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Treatment results of fast neutron irradiation in soft tissue sarcomas.

PURPOSE: Surgery is the standard treatment of soft-tissue sarcomas. Adjuvant radiotherapy with photons after less radical resection can improve local control. The rate of tumor control achieved in patients with G1 and G2 soft tissue sarcomas incompletely resected and treated postoperatively with neutron irradiation is similar to that seen in patients undergoing complete tumor resection and adjuvant photon irradiation. PATIENTS AND METHODS: At the Department of Radiotherapy and Radiation Oncology of the University of Münster, 61 patients with soft tissue sarcomas were irradiated postoperatively with fast neutrons. Mainly tumors of low or intermediate malignancy (R0; 27%; R1, 21%; R2, 52%) were treated. Malignant fibrous histiocytoma, liposarcoma, and neurogenic sarcomas dominated. 46 patients were irradiated with fast neutrons alone, and 15 patients were treated with mixed beam therapy (photons and neutrons). RESULTS: The median follow-up period was 44 months. Overall five-year survival probability analysed by Kaplan-Meyer method was 42.5%. The local control rate was 57.7%. 15 patients showed complete remission, 18 patients had a partial remission. Only 11% of the patients showed grade III and IV side effects during neutron irradiation. CONCLUSION: Neutron irradiation is efficocious in treating highly and intermediately differentiated soft tissue sarcomas. The result of surgical resection seems to be a very important prognostic factor for patients with soft tissue sarcomas.

Adult↗

Bulky cervical liposarcoma associated with sleep apnea syndrome.

Sarcomas of the head and neck are relatively infrequent tumors. Liposarcomas represent 10-16% of all soft tissue sarcomas and <5% of them arise from the head and neck. The retropharyngeal area is rarely involved. We present the case of a middle-aged male with clinical symptoms of sleep apnea. A fiber-optic examination revealed a soft submucosal lesion compressing the posterior pharyngeal wall, with partial obstruction of the airway tract. CT showed a large heterogeneous prevertebral mass (6 x 20 cm(2)) occupying the posterior mediastinum. A transoral submucosal biopsy was performed. Histological findings were compatible with a low-grade liposarcoma. The mass was removed in monoblock using a classical laterocervical neck incision. The surgical specimen weighed 500 g. After 24 months of follow-up no signs of tumor recurrence or sleep apnea syndrome were noted.

Biopsy↗

Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma.

The presence of abundant inflammation is uncommon in well-differentiated liposarcomas (WDLPS). We describe ten cases of WDLPS in which an extensive lymphoplasmacytic infiltrate was present to such a degree that the differential diagnoses included inflammatory pseudotumor and Castleman's disease, and in which the lipogenic component could easily be overlooked. The median age (62 years) and tumor location (six retroperitoneal, three head/neck, one paratesticular) was comparable to usual WDLPS, as was the fact that six of ten cases recurred locally, but none metastasized during the period of follow-up (range 13 to 228 months, median 72 months). Key histologic features included (1) nodular lymphoplasmacytic aggregates; (2) intervening paucicellular stroma containing fibroblastic elements, frequently with plasma cell-rich zones and scattered atypical, often multinucleate cells; (3) merging of atypical adipocytic and inflammatory elements; and (4) adjacent clearly defined zones of lipoma-like or, more rarely, sclerosing-type liposarcoma. In recurrences, two cases "reverted" to purely lipoma-like liposarcoma, and two cases dedifferentiated. Immunoperoxidase studies demonstrated that B cells predominated in the lymphoid aggregates, and that the abundant plasma cells were polytypic in nature. We support the use of the term well-differentiated inflammatory liposarcoma for tumors of this type, which should be distinguished from high-grade liposarcoma containing inflammatory cells, and in order to identify a subgroup of WDLPS at high risk for misdiagnosis.

Adipocytes↗

Alterations of cancer-related genes in soft tissue sarcomas: hypermethylation of RASSF1A is frequently detected in leiomyosarcoma and associated with poor prognosis in sarcoma.

Aberrant methylation is a main mechanism of tumor suppressor gene inactivation in carcinogenesis. In this study, the methylation status of RASSF1A, p16, MLH1, MSH2 and ERalpha was investigated in 84 primary soft tissue sarcomas (STSs), including 22 liposarcomas, 18 malignant fibrous histiocytomas (MFHs), 18 leiomyosarcomas, 6 rhabdomyosarcomas, 6 neurogenic sarcomas and several other sarcoma entities. RASSF1A hypermethylation was detected in 17 of 84 (20%) STSs; however, methylation was more frequent in leiomyosarcomas (39%) compared to MFHs (6%; p < 0.015) and liposarcomas (18%). The p16 CpG island was methylated in 22 out of 82 (27%) cases. In 7 out of 81 (9%) STS samples, the promoter of MLH1 was methylated and in liposarcoma the methylation frequency was higher (14%). For MSH2, no hypermethylation was detected. Methylation of ERalpha was detected in 48 of 63 (76%) STSs, but also in 4 of 8 (50%) normal tissue samples. Furthermore, we analyzed mutational activation of K-ras and BRAF. In 4 out of 84 (5%) of STSs, a substitution at codon 599 of BRAF was found; however, no alteration of K-ras was detected. In an univariate Cox proportional-hazards regression model, we found that the risk of a tumor-related death for STS patients with methylated RASSF1A was significantly increased (RR = 2.9; p = 0.037). In summary, our data indicate that inactivation of RASSF1A is a common event in STS, especially in leiomyosarcoma. Thus, the methylation status of cancer-related genes was distinct in different STS and methylation of RASSF1A promoter can serve as prognostic marker in STSs.

Adult↗

From morphological to molecular diagnosis of soft tissue tumors.

Cytogenetic discoveries of balanced translocations in soft tissue tumors have opened the way to molecular genetic definition of these translocations as gene fusions from the late 1980s. Many sarcomas are known to have such fusions, and the demonstration of the fusion transcripts in tumor tissue is of great value in specific diagnosis of synovial sarcoma (SYT-SSX), Ewing sarcoma (EWS-Fli1), clear cell sarcoma (EWS-ATF1), myxoid liposarcoma (FUS-CHOP), and other sarcomas. These translocations are believed to be disease-specific and pathogenetic forces, despite occasional observations to the contrary. Demonstration of SYT-SSX and EWS-ATF1 fusion assists in the diagnosis of synovial and clear cell sarcomas in unusual locations, such as the gastrointestinal tract, where these tumors occur with low frequency. Demonstration of sarcoma translocations and their fusion by different assays is well established; use of in situ hybridization is limited by availability of specific probes. In two exceptional instances, the same translocation and gene fusion occurs in two unrelated diseases: ETV6-NTRK fusion in infantile fibrosarcoma and secretory carcinoma of the breast, and ALK-TPM3 fusion in inflammatory myofibroblastic tumor and large cell anaplastic lymphoma. Thus, the target cell of the genetic change is an important factor to define the resulting disease. Activating mutations in two related receptor tyrosine kinases (RTKs), KIT, and platelet-derived growth factor receptor alpha (PDGFRA) is central to the pathogenesis of gastrointestinal stromal tumors (GISTs), and countering the mutational activation by specific tyrosine kinase inhibitors, such as Imatinib mesylate, is now standard treatment for metastatic GISTs. KIT exon 11 mutations (in frame deletions, point mutations, and duplications) occur in GISTs of all locations, whereas a characteristic exon 9 insertion-duplication AY502-503 is nearly specific for intestinal vs gastric tumors. In contrast, PDGFRA mutations are nearly specific for gastric GISTs, especially those with epithelioid morphology. Mutation type influences therapy responsiveness, but fortunately very few GISTs carry primarily Imatinib-resistant mutations. Secondary drug resistance acquired during Imatinib treatment based on new, Imatinib-resistant mutations is a major problem limiting treatment success. Loss of NF2 tumor suppressor gene in a biallelic fashion is believed to be central in the pathogenesis of neurofibromatosis 2 (NF2) associated and sporadic schwannomas and meningiomas. The mechanism includes nonsense or missense mutation in NF2 gene, and loss of the other NF2 allele as a part of losses in chromosome 22q. Schwannoma types may differ in their pathogenesis: gastrointestinal schwannomas lack NF2 changes suggesting a different pathogenesis. Intraneural and sclerosing perineuriomas display similar NF2 gene alterations as seen in meningioma, indicating a similar pathogenesis and molecular homology. Specific viral sequences of human herpesvirus 8 (HHV8) are diagnostic markers for Kaposi sarcoma (KS), and are absent in angiosarcoma. Despite discovery on simian virus SV40 sequences in mesothelioma as a possible pathogenetic factor, recent studies suggest that the presence of these sequences may be artifactual and based on common presence of some SV40 sequences as PCR contaminants.

Genetic Testing↗

Mammary NOS-type sarcoma with CD10 expression: a rare entity with features of myoepithelial differentiation.

We present an extensive immunohistochemical analysis of 7 mammary sarcomas that did not fit into any specific soft tissue sarcoma category. Histologically, they were composed of spindle cells with highly pleomorphic nuclei and abundant mitoses. Our immunohistochemical antibody panel included pan-cytokeratin (CK), basal cell type CKs (34betaE12, CK5/6, CK14, CK17) and vimentin antibodies, antibodies to established (SMA, CD10, p63, S-100, maspin, calponin, GFAP, SM-myosin), and novel (CD29, 14-3-3sigma) myoepithelial markers, as well as antibodies to CD34, desmin, h-caldesmon, steroid receptors (estrogen, progesterone, androgen), and EGFR (Her-1). Whereas CKs, CD34, desmin, and h-caldesmon were not expressed, all tumors were positive for CD10 and vimentin. CD29 and SMA were observed in 3 cases each (43%), and p63 and calponin in 2 cases each (29%). Other myoepithelial markers and steroid receptors were absent, except androgen receptors, which were expressed in one sarcoma. Five sarcomas showed positivity for EGFR. The distinction of specific, histogenetically defined sarcoma entities (such as leiomyosarcoma, angiosarcoma, liposarcoma) from NOS-type sarcoma with CD10 expression is usually clear-cut because the former exhibit a characteristic histomorphology and immunoprofile. Phyllodes tumors with stromal overgrowth or recurrent phyllodes tumors lacking epithelial structures as well as periductal stromal sarcomas can be ruled out by their frequent expression of CD34 and negativity for myoepithelial markers. The most important differential diagnosis is sarcomatoid metaplastic carcinoma because its treatment includes axillary lymphadenectomy. Since some NOS-type sarcomas with CD10 expression and most metaplastic carcinomas show positivity for CD29, SMA, and p63, differential diagnosis can be extremely difficult and requires extensive immunohistochemical evaluation for CKs and additional myoepithelial markers such as S-100, 14-3-3sigma, and maspin. The immunophenotype of NOS-type sarcomas with CD10 expression suggests that these neoplasms represent a mammary sarcoma variant with myoepithelial features.

Adult↗

Influence of biologic factors and anatomic site in completely resected liposarcoma.

PURPOSE: Soft tissue sarcoma (STS) encompasses a group of neoplasms that are anatomically and biologically diverse. Retroperitoneal/visceral (RP/V) tumors have a poorer prognosis than extremity/trunk (E/T) lesions, and this has been attributed to frequent presentation with tumors of large size and multiorgan involvement that precludes complete resection. The worse prognosis that is associated with RP/V tumors has also been thought to be histopathologically dependent and not necessarily related to anatomic site. The aim of this study was to determine the role of anatomic site and biologic features in prognosis and outcome in patients after complete resection by examining a large cohort of STS patients with a single histopathology, ie, liposarcoma. METHODS: All patients who were treated for liposarcoma from July 1, 1982, through July 1, 1998, were included. Univariate analyses were performed using log-rank test and Kaplan-Meier estimates, and multivariate analyses were performed using Cox regression. The three end points examined were local recurrence (LR), distant recurrence, and disease-specific survival (DSS). RESULTS: Seven hundred twenty patients with liposarcoma were evaluated, and of these, 460 had completely resected primary or completely resected locally recurrent disease. Breakdown of anatomic site was 65% E/T (n = 301) and 35% RP/V (n = 159). The median follow-up period for patients who underwent complete resection was 42 months (range, 1 to 194 months). We found that RP/V site is a poor prognosticator that is independent of patient sex and age; tumor size, grade, and margin; and recurrent presentation. Sixty-nine percent of patients with RP/V tumors who died had local disease only and no distant metastasis at the time of death. CONCLUSION: In liposarcoma, tumor location exerts as strong an influence on prognosis as biology. In contrast to extremity liposarcoma, LR without distant metastasis often results in death for patients with RP/V tumors. For these patients, local control accomplished by complete surgical resection +/- adjuvant radiation therapy should impact strongly on DSS.

Disease-Free Survival↗

Sclerosing extramedullary hematopoietic tumor in chronic myeloproliferative disorders.

Sclerosing extramedullary hematopoietic tumor (SEMHT) occasionally may arise in patients with chronic myeloproliferative disorders (CMPDs). Morphologically, these tumors may be mistaken for sarcomas or other neoplasms, especially if the clinical history is unknown. We analyzed four cases to identify features to aid in this differential diagnosis. Clinically, there were four men (mean age, 64.5 years), each with a history of CMPD. Grossly, the SEMHTs formed solitary renal or perirenal masses or multiple mesenteric or omental nodules. Morphologically, each SEMHT had a sclerotic to myxoid background with thick collagen strands and trapped fat. Atypical megakaryocytes, maturing granulocytic and erythroid precursors, and few to no blasts were identified in all cases. The megakaryocytes, granulocytic precursors, and erythroid precursors reacted strongly with antibodies to factor VIII, myeloperoxidase, and hemoglobin, respectively, in immunohistochemical studies performed in selected cases. SEMHT is a rare manifestation of CMPD that may be mistaken for a sarcoma, especially sclerosing liposarcoma, Hodgkin's disease, especially lymphocyte depletion type, or a myelolipoma. In a myxoid tumor with trapped fat and atypical cells, morphologic and immunohistochemical identification of maturing hematopoietic precursors helps distinguish SEMHT from sarcoma or Hodgkin's disease. The presence of sclerosis and atypical megakaryocytes helps distinguish SEMHT from myelolipoma.

Aged↗

Sarcomas near extremity joints in adults.

BACKGROUND AND OBJECTIVES: There are technical difficulties in resecting soft tissue sarcomas extending to or crossing a joint. The objective of this study was to determine the rate of amputation and local recurrence rate for these sarcomas and compare them with the respective rates for overall extremity sarcomas. METHODS: Retrospective review of 78 patients with sarcoma near a joint compared with 215 patients with extremity sarcomas accrued during the same period, 1977-1994. Of these 78 patients, 64 were in the lower and 14 in the upper extremity. Most common histologic subtypes were malignant fibrous histiocytoma (15/78, 19%), synovial sarcoma (11/78, 14%), liposarcoma (11/78, 14%), and leiomyosarcoma (10/78, 13%). The surgical treatment consisted of local excision in 10 (13%), wide excision in 56 (72%), and amputation in 12 (15%). Adjuvant radiation was given to 26 patients. RESULTS: Local recurrence was noted in 20% (16/78) patients. The incidence of local recurrence in the surgery alone group (n = 52) was 15% (8/52) and in the surgery plus adjuvant radiation group (n = 26) it was 31% (8/26); P = 0.11. Of the 16 patients with local recurrence, 9 (56%) required amputation. The 5-year and 10-year survival rates for the entire group of patients were 68% and 60% respectively. On multivariate analysis survival varied according to grade (P = 0.05) and tumor size (P = 0.02). CONCLUSIONS: Amputation was finally required in 27% (21/78) for local control of the disease. The local recurrence rate was 20%. These rates appear to be somewhat higher than those reported in our overall extremity sarcoma series and those in most modern series of overall extremity sarcomas, but the 5- and 10-year survival rates are similar to those of the latter.

Adult↗

Translocation t(12;22)(q13;q12.2-12.3) in a clear cell sarcoma of tendons and aponeuroses.

Cytogenetic analysis of a short-term culture from a clear cell sarcoma revealed a complex karyotype with the mainline of 49,XY,t(7;18)(p11.2;q21.3), +der(7)t(7;18)(p11.2;q21.3), +8, +der (8;17)(q10;q10),t(12;22)(q13;q12.2-12.3),add(13)(p13). An apparently identical translocation t(12;22) has been described recently in four clear cell sarcomas, indicating that this constitutes a primary cytogenetic change specific for this type of tumor. In our case, the breakpoint on chromosome 22 could be assigned to band 22q12.2 or 22q12.3. Together with the present case, trisomy or tetrasomy 8 has been found in six of nine clear cell sarcomas, suggesting that, as in Ewing's sarcoma and myxoid liposarcoma, trisomy/tetrasomy 8 represents a nonrandom secondary aberration. We conclude that the finding of the specific translocation t(12;22) may prove to be an important marker in the differential diagnosis of clear cell sarcoma from some other soft tissue sarcomas and malignant melanoma.

Adult↗

Matrix "blues": clue to a cranial thoracic mass in a dog.

A 5-year-old, intact male Italian Spinone dog was presented for progressive, severe dyspnea and coughing. Thoracic radiographs revealed a large mass in the right cranial thorax. Fine needle aspiration of the mass yielded a highly cellular sample containing dense clumps of oval to spindle-shaped mesenchymal cells with distinct intracytoplasmic vacuolation, consistent with lipoblasts and lipocytes. Cell clusters were associated with abundant eosinophilic matrix, which was identified as mucin, based on Alcian blue staining. At exploratory thoracotomy, the mass was found to be nonresectable, and the dog was euthanized. Histologic sections of the multilobular mass had discrete regions of variable cellular differentiation, including highly cellular areas of pleomorphic cells, areas of spindle cells and lipoblasts in a myxoid background, and areas of well-differentiated lipogenic cells. The histologic diagnosis was myxoid liposarcoma. The thoracic cavity is a rare site for liposarcoma in the dog. The cytologic features of lipoblasts together with a mucopolysaccharide matrix were useful for distinguishing the myxoid variant of liposarcoma from other forms of liposarcoma and myxoid sarcomas.

Animals↗

Prognostic significance of augmented metallothionein (MT) expression correlated with Ki-67 antigen expression in selected soft tissue sarcomas.

In soft tissue sarcomas, the most important prognostic criteria include extent of malignancy (G), size of the tumour and intensity of Ki-67 antigen expression. In recent times expression of metallothionein (MT) in cells of some malignant processes of epithelial origin was found to correlate with intensity of Ki-67 antigen expression and to carry a possible prognostic significance. The present study aimed at a demonstration of prognostic value of MT expression and at comparing it with Ki-67 antigen expression and G grade in selected soft tissue sarcomas. Immunohistochemical studies were performed on paraffin sections in 54 cases of malignant fibrous histiocytoma (MFH), 18 cases of liposarcoma and 20 cases of synovial sarcoma. The extent of MT and Ki-67 antigen expression was evaluated and an attempt was made to correlate the results with each other and with grade of the tumour. Expression of MT was evident both in the cytoplasm and in cell nuclei of all studied sarcomas. The most pronounced MT expression was noted in MFH-type tumours. The extent of Ki-67 antigen expression was similar in MFH and liposarcoma and was the lowest in synovial sarcoma. In MFH, liposarcoma and synovial sarcoma a pronounced positive correlation was documented between expression of MT and Ki-67 antigen (r=0.85; p<0.001; r=0.93, p<0.0001; r=0.79, p<0.0001). In all types of the tumours a positive relation was detected between MT expression, expression of Ki-67 and G grade of malignancy in the tumour. Moreover, patients with higher MT expression in the studied tumours demonstrated a shorter survival. MT expression in soft tissue tumours of MFH, liposarcoma and synovial sarcoma type strongly correlated with intensity of proliferation (Ki-67) and G grade and could be useful in defining the extent of malignancy and in prognostic appraisal in the tumours.

Humans↗

P-glycoprotein expression in soft-tissue sarcomas.

Multidrug resistance (MDR) is an important problem in chemotherapy for neoplastic disease. In humans. MDR is mainly mediated by P-glycoprotein (P-gp) a product of the MDRI gene, which acts as a transmembrane protein pump and eliminates chemotherapeutic agents from the cells. Expression of P-gp was immunohistochemically studied by using two monoclonal antibodies, JSB-1 and C-219, on paraffin-embedded sections from 55 patients with soft-tissue sarcoma. The histological diagnosis of tumors was malignant fibrous histiocytoma in 24 cases, liposarcoma in 9, synovial sarcoma in 7, malignant neurogenic tumors in 6, leiomyosarcoma in 5, others in 4. The histological grade was determined on the basis of criteria previously proposed by us. Out of 55 cases, 34 (62%) were positive for P-gp expression. There was a significant difference in P-gp expression between high-grade (90%) and intermediate and low-grade tumors (46%) (P < 0.005). Tumors expressing P-gp had a less favorable prognosis than P-gp-negative tumors in the high- and intermediate-grade tumors. The current study demonstrated that the estimation of P-gp expression could be used to select appropriate therapeutic modalities.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

Tumor suppressor genes and related molecules in leiomyosarcoma.

Soft tissue sarcomas represent a heterogeneous group of mesenchymal malignancies, and the majority of the previous scientific studies that have analyzed the occurrence of cell cycle regulators aberrations within soft tissue sarcomas have dealt with broad categories of different tumors. As a consequence, data concerning single classes of sarcomas are very limited. The authors analyze herein a histologically homogeneous series of 23 cases of leiomyosarcoma of the deep soft tissue. The p53 pathway was studied by investigating the p53 gene and protein, MDM2 protein, and p21waf1 protein. The Rb-cyclin D pathway was analyzed by studying the Rb gene and protein, p16MTS1/INK4A gene and protein, cyclin D1Prad1/bcl1 and cyclin D3 proteins. Aberrations of the p53 pathway were observed in about 16 percent of cases and were limited to the p53 gene. Such a finding contrasts with the higher rates of p53/MDM2 abnormalities reported in other types of sarcomas such as liposarcoma. Interestingly, abnormalities involving the Rb-cyclin D pathway were detected in about 90 percent of cases. The Rb-cyclin D pathway therefore emerges as the preferred target for molecular abnormalities in this subset of soft tissue sarcomas.

Base Sequence↗

A robust assay for alternative lengthening of telomeres in tumors shows the significance of alternative lengthening of telomeres in sarcomas and astrocytomas.

PURPOSE AND EXPERIMENTAL DESIGN: Telomeres of tumor cells may be maintained by telomerase or by alternative lengthening of telomeres (ALT). The standard ALT assay requires Southern analysis of high molecular weight genomic DNA. We aimed to establish and validate an ALT assay suitable for archived paraffin-embedded tumors and to use it to examine the prevalence and clinical significance of ALT in various types of tumors that are often telomerase negative. RESULTS: To assay for ALT, we detected ALT-associated promyelocytic leukemia (PML) bodies (APBs) by combined PML immunofluorescence and telomere fluorescence in situ hybridization. APBs are PML nuclear domains containing telomeric DNA and are a known hallmark of ALT in cell lines. The APB assay concurred with the standard ALT assay in 62 of 62 tumors and showed that 35% of 101 soft tissue sarcomas (STS), 47% of 58 osteosarcomas (especially younger patients), 34% of 50 astrocytomas, and 0% of 17 papillary thyroid carcinomas were ALT positive (ALT+). The prevalence of ALT varied greatly among different STS subtypes: malignant fibrous histiocytomas, 77%; leiomyosarcomas, 62%; liposarcomas, 33%; synovial sarcomas, 9%; and rhabdomyosarcomas, 6%. ALT correlated with survival in glioblastoma multiforme and occurred more often in lower-grade astrocytomas, but ALT+ and ALT- sarcomas were equally aggressive in terms of grade and clinical outcome. CONCLUSION: The APB assay for ALT is suitable for paraffin-embedded tumors. It showed that a substantial proportion of STS, osteosarcomas, and astrocytomas, but not papillary thyroid carcinomas use ALT. APB positivity correlated strongly with survival of patients with astrocytomas.

Adult↗

[Adjuvant chemotherapy in the treatment of primary soft tissue sarcomas, with special reference to intra-arterial infusion chemotherapy].

Seventy-eight cases of adjuvant chemotherapy for primary soft tissue sarcoma including 51 cases of intra-arterial infusion chemotherapy were studied. The patients ranged in age from 1 to 92 years with a median age of 34 years. Thirty-nine patients were male and 39 were female. The seventy-eight cases were comprized of 17 rhabdomyosarcoma, 12 liposarcoma, 12 neurogenic sarcoma, 10 malignant fibrous histiocytoma, 8 leiomyosarcoma, 7 angiosarcoma, 8 others and 4 unclassified sarcomas. Fifty-one patients with soft tissue sarcoma of the extremities were treated by intra-arterial infusion chemotherapy with either VCQ (Vincristine and Carbazilquinone) or VCQ, A (Vincristine, Carbazilquinone and Adriamycin). Out of 42 patients with measurable lesions, 2 CR, 4 PR, 33 NC and 3 PD were obtained. Histological examinations demonstrated histological effect of GI 19, G IIa 11 and G IIb 7 by Ohboshi and Shimosato's criteria. Remarkable effects of treatment were noted in most rhabdomyosarcoma patients. After intra-arterial infusion chemotherapy, a variety of surgical procedures ranging from marginal resection and wide resection to radical amputation were employed in 44 patients. Local recurrence was 27% and distant metastasis developed in 47% of cases.

Adolescent↗