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PubMed1979

[Prognosis in malignant tumors of soft tissues].

The author reports the data on 348 patients with malignant tumors of soft tissues, operated upon within the period of 1946--70 inclusively, children being excluded from this number. Various factors were studied from the prognostic viewpoint. The data obtained enabled estimating the morphological tumor structures, other conditions being equal, as a factor of great importance. None of the factors should be regarded separately. The accumulation of observations and systematization of prognostic criteria seems to be absolutely essential. Prognostication in malignant tumors of soft tissues may aid in selecting an adequate extent of surgical intervention, in avoiding too traumatic procedures, thus contributing to patients' rehabilitation.

Fibrosarcoma↗
PubMed1986

Estrogen receptor protein in bone and soft tissue tumors.

Thirty-three histologically diverse bone and soft tissue tumors were analyzed biochemically for the presence of estrogen receptor protein (ERP) and progesterone receptor by means of a conventional, commercially available, steroid-binding assay (dextran-coated charcoal method) on fresh frozen tissue. These results were compared with analysis of ERP by using a specific monoclonal antibody both in an enzyme immunoassay and on frozen tissue sections by using immunohistochemical procedures. Frozen tissue sections were also examined for the presence of estrogen and progesterone receptors using fluorescein-labeled steroids. Six of the 33 tumors (18%) contained low levels of ERP ranging from 19 to 73 fmol/mg as determined by the dextran-coated charcoal method. The remaining 27 cases contained no (less than 10 fmol/mg) ERP. The ERP-positive group included a fibromatosis, leiomyosarcoma, liposarcoma (2 cases), neural sarcoma, and a synovial sarcoma. Four were high grades sarcomas, and two were low grade sarcomas. There was excellent agreement between the ERP levels determined by the dextran coated charcoal method and those determined by enzyme immunoassay. ERP could not be demonstrated immunohistochemically on frozen tissue sections of the tumors even though it could be demonstrated in breast carcinomas serving as positive controls. The failure of the immunohistochemical technique may be related to the low levels of ERP in these tumors and the difficulty of detecting antigen at threshold levels. Cytochemical localization of receptor protein employing fluoresceinated steroids did not correlate with cytosolic ERP as determined by enzyme immunoassay or the dextran coated charcoal method. Moreover, the high level of background fluorescence gave rise to a significant amount of intraobserver and interobserver variation. Although the clinical significance of ERP protein in mesenchymal tumors is still uncertain, the present findings, coupled with various clinical observations suggesting hormonal dependency of some mesenchymal tumors, indicate that investigation of a larger group of patients amenable to statistical analysis is warranted.

Adolescent↗
PubMed1981

[Xeroradiography in radiotherapy of tumors of the bone and soft parts: value in the beginning of therapy in long-term follow-up studies].

Xeroradiography was performed on 38 cases of bone or soft tissue tumors submitted to radiotherapy. The examination was done before, during and after the radiological treatment and at longer intervals after the completion of its course. The indications and the drawbacks of xeroradiography for the follow-up of irradiated cases and for the search of recurrences are discussed, taking also into account the other radiological techniques used in this field. Xeroradiography is held to be very useful in planning the treatment of malignant bone tumors with extraosseous spread, since it allows to establish the real limits of the tumor to be irradiated. Moreover, xeroradiography is helpful to recognize the radiotherapeutic sequelae on superficial soft tissues, as oedema, fibrosis and necrosis of the subcutaneous fat and skin.

Bone Neoplasms↗
PubMed2001

DNA copy number amplifications in sarcomas with homogeneously staining regions and double minutes.

To identify DNA amplifications in sarcomas, comparative genomic hybridization was performed on 27 cases that were likely to display high-level DNA copy number gains. In all cases, chromosome banding analysis had revealed homogeneously staining regions or double minutes, i.e., cytogenetic signs of gene amplification. In most cases, gains predominated over losses. Low-level amplifications (ratio 1.3:1.5) were seen in 20 cases. High-level amplifications (ratio >1.5) exceeded the frequencies seen in published, unselected sarcomas of similar histotypes and were detected in 16 tumors: 4/4 osteosarcomas, 5/8 malignant fibrous histiocytomas, 3/7 leiomyosarcomas, 1/2 myosarcomas, 0/1 liposarcoma, 0/1 rhabdomyosarcoma, 1/1 pleomorphic sarcoma, 0/1 myxofibrosarcoma, 1/1 malignant mesenchymona, and 1/1 malignant schwannoma, with two to four chromosomal regions involved in nine tumors. Recurrent amplifications involved 1p33-p32, 5p15-p14, 7pter-p12, 7q21-qter, 8q21.3-qter, 11q22-q23, 16p13.2-p12, 19q12-q13.1, 20q11.2-qter, and 22q12-q13. Most of the recurrent gains/amplifications we detected have been reported in sarcomas previously. A novel gain/amplification was seen at 2q14.3-q21 in five cases of four sarcoma types. The disparate pattern of amplified sequences, the poor correspondence between the localization of low- and high-level amplifications, and the chromosomal position of homogeneously staining regions suggest the involvement of many genes in the amplifications and that the genes rarely maintain their native position in these tumors.

Adolescent↗
PubMed2006

The usefulness of cytogenetic analysis in fine needle aspirates for the histologic subtyping of sarcomas.

Conventional cytogenetic analysis performed from open biopsy tissue samples may be a useful adjunct for the histologic subtyping of bone and soft tissue sarcomas. However, its diagnostic utility in fine needle aspiration biopsy (FNAB) specimens is unclear. We retrospectively reviewed 24 consecutive FNAB bone and soft tissue sarcoma specimens, procured from 1995 to 2003, in which aspirated material was obtained for cytogenetic analysis. The study sample included eight Ewing sarcomas, six synovial sarcomas, five rhabdomyosarcomas, two myxoid liposarcomas, and one each of myxoid chondrosarcoma, osteosarcoma, and atypical lipoma. Cytogenetic analysis confirmed the t(X;18) in all six synovial sarcomas and the t(11;22) in three Ewing sarcomas. The t(2;13) was strongly suggested in one alveolar rhabdomyosarcoma. For two of these cases (both of which were synovial sarcomas), cytogenetic analysis was necessary for definitive diagnosis. While the positive cytogenetic results were supportive in the remainder, all were initially and accurately subtyped based on cytomorphology and/or immunohistochemistry. Cytogenetic analysis was noncontributory (eg no growth) in 14 sarcoma cases, but excluding the case of atypical lipoma, this did not preclude the rendering of an accurate diagnosis. Cytogenetic analysis can be performed on FNAB specimens from bone and soft tissue sarcomas and may be a useful diagnostic aid in difficult cases. However, when cell block material is available for immunohistochemistry, the majority of such cases are successfully subtyped.

Adolescent↗
PubMed1999

Bone Metastases from Soft Tissue Sarcomas.

The incidence, distribution, time of appearance, and radiologic findings of bone metastases from soft tissue sarcomas, exclusive of lymphomas, were evaluated in 320 patients with soft tissue sarcomas. Thirty patients (9.4%) had evidence of 58 bone metastases. Five of 30 patients presented with metastases, and 25 of 30 patients developed metastases up to 66 months after presentation with a mean time interval of 21.3 months. The incidence of skeletal metastases differed among histologic subtypes of sarcomas; alveolar soft part sarcoma (5 of 8), dedifferentiated liposarcoma (2 of 4), angiosarcoma (2 of 4), and rhabdomyosarcoma (5 of 16) tended to show a higher incidence of bone metastases. The sarcomas metastasized to the regional bones close to the primary tumor in 16 (53%) of 30 patients and to the axial bones in 18 (60%). On conventional radiographs, the osseous metastases demonstrated predominantly osteolytic changes, and evidence of pathological fracture was observed in 31% of 58 metastases.

Journal Article↗
PubMed2005

Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.

BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity. The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period. METHODS: Fifty-two patients age 8-18 years (median 8 years) were observed (1979-2002). Primary sites were on the lower limbs in 41 and on the upper limbs in 11 cases. Clinical TNM and surgical Intergroup Rhabdomyosarcoma Staging systems were adopted. Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy. Aggressive delayed surgery of residual disease, including compartmental resections, was recommended. Radiotherapy was suggested only for patients age > 3 years when surgery was incomplete or not feasible. RESULTS: The evaluation was performed dividing the patients into two categories according to their chemosensitivity. Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5. Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1. Nineteen of 21 patients with CTs-NRSTS were alive without disease: the 5-year overall survival (OS) and progression-free survival (PFS) were 94.4% and 79.3%, respectively; 23 of 31 patients with CTns-NRSTS were alive without disease: 5-year OS and PFS were 75.3% and 68.3%, respectively. Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors. The achievement of complete delayed resection was particularly important for CTns-NRSTS. Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor. CONCLUSIONS: Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS. Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread.

Adolescent↗
PubMed1997

[Liposarcoma of the spermatic cord. Clinical case].

Sarcomas are neoplasms originating from connective tissues of any anatomical region; the sarcoma of spermatic cord is a rare neoplasm with only little more than two hundred cases described in the literature. Rhabdomyosarcomas in childhood and leiomyosarcomas in adults are the most frequent histological types. Liposarcomas, fibrosarcomas and osteosarcomas are less frequent. The spermatic cord liposarcoma originates from the spermatic cord fatty tissue and therefore it consist of adipose cells, fibroblasts and myxomatosus cells; such neoplasms are usually very well differentiated. Diagnosis is suggested by the appearance of a progressively enlarging mass and by echotomography which shows a solid or liquid mass of the spermatic cord. Therapy consists of surgical intervention (orchifunicolectomy) which also allows a correct histological diagnosis. The case of a 56 year-old male who underwent left orchifunicolectomy for a sarcoma of spermatic cord occasionally discovered during surgical repair of a left groin hernia is reported. Six months after the intervention the patient is in good condition and there is no evidence of relapse.

Genital Neoplasms, Male↗
PubMedDate not supplied

[A case of pleomorphic liposarcoma involving a parahypopharyngeal site].

Soft tissue sarcomas are uncommon, accounting for fewer than 1% of malignant neoplasms of the head and neck. The most frequent histotype is malignant fibrohistiocytoma (29%), while the least common is liposarcoma (1%). We report the case of a 20-year-old man with a malignant hypopharyngeal pleomorphic liposarcoma, successfully treated with surgery and adjuvant radio- and chemotherapy. The patient is doing well 8 years after the operation, without any signs of local recurrence or distant metastasis.

Adult↗
PubMed1991

Tumours in Iceland. 14. Malignant tumours of soft tissues. Histological classification and epidemiological considerations.

All malignant tumours of soft tissues diagnosed in Iceland between 1955 and 1988 were reviewed histologically. Of a total of 155 tumours, 129 were, on review, soft tissue sarcomas, while 26 had been erroneously diagnosed and were excluded from the study. In 25% of the tumours the original sarcoma diagnosis was changed to another sarcoma type. Malignant fibrous histiocytoma, liposarcoma and leiomyosarcoma together comprise more than half of all tumours diagnosed. Contrary to previous estimations, according to this study, the age-standardized incidence of soft tissue sarcoma in Iceland is 1.8 per 1000,000 for males and 1.6 per 100,000 per females, which is similar to incidence rates in the other Nordic countries. The majority of sarcomas were of relatively high grade. The tumours were graded using both three and four grades of malignancy. Both systems yielded prognostic information although it was not possible to detect significant differences in survival for grade I and grade II tumours when the four grade system was used. The results of this study show that the epidemiology of soft tissue sarcoma in Iceland is similar to that found in other Western countries.

Age Factors↗
PubMed1992

p53 immunohistochemistry in malignant fibrous histiocytomas and other mesenchymal tumours.

In this study we analysed by immunohistochemistry the expression of p53 protein in 14 malignant fibrous histocytomas (MFHs), 22 other types of sarcoma (eight leiomyosarcomas, four rhabdomyosarcomas, four liposarcomas, two fibrosarcomas, two chondrosarcomas, one malignant schwannoma, and one dermatofibrosarcoma protuberans), and 25 non-malignant mesenchymal lesions (eight dermatofibromas, four cases of nodular fasciitis, three leiomyomas, three fibromatoses, two epithelioid.leiomyomas, two neurofibromas, one schwannoma, one myositis ossificans, and one giant cell tumour of tendon sheath). Four MFHs and nine other types of sarcoma (four leiomyosarcomas, two chondrosarcomas, one liposarcoma, one fibrosarcoma, and one dermatofibrosarcoma protuberans) showed nuclear positivity for p53. Of the benign soft tissue lesions, p53 positivity was observed in two fibromatoses, one nodular fasciitis, and one dermatofibroma. The number of p53-positive cells in these benign lesions was considerably smaller than that in most of the p53-positive sarcomas. The p53 positivity in MFHs and other types of sarcoma indicates that p53 gene alterations may play a part in the neoplastic transformation of these tumours. The occurrence of p53 positivity in benign mesenchymal lesions suggests that sometimes p53 protein may accumulate in cells without an associated malignancy. Because of this, p53 immunoreactivity cannot, by itself, be used as a criterion of malignancy. According to our results, p53 positivity in over 1 per cent of tumour cells in mesenchymal lesions favours malignancy.

Genes, p53↗
PubMed1986

[Mesenchymal (non-epithelial) salivary gland tumors. Analysis of 167 tumor cases of the salivary gland register].

The total material of the Salivary Gland Register (1965-1985) contained 167 mesenchymal tumours with an intra- or paraglandular localisation. This corresponds to 1.5% of all cases of the Salivary Gland Register or to 5% of all salivary gland tumours. Periglandular tumours without relation to the salivary gland tissue, metastatic tumours or malignant lymphomas were not considered in this study. With respect to the localisation, the age and sex distribution and the histopathological classification the following results were obtained: 90% of all mesenchymal tumours were localised in the parotid gland and 10% in the submandibular gland. In lipomas, the percentage of the parotid gland was even 95%; in lymphangiomas, however, it was only 80%. Angiomas occur preferably in the first and second decades and represent in this age period nearly 90% of all mesenchymal tumours. The age peak of lipomas lies in the fifth to sixth decades, whereas neurogenic tumours are distributed relatively equal over the fourth to seventh decades. A sex disposition was seen in lipomas (85% in males), neurinomas (65% in females) and neurofibromas (75% in males). 90% were benign mesenchymal tumours, 10% sarcomas. Within the benign tumours 47.5% were angiomas, 22.5% lipomas, 16% neurogenic tumours and 4% rare benign tumours. In angiomas (79 cases) haemangiomas (50 cases), lymphangiomas (17 cases), mixed haemangiomas and lymphangiomas (9 cases) and haemangiopericytomas (3 cases) could be seen, in neurogenic tumours (27 cases) neurinomas (12 cases), neurofibromas (12 cases) and neurofibromatoses (3 cases), in sarcomas (17 cases) malignant fibrous histiocytomas (5 cases), malignant schwannomas (5 cases), embryonal rhabdomyosarcomas (4 cases) and other rare sarcomas (one case each myxoid liposarcoma, leiomyosarcoma, malignant haemangioendothelioma).(ABSTRACT TRUNCATED AT 250 WORDS)

Hemangioma↗
PubMed1996

Late onset of isolated central nervous system metastasis of liposarcoma--a case report.

Metastatic soft-tissue sarcoma of the central nervous system (CNS) is exceedingly rare. We report a case of a 56-year-old male treated for a right lower extremity liposarcoma at the age of 30 years, whose first recurrence was an intracerebral metastasis occurring 26 years after resection of the primary tumor. Initial treatment of the metastasis with surgical resection and adjuvant radiotherapy was followed rapidly by CNS recurrence in 3 months. Further debulking and interstitial brachytherapy were unsuccessful in controlling disease progression. Clinical presentation and treatment of brain metastases in soft-tissue sarcoma are discussed.

Brachytherapy↗