almost 2 years (22 months; range, 6-58 months), limb salvage was achieved in 82%. Regional toxicity was limited and systemic toxicity minimal to moderate, easily managed, with no toxic deaths. CONCLUSIONS: In the setting of isolated limb perfusion, TNF is an active anticancer drug in patients. The ILP with TNF + melphalan can be performed safely in many centers and is an effective induction treatment with a high response rate that can achieve limb salvage in patients with locally advanced extremity soft tissue sarcoma.

Adolescent↗
PubMed1989

Solid tumor cytogenetics. Progress since 1979.

Some of the advances in the past decade in the field of solid tumor cytogenetics are described, with particular reference to nonrandom structural chromosome changes. Although it had been known for many years that meningiomas and salivary gland tumors were associated with changes involving particular chromosomes, it has only quite recently become clear, following the application of suitable culture techniques, that other benign tumors such as lipomas and leiomyomas may also be characterized by specific changes, particularly reciprocal translocations. Reciprocal translocations may also be found in malignant soft-tissue tumors such as liposarcomas (involving 12q as in lipomas) and Ewing's sarcoma. In contrast, the common forms of carcinoma present a more variable picture, although certain chromosomes may undergo nonrandom changes of various types, including translocations, which, however, are generally nonreciprocal. Some of these chromosomes may be quite specific (e.g., chromosome 10 in prostatic and #18 in colorectal cancer), while others appear to be common to many or all types of carcinoma, such as chromosomes 1, 3, 11, and 17, and a small metacentric that may be an i(5p). In carcinoma of the bladder, different chromosome changes may characterize subsets of the tumors. In carcinoma of the cervix, however, the commonly involved chromosomes, 1, 3, ?5, 11, and 17, appear in markers in any combination and are thus not mutually exclusive. Although further study of the chromosome changes in carcinomas is essential to an understanding of their relationship to the molecular changes that are associated with malignant transformation, it can be hypothesized that, while some of the changes result in the duplication of particular genes, e.g., on chromosome 1q, a more important role may be to bring about the loss of chromosomal segments containing tumor-suppressor genes. Evidence from molecular studies that has recently been accumulating for the loss of alleles on, for instance, 3p, 11p, and 17p, which could in part be due to gross chromosomal rearrangements, also strongly suggests the importance of genic loss in malignant transformation. In carcinomas, at least, the changes probably involve a number of genes, each change representing one of the several steps necessary for tumorigenesis.

Chromosome Aberrations↗
PubMed1999

Unique cytological features and chromosome aberrations in chondroid lipoma: a case report based on fine-needle aspiration cytology, histopathology, electron microscopy, chromosome banding, and molecular cytogenetics.

Chondroid lipoma is a rare, benign tumor that may mimic soft-tissue sarcoma clinically. Its histopathologic features may resemble hibernoma, myxoid liposarcoma, myxoid chondrosarcoma, and other lipomatous or chondroid neoplasms. In this study, a chondroid lipoma was analyzed by fine-needle aspiration cytology, histopathology, electron microscopy, chromosome banding, and metaphase fluorescence in situ hybridization. The results demonstrate that chondroid lipoma exhibits a characteristic pattern by fine-needle aspiration cytology, including a mixture of benign adipose tissue with lipoblastlike cells, and chondroblastlike cells with a fibrochondroid matrix. Cytogenetically, a three-way rearrangement between chromosomes 1, 2, and 5 was found, together with an 11;16 translocation with a breakpoint in 11q13, approximately 1 Mb proximal to the MEN1 region shown to be rearranged frequently in hibernoma. The presence of a karyotype of low complexity, but without any of the genetic aberrations characteristic for other types of soft-tissue tumors, indicate that chondroid lipoma develops along a unique pathogenetic pathway.

Adult↗
PubMed2001

Phase I and pharmacokinetic study of ecteinascidin-743, a new marine compound, administered as a 24-hour continuous infusion in patients with solid tumors.

PURPOSE: To define the maximum-tolerated dose (MTD) and the phase II recommended dose (RD) of ecteinascidin-743 (ET-743) given as a 24-hour continuous infusion every 3 weeks to patients with treatment-refractory solid tumors. PATIENTS AND METHODS: Fifty-two patients received a total of 158 cycles of ET-743 at one of nine dose levels (DLs) ranging from 50 to 1,800 microg/m(2). RESULTS: The MTD was defined as 1,800 microg/m(2) (DL 9), and the phase II RD was 1,500 microg/m(2) (DL 8) for moderately pretreated patients with performance status (PS) 0 to 1 and good hepatobiliary function. Neutropenia and thrombocytopenia were the dose-limiting toxicities (DLTs) and were severe at the MTD (1,800 microg/m(2)) in 94% and 25% of cycles, respectively. At the RD (1,500 microg/m(2)), neutropenia and thrombocytopenia were present in 33% and 10% of cycles, respectively. Transient acute elevated transaminase levels occurred in almost all cycles and was severe in 38% of cycles. Severe toxicities and DLTs were observed in patients with poor PS or abnormal liver function or who had received a large number of previous chemotherapy regimens. Antitumor activity was observed at the three highest DLs, including three partial responses (breast cancer, osteosarcoma, and liposarcoma), and four patients (all with progressing soft tissue sarcomas) had stable disease lasting > or = 3 months. Pharmacokinetic studies were performed on all patients for at least the first cycle, giving a linear pharmacokinetic profile; this showed a relationship between area under the curve (AUC) and transaminitis grade and a clear correlation between AUC and severe hematologic toxicity likelihood. CONCLUSION: The RD for a 24-hour continuous intravenous infusion of ET-743 is 1,500 microg/m(2), with the most prevalent DLTs being hematologic. Patients with minor baseline hepatobiliary function abnormalities have a higher likelihood of severe hematologic toxicities and AUC-related DLTs, requiring dose adjustments or delays.

Adult↗
PubMedDate not supplied

Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft tissue). A case report.

A case of a 55 year old patient with a tumour in the inguino-scrotal region infiltrating into the depth of the thigh was reported. The most tumoral cells appeared clear, rich in glycogen and in melanic pigment, with positive reaction for S-100 protein; between tumoral cells fine vasculo-conjunctival axes were observed. Excluding a Grawitz tumour metastasis one made the differential diagnosis with synovial sarcoma, epitheloid form of malignant schwanomas, spindle cell melanomas, liposarcoma, fibrosarcoma, the tumour was included in the distinct oncologic entity described by Enzinger, with neuroectodermal origin. This case is the first published in Romania.

Diagnosis, Differential↗
PubMed1986

Primary liposarcoma of the heart.

A rare form of primary heart tumour, a liposarcoma, is reported. A discussion of diagnosis and modalities of treatment of primary heart sarcomas is presented.

Female↗
PubMed2006

[Unique massive recurrence liposarcoma of the retroperitoneum].

Liposarcoma constitutes a heterogeneous group of tumors which rarely arise from the retroperitoneal area. They are the single most common tumor in all sarcoma of the retroperitoneum space. Surgery plays a dominant role in the treatment of retroperitoneal liposarcoma. The histologic subtype and margin of resection are prognostic for survival in primary tumors. Local recurrences are the most frequent cause of failure of the surgery. We report the case of a patient with massive recurrences ofretroperitoneal liposarcoma. A 45 year-old male had low-grade liposarcoma of the retroperitoneum which recurred 6 times within 10 years. The next retroperitoneal recurrence tumor excised weighted 25 kg in total. Seven months after resection the patient feels well. We conclude that aggressive surgery in recurrent tumors can produce prolonged survival in patients with low-grade retroperitoneal liposarcoma.

Follow-Up Studies↗
PubMed1984

An evaluation of reports of dioxin exposure and soft tissue sarcoma pathology among chemical workers in the United States.

A review of employment records and tissue specimens of seven workers, reported previously as having occupational dioxin exposure and soft tissue sarcomas, confirms that four workers had employment of 2 to 19 years in the production of 2,4,5-trichlorophenoxyacetic acid (2,4,5-T) or trichlorophenol, products contaminated with 2,3,7,8-tetrachlorodibenzodioxin, the most toxic dioxin isomer. Of these individuals, two have confirmed soft tissue sarcomas. In addition three individuals who worked for companies which made 2,4,5-T also have confirmed soft tissue sarcomas. Their employment records do not show specific assignment to 2,4,5-T or trichlorophenol departments; however, one individual worked for 10 d in the production of pentachlorophenol, which is contaminated with different isomers of dioxin. Methodological problems are discussed which must be addressed in the epidemiologic evaluation of the outcome of soft tissue sarcoma.

Chemical Industry↗
PubMed1982

Vascular spindle cell lipoma.

Spindle cell lipoma is a neoplasm of adipose tissue that primarily affects posterior necks and shoulders of men in their fifth to seventh decades. It consists of a mixture of adipose tissue and spindle-shaped cells that could be misinterpreted as sarcoma. A clinical and pathological study of two patients, both men, with vascular variants of spindle cell lipoma is presented. These tumors were unusual clinically, in that one caused pain on motion and the other was painful to palpation. On microscopic examination, their vascularity was augmented over previously described examples of spindle cell lipoma.

Adult↗
PubMed1987

Soft tissue sarcomas apparently arising in chronic tropical ulcers.

Of 720 soft tissue sarcomas received from Malawi over a 15 year period, eight had apparently arisen in chronic tropical ulcers which are endemic in that part of Africa. These eight tumours were classified as leiomyosarcoma (three), extraskeletal osteosarcoma (two), malignant fibrous histiocytoma (one), myxoid liposarcoma (one) and unclassifiable (one). All had a history suggestive of malignant change in a long-standing ulcer, in each case clinically thought to be a squamous carcinoma. However, in none was there evidence of an epithelial origin and all were negative for epithelial membrane antigen and cytokeratin. This association has not previously been reported. The validity of this proposed association is discussed.

Adult↗