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PubMed1999

Soft tissue sarcoma in adults.

Soft tissue sarcomas are a heterogeneous group of tumors, consisting of numerous histiotypes that all share a putative common mesenchymal origin. Although prognosis of these tumors is determined by clinical parameters (size, location, and resection margin status) and pathologic features (mitotic activity and necrosis), the histologic subtype has never been shown to be a consistent independent prognostic factor. Some relevant differences among these histiotypes are emerging, in specific biological parameters such as proliferation indices, in integrin expression profiles, and with regard to drug sensitivity. Several biological factors are considered to be prognostically important. Most attention is directed to regulators of cell-cycle progression. The significance of p53 dysregulation is confirmed by the inhibition of cellular proliferation, both in in-vitro and in in-vivo sarcoma models, after reintroduction of wild type p53. A multidisciplinary approach is essential for the optimal treatment of soft tissue sarcomas. Multimodality treatment has led to a patient-tailored approach with limb-sparing resections integrated with external and/or interstitial irradiation. The value of chemotherapy both in the neoadjuvant and the adjuvant setting, although of critical value in other sarcomas such as Ewing's sarcoma and osteosarcoma, remains to be established for soft tissue sarcomas.

Adult↗
PubMed2001

Angiogenesis and antiangiogenic approaches to sarcomas.

Established chemotherapy regimens for advanced or metastatic sarcoma generally have low response rates and substantial toxicity, and resistance often arises quickly, making sarcoma an ideal target for alternative treatment approaches. Antiangiogenic therapies have a number of potential advantages including decreased resistance, fewer side effects, and a broad spectrum of activity. This paper reviews the evidence supporting the use of antiangiogenic therapies for adult soft tissue sarcomas. Human sarcomas express a number of proangiogenic factors that may represent therapeutic targets, with vascular endothelial growth factor being the best characterized, and inhibitors of angiogenesis have antitumor activity in animal models of human sarcomas. Clinical trials are in early stages, although promising results have already been seen. In the future, improved drugs, refined molecular profiling of tumors, and new ways of combining antiangiogenic agents with cytotoxic agents may lead to more effective and tolerable therapies for sarcomas.

Angiogenesis Inhibitors↗
PubMed1997

Survival of European patients with Kaposi's sarcoma as AIDS-defining condition during the first decade of AIDS. AIDS in Europe Study Group.

OBJECTIVES: To determine whether the survival of patients with Kaposi's sarcoma as first manifestation of AIDS has changed between 1979 and 1989; and to define whether prognosis factors could be identified. PATIENTS AND METHODS: This is a multicentric retrospective cohort study from 52 centers in 17 European countries involving adults AIDS patients diagnosed between 1979 and 1989. Variables such as age, sex, geographical regions, transmission groups, date of Kaposi's sarcoma diagnosis, zidovudine use, CD4+ cell count and concomitant opportunistic infections or AIDS-related malignancies were evaluated by using uni- and multivariable proportional hazard models. Log-rank tests were used to determine which variables were associated with survival. RESULTS: From the 6,546 AIDS patients recruited in the database of the AIDS in Europe Study Group, 1,394 were diagnosed with Kaposi's sarcoma at the time of AIDS diagnosis, from 1979 and 1989. A total of 1,047 Kaposi's sarcoma patients died during the follow-up period. By Kaplan-Meier analyses, the median and mean survival for these Kaposi's sarcoma patients were 17 and 25 months, respectively, with no change over time. However, age, sex (female), geographic region, low CD4+ cell count (< 150 x 10(6)/l) and some opportunistic infections and non-Hodgkin's lymphoma were associated with a poorer prognosis. Zidovudine use, year of diagnosis and risk factor for HIV-1 infection brought no additional information as predictor of mortality. CONCLUSIONS: This study suggests that the survival of patients with Kaposi's sarcoma as first manifestation of AIDS has remained poor during the last decade in contrast with the overall AIDS survival which had significantly improved from a median of 13-18 months during the same period of observation. There is a need for further prospective information to explain the worse prognosis in women and the geographical variations.

AIDS-Related Opportunistic Infections↗
PubMed1998

Kaposi's sarcoma in HIV infection: impact on opportunistic infections and survival.

OBJECTIVE: To determine the effect of Kaposi's sarcoma on survival of HIV-infected patients. METHODS: Retrospective cohort study to compare the survival of 241 HIV-infected homosexual patients with Kaposi's sarcoma (cases) with that of 241 HIV-infected homosexual patients without Kaposi's sarcoma (control subjects) but with a similar level of immunosuppression (measured by the absolute CD4+ lymphocyte count). RESULTS: Cases and control subjects were similar in age, occurrence of previous opportunistic infections, and the use of antiretroviral therapy. The mean CD4+ lymphocyte counts were similar for cases and control subjects (185 x 10(6) versus 184 x 10(6)/l, respectively). Cases had a higher incidence of opportunistic infections (5.95 versus 3.88 infections, respectively, per 100 person-months of observation) and a greater number of infections typical of late-stage HIV infection. Cases had a shorter overall survival than did control subjects (P=0.0025). Kaposi's sarcoma was associated with an increased risk of death (odds ratio, 1.28), even when adjusting for age, previous opportunistic infection, baseline CD4+ lymphocyte count, and antiretroviral therapy. CONCLUSION: Kaposi's sarcoma appears to accelerate the clinical course of HIV infection. Opportunistic infections develop earlier and more often in patients with the disease than in control subjects. Survival was significantly shorter in patients with Kaposi's sarcoma.

AIDS-Related Opportunistic Infections↗
PubMed1999

Sarcomas of the head and neck.

Sarcomas are rare malignancies of mesenchymal origin. Computed tomographic and magnetic resonance imaging characteristics, as well as histologic findings and epidemiology, of sarcomas of the head and neck are reviewed. The sarcomas discussed include rhabdomyosarcoma, fibrosarcoma, osteosarcoma, chondrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, alveolar soft part sarcoma, Ewing's sarcoma, and synovial sarcoma. The imaging findings of these entities frequently are nonspecific. Imaging, particularly magnetic resonance imaging, has a major role in defining the extent of these tumors. This is important because complete surgical excision is the preferred method of treatment. Imaging also is useful in planning radiation therapy and determining prognosis.

Head and Neck Neoplasms↗
PubMed1983

Dose-response relationships for radium-induced bone sarcomas.

The incidence of bone sarcomas among 3055 female radium-dial workers who entered the dial industry before 1950 was used to determine dose-response relationships for the induction of bone sarcomas by radium. Two subpopulations were analyzed: all measured cases who survived at least 5 yr after the start of employment and all cases who survived at least 2 yr after first measurement. The first constituted a group based on year of entry; it contained 1468 women who experienced 42 bone sarcomas; the expected number was 0.4. The second comprised a group based on first measurement; it contained 1257 women who experienced 13 bone sarcomas; the expected number was 0.2. The dose-response function, I = (C + alpha D + beta D2)e-gammaD, and simplifications of this general form, were fit to each data set. Incidence (I) was in units of bone sarcomas per person-yr; (D) was the quantity (muCi) of radium that entered the blood. Two functions, I = (C + alpha D + beta D2)e-gammaD and I = (C + beta D2)e-gammaD, fit the data for year of entry (p greater than or equal to 0.05); both these functions and I = (C + alpha D) fit the data for first measurement. The function I = (C + beta D2)e-gammaD was used to predict the number of bone sarcomas in all other pre-1950 radium cases (medical, laboratory and other exposures); fewer were actually observed than the fit of this function to the female dial workers predicted.

Adult↗
PubMedDate not supplied

Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease.

OBJECTIVE: The purpose of this study was to present the computed tomography (CT) findings of pulmonary artery sarcoma in 7 patients with a focus on the distinguishing features of pulmonary embolic disease. METHODS: For the 9 years from December 1993 to November 2002, we treated 7 patients with pathologically proven pulmonary artery sarcoma, and during the 2 years from December 2000 to November 2002, we treated 40 patients with acute (n = 33) or chronic (n = 7) pulmonary embolism. In these patients, pulmonary embolism was diagnosed from serial CT or clinical findings. Two chest radiologists, blinded to the diagnoses, independently reviewed the scans of all 47 patients in random order, and the so-documented CT features of sarcoma and pulmonary embolism were compared by using Fisher exact test or the generalized estimating equations test. RESULTS: The two most frequent CT findings of pulmonary artery sarcomas were a low-attenuation filling defect occupying the entire luminal diameter of the main (n = 1) or proximal (n = 6) pulmonary artery and an expansion of any segment of the pulmonary artery with extensive intraluminal filling defect, as observed in six (86%) of 7 patients. In contrast, the finding of a lesion occupying the entire luminal diameter at the level of proximal pulmonary arteries was absent in all 40 patients with pulmonary embolism (P < 0.0001) (kappa = 0.9111). Expansion of the pulmonary arteries was seen in one (3%) of 40 patients with pulmonary embolism (P < 0.0001) (kappa = 0.9108). Extraluminal extension was observed in 5 of 7 (71%) patients with sarcoma, but in no patient with an embolism (P < 0.0001) (kappa = 0.8773). CONCLUSION: CT can help differentiate pulmonary artery sarcoma from pulmonary embolism by indicating a low-attenuation filling defect occupying the entire luminal diameter of the proximal or main pulmonary artery, expansion of the involved arteries, or extraluminal tumor extension.

Adult↗
PubMed2003

Primary synovial sarcoma of the chest: radiographic and clinicopathologic correlation.

Primary synovial sarcoma of the thorax is rare. Origin of thoracic synovial sarcoma in the pleura or lung was first described only 7 years ago. Radiologic characteristics of this disease have not yet been studied in a formal series. The authors sought to define the radiologic features of primary thoracic synovial sarcoma and to correlate the findings with clinical and pathologic features. They examined clinical, radiologic, and pathologic features of five patients with primary synovial sarcoma of the chest. Radiologic evaluation included conventional radiographs, computed tomographic scans, and magnetic resonance images of the chest. Patients included three men and two women who ranged in age from 28 to 40 years. Primary tumors involved the chest wall (n = 2), lung (n = 1), or both (n = 2). Chest pain was the most common presenting symptom. Although conventional radiographs often showed the lesions to be ill defined, computed tomographic scans showed well-defined masses in every case. Heterogeneous enhancement and an absence of calcification were also seen. Pathologic evaluation demonstrated synovial sarcoma with equal distribution between the monophasic and biphasic variants. The chromosomal translocation X;18 was demonstrated in four of four cases tested. All patients were treated by resection. Recurrence was demonstrated radiologically in four patients at 2 to 14 months. All patients were alive at 9 to 58 months of follow-up. The authors conclude that primary synovial sarcoma of the chest occurs in young adults, most commonly presenting with chest pain. It is characterized radiologically by a heterogeneously enhancing well-defined mass without calcifications.

Adult↗
PubMed2002

Synovial cell sarcoma: diagnosis, treatment, and outcomes.

OBJECTIVES/HYPOTHESIS: Synovial cell sarcoma is a mesenchymal tumor predominantly of the lower extremities. Three percent of cases arise in the head and neck region. It is thought that head and neck synovial sarcoma has a better prognosis than tumors of the extremities. Our experience has demonstrated aggressive behavior of this neoplasm in the head and neck. This compelled us to compare our experience with other studies. STUDY DESIGN: Retrospective chart review. METHODS: We obtained the records of patients diagnosed with head and neck synovial sarcoma from the Tumor Registry of the University of Louisville School of Medicine (Louisville, KY) and affiliated hospitals for data compiled between January 1990 and December 2000. Data on patient demographics, clinical findings and symptoms, histological findings, treatment, extent of disease, recurrence, and survival were recorded. The literature was reviewed identifying reports of synovial cell sarcoma. RESULTS: Five consecutive patients with synovial cell sarcoma were assessed at our facility. The median patient age was 28.2 years. All of the patients underwent an aggressive primary surgical excision followed by irradiation. All patients received chemotherapy after recurrence. Four of the five patients had local recurrence, and all five of the patients developed distant metastases. Three of the patients have died, and two are alive with evidence of disease. Novel sites are reported including the ethmoid sinus and the parotid gland. This group demonstrated a 40% 5-year overall survival, which was lower than the 60% 5-year survival reported in the literature for all sites. CONCLUSIONS: Synovial cell sarcoma of the head and neck is a disease of young people and carries a poor prognosis. The aggressive nature of the disease may require modification of accepted treatment modalities and sequence.

Adult↗
PubMed1998

Functional outcome after soft-tissue reconstruction for limb salvage after sarcoma surgery.

Limb salvage has been achieved for patients with sarcoma by means of compartmental resection, soft-tissue reconstruction, and adjuvant therapy without increased rates of local recurrence, metastasis, or mortality. Despite the prevalence of limb salvage procedures in the treatment of these tumors, relatively little information has been published regarding late functional results in these reconstructed extremities. This study reports on the functional outcome for soft-tissue reconstruction for limb salvage in patients with sarcoma. Over the past 6 years, 28 patients were treated for sarcomas of the extremity in which soft-tissue reconstruction was needed for complete limb salvage. The mean age of these patients was 48 years (range, 14 to 83 years); there were 14 male and 14 female patients. Of the 28 sarcomas, 23 cases involved the lower extremity and 5 cases were in the upper extremity. Reconstruction was performed primarily in 12 patients; 16 reconstructions were performed secondarily because of wound complications after initial extirpation. Adjuvant radiation therapy was administered either preoperatively or postoperatively in all cases. Of the 33 reconstructive procedures performed in these 28 patients, 16 involved free flaps and 17 involved local flaps. All patients achieved initial limb salvage after the reconstructive procedure(s). Mean follow-up was 38 months. Twenty patients were available for the evaluation portion of the study. Two patients had delayed amputations: one for recurrent disease and another for osteoradionecrosis. Two patients died before beginning the examination process: one patient from the sarcoma and another patient from colon cancer. Twenty of the remaining 24 patients agreed to participate and were examined using the Enneking outcome measurement scale. Patients were examined for range of motion, deformity, stability, pain level, strength, functional activity, and emotional acceptance and assigned a numerical score for each category. Based on this, an overall rating of excellent, good, fair, or poor was assigned. Nine patients (45 percent) achieved an overall rating of excellent, five patients (25 percent) achieved a rating of good, and six patients (30 percent) achieved a fair score. None had received a rating of poor. There were no differences in the results obtained comparing upper versus lower extremity, immediate versus delayed reconstruction, or reconstructions performed with a free flap versus a pedicled flap. This study supports the continued use of soft-tissue reconstruction for limb salvage in sarcoma surgery with good to excellent late functional results obtained in the majority of patients.

Adolescent↗
PubMed1980

Primary lymphomas and sarcomas of the stomach.

The records of patients with primary gastric lymphoma and sarcoma treated at M. D. Anderson Hospital and Tumor Institute between 1945 and 1975 were reviewed. Weight loss, abdominal pain, nausea, and vomiting were the most common presenting symptoms, while palpable abdominal mass was the most common sign. The lymphomas were predominantly located in the distal portion of the stomach, in contrast to the sarcomas, which were commonly located in the body and the proximal portion of stomach. Curative gastric resection was performed in 96% of patients with lymphoma and in 67% of patients with sarcoma. Diffuse histiocytic lymphoma and leiomyosarcoma were the most common histologic types. Patients with lymphoma survived significantly longer than patients with sarcoma (median 75 vs 22 months, P = 0.009). Adjuvant radiotherapy seemed to improve the survival of patients with lymphoma, while curative gastric resection provided the only hope for long-term survival for patients with gastric sarcoma. The place of adjuvant chemotherapy after curative resection of gastric lymphoma and sarcoma remains to be investigated.

Female↗
PubMed2003

Detection of fusion gene transcripts in fresh-frozen and formalin-fixed paraffin-embedded tissue sections of soft-tissue sarcomas after laser capture microdissection and rt-PCR.

The diagnosis of small round cell sarcomas is often very difficult, especially when only small biopsy specimens are available for examination. Recent studies have shown that some sarcomas have specific recurrent chromosomal translocations producing chimeric gene fusions, which can be detected by reverse transcription-polymerase chain reaction (RT-PCR), fluorescent in situ hybridization (FISH), or cytogenetic analysis. In this study, 12 cases of well-defined sarcomas including Ewings sarcoma/primitive neuroectodermal tumors (ES/PNET), synovial sarcoma (SS), alveolar rhabdomyosarcoma (ARMS), and desmoplastic small round cell tumors (DSRCT) were used to collect specific numbers of cells by laser capture microdissection (LCM), subsequently used for RT-PCR to detect specific chimeric gene transcripts. Tumor cells from fresh-frozen (FS) tissue sections and paraffin-embedded (PS) tissue sections from the same cases were compared directly to evaluate the sensitivity of FS and PS sections as the starting material for analysis. Samples were used for RNA extraction, RT-PCR analysis, and Southern hybridization with fluorescein-labeled internal probes followed by enhance chemiluminescence (ECL) detection. The fusion gene transcripts could be detected using 50 cells from FS materials in all cases and from 1 cell in 9 of 12 cases. For PS, a positive signal could be detected using 200 to 1000 cells in all cases, while weaker signals were detected using 50 cells in most cases. These results indicate that the fusion gene products from small round cell sarcomas can be detected by RT-PCR with 10 to 200 cells from FS and PS tissues. The sensitivity of RT-PCR with FS was 10- to 50-fold greater than with PS. These results also suggest that RT-PCR analysis for sarcoma fusion gene products can be successfully performed when only a few cells are available for analysis, although this is not recommended for routine clinical use.

Blotting, Southern↗
PubMed2004

hTERT Is highly expressed in Ewing's sarcoma and activated by EWS-ETS oncoproteins.

Although the fusion proteins EWS-ETS are uniquely associated with Ewing's sarcoma and have been shown to have transformational properties, they are not the only determinants of oncogenesis. Therefore, before molecular-based therapy can be initiated, a better understanding of the molecular network specific to Ewing's sarcoma is mandatory. Specimens from 31 patients with Ewing's sarcoma were analyzed immunohistochemically. We found that human telomerase reverse transcriptase was expressed highly (78%) in Ewing's sarcoma. The mean followup was 7 years (range, 1-21 years), and human telomerase reverse transcriptase expression was correlated with outcome. Because we did not find an association between expression pattern and survival, human telomerase reverse transcriptase may not serve as a tumor marker in Ewing's sarcoma. However, the human telomerase reverse transcriptase promoter is shown to be activated by the fusion proteins. Therefore, transcriptional regulation via EWS-ETS may account for the high human telomerase reverse transcriptase expression in Ewing's sarcoma.

Adolescent↗
PubMed2005

Multilevel en bloc spondylectomy and chest wall excision via a simultaneous anterior and posterior approach for Ewing sarcoma.

STUDY DESIGN: A case study of a patient with Ewing sarcoma of T8 and T9 with paravertebral and chest wall involvement, who underwent neoadjuvant chemotherapy and subsequent multilevel en bloc spondylectomy and chest wall excision using a simultaneous anterior and posterior approach. OBJECTIVE: To show the feasibility of treating Ewing sarcoma of the thoracic spine with paravertebral and chest wall extension by multiagent chemotherapy followed by a multilevel en bloc spondylectomy and chest wall excision using a simultaneous anterior and posterior approach. SUMMARY OF BACKGROUND DATA: Ewing sarcoma is a primary malignant bone tumor that occasionally involves the spinal column. Most patients with Ewing sarcoma of the spine are treated with systemic chemotherapy followed by definitive local control. Radiation therapy is the usual mode of local control in these patients because the spinal column has historically been considered a surgically inaccessible site where wide surgical margins are difficult to obtain. However, en bloc spondylectomy techniques have been described that can probably further decrease the risk of local recurrence, thereby minimizing or even eliminating the need for radiation therapy. To our knowledge, a combined en bloc spondylectomy and chest wall excision in a patient with Ewing sarcoma in the spine has not been previously reported. METHODS: Neoadjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide was administered. After completion of the chemotherapy, an en bloc spondylectomy of T8 and T9 with removal of the chest wall was achieved using a simultaneous anterior and posterior approach to the spine. A stackable carbon fiber cage filled with autograft and allograft bone was inserted between T7 and T10. The spine was stabilized with anterior and posterior instrumentation. The chest wall was reconstructed with contoured polymethylmethacrylate and polypropylene (Marlex, Textile Development Associates, Inc., Franklin Square, NY) mesh. RESULTS: The patient maintained normal neurologic function, and pain was lessened. The margins were free of tumor, and tumor necrosis was 100%. After surgery, radiotherapy was not administered. No local tumor recurrence or distant metastases were evident at the last follow-up. Balance in the coronal and sagittal planes was maintained. The patient has returned to work and resumed normal activities of daily living. CONCLUSIONS: Multilevel en bloc spondylectomy and chest wall excision performed using a simultaneous anterior and posterior approach is a safe and effective technique that may be used to achieve adequate margins in select patients with malignant tumors involving the thoracic spine and chest wall. This technique can eliminate the need for radiation therapy in patients with Ewing sarcoma and probably decreases the risk of local recurrence compared with radiation therapy alone.

Adult↗
PubMed2005

Genetic predictors for drug resistance in soft tissue sarcoma: a review of publications in 2004.

PURPOSE OF REVIEW: Sarcomas are generally managed through a multidisciplinary approach including surgery, radiotherapy, and chemotherapy. Metastatic tumor cells frequently develop resistance to cytotoxic agents. Several molecular mechanisms of drug resistance have been uncovered recently. RECENT FINDINGS: Among genes governing the apoptosis pathway, overexpression of the bcl2 family or mutations of p53 have recently been reported to be involved in drug resistance in sarcoma. The multidrug resistance genes are involved in the efflux of chemotherapeutic agents. Identification of members of this family of genes may predict resistance for developing new strategies. Several histologic subtypes of sarcomas have specific mechanisms of resistance. Methotrexate is one of the four active drugs for osteosarcoma and penetrates in tumor cell through specific carrier (reduced folate carrier) whose levels of expression or changes in amino acid sequence correlate to resistance to methotrexate. Specific molecular alteration defining nosological entities among sarcoma also correlates to drug resistance. Thirty percent of human sarcomas are characterized by specific translocations that may predict, for specific subtypes, survival and response to treatment. Finally, gastrointestinal tumors harbor specific activating mutations in KIT or PDGFRA genes, which are responsive to imatinib. Specific mutations of the KIT and PDGFRA genes have now repeatedly been found correlated to response or resistance to imatinib. SUMMARY: The molecular alterations present in tumor cells predict influence sensitivity or resistance to chemotherapy. Ultimately, this may delineate specific therapeutic strategies for both cytotoxic and targeted therapies in sarcomas.

Antineoplastic Agents↗
PubMed2006

Future directions for immunotherapeutic intervention against sarcomas.

PURPOSE OF REVIEW: To discuss advances in immunotherapy as pertaining to systemic therapy for sarcomas. RECENT FINDINGS: Developments in immunology will have direct relevance to studies in sarcomas and other cancers in the near future. Vaccines employing peptides are the backbone of many studies today due to issues of cost and ease of production, but intact or transfected whole tumor cells or antigen-pulsed or transfected dendritic cells are now being investigated as the immunogenic principle in sarcomas and other cancers. The importance of dendritic cells in generating immune responses is better appreciated than ever, as is the role of CD4+ CD25+ regulatory T cells in mediating immune responses. Enhancing costimulatory signals to T cells using anti-CD152 (CTLA4) and other antibodies or expanding anti-tumor cytotoxic T lymphocytes ex vivo are other means to enhance immunity to sarcoma-specific antigens. SUMMARY: As of 2006, few studies are examining the utility of immunotherapy in sarcomas. However, a detailed understanding of the remarkable mechanics of how an immune response is mounted and how T cell activation and/or proliferation can be halted by the tumor will be central to properly design new studies of immunological agents against sarcomas and other cancers.

Allergy and Immunology↗
PubMed2005

Bilateral breast metastases from ewing sarcoma of the femur.

We report a case of bilateral breast metastases from Ewing sarcoma of the femur. A 40-year-old woman presented with Ewing sarcoma of the left thigh, treated by complete surgical exeresis and chemotherapy. Secondary, a large tumor appeared in the left breast. Bone scintigraphy, chest, and abdominal computed tomographic scan were normal. A breast biopsy found a malignant tumor composed of small round cells consistent with the initial diagnosis. After the first cycle of chemotherapy, a tumor was discovered in the controlateral breast. After 5 cycles, residual tumors persisted in the 2 breasts. Tumor exeresis was performed and found bilateral breast metastases of Ewing sarcoma. Because of the early recurrence of the left breast tumor, segmentectomy of the right breast and left mastectomy were performed. The histopathological analysis confirmed Ewing sarcoma metastases in the left breast. Despite local radiotherapy, the clinical course was marked by lumbar bone metastasis, local chest evolution, and progression of the disease. Metastases to the breast by extramammary malignant neoplasms are unusual. Sarcoma is an extremely rare cause of breast metastases and our case is the first report of breast metastases from Ewing sarcoma.

Adult↗
PubMed2005

Myxoid clear cell sarcoma.

Clear cell sarcoma is a rare soft-tissue tumor presenting typically in the extremities of young adults. It has been also known as malignant melanoma of the soft parts because of the presence of melanin and cytoplasmic melanosomes. However, clear cell sarcoma is, at present, usually considered as a unique lesion because the t(12;22)(q13;q12) translocation is present only in clear cell sarcoma. Myxoid malignant melanoma is now a well-recognized morphologic variant of malignant melanoma. However, a myxoid variant of clear cell sarcoma has not been well described yet. We report a case of myxoid clear cell sarcoma occurring on the heel in a 22-year-old man. The tumor was composed of nests and fascicles of oval to fusiform cells with clear to pale eosinophilic cytoplasm, often separated by fibrous septa. The tumor cells were reactive for S-100 protein, HMB-45, and MART-1. Variably sized cysts lined by one or several layers of tumor cells were observed. Alcian blue and mucicarmine stains demonstrated prominent mucin deposition in the tumor stroma and especially in the lumen of the cysts. Fluorescence in situ hybridization for the Ewing sarcoma gene showed rearrangement in nearly all of the neoplastic cells.

Adult↗