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PubMed1998

[Primary cardiac synovial sarcoma--a case report].

We report a case of cardiac synovial sarcoma, a very rare primary tumor of the heart. The patient was a 29-year-old man with chief complaint of dyspnea. On echocardiography, a tumor 42 mm in diameter occupying the most space of the left atrium was found. The tumor was obstructed the opening of the mitral valve. He was diagnosed with heart failure due to disturbance of the pulmonary venous return and hemodynamic mitral valve stenosis, and underwent surgery. The tumor was a 5-cm sphere originating in the left atrial posterior wall, and was covered with a sheath and solid. The atrial posterior wall was covered with a peel that appeared to be tumor tissue, and this tissue expanded toward the openings of the pulmonary veins bilaterally and the posterior cusp of the mitral valve. The tumor was resected together with the posterior wall. On pathologic examination, the tumor was diagnosed as a synovial sarcoma. Since no other primary focus was found, this tumor was judged to be a primary sarcoma of the heart. The patient was discharged with elimination of symptoms. The sarcoma recurred after 4 months, and the patient underwent a second operation. The recurrent tumor began from the site of resection of the previous operation and expanded up to the inferior pulmonary veins bilaterally. A wide area of the left atrial wall including the tumor was resected, and he was discharged again. The sarcoma recurred again, and he died of progression of heart failure and aggravation of general conditions after 8 months from the first operation.

Adult↗

PubMed1997

[Kaposi sarcoma in cyclosporin A therapy of actinic reticuloid].

A 76 year old patient with actinic reticuloid was treated with Cyclosporin A. 11 months after beginning the immunosuppressive therapy, he developed lymphoedema and livid nodular skin lesions of the right leg, which histologically showed Kaposi's sarcoma. After stopping the Cyclosporin A and intralesional therapy with Interferon alpha, no regression of the Kaposi sarcoma could be seen. The characteristics of Kaposi sarcoma arising during immunosuppressive therapy and the differences in incidence, risk groups and distribution pattern compared to the classic sporadic and the AIDS-related Kaposi sarcoma will be described. The development of Kaposi sarcoma during immunosuppressive therapy for actinic reticuloid has not been previously described.

Aged↗

PubMed2005

Imaging characteristics of primary cranial Ewing sarcoma.

BACKGROUND: Ewing sarcoma accounts for 10-15% of all childhood malignant bone tumours and is second in prevalence to osteosarcoma. The skull bones are an unusual site of origin of primary Ewing sarcoma in children. Previous reports concentrate on the neurosurgical aspects and relatively good outcome compared to other bone tumours of the calvarium. Reported cases mainly describe the imaging characteristics on CT. OBJECTIVE: To describe the MRI and CT features of primary cranial Ewing sarcoma. MATERIALS AND METHODS: The neuroimaging of three cases of primary cranial Ewing sarcoma are reviewed. RESULTS: Our three cases show an extra-axial mass that is high attenuation on CT and low signal on T2-weighted MRI. Haemorrhagic components, dural extension and contrast enhancement are also characteristic features. CONCLUSION: CT attenuation and magnetic resonance signal characteristics reflect sheets of densely packed cells seen in Ewing sarcoma.

Child↗

PubMed2003

Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation.

OBJECTIVE: To characterize the radiological and clinicopathologic features of cystic synovial sarcoma. DESIGN AND PATIENTS: Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed. RESULTS: CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma. CONCLUSION: All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging.

Adolescent↗

PubMed1997

The abundant presence of Söderström bodies in cytology smears of fine-needle aspirates contributes to distinguishing high-grade non-Hodgkin's lymphoma from carcinoma and sarcoma.

Söderström bodies, also termed lymphoglandular bodies (LGB) and detectable in fine-needle aspiration cytology smears, have long been accepted as indicative of lymphoid tissues. To investigate the validity of this association, we examined 588 cytologic smears from high-grade non-Hodgkin's lymphoma (NHL), carcinoma, and sarcoma. Slides with lymphocytes in the vicinity of carcinoma and sarcoma cells had been excluded. Two independent observers scored smears to number, size, color, form, and smear background of the LGB. In 68 of 359 (19%) nonlymphoid malignancies rare (defined as < 1 LGB per high-power field) or occasional LGB (defined as 1-20 LGB per high-power field) were detectable. Half of these tumors consisted of melanomas, small cell lung carcinomas, and teratomas; the other half encompassed undifferentiated sarcomas. However, none of the smears obtained from carcinoma or sarcoma tissue had abundant LGB (defined as > 20 LGB per high-power field). When number of LGB was estimated to be abundant, the sensitivity for diagnosing a lymphoma was 54%; however, specificity was 100%. The difference in showing LGB between high-grade NHL and carcinoma/sarcoma was highly significant (p = 0.0001). The presence of abundant LGB in cytologic smears strongly suggests the diagnosis of lymphoma, while the absence of LGB nearly excludes this diagnosis. No trends were observed with the other criteria which were tested. LGB in aspiration cytology smears from malignant tumors thus represent a useful tool to distinguish high-grade NHL from carcinoma and sarcoma.

Biopsy, Needle↗

PubMed1994

[The histological features of local recurrences of soft tissue sarcomas].

Local recurrence is a well-recognised problem in soft tissue sarcomas, especially at difficult anatomical locations or after inadequate surgery. It is logical to assume that such recurrence will have an adverse effect on prognosis. In most cases the histological appearance of the recurrence is very similar to that of the primary neoplasm, in view of the fact that recurrence is most common in high-grade (poorly differentiated) sarcomas. However, in some cases, the appearance may change in one of three ways: (1) Gradual progression in grade. This phenomenon is seen most often in leiomyosarcoma or myxofibrosarcoma, in which the tumour shows progressive loss of differentiation and there is increasing risk of metastasis; (2) Abrupt change to a higher grade. This phenomenon, known as dedifferentiation, is commonest (among soft tissue lesions) in liposarcoma and is characterised usually by the emergence of an "MFH-like" pattern and an increased risk of metastasis; (3) Acquisition of differentiation. Some round cell sarcomas in children become better differentiated after chemotherapy, and some dedifferentiated adult sarcomas may revert to their well-differentiated form; occasionally the appearance of greater differentiation reflects sampling error in the primary tumour. Pathologists should be aware that significant morphological changes may occur in recurrent soft tissue sarcomas and, in analysing and diagnosing such lesions, they should always review material from the primary tumour.

Cell Differentiation↗

PubMed1998

[Synovial sarcoma of the pericardium].

A precordial tumor of the pericardium was radiologically diagnosed as the cause of an untypical clinical picture of heart disease in a 41-year-old soldier. As the patient had an increased asbestos exposure due to his profession, he was admitted to operation under the tentative diagnosis of a pericardial mesothelioma and the question of an occupational disease (BK 4105). Microscopic and immunohistochemical findings are compatible with the diagnosis of a synovial sarcoma of the pericardium. The present immunohistochemical marker spectrum allowed a reliable differentiation between synovial sarcoma and pericardial mesothelioma, which is more frequent than synovial sarcoma. The epithelioid component was determined using the following antibodies: MNF 116, CK 19, CK 7, EMA and Ber EP-4 were positive while Factor VIII, Calretinin, S100, Vimentin, CEA, CD 31, bcl-2 and HBA-71 were negative. The sarcomatous component was determined with antibodies to Vimentin, bcl-2 and HBA-71 which were positive, and to MNF 116, CK 19, CK 7, Factor VIII, Calretinin, S100, EMA, CEA, Ber EP-4 and CD 31 which were negative. Synovial sarcomas of the pericardium in the lower anterior mediastinum or the myocardium are exceedingly rare. A causal relationship between tumor formation and an increased asbestos exposure--similar to the epidemiologically based experiences with pericardial mesothelioma--is not likely. Primary extrapericardial synovial sarcoma could be excluded.

Adult↗

PubMed1999

Penile epithelioid sarcoma: MR imaging findings.

Magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie's disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis.

Adult↗

PubMed2002

Isolated meningeal chloroma (granulocytic sarcoma) in a child with acute lymphoblastic leukemia mimicking a falx meningioma.

BACKGROUND: Isolated chloromas (granulocytic sarcomas) are rare tumors. Chloromas are masses composed of immature granulocytic cells. Granulocytic sarcoma occurs primarily in patients with acute myelogenous leukemia, but can also arise in patients with other myeloproliferative disorders, though rarely in patients with acute lymphoblastic leukemia (ALL). When dural-based, granulocytic sarcoma may be indistinguishable from meningioma radiologically. CASE HISTORY: We now describe one patient affected by ALL with isolated granulocytic sarcoma mimicking meningioma as initial CNS relapses. A 12-year-old girl who had been diagnosed with ALL and undergone chemotherapy presented with generalized tonic-clonic seizure while in complete remission. Computed tomographic scan and magnetic resonance imaging showed a small mass mimicking a meningioma at the anterior falx. The patient was developed speech disturbance 6 days later. Follow-up magnetic resonance imaging demonstrated a rapidly growing mass with intralesional hemorrhage. Bone marrow biopsy and cerebrospinal fluid study were negative for leukemia. The patient underwent open surgery. The pathological diagnosis was acute lymphoblastic leukemia. CONCLUSIONS: These unusual clinical manifestations and radiological findings in acute lymphoblastic leukemia should be regarded as a recurrence of leukemia. Early detection and antileukemic treatment of granulocytic sarcoma are necessary and important for a favorable prognosis.

Child↗

PubMed2003

A case of primary Ewing's sarcoma of the occipital bone presenting with obstructive hydrocephalus.

BACKGROUND: Ewing's sarcomas account for approximately 10% of primary malignant bone tumors. While most of the primary Ewing's sarcomas occur in the long bones, pelvis, or ribs, approximately 1-6% of these sarcomas arise in the skull. We found approximately 50 cases of Ewing's sarcoma of the calvarium reported in our search of the literature. Of these, 36 cases were reported in detail. CASE REPORT AND DISCUSSION: In this article we describe a rare case of primary Ewing's sarcoma of the occipital bone presenting with acute obstructive hydrocephalus and review the literature.

Combined Modality Therapy↗

PubMed2003

Synovial sarcoma--unusual presentation with cerebral hemorrhage.

INTRODUCTION: Soft-tissue sarcomas account for 1% of all malignancies in adults. Typical symptoms comprise a growing and painless mass. Occasionally, the diagnosis is facilitated by secondaries. MATERIALS AND METHODS: Here we report the first case presented with intracranial hemorrhage caused by a metastasis of a previously unknown synovial sarcoma. The 74-year-old female patient was under anticoagulation for recurrent thrombosis of the right popliteal vein due to compression by the sarcoma, misdiagnosed as Baker's cyst. The brain metastasis was resected, and after an incisional biopsy of the suspected tumor, which confirmed the diagnosis of a synovial sarcoma, an above knee amputation was performed because of the invasion of the neurovascular structures in the popliteal fossa. RESULTS: The patient died 11 months after the initial event from pulmonary and further brain metastases. CONCLUSION: Brain metastases are seldom the primary factor in the management of advanced synovial sarcoma. Initially, the primary tumor was thought to be a benign cyst; we therefore recommend a biopsy/excision of lesions greater than 5 cm in size and with growth tendency to allow appropriate treatment.

Aged↗

PubMed2000

Expression of lung resistance protein in epithelioid sarcoma in vitro and in vivo.

The incidence of epithelioid sarcoma among patients with malignant soft tissue tumors is small, but the rates of recurrence and metastasis of this type of sarcoma are high. To date, effective chemotherapy for advanced epithelioid sarcoma has not been established and, furthermore, epithelioid sarcoma is known to exhibit multidrug resistance (MDR). The chemosensitivities to anticancer agents of two cell lines established from epithelioid sarcoma were examined in this study. The results showed that the ES-OMC-MN and SFT-8606 cell lines were resistant to vincristine (IC50 1190 nM and 872 nM, respectively) and Adriamycin (IC50 921 nM and 650 nM, respectively), but sensitive to actinomycin D (IC50 < 10 nM). P-glycoprotein (p-Gp) and MDR-associated protein (MRP) were not expressed in these cell lines, but a high expression level of lung resistance protein (LRP) was observed. The original tumor tissues from which the two cell lines were established were also found to be LRP-positive but not to express p-Gp or MRP. Their chemosensitivities to Adriamycin were not significantly altered in the presence of 2.5 microg/ml anti-LRP antibody (LRP-56), but the IC50 of vincristine was much less (IC50 128 nM and 27 nM, respectively) than that for an untreated cell line. It is thus suggested that the vincristine resistance in the two cell lines is LRP-mediated. Since cyclosporin A, known to be a modifier of p-Gp, also induced reversal of vincristine resistance in the ES-OMC-MN and SFT-8606 cell lines (IC50 6.2 nM and 17 nM, respectively), it is suggested that cyclosporin A acts as a modifier of MDR mediated by LRP.

ATP Binding Cassette Transporter, Subfamily B↗

PubMed2006

Follicular dendritic cell sarcoma of the tonsil: case report.

Follicular dendritic cell sarcomas are unusual, and extranodal origin is extremely rare. The English literature contains only eight cases in which this sarcoma has been presented as a tonsillar mass. We report a new case of follicular dendritic cell sarcoma of the tonsil in a 76 year old woman. The patient underwent diagnostic tonsillectomy for a left tonsillar mass, and follicular dendritic cell sarcoma was diagnosed based on histopathological and immunoperoxidase findings. Postoperative radiotherapy was performed. The patient is alive and disease-free at 4 years of follow-up. Without a high index of suspicion, this entity can easily be missed. We believe that follicular dendritic cell sarcoma should be included in the differential diagnosis for any tonsillar mass.

Aged↗

PubMed2002

A gastric carcinosarcoma with neuroendocrine cell differentiation and undifferentiated spindle-shaped sarcoma component possibly progressing from the conventional tubular adenocarcinoma; an immunohistochemical and ultrastructural study.

A 56-year-old Japanese man underwent total gastrectomy with lymph-node dissection for a gastric tumor. In the oral rim of the excised large ulcerated tumor, a small nodular mass demonstrated a unique histology of a carcinosarcoma composed of more than three distinctive components. A major part of the large ulcerated tumor tissue consisted of conventional tubular adenocarcinoma, with the coincidental focal distribution of solid cell nests of poorly differentiated or neuroendocrine cell (small cell) carcinoma. Sarcoma cells were another of the constituents of the small nodular tumor with atypical spindle-shaped cells. All the excised lymph-node metastasis demonstrated the histology of tubular adenocarcinoma. In the sarcoma cells, many of the lineage-specific immunohistochemical markers were negative. Only vimentin was positive. Electron microscopy revealed that the neuroendocrine cell carcinoma contained cells with abundant small aggregations of neurosecretory-type granules in their cellular cytoplasm. Primitive cellular junctions and incomplete basal lamina were seen in the sarcoma cells. No transitional or hybrid-type cells were seen between carcinoma and sarcoma cells. The interfaces between the cancer cell nests and the proliferation of sarcoma cells are clearly discerned. It was speculated that the unique histology of carcinosarcoma might have progressed from the conventional type of pre-existing advanced tubular adenocarcinoma.

Adenocarcinoma↗

PubMed2005

Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts.

Clear cell sarcoma is a high-grade sarcoma with morphological features resembling those of malignant melanoma. An osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts is very rare. Herein, we report an unusual stomach tumor with microscopic and immunohistochemical characteristics of an osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts. The tumor cells were predominantly oval, admixed with some round and spindle elements arranged in nests and fascicles, and admixed with scattered osteoclast-like multinucleated giant cells. Neoplastic cells were positive for S-100 protein, and osteoclast-like multinucleated giant cells were immunoreactive to CD68. The unusual morphology of the tumor caused significant diagnostic difficulties. The differential diagnosis included gastrointestinal stromal tumor, primary or metastatic melanoma, and epithelioid malignant peripheral nerve sheath tumor. To the best of our knowledge, this is possibly the second description of an osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts.

Adult↗

PubMed2006

Adamantinoma-like Ewing's sarcoma with EWS-FLI1 fusion gene: a case report.

Recent studies have advocated the genotypic and phenotypic delineation of a novel Ewing's sarcoma histologic variant showing epithelial features defined as "adamantinoma-like Ewing's sarcoma". We described an 18-year-old girl with a primary small round-cell sarcoma of the right tibia showing polyphenotypic differentiation with epithelioid features. The neoplastic cells had mainly round or oval nuclei with fine chromatin with a portion of epithelial arrangements. The immunohistochemical analysis showed the epithelial markers of cytokeratin 5/6/18, AE1/AE3, and cytokeratin high molecular weight were stained especially in the foci with epithelioid features, as well as MIC2, S100, and NSE. The diagnosis of the lesion was confirmed as Ewing's sarcoma by the presence of the EWS-FLI1 fusion transcript, and could be defined as the so-called "adamantinoma-like Ewing's sarcoma". After wide excision and high-dose chemotherapy with peripheral blood stem cell transfusion, the patient has been well and continuously event-free for 3 years since the initial diagnosis.

Adamantinoma↗

PubMed1999

Prognostic significance of p53 gene mutations and p53 protein expression in synovial sarcomas.

Alterations to p53 seem to be of prognostic significance in soft tissue sarcomas, but their significance for synovial sarcomas has not been studied. We analysed 34 synovial sarcomas in 19 patients for p53 alterations (p53 gene mutations + p53 immunopositivity) and examined this factor for its prognostic value in a group of 15 primary tumours. DNA was prepared from paraffin-embedded tumour material by a modified proteinase K/phenol/chloroform extraction. p53 gene mutations of exons 5-8 were analysed by the PCR-SSCP-sequencing method. p53 protein expression was evaluated by immunohistochemistry using the murine monoclonal antibody DO1. We found two missense mutations (5.9%) and ten p53 immunopositive cases (29.4%). Both tumours with p53 mutations showed p53 protein expression. There was no significant correlation between p53 alteration and histological subtype, age, sex, or tumour size. The 5-year survival rate was 24.1%. Overall survival was significantly reduced in patients having synovial sarcomas with p53 alterations (P<0.001). In the multivariate Cox's analysis, only p53 alterations (P=0.032) and tumour size (P=0.023) emerged as independent prognostic factors. We suggest that p53 alterations may be a useful prognostic indicator in synovial sarcomas, allowing rational clinical treatment and follow-up.

Adolescent↗

PubMed1999

Kaposi's sarcoma following malignant mesothelioma.

We report the unusual occurrence of Kaposi's sarcoma following asbestos-related malignant mesothelioma, in a human deficiency virus (HIV)-negative Italian man. Seropositivity to human herpes virus 8 (HHV8) was documented at the time of mesothelioma diagnosis and preceded the onset of Kaposi' sarcoma with a time lapse of 13 months. HHV8 DNA was detected by polymerase chain reaction in lesional Kaposi's sarcoma but not within mesothelioma. By immunostaining, mesothelioma cells expressed interleukin-6 and platelet-derived growth factor, which are important for survival of Kaposi's sarcoma cells. Besides the possibility of a casual association, we hypothesize that mesothelioma-linked factors may have contributed to the development of Kaposi's sarcoma in the presence of HHV8 infection.

Antibodies, Viral↗