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 289 records · Page 16Linked to original sources
PubMed1989

Orbital nonchromaffin paraganglioma. A case report and review of the literature.

Nonchromaffin paraganglioma (NCP), also called glomus body tumor or chemodectoma, is rarely found in the orbit. The behavior of orbital nonchromaffin paraganglioma may potentially be more aggressive than in other head and neck locations. Diagnosis depends on electron microscopic demonstration of membrane-bound neurosecretory granules. Results of histopathologic study show a well-circumscribed lesion without a true capsule with alveolar or organoid arrangements of epithelioid cells within a reticulin framework with thin-walled blood vessels. Cells are polygonal with round or oval nuclei containing rare mitotic figures and pale-staining cytoplasm. Differential diagnosis includes alveolar soft-part sarcoma, alveolar rhabdomyosarcoma, neuroblastoma, carcinoid, and granular cell tumor. Of 29 previously reported cases of orbital NCP, 16 have been reclassified as alveolar soft-part sarcoma. The authors report a patient with an electron microscopically established orbital NCP, with the history of a contralateral glomus jugulare tumor irradiated 14 years previously.

Adult↗
PubMedDate not supplied

Alveolar soft-part sarcoma of the orbit.

A 10-year-old girl with gradual-onset proptosis in her left eye was diagnosed with orbital alveolar soft-part sarcoma. The tumor and the contiguous medial rectus muscle in the left eye were excised and compensatory strabismus procedures were performed. Five years postoperatively, she has central binocular vision with no evidence of tumor recurrence.

Child↗
PubMed1991

Alveolar soft-part sarcoma of the uterine corpus: histological, immunocytochemical and ultrastructural study of a case.

A case of alveolar soft-part sarcoma located in the uterine corpus is reported. It was an incidental finding in the hysterectomy specimen of a 40-year-old woman. Light and electron microscopic examination revealed periodic-acid-Schiff-positive, diastase-resistant, membrane-bound cytoplasmic granules and crystalloids. Tumour cells expressed immunoreactivity with vimentin, desmin, cytokeratins, NK1/C3 and HMB-45 antibodies. Four years postoperatively, the patient is still alive without evidence of disease. Differential diagnoses, immunocytochemistry and clinical management of uterine alveolar soft-part sarcoma are discussed and the literature reviewed.

Adult↗
PubMed1981

[A case of alveolar soft-part sarcoma with lung and cerebral metastasis (author's transl)].

A 37-year-old housewife who had episodes of surgical removal for an alveolar soft-part sarcoma of her right leg 15 years ago, and total removal for right lung metastasis 2 years ago, was admitted to our service with complaints of headache, vomiting, dyscalculia and recent memory disturbance which started about 4 months prior to admission. Neurological examination revealed papilledema and disorientation. Left carotid angiography and CT scan showed a ball-like vascular tumor in the left frontal lobe. Presumptive diagnosis of metastatic brain tumor in the left frontal lobe was made in our service. Left fronto-parietal craniotomy was carried out on Feb. 22, 1977 and total removal for the metastatic tumor were made. Specimens of the tumor showed typical feature of alveolar soft-part sarcoma histologically. Postoperative course was well and she was discharged without neurological deficits. Two years after the previous surgery for cerebral metastasis, another metastatic tumor was found in the left frontal lobe again. Total removal for this solitary metastatic tumor was performed again. Histological and electron microscopical examination was made with this tumor specimen. Typical feature of alveolar soft-part sarcoma was confirmed. She was discharged with a marked improvement of her symptoms. At present, it has been passed about a year after the second surgery on her, she is doing well without neurological deficits. Clinical features of alveolar soft-part sarcoma were discussed, especially with the comparison of clinical feature between the reported cases of Japan and the cases of foreign countries. Regarding the choice of the treatment of this tumor, total removal was seemed to be the best management. Therefore, all efforts should be focussed to discover in the earlier stage of this tumor.

Adult↗
PubMed1985

Alveolar soft-part sarcoma. A study of 13 typical examples and one with a histologically atypical component.

Thirteen cases of typical alveolar soft-part sarcoma and one in which the tumor had a histologically atypical component are presented. The atypical zone in the latter case was characterized by increased nuclear hyperchromatism and pleomorphism, increased nuclear-cytoplasmic ratio, decreased cytoplasmic eosinophilia and granularity, a less distinct "nesting" pattern, and more numerous mitotic figures. The tumors occurred almost entirely in the younger decades and involved a variety of soft tissue sites. Sex distribution was 1:1, and there was a slight left-sided predominance in tumor location. Median survival of the patients with typical alveolar soft-part sarcoma was 79 months (the patient with the atypical neoplasm died at 36 months). The major factor related to both survival and the likelihood of metastasis was tumor size.

Adolescent↗
PubMed2001

Cytogenetic abnormalities of alveolar soft-part sarcomas using interphase fluorescent in situ hybridization: trisomy for chromosome 7 and monosomy for chromosomes 8 and 18 seem to be characteristic of the tumor.

Four alveolar soft-part sarcomas were investigated by means of standard immunohistochemistry and interphase cytogenetics to further characterize the immunophenotype and proliferative activity of this tumor. The main goal of this study was to explore the chromosomal changes of this rare soft-tissue sarcoma. One epithelial (KLI), three neurogenic [neuron specific enolase (NSE), PGP 9.5, and S100], and five myogenic (desmin, myoglobin, alpha-smooth mnuscle actin, alpha-sarcomeric actin, and MyoD1) markers were used for the immunophenotypical analysis. Proliferative activity was assessed using the Ki67 index. Twelve (peri)centromeric (1, 3, 4, 6, 7, 8, 10, 12, 15, 17, 18, and X) and one telomeric (17q25-qtel.) chromosomal probes were used for interphase cytogenetic analysis. Three of the cases showed cytoplasmic desmin and/or myoglobin, and one showed smooth muscle actin positivity. All of the four tumors had granular, cytoplasmic, possibly nonspecific MyoD1 and sarcomeric actin positivity. Two of the tumors were positive for vimentin, four gave focal and weak staining with neurogenic markers (four of four NSE, one of four S100, and four of four PGP 9.5), but none of them was positive with KLI. Alveolar soft-part sarcomas may show myogenic immunophenotype in a number of cases, which supports myogenic differentiation. Fluorescent in situ hybridization using alpha satellite chromosomal probes revealed significant alterations in all of the cases. Most frequent and repeated numerical changes, which seem to be characteristic of the neoplasm and may play an important part in its pathogenesis and/or progression, were trisomy 7, monosomy 8 and monosomy 18.

Adolescent↗
PubMed1996

Does alveolar soft-part sarcoma exhibit skeletal muscle differentiation? An immunocytochemical and biochemical study of myogenic regulatory protein expression.

There has been persistent controversy regarding the nature of cell differentiation in alveolar soft-part sarcoma (ASPS) since its first description in 1952. Some studies suggest that ASPS might represent an unusual variant of skeletal muscle tumor. Given the availability of new monoclonal antibodies to probe for skeletal muscle differentiation and the rapid advance in immunocytochemical techniques for deparaffinized, formalin-fixed tissue sections, we wished to test the proposed hypothesis that ASPS might represent a new type of rhabdomyosarcoma. Twelve archival samples of ASPS were retrieved, and we investigated the expression of two myogenic regulatory proteins, MyoD1 and myogenin, as well as other muscle-associated proteins, using sensitive immunocytochemical techniques. Despite the presence of desmin immunostaining in six ASPSs, no tumors were positive for either muscle actin or myoglobin. Most importantly, no specimen showed nuclear expression of MyoD1 or myogenin. In 11 tumors, however, there was considerable granular immunostaining in the tumor cell cytoplasm with the anti-MyoD1 monoclonal antibody 5.8A, a phenomenon observed in various nonmuscle normal and neoplastic tissues with this antibody. To analyze the exact nature of immunostaining of MyoD1 and desmin in ASPS, biochemical analyses using available fresh frozen tumor tissue were performed. Although a 53-kDa band was noted with antidesmin antibody on Western blot analysis, no specific protein band that corresponds to the 45-kDa MyoD1 was detected with antibody 5.8A. These results confirm the presence of desmin in ASPS but argue against authentic expression of MyoD1. They also suggest that the cytoplasmic immunostaining observed with anti-MyoD1 antibody 5.8A most likely represents a nonspecific cross-reaction with an unknown cytoplasmic antigen. Considering the master role that MyoD1 and myogenin play in skeletal muscle commitment and differentiation and the lack of expression of these two proteins in ASPS as determined immunocytochemically and biochemically, we think that the histogenesis of ASPS remains unknown.

Adolescent↗
PubMed1991

Multiple and complex abnormalities in a case of alveolar soft-part sarcoma.

Chromosomal analysis of two pulmonary metastases from a 25-year-old male with alveolar soft-part sarcoma of the right lower extremity revealed multiple and complex chromosomal abnormalities. The rearrangements included deletions and translocations affecting chromosomes 1, 2, 3, 10, 11, 14, 15, and 16. Trisomy of chromosome 12 and loss of chromosome 17 was also observed.

Adult↗
PubMed2003

Alveolar soft-part sarcoma responding to interferon alpha-2b.

A 23-year-old woman with an alveolar soft-part sarcoma of her calf with pulmonary metastases unresponsive to chemotherapy is described. Interferon (IFN) alpha-2b induced an impressive tumour response still ongoing after IFN treatment had to be stopped because of a psychosis. An explanation of this effect is still speculative.

Adult↗
PubMed1993

Alveolar soft-part sarcoma. A report of ten cases.

The clinical and pathologic features of ten cases of alveolar soft-part sarcoma (ASPS) were observed in subjects aged ten to 49 years. Eight were male and two were female. The lesion involved the thigh in six cases and the adjacent bone in three. All lesions were retrospectively classified as high risk (Stage II or III of Enneking's staging system), and distant metastasis to lung or bone was noted at initial presentation in four patients. Three patients died with distant metastases at a mean time of three years seven months after diagnosis. The mean interval from initial presentation to detection of distant metastasis was nine months; and the mean interval from detection of metastasis to death was three years two months. Ultrastructural studies demonstrated typical organoid arrangement of the neoplastic cells surrounded by a basement membrane with close attachment of individual cells to the basal lamina. No local recurrence, even in cases with inadequate tumor control, and the tendency for distant metastasis demonstrated in the current series, reemphasized that management of metastasis is most important in the treatment of ASPS.

Adolescent↗
PubMedDate not supplied

Fine needle aspiration cytology of alveolar soft-part sarcoma. A case report.

Transthoracic fine needle aspiration was performed on pulmonary nodules of unknown etiology in a 22-year-old male. Cytologic examination of variously stained smears of the aspirate suggested a metastatic tumor, consistent with alveolar soft-part sarcoma. This diagnosis was confirmed by subsequent histologic examination of an excised thigh mass, which revealed alveolar soft-part sarcoma with an associated arteriovenous malformation. The cytologic findings of alveolar soft-part sarcoma are discussed along with a brief review of this uncommon neoplasm.

Adult↗
PubMed1982

Alveolar soft-part sarcoma--A malignant angioreninoma: Histochemical, immunocytochemical, and electron-microscopic study of four cases.

This combined ultrastructural and immunohistochemical study of four alveolar soft-part sarcomas analyzes the characteristic granules in order to elucidate the histogenesis of this unusual neoplasm. The granules have the ultrastructural features and staining characteristics of renin, react with antiserum to renin, and like renin, incorporate zinc into the crystalline storage granules. The cell appears to be a modified smooth muscle cell analogous to renal juxtaglomerular cells and the cells of juxtaglomerular cell tumors. As there is no association with hypertension, it would appear that alveolar soft-part sarcomas either do not secrete or the product is a form of renin that does not act upon the angiotensin system.

Adult↗
PubMed1996

Diagnostic value of electron microscopy in rare malignant musculoskeletal tumors. Experience from an orthopedic tumor center.

Rare musculoskeletal tumors can be difficult to diagnose by light microscopy or immunohistochemistry. Electron microscopy can be of diagnostic assistance especially if histotype specific ultrastructural features exist. In particular, electron microscopy for uncommon sarcomas such as alveolar soft part sarcoma, parachordoma, atypical Ewing's sarcoma and epithelioid sarcoma may be the diagnostic modality of choice.

Adult↗
PubMed1979

The ultrasonographic and radiologic features of a histologically proven case of alveolar soft-part sarcoma of the orbit.

An 18-year-old woman had a rapidly progressive proptosis, vascular congestion, and orbital pain. Ultrasonographic and radiologic tests supported the diagnosis of a solid vascularized mass that proved histologically to be an alveolar soft-part sarcoma of the orbit. Uncertainty as to the histogenesis of alveolar soft-part sarcoma still exists, but prognosis for patient survival is poor.

Adolescent↗
PubMed2005

Brain metastasis in pediatric extracranial solid tumors: survey and literature review.

OBJECTIVES: Brain is a rare site of metastasis in most extracranial pediatric solid tumors. The aim of this study is to investigate the incidence, treatment, prognosis of brain metastasis in extracranial pediatric malignant tumors in a single institution and to review the literature. METHODS: From September 1989 to December 2002, 1100 children <or=16 years of age with extracranial solid tumors including lymphomas were diagnosed and treated in the Division of Pediatric Oncology, Oncology Institute, Istanbul University. Patients with parenchymal metastases in the brain were assessed. RESULTS: Sixteen (10 female, 6 male) of 1100 patients (1.45%) with extracranial solid tumors developed brain metastases. The median age of the patients was 10.5 (1-16) years. The diagnosis was sarcomas in 12 patients: 5 osteosarcomas, 4 Ewing's sarcoma family tumors, 1 rhabdomyosarcoma, 1 clear cell sarcoma of the soft tissue, 1 alveolar soft part sarcoma. Two patients had Wilms' tumor and two had germ cell tumors. Four patients (25%) had brain metastasis at diagnosis. Twelve (75%) developed brain metastasis during therapy or relapse at a median duration of 16 (1-70) months from initial diagnosis. All patients had metastases to various sites, mostly lung, at the time the brain metastases were detected. Treatment included surgery, followed by postoperative radiotherapy (RT) and chemotherapy (CT) in 1, S and RT in 1, S in 1, RT and CT in 6, RT in 1, CT in 1 and no treatment in 5. Only one patient with alveolar soft part sarcoma is alive with disease 20 months from diagnosis of brain metastasis. All other patients died at a median time of 2 months (2 days-6 months) from the time of brain metastasis. CONCLUSIONS: Children with metastatic cancer who develop headaches or any other neurologic symptom should be investigated for possible brain metastasis. Although, the outcome for these patients is dismal in this series and in the literature; reports of long term survival in a few cases with Wilms' tumor, osteosarcoma and alveolar soft part sarcoma who had isolated brain metastasis, suggest that a subset of patients may benefit from therapy.

Adolescent↗
PubMed1976

Alveolar soft-part sarcoma in the oral cavity: report of a case.

This case presents a rare tumor of the oral cavity--an alveolar soft-part sarcoma. Difficulties developed in the diagnosis, and therefore, consultations were required. Because of the radioresistant qualities of the lesion, wide surgical excision was performed. The patient's condition has been followed for 40 months without evidence of recurrence. The conclusion arrived at from review of the literature is that this is an indolent but malignant tumor and has so far proved to be 100% fatal. However, it may be 20 years before it proves fatal.

Child, Preschool↗
PubMed1998

Expressions of basic fibroblast growth factor and fibroblast growth factor receptor mRNA in soft tissue tumors by in situ hybridization.

Basic fibroblast growth factor (bFGF) is a mitogenic and angiogenic polypeptide produced by diverse cell types, including the cells derived from normal tissue and neoplastic lesions. To evaluate the possible differential expression of bFGF in soft tissue tumors, we examined 17 frozen primary soft tissue tumors including extra-abdominal desmoids, leiomyosarcomas, synovial sarcomas, myxoid liposarcomas, and alveolar soft part sarcomas by nucleic acid in situ hybridization. Three of the four leiomyosarcomas and two of the three synovial sarcomas studied expressed bFGF mRNA, whereas all of the myxoid liposarcomas and alveolar soft part sarcomas were negative. Two of the leiomyosarcomas were also positive for FGF receptor mRNA. These results indicated the possibility of an autocrine or paracrine mechanism in the function of bFGF and also lent support to the opinion that in situ hybridization is useful for examining cell character.

Adolescent↗