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PubMed1999

CD40 upregulation is independent of HHV-8 in the pathogenesis of Kaposi's sarcoma.

AIMS: Human herpesvirus 8 (HHV-8) is now acknowledged as the infective cofactor in the pathogenesis of Kaposi's sarcoma. The mode by which HHV-8 causes Kaposi's sarcoma is unresolved and it is probable that it acts in conjunction with other factors including cytokines, anti-apoptosis proteins, and cell surface receptors. CD40, a cell membrane receptor belonging to the tumour necrosis factor receptor super family, promotes B cell survival and is expressed constitutively on endothelial cells. It is upregulated on cytokine treatment and has been documented recently in Kaposi's sarcoma. Because the HHV-8 genome contains cytokine homologues, this study investigated whether CD40 expression in Kaposi's sarcoma correlated with HHV-8 status, using a unique set of HHV-8 positive and negative specimens. METHODS: Twenty one paraffin wax embedded samples of Kaposi's sarcoma were selected, of which 18 were screened for the presence of HHV-8 using both conventional solution phase and TaqMan polymerase chain reaction (PCR). CD40 immunohistochemistry was assessed using a biotinylated amplification system. Staining was scored semiquantitatively. RESULTS: The results indicated that the expression of CD40 is independent of viral status, being present in both HHV-8 positive and negative specimens. CONCLUSIONS: This suggests that HHV-8 promotes Kaposi's sarcoma cell survival following infection by mechanisms other than those involving CD40.

Antigens, Neoplasm↗
PubMed1987

Pulmonary Kaposi's sarcoma in patients with acquired immune deficiency syndrome: a clinicopathological study.

Pulmonary Kaposi's sarcoma may contribute to respiratory dysfunction in patients with acquired immune deficiency syndrome (AIDS) and features of pneumonitis. Opportunistic infections are readily recognised in endoscopic material, but pulmonary Kaposi's sarcoma is easily missed, so that patients are deprived of specific treatment. The clinical and pathological findings from nine cases of pulmonary Kaposi's sarcoma have been reviewed; these were found among 84 patients with AIDS and pneumonitis undergoing fibreoptic bronchoscopy and bronchoalveolar lavage. Diagnosis was established before death in eight patients (in five by bronchial biopsy and in three by open lung biopsy). Examination of lavage fluid showed alveolar haemorrhage in six patients. It is concluded that: (1) fibreoptic bronchoscopy may be useful in the diagnosis of endobronchial lesions of Kaposi's sarcoma; (2) alveolar haemorrhage in patients with AIDS is suggestive of pulmonary Kaposi's sarcoma. Factors that may cause difficulties in diagnosis include the focal nature of some lesions and the pleural or parenchymatous location of others. In addition, in the lung as in the skin, the early stages of Kaposi's sarcoma resemble granulation tissue. Such lesions are far more difficult to recognise than is the late nodular stage.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Renal sarcoma and sarcomatoid renal cell carcinoma: CT and angiographic features.

Computed tomographic (CT) and angiographic findings in 14 patients (eight men, six women, aged 30-72 years) with sarcomatoid renal cell carcinoma (SRCC) and various types of renal sarcoma are described. There were four patients with SRCC; three, leiomyosarcoma; two, liposarcoma; two, fibrosarcoma; and one each of unclassified renal sarcoma, clear cell sarcoma, and malignant fibrous histiocytoma. The most frequent presenting symptom was an abdominal mass or pain. All 14 patients underwent CT, and 11 underwent selective renal angiography. The diagnosis of renal sarcoma should be suspected when CT findings suggest that the tumor arises from the renal capsule or renal sinus and when the tumor is hypovascular or avascular on angiograms. The characteristic negative attenuation values for liposarcomas permit a specific diagnosis. Sarcomas that originate in the renal parenchyma and SRCC cannot be easily differentiated from renal cell carcinoma; however, renal sarcomas do not appear to have a propensity for extension into the renal vein or the inferior vena cava.

Adult↗
PubMed2005

Expression of the EWS/FLI-1 oncogene in murine primary bone-derived cells Results in EWS/FLI-1-dependent, ewing sarcoma-like tumors.

Ewing sarcoma is the second most common malignant pediatric bone tumor. Over 80% of Ewing sarcoma contain the oncogene EWS/FLI-1, which encodes the EWS/FLI-1 oncoprotein, a hybrid transcription factor comprised of NH2-terminal sequences from the RNA-binding protein EWS and the DNA-binding and COOH-terminal regions of the Ets transcription factor FLI-1. Although numerous genes are dysregulated by EWS/FLI-1, advances in Ewing sarcoma cancer biology have been hindered by the lack of an animal model because of EWS/FLI-1-mediated cytotoxicity. In this study, we have developed conditions for the isolation and propagation of murine primary bone-derived cells (mPBDC) that stably express EWS/FLI-1. Early-passage EWS/FLI-1 mPBDCs were immortalized in culture but inefficient at tumor induction, whereas later-passage cells formed sarcomatous tumors in immunocompetent syngeneic mice. Murine EWS/FLI-1 tumors contained morphologically primitive cells that lacked definitive lineage markers. Molecular characterization of murine EWS/FLI-1 tumors revealed that some but not all had acquired a novel, clonal in-frame p53 mutation associated with a constitutive loss of p21 expression. Despite indications that secondary events facilitated EWS/FLI-1 mPBDC tumorigenesis, cells remained highly dependent on EWS/FLI-1 for efficient transformation in clonogenic assays. This Ewing sarcoma animal model will be a useful tool for dissecting the molecular pathogenesis of Ewing sarcoma and provides rationale for the broader use of organ-specific progenitor cell populations for the study of human sarcoma.

Amino Acid Sequence↗
PubMed2005

Herceptin down-regulates HER-2/neu and vascular endothelial growth factor expression and enhances taxol-induced cytotoxicity of human Ewing's sarcoma cells in vitro and in vivo.

We have previously shown that high levels of HER-2/neu protein were overexpressed in human Ewing's sarcoma cells (TC71, SK-ES1) relative to normal human osteoblasts. The purpose of this study was to determine whether herceptin alone or in combination with chemotherapeutic agents could inhibit the growth of Ewing's sarcoma in vitro and in vivo. Western blot analysis showed that the protein levels of HER-2/neu were decreased following herceptin treatment. Cell growth was also inhibited by herceptin in a dose-dependent manner with an IC(50) of 4 mg/mL in TC71 and SK-ES1 cell line, whereas human immunoglobin had no effect. Northern blot and ELISA showed the RNA expression and protein levels of vascular endothelial growth factor were also inhibited by herceptin treatment with no alteration in HIF-1alpha protein and topoisomerase IIalpha expression. Furthermore, Ewing's sarcoma tumor growth was significantly delayed by 100 mg/kg herceptin treatment in our Ewing's sarcoma xenograft mouse model. Combining taxol with herceptin resulted in additive cytotoxicity, whereas herceptin-etoposide, doxorubicin, and 9-nitrocamptothecin combinations did not. Taxol-herceptin enhanced growth inhibition in TC71 cells in vitro compared with either agent alone. Ewing's sarcoma growth was also delayed in vivo and mean tumor size was significantly lower in mice treated with herceptin plus taxol than in those receiving taxol or herceptin alone. These data suggest that herceptin in combination with taxol may be a therapeutic option in the treatment of Ewing's sarcoma.

Antibodies, Monoclonal↗
PubMed2006

NR0B1 is required for the oncogenic phenotype mediated by EWS/FLI in Ewing's sarcoma.

A number of solid tumors, such as alveolar rhabdomyosarcoma, synovial sarcoma, and myxoid liposarcoma, are associated with recurrent translocation events that encode fusion proteins. Ewing's sarcoma is a pediatric tumor that serves as a prototype for this tumor class. Ewing's sarcomas usually harbor the (11;22)(q24;q12) translocation. The t(11;22) encodes the EWS/FLI fusion oncoprotein. EWS/FLI functions as an aberrant transcription factor, but the key target genes that are involved in oncogenesis are largely unknown. Although some target genes have been defined, many of these have been identified in heterologous model systems with uncertain relevance to the human disease. To understand the function of EWS/FLI and its targets in a more clinically relevant system, we used retroviral-mediated RNAi to "knock-down" the fusion protein in patient-derived Ewing's sarcoma cell lines. By combining transcriptional profiling data from three of these lines, we identified a conserved transcriptional response to EWS/FLI. The gene that was most reproducibly up-regulated by EWS/FLI was NR0B1. NR0B1 is a developmentally important orphan nuclear receptor with no previously defined role in oncogenesis. We validated NR0B1 as an EWS/FLI-dysregulated gene and confirmed its expression in primary human tumor samples. Functional studies revealed that ongoing NR0B1 expression is required for the transformed phenotype of Ewing's sarcoma. These studies define a new role for NR0B1 in oncogenic transformation and emphasize the utility of analyzing the function of EWS/FLI in Ewing's sarcoma cells.

Animals↗
PubMed1998

Classic Kaposi's sarcoma: low-dose interferon alfa treatment.

BACKGROUND: Classic Kaposi's sarcoma, a rare form of cancer, has no definitive cure. A beneficial effect of low-dose interferon (IFN) alfa was indicated by some case reports. In the present report, we summarize the results of our experience with subcutaneous low-dose IFN alfa treatment of 11 patients with extensive classic Kaposi's sarcoma and review the literature. DESIGN: Eleven patients, 56-96 years old, were treated: 10 men, 1 woman, 3 of whom had an associated malignancy. Subcutaneous injections of IFN alfa were given, starting with 3 million units 5 times a week, and modified according to side effects and response after 2 weeks (2-6 million units, 3-6 times a week). This treatment was continued for 6 months, except for 1 patient who died after 4 months and another who stopped treatment at 4 months due to surgery but was treated again after recurrence. Treatment was continued after 6 months when a partial response was noted, but further improvement was desired (1 patient). RESULTS: In 9 out of the 11 patients, initial response was noted after 3-13 weeks of treatment. This was manifested by a reduction of lesion size and fading of color, leading to a partial resolution. Maximum response was achieved after 4-6 months. Remission lasted 4-72 months. Recurrences were retreated, with additional remissions after only 5-8 weeks of treatment. Side effects included fever and fatigue, which were overcome by dose reduction. CONCLUSION: Whereas in AIDS-related Kaposi's sarcoma patients become refractory to IFN, this was not observed in our patients with classic Kaposi's sarcoma. Moreover, unlike AIDS-related Kaposi's sarcoma, continuous treatment was not needed in classic Kaposi's sarcoma, and recurrences were responsive to retreatment.

Aged↗
PubMed2001

Human herpesvirus 8 DNA in the skin and blood of patients with Mediterranean Kaposi's sarcoma: clinical correlations.

BACKGROUND: Kaposi's sarcoma is a multifocal lympho-angioproliferative disease that appears in elderly subjects of Mediterranean origin (classical form), young Africans and immunodepressed patients (as a result of organ transplantation or AIDS). In 1994, DNA sequences of a new human herpesvirus, called HHV-8, were detected in skin lesions and peripheral blood of patients with AIDS-related Kaposi's sarcoma by confirmational display analysis and polymerase chain reaction. OBJECTIVE: As HHV-8 in peripheral blood mononuclear cells is detected in about 50% of Mediterranean Kaposi's sarcoma patients and its presence fluctuates in time in the same patient, maybe its detection correlates with the clinical behaviour of the disease. METHODS: By using routine and nested polymerase chain reaction we evaluated the presence of HHV-8-specific DNA sequences in the skin lesions, perilesional healthy skin and peripheral blood mononuclear cells of a group of 40 HIV-negative patients with Mediterranean Kaposi's sarcoma. RESULTS: HHV-8 DNA sequences have been found in 40/40 (100%) lesional skin of Mediterranean Kaposi's sarcoma, in 35/40 (85%) perilesional apparently normal skin and in 24/40 (60%) peripheral blood monuclear cell samples. The results of polymerase chain reaction on peripheral blood monuclear cells were positive in 41% of the patients with slowly evolving disease as opposed to 74% of those with rapidly evolving disease, and in 47.6% of the patients with stage I-II disease as opposed to 73.6% of those with stage III-IV. CONCLUSION: The detection of HHV-8 in peripheral blood monuclear cells seems to correlate with the more aggressive stages and the rapid evolution behaviour of Mediterranean Kaposi's sarcoma.

Adult↗
PubMed2003

Central nervous system involvement in children with sarcoma.

OBJECTIVES: To summarize and analyze the experience in CNS involvement (CNSI) in children with sarcomas treated in the above-mentioned institutions. PATIENTS AND METHODS: From 1990 to 2001, all medical charts were retrospectively reviewed: 19 sarcoma patients (12 boys and 7 girls) were diagnosed with CNSI (4 osteogenic sarcomas, 11 Ewing sarcomas, 2 rhabdomyosarcomas, 1 alveolar soft part sarcoma and 1 mesenchymal chondrosarcoma). Mean age of all patients at the time of initial diagnosis was 14.9 years (range: 4-24 years), mean age at the time when CNSI was diagnosed was 16.9 years (range: 5.5-27 years). RESULTS: The frequency of CNSI among our patients was 6.17%. The following symptoms and signs (sometimes combined) presented: headache (10 patients), nausea and vomiting (6 patients), seizures (11 patients) and focal neurological signs (9 patients). The mean duration of time elapsed since diagnosis of CNSI till death or last follow-up was 5.2 months (SD: +/-5.7 months). Four patients received chemotherapy (CT) alone, 8 CT and radiotherapy (RT), 2 RT alone, 3 supportive treatment only, 1 CT and surgery and 1 surgery alone. Sixteen patients died; there was no significant difference in the duration of survival between those who were treated with RT or surgery (mean +/- SD: 6.77 +/- 6.56 months) and those who received only CT or supportive treatment (mean +/- SD: 2.60 +/- 2.94 months) (p = 0.07). Brain disease was the main cause of death in all but 1 patient who died 4 days after autologous bone marrow transplantation from uncontrolled sepsis. In 16 patients, CNSI was part of a metastatic disease. CONCLUSIONS: Among children with sarcoma, CNSI is encountered in 6.17% of cases. More effective therapy has to be developed in order to improve their outcome.

Adolescent↗
PubMed1988

Ewing's sarcoma: ex vivo sensitivity towards natural and lymphokine-activated killing.

We studied the ex vivo cell-mediated cytotoxicity of natural killer (NK) and lymphokine-activated killer (LAK) cells against continuously cultured Ewing's sarcoma cells from 3 different patients. Target cell lysis was measured in a 4-hour 51Cr radioisotope release assay. At an effector to target (E:T) ratio of 50:1, the mean (+/- 1 SD) cytolysis by fresh purified large granular lymphocytes (NK cells) was 20 +/- 8, 25 +/- 2, and 21 +/- 3% in Ewing's sarcoma cell lines 6647, 5838, and A4573, respectively. Under identical conditions, NK cells lysed 56 +/- 7% of K562 (a standard NK target), and 3 +/- 3% of Daudi (a standard NK-resistant LAK target). When compared to fresh unseparated peripheral blood mononuclear cells (PBMC), purified NK cells did not exhibit an enhanced cytotoxic reactivity against either Ewing's sarcoma target. In contrast, LAK cells (i.e., PBMC that were preincubated for 4 days in the presence of rIL-2) were highly cytotoxic against all three Ewing's sarcoma targets. LAK activity was dependent on the concentration of rIL-2 used in PBMC cultures. Optimum cell-mediated toxicity against the standard LAK target Daudi (99 +/- 10% cytolysis at 50:1 E:T ratio) was achieved at rIL-2 concentrations of 1,000 u/ml. LAK cells grown under these conditions were also effective against Ewing's sarcoma cells. At an E:T ratio of 50:1, 86 +/- 16, 85 +/- 16, and 67 +/- 13% inhibition was observed in 6647, 5838, and A4573 cells, respectively, as compared to 17 +/- 10, 19 +/- 15, and 29 +/- 11% cytolysis by fresh uninduced PBMC. In summary, our results suggest that rIL-2-induced LAK-type immune effector cells may be of some therapeutic value in the treatment of poor prognosis Ewing's sarcoma.

Cell Line↗
PubMedDate not supplied

Synovial sarcoma of the heart: Report of a case with diagnosis by endoscopic ultrasound-guided fine needle aspiration biopsy.

BACKGROUND: Primary synovial sarcomas of the heart are aggressive and extremely rare tumors. At least 17 cases have been reported in the literature. In all the published cases the diagnosis was based on histologic sections. To our knowledge, this is the first case of primary synovial sarcoma of the heart diagnosed by fine needle aspiration (FNA). CASE: A 36-year-old male with an unremarkable past medical history presented with a 4.4-cm mass arising from the left ventricular wall of the heart. Endoscopic ultrasound guided fine needle aspiration biopsy of the mass revealed a high grade tumor showing an intimate admixture of spindle and epithelial cells. A diagnosis of undifferentiated sarcoma, favor synovial sarcoma, was rendered. Reverse transcription-polymerase chain reaction demonstrated the presence of a SYT-SSX fusion transcript. The patient received 6 cycles of chemotherapy followed by resection of the residual tumor. The histology of the viable tumor showed histologic findings typical of biphasic synovial sarcoma. CONCLUSION: Synovial sarcoma rarely presents as a primary tumor of the heart. Sampling by FNA allows demonstration of the cytomorphologic appearance typical of the tumor and other ancillary studies. The specific genetic abnormality of these tumors allows confirmation by cytogenetic and molecular studies.

Adult↗
PubMedDate not supplied

Fine needle aspiration cytology of primary proximal-type epithelioid sarcoma of the perineum: a case report.

BACKGROUND: Epithelioid sarcoma is a malignant soft tissue tumor of unknown histogenesis. We describe the cytologic findings in a case of primary proximal-type epithelioid sarcoma of the perineum and results of an immunofluorescence analysis of rhabdoid cells from this tumor. To the best of our knowledge, the 3-color immunofluorescence features of proximal-type epithelioid sarcoma have never before been reported. CASE: An 8-cm-diameter mass with a 2.5-cm ulcer was found in the perineum of a 36-year-old man. After excision of the tumor, histopathologic examination of the resected specimen suggested a diagnosis of proximal-type epithelioid sarcoma. Fine needle aspiration cytology showed numerous rhabdoid cells with globular intracytoplasmic inclusions. A few isolated cells and polygonal cells were also observed in the smears. Three-color immunofluorescence analysis indicated that the intracytoplasmic inclusions in the rhabdoid cells were positive for cytokeratin, vimentin and CD34. The cytoplasmic staining pattern differed between rhabdoid and epithelioid sarcoma cells. CONCLUSION: Immunofluorescent staining of rhabdoid cells from a primary perineal proximal-type epithelioid sarcoma revealed an uneven distribution of cytokeratin in intracytoplasmic inclusions, with the highest concentration at the periphery of the inclusions.

Adult↗
PubMed1991

Hyaline droplet accumulation in rodent kidney proximal tubules: an association with histiocytic sarcoma.

Since recognition during the last decade that certain renal carcinogens can initially cause an accumulation of hyaline (protein) droplets in proximal tubules of male rats, it has become appropriate to establish whether this phenomenon of protein overload can also occur in rodent kidneys unrelated to chemical treatment. Kidney tissue from a number of selected rodent studies held in the National Toxicology Program (NTP) or Food and Drug Administration (FDA) archives were evaluated for hyaline droplet accumulation in proximal tubules. The survey concentrated on rats and mice of both sexes bearing hematopoietic tumors, as our preliminary observations had suggested this direction of study. The tissues of 101 Sprague-Dawley, 25 Osborne-Mendel, and 70 Fischer 344 rats and 96 B6C3F1 mice were examined. These animals provided an assortment of tumors including histiocytic sarcoma, lymphocytic lymphoma, mononuclear cell leukemia, and sarcoma. Hyaline droplet accumulation, primarily involving the P2 segment of proximal tubules, was diagnosed in 96% of rats with histiocytic sarcoma (74/77 cases in Sprague-Dawley, 17/18 in Osborne-Mendels, 7/7 in Fischers) and in 55% of B6C3F1 mice with histiocytic sarcoma (18/33 cases). There appeared to be a qualitative correlation between hyaline droplet accumulation and degree of tumor burden. Thus, in cases negative for hyaline droplets, the tumor was often confined to a single location, while increasing involvement of proximal segments beyond P2 occurred with more extensive multi-organ dissemination of the tumor. By immunohistochemistry on 11 cases of rat and 8 cases of mouse histiocytic sarcoma, the protein in hyaline droplets was identified as lysozyme, a known major secretory product of monocytes and macrophages. The hyaline droplets were negative for alpha 1-antitrypsin, alpha 2u-globulin, rat or mouse immunoglobulin, and albumin. More sparsely scattered droplets and granules present in proximal tubules of Fischer rats with mononuclear cell leukemia were negative for lysozyme but positive for either iron or lipofuscin pigment. The study establishes a clear association between renal tubule hyaline droplet and lysozyme accumulation in rats and mice with histiocytic sarcoma. Hyaline droplets secondary to neoplasia should be distinguished from chemically-induced hyaline droplet nephropathy in the male rat involving alpha 2u-globulin.

Animals↗
PubMedDate not supplied

Soft tissue sarcomas in the general population living near a chemical plant in Northern Italy.

BACKGROUND AND OBJECTIVES: After the notification by a general practitioner of a high frequency of soft tissue sarcomas among subjects living close to the industrial area of the city of Mantua (northern Italy), the local Medical Association carried out a formal epidemiologic investigation to corroborate or falsify the hypothesized excess. Several industrial activities of the area were hypothesized to be a source of environmental pollution that might cause soft tissue sarcomas. METHODS: All general practitioners working in the area were requested to report the cases of soft tissue sarcoma diagnosed in the study area. Person-years of observation were computed for all subjects who ever resided in the area between 1984 and 1997. Expected incidence was computed from the cancer registry covering a nearby province (Varese) and from the pool of Italian cancer registries. Peripheral soft tissue sarcoma and visceral sarcomas were included in the analysis. RESULTS AND CONCLUSIONS: Overall, 20 cases were observed in a 13-year period, 8.87 were expected from the Varese province cancer registry and 7.72 from the pool of Italian registries. The corresponding standardized morbidity ratios were 2.25 (95% confidence interval, 1.34-3.47) and 2.6 (95% confidence interval, 1.6-4.0), respectively. A significant excess persisted after excluding 5 cases that had been the object of the original notification. We hypothesize that the soft tissue sarcoma excess may have resulted from environmental pollution by industrial toxic emissions, which likely included 2,3,7,8-tetrachloro-dibenzo-dioxin.

Adult↗
PubMedDate not supplied

The first report of extraosseous Ewing's sarcoma in the rectovaginal septum.

AIMS AND BACKGROUND: To report an extremely rare case of Ewing's sarcoma located in the rectovaginal septum. Ewing's sarcoma is a highly malignant neoplasm of bone, which usually occurs during childhood. Common extraosseous localizations of Ewing's sarcoma include the trunk, extremities, uterus, cervix and vagina. METHODS: A 45-year-old woman presented to us with a six-month history of pain in the lower abdomen during intercourse. Pelvic examination was performed and a palpable mass was found. The mass had a size of 9 x 6 cm, a soft tissue consistency, was partially movable and the patient felt the pain during palpation. Examination of the inguinal lymph nodes revealed no signs of inguinal adenopathy. The results of laboratory tests, rectoscopy, chest X-rays, barium enema and bone scan were normal. Computed tomography (CT) showed an inhomogeneous expansive mass in the rectovaginal septum measuring 8.7 x 6.1 cm, without any signs of rectum or bladder invasion. The vascular structures of the pelvis were normal. At laparotomy the process was judged inoperable and only biopsy of the tumor mass was carried out. Histology showed a neoplasm with small, round to oval cells with scarce cytoplasm. Immunohistology with the monoclonal antibody CD99 (MIC-2 gene product, Ewing's sarcoma marker, clone 12E7, DAKO A/S, Glostrup, Denmark) revealed an extraosseous Ewing's sarcoma. The patient was treated with chemotherapy followed by whole-pelvis external beam radiation and intracavitary brachytherapy. RESULTS: A residual mass measuring 3.5 x 2.5 cm was visible on a control CT scan 18 months after treatment; however, the patient was feeling well and refused surgery to remove the residual mass. CONCLUSIONS: To our knowledge this is the first reported case of extraosseous Ewing's sarcoma in the rectovaginal septum.

Female↗
PubMedDate not supplied

Lymphographic evaluation in bone and soft tissue sarcomas.

A group of 79 patients with soft tissue sarcomas and 46 with primary malignant bone tumors underwent lymphangiography as part of the initial diagnostic workup. In the group of soft tissue sarcomas, the overall incidence of metastases was 22 of 79 (28%). According to the site of origin, the highest incidence was found in tumors originating from the buttocks (57%), followed by those from the inferior limbs (31%). Considering the histology, the incidence ranges from 50% in anaplastic sarcoma, to 43% in rhabdomyosarcoma, to 23% for liposarcoma and fibrosarcoma. The incidence of lymphographically proven metastases in bone tumors was 8 to 46 (17%), with 1 out of 4 in reticulum cell sarcoma, 1/1 in chordoma, and 22% in Ewing's sarcoma. A radiographic/histologic correlation on the lymph nodes was obtained in 19/79 (24%) soft tissue sarcomas and in 4/46 (9%) bone tumors. In the 12 radiographically negative and 11 radiographically positive cases, this correlation was always correct. The results of this study suggest a larger use of lymphography in these tumors.

Bone Neoplasms↗
PubMed1993

Sarcomas involving the abdominal and pelvic cavity.

The principles of management of all sarcomas that involve the abdominal and pelvic cavity are presented. The anatomic sites for the primary malignancy include retroperitoneal sarcomas, pelvic side-wall sarcomas, sarcomas arising from the abdominal viscera, and sarcomas arising from the pelvic organs. All histologic types of sarcomas may be considered together when therapeutic options are being discussed. This presentation stresses surgical removal with an adequate margin of resection as the principal goal of management. The curative treatment of these cancers places great emphasis on the surgeon's knowledge of anatomy, technical skills, innovation, and surgical courage. Systemic chemotherapy and radiotherapy have not shown reproducible efficacy. Complete resection in the absence of tumor spillage remains the only reliable treatment option. Possible benefits of induction chemotherapy and intraperitoneal chemotherapy using cisplatin and doxorubicin in the early postoperative period are presented.

Abdominal Neoplasms↗
PubMed1984

Treatment of epidemic Kaposi's sarcoma with etoposide or a combination of doxorubicin, bleomycin, and vinblastine.

An epidemic of disseminated Kaposi's sarcoma in male homosexuals has recently been described. Forty-one evaluable patients with epidemic Kaposi's sarcoma were treated with etoposide. The majority of these patients had early stage disease, no prior opportunistic infections, and no prior therapy. Twelve patients (30%) achieved complete remission, 19 (46%) partial remission, and ten (24%) no response. With follow-up time to 31 months, the median response duration is nine months. The median survival of patients with complete and partial remissions has not been reached. A combination of doxorubicin (Adriamycin, Adria Laboratories, Columbus, Ohio), bleomycin, and vinblastine (ABV) was used in 31 evaluable patients with epidemic Kaposi's sarcoma. The majority of these patients had late stage disease, prior opportunistic infections, or had failed prior treatment. Seven patients (23%) achieved complete remission, 19 (61%) partial remission, and five (61%) no response. With follow-up time to 24 months, the median response duration is eight months. The projected median survival for all patients treated with ABV is nine months. Both regimens were well tolerated, with an overall response rate of 76% for etoposide and 84% for ABV. However, while successfully treating the Kaposi's sarcoma, the underlying immune deficiency in these patients has persisted. Future treatments of Kaposi's sarcoma will need to focus on reversing the underlying immune incompetence as well as controlling the malignant manifestations of Kaposi's sarcoma arising in relation to the acquired immune deficiency syndrome.

Adult↗