[Botryoid sarcoma or racemose sarcoma of the vagina in children. Histology and clinical aspects].
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Diagnosis, Differential↗
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Specificity of antibody induced in sarcoma patients immunized with allogeneic sarcoma cells.
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Absorption↗
[What is your roentgen diagnosis? Osteogenic sarcoma of the distal femoral metaphysis (osteoplastic sarcoma)].
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Adolescent↗
Epidemiologic analysis of Kaposi's sarcoma as an early and later AIDS outcome in homosexual men.
The authors separately studied the epidemiology (risk and risk factors) of Kaposi's sarcoma occurring as an initial acquired immunodeficiency syndrome (AIDS) outcome (early Kaposi's sarcoma) and later after a different initial AIDS outcome (later Kaposi's sarcoma) in a cohort of 2,591 human immunodeficiency virus type 1-infected gay men of the Multicenter AIDS Cohort Study between 1984 and 1992. Among 844 AIDS cases, 202 presented with early Kaposi's sarcoma, 101 subsequently developed later Kaposi's sarcoma, and 541 were not diagnosed with Kaposi's sarcoma. Overall, 37.4% of AIDS cases were diagnosed with Kaposi's sarcoma prior to death. Kaposi's sarcoma diagnosed on the skin was significantly more common with early Kaposi's sarcoma (77.3%) than with later Kaposi's sarcoma (65.1%). Men presenting with an AIDS outcome other than Kaposi's sarcoma were at high risk for later Kaposi's sarcoma. Later Kaposi's sarcoma onset in men with a previous AIDS outcome was associated with the following characteristics: 1) lower immune status prior to AIDS and 2) longer post-AIDS survival. A Kaposi's sarcoma diagnosis in a man with a previous AIDS illness approximately doubled the risk (hazard) for death. Histories of urethral gonorrhea and scabies prior to study entry were more common in early Kaposi's sarcoma cases than in later Kaposi's sarcoma cases. However, self-reported sexual activity at study entry and prior to AIDS onset was highest in the later Kaposi's sarcoma group. In this cohort, cigarette smoking had a protective association against all Kaposi's sarcoma in univariate and multivariate models. Only 21.0% of the later Kaposi's sarcoma and 25.0% of the early Kaposi's sarcoma men smoked at least one-half pack of cigarettes daily at study entry compared with 33.8% of non-Kaposi's sarcoma and 35.5% of seroprevalent men still AIDS free. The reasons for this surprising association are unclear. However, other evidence which documents that habitual smoking alters the immune system (and possibly cytokine levels) in ways that could perhaps influence Kaposi's sarcoma pathogenesis should be considered.
Acquired Immunodeficiency Syndrome↗
Is fine-needle aspiration biopsy a practical alternative to open biopsy for the primary diagnosis of sarcoma? Experience with 140 patients.
We reviewed the clinicopathologic features of 145 consecutive fine-needle aspiration biopsy (FNAB) specimens from 140 patients without a previous diagnosis of sarcoma. Among 138 adequate specimens, 42 bone sarcomas and 80 soft tissue sarcomas were recognized as sarcomas; histologic subtyping was easier in bone than in soft tissue sarcomas and in pediatric than in adult cases. There was no correlation in accuracy of subtyping in low- vs high-grade sarcomas. FNAB was most accurate for subtyping of skeletal osteosarcoma, pediatric small round cell bone/soft tissue sarcomas, synovial sarcoma, skeletal chondrosarcoma, and adult myxoid soft tissue sarcomas. Although almost always recognized as sarcoma, subtyping of adult pleomorphic soft tissue sarcomas generally was not possible but did not influence therapy; all were considered high-grade sarcomas for treatment purposes. There were 4 misinterpretations of subtype in soft tissue sarcomas; none resulted in a change in therapy. Cytogenetic analysis on aspirated material confirmed t(11;22) in 2 Ewing and t(X;18) in 3 synovial sarcomas. No procedure-related complications occurred. Among bone and soft tissue sarcomas, FNAB was sufficient for initiation of definitive therapy in 87% and 83% of patients, respectively. Most FNAB specimens from bone and soft tissue sarcomas are recognized easily as sarcoma, but subtyping seems more accurate in bone sarcomas. Although histologic subtyping of adult soft tissue sarcomas is often impossible, no influence on initial therapy is usually observed. In contrast, subtyping of pediatric sarcomas by FNAB seems highly accurate and is necessary for appropriate therapy.
Biopsy↗
Kaposi's-sarcoma-associated herpesvirus is detected in peripheral blood mononuclear cells of HIV-infected homosexuals more often than in heterosexuals.
PURPOSE: The Kaposi's-sarcoma-associated herpesvirus is thought likely to play an important part in the pathogenesis of Kaposi's sarcoma, which is the most common tumor in AIDS patients. To determine whether peripheral blood is also infected by the virus, we prospectively tested mononuclear cells from HIV-infected individuals, both with and without Kaposi's sarcoma. PATIENTS AND METHODS: Thirty-three patients with AIDS were studied. Twenty-three were homosexuals, and 10 of these patients had Kaposi's sarcoma. All 10 nonhomosexual patients were free of Kaposi's sarcoma. PCR amplification of peripheral blood mononuclear cells was performed for a 233-base-pair segment of Kaposi's-sarcoma-associated herpesvirus on all patients. Fisher's exact test was used to compare the patient groups. RESULTS: Kaposi's-sarcoma-associated herpesvirus sequences were detected in the peripheral blood mononuclear cells of homosexual men both with (7/10) and without (5/13) Kaposi's sarcoma. However, none of the nonhomosexual patients (0/10) had detectable virus. Therefore, those with Kaposi's sarcoma are at greater risk for Kaposi's-sarcoma-associated herpesvirus infection than patients without Kaposi's sarcoma, and among HIV-positive patients without Kaposi's sarcoma, homosexuals are more likely to have detectable Kaposi's-sarcoma-associated herpesvirus than are nonhomosexuals. In the homosexual patients, the presence of virus was unrelated to total CD4+ cell counts. A comparative dilutional analysis showed that the nonhomosexual patients had a low viral load in their peripheral blood mononuclear cells relative to most homosexuals with Kaposi's sarcoma. Sequential studies on two patients revealed clearing of the virus while on therapy; one patient was treated for HIV with the protease inhibitor indinavir, and the other patient was treated for Kaposi's sarcoma with liposomal doxorubicin. CONCLUSIONS: These results indicate that Kaposi's-sarcoma-associated herpesvirus is harbored in peripheral blood mononuclear cells of HIV-infected patients, and that the rates of infection are significantly higher in homosexual men compared with their nonhomosexual counterparts. The significance of viral clearing in response to therapy is unknown but warrants further study, as prophylactic treatment for this virus might alter the occurrence of Kaposi's sarcoma in this susceptible patient population.
AIDS-Related Opportunistic Infections↗
Distinction of endometrial stromal sarcomas from 'hemangiopericytomatous' tumors using a panel of immunohistochemical stains.
Endometrial stromal sarcomas are low-grade malignant tumors that may pose a diagnostic challenge, especially when they are present in an extrauterine site. Owing to the presence of an arborizing vasculature and cells with an undifferentiated appearance, endometrial stromal sarcomas can be confused with several soft-tissue neoplasms. We studied 17 endometrial stromal sarcomas, eight hemangiopericytomas, 14 solitary fibrous tumors, and 16 synovial sarcomas immunohistochemically, detecting the following antigens: CD10, estrogen receptor, progesterone receptor, bcl-2, CD34, smooth muscle antigen, epithelial membrane antigen and cytokeratin (AE1/AE3). Most endometrial stromal sarcomas stained positively for CD10 (16/17), estrogen receptor (17/17), progesterone receptor (15/17), and bcl-2 (17/17). Staining with antismooth muscle antigen was seen in 11 of 17 cases of endometrial stromal sarcoma, with more intense staining seen in areas showing smooth muscle differentiation. Staining with AE1/3 was seen in four of 17 endometrial stromal sarcomas, with two of the positive cases containing epithelioid cells. None of the endometrial stromal sarcomas expressed epithelial membrane antigen or CD34. More than half of the hemangiopericytomas (4/8) and solitary fibrous tumors (9/14) cases demonstrated CD10 expression either focally or in a patchy cytoplasmic and membranous pattern. Hemangiopericytomas, solitary fibrous tumors, and synovial sarcomas did not express estrogen receptor. Four of eight hemangiopericytomas and seven of 14 solitary fibrous tumors also showed patchy progesterone receptor expression. CD34 expression was identified in six of eight hemangiopericytomas and 13 of 14 solitary fibrous tumors, but we did not find expression of CD34 in synovial sarcoma. Differences between endometrial stromal sarcoma and other soft-tissue tumors were detected for all of the immunohistochemical markers (P<0.05), except anti-bcl-2 and AE1/3. Antibodies against CD10 mark a substantial number of hemangiopericytomas and solitary fibrous tumors (albeit not diffusely) and should always be combined with antiestrogen receptor and CD34 when the differential diagnosis includes endometrial stromal sarcoma. Unlike estrogen receptor antibodies, progesterone receptor antibodies show at least focal nuclear staining in most hemangiopericytomas, solitary fibrous tumors and rare synovial sarcomas, and are not useful for this differential diagnosis. All endometrial stromal sarcomas expressed bcl-2, mostly in a diffuse pattern, but this did not distinguish between endometrial stromal sarcoma and mimics. We therefore recommend the use of a small antibody panel comprising anti-CD10, anti-estrogen receptor, and anti-CD34 to distinguish endometrial stromal sarcomas from tumors with a predominant hemangiopericytomatous growth pattern.
Adolescent↗
Detection of herpesvirus-like DNA sequences in Kaposi's sarcoma in patients with and those without HIV infection.
BACKGROUND: Herpesvirus-like DNA sequences have recently been found in lesions from patients with Kaposi's sarcoma and the acquired immunodeficiency syndrome (AIDS). It is not known whether these sequences are also present in classic Kaposi's sarcoma or in the Kaposi's sarcoma that occurs in homosexual men who are seronegative for the human immunodeficiency virus (HIV). METHODS: We analyzed DNA in tissue samples from patients with AIDS-associated Kaposi's sarcoma, patients with classic Kaposi's sarcoma, and HIV-seronegative homosexual men with Kaposi's sarcoma. We also analyzed DNA in samples of uninvolved tissue from these patients and in control tissue from healthy subjects. All samples were tested blindly by polymerase chain reaction (PCR) with specific primers to amplify KS330(233), a herpesvirus-like DNA sequence. RESULTS: The KS330(233) PCR product was found in 20 of 21 tissue samples (95 percent) from the patients with Kaposi's sarcoma, including 10 of the 11 samples from the patients with AIDS-associated Kaposi's sarcoma, all 6 samples from the patients with classic Kaposi's sarcoma, and all 4 samples from the HIV-negative homosexual men with Kaposi's sarcoma. Only 1 of the 21 control samples (5 percent) was positive (odds ratio, 400; 95 percent confidence interval, 19 to 17,300). Of the 14 samples of uninvolved skin from the patients with Kaposi's sarcoma, 3 were positive for KS330(233). Representative PCR-product sequences were more than 98 percent identical for the three types of Kaposi's sarcoma, suggesting that all three are caused by the same agent. CONCLUSIONS: The same herpesvirus-like DNA sequences are present in AIDS-associated Kaposi's sarcoma, classic Kaposi's sarcoma, and the Kaposi's sarcoma that occurs in HIV-negative homosexual men. Therefore, this presumably new human herpesvirus is not solely an opportunistic infection in patients with AIDS, and the three forms of Kaposi's sarcoma may be caused by the same infectious agent.
Acquired Immunodeficiency Syndrome↗
Advances in the pathobiology and treatment of Kaposi sarcoma.
PURPOSE OF REVIEW: Kaposi sarcoma, which has reached epidemic proportions in parts of Africa and in the Western world, continues to pose a problem in patients with AIDS receiving highly active antiretroviral therapy (HAART). This article reviews the new and important information regarding the epidemiology, biology, and management of Kaposi sarcoma that was published in the last year. RECENT FINDINGS: Worldwide Kaposi sarcoma herpesvirus/human herpesvirus 8 (HHV8) seropositivity has been found to exceed the incidence of Kaposi sarcoma. Therefore, investigators have justifiably implicated other cofactors (eg, blood-sucking arthropods, angiotensin converting enzyme inhibitors, hemodialysis, and iron) in the development of Kaposi sarcoma. In the transplant setting, Kaposi sarcoma lesions have been shown to originate from the engraftment of donor tumor cells. The detection of HHV8 in Kaposi sarcoma lesions has provided a new diagnostic tool to help differentiate Kaposi sarcoma from its mimics. Kaposi sarcoma lesional cells, now confirmed to be of lymphatic origin, have been shown to express several chemokine receptors, some of which may help explain the predilection for skin. Regression of Kaposi sarcoma has been characterized histologically for the first time. The finding that some tumor cells can remain in an atrophic state even in completely regressed lesions suggests that they have the potential to recur. Protease inhibitor-based and nonnucleoside reverse transcriptase inhibitor-based HAART regimens have been verified to be similarly effective. Although antiretrovirals have been noted to favorably alter the clinical characteristics of Kaposi sarcoma favorably, they seem not to alter the natural history of this disease. In the HAART era, because CD4 cell count was shown no longer to provide prognostic information about AIDS-related Kaposi sarcoma, the traditional classification system for staging AIDS-related Kaposi sarcoma has been refined. Also, a new staging system for classic Kaposi sarcoma has been proposed. SUMMARY: Numerous advances have emerged regarding Kaposi sarcoma during the last year, many of which still need to be translated into clinically useful information. The results of new clinical trials involving antivirals purposely directed against HHV8 and antiretrovirals for HIV-uninfected people are anticipated. Finally, although investigators during this period did provide us with additional potential therapeutic targets, more novel approaches such as RNA interference and gene therapy have been also proposed as options in the future management of Kaposi sarcoma.
Acquired Immunodeficiency Syndrome↗
Lymphatic dissemination of bone and soft tissue sarcomas: a lymphographic investigation.
The series consisted of 132 patients, 61 with primary bone sarcomas and 71 with primary soft tissue sarcomas. The patients were all evaluated by lymphography. The investigation included both patients who had not yet been treated and patients with suspected or confirmed metastases. All tumour diagnoses were confirmed microscopically. The findings as regards dissemination were based on clinical examinations, laboratory tests, roentgen examinations and lymphographies. In some cases, lymph node biopsies and surgical observations were also used. A total of 151 lymphographies were performed and 281 follow-up films taken. Preoperative lymphography was performed using the technique introduced by Kinmonth. For postoperative lymphography on the stumps of amputated extremities, two simple but useful methods were developed, which are presented here. Changes in the lymphographic appearance of lymph node metastases, the occurrence of new metastases, and the results of treatment were assessed by survey films and repeat lymphography. The generally accepted criteria for metastasis were used as a basis for the analysis of the lymphographic findings. The results may be summarized as follows: 1. Incidence of lymphatic dissemination. Different sarcomas varied greatly in their clinical course, including the frequency of dissemination. The lymphatic involvement in the metastatic cases was as follows: Bone sarcomas: 16 out of 28 (Table 10); of these, 13 were to regional lymph nodes, 8 to distant nodes and 5 to both (Table 14). Soft tissue sarcomas: 24 out of 40 (Table 11). All 24 had metastases in regional nodes, and 8 in distant nodes as well (Table 15). The highest frequencies of lymphatic spread in the different metastasized tumours were found to be: Bone sarcomas: reticulosarcoma 100%, Ewing's sarcoma 50%, osteosarcoma 47%. Soft tissue sarcomas: rhabdomyosarcoma 100%, synovial sarcoma 80%, neurogenic sarcoma 78%, leiomyosarcoma 67%. 2. Time-relation between lymphatic and haematogenic dissemination; The tendency to metastasize first via the lymphatics or via the blood vessels varied. Half of the cases of Ewing's sarcoma and reticulosarcoma had evidence of lymphatic spread before blood-borne metastases were detected. In the osteosarcoma cases, however, lymphatic dissemination was always preceded by haematogenic spread (Table 12). In synovial sarcoma, rhabdomyosarcoma and neurogenic sarcoma, the first dissemination was more frequently lymphatic than haematogenic (Table 13). 3. Possible existence of special lymphographic features of sarcoma metastases. Only reticulosarcoma displayed special characteristics. The lymph node metastases of reticulosarcoma of bone had lymphographic appearances similar to those found in reticulosarcoma of soft tissue or lymph node origin (Fig. 12). The lymph node metastases of other primary bone and soft tissue sarcomas had no specific lymphographic features and were indistinguishable from carcinomatous metastases (Figs 7, 9, 13, 15, 18, 19, 20, 22, 23). 4...
Adolescent↗
The value of FDG-PET in the detection, grading and response to therapy of soft tissue and bone sarcomas; a systematic review and meta-analysis.
BACKGROUND: Sarcomas represent a significant diagnostic and therapeutic challenge that requires techniques to provide better assessment of the disease than provided by traditional means. FDG-PET depicts the increased metabolism in abnormal tissues, enabling visualisation and quantification in vivo. The objective of this review was to assess the diagnostic value of FDG-PET in the detection, grading and therapy response of soft tissue and bone sarcomas. METHODS: A systematic review and meta-analysis of clinical studies on FDG-PET and sarcomas was conducted. Databases of PubMed, Embase and Cochrane were searched for studies. Besides that, the references of identified studies were reviewed. Three reviewers independently assessed the methodological quality. Statistical pooling was possible for studies concerning detection and grading of studies with mixed sarcomas (soft tissue and bone) and studies with soft tissue sarcomas only. RESULTS: Twenty-nine studies met the inclusion criteria. There was disagreement between the reviewers in 21.5% of the questions from the criteria list. The methodological quality of most of the included studies was poor. Pooled sensitivity, specificity and accuracy of PET for the detection of sarcomas were 0.91, 0.85 and 0.88, respectively. The difference between the mean Standard Uptake Value (SUV) in malignant and benign tumours for the studies concerning mixed and soft tissue sarcomas was statistically significant, as well as the difference in FDG uptake between low and high grade mixed sarcomas. CONCLUSIONS: The meta-analysis in this study was limited by the fact that only a few studies had mutual comparable outcome parameters. Moreover, the methodological quality of the studies was generally poor. Nevertheless, our results indicate that FDG-PET can discriminate between sarcomas and benign tumours and low and high grade sarcomas based on the mean SUV. The diagnostic implications of these results have to be investigated, especially the discrimination between benign tumours and low grade sarcomas. Based on this meta-analysis, there is no indication to use FDG-PET in the standard treatment of sarcomas. In the future PET imaging in bone and soft tissue sarcomas should be directed to the clinical implication for the detection and grading of sarcomas and the treatment evaluation of locally advanced sarcomas.
Adolescent↗
Radiation-associated sarcomas are characterized by complex karyotypes with frequent rearrangements of chromosome arm 3p.
Ionizing radiation is a well-known risk factor for sarcoma development. To investigate whether radiation-associated sarcomas are characterized by chromosome aberrations that distinguish them from de novo sarcomas, we identified those patients in our series of more than 500 cytogenetically abnormal sarcomas that fulfilled the following criteria: (1) each patient should have been irradiated for another malignancy at least 3 years prior to the sarcoma diagnosis, and (2) the sarcoma should have developed within the field of radiation. Ten patients fulfilling these criteria could be retrieved (median age at sarcoma diagnosis was 55 years, range 17-79; median latency period between primary tumor and radiation-associated sarcoma was 9 years, range 4-30). The diagnoses were typical for radiation-associated sarcomas: 2 each of malignant fibrous histiocytoma, leiomyosarcoma, and pleomorphic sarcoma, and 1 each of osteosarcoma, fibrosarcoma, myxofibrosarcoma, and spindle cell sarcoma. All 10 cases had relatively complex karyotypes with multiple, mostly unbalanced, structural rearrangements, similar to what has been reported in de novo sarcomas of the corresponding histologic subtypes. The only cytogenetic features that were unusually frequent among the radiation-associated sarcomas were the finding of unrelated clones in 3 cases, and loss of material from chromosome arm 3p, in particular 3p21-3pter, in 8 cases. Loss of the same chromosome segment has been described in 4 of the 8 previously published cases of radiation-associated sarcomas that have been analyzed after short-term culturing, which makes this imbalance significantly (P < 0.001) more frequent among radiation-associated sarcomas (12 of 18 cases) than among unselected cases of the corresponding histologic subtypes (74 of 282 cases). In contrast to the cytogenetic results, no 3p deletions were detected among the 6 cases of the present series that could be analyzed by comparative genomic hybridization (CGH). The most frequent imbalance detected by CGH was gain of 15cen-q15 (3 cases), followed by loss of chromosome 13 and gain of 5p, and 7cen-q22, each detected in 2 cases.
Adolescent↗
Overview of sarcomas in the adolescent and young adult population.
Based on the data of the Surveillance, Epidemiology and End Results Section of the National Cancer Institute (SEER) program, soft tissue and bone sarcomas account for about 1% of all new malignancies diagnosed in the United States each year. However, there are numerous different histologic types, and any given type of sarcoma is extremely rare. Determining the incidence of sarcomas by age and type is difficult due to the limited data reported. The SEER program collects data regarding age but only limited data on histology, while most series reported in the literature include either adults or pediatric patients, but rarely both. In an effort to estimate the frequency and absolute numbers of different sarcomas in the adolescent and young adult population, the University of Texas M. D. Anderson Cancer Center (MDACC) tumor registry was queried for all soft tissue sarcomas from 1990 through 2003, and all bone sarcomas from 1990 through 2002. Based on this query, an overview of sarcomas that occur predominantly in the adolescent and young adult (AYA) population is presented. These sarcomas include rhabdomyosarcoma, synovial sarcoma, neurogenic sarcoma, epithelioid sarcomas, alveolar soft parts sarcoma, Ewing sarcoma, and osteosarcoma. Using the percentages for occurrence of each histologic type determined from the MDACC database, and the SEER estimate of overall sarcoma incidence, an estimate of the number of new cases in 2004 for the predominant histologic types occurring in the AYA population are presented. Also reviewed are the chromosomal translocations that occur frequently in sarcomas presenting in the AYA population.
Adolescent↗
Sarcomas in hamsters after injection with Rous chicken tumor material.
Newborn hamsters were injected subcutaneously with a suspension of finely minced Rous chicken sarcoma (Schmidt-Ruppin strain). After an interval of about 2 weeks, progressively growing sarcomas developed at the site of injection in almost all animals. Also in adult hamsters inoculated intramuscularly with the same material sarcomas developed at the site of injection within 2 to 4 months. Secondary growths appeared on the peritoneal surface, in the retroperitoneal and mediastinal lymph nodes and in the lungs. The sarcomas usually had a pleomorphic appearance and showed a certain resemblance to rhabdomyosarcoma, but sometimes they had the character of spindle cell sarcomas of varying degree of maturity. Sarcomas were not only obtained in hamsters injected with cellular material from the Rous chicken sarcoma but were also seen in hamsters which were injected at birth or when 2 months' old with supernatant fluid obtained by repeated centrifugation of suspensions of homogenized chicken sarcoma, and presumed to be cell-free. The hamster sarcoma was transplanted to a newborn hamster and could then without difficulties be passed in series in hamsters. All attempts to transfer the sarcoma from hamster to hamster by means of cell-free material from the hamster sarcoma failed. On the other hand, material from the hamster sarcomas inoculated into chickens induced rapidly growing Rous sarcomas at the site of inoculation. This proved possible not only with material from the first but also from later passages of the tumor in hamsters. It is concluded that the strain of Rous virus used has the capacity to induce sarcomas not only in chickens but also in hamsters.
Animals↗
Human sarcomas in culture. Foci of altered cells and a common antigen; induction of foci and antigen in human fibroblast cultures by filtrates.
In a study of human sarcomas maintained in culture for periods up to two years, the following observations were made. The most prominent cell type in serially cultured osteosarcomas was fibroblastic in appearance. After 16-20 wk in culture some lines spontaneously developed foci of altered cells resembling the foci produced in monolayer cultures by oncogenic viruses. The presence of these foci in the sarcoma cultures was transient, and usually they did not reappear; but in one instance they recurred with a characteristic periodicity of several weeks. From one of the sarcoma lines, in which foci appeared after 5 months in culture, two subcultures were established from stored frozen cells and these both exhibited foci after approximately the same lapse of time. The same phenomenon has been seen with another line, suggesting that the time of appearance of foci is characteristic for particular sarcomas. Foci of similar type could sometimes be induced in monolayer cultures of human fibroblasts by filtered medium from cultured sarcomas; this bore no relation to the presence or absence of foci in the sarcoma cultures at the time the filtrate was prepared. Electron microscopy of the spontaneous and induced foci, and of the sarcoma cultures, revealed no demonstrable virus. 12 out of 15 sarcoma cultures contained an antigen (S) demonstrable by indirect immunofluorescence with human sera. It was not present in any of the original sarcoma specimens, nor in any culture lines other than sarcomas. At least 3-4 wk in culture appear to be required for its demonstration. The antigen was cytoplasmic, occurred in only a small proportion of the cells, and was unpredictably variable in its expression, even in the same culture line. It could be induced in monolayer cultures of human fibroblasts by filtrates of medium from sarcoma cultures. As with the foci, the induction of S antigen in indicator cultures was not dependent upon the expression of antigen in the sarcoma line from which the filtrates were obtained. There was no association between the presence of foci and of antigen, nor was there any apparent relation between the ability of filtrates to induce foci and their ability to induce antigen. 80% or more of the general population have S antibody, and the titer of antibody in patients with sarcoma is no higher than in normal subjects. Thus, as in the case of Burkitt's lymphoma antigen, it appears that most individuals have been exposed to S antigen. But unlike Burkitt's lymphoma, no relation has so far been established between any particular disease and a corresponding high titer or frequency of occurrence of S antibody. The occurrence of foci of altered cells and of a common antigen, and the transmission of these two characters to indicator cells by filtrates, are all suggestive of a virus specifically associated with human sarcomas, one to which the general population is widely exposed, as indicated by the prevalence of antibody.
Adenocarcinoma↗
Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the Institute of Oncology, Ljubljana.
BACKGROUND AND OBJECTIVES: Retroperitoneal sarcomas are rare malignant tumors with an aggressive course of disease and high local recurrence rate. Local disease is the main cause of death. A retrospective study was undertaken to evaluate the results of aggressive surgical treatment in a series of patients of primary and recurrent retroperitoneal sarcomas. PATIENTS AND METHODS: From 1975 to 2000, 155 patients with retroperitoneal tumors were operated on at the Institute of Oncology, Ljubljana. Patients with fibromatosis and children less than 16 years were excluded from further calculation. Out of the rest 139 patients, 102 patients had localized retroperitoneal sarcoma, and 37 patients had retroperitoneal sarcoma with distant metastases Leiomyosarcoma was the most common type of histology, followed by liposarcoma and schwannoma. Their referral status was as follow: 56 had primary sarcoma, 20 residual sarcoma after operation elsewhere and 26 already recurrent sarcoma. Our treatment approach was aggressive. We removed surgically primary tumor, recurrent sarcoma and metastases, whenever possible. To this end, we performed, 235 operations for retroperitoneal sarcomas on 139 patients (up to 8 operations on 1 patient). RESULTS: Complete resection was done in 97 of 102 patients with localized sarcoma (resectability rate 95%); in 55 patients, R0 resection was made, in 42 patients, R1 resection, in 3 patients, debulking procedure, and in 2 patients biopsy alone. In 118, patients at least one organ was resected completely or partially en bloc with tumor. The 5-, 10-, and 15-year survivals of patients with localized sarcoma were 52%, 36%, and 22%, respectively. The 5-year survival of patients with metastases was 12% (P = 0.0002), the 5- and 10-year survival rates of patients with R0 resection were 75% and 64%, respectively, and of those with R1 resection, 25% and 8%, respectively (0 < 0.00001). High tumor grade was associated with poor survival. The 5-year local recurrence rate of patients with primary sarcoma and of those with locally recurrent sarcoma was 37% and 71%, respectively (P = 0.04). After the first local recurrence, the 5- and 10-year survival rates were 42% and 26%, after the second local recurrence 45% and 22%, after the third local recurrence 43% and 11%, and after the fourth local recurrence 51% and 17%, respectively. CONCLUSION: Complete surgical resection without microscopic residuum and contamination is likely to offer the best chances for long-term survival. Until there are no other treatment modalities, aggressive surgery for recurrent sarcoma is recommended.
Adolescent↗
Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma.
BACKGROUND: Survivors of hereditary retinoblastoma have an increased risk for second malignancies, especially soft tissue sarcomas. However, the risks of individual histologic subtypes of soft tissue sarcomas have not been evaluated. METHODS: We estimated the risk for six subtypes of soft tissue sarcomas (fibrosarcoma, liposarcoma, histiocytoma, leiomyosarcoma, rhabdomyosarcoma, and others) in a cohort of 963 one-year survivors of hereditary retinoblastoma among patients diagnosed at two US institutions from 1914 through 1984. We calculated standardized incidence ratios (SIRs) for specific subtypes of soft tissue sarcomas by comparison with population data from the Connecticut Tumor Registry or from National Cancer Institute Surveillance, Epidemiology, and End Results database. We also calculated the cumulative risk for all soft tissue sarcomas combined. RESULTS: We observed 69 soft tissue sarcomas in 68 patients with hereditary retinoblastoma. Risks were elevated for soft tissue sarcomas overall (SIR = 184, 95% confidence interval [CI] = 143 to 233) and for individual subtypes. Leiomyosarcoma was the most frequent subtype (SIR = 390, 95% CI = 247 to 585), with 78% of leiomyosarcomas diagnosed 30 or more years after the retinoblastoma diagnosis (SIR = 435, 95% CI = 258 to 687). Among patients treated with radiotherapy for retinoblastoma, we found statistically significantly increased risks of soft tissue sarcomas in the field of radiation. Irradiated patients also had increased risks of soft tissue sarcomas, especially leiomyosarcomas, outside the field of radiation, and risks of soft tissue sarcomas were increased in nonirradiated patients as well, indicating a genetic predisposition to soft tissue sarcomas independent of radiation. The cumulative risk for any soft tissue sarcoma 50 years after radiotherapy for retinoblastoma was 13.1% (95% CI = 9.7% to 17.0%). CONCLUSION: Long-term follow-up of a cohort of survivors of hereditary retinoblastoma revealed a statistically significant excess of leiomyosarcoma and other soft tissue sarcomas that persists decades after the retinoblastoma diagnosis. Retinoblastoma survivors should undergo regular medical surveillance for sarcomas in their adult years.
Adult↗
The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma.
The etiology of Kaposi's sarcoma remains somewhat obscure. While lesions of classic Kaposi's sarcoma, African Kaposi's sarcoma, and immunosuppressed Kaposi's sarcoma have been found to be indistinguishable from one another, the reasons for the variations in type and severity have not been established. The origin of the spindle cell is yet to be agreed on. Geographic variation does not seem as important as ethnic variation. The very young and the very old, perhaps two ages of weakened immunity, tend to have a higher incidence of Kaposi's sarcoma. Children and AIDS patients tend to develop more virulent disease. Males tend to get Kaposi's sarcoma at higher rates than do females. Jewish and Mediterranean males have the highest incidence of classic Kaposi's sarcoma, and African Bantu have the highest incidence of African Kaposi's sarcoma, classifications which do not apply to the Kaposi's sarcoma population in the United States. Male homosexuals have much higher incidence of Kaposi's sarcoma than do male heterosexuals, but since the early 1980s, its incidence as the presenting manifestation of AIDS has decreased dramatically. There is no unequivocal association with HLA haplotype (though DR5 carriers may be at especially high risk) or evidence of family clustering. There is an impressive but not always consistent association between Kaposi's sarcoma development and immunodeficiency. Environmental factors, such as nitrite use, immunosuppression, and repeated cytomegalovirus infection, are associated with Kaposi's sarcoma, but the exact mechanism is unclear and the associations remain inconsistent. Finally, it is still unclear if there is a causative infectious agent for Kaposi's sarcoma. While cytomegalovirus has been linked to Kaposi's sarcoma, there are weaknesses in its hypothetical role as an etiologic agent as is the case for HIV itself.(ABSTRACT TRUNCATED AT 400 WORDS)
Acquired Immunodeficiency Syndrome↗