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PubMed1999

Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.

Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the DNA extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the DNA extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. In situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association.

Biomarkers, Tumor↗
PubMed2000

Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas.

Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma characterized by bland histologic features and a paradoxically aggressive clinical course. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is a closely related tumor characterized by the presence of giant collagen rosettes. Only a single example of a metastasizing HSCT has been reported. A small subset of both LGFMS and HSCT display areas of increased cellularity and atypia which qualify as intermediate- to high-grade sarcoma; the significance of these features has not been definitively assessed. We present the clinicopathologic features of 77 cases of LGFMS and HSCT to determine the degree of overlap of these two lesions, their biologic behavior, and the significance of the occasional presence of intermediate- to high-grade sarcoma within both. The patients (33 female, 40 male) ranged from 3 to 78 years of age (median, 34 yrs). Fourteen cases occurred in patients less than 18 years of age. The tumors measured from 1 to 23 cm (median, 4.5 cm) and occurred predominantly in the trunk and lower extremities in both the deep (66 cases) and superficial (7 cases) soft tissues. In 15 cases, the tumor was present > 1 year before diagnosis. All tumors showed predominantly the typical hypocellularity and bland cytologic features of typical LGFMS; however, areas of hypercellularity and nuclear enlargement and hyperchromatism were identified in 12 of 73 (16%) and 7 of 73 (10%), respectively. Necrosis and mitotic activity >5/50 high-powered fields (HPF) were present in 6 of 73 (8%) and 5 of 73 (7%), respectively. Epithelioid areas were present in 33 of 73 (45%) and rosettes in 22 of 73 (30%). Follow up (54 cases; range, 2-192 mos; median, 24 mos; mean, 38 mos) showed 5 recurrences, 3 metastases, and 1 death. The diagnosis of LGFMS or HSCT was made prospectively in 51 patients; none had metastatic disease. Two of the metastatic tumors were LGFMS and one was a HSCT. LGFMS may occur more often in the pediatric population and show a much wider histologic spectrum than previously thought. A significant number of LGFMS possess inconspicuous collagen rosettes characteristic of HSCT, indicating that these two tumors are ends of a common spectrum rather than distinct entities. HSCT, like LGFMS, are low-grade sarcomas with metastatic potential. The presence of focal areas of intermediate- to high-grade sarcoma does not portend a worse outcome in the short term. The better prognosis reflected in this study compared with previous ones might reflect the fact that all were initially diagnosed as sarcomas and treated with aggressive surgery. The fact that the only three patients to develop metastatic disease were patients whose LGFMS or HSCT was identified retrospectively supports this concept.

Adolescent↗
PubMed1987

Gastrointestinal lymphoma and sarcoma. A case for aggressive search and destroy.

The incidence of sarcomas of the gastrointestinal tract has remained the same, but gastrointestinal lymphomas are gradually contributing a larger percentage of malignant gastrointestinal neoplasms. The authors have examined their more recent experience with these relatively rare lesions. Twenty-eight patients (13 with lymphoma, 15 with sarcoma) have been treated at the Vanderbilt University and the Metropolitan Nashville General Hospital since 1976. There were eight men in the group with lymphoma and six in the group with sarcoma. The average age for patients with lymphoma was 66 years; the average age was 57 years in the patients with sarcoma. Seven patients with lymphoma and eight patients with sarcoma had been treated for 6 months to 3 years for presumed peptic ulcer disease. Eight of these 15 patients were found to have perforated tumors at the time of surgical exploration. Three patients (all in the group with sarcoma) had metastatic liver disease or peritoneal implants at the time of diagnosis. Treatment for most patients included resection of the tumor, followed by chemotherapy or radiation in cases of tumor perforation or metastatic disease. The survival rate for patients with lymphoma has averaged 5.5 years, with a 55% 5-year survival rate. Patients with cleaved cell tumors survived longer than those with other types of lymphoma. In the group with sarcoma, the survival rate has been 3.1 years on the average, with a 21% 5-year survival rate.

Aged↗
PubMed1992

Advances in the diagnosis and management of sarcomas.

Cytogenetic alterations that characterize different histologic subtypes of soft tissue sarcomas have been identified. In a few situations, more precise chromosomal mapping has allowed identification of certain genes that may be involved in the development or tumor progression of sarcomas. Careful family histories must be elicited in sarcoma patients. While "cancer families" are rarely identified when screening close relatives of sarcoma patients, the discovery of the currently known tumor suppressor gene syndromes associated with germ line retinoblastoma gene and p53 gene defects were made possible by their association with sarcomas. Optimal management of primary sarcomas includes function-sparing complete resection and radiotherapy. Innovative radiotherapy, utilizing radiation sensitizers or brachytherapy, may increase local control in patients with unresectable tumors. New drugs are needed. Epirubicin and other anthracycline analogues do have significant activity; however, no other novel drugs have documented efficacy. Dose intensity is being explored with sarcoma trials providing the "vehicle" to evaluate new cytokines. Several mechanisms of doxorubicin resistance have been identified in cell lines and fresh tumors, including alterations in glutathione peroxidase activity and MDR-1 gene expression. These observations need to be taken to the clinic.

Antineoplastic Agents↗
PubMed1993

Clinical aspects of AIDS-related Kaposi's sarcoma.

Kaposi's sarcoma was one of the first conditions defining the new disease AIDS in 1981. From the outset, it was quite apparent that the biology of Kaposi's sarcoma in the setting of AIDS was quite different from that of most other known neoplasms. This observation led many investigators to speculate that Kaposi's sarcoma is, in fact, a reactive tumor and not a true malignancy. Our understanding of the pathogenesis of Kaposi's sarcoma has evolved over the years, and it now believed that the tumor arises from mesenchymal cells, which are influenced by HIV, various cytokines or growth factors, and perhaps other viruses or environmental factors operating in the setting of immune suppression. The tumor itself has various clinical manifestations, ranging from indolent cutaneous tumors to rapidly growing tumors involving lung and other viscera. New approaches to the treatment of Kaposi's sarcoma include inhibition of angiogenic and Kaposi's sarcoma stimulating growth factors, eg, interleukin-6, fibroblast growth factor, tumor necrosis factor, oncostatin-M, and the HIV-tat gene product. Additionally, improvement in our use of cytotoxic chemotherapy, interferons, and antiretroviral drugs has led to better management of complications of the tumor and of HIV itself. This review highlights recent advances in our understanding of Kaposi's sarcoma and its treatment, with a focus on pathogenesis and novel therapies.

Acquired Immunodeficiency Syndrome↗
PubMed1998

Clinical and biological impact of antiretroviral therapy with protease inhibitors on HIV-related Kaposi's sarcoma.

OBJECTIVE: To evaluate the clinical and biological impact of protease inhibitors on HIV-associated Kaposi's sarcoma. DESIGN AND SETTING: A cohort of 10 patients included prospectively from April 1996 to June 1997 were studied in one institutional centre after initiation of protease inhibitors. PATIENTS AND METHODS: All patients but one (stable disease) had progressive Kaposi's sarcoma. Three out of 10 patients had stopped specific chemotherapy for Kaposi's sarcoma for more than 4 weeks, three were still under chemotherapy, and four had never received specific treatment of Kaposi's sarcoma. Plasma HIV viral load, human herpesvirus (HHV)-8 viraemia in peripheral blood mononuclear cells (PBMC), and CD4 cell count were sequentially assessed from the beginning of therapy. For six patients, a semiquantitative evaluation of HHV-8 viral load in the Kaposi's sarcoma lesions was performed during treatment using polymerase chain reaction. RESULTS: After initiation of HIV triple therapy with protease inhibitors, we observed six complete responses, two partial responses, and two patients with progressive disease. All patients had undetectable plasma HIV viral load within 2 months of treatment. Undetectable HHV-8 viraemia in PBMC occurred in seven out of eight patients with partial or complete response and in none of the progressive patients. A decrease or negation of HHV-8 viral load in Kaposi's sarcoma lesions was observed in two complete responders. CONCLUSION: Our results suggest that antiviral therapy with protease inhibitors are clinically efficient in HIV-associated Kaposi's sarcoma and that there exists a correlation between clinical response and negation of HHV-8 viraemia.

AIDS-Related Opportunistic Infections↗
PubMedDate not supplied

MR characteristics of epithelioid sarcoma.

OBJECTIVE: The purpose of this study was to characterize MRI findings of epithelioid sarcomas and correlate them with the presence or absence of metastasis and/or recurrence. MATERIALS AND METHODS: Thirty-six MRI studies were analyzed retrospectively in 12 patients with histologically proven epithelioid sarcomas. The following parameters were evaluated: lesion location, signal characteristics, and bone or vascular involvement. Also, the time of the appearance and the frequency of local recurrences were determined, as well as location, frequency, and timing of appearance of metastatic disease. RESULTS: Epithelioid sarcomas were located mainly in the upper extremities (58%). The MRI findings divided the tumor into two major categories: hemorrhagic (two patients) and nonhemorrhagic (ten patients). The study showed recurrence in 33% of the cases and metastasis in 50% overall. Both patients with hemorrhagic epithelioid sarcoma showed metastases that appeared earlier than metastases from nonhemorrhagic sarcomas. Only 33% of the nonhemorrhagic sarcomas showed metastases; and 17% of the cases revealed vascular invasion. CONCLUSION: Magnetic resonance plays a role in recognizing intratumoral hemorrhage in patients with epithelial sarcoma. Patients with hemorrhagic tumors need more aggressive treatments and follow-up studies.

Adult↗
PubMed2000

Seroprevalence of human herpesvirus 8 and its association with Kaposi sarcoma in Brazil.

BACKGROUND: Epidemiologic studies have indicated that human herpesvirus 8 is implicated in the development of Kaposi sarcoma in different geographic areas worldwide. GOAL: To provide information on the prevalence of human herpesvirus 8 in Brazil and its association with Kaposi sarcoma. STUDY DESIGN: An immunofluorescence assay was performed to test 1,044 serum samples from 747 blood donors, 73 patients presenting to casualty departments, and 224 patients attending sexually transmitted disease/AIDS clinics. The sexually transmitted disease group was composed of 88 patients with HIV (40 Kaposi sarcoma positive, 48 Kaposi sarcoma negative) and 136 patients without HIV. RESULTS: Antibodies to human herpesvirus 8-latent nuclear antigens were found in 34 blood donors (4.6%), and in seven casualty patients (9.6%). The highest frequency of human herpesvirus 8 antibodies was found in the sexually transmitted disease group: 32 HIV-positive patients with Kaposi sarcoma (80%) and seven patients without Kaposi sarcoma (14.6%). CONCLUSION: The presence of human herpesvirus 8 in patients with HIV was strongly associated with Kaposi sarcoma (odds ratio, 23.4; 95% CI, 7.7-71.4), male gender, homosexual or bisexual orientation, and hepatitis B virus infection, but not with the other sexually transmitted diseases that were investigated.

Adult↗
PubMed2001

Intraneural monophasic synovial sarcoma: a case report.

STUDY DESIGN: A case report. OBJECTIVES: To illustrate a rare case of synovial sarcoma arising within a peripheral nerve. SUMMARY OF BACKGROUND DATA: A synovial sarcoma arising within a peripheral nerve is very unusual. Only five cases of primary synovial sarcoma within a peripheral nerve have been reported. This is the first case with involvement of the nerve root. The authors diagnosed the tumor arising within the S1 nerve root as synovial sarcoma using cytogenetic analysis that detected the chimeric SYT/SSX gene. METHODS: In addition to the immunohistochemical study, a reverse transcription-polymerase chain reaction (RT-PCR) assay was conducted for the SYT-SS10 fusion gene using archival formalin-fixed paraffin-embedded tumor specimens. RESULTS: Computed tomography scan, magnetic resonance imaging performed before surgery, and the intraoperative findings showed that the tumor was embedded within the S1 nerve root. Although the histologic findings were suggestive of a malignant peripheral nerve sheath tumor, the results of the cytologic study confirmed its diagnosis of synovial sarcoma. CONCLUSION: Primary intraneural synovial sarcoma, although rare, must be distinguished from malignant peripheral nerve sheath tumor. The molecular assay of the detection of the SYT/SSX fusion gene is useful to make a definite diagnosis of monophasic synovial sarcoma.

Female↗
PubMed2001

Osteogenic sarcoma associated with Diamond-Blackfan anemia: a report from the Diamond-Blackfan Anemia Registry.

PURPOSE: Diamond-Blackfan anemia (DBA) is a congenital pure red cell aplasia, usually presenting in infancy or early childhood. A review of the literature strongly supports a predisposition to hematopoietic malignancy. Recently, solid tumors have been reported, some attributable to hemosiderosis and/or androgen therapy. Two cases of osteogenic sarcoma have also been documented. An analysis from the Diamond-Blackfan Anemia Registry was performed to evaluate the cancer risk in patients with DBA. METHODS: The Diamond-Blackfan Anemia Registry of North America (DBAR) is a comprehensive database of patients with DBA enrolled, after informed consent, through outreach to pediatric hematologists and family groups. The patients and/or their families complete a detailed questionnaire, and a review of medical records and telephone interviews are performed to complete and clarify the information provided. RESULTS: Of the 354 patients registered in the DBAR, there were six patients meeting the accepted diagnostic criteria for DBA who were found to have malignancies. Three patients had osteogenic sarcoma diagnosed, one with myelodysplastic syndrome, one with colon carcinoma, and one with a soft tissue sarcoma. CONCLUSION: There appears to be an association of osteogenic sarcoma with DBA. A young age at presentation may be a feature of DBA-associated osteogenic sarcoma. Because of the immaturity of the database, the actuarial risk for osteogenic sarcoma and other cancers in individuals with DBA cannot be ascertained. Speculation is made regarding the nature of the molecular defect leading to the association of DBA and osteogenic sarcoma.

Adolescent↗
PubMed2006

Epithelioid sarcoma of Enzinger.

Epithelioid sarcoma was named in 1970 in a classic paper by Enzinger, who expanded the observations in a larger series in 1985. He defined a sarcoma with a peak incidence in young adult males and a predilection for extremities, involving subcutis or deeper tissue and extending along tendon sheaths or aponeuroses. The tumor forms nodules with central necrosis surrounded by bland polygonal cells with eosinophilic cytoplasm and peripheral spindling. Fibromalike, angiomatoid, and proximal aggressive variants (with larger cells, prominent nuceloi, and rhabdoid change) have since been described. Epithelioid sarcomas regularly express vimentin, cytokeratins, and epithelial membrane antigen, and about half are positive for CD34, but a wide range of other antigens can be expressed. S100 protein, desmin, and FLI-1 are usually negative. The ultrastructure displays epithelial and mesenchymal features including myofibroblastic differentiation. There are no specific genetic findings but several cases display chromosomal abnormalities in the 22q region. The tumor has no normal cellular counterpart and differs from both synovial sarcoma and carcinoma. There is a wide differential diagnosis from numerous benign and malignant conditions, including granuloma annulare, melanoma, and epithelioid vascular neoplasms. Epithelioid sarcoma has a high recurrence rate, which can be reduced by adequate surgery, and up to 40% metastasize, to regional lymph nodes, to lung, and other locations including scalp. Adverse prognostic factors include large size, male sex, older age, necrosis, vascular invasion, rhabdoid cytomorphology, and inadequate excision. Thirty-six years after Enzinger's original account, epithelioid sarcoma remains a clinically and pathologically distinct, indolent but aggressive sarcoma of indeterminate lineage.

Biomarkers, Tumor↗
PubMed2006

Paget sarcoma of the spine: Scottish Bone Tumor Registry experience.

STUDY DESIGN: Retrospective case study of 13 cases of Paget sarcoma of the spine accrued from a prospectively collected Tumor Registry database. OBJECTIVES: To analyze the clinical, radiologic, and histologic features of Paget sarcoma of the spine and to determine the factors influencing the prognosis. SUMMARY OF BACKGROUND DATA: Paget disease of bone is a common disorder with the spine being involved in over 50% of patients. However, sarcomatous degeneration in the vertebral column is an extremely rare complication. There is very little in the literature with regard to clinical presentation and prognosis of patients with Paget sarcoma affecting the vertebral column. METHODS: Between January 1944 and December 2003, 89 patients were registered with a diagnosis of Paget sarcoma in the Scottish Bone Tumor Registry. Thirteen patients with Paget sarcoma of the spine were analyzed with regard to their clinical, radiologic, and histopathologic features along with the prognostic predictors. RESULTS: The mean age was 66.9 years (range: 56-79 years). There were 10 males and three females. There were seven cases involving the sacral spine (63.6%), three cases involving lumbar vertebrae, two affecting the dorsal spine, and one with diffuse dorsolumbar involvement (D11-L3). The mode of presentation was progressively increasing low back pain (in all 13), unilateral sciatica (six; left-sided, five; right-sided, one), bilateral sciatica (two), lower limb weakness (eight), and autonomic dysfunction (four). Ten of 13 cases (76.9%) were osteosarcoma. The rest were chondrosarcoma (n = 1), fibrosarcoma (n = 1), and malignant fibrous histiocytoma (n = 1). Decompression laminectomy was performed in three patients with progressive neurologic deficit. Eight patients had received radiotherapy. The mean survival was 4.22 months. CONCLUSIONS: This series confirmed that Paget sarcoma of the spine has a very poor prognosis. We found a constellation of symptomatology in patients with sarcomatous Paget spine resulting from radiculomedullary compression, primarily lumbosacral involvement and predominantly osteosarcomatous histology. There was no significant difference observed on the overall prognosis of the patients with Paget sarcoma of the spine in the last 6 decades.

Adult↗
PubMed2006

Extent, relationship and prognostic significance of apoptosis and cell proliferation in synovial sarcoma.

AIMS: To analyse the extent, relationship and clinical significance of apoptosis and cell proliferation in synovial sarcoma. METHODS: Apoptosis was detected by TUNEL, and expression of Ki-67, Bcl-2, Bax and p53 was examined immunohistochemically in 72 synovial sarcomas. Their relation and correlation with clinicopathological parameters and survival rate were analysed. RESULTS: The average values of apoptosis index (AI) and Ki-67 labelling index (LI) were 0.76% and 28.30%, respectively. Both AI and Ki-67 LI in large-volume, high-grade and advanced-stage synovial sarcomas were significantly higher than those in small-volume, low-grade and early-stage ones (P<0.05 for all). And there was a linear relationship between AI and Ki-67 LI (r=0.751, P<0.001). All examined synovial sarcomas were positive for Bcl-2 and Bax, and only 20.8% cases showed expression of p53 protein. The expressions of Bcl-2, Bax and p53 were also significantly correlated with AI (P=0.005, P=0.002, P=0.037, respectively). In addition, patients with high AI (>0.76%) had poor prognosis (log-rank test; P=0.007). CONCLUSIONS: Alterations in apoptosis and cell proliferation activity might be responsible for the pathogenesis and behaviour of synovial sarcoma. Increased rate of apoptosis in synovial sarcoma was considered to be an indicator of poor prognosis. In addition, apoptosis in synovial sarcoma may be controlled by multiple apoptosis-regulating mechanisms, including the Bcl-2 family and p53 protein.

Adolescent↗
PubMed2006

Clear cell sarcoma of tendons and aponeuroses: a historical perspective and tribute to the man behind the entity.

Clear cell sarcoma of tendons and aponeuroses is a unique sarcoma initially described by Dr Franz M. Enzinger. The tumor has a proclivity to involve the tendons and aponeuroses of distal extremities of relatively young individuals and is characterized by multiple local recurrences with late metastases and a high rate of tumor deaths. Since its seminal description in 1965, there have been many studies verifying the uniqueness of this entity and probing its differentiation. Ultrastructural and immunohistochemical studies have shown melanocytic differentiation, whereas molecular genetic studies have shown cytogenetic rearrangements resulting in a EWSR1-ATF1 fusion gene that is characteristic but not entirely unique for clear cell sarcoma (similar fusion genes are also seen in angiomatoid fibrous histiocytoma). Detection of this fusion gene and the absence of BRAF gene mutations clearly distinguish clear cell sarcoma from cutaneous melanoma. Adverse prognostic factors identified to date include larger tumor size and any microscopic tumor necrosis. Surgery is the mainstay of treatment for this high grade sarcoma, with chemotherapy having little effect. Although the melanocytic differentiation of clear cell sarcoma is indisputable, its precise lineage remains unclear. Thus, clear cell sarcoma maintains the status of a unique yet enigmatic clinicopathologic entity of ever increasing complexity 40 years after its original description by an extraordinarily gifted man.

Fascia↗
PubMed2005

Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature.

Intimal sarcoma (IS) is defined as a malignant tumor arising in the tunica intima of large blood vessels. In systemic circulation, the majority of IS develop in the aorta, where close to three fourths of published cases lack specific differentiation and are called undifferentiated intimal sarcomas (UIS). The remaining cases are intima-associated sarcomas of recognized types, also called differentiated intimal sarcomas (DIS). In this report, we further characterize UIS, including its immunohistochemical profile and results of comparative genomic hybridization. A total of 14 cases of UIS were collected from 17 medical institutions, including slides, blocks, electron photomicrographs, clinical abstracts, and reports of surgical pathology specimens and autopsies. The patients, 7 women and 7 men, were 41 to 85 years of age (median, 65.6 years). Twelve tumors arose from the aorta, one from the left external iliac and femoral arteries, and one in a large systemic vein (the venous tumor was included due to histologic similarity with the arterial lesions). Tumors ranged from 1 cm to over 10 cm in diameter. Histopathology was that of a largely necrotic, poorly differentiated epithelioid and pleomorphic malignant neoplasm relating to the tunica intima. Usually there was only a thin layer of viable tumor cells overlying a large thrombus. All tumors stained at least focally with the endothelial markers CD31 and Fli-1; however, there was otherwise considerable variability in immunophenotype. The distinctive histopathologic appearance of the primary luminal lesion was lost whenever tumor invaded outside the vessel wall (into adventitia and beyond) or in metastatic sites. Such extravascular tumors assumed a variety of patterns reminiscent of undifferentiated pleomorphic sarcoma (UPS; in older literature also known as pleomorphic malignant fibrous histiocytoma, MFH) or other distinct types of sarcomas, including osteosarcoma, angiosarcoma, and rhabdomyosarcoma. The results of comparative genomic hybridization were nonspecific. Eleven patients died of the disease, in an average of 11 months after diagnosis. Three patients are still alive and free of disease at 4, 16, and 27 years. UIS of large systemic vessels represents a distinct clinical entity where intraluminal sarcoma presents with thrombosis and occlusion of large vessels. It is associated with a highly characteristic, although not entirely specific, histology and immunohistochemical phenotype. The histogenesis of UIS is not certain; however, it seems that the cell of origin must leave the confines of the vessel wall to show altered morphology. Although there are rare long-term survivors, UIS behaves as a fully malignant neoplasm that is almost uniformly associated with metastases and tumor-related death.

Adult↗
PubMed2007

Primary synovial sarcoma in fallopian tube: case report and literature review.

Synovial sarcoma, a malignant mesenchymal neoplasm, occurs mostly near the joints of the extremities and occasionally outside the joint such as lung. We report a case of soft tissue sarcoma arising in the fallopian tube origin that showed characteristic pathological appearance of biphasic synovial sarcoma. Molecular analysis detected a fusion gene transcript of synovial sarcoma translocation (SYT) gene from chromosome 18 and synovial sarcoma X chromosome breakpoint 1 (SSX1) gene, which is believed to pathognomonic for synovial sarcoma of joint origin. Recurrent abdominal tumor, observed at 12 month after the initial surgery and following chemotherapy using doxorubicin, cisplatin and ifosfamide, partially responded to chemotherapy using paclitaxel and carboplatin and, then, optimal surgery was performed. This is the first report of a synovial sarcoma arising in the fallopian tube.

Adult↗
PubMed1994

International variations in the incidence of childhood soft-tissue sarcomas.

In the study of international childhood cancer incidence coordinated by the International Agency for Research on cancer, soft-tissue sarcomas comprised between 4 and 8% of all cancers between 0 and 14 years of age. Among predominantly white populations, the age-standardised annual incidence rate (ASR) for all soft-tissue sarcomas was between 5 and 9 per million. The most common histological subtypes were rhabdomyosarcoma (ASR 4-7 per million) and fibrosarcoma, including other malignant fibromatous tumours (ASR 1-2 per million). In the United States the incidence of rhabdomyosarcoma for black girls was only half that for white girls, while the rates for boys were similar in the two ethnic groups; fibrosarcoma had a higher incidence among black people than white people for both sexes. Throughout most of Asia, soft-tissue sarcomas almost invariably had a total ASR below 6 per million, rhabdomyosarcoma and fibrosarcoma again being the most common histological types. In Africa, incidence rates could not generally be calculated, but there were substantial numbers of registrations for rhabdomyosarcoma and fibrosarcoma. The majority of cases of Kaposi's sarcoma were in African children, and in eastern and southern Africa where Kaposi's sarcoma is endemic among adult men it comprised 25-50% of soft-tissue sarcomas and 2-10% of all childhood cancers; the sex ratio (M/F) was 2.2:1, in contrast to the overwhelming male predominance among adults. Variations between ethnic groups in the incidence of rhabdomyosarcoma and fibrosarcoma, together with their occurrence in a number of heritable syndromes, suggest that genetic predisposition is important in their aetiology. There was little evidence for an environmental aetiology in rhabdomyosarcoma and fibrosarcoma but an infectious agent may be responsible for childhood Kaposi's sarcoma.

Adolescent↗
PubMed1997

Chromosomal translocations in human soft tissue sarcomas by interphase fluorescence in situ hybridization.

In soft tissue sarcomas, clonal rearrangement of chromosomes has been shown by cytogenetic analysis to be unique and specific for tumor types. The development of fluorescence in situ hybridization (FISH) has allowed detection of chromosomal rearrangements in the interphase nuclei isolated from paraffin-embedded tissues. Three kinds of translocations in the interphase nuclei that were isolated from 47 cases of soft tissue sarcomas were examined by FISH with chromosome-specific DNA probes of centromeric and total probes. Of 47 soft tissue sarcomas 42 (89.4%) revealed tumor-specific translocations by retrospective cytogenetic analysis. Translocation t(X;18) was detected in 25/28 synovial sarcomas; translocation t(11;22) in 5/6 Ewing's sarcomas and primitive neuroectodermal tumors (PNET); and translocation t(12;16) was found in 12/13 liposarcomas, including 10 myxoid and two round cell types as clonal chromosomal aberrations specific for both subtypes. Based on the cytogenetic analysis, Ewing's sarcoma is related closely with PNET as shown by MIC2-protein reactivity. Other cytogenetic findings of translocation t(12;16) indicate that round cell liposarcomas share chromosomal changes with myxoid liposarcomas, and further suggest that both tumor subtypes of liposarcoma may possess common precursor cells. FISH is a useful aid in determining the tumor type of soft tissue sarcomas with regard to histogenetic origin.

Adolescent↗