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PubMed1998

Soft tissue sarcomas: pattern diagnosis or entity?

Soft tissue sarcomas (STS) are a diverse and heterogeneous group of tumours. The sub-classification of these tumours is of importance for both prognosis and treatment. Classically, sub-categorization is based purely on histomorphological grounds, but as new techniques evolve, a more conclusive and accurate diagnosis can be made. This study describes the prevalence of soft tissue sarcomas in adults diagnosed at The Aga Khan University Hospital (AKUH) and the impact of immunohistochemistry (IHC) on the precise sub-categorization of these tumours. The study included 364 adults (age 16+) who were diagnosed as soft tissue sarcoma in the past six years (May 1991-July 1997) at the Histopathology lab of the AKUH. Where indicated, tumours were stained with a panel of antibodies using the PAP technique. Of these, 237 (65%) were male and 127 (35%) were female. The median age at which all sarcomas were diagnosed was 39.5 years. The most common site was the lower extremity (29%). The most frequently diagnosed sarcoma was leiomyosarcoma (13%), followed by malignant nerve sheath tumour (12%), rhabdomyosarcoma (10%) and liposarcoma (10%). Cases were further analyzed by dividing them into two groups, each group comprised of all sarcomas diagnosed during the specified period. In the period 1991-1994, only 16% of cases were further analyzed using IHC, while in the period 1995-1997, IHC was performed on 59% of cases. In the 1991-1994 group, a conclusive diagnosis was made in 57% of the cases and in the 1995-1997 group in 78%. A Chi-square test was performed, which proved that these results were statistically significant. Soft tissue sarcoma is one of the key areas in surgical pathology where immunohistochemistry plays an important role in both precise diagnosis and sub-categorization.

Abdominal Neoplasms↗
PubMed1998

[Demonstration of SYT-SSX11/2 fusion transcripts in synovial sarcomas using RT-PCR].

Various mesenchymal malignancies with a spindle cell morphology may mimic monophasic synovial sarcomas. We analysed, whether detection of SYT-SSX1/2 fusion transcripts, present as a consequence of a specific translocation [t(X;18)(p11.2;q11.2)] found in synovial sarcomas, are useful to confirm the diagnosis of synovial sarcoma. A nested RT-PCR protocol was established for the detection of SYT-SSX1/2 fusion transcripts. After RNA extraction of snap frozen tissue reverse transcription was carried out. In a nested PCR, the resulting cDNA samples were amplified and visualized on agarose gels. DNA sequencing of PCR products enabled the assignment of fusion transcripts to either the SYT-SSX1 or the SYT-SSX2 variant. We detected SYT-SSX1/2 fusion transcripts in seven monophasic and three biphasic synovial sarcomas. 20 out of 21 control tumour including leiomyosarcomas, malignant peripheral nerve sheath tumours, gastrointestinal stromal sarcomas and fibrosarcomas were negative in RT-PCR analysis. One case of recurrent spindle cell sarcoma originally classified as a fibrosarcoma revealed a SYT-SSX2 fusion transcript. These data provide further evidence that the RT-PCR amplification of SYT-SSX1/2 fusion transcripts permits the specific identification of synovial sarcomas. This diagnostic approach may be especially useful in cases with equivocal histomorphology.

Chromosome Mapping↗
PubMed1999

Myxoid synovial sarcoma: an underappreciated morphologic subset.

Focal myxoid change is a well-recognized feature of synovial sarcoma, but the presence of a predominantly myxoid stroma is rare. We describe seven cases of myxoid synovial sarcoma in which marked myxoid change initially obscured the diagnosis, leading to confusion (principally with malignant peripheral nerve sheath tumor). The median age (20 yr) and tumor location (four lower extremity, two upper extremity, and one head and neck region) were similar to those found in typical synovial sarcoma. Histologically, five cases were monophasic spindle cell lesions with a lacy appearance in areas with a prominent Alcian blue-positive myxoid stroma. Each case had foci with more typical features of synovial sarcoma, including a fascicular growth pattern with a variably collagenized stroma, stromal mast cells, a hemangiopericytoma-like vascular pattern, and calcification. Two cases showed small foci of glandular (biphasic) differentiation. Immunohistochemically, all of the seven cases were positive for epithelial membrane antigen, four of six were positive for pan-keratin, three of six were positive for S-100, two of four were positive for CD99, and six of six were negative for desmin. Clinical follow-up in six cases ranged from 8 to 48 months (median, 21 mo). Local recurrence developed in three patients at 9, 20, and 24 months, respectively. In one of these three patients, lung metastases developed at 13 months, and the patient died of disseminated disease at 23 months. In another of the three patients, lung metastases developed at 27 months. Three patients had no evidence of disease at 8, 15, and 15 months. Our data are too limited to indicate any clinical differences between myxoid synovial sarcoma and conventional synovial sarcoma Recognition of this rare histologic variant of synovial sarcoma is important because it can easily be mistaken for other myxoid spindle cell neoplasms, potentially resulting in suboptimal therapy.

Adolescent↗
PubMed2001

[Epidemiology of soft tissue sarcomas in adults].

INCIDENCE: Soft tissue sarcoma accounts for a approximately 1% of all cancers. Excluding Kaposi sarcoma, the incidence of soft tissue sarcoma has remained stable in Europe (3-4/100,000). ENDOGENOUS FACTORS: Endogenous factors are related to rare genetic syndromes (Li-Fraumeni syndrome, neurofibromatosis, Werner syndrome, bilateral retinoblastom). EXOGENOUS FACTORS: Exogenous factors associated with soft tissue sarcoma are essentially iatrogenic: radiation therapy (prevalence of radiation-induced sarcoma: 0.17-0.21%) and chronically edematous arms after radical mastectomy for breast cancer (Stewart-Treves syndrome). Many studies suggest that environmental or occupational exposure to chlorophenol, phenoxy-herbicides or dioxin increase the risk of soft tissue sarcoma. FURTHER RESEARCH: Little is known about the epidemiology of soft tissue sarcoma. Case-control studies are needed.

Adult↗
PubMed2002

Telomerase activity in needle biopsied uterine myoma-like tumors: differential diagnosis between uterine sarcomas and leiomyomas.

Preoperative differential diagnoses between uterine sarcomas and leiomyomas are difficult. As telomerase activation is thought to be essential for the immortality of malignant cells, it is considered a potentially useful diagnostic marker. The aim of the present study was to evaluate the potential diagnostic use of measuring telomerase activity in needle biopsy samples to distinguish uterine sarcoma from leiomyoma. Sixty-two patients with suspected uterine sarcomas based on clinical findings or magnetic resonance imaging findings, and who were scheduled for surgery, underwent transcervical ultrasound-guided needle biopsy. Three samples were obtained per patient for histopathological examination and telomerase activity measurement. Telomerase activity was measured using the telomeric repeat amplification protocol and correlated with final histopathological findings of surgical specimens. Of the 62 patients, 6 leiomyosarcomas and 1 endometrial stromal sarcoma (high grade) were diagnosed by histopathology. In 6 of the 7 samples from uterine sarcomas, relatively high telomerase activity (22-102 units) was detected, whereas only low telomerase activity (11-18 units) existed in 3 of the remaining 55 samples from benign or borderline uterine smooth muscle tumors. At a cut-off value of 20 units, sensitivity, specificity, positive predictive, and negative predictive values for detecting uterine sarcoma were 86% (95% confidence interval, 59-100%), 100% (94-100%), 100% (54-100%) and 98% (95-100%), respectively. The results indicated that telomerase activity in needle biopsy samples is a useful diagnostic marker to distinguish uterine sarcoma from leiomyoma.

Adult↗
PubMed2002

[CT manifestations of primary hepatic sarcoma].

OBJECTIVE: To assess the diagnostic value of CT to primary hepatic sarcoma. METHODS: The CT findings of 12 cases of primary hepatic sarcomas confirmed by operation and pathology, including leimyosarcoma, liposarcoma, and malignant fibrous histocytoma, 2 cases each, and angiosarcoma, carcinosarcoma, cystadenocarcinosarcoma, mesotheliosarcoma, fibrosarcoma, and malignant lymphoma, one case each, were analysed retrospectively. RESULTS: Only 2 out of the 12 cases of primary hepatic sarcoma were correctly diagnosed by CT before operation. The CT manifestations of primary hepatic sarcoma, against the operational and pathological findings, could be summarized as two types: solid mass type and cystic mass type. Eight cases were of solid mass type, including 2 cases of liposarcoma, and leiomyosarcoma, angiosarcoma, carcinosarcoma, methotheliosarcoma, fibrosarcoma, and malignant lymphoma, one case each. Plain scanning showed homogeneous or inhomogeneous low-density masses; enhanced scanning showed no marked enhancement or inhomogeneous enhancement, ringed peripheral enhancement, or nodular peripheral enhancement. Peripheral enhancement was a CT feature common to angiosarcoma and lymphoma. The characteristic CT manifestation of liposarcoma was an inhomogeneous mass with well-defined border and multiple intermingled septa. Four cases were of cystic mass type, including 2 cases of malignant fibrous histocytoma, and leimyosarcoma with post-operative recurrence and cystadenocarcinosarcoma, one case each, all manifesting a huge single cystic mass: unilocular in the case of leimyosarcoma with post-operative recurrence and multilocular in the other 3 cases. The wall and/or septa of the cyst had an uneven thickness and were markedly enhanced after contrast administration. Clear solid element and wall nodules were seen in the cystic mass of cystadenocarcinosarcoma. CONCLUSION: The CT findings of primary hepatic sarcoma are associated with the pathology of tumor. Some pathological patterns of primary hepatic sarcoma, such as liposarcoma and angiosarcoma, have their own characteristic CT manifestations. However, the diagnosis of most primary hepatic sarcoma lacking characteristic CT manifestations must be based on clinical and laboratory examinations and confirmed by pathology.

Adolescent↗
PubMedDate not supplied

Recovery of stromal stem cells in bone sarcoma patients after chemotherapy: implication for cell-based therapy in bone defect reconstruction.

Bone sarcomas, such as osteosarcoma (OS) or Ewing sarcoma (ES), frequently arise in the intramedullary region of long bones. Patients affected by bone sarcomas are treated with preoperative aggressive chemotherapy immediately after diagnosis. After chemotherapy, patients undergo surgery that frequently entails the excision of wide bone segments. If a large segment of the bone is lost (defined as a critical defect) the patient undergoes bone reconstruction. Because bone marrow derived stromal stem cells (SSC) offer great promise for cell-based regenerative medicine in bone reconstruction, we investigated whether SSC could be isolated from chemotherapy-treated bone sarcoma patients. We also investigated whether chemotherapy modified SSC differentiation capability. We studied 9 SSC derived from OS and ES patients that had undergone chemotherapy and 5 SSC derived from bone sarcoma patients that had not undergone chemotherapy. SSC could be obtained from all the patients analyzed regardless of whether the patients received chemotherapy or not. Our results also showed that post-chemotherapy SSC can be cultured and expanded ex vivo, these cells retained the ability to differentiate toward the osteogenic lineage in vitro. In conclusion, our results support that SSC cells can be obtained from bone sarcoma patients that undergo chemotherapy and suggest that SSC can be used for cell-based bone reconstruction techniques in bone sarcoma patients treated with preoperative chemotherapy.

Adolescent↗
PubMed2003

[Kaposi's sarcoma during HIV infection in Morocco (apropos of 50 cases)].

Kaposi's sarcoma is the most common neoplasm to be found among AIDS patients. The purpose of this retrospective study is to determine the epidemiological, clinical, therapeutic features and the evolution of Kaposi's sarcoma in Moroccan patients suffering from AIDS. Between January 1987 and December 1999, 696 patients with AIDS were hospitalized in the department of infectious diseases of Casablanca. Fifty of them (7%) had Kaposi's sarcoma. AIDS was confirmed by ELISA and Western-Blot. The diagnosis of Kaposi's sarcoma was established by clinical aspect and I or by histologically study. It concerned 46 men and 4 women. The average age was 32.5 years. The homosexuality was noted in 38% of patients and the heterosexuality in 56% of cases. AIDS was revealed by Kaposi's sarcoma in 82% of cases. The Lesions were found in the skin (96%), in the skin and mucous membranes (56%), in the lung (14%), in the gastrointestinal tract (12%) and in the lymphatic nodes (8%). The average CD4 cells count was 212.8/mm3. The chemotherapy was instituted in 43 patients and the radiotherapy in 5 patients (alone or associated with chemotherapy). Four patients received HAART. Six patients didn't received any treatment. The evolution has been characterized by the death in 61% of patients. This death was due to Kaposi's sarcoma in six patients and to opportunistic infections in the others cases. Our study demonstrates the high incidence of Kaposi's sarcoma as an inaugurating event of AIDS, the severity of the visceral involvement, the limit of our therapeutic materials and the wrong prognosis of this disease.

Acquired Immunodeficiency Syndrome↗
PubMed2004

Costs of follow-up after potentially curative treatment for extremity soft-tissue sarcoma.

Soft-tissue sarcoma is an uncommon cancer with the potential for high rates of recurrence after initial therapy. Multiple surveillance strategies have been developed to follow patients after primary treatment. The purpose of this study was to quantify the costs associated with various published post-treatment surveillance strategies. A literature review covering the years 1982-2003 was performed to find all modern published surveillance methods for extremity soft-tissue sarcoma. Only articles describing an explicit 5-year follow-up strategy were included. Total costs of 5-year follow-up were calculated for each strategy using Medicare-allowed charges as a proxy. Thirty-four articles depicting 54 strategies were identified. Total Medicare-allowed charges in year 2003 dollars ranged from 485 dollars for follow-up of low-grade sarcoma to 21,235 dollars for follow-up of high-grade sarcoma, a 42.8-fold cost differential. The average charge for these 54 strategies was 6,401 dollars. Physical examination and chest x-ray were the most commonly used screening modalities. Several guidelines have been proposed for extremity soft-tissue sarcoma patient follow-up, most prominently those of the National Comprehensive Cancer Network. The literature has yet to reflect the consensus these guidelines suggest. This study shows wide disparity in the costs of 54 specific methods of following soft-tissue sarcoma patients. Clinical trials are needed to identify an optimal surveillance strategy, one balancing gains in survival, quality of life, costs, and societal willingness to expend resources. Such trials have not been conducted due to the rarity of extremity soft-tissue sarcomas and the costs associated with conducting long-term trials. Alternatively, prospective evaluation of imaging modalities used in follow-up should be assessed as part of other trials. Computer simulation analysis also holds great promise as an assessment tool for surveillance strategies because patient participation is not required.

Extremities↗
PubMedDate not supplied

Metastatic follicular dendritic cell sarcoma of the stomach: a case report and review of the literature.

Follicular dendritic cell (FDC) sarcomas are rare tumours, typically seen in lymph nodes. However, in about one third of the reported cases, a FDC sarcoma presents as an extranodal mass. Involvement of the gastrointestinal tract is extremely rare, and only 3 cases have been described to date. We report on a 40-year-old female patient with a follicular dendritic cell sarcoma located in the stomach and the presence of a metastasis in the liver at the time of diagnosis. Severe asthenia, nausea, back pain and loss of weight were the presenting symptoms. A CT scan of the abdomen and an upper gastrointestinal endoscopy revealed a tumour mass in the stomach. The diagnosis of a FDC sarcoma was made on histological and immunohistochemical findings. We report the second case of a FDC sarcoma presenting in the stomach. Due to its rarity, a FDC sarcoma seldom enters the differential diagnosis of spindle cells neoplasms of the gastrointestinal tract. Complete surgical resection is the treatment of choice for FDC sarcoma.

Adult↗
PubMed2004

The frequent expression of cancer/testis antigens provides opportunities for immunotherapeutic targeting of sarcoma.

Sarcomas are rare but aggressive malignant tumors associated with high mortality, for which the efficacy of standard therapies remains limited. In order to develop immunotherapeutic approaches for the treatment of sarcoma, we studied the relevance of cancer/testis antigens (CTAs), a group of antigens whose expression is developmentally regulated and that is specifically found in some tumor types, as sarcoma vaccine targets. CTA expression was assessed by PCR and/or immunohistochemistry (IHC) in sarcoma tumor samples that included different histological subtypes and sarcoma cell lines. Expression of HLA class I was assessed by IHC in tumor samples and by FACS analysis in cell lines. More than 70% of the tumor samples expressed at least one CTA. The majority of tumors and cell lines expressed normal levels of HLA class I. HLA class I expression in cell lines was enhanced upon treatment with IFN-gamma. CTA expression was enhanced or induced by treatment with the demethylating agent 5-aza-2'-deoxycytidine, resulting in recognition by specific CTLs. Interestingly, a spontaneous humoral and CD8+ T cellular response to the CTA NY-ESO-1 was detected in a synovial sarcoma patient. Together, these findings strongly support the implementation of CTA-based immunotherapy of sarcoma as a means to improve the efficacy of the standard therapy.

Antibodies, Neoplasm↗
PubMed1992

Flow and image cytometric DNA analysis in Ewing's sarcoma.

Ewing's sarcoma is the second most common bone tumor in childhood, with an overall 5-yr survival of 40%. It is one of the poorly differentiated small spherical cell tumors frequently requiring distinction from rhabdomyosarcoma, neuroblastoma, osteosarcoma, primitive neuroectodermal tumor, and lymphoma. The majority of rhabdomyosarcomas, neuroblastomas, and osteosarcomas are aneuploid, whereas Ewing's sarcomas are usually diploid. To determine whether there is any correlation between DNA content, morphology, site, and survival in Ewing's sarcoma and extraosseous Ewing's sarcoma, 21 tumor samples were studied retrospectively (3 extraosseous Ewing's and 18 Ewing's sarcomas). The DNA analysis was performed on disaggregated paraffin-embedded tissue nuclei by flow (FCM) and image (IC) cytometry and correlated with the histology and clinical history. The DNA ploidy by FCM on 17 of 18 Ewing's sarcoma samples was 12 diploid, 1 aneuploid, and 4 tetraploid. By IC, the DNA ploidy on 16 samples was 13 diploid, 1 aneuploid, and 2 tetraploid. Three samples were nonevaluable (1 by FCM and 2 by IC). The agreement between FCM and IC was 12 of 16 (75%). The extraosseous Ewing's sarcoma tumors were 2 diploid and 1 aneuploid by IC. In this study there was no correlation between the DNA ploidy and either the histology, site, or survival.

Adolescent↗
PubMed2006

[Diagnosis and treatment of HIV-related Kaposi's sarcoma].

A 42-year-old heterosexual man presented with bluish-purple spots on his skin and in his mouth cavity that had been present for a few months; a 48-year-old homosexual man had painful lymphadenopathy in the groins and left axilla. Both men appeared to have a Kaposi's sarcoma and to be HIV-positive. During highly active antiretroviral therapy (HAART) and radiotherapy or chemotherapy, both the AIDS parameters and the skin lesions improved. Kaposi's sarcoma is AIDS-defining in HIV-seropositive patients. Human herpesvirus-8 infection seems to play a role in the development of Kaposi's sarcoma. The incidence of Kaposi's sarcoma has declined since the introduction of HAART. Nowadays, Kaposi's sarcoma is frequently the presenting symptom of HIV-seropositivity. Patients present with purple cutaneous lesions and/or generalised lymphadenopathy. Visceral lesions are associated with a shorter median survival. The treatment of Kaposi's sarcoma is palliative, whereas immune restitution can lead to regression of the sarcoma.

Adult↗
PubMed1977

Oncornavirus-induced sarcoma formation obscured by rapid development of lethal leukemia.

Injection i.v. of avian erythroblastosis virus (AEV) strain ES4 causes a high incidence of leukemia and the death of most of the inoculated chicks within 2 weeks. As found earlier, the virus is defective for replication and transforms bone marrow cultures in vitro, and surprisingly, also chick embryo fibroblasts. Inoculation of transformed AEV cells negative for virus production into newborn chicks induced the formation of sarcomas only, whereas cells superinfected with helper virus induced the formation of erythroblastosis in addition to sarcomas. The helper virus alone caused neither sarcomas nor erythroblastosis during the experimental period. These findings were explained by the hypothesis that AEV-induced erythroblastosis develops more rapidly than do AEV-induced sarcomas and that animals receiving i.v. injections die of the leukemia before sarcomas become detectable. The observation that animals receiving i.m. injections of AEV developed sarcomas at the site of injection strongly supports this concept. Most of the animals that received i.m. injections also developed an erythroblastosis that was delayed, however, in comparison to the animals receiving i.v. injections. Our data also suggest that the erythroblastosis induced by AEV does not suppress the formation of sarcomas in the same animal.

Alpharetrovirus↗
PubMed1991

[Thoraco-scapular amputation in sarcomas of the shoulder girdle].

A total of 121 patients with sarcomas localized to the shoulder girdle were referred to the Sarcoma Centre in Arhus. Of these, 17 (14%) underwent interscapulothoracic amputation. At the time of treatment, the average age was 51 years (17-82 years). Eleven of these patients had sarcomas of bone and six had soft tissue sarcomas. Late diagnosis or previous surgical interventions contributed to the indication for the mutilating procedure. At the time of referral, six of the 11 cases of bone sarcomas were complicated by a pathological fracture and all six soft tissue sarcomas had been submitted to incisional biopsy or non-radical treatment. The soft tissue sarcomas were usually large with an average maximum diameter of 10 cm (4-15 cm). Postoperative recovery was uncomplicated in all cases. Local recurrence occurred in three patients (18%). Eight patients (47%) developed metastases and died from the tumour on an average of 32 months (7-94 months) after operation. Two patients died from other causes without tumour. Seven patients (41%) were tumourfree and alive for an average of 69 months (21-128 months) after operation. Only three of these seven patients wore their shoulder-arm prosthesis regularly while the remainder preferred to be either without a prosthesis or to use a lightweight shoulder prosthesis. None of the seven patients still experienced phantom pain necessitating analgetics. All of the patients were self-reliant in everyday life and the five patients who had been occupationally active until the time of operation had returned to work. The prognosis after interscapulothoracic amputation depends upon the primary malignant disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

Monoclonal antibody identification and characterization of two human sarcoma-associated antigens.

Two murine monoclonal antibodies, 29-13 (IgG1) and 29-2 (IgG2a), generated against malignant fibrous histiocytoma plasma membranes immunoprecipitated a Mr 200,000 protein (p200), with an isoelectric point between 6.3 and 7.5. Two additional antibodies, 35-16 (IgG1) and 30-40 (IgG2a), generated against Ewing's sarcoma membranes, immunoprecipitated an acidic protein of Mr 160,000 (p160), with an isoelectric point between 5.8 and 6.7. Monoclonal antibodies 29-13 and 29-2 recognize a similar determinant(s) on p200 while 35-16 and 30-40 recognize different determinants on p160. Monoclonal antibody 29-13 exhibited significant binding to membranes isolated from fibrosarcoma and aggressive fibromatosis; moderate binding to osteosarcoma, hemangiopericytoma, and malignant fibrous histiocytoma; and minimal to no binding to other soft tissue sarcoma plasma membranes. The p200 protein was not expressed in 16 other malignant tumors and in only 3 of 35 normal human tissue specimens. High levels of p200 were selectively expressed by leiomyosarcoma, Ewing's sarcoma, and fibrosarcoma cells as well as neonatal fibroblasts in vitro, but not by other carcinoma cell lines or B-lymphoblasts. The p160 protein appeared to be selectively expressed by Ewing's sarcoma with little or no expression on other sarcomas, carcinomas, or normal tissues. However, the p160 antigen was expressed in Ewing's sarcoma, leiomyosarcoma, melanoma, 4 of 9 carcinomas, and neonatal fibroblasts in vitro. The affinity of MoAbs 29-13, 29-2, 35-16, and 30-40 ranged from 5.3 x 10(8) to 4.7 x 10(9) M-1 for sarcoma membranes with approximately 5 x 10(4) binding sites/sarcoma cell.

Antibodies, Monoclonal↗
PubMed1990

Dana-Farber Cancer Institute studies in advanced sarcoma.

In a phase II trial of ifosfamide 2 g/m2 days 1 to 4 with mesna uroprotection in 124 patients who had previously failed treatment for sarcomas, 3% achieved a complete response (CR), and 18% had a CR or partial response (PR). In the subset of patients with soft-tissue sarcomas, the response rate for the 64 patients who received bolus administration was 26% compared with 9% for the 60 patients who received a continuous infusion (Cl) schedule (P = .03). When mesna was unavailable, the incidence of hematuria was significant in patients, with three of four patients affected. Microscopic hematuria was uncommon in patients receiving mesna. Hematuria in the group as a whole was not associated with prior treatment with cyclophosphamide, pelvic radiotherapy, age, or a bolus versus Cl schedule. Thus, this and other studies confirm that ifosfamide is active in failed sarcomas. In sequential phase I/II trials, 111 patients who were previously untreated for metastatic or inoperable sarcomas received doxorubicin, ifosfamide, dacarbazine at doses of 60, 7,500, and 900 mg/m2, respectively, by Cl over 72 hours; myelosuppression was dose-limiting. Eleven patients (10%) achieved CRs, with an overall response rate of 47%. Most responses (approximately 70%) were observed within two cycles, and median times to progression were 10 and 9 months for CR and PR, respectively. Following CR, two patients remained disease-free at 32 and 16 months. Of 15 additional patients rendered disease-free with surgery following at least a minor response to chemotherapy, two remained disease-free at 30 and 18 months with no further therapy. Central nervous system (CNS) metastases developed in 11 patients, all of whom had high-grade sarcomas; seven of these patients were still responding systemically (three CRs). This regimen is undergoing evaluation in a randomized trial versus doxorubicin and dacarbazine alone in untreated advanced sarcoma and is being compared with observation in the adjuvant treatment of high-grade sarcomas.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1990

[Sarcoma treatment in Denmark].

Approximately 240 sarcomas are registered annually in Denmark. Of these, 5/6 are soft-tissue sarcomas while the remainder consist of various types of sarcomas of bone. Determination of the histogenetic type, the degree of aplasia and the anatomical spread of the primary tumour is decisive for optimal prognostic assessment and for planning of the primary local treatment. The surgical treatment is aimed at radical treatment and, where tumours in extremities are concerned, this frequently involves reconstruction with endoprostheses with the object of salvaging the limb. Irradiation is indicated in cases of soft-tissue sarcoma which are not amenable to radical operation and as palliative treatment. In cases of soft-tissue sarcoma, cytostatic therapy with adriamycin alone or combined with iphosphamide in metastasizing disease can result in response in 30-50% of the patients. On the other hand, the value of adjuvant treatment is not yet elucidated. In cases of osteogenic sarcoma, the data available at present suggest that prolongation of the survival rate may be obtained by means of intensive adjuvant cytostatic treatment, but the optimal regimen is not yet defined. In other situations, cytostatic therapy must be considered as experimental and not indicated outside the fields of controlled investigations. Optimal diagnosis and treatment of sarcoma requires extensive multidisciplinary contributions which should be centralized in the fewest possible centres. Intimate national and international cooperation is also essential.

Antineoplastic Agents↗