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PubMed1999

[MRI diagnosis in longitudinal stress fractures: differential diagnosis of Ewing sarcoma].

PURPOSE: To compare MR imaging features of stress fractures, simulating malignancies, and Ewing sarcomas. MATERIALS AND METHODS: MR imaging studies of 4 patients with longitudinal stress fractures of the tibia (n = 2) and the femur (n = 2) simulating malignancy were retrospectively compared with the MRI scans of 10 patients with histologically proven Ewing sarcoma (femur n = 5; tibia n = 3, fibula n = 1, humerus n = 1). The diagnosis of stress fractures was confirmed by follow-up examinations. An additional biopsy was performed in two patients. RESULTS: Despite negative x-ray examinations, MRI showed the fracture line in all patients with stress fractures. In these cases marrow edema was irregular and there was no well defined margin towards normal fatty marrow. In contrast Ewing sarcomas were sharply demarcated in 9/10 cases. Extraosseous enhancing soft tissue was found in Ewing sarcomas as well as in stress fractures. In stress fractures the enhancing mass was repair tissue. Areas of necrosis within the enhancing mass was seen in (8/10) Ewing sarcomas, only. In follow-up studies we observed a decrease of the marrow edema in patients with stress fractures. Occurrence of low signal areas in T1- and T2-weighted sequences within the initial enhanced extraosseous tissue corresponded to bony callus on x-rays films. CONCLUSIONS: Repair tissue in stress fractures can imitate malignancy. The irregularity of the marrow edema without well defined margins, the lack of necrosis in the small enhancing tissue and the proof of the fracture line in the MRI are criteria to differentiate stress fractures from Ewing sarcomas. Short-term follow up studies are helpful to underline the diagnosis.

Adolescent↗
PubMed1992

Prevalence and spectrum of germline mutations of the p53 gene among patients with sarcoma.

BACKGROUND: Recent studies have identified germline mutations of the p53 tumor-suppressor gene in families with the Li-Fraumeni syndrome, a rare inherited disorder characterized by a high risk of sarcomas of bone and soft tissue, breast cancer, and other tumors. In this report, we address the possibility that some sporadic sarcomas may be associated with new germline mutations of the p53 gene, which would not be manifested as familial cancer unless the patient survived to reproduce. METHODS: We studied DNA from peripheral leukocytes of 196 patients with sarcoma and from 200 controls. Of the 196 patients with sarcoma, 15 were selected because they had had multiple primary cancers or had a family history of cancer. The entire coding sequence and splice junctions of the p53 gene were analyzed for mutations. RESULTS: Eight germline mutations were found, three in patients with no known family history of cancer and five in patients with an unusual personal or family history of cancer. Four mutations caused amino acid substitutions, and four caused stop codons. These mutations were not present in any of the 200 controls. CONCLUSIONS: New germline mutations of the p53 gene are rare among patients with "sporadic" sarcoma but may be common in patients with sarcoma whose background includes either multiple primary cancers or a family history of cancer. Diverse mutations of this gene were associated with an increased likelihood of cancer; hence, the entire gene should be considered a target for heritable mutation. It appears that the group of patients with cancer who carry germline mutations of the p53 gene is more diverse than is suggested by the clinical definition of the Li-Fraumeni syndrome. The identification of carriers could be of substantial clinical importance.

Adolescent↗
PubMed1995

Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas.

BACKGROUND: DNA fragments that appeared to belong to an unidentified human herpesvirus were recently found in more than 90 percent of Kaposi's sarcoma lesions associated with the acquired immunodeficiency syndrome (AIDS). These fragments were also found in 6 of 39 tissue samples without Kaposi's sarcoma, including 3 malignant lymphomas, from patients with AIDS, but not in samples from patients without AIDS. METHODS: We examined the DNA of 193 lymphomas from 42 patients with AIDS and 151 patients who did not have AIDS. We searched the DNA for sequences of Kaposi's sarcoma-associated herpesvirus (KSHV) by Southern blot hybridization, the polymerase chain reaction (PCR), or both. The PCR products in the positive samples were sequences and compared with the KSHV sequences in Kaposi's sarcoma tissues from patients with AIDS. RESULTS: KSHV sequences were identified in eight lymphomas in patients infected with the human immunodeficiency virus. All eight, and only these eight, were body-cavity-based lymphomas--that is, they were characterized by pleural, pericardial, or peritoneal lymphomatous effusions. All eight lymphomas also contained the Epstein-Barr viral genome. KSHV sequences were not found in the other 185 lymphomas. KSHV sequences were 40 to 80 times more abundant in the body-cavity-based lymphomas than in the Kaposi's sarcoma lesions. A high degree of conservation of KSHV sequences in Kaposi's sarcoma and in the eight lymphomas suggests the presence of the same agent in both lesions. CONCLUSIONS: The recently discovered KSHV DNA sequences occur in an unusual subgroup of AIDS-related B-cell lymphomas, but not in any other lymphoid neoplasm studied thus far. Our finding strongly suggests that a novel herpesvirus has a pathogenic role in AIDS-related body-cavity-based lymphomas.

Acquired Immunodeficiency Syndrome↗
PubMed2003

Immunomic analysis of human sarcoma.

The screening of cDNA expression libraries from human tumors with serum antibody (SEREX) has proven to be a powerful method for identifying the repertoire of tumor antigens recognized by the immune system of cancer patients, referred to as the cancer immunome. In this regard, cancer/testis (CT) antigens are of particular interest because of their immunogenicity and restricted expression patterns. Synoivial sarcomas are striking with regard to CT antigen expression, with >80% of specimens homogeneously expressing NY-ESO-1 and MAGE-A3. In the present study, 54 sarcoma patients were tested for serum antibodies to NY-ESO-1, SSX2, MAGE-A1, MAGE-A3, MAGE-A4, MAGE-A10, CT7, and CT10. Two patients had detectable antibodies to CT antigens, and this seroreactivity was restricted to NY-ESO-1. Thus, although highly expressed in sarcoma, CT antigens do not induce frequent humoral immune responses in sarcoma patients. Sera from these two patients were used to immunoscreen cDNA libraries from two synovial sarcoma cell lines and normal testis, resulting in the identification of 113 distinct antigens. Thirty-nine antigens were previously identified by SEREX analysis of other tumor types, and 2339 antigens (59%) had a serological profile that was not restricted to cancer patients, indicating that only a proportion of SEREX-defined antigens are cancer-related. A novel CT antigen, NY-SAR-35, mapping to chromosome Xq28 was identified among the cancer-related antigens, and encodes a putative extracellular protein. In addition to testis-restricted expression, NY-SAR-35 mRNA was expressed in sarcoma, melanoma, esophageal cancer, lung cancer and breast cancer. NY-SAR-35 is therefore a potential target for cancer vaccines and monoclonal antibody-based immunotherapies.

Antigens, Neoplasm↗
PubMed1974

Sarcoma-virus-related RNA sequences in normal rat cells.

A rat type C virus spontaneously activated from the NRK (normal rat kidney) cell line was found to have two major size classes of viral RNA subunits sedimenting at 35 and 30 S. Virus-producing cells contained both RNA species, while normal "virus-free" rat cells contained primarily virus-specific 30S RNA species. A DNA transcript, specific for Kirsten sarcoma virus, prepared from virus activated in nonproducer BALB/c cells originally transformed by Kirsten sarcoma virus and rendered specific for the virus by absorption of sequences related to mouse helper virus hybridized only with the 30S RNA species of virus-producing rat cells and normal rat cells. These findings are consistent with the hypothesis that sarcoma-specific nucleic acid sequences in kirsten sarcoma virus emerged through a process that incorporated some portions of 30S RNA species from rat cells (either normal or virus-producing) into the Kirsten leukemia virus during passage in vivo of that virus. The virus designated M-MSV(RaLV), which originally derived from tumor induced by Moloney sarcoma virus (M-MSV) in rats, contained 35S RNA species of rat type C viruses and 30S RNA species specific for both rat and mouse viruses. It appears striking that for these two animal species, sarcoma-virus-specific information resides on a 30S subunit.

Animals↗
PubMed1997

Diet and risk of lymphoid neoplasms and soft tissue sarcomas.

The relationship between frequency of intake of selected indicator foods, lymphoid neoplasms, and soft tissue sarcomas was investigated in an updated case-control study conducted in Northern Italy between 1983 and 1992 on 158 incident, histologically confirmed cases of Hodgkin's disease (HD), 429 cases of non-Hodgkin's lymphoma (NHL), 141 cases of multiple myelomas, 101 cases of soft tissue sarcomas, and 1,157 controls admitted to hospital for acute, nonneoplastic diseases unrelated to long-term modifications of diet. Compared with the lowest tertile, the odds ratio (OR) for the highest tertile of milk intake was 1.8 for NHL and 1.9 for sarcomas. Liver intake was an indicator of the risk of HD (OR = 1.8), NHL (OR = 1.6), and myelomas (OR = 2.0), ham an indicator of HD (OR = 1.7), and butter an indicator of myelomas (OR = 2.8). A high consumption of green vegetables was inversely related to myelomas (OR = 0.4), and frequent use of whole-grain foods was inversely related to NHL (OR = 0.4) and soft tissue sarcomas (OR = 0.2). No material association with meat was observed for any of the neoplasms considered. Likewise, coffee and alcohol intakes were not associated with lymphoid neoplasms and soft tissue sarcomas. The OR for the highest tertile of intake of beta-carotene ranged between 0.5 and 0.7, whereas the OR for retinol ranged between 1.5 and 2.3. Although available data do not point to any specific inference, this study suggests that certain aspects of diet are a correlate or an indicator of the risk of lymphoid neoplasms and soft tissue sarcomas.

Adolescent↗
PubMed1987

Epithelioid sarcoma. An immunohistochemical study.

The immunohistochemical findings in 14 epithelioid sarcomas, neoplasms of uncertain histogenesis, indicate that they react with antibodies against cytokeratin, epithelial membrane antigen, and vimentin. All cases were nonreactive for leukocyte common antigen, myoglobin, and Factor VIII-related antigen. These results point to the fact that epithelioid sarcoma expresses phenotypic characteristics more often associated with epithelioid neoplasms, rather than the mesenchymal profile of most soft tissue sarcomas. One explanation for this observation is that epithelioid sarcoma is in fact a carcinoma originating in the deep soft tissues. On the other hand, the pluripotential mesoderm has a known embryonic capacity to differentiate into epithelium and, therefore, it is plausible that epithelioid sarcoma is a mesenchymally derived neoplasm. Aside from histogenetic considerations, epithelioid sarcoma may be confused with a number of other neoplastic and granulomatous processes. Differential immunohistochemical stains are useful in selected instances wherein light and electron microscopic findings are diagnostically equivocal.

Adolescent↗
PubMed1997

Risk of Kaposi's sarcoma and oroanal sexual contact.

After contradictory findings from a number of previous studies, behavioral risk factors for Kaposi's sarcoma were examined in a case-control study of 202 people diagnosed with acquired immunodeficiency syndrome (AIDS) in 1991-1993 in Sydney, Australia. Cases comprised 67 men who developed Kaposi's sarcoma at or after a diagnosis of acquired immunodeficiency syndrome, and controls were 135 people who did not have Kaposi's sarcoma at the time of diagnosis of acquired immunodeficiency syndrome or during follow-up until 1995. Men who developed Kaposi's sarcoma were more likely to report having a history of sexually transmissible diseases and having engaged more frequently than controls in a number of sexual practices with casual partners in the period before they became aware of their human immunodeficiency virus (HIV) infection. However, the only sexual practice reported significantly more often by cases at the 0.05 significance level was insertive oroanal contact with casual partners (odds ratio = 2.6, 95 percent confidence interval 1.3-5.3). This association was not present for insertive oroanal contact with regular partners or for insertive oroanal contact after subjects became aware of their HIV infection. The relation was present both in men who had Kaposi's sarcoma at the time of interview and in those who developed it later. The relation was not affected by adjustment for time of HIV infection and diagnosis or for other sexual practices. These results can be interpreted as supporting the hypothesis that Kaposi's sarcoma in people with HIV is caused by an infectious agent transmitted by oral contact with feces.

Acquired Immunodeficiency Syndrome↗
PubMed1992

The value of immunohistochemistry in the diagnosis of soft tissue sarcomas.

Histological classification of soft tissue sarcomas can be made by light microscopy alone for those tumors with unique appearances, while a significant proportion of sarcomas require the implementation of electron microscopy or immunohistochemistry to obtain a final diagnostic classification. Immunostaining methods are particularly valuable in certain classes of sarcomas such as epithelioid sarcomas, malignant schwannomas, hemangiosarcomas, clear cell sarcomas, rhabdomyosarcomas, pleomorphic liposarcomas, leiomyosarcomas and synovial sarcomas through documentation of their cellular products. Immunohistochemistry is not a substitute for skilled interpretation of conventionally stained microscopic specimens; however, the availability of more specific determinants in the soft tissue neoplasms will expand the value of this approach in this area of diagnostic pathology.

Humans↗
PubMed2005

Prognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden Hospital.

AIMS: Key prognostic factors at diagnosis of synovial sarcoma are well defined from the literature. There are few data regarding prognostic parameters in the setting of advanced disease. Our aim was to look specifically at a cohort of patients with advanced synovial sarcoma and to identify potential prognostic factors. PATIENTS AND METHODS: One hundred and four patients with advanced synovial sarcoma were identified from the Royal Marsden Hospital's sarcoma database between 1978 and 2003. Patient data were analysed retrospectively. Most patients were aged between 20 and 50 years at diagnosis. Seventy-one patients were deceased at the time of analysis. Ninety-two patients received chemotherapy for management of advanced disease (most commonly doxorubicin + ifosfamide). RESULTS: Median survival following development of advanced disease was 22 months. Predictors of survival with advanced disease were age <35 years (P = 0.03) and response to first-line chemotherapy (P = 0.05). The response rate to doxorubicin plus ifosfamide was 58.6%, and this was superior to either agent when given singly. Metastasectomy was not associated with improved prognosis in this series. CONCLUSIONS: Synovial sarcoma is a chemosensitive soft tissue sarcoma. Compared with historical controls, survival with advanced disease seems to have improved over the years, possibly as a result of better use of chemotherapy. Age <35 years and response to first-line chemotherapy predict for improved survival with advanced disease.

Adult↗
PubMed1980

Manganese inhibition of sarcoma induction by benzo[a]pyrene in rats.

The effects of Mn and Cr dusts upon carcinogenesis by polycyclic aromatic hydrocarbons were tested in male Fischer 344 rats. In Experiment I, rats were given i.m. injections of benzo[a]pyrene (BP, 1.2 mg), Mn dust (4.4 mg), and Cr dust (4.4 mg), alone, and in various combinations. By 100 weeks, sarcomas occurred at the injection site in 17/20 rats that received BP alone, versus 10/19 rats that received BP plus Mn dust (P < 0.05). The sarcoma incidences were 0/20 in 3 control groups that received the vehicle, Mn dust alone, or Cr dust alone, and 20/20 in rats that received BP plus Cr dust. In Experiment II, rats were given i.m. injections of BP (0.6 mg), 7,12-dimethylbenz[a]anthracene (DMBA, 0.6 mg), Mn dust (4.4 mg), and Cr dust (4.4 mg), alone, and in various combinations. Local sarcomas occurred in 17/20 rats that received BP alone, versus 5/17 rats that received BP plus Mn dust (P < 0.001). Sarcoma induction was not inhibited when BP was injected in the right thigh and Mn dust in the left thigh (sarcoma incidence = 16/20). The sarcoma incidences were 0/19 in a vehicle control group; 0/18 in 2 control groups that received Mn dust or Cr dust alone; 18/20 in rats that received BP plus Cr dust; 14/20 in rats that received DMBA alone; 17/19 in rats that received DMBA plus Mn dust; and 13/18 in rats that received DMBA plus Cr dust. These experiments show that Mn dust inhibits the carcinogenicity of BP when the Mn dust and BP are administered to rats by a single i.m. injection. Under identical conditions, Mn dust does not affect the carcinogenicity of DMBA; Cr dust does not affect the carcinogenicity of BP or DMBA.

9,10-Dimethyl-1,2-benzanthracene↗
PubMed2005

Primary pulmonary synovial sarcoma confirmed by molecular detection of SYT-SSX1 fusion gene transcripts: a case report and review of the literature.

This is a case report of a rare patient with primary pulmonary synovial sarcoma. The patient was a 58-year-old woman who presented with a well-defined giant mass in the right lower field on a chest radiograph. A malignant pulmonary tumor was suspected and consequently a right middle and lower lobectomy was performed. Grossly, the tumor measured 10 x 8 x 7 cm, was whitish-yellow in color and friable with hemorrhage. Histologically, the tumor showed a dense proliferation of spindle cells. In some areas, a herringbone-like pattern with coagulation necrosis of large size was noted. Immunohistochemically, the tumor cells were focally positive for cytokeratin and epithelial membrane antigen (EMA). As these features suggested a monophasic synovial sarcoma, we looked for the presence of SYT-SSX fusion gene transcripts using RNA samples from the paraffin-embedded tissue. A reverse transcription-polymerase chain reaction (RT-PCR) amplified a single 118 bp fragment characteristic of the SYT-SSX1 fusion gene transcripts. As no tumor was found at other sites, it was diagnosed as primary pulmonary synovial sarcoma. Molecular testing proved to be very helpful or necessary when monophasic spindle cell synovial sarcoma was recognized in uncommon/unexpected sites. In our review of primary pulmonary synovial sarcomas confirmed by molecular detection of SYT-SSX fusion gene transcripts, the SYT-SSX2 fusion protein expression correlates with poorer prognosis. This is in contrast to the association between the SYT-SSX1 fusion protein expression and poorer prognosis in soft tissue synovial sarcomas.

Female↗
PubMed1993

Administration of pentosan polysulfate to patients with human immunodeficiency virus-associated Kaposi's sarcoma.

BACKGROUND: Neovascularization induced by basic fibroblast growth factor (basic FGF) or FGF-like cytokines is thought to play a substantial role in the pathogenesis of human immunodeficiency virus (HIV)-associated Kaposi's sarcoma. Pentosan polysulfate has been shown to inhibit basic FGF and FGF-like dependent tumor growth both in vitro and in vivo. Moreover, it has been found to inhibit the growth of Kaposi's sarcoma-derived spindle cells in vitro. These observations suggested that pentosan polysulfate might be worth exploring as a potential agent for the treatment of Kaposi's sarcoma. PURPOSE: The purpose of this phase 1 clinical trial was to determine the maximum tolerated dose of pentosan polysulfate in patients with HIV-associated Kaposi's sarcoma and whether or not this compound had activity against this neoplasm. METHODS: Sixteen HIV-seropositive patients with Kaposi's sarcoma received pentosan polysulfate via continuous venous infusion for 3-6 weeks and then received a subcutaneous dose three times per week. Three different doses of pentosan polysulfate were administered: 2 mg/kg per day by infusion followed by 2 mg/kg per dose given subcutaneously (six patients), 3 mg/kg per day by infusion followed by 3 mg/kg per dose given subcutaneously (five patients), and 4 mg/kg per day by infusion followed by 4 mg/kg per dose given subcutaneously (five patients). Five of the 16 patients in the study also received injections of 1 mg of pentosan polysulfate into two different lesions two times a week for 3 weeks, followed by intralesional therapy once weekly. After receiving pentosan polysulfate for 6 weeks, patients were administered 100 mg zidovudine (AZT) orally every 4 hours in conjunction with pentosan polysulfate. RESULTS: The maximally tolerated dose of pentosan polysulfate given by continuous venous infusion was found to be 3 mg/kg per day. No patient had an objective clinical antitumor response to either systemic or intralesional pentosan polysulfate administration; however, three patients had stable Kaposi's sarcoma for 3-27 weeks. No statistically significant effect on CD4 cells or serum HIV p24 antigen was noted during pentosan polysulfate administration. Dose-limiting toxic effects were characterized by anticoagulation and thrombocytopenia and were reversible. CONCLUSION: Pentosan polysulfate was well tolerated in this patient population. However, no objective tumor response or evidence of anti-HIV activity was noted; therefore, no claim of activity can be made in this trial. IMPLICATION: Continued investigation into the use of angiogenesis inhibitors with improved activity and toxicity profiles or different mechanisms of action is warranted.

Adult↗
PubMed1994

MDM2 gene amplification and transcript levels in human sarcomas: relationship to TP53 gene status.

BACKGROUND: Alterations of the TP53 tumor suppressor gene appear to be implicated in the tumorigenesis and progression of several types of human cancer, including different histologic subtypes of sarcomas. The MDM2 (murine double minute-2) gene encodes a nuclear phosphoprotein that may interact with both mutant and wild-type p53 proteins, thereby inhibiting p53-mediated transactivation in a dose-dependent manner. Recently it has been suggested that mdm2 and p53 proteins are components of an autoregulatory loop in which the MDM2 gene is transactivated by p53. PURPOSE: Our purpose was to examine the frequency of MDM2 amplifications in larger panels of sarcomas, determine if the mRNA level could be elevated in tumors without concomitant gene amplification, and relate MDM2 findings to the TP53 status of the tumors. METHODS: Sarcoma tissue of different histologic subtypes was obtained from 68 patients at the time of surgery and from 26 human xenografts in nude mice. In addition, two human sarcoma cell lines (OSA and U2OS) were studied. Genomic DNA from tumor tissue, in vitro cell lines, and peripheral blood cells were isolated by Southern-blot analysis methods to determine MDM2 gene amplification. Tumor DNA was analyzed for possible TP53 gene mutations in exons 5, 7, and 8 by constant denaturing gel electrophoresis. To determine the MDM2 and TP53 mRNA levels, Northern-blot analysis was performed. RESULTS: Amplification of the MDM2 gene was detected in 10 tumors (10.3%). Whereas MDM2 amplification and/or over-expression were found only in two (U2OS and OSA cell lines) of 18 osteosarcomas, one of 20 malignant fibrous histiocytomas (MFHs), and in none of 14 leiomyosarcomas, such alterations were observed in two of two fibrosarcomas, three of six malignant schwannomas, three of 19 liposarcomas, and in the one hemangiopericytoma examined. MDM2 overexpression was found in all nine examined cases with and in three tumors without amplification. TP53 mutations were detected in 12 cases (five osteosarcomas, four MFHs, and three leiomyosarcomas), of which none showed amplification, but one had increased levels of MDM2 mRNA. None of the fibrosarcomas, malignant schwannomas, and liposarcomas examined had mutated TP53. The six sarcomas that showed high TP53 mRNA expression in the absence of gene mutation also had elevated levels of MDM2 mRNA. CONCLUSIONS: The present data provide further indications that increased MDM2 expression level, caused by gene amplification or altered regulation of transcription, is involved in tumor progression of some, but not all, sarcoma subtypes.

Animals↗
PubMed1994

Risk of other cancers following Kaposi's sarcoma: relation to acquired immunodeficiency syndrome.

To evaluate the risk of another cancer among persons who initially developed Kaposi's sarcoma, the authors used data from the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute for the years 1973-1990. In persons under 70 years of age, 4,946 cases of Kaposi's sarcoma were observed during the period 1980-1990 (6,217 person-years of follow-up). On the basis of rates seen during the period prior to the epidemic of acquired immunodeficiency syndrome (AIDS), 169 cases were expected. Therefore, cases of Kaposi's sarcoma in this group were assumed to be AIDS-related, while cases occurring in older persons or during the 1970s were assumed to be non-AIDS-related. Rates were compared with the numbers of cases expected overall and by site on the basis of age-, sex-, and calendar year-specific rates from the SEER data. Among the 4,946 persons with AIDS-related Kaposi's sarcoma, the risk of developing non-Hodgkin's lymphoma through 1990 was increased 198-fold (95% confidence interval 169-232). However, the risk of all other cancers was only marginally increased (1.5-fold; 95% confidence interval 0.95-2.3), a risk that was probably biased upward because of ascertainment and misclassification. Among 491 persons with non-AIDS-related Kaposi's sarcoma, the relative risk of all cancers, including non-Hodgkin's lymphoma, was 0.9 (upper 95% confidence limit 1.2), and the risk of non-Hodgkin's lymphoma alone was 0.6 (upper 95% confidence limit 3.3). As of 1990, the risk of having another cancer following Kaposi's sarcoma was increased only in persons infected with human immunodeficiency virus, who were at high risk of non-Hodgkin's lymphoma but probably not of other cancers as a whole.

Acquired Immunodeficiency Syndrome↗
PubMed1997

"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.

Eighteen examples of an unusual malignant soft-tissue neoplasm, the morphology of which ranged from that of "atypical" epithelioid sarcoma to that of a rhabdoid tumor or undifferentiated carcinoma (with transitional forms) are described. Patients included 11 males and seven females; their median age was 35.5 years with most patients aged 20 to 40 years. Development of a mass was the main presenting symptom. Six tumors developed in the pelvis and perineal region, four in the pubic region and vulva, three in the buttocks, one in the deep soft tissues of the left hip, one on the penis, one in left forearm, one in left axilla, and one on the occiput. Tumor size ranged from 1 to 20 cm (median, 4 cm). On microscopic examination, the tumor cells invaded the subcutaneous or deep soft tissues, had prominent epithelioid or rhabdoid features, had marked cytologic atypia, and grew in a multinodular pattern in half of the cases. Areas of necrosis were often seen. A granuloma-like pattern reminiscent of that observed in classic epithelioid sarcoma was observed in only two cases. Immunohistochemically, positivity for cytokeratin, epithelial membrane antigen, and vimentin was seen in all but one of the cases. Of 16 cases, 10 and eight tumors reacted with desmin and CD34, respectively; five of 15 reacted at least focally with smooth-muscle actin, whereas three of 13 and one of 10 reacted for HMB-45 and carcinoembryonic antigen, respectively. S-100 protein and CD31 yielded negative results. Seven tumors were investigated at the ultrastructural level, four of which showed prominent intracytoplasmic intermediate filament aggregates, often accumulating into paranuclear whorls, which is in keeping with the rhabdoid phenotype. Five tumors showed features of epithelial differentiation (i.e., tonofilament-like structures or desmosomes or both), whereas one tumor displayed features of myofibroblastic differentiation. Differential diagnoses include mainly conventional epithelioid sarcoma, extrarenal malignant rhabdoid tumor, epithelioid malignant peripheral nerve sheath tumor, melanoma, rhabdomyosarcoma, and undifferentiated carcinoma. Follow-up information on 14 patients (range, 4 months to 8 years; median, 19 months) revealed local recurrence in one case and metastatic dissemination in six patients, leading to death in five. In our opinion, the above-described neoplasms represent a usually "proximal-type" of epithelioid sarcoma. In contrast to the conventional, "distal-type" epithelioid sarcoma, the proximal variant is characterized by a predominantly large-cell, epithelioid cytomorphology, marked cytologic atypia, frequent occurrence of rhabdoid features, and lack of a granuloma-like pattern in most cases. It appears to be somewhat more aggressive (or at least metastasizes earlier) than usual epithelioid sarcoma.

Adolescent↗
PubMed1998

Poorly differentiated synovial sarcoma: immunohistochemical distinction from primitive neuroectodermal tumors and high-grade malignant peripheral nerve sheath tumors.

Synovial sarcoma is a relatively common sarcoma in adults, which in its classic bimorphic form infrequently poses a diagnostic problem. Monomorphic spindled variants, as well as the less common poorly differentiated variants, may be confused with other soft-tissue sarcomas; the poorly differentiated variant (PDSS), in particular, may be histologically indistinguishable from other small, blue, round cell tumors, including primitive neuroectodermal tumors (PNETs). Detection of the synovial sarcoma-associated t(X;18) by either cytogenetic or molecular genetic approaches may be necessary to confirm the diagnosis of synovial sarcoma in difficult cases. We evaluated 10 cases of PDSS from eight patients using a panel of antibodies (including those to intermediate filament proteins, nerve-sheath associated markers, and neuronal and neuroectodermal associated markers) in order to better establish the immunophenotype of this tumor and to help distinguish it from the tumors with which it may be confused, particularly PNETs and high-grade malignant peripheral nerve sheath tumors (MPNSTs). Our results showed PDSS to have significant immunophenotypic overlap with both PNETs and MPNSTs. In most instances these three entities may be differentiated by a panel of antibodies that should include those to both low and high molecular weight cytokeratins, epithelial membrane antigen, type IV collagen, CD99, CD56, and S-100 protein. Our results also suggest that synovial sarcoma may be a tumor showing combined neuroectodermal and nerve sheath differentiation--perhaps because of translocation-associated expression of specific proteins--rather than a carcinosarcoma of soft tissues or a tumor of specialized arthrogenous mesenchyme.

12E7 Antigen↗
PubMed1980

Is amputation necessary for sarcomas? A seven-year experience with limb salvage.

The rationale for amputation for local tumor control of skeletal and soft tissue sarcomas was based on results obtained from surgical therapy alone. However, our previous results from a pilot trial of multimodality therapy of preoperative chemotherapy and radiation therapy followed by surgical resection indicated that limb salvage (without amputation) could be accomplished in most patients with little morbidity and low recurrence rate. This report summarizes our experience in a prospective trial from January 1972 to December 1979. A total of 105 consecutive patients with soft tissue sarcomas (65 patients) or bone sarcomas (40 patients) were treated with preoperative intraarterial adriamycin, 3500 rads of rapid-fraction radiation and radical en bloc resection of primary tumor. Diseased bones were replaced with cadaver allografts (22 patients), metallic endoprostheses (10 patients) autologous bone (2 patients), or no replacement (ilium or fibula-4 patients). Salvage of a viable, neurologically intact, functional extremity was achieved in 98/105 patients (98%); 97% of limb salvage patients were free of local recurrence after a median follow-up period of 28 months. Major complication rate that required amputation was 3/105 patients (2%). Postoperative adjuvant chemotherapy with cyclical adriamycin and high-dose methotrexate was employed for all patients with osteosarcoma and 35 patients with grade III soft tissue sarcomas. The overall disease-free rate is 50% (18/35) for osteosarcomas and 65% (42/65) for soft tissue sarcomas. These results indicate that local tumor control can be achieved in 91% of patients without amputation. Their functional capabilities are excellent with a low complication rate. Since the advent of adriamycin and methotrexate has significantly improved the overall survival for patients with skeletal and soft tissue sarcomas, the quality of this survival has become even more important. Preoperative multimodality therapy is a major advance in this direction and since results of limb salvage procedures appear to be equal or superior to those achieved by amputation we believe these alternatives should be offered to all patients.

Adolescent↗