PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Sarcoma, Alveolar Soft Part”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 325 records · Page 18Linked to original sources
PubMed1978

Ultrastructure of malignant paraganglioma of organ of Zuckerkandl.

Electron microscopic study of a malignant paraganglioma of the organ of Zuckerkandl revealed similarities between the tumor and the normal paraganglia. The well-differentiated portion of the tumor recapitulated the structure of the basic functional units of the paraganglion. In addition there signs of anaplasia both at the histologic and ultrastructural level. Large neurosecretory granules were noted in some of the tumor cells, but most of the cells were agranular. Crystaloids resembling those seen in alveolar soft part sarcoma were also noted. The study supports the theory on the common origin and histogenesis of paragangliomas and alveolar soft part sarcomas.

Chromaffin System↗
PubMed1981

Alveolar soft-part sarcoma of the orbit.

An 11-month-old white female with a three-month history of swelling of soft tissue around the right eye presented with a proptotic globe and marked limitation of motility. A CT scan showed a soft-tissue mass in the orbit, which was biopsied and examined by light and electron microscopy. The specimen consisted of large polygonal and cuboidal cells of uniform appearance arranged in pseudo-alveoli around capillaries. The nuclei were pale and ovoid with prominent nucleoli and rare mitoses. The cytoplasm was packed with lipid droplets and the paranuclear region contained clusters of periodic acid-Schiff (PAS)-positive, diastase-resistant granules and needle-shaped crystals, which showed a linear periodicity of 100 nm by electron microscopy. These cytoplasmic crystals, which were diagnostic for alveolar soft-part sarcoma, were also found to be acid phosphatase-positive and, therefore, components of the lysosomal system. A lid-sparing exenteration of the right orbit was performed, and subsequent radiation therapy by cesium implant delivered 24,000 rads to the operative site. The patient is alive at 22 months following initial biopsy with no evidence of tumor recurrence.

Female↗
PubMed2000

Expression of the intermediate filament peripherin in extraskeletal myxoid chondrosarcoma.

The embryologic histogenesis of cartilage is not well characterized. While cranial cartilage is believed to be derived from pluripotential precursor cells of the neural crest, chondrocytes found elsewhere in the body are thought to be derived from mesoderm. As such, soft tissue tumors with cartilaginous differentiation may be related to neural crest or mesoderm. Peripherin is an intermediate filament encoded on chromosome 12, involved in growth and development of the peripheral nervous system. Peripherin is apparently expressed exclusively in cells derived from the neural crest and neural tube. A group of six soft tissue tumor types was selected because they are either of controversial differentiation or cytogenetically related to chromosome 12. A total of 41 cases was evaluated with antibodies against the intermediate filament peripherin. A panel of neural and neuroendocrine differentiation markers was used in selected cases. Three of five extraskeletal myxoid chondrosarcomas showed strong cytoplasmic reactivity with anti-peripherin. No peripherin expression was noted in any of eleven epithelioid sarcomas, eight liposarcomas, seven conventional chondrosarcomas, four neurothekeomas, three alveolar soft part sarcomas, or three clear cell sarcomas. The finding of peripherin expression in some extraskeletal myxoid chondrosarcomas may suggest the ability of some tumors to demonstrate both neural and chondroid differentiation.

Adolescent↗
PubMed2002

Chromosomal translocations and sarcomas.

This review examines how the identification of tumor-specific translocations and fusion proteins has advanced the basic scientific and clinical understanding of sarcomas. Recent genetic advances, including the ASPL-TFE3 fusion of alveolar soft part sarcoma, the JAZF1-JJAZ1 fusion of endometrial stromal sarcoma, and HMGIC fusions in liposarcoma, are discussed. Next, the review addresses the ways in which molecular genetic data have influenced diagnostic and prognostic paradigms. For example, recent studies describe the detection of occult tumor cells and the identification of primary renal neoplasms that are genetically related to alveolar soft part sarcoma. In addition, the review discusses potential therapies based on the targeting of sarcoma-specific fusion proteins. These reports describe the potential use of Gleevec (STI571) for dermatofibrosarcoma protuberans and the use of tumor-specific fusion proteins as potential targets for immunotherapy. Finally, basic scientific findings are reviewed that elucidate, for example, the aberrant functions of SYT-SSX in chromatin remodeling and of EWS-FLI1 in transcription and mRNA splicing. These and other emerging models of tumorigenesis will help identify new therapeutic targets.

Antineoplastic Agents↗
PubMed1993

B72.3 and CD34 immunoreactivity in malignant epithelioid soft tissue tumors. Adjuncts in the recognition of endothelial neoplasms.

The accurate immunohistochemical diagnosis of epithelioid angiosarcoma--as distinct from carcinomas and other epithelioid neoplasms--is hindered by the lack of endothelial-selective reagents that are both sensitive and specific. To evaluate the potential utility of an "epithelial-specific" and an "endothelial-selective" monoclonal antibody in this differential diagnosis, B72.3 [anti-TAG 72 (tumor-associated glycoprotein-72)] and My 10 [anti-CD34 (the hematopoietic progenitor cell antigen)] were applied to 14 examples of angiosarcoma (seven with an epithelioid phenotype), nine epithelioid hemangioendotheliomas, and eight capillary hemangiomas, together with antibodies to factor VIII-related antigen (F8rag) and cytokeratin (CK), and the lectin Ulex europaeus I (UEA). The results of these analyses were compared with stains fro B72.3, CD34, and Ck in a variety of other epithelioid soft tissue neoplasms, including four epithelioid sarcomas, three epithelioid malignant peripheral nerve sheath tumors, three alveolar soft part sarcomas, four clear cell sarcomas (malignant melanomas of soft parts), five malignant fibrous histiocytomas, and seven epithelioid leiomyosarcomas. The endothelial phenotype of each vascular tumor was supported by staining for either F8rag or UEA in all examples. Only angiosarcomas were reactive with B72.3, including five of seven epithelioid variants. None was positive for cytokeratin. Four epithelioid angiosarcomas were also reactive for CD34, but so were the majority of epithelioid leiomyosarcomas and at least one example each of all other epithelioid soft tissue tumors, except alveolar soft part sarcoma. In contrast, none of the latter tumor types was reactive with B72.3. These data indicate that anti-CD34 is not specific for endothelial tumors, whereas B72.3 reactivity is selective for epithelioid angiosarcomas. Despite published reports to the contrary, no tumor in this series was reactive for cytokeratin. An immunohistochemical panel that includes antibodies to both cytokeratin and B72.3, in addition to other lineage-selective mesenchymal markers, is useful in the diagnostic recognition of epithelioid soft tissue neoplasms.

Adolescent↗
PubMed1990

Breast sarcoma. A clinicopathologic review of 25 cases.

Sarcoma of the breast represents less than 1% of primary mammary malignancies; this study reports 25 such cases. The largest group had malignant fibrous histiocytoma (44%), followed by liposarcoma (24%) and fibrosarcoma (16%). Also represented were clear cell sarcoma, neurogenic sarcoma, leiomyosarcoma, and alveolar soft part sarcoma (4% each). Of 19 patients treated by wide local excision or simple mastectomy with or without adjuvant radiotherapy, 11 had local recurrence develop, of which one patient died and nine of the remaining ten had metastases develop. Of the remaining eight patients in this group with no local recurrence, only two had metastases develop. Of the six patients treated by radical or Patey mastectomy, none had local recurrence develop, but two died of metastases. No patient had metastases develop more than 5 years after diagnosis. Regional lymph node involvement with tumor was observed in only one patient (with malignant fibrous histiocytoma) despite regional lymphadenopathy in seven. The overall mortality at 5 years is 64% but does not increase thereafter. The authors' findings suggest that failure to establish local control is associated with a poor prognosis and that wide local excision or simple mastectomy does not provide sufficient clearance to be used as first-line treatment. Excision of the axillary lymphatics and adjuvant radiotherapy are unlikely to be beneficial.

Adult↗
PubMed2005

Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.

BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity. The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period. METHODS: Fifty-two patients age 8-18 years (median 8 years) were observed (1979-2002). Primary sites were on the lower limbs in 41 and on the upper limbs in 11 cases. Clinical TNM and surgical Intergroup Rhabdomyosarcoma Staging systems were adopted. Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy. Aggressive delayed surgery of residual disease, including compartmental resections, was recommended. Radiotherapy was suggested only for patients age > 3 years when surgery was incomplete or not feasible. RESULTS: The evaluation was performed dividing the patients into two categories according to their chemosensitivity. Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5. Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1. Nineteen of 21 patients with CTs-NRSTS were alive without disease: the 5-year overall survival (OS) and progression-free survival (PFS) were 94.4% and 79.3%, respectively; 23 of 31 patients with CTns-NRSTS were alive without disease: 5-year OS and PFS were 75.3% and 68.3%, respectively. Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors. The achievement of complete delayed resection was particularly important for CTns-NRSTS. Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor. CONCLUSIONS: Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS. Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread.

Adolescent↗
PubMed1995

p53 immunostaining in the differentiation of inflammatory pseudotumor from sarcoma involving the lung.

Inflammatory pseudotumor (IPT) of the lung is a non-neoplastic process that consists of proliferating spindle cells (fibroblasts and myoblasts), with variable numbers of mitoses, and inflammatory cells, particularly plasma cells. These lesions clinically, radiographically, and grossly mimic malignant neoplasms but are usually easily distinguished from malignancy on routine histopathology. However, in occasional cases the proliferating spindle cells may histopathologically mimic sarcoma, particularly on small biopsies and needle aspirates. Strong intranuclear immunopositivity for p53 protein is presumed to be indirect evidence of mutation of the p53 tumor suppressor gene and can be detected in many malignancies. In order to determine the utility of p53 immunostaining in differentiating IPT occurring in the lung from sarcoma involving the lung, we immunostained eight solitary IPTs, one IPT that recurred repeatedly over a 10-year period, six sarcomas (two malignant fibrous histiocytomas, two metastatic high-grade sarcomas, one metastatic alveolar soft part sarcoma, and one fibrosarcoma) involving the lung, and one IPT from which a sarcoma arose 10 years after radiation therapy. Immunohistochemistry was performed on 5-microns formalin-fixed sections using a commercially available antibody to the p53 protein (Biogenex, monoclonal 1:200) and a standard antigen retrieval technique. Weak intranuclear staining occurring in less than 10% of proliferating cells was not considered a true immunopositive. All eight of the solitary IPTs were immunonegative for p53 protein by our criteria. The IPT that recurred a number of times and the IPT from which a sarcoma later developed were also immunonegative for p53 protein. Four of the six sarcomas were immunopositive, as was the postradiation sarcoma arising from a p53-immunonegative IPT.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2005

Pediatric renal cell carcinomas with Xp11.2 rearrangements are immunoreactive for hMLH1 and hMSH2 proteins.

Alveolar soft part sarcoma and pediatric renal cell carcinoma share a similar chromosomal abnormality, t(X;17)(p11.2;q25). Recently, it has been suggested that the inactivation of DNA mismatch repair genes hMLH1 and hMSH2 may play an additional role in the pathogenesis of alveolar soft part sarcoma. Immunohistochemical expression of the proteins hMLH1 and hMSH2 is indicative of the activation status of the corresponding genes. We performed immunohistochemistry for hMLH1 and hMSH2 in 4 cases of pediatric renal cell carcinomas with Xp11.2 rearrangements. All cases showed nuclear immunoreactivity for both proteins, although the staining was patchy. Our study demonstrates that inactivation of the DNA mismatch repair genes hMLH1 and hMSH2 does not appear to play a role in the tumorigenesis of pediatric renal cell carcinomas with Xp11.2 rearrangements.

Adaptor Proteins, Signal Transducing↗
PubMed1986

Distribution of collagen type IV in soft tissue tumors. An immunohistochemical study.

The distribution of collagen type IV, one of the major constituents of basement membrane, was studied immunohistologically in a series of 103 soft tissue tumors including those of peripheral nerve origin, smooth muscle origin, striated muscle origin, fibrous tissue origin, fibrohistiocytic origin, adipose tissue origin, synovial tissue origin, and blood vessel origin, paragangliomas, alveolar soft part sarcomas, granular cell tumors, and epithelioid sarcomas. Intensely positive staining for collagen type IV was observed in neurilemomas, neurofibromas, malignant schwannomas, and blood vessel tumors. Weakly to moderately positive staining was seen in leiomyomas, angiomyomas, and leiomyosarcomas. In contrast, synovial, fibroblastic and fibrohistiocytic tumors, benign or malignant, were negative. In paragangliomas, granular cell tumors, and alveolar soft part sarcomas, positive staining was evident surrounding nests or clusters of tumor cells. In all tumors, staining for collagen type IV clearly illustrated the vascular pattern.

Collagen↗
PubMed2000

Lymphatic mapping with sentinel node biopsy in pediatric patients.

BACKGROUND/PURPOSE: Lymphatic mapping with sentinel node biopsy is used widely in adult melanoma and breast cancer to determine nodal status without the morbidity associated with elective lymph node dissection. This technique can be used in children to determine lymph node status with limited dissection and accurate interpretation. The authors report their initial experience. METHODS: The charts of patients who underwent lymphatic mapping with sentinel node biopsy were reviewed retrospectively. Lymphoscintigraphy was performed in patients with truncal lesions 24 hours before surgery to determine the draining nodal basin (for surgical mapping). The tumors were injected 1 hour preoperatively with technetium sulfur colloid and in the operating room with Lymphazurin blue. The draining basin was examined using a radioisotope detector. The blue nodes with high counts were localized and removed. If nodal metastases were identified, lymph node dissection was recommended. Four patients were injected only with Lymphazurin blue. RESULTS: Thirteen children (7 girls, 6 boys; mean age, 7 years) underwent lymphatic mapping with sentinel node biopsy. The tumor types were as follows: 8 malignant melanoma (6 extremity, 2 truncal), 1 malignant peripheral nerve sheath tumor, 1 alveolar soft part sarcoma, and 3 rhabdomyosarcoma. A mean of 2.4 nodes (range, 1 to 6) were removed from each patient. Six patients had a positive sentinel node. Formal lymph node dissection was performed on 4 of the 6 patients, 1 of whom had further nodal disease with 2 of 13 nodes containing micrometastases. One of the 6 patients refused lymph node dissection and adjuvant therapy; the final patient had rhabdomyosarcoma, a malignancy for which lymph node dissection is not indicated. Pulmonary metastasis developed 26 months after diagnoses in the patient with alveolar soft part sarcoma and a negative sentinel node. This patient was injected only with Lymphazurin blue at the time of sentinel node biopsy and refused adjuvant therapy. There have been no other recurrences. There were no complications related to lymphatic mapping or sentinel node biopsy. CONCLUSIONS: Lymphatic mapping with sentinel node biopsy, using both technetium-labeled sulfur colloid and Lymphazurin blue, can be performed safely in pediatric skin and soft tissue malignancies. Further study with long-term follow-up will determine the utility and accuracy of this technique in pediatric malignancies.

Biopsy↗