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PubMed1989

Response to ifosfamide and mesna: 124 previously treated patients with metastatic or unresectable sarcoma.

European and American investigators have reported response rates of 38% to 83% for ifosfamide alone in pretreated sarcomas. In a phase II trial of ifosfamide 2.0g/m2 days 1 to 4 with mesna uroprotection in 124 patients with previously failed sarcomas, four (3%) responded completely (95% exact confidence interval, 1% to 8%) and 26 (21%) had a complete or partial response (95% exact confidence interval, 14% to 29%). The median time to progression was 5 and 9 months for partial and complete responders, respectively. In the subset of soft tissue sarcomas, the response rate for the patients receiving bolus administration was 26%, compared with 9% for the patients receiving a continuous infusion schedule (P = .03). The response rates among patients with soft tissue and bony sarcomas with a performance score of 0-2 and 0-1 prior to chemotherapy administration were 20% and 40%, respectively. Somnolence or confusion developed in 19%. Neurotoxicity was significantly associated with poor performance status (P less than .01), elevated creatinine (P less than .01), and low bicarbonate levels (P = .05). A serum bicarbonate less than 20 developed in 31% of the patients and was significantly associated with older age (P = .01), elevated creatinine (P = .02), and female sex (P = .06). Hematuria was significantly associated with no uroprotection (the first four patients did not receive mesna because it was unavailable), but was not associated with prior cyclophosphamide, pelvic radiotherapy, age, or bolus v continuation infusion schedule. Thus, ifosfamide is active in failed sarcomas and warrants further study in previously untreated patients with sarcoma.

Adolescent↗
PubMed2003

Buttock soft tissue sarcoma: clinical features, treatment, and prognosis.

BACKGROUND: Primary buttock soft tissue sarcomas in adults are common entities that have been infrequently reported (three clinical series and isolated case reports). We present our experience of buttock sarcomas to better characterize and define the natural history of this condition. METHODS: Buttock tumors occurring in adults (>16 years) between January 1990 and January 2002 were identified from the Royal Marsden Hospital's Sarcoma Unit prospective database. RESULTS: Seventy-three buttock sarcomas were evaluated and treated at the Royal Marsden Hospital during this period. Liposarcoma (n = 19), leiomyosarcoma (n = 13), and synovial sarcoma (n = 9) were the most frequent histological types. There were 8 T1 and 61 T2 tumors, and size was not available in 4 patients. Most tumors (n = 64) were located deep to the deep fascia. There were 15 grade 1, 20 grade 2, and 37 grade 3 tumors, and grade was not available in 1 patient. There were 29 tumors contained within the gluteus maximus. Wide excision was performed in 50 patients. Local recurrence and distant metastasis occurred in 15 and 35 patients with a median time of 18 and 8 months, respectively. The rate of local recurrence at 2 years was 20.9% (SE, 6.8%). The 2-year overall and disease-free survival rates were 64.1% (SE, 6.7%) and 48.5% (SE, 6.4%), respectively. CONCLUSIONS: Buttock sarcomas present special surgical difficulties because of proximity of the sciatic nerve and the ability of tumors at this site to extend into the pelvis and perineum. Size and grade of the tumor were independent predictors for disease-free and overall survival.

Adolescent↗
PubMed2006

Preoperative isolated limb infusion of Doxorubicin and external irradiation for limb-threatening soft tissue sarcomas.

BACKGROUND: At present, limb-sparing surgery is the most appropriate and acceptable treatment option for soft tissue sarcomas of the extremities. To increase the number of limb-sparing resections in the treatment of locally advanced soft tissue sarcomas of the extremities, preoperative radiotherapy and/or chemotherapy are often used. Isolated limb perfusion of cytostatic agents is an effective alternative option but technically complex. Isolated limb infusion, essentially a low-flow isolated limb perfusion without oxygenation via a percutaneous catheter, had been developed as a simple alternative. OBJECTIVE: The objective of this study was to achieve limb-sparing surgery in patients with locally advanced soft tissue sarcomas of the extremities that would otherwise have required an amputation or a functionally mutilating surgery by performing preoperative isolated limb infusion with doxorubicin and external beam irradiation to obtain local control and make limb-sparing surgery feasible. METHODS: A total of 40 patients with locally advanced soft tissue sarcomas of the extremities were evaluated between 2002 and 2005. Tumors were located in the lower limb in 28 patients (70%) and in the upper limb in 12 patients (30%). All of these patients were felt to be unresectable and were referred because amputation was considered the only available treatment option. They underwent preoperative isolated limb infusion with doxorubicin (0.7 and 1.4 mg/kg for the upper and lower limbs, respectively). Preoperative external beam radiotherapy started within 3-7 days after isolated limb infusion was administered. The total dose was 35 Gy in ten fractions. After 3-7 weeks, surgery was performed aiming at limb preservation. RESULTS: Tumor response was seen in 85% of patients, rendering these large sarcomas resectable in most cases. The mean values of pretreatment tumor volume and post-treatment volume were 2797 cm(3) and 1781 cm(3), respectively, with a significant p value of 0.0001. Histologic response was seen in 80% of patients. At a median followup of 15 months (range = 5-35), limb salvage was achieved in 82.5%. Procedure-related complications were limited and easily managed. CONCLUSION: Isolated limb infusion with doxorubicin is a simple and safe method of regional chemotherapy. The addition of preoperative external beam irradiation helped to increase the rate of limb salvage in patients with large and/or high-grade soft tissue sarcomas of the extremities.

Adult↗
PubMed2005

Soft-tissue sarcomas of the chest wall: prognostic factors.

OBJECTIVE: To evaluate factors that are predictive of outcome for patients with chest wall soft-tissue sarcomas. PATIENTS AND METHODS: A retrospective review of 55 surgically treated patients, from March 1964 to October 1996. RESULTS: The median age of the patients was 47.5 years (age range, 15 to 76.3 years), and 56.4% were men. The most common presenting symptom was chest wall mass in 29 patients (52.7%). The median symptom duration was 12 months. Tumor size ranged from 1 to 26 cm (median size, 9.7 cm). The most common histologic type of tumor was fibrosarcoma (52.7%). Twenty-three sarcomas (41.8%) were high-grade, and 32 sarcomas (52.8%) were low-grade. Of the 55 patients, 27 (49.1%) had previously been treated elsewhere (surgical resection, 23 patients; radiation therapy and surgery, 3 patients; chemoradiation therapy, 1 patient). Previously treated patients presented either with residual disease (10 cases) or recurrence of disease (17 cases). All 55 patients underwent surgical resection, 15 patients (27.3%) were treated by neoadjuvant chemoradiation therapy, and 2 patients were treated by adjuvant radiotherapy. Wide surgical resection was performed in 45 patients (81.8%), and marginal resection was performed in 10 patients (18.2%). The median follow-up time was 51.9 months. Local recurrence of disease developed in 6 patients, and metastases developed in 10 patients. The overall survival rates at 5 and 10 years were 87.3% and 79.3%, respectively. Tumor size < 5 cm and low histologic grade were determinants of better survival at univariate analyses. Multivariate analyses disclosed only histologic grade as an independent predictor for the risk of death. Disease-free survival rates at 5 and 10 years were 75.3% and 64.2%, respectively. Tumor size < 5 cm, performance of wide surgical resection, and low histologic grade were determinants of a better disease-free survival rate. Independent prognostic factors for disease-free survival were histologic grade and type of surgical resection. CONCLUSION: The clinical behavior of chest wall soft-tissue sarcomas is similar to that of extremity sarcomas. Thoracic wall soft-tissue sarcomas are best controlled by wide surgical resection.

Adolescent↗
PubMed2005

[Orbital granulocytic sarcoma: case report].

Orbital granulocytic sarcoma is a localized tumor consisting of malignant cells of myeloid origin. This tumor may present in association with acute myelogenous leukemia. Granulocytic sarcoma may be found in a variety of locations throughout the body including the orbit and typically affects children and young adults. There is a slight male predominance in these cases. This is an uncommon case report of a 33-year-old Latin-American woman who was admitted to the Hospital for rapidly progressive orbital proptosis. There was no systemic manifestation of leukemia. The occurrence of orbital granulocytic sarcoma before the development of systemic leukemia in children and young adults is not uncommon and these cases frequently develop hematological evidence within 2 months after initial orbital disease. In this case report, there was no systemic manifestation of leukemia in the last 30 months, even in the presence of orbital tumors. Granulocytic sarcoma is most frequently confused with malignant lymphoma, rhabdomyosarcoma and neuroblastoma. The differential diagnosis of these cases can be challenging, particularly when there is no evidence of systemic leukemia, when imaging features are not sufficiently specific to distinguish granulocytic neoplasms from other tumors. To establish the diagnosis often a biopsy is required. The treatment in such cases (orbital granulocytic sarcoma) is not standardized. Orbital granulocytic sarcoma may be suspected in cases of orbital tumors even in the absence of systemic manifestations of leukemia at any age.

Acute Disease↗
PubMed2006

Sarcoma derived from cultured mesenchymal stem cells.

To study the biodistribution of MSCs, we labeled adult murine C57BL/6 MSCs with firefly luciferase and DsRed2 fluorescent protein using nonviral Sleeping Beauty transposons and coinfused labeled MSCs with bone marrow into irradiated allogeneic recipients. Using in vivo whole-body imaging, luciferase signals were shown to be increased between weeks 3 and 12. Unexpectedly, some mice with the highest luciferase signals died and all surviving mice developed foci of sarcoma in their lungs. Two mice also developed sarcomas in their extremities. Common cytogenetic abnormalities were identified in tumor cells isolated from different animals. Original MSC cultures not labeled with transposons, as well as independently isolated cultured MSCs, were found to be cytogenetically abnormal. Moreover, primary MSCs derived from the bone marrow of both BALB/c and C57BL/6 mice showed cytogenetic aberrations after several passages in vitro, showing that transformation was not a strain-specific nor rare event. Clonal evolution was observed in vivo, suggesting that the critical transformation event(s) occurred before infusion. Mapping of the transposition insertion sites did not identify an obvious transposon-related genetic abnormality, and p53 was not overexpressed. Infusion of MSC-derived sarcoma cells resulted in malignant lesions in secondary recipients. This new sarcoma cell line, S1, is unique in having a cytogenetic profile similar to human sarcoma and contains bioluminescent and fluorescent genes, making it useful for investigations of cellular biodistribution and tumor response to therapy in vivo. More importantly, our study indicates that sarcoma can evolve from MSC cultures.

Animals↗
PubMed1997

Polyethylene glycol-liposomal doxorubicin. A review of its pharmacodynamic and pharmacokinetic properties, and therapeutic efficacy in the management of AIDS-related Kaposi's sarcoma.

Doxorubicin is an antineoplastic drug which has in vitro and in vivo activity against a number of malignancies including Kaposi's sarcoma. Incorporation of doxorubicin into polyethylene glycol-coated (pegylated) liposomes alters the pharmacokinetics of the drug. Liposomal doxorubicin has a smaller volume of distribution and slower plasma clearance than standard free doxorubicin. The liposomal formulation achieves higher concentrations in the highly vascularised lesions of Kaposi's sarcoma than in normal tissue. Liposomal doxorubicin monotherapy in patients with AIDS-related Kaposi's sarcoma produced overall response rates (complete plus partial) of 43 and 59% in large comparative studies and 67 to 100% in noncomparative studies which included > or = 20 patients. In comparative studies, liposomal doxorubicin was significantly more effective than the combination of standard doxorubicin, bleomycin and vincristine (overall response rates of 43 and 25%, respectively) and bleomycin and vincristine (BV) [overall response rates of 59 and 23%, respectively]. In addition, overall response rates to the liposomal drug were higher in both treatment arms of 2 smaller comparative studies which compared liposomal doxorubicin with BV, but significant between-treatment differences were not detected. Patient numbers in these 2 studies, however, may have been too small to detect significant differences. Liposomal doxorubicin is generally well tolerated. Myelosuppression is the most common dose-limiting adverse effect in patients with AIDS and Kaposi's sarcoma. Neutropenia occurs most often; anaemia and thrombocytopenia occur less frequently, as do nausea and vomiting and stomatitis. Palmar-plantar erythrodysaesthesia occurs in some patients, most commonly after 6 to 8 weeks of chemotherapy. Although symptoms may occasionally be severe, the syndrome usually does not require dosage reduction or treatment delay. Limited data suggest that the incidence of cardiotoxicity may be lower after liposomal doxorubicin than after equivalent doses of standard doxorubicin. Overall, liposomal doxorubicin appears to be one of the most active single agents available for treating patients with AIDS-related Kaposi's sarcoma. The therapeutic potential of liposomal doxorubicin administered in combination with other active agents to patients with Kaposi's sarcoma is, as yet, unknown. However, administered alone, the drug seems to be more effective than the best available combination chemotherapy regimens.

Acquired Immunodeficiency Syndrome↗
PubMed2007

High Prevalence of Torque teno (TT) virus in classical Kaposi's sarcoma.

Human herpes virus 8 infection is the primary and necessary factor in the development of Kaposi's sarcoma, but is not sufficient per se to trigger the onset of the disease. In order to search for virological cofactors associated with the occurrence of the disease, we investigated the prevalence of active infection by two newly discovered viruses, hepatitis G virus and TT virus, among patients with classical Kaposi's sarcoma. Serum of 24 patients with Mediterranean Kaposi's sarcoma was investigated using polymerase chain reaction and compared with that of 68 healthy subjects. Cutaneous samples from patients with Kaposi's sarcoma and healthy subjects were investigated for TT virus DNA. No patient had serum markers for hepatitis G virus. TT virus DNA was present in the serum of 21/24 (87.5%) patients and 32/68 (47%) controls (p=0.002). TT virus DNA was present in the lesional skin of 5/18 patients with Kaposi's sarcoma (27.7%), but not in the skin of controls. TT virus might play a role as a cofactor in the clinical emergence of Kaposi's sarcoma in patients infected with Human herpes virus 8, perhaps by immunosuppressive effects or by a common transmission pathway for these two viruses.

Adult↗
PubMed2002

World Wide Web-based survey of vaccination practices, postvaccinal reactions, and vaccine site-associated sarcomas in cats.

OBJECTIVE: To quantify incidence of vaccination practices, postvaccinal reactions, and vaccine site-associated sarcomas in cats. DESIGN: Epidemiologic survey. Animals-31,671 cats vaccinated in the United States and Canada by veterinarians with World Wide Web access. PROCEDURE: Veterinarians used secure Web-based survey forms to report data regarding administered vaccines, postvaccinal inflammatory reactions, vaccine site-associated sarcomas, and detailed information and history on each sarcoma. Data were collected from Jan 1, 1998 to Dec 31, 2000, allowing a 1- to 3-year follow-up of vaccinated cats. RESULTS: Participants reported administering 61,747 doses of vaccine to 31,671 cats; postvaccinal inflammatory reactions developed in 73 cats (11.8 reactions/10,000 vaccine doses), and qualifying vaccine site-associated sarcomas developed in 2 cats (0.63 sarcomas/10,000 cats; 0.32 sarcomas/10,000 doses of all vaccines). CONCLUSIONS AND CLINICAL RELEVANCE: These findings indicate that the incidence of vaccine site-associated sarcomas is low and is not increasing. Thoughtful consideration of the relative risks and benefits of specific vaccines remains the best means of reducing the incidence of sarcomas. It is not necessary to remove postvaccinal granulomas unless malignant behavior is apparent or they persist > 4 months.

Animals↗
PubMedDate not supplied

Variability of differentiation patterns in xenotransplanted spindle cell sarcomas: a histomorphological, immunohistochemical, and ultrastructural study.

Three leiomyosarcomas, 3 nerve sheath sarcomas, 1 rhabdomyosarcoma, and 1 sarcoma not otherwise classifiable with 17 of their xenografts, grown on nude mice, were analyzed to assess the degree of concordance between histomorphology, immunohistochemistry, and ultrastructure in spindle cell sarcoma xenograft differentiation. Histomorphology was inconclusive or misleading in 4/8 sarcoma strains and immunohistochemistry in 4/8 originals and in 10/17 xenografts, although specific patterns had been identified ultrastructurally. Electron microscopy was superior to immunohistochemistry and histomorphology in spindle cell sarcoma differential diagnosis. A further purpose of this study was to clarify whether spindle cell sarcoma xenografts retain the morphological characteristics of their primaries. Histomorphological features of the primaries were preserved over all passages, whereas the immunohistochemical marker profiles as well as the ultrastructural phenotypes changed in 14/17 xenografts and in 8/17 xenografts, respectively. Moreover, unusual bidirectional or tridirectional patterns of differentiation were identified ultrastructurally with leiomyomatous as well as Schwann cells occurring side by side and with MFH-like areas in 5/17 xenotransplants. These findings suggest genetic instability of tumor cells and may be important in the consideration of mesenchymal differentiation pathways.

Animals↗
PubMed1996

Soft tissue sarcomas.

This synthesis of the literature on radiotherapy for sarcomas originating in the body's soft, supportive tissues, ie, muscle, connective tissue, and fatty tissue is based on 71 scientific articles, including 4 randomized studies, 5 prospective studies, and 26 retrospective studies. These studies involve 3,444 patients. Over 90% of patients with soft tissue sarcomas in the arms and legs can be treated in a way that preserves the extremities. Subcutaneous and intramuscular sarcomas can be treated surgically with little functional loss or risk for local recurrence without adjuvant radiotherapy. To avoid amputation, surgery is often combined with radiotherapy for treatment of local relapse. Adequate surgical margins are usually difficult to achieve for head/neck tumors and retroperitoneal tumors, and therefore surgery is often combined with radiotherapy to reduce the risk for local relapse. Pre- and postoperative radiotherapy are similar. A disadvantage of preoperative radiotherapy is that it reduces the opportunity for exact diagnosis and determining morphobiologic sarcoma parameters. To further improve treatment results for advanced sarcomas, it is necessary to introduce other fractionation schedules, mainly hyperfractionation. This places greater demands on radiotherapy, mainly for staff resources. Combining radiotherapy and local intraarterial chemotherapy involves greater risks for complications and has not shown better treatment results than pre- or postoperative radiotherapy alone, and it is not recommended as standard treatment for soft tissue sarcomas. Intraoperative treatment methods should be targeted for further study and development.

Combined Modality Therapy↗
PubMed1990

Conditioned medium from mouse sarcoma 180 cells contains vascular endothelial growth factor.

Medium conditioned by mouse sarcoma 180 cells stimulates the growth of capillary endothelial cells. The growth factor produced by mouse sarcoma 180 cells is heparin-binding, dithiothreitol-sensitive, endothelial cell specific, and secreted into the medium. The characteristics of this mouse sarcoma-derived growth factor are very similar to those of vascular endothelial growth factor (VEGF) first described by Ferrara and Henzel (1989). The N-terminal amino acid sequences of the two growth factors are similar. Since the amino acid sequence of vascular permeability factor (VPF) is essentially identical to that of VEGF, a Western blot of mouse sarcoma 180-derived endothelial growth factor was probed with a polyclonal antibody raised against human VPF. This antibody reacted with several proteins of approximately 23 kDa, suggesting the presence of multiple forms of a VEGF-like protein. A full length cDNA probe for bovine VEGF reacted strongly with RNA isolated from mouse sarcoma 180 cells. We conclude that an endothelial growth factor found in conditioned medium from mouse sarcoma 180 cells is VEGF.

Amino Acid Sequence↗
PubMedDate not supplied

Angiogenesis does not correlate with prognosis or expression of vascular endothelial growth factor in synovial sarcomas.

To investigate the significance of tumour angiogenesis in synovial sarcoma on prognosis, we analysed the correlation between microvessel density (MVD) and various clinicopathological factors or immunohistochemical expression of vascular endothelial growth factor (VEGF) in 54 primary and recurrent synovial sarcomas. MVD in synovial sarcomas did not correlate with prognosis or VEGF expression. Furthermore, VEGF expression in synovial sarcomas did not have any prognostic value on overall survival. Our results indicate that angiogenesis does not play an important role in metastasis of synovial sarcoma and overall survival. Angiogenesis in synovial sarcoma may be controlled by angiogenesis activators other than VEGF.

Adolescent↗
PubMed2006

Expression of EWS-ETS fusions in NIH3T3 cells reveals significant differences to Ewing's sarcoma.

Ewing's sarcomas contain specific chromosomal translocations that fuse EWS to ETS family members, including FLI, ERG, FEV, ETV1 and ETV4. Prior work has suggested that functional differences exist between some of these EWS-ETS fusions. However, as the cell of origin of Ewing's sarcoma is unknown, this prior work was conducted in NIH3T3 cells, which have not been validated as an appropriate model for the study of EWS-ETS fusions. To determine if NIH3T3 cells are a good model for Ewing's sarcoma, we introduced all five EWS-ETS fusions into these cells, and analyzed their phenotypes and gene expression patterns. EWS-FLI, EWS-ERG, and EWS-FEV caused NIH3T3 cells to exhibit anchorage independent growth whereas EWS-ETV1 and EWS-ETV4 did not. In contrast, all the EWS-ETS fusions induced tumor formation in a xenograft model. We defined the core transcriptional profile of the EWS-ETS fusions using cDNA microarrays, and compared these to data derived from patient-derived Ewing's sarcoma cell lines. The NIH3T3 model did not recapitulate the gene expression pattern of bona fide Ewing's sarcoma. Based on these results, we conclude that while there may be functional differences between the various EWS-ETS fusions, the NIH3T3 cell model is inadequate to study the gene expression pattern induced by EWS-ETS proteins in Ewing's sarcoma. Thus, data derived from the NIH3T3 model system needs to be appropriately validated before they can be accepted as relevant to the human disease.

Animals↗
PubMed2000

Sarcomas of the head and neck at Kenyatta National Hospital.

OBJECTIVE: To determine the pattern of occurrence of sarcomas afflicting the neck and craniofacial region. DESIGN: A retrospective study (1982-1991). SETTING: Cancer Registry, Kenyatta National Hospital, Nairobi, Kenya. METHOD: Examination was performed of the cancer records in the registry over the period 1982 to 1991 inclusive. Histopathological types of the sarcomas were then analysed to indicate the numbers that occurred per year; and also the pattern of occurrence according to age groups, gender and site. RESULTS: Of the 10,897 whole body neoplasms, 985 were sarcomas. Among these sarcomas, 160 (16%) were located in the neck and craniofacial region. The histopathological types of the neoplasms included Kaposi's sarcoma (39%), osteosarcoma (23%), rhabdomyosarcoma (21%), fibrosarcoma (13%), chondrosarcoma (two per cent), malignant fibrous histiocytoma (one per cent) and dermatofibrosarcoma protuberans (one per cent). CONCLUSION: The results of this investigation, in concordance with those of other series worldwide, indicate that the various sarcomas afflicting the neck and craniofacial area are generally rare.

Adolescent↗
PubMed2002

Expression of E-cadherin and p53 proteins in human soft tissue sarcomas.

OBJECTIVE: To investigate the expression of E-cadherin in human soft tissue sarcomas and its potential relationship to p53 alterations. DESIGN: Tissue sections of 91 soft tissue sarcomas were analyzed by immunohistochemistry for E-cadherin and p53 proteins. Sixty-one tumors were investigated further by the application of the polymerase chain reaction technique and a direct sequence analysis procedure of exons 5 through 8 in the p53 gene. SETTING: Tertiary-care teaching hospital. PATIENTS: Ninety-one patients with soft tissue tumor were treated surgically. Thirteen of these patients had tumors with epithelial differentiation. RESULTS: E-Cadherin was expressed at the cell-cell boundaries in 11 samples (12%): 9/13 (69%) with and 2/78 (3%) without histologic evidence of epithelial elements. Other sarcomas were completely negative for E-cadherin. Overexpression of p53 was detected in 30 cases (33%), 7 of which also demonstrated mutations in the p53 gene. The frequencies of p53 abnormalities in tumors with and without epithelial components were 8% and 37%, respectively. No association was established between E-cadherin immunoreactivity and p53 abnormalities (P =.13). Tumor grade strongly correlated with p53 alterations (P =.01), but not with E-cadherin expression (P =.07). CONCLUSIONS: These data support the involvement of p53 alterations in the pathogenesis of soft tissue sarcomas. The lack of E-cadherin expression in these tumors, with the exception of lesions showing epithelial differentiation, indicates that E-cadherin is not an important factor involved in cell-cell adhesion in sarcomas. It is, however, suggested that E-cadherin may play a role in the development and/or maintenance of epithelial architecture in sarcomas, regardless of p53 status.

Cadherins↗
PubMed2003

Epithelioid sarcoma: new insights based on an extended immunohistochemical analysis.

CONTEXT: Epithelioid sarcoma has a distinctive epithelioid phenotype and characteristically exhibits immunohistochemical reactivity for epithelial markers (keratins and epithelial membrane antigen) and mesenchymal markers (most notably vimentin and CD34). Antibodies to certain keratin subunits and other novel antigens now available to surgical pathologists have not been tested on a large number of cases. OBJECTIVE: To assist in the differential diagnosis of epithelioid sarcoma and to help elucidate its histogenesis through an expanded immunohistochemical profile. DESIGN: Immunohistochemical testing with diverse antibodies was performed on 95 archived epithelioid sarcomas including 73 classic and 22 histologically variant subtypes retrieved from the files of the Armed Forces Institute of Pathology. RESULTS: Immunohistochemical reactivity (number positive/number of cases tested [percent positive], frequency of staining) included keratin 14 (31/64 [48%], variable), gamma-catenin (35/74 [47%], variable), keratin 5/6 (10/33 [30%], focal), calretinin (8/40 [20%], focal), keratin 20 (11/71 [15%], focal), p63 (3/20 [15%], focal), whereas 9 invasive cutaneous squamous cell carcinomas showed strong p63 positivity, epithelial-specific antigen (10/74 [14%], variable), CD117/Kit (5/37 [14%], focal), keratin 15 (3/23 [13%], rare cell), mesothelin (2/64 [3%], rare cell), and CD10 (1/41 [2%], rare cell). No reactivity was observed for keratins 2, 5, and 10. CONCLUSIONS: Diagnostically, p63 and keratin 5/6 distinguish cutaneous squamous cell carcinoma (positive) from epithelioid sarcoma (usually negative). No single immunomarker was able to distinguish the main 4 histologic subtypes of epithelioid sarcoma, indicating that they are all histogenetically related lesions. The limited expression of specific keratin subtypes used in our study supports the notion that epithelioid sarcoma is a mesenchymal neoplasm capable of partial epithelial transformation.

Adolescent↗
PubMed2003

SYT-SSX2 variant of primary pulmonary synovial sarcoma with focal expression of CD117 (c-Kit) protein and a poor clinical outcome.

Pulmonary synovial sarcoma is a rare neoplasm recently recognized as a distinct entity and characterized by t(X;18) translocation and production of at least 2 fusion genes, SYT-SSX1 and SYT-SSX2. We report a case of primary pulmonary synovial sarcoma with the SYT-SSX2 phenotype and a rapidly progressive downhill course. Previous reports have suggested that the soft tissue synovial sarcomas with SYT-SSX2 phenotype have a favorable clinical outcome. To the best of our knowledge, this is also the first report of CD117 (c-Kit) expression in a pulmonary synovial sarcoma. A 45-year-old woman presented with left chest pain and was found to have a left lower lobe tumor that was originally diagnosed as a sarcomatoid carcinoma. After the patient underwent chemotherapy and brachytherapy, the specimen from a left pneumonectomy showed a large spindle cell tumor, which was reclassified as a synovial sarcoma based on the results of immunophenotyping and molecular genetic studies. Differentiation between sarcoma and carcinosarcoma is crucial for implementing appropriate therapy. Furthermore, if the tumor expresses c-Kit, it may respond to target-based therapy.

Biomarkers, Tumor↗