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A case of sarcoma of thymic stroma with features of liposarcoma associated with breast carcinoma is presented. No previous reference was found in the literature to this association. It is possible that this event could be not entirely fortuitous but an element sustaining the pathogenetic theory relating stromal and epithelial neoplasias in the thymus.
Twelve patients presenting with a bone tumor were operated on using autoclaved bone autografts for reconstruction following carcinologic resection. According to the Enneking grading system, 6 were high malignancy tumors (3 osteosarcomas and 3 grade 2 chondrosarcomas), 4 were low grade tumors (1 paraosteal sarcoma, 1 chondrosarcoma, 1 secondary chondrosarcoma, 1 liposarcoma). One was a metastasis from a kidney tumor. The last patient had a femoral osteoid osteoma. Six local recurrences were responsible for 4 reoperations: 2 disarticulations and 2 iterative resections. With a 1-6 years range of follow-up (average follow-up 2.5 years), osseointegration of autoclaved grafts was studied. Fusion at the host/graft junction was roentgenographically observed. In three cases, proximal resorption of the humeral graft occurred. Five biopsies were obtained during reoperation after 1 year, which showed partial revascularization of autoclaved bone autografts. The authors conclude that autoclaved tumoral bone grafts, are reliable and discuss indications. They point out the main contraindication, represented by chemosensitive bone tumors, in which conservation of the removed tumor is necessary to quantify the response to chemotherapy.
The expression of the three catalytic subunits of protein phosphatase (PP) type 1 and 2A, PP1 alpha, PP1 gamma 1, and PP2AC, was examined in osteogenic tumors and soft tissue tumors by immunohistochemical analysis. The percentage of cells stained positively with antiserum against PP1 catalytic subunit isoform PP1 gamma 1, was significantly higher in malignant osteogenic tumors (chondrosarcoma, osteosarcoma, and Ewing's sarcoma) and in malignant soft tissue tumors (liposarcoma and malignant fibrous histiocytoma [M.F.H.]) than in benign tumors (osteochondroma, osteoblastoma, ossifying fibroma, enchondroma and lipoma). Furthermore, the malignant tumor lesions showed a markedly high number of cells in the S-phase fraction of the cell cycle, as compared to benign tumors. These results suggest that PP1 gamma 1 is involved in the accelerated growth of malignant tumor cells.
Between 1970 and 1988 surgery was performed on 124 patients with soft tissue sarcomas of the extremities and trunk in the University Clinics of Hamburg. Liposarcoma, malignant fibrous histiocytoma, fibrosarcoma and malignant schwannoma were the most common histological types. High-grade sarcomas (G3) predominated, with 41%, while 26% were graded G2 and 33% G1. Resection with wide margins all round was achieved in only 54% of the patients. The quality of the operation proved to be only therapy-related prognostic factor. In addition, tumour grade, size, regional lymph node and distant metastasis and histological type proved to be relevant to the prognosis. With multivariate analysis, distant metastasis, grade, resectability and histological type retained prognostic significance. The efficacy of adjuvant chemo- and radiotherapy was related to the quality of the preceding tumour resection. In case of gross tumour the rate of either partial or complete response was 28% for chemotherapy and 22% for radiotherapy. The mean survival time was 102 months; the 5- and 10-year survival rates were 48% and 37%, respectively.
Complete surgical resection is the most effective modality for the treatment of retroperitoneal sarcomas. Previous studies of all types of retroperitoneal sarcomas have not shown a survival benefit of incomplete resection over no resection. Because death often occurs as a result of local progression in retroperitoneal liposarcomas (RPLS), it is possible that incomplete resection may be beneficial in this histologic type. In this study we have sought to determine the clinical outcomes in patients with incompletely resected and unresected RPLS with the aim of defining patients who may benefit from palliative resection. From a prospective clinical database 55 patients with incompletely resected (n = 43) or unresected (n = 12) RPLS were identified between 1982 and 1999. Statistical analyses were performed using the log-rank test and Kaplan-Meier estimates with disease-specific survival as the primary end point. Variables studied included age, gender, recurrent versus primary disease, tumor grade, and tumor size. The patient population consisted of 34 men and 21 women with a mean age of 61 +/- 14 (SD) years. The median time to death was 10 months (range 1 to 83 months) with a median followup of 12 months (range 1 to 60 months) for survivors. Partial resection was an independent factor for increased survival as compared with exploration or biopsy only (median survival 26 versus 4 months, p < 0.0001). Of patients who received incomplete resections, locally recurrent presentation (n = 19) versus primary disease (n = 24) was a negative prognostic variable (median survival 17 versus 46 months, p = 0.009). Successful palliation of symptoms was achieved in 24 of 32 patients (75%) with preoperative symptoms. In select patients with unresectable RPLS, incomplete surgical resection can provide prolongation in survival and successful symptom palliation. Most likely to benefit are those patients presenting with primary tumors, suggesting that surgical resection should be attempted in the majority of patients.
Recent studies have shown that expression of alternatively splicing variants of CD44 is correlated with prognosis for several kinds of malignant tumors. However, little is known about the expression of CD44 standard and variant isoforms in soft tissue sarcomas. In this study 47 cases of soft tissue sarcoma [18 malignant fibrous histiocytomas (MFHs), 13 synovial sarcomas (SSs), 7 malignant schwannomas (MSs), and 9 liposarcomas (LSs)] were examined immunohistochemically. The monoclonal antibodies to the standard form of CD44 (CD44H) and variant exons of CD44v3, 4, 5, 6, 7, 9, and v10 were used. We analyzed the membranous expression pattern of CD44H and CD44 variant exons and assessed the relation between expression of CD44s and metastasis-free survival rates (MFSR) of patients with soft tissue sarcoma. A few sarcomas expressed CD44v3 (2/47) and v7 (2/47), but none of the sarcomas expressed CD44v10. CD44v4 (5/47), v5 (4/47), v6 (10/47), and v9 (9/47) are relatively common types of variant isoforms in soft tissue sarcomas. Expression of CD44v6 is more frequently detected in high-grade than in low-grade tumors. CD44v6 or CD44v9 expression was correlated with metastasis-free survival of patients with soft tissue sarcomas.
The lymph node is the site of antigen presentation, and dendritic cells are sentinels for anti-tumor immunity. However, little is known about the histological features of lymph nodes and dendritic cells in soft tissue sarcomas. The reactive lymph node and infiltration of dendritic cells or effector cells were studied histologically in 10 soft tissue sarcomas with reactive lymphoid hyperplasia. The cases included four malignant fibrous histiocytomas, two malignant peripheral nerve sheath tumors, one synovial sarcoma, one epithelioid sarcoma, one malignant granular cell tumor, and one liposarcoma. The proportions of the T zone, lymphoid follicle, and lymphoid sinus (which was occupied by cells immunopositive for antibodies against CD3, CD20, or CD68) were 33.4% +/- 11.0%, 6.1% +/- 4.9%, and 13.5% +/- 6.5%, respectively. T zone hyperplasia was observed in all cases, and sinus histiocytosis was found in four. The proportion of the T zone in regional lymph nodes of soft tissue sarcoma patients was significantly higher than that in adult autopsy cases without a cancer history. CD8-, TIA-1-, or granzyme B-positive effector cells were found in each sarcoma tissue. Whereas CD1a-positive dendritic cells were not detected, S-100 protein-positive or CD83-positive dendritic cells were observed in five sarcoma tissues. The coefficient correlation between the numbers of effector cells and dendritic cells positive for CD83 or S-100 protein were demonstrated. Although this is a preliminary report, the present study demonstrated that some soft tissue sarcoma patients showed reactive lymphoid hyperplasia. Furthermore, the association between the infiltration of dendritic cells and that of effector cells was observed in patients with soft tissue sarcomas.
We have studied 107 bone and soft-tissue sarcomas and 8 lipomas for amplification of the MDM2 gene. This gene was amplified in 3 out of 67 osteosarcomas, 3 out of 20 malignant fibrous histiocytomas, 4 out of 20 liposarcomas, and 4 out of 8 lipomas. The amplification was associated with overexpression of mRNA. In osteosarcomas, contrary to previous findings, all amplifications were observed in primary lesions. In liposarcomas, the amplification was seen exclusively in well-differentiated tumors with high frequency (4/5) but not in other subtypes (0/15). In addition, MDM2 amplification was also frequently found in deep-seated intra- or intermuscular lipomas (4/5). Hence, it is suggested that MDM2 amplification plays a significant role in the development of differentiated adipose-tissue tumors. Three well-differentiated liposarcomas with MDM2 amplification coexisted with high-grade dedifferentiated sarcomas, in which MDM2 amplification was also observed. Interestingly, in 2 of these cases, the grades of amplification correlated with the histological grades, indicating an important role of MDM2 overexpression in tumor progression.
The glutathione S-transferases (GSTs) are a multi-gene family of enzymes involved in detoxifying electrophilic compounds and the expression of these enzymes in tumours has been proposed as one important mechanism of anti-cancer drug resistance. In this study, the localization of the major cytoplasmic forms of GST has been studied in soft tissue sarcomas by immunohistochemistry. The alpha, mu, and pi forms of GST were identified in 59, 68, and 51 per cent of tumours, respectively. In addition, GST pi immunoreactivity was consistently identified in fibroblasts in adjacent non-neoplastic tissue. The presence of specific forms of GST in soft tissue sarcomas may contribute to the drug resistance frequently observed in these tumours.
Intensive chemotherapy and radiotherapy of Hodgkin's Disease has resulted in improved survival rates. With long-term follow-up, however, a risk of secondary malignancy in these individuals is now appreciated. The authors of this report have encountered five patients who developed bone or soft tissue sarcomata more than 5 years after treatment of Hodgkin's Disease. The four males and one female ranged in age from 14 to 74 years at the time of diagnosis of Hodgkin's disease. Two had received radiotherapy alone for treatment of Hodgkin's disease, two were treated with radiation and chemotherapy, and one received only chemotherapy. The latent period prior to diagnosis of sarcoma ranged from 6 to 11 years. There was one case each of neurofibrosarcoma, fibrosarcoma, osteosarcoma, liposarcoma and leiomyosarcoma. Four patients died within 1 year of the diagnosis of sarcoma. One is alive with no evidence of disease 2 years following diagnosis and surgical excision of the sarcoma. On the basis of the Massachusetts General Hospital experience in the treatment of Hodgkin's Disease, the authors calculate a risk of 0.9% of sarcoma occurring in five year survivors of Hodgkin's disease. Previously reported cases of sarcoma following treatment of Hodgkin's disease are summarized. The pertinent literature is reviewed.
A case of retroperitoneal sarcoma in a kidney transplanted patient is presented. We describe the presentation pattern and the aggressive outcome despite the surgical removal of 5.900 gr. retroperitoneal tumor, included native kidney. The patient died due multiple metastasis six months later. An interesting iconography shows the great volume of the tumor.
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We will report the result obtained from sensitivity tests on various anti-cancer agents for malignant bone and soft-tissue tumors based on SDI (Succinic Dehydrogenase Inhibition Test) method with the use of enzymic activities as marker since 1976. Our study comprised 27 cases altogether 15 cases of osteosarcoma, one case each of Ewing's sarcoma, malignant fibrous histiocytoma and malignant lymphoma, 3 cases of metastatic bone tumor and one case each of angiosarcoma, fibrosarcoma, rhabdomyosarcoma, liposarcoma, 2 cases of metastatic lung tumor among soft-tissue sarcomas. In all cases, sensitivity tests were done on the tumor tissues according to SDI method at the same time as biopsy for the determination of the appropriate medications. Four to six weeks of pre-operative intra-arterial infusion was done followed by radical operation. The results obtained are as follows. Observing the long-term results between subjects that applied anti-cancer agents decided by sensitivity test and those without sensitivity test. The 5 years cumulative survival rate jumped from 30.5% to 50.5%, showing a clear improvement.
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To evaluate the efficacy of aggressive pulmonary metastasectomy for treating osteosarcomas and soft tissue sarcomas, we reviewed 105 cases treated in our hospital between 1990 and 2002. There were 57 males and 48 females, 44 osteosarcomas (OS), 21 synovial sarcomas (Syno), 16 malignant fibrous histiocytomas (MFH), 4 leiomyosarcomas (Leio), 4 alveolar soft part sarcomas (ASPS) and 16 others (including chondrosarcoma and liposarcoma). A total of 904 metastases were resected (8.6(0-49)/patients) and 244 thoracotomies (2.3/patients) were performed. Mean number of initial metastasectomies was 3.7 (0-26), disease-free interval (DFI) was 13. 8 months (0-96), and mean diameter of maximum resected metastases for one patient was 20.4 (5-90) mm. Five-and 10-year survival rates of all cases were 44.9% and 32.0% respectively. Those of OS were 45.8% and 38.5%, and those of soft tissue sarcomas were 44.2% and 25.5%, respectively. The highest 5-and 10-year survival rates among soft tissue sarcomas was attained in ASPS and Leio (75%), and others (51.6% and 38.7%), followed by Syno (42.9% and 12.4%). Long-term survival was not attained in MFH (30.9%: 5 years). Analysis of histological types of the tumors and numbers of resected pulmonary metastases showed that the largest number of metastases were resected in ASPS (16/case) and Syno (13.8/case), followed by OS (9.0/case). MFH had the largest (27.1 mm) mean diameter of maximum resected metastases, followed by Leio (27.0 mm). Analysis of prognostic factors indicated that curativity was the most important prognostic factor: curative cases 42.2% (10-year survival) vs noncurative cases 4.2% (6-year survival). Number of resected metastases and mean diameter of maximum tumor size also affected the patient survival.