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PubMedDate not supplied

Alveolar soft part sarcoma: an electron microscopic study especially of uncrystallized granules using a tannic acid-containing fixative.

Electron microscopic observation of alveolar soft part sarcoma reveals crystalline structure as well as granules that are only partially composed of crystalloids. The uncrystallized part has been observed only as a dense amorphous substance by the conventional fixation method with glutaraldehyde or osmium tetroxide (or both). Some investigators have explained the uncrystallized areas as artifacts due to the fixation method and as being essentially crystallized; other investigators consider the dense amorphous material the prodromal state of crystallization. In this study, however, which used the fixation method most appropriate for the intracytoplasmic filaments (fixation in tannic acid-containing glutaraldehyde and postfixation in osmium tetroxide) after increasing the permeability of the filaments to the fixative by pretreatment with a nonionic detergent (Triton X-100), the uncrystallized part was revealed to consist of an aggregation of many uncrystallized filaments with the same diameter (6 nm) as that of the crystallized filamentous structures. That is, the uncrystallized part was found to have been present as the prodromal state of crystallization and as filaments.

Adult↗
PubMed1985

[Alveolar soft part sarcomas. Apropos of 6 cases and review of the literature].

This is a clinicopathologic study of 6 cases of alveolar soft-part sarcoma. The patients age ranges from 8 to 47 years. All patients are women. In 5 cases the localization of the primary tumor is the lower extremities. Histologically the tumors have a distinctive organoid pattern outlined by thin-walled capillaries and are composed of nests of large polyhedral cells with abundant finely granular, acidophilic cytoplasm. Two tumors, among the 4 tested cases, have cytoplasmic PAS positive diastase-resistant crystalline structures. Electron microscopic study of one tumor shows intracytoplasmic glycogen, small membrane-bound electron-dense granules, Golgi lamellae and crystalline structures. Immunoperoxidase study performed in one case reveals a positivity with antikeratin and anti-enolase (NSE) antibodies. The prognosis of this tumor is poor. The main metastasis are in the lung and in the brain. Often the patients develop metastasis before detection of the primary tumor. The histogenesis of alveolar soft part sarcoma and the identity of the characteristic crystalloids remain open for discussion.

Adult↗
PubMed1994

Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass.

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.

Adult↗
PubMed1989

Alveolar soft part sarcoma. Assessment of immunohistochemical demonstration of desmin using paraffin sections and frozen sections.

The many different theories on the histogenesis of alveolar soft part sarcoma (ASPS) have caused great confusion. Owing to the recent rapid advance in immunohistochemical studies, two major hypotheses have been proposed. One group of researchers supports the idea that ASPS shows myogenic differentiation, while the other group opposes the idea. This confrontation is essentially one between a group that believes in the immunohistochemically demonstrated presence of desmin in ASPS and a group that denies it. In the present study we detected desmin in 6 of 10 formalin-fixed paraffin sections (although there were differences due to the use of five commercially available types of anti-desmin antibodies). When acetone-fixed paraffin sections and periodate-lysin-paraformaldehyde (PLP)-fixed frozen sections were used in one and three cases, respectively, they were found to be desmin positive, regardless of the type of antibody. The consistent positivity for all anti-desmin antibodies in the cases treated with acetone or PLP is very suggestive of a myogenous origin of ASPS. It is important to take into consideration the fact that formalin-fixed paraffin sections are not very suitable for immunohistochemical study of desmin.

Adolescent↗
PubMed1995

[A case of alveolar soft part sarcoma found by pulmonary metastasis].

A 34-year-old housewife presented to a hospital because of dry cough. Her chest radiograph showed bilateral multiple nodular lesions. Smaller but similar lesions had been seen on the chest radiograph 2 years earlier. Because the tissue taken during a trans bronchial biopsy was non-diagnostic, open lung biopsy was done and the diagnosis was pulmonary metastasis of alveolar soft part sarcoma. The primary tumor was found in her left calf by MRI. Malignant tumors are important for differential diagnosis of slow-growing multiple pulmonary nodules, and in some cases MRI is useful for finding the primary site.

Adult↗
PubMedDate not supplied

Non-myogenic tumors involving skeletal muscle. A survey with special reference to alveolar soft part sarcoma.

Skeletal muscle is remarkably free of neoplasms despite its great body mass. Metastatic tumors to skeletal muscle are distinctly uncommon. Primary myogenous tumors (defined as those whose cytoplasm contains myofibrils) are infrequent, compared to primary lesions of supporting stroma and vascular origin. Almost all non-myogenic tumors of striated muscle occur also in other areas of the body and many represent extension from contiguous structures. The distinctive non-myogenic tumor which occurs exclusively in skeletal muscle, alveolar soft part sarcoma, has ultrastrucutral features of a secretory lesion with distinctive membrane bound crystalloids which may be lipid in nature. The ultrastructural features of alveolar soft part sarcoma do not support a myogenous derivation.

Adult↗
PubMed1988

Alveolar soft part sarcoma. An immunohistochemical, cytologic and electron-microscopic study and a quantitative DNA analysis.

The type, differentiation and histogenesis of the tumor cells of alveolar soft part sarcoma (ASPS) have been analyzed in a series of ten cases by a light-microscopic, ultrastructural, immunohistochemical and cytologic investigation and quantitative DNA analysis. Four tumors deviated from ordinary ASPS: three were wholly or partly of the so-called pleomorphic variant of ASPS and a fourth tumor showed calcifications of the psammoma body type. The ultrastructural findings and immunohistochemical demonstration of desmin supported the hypothesis of a rhabdomyomatous differentiation and gave no support to epithelial (negative immunoreactions for cytokeratins, epithelial membrane antigen, HMFG-1 and -2, tissue polypeptide antigen (TPA] or neuroectodermal (negative for S-100 protein, glial fibrillary acidic protein, neurofilaments) differentiation. The negative immunoreactions for vimentin and myoglobin and the positive reaction for neuron specific enolase (NSE) do not exclude a rhabdomyomatous differentiation since in rhabdomyosarcomas the undifferentiated rhabdomyoblasts generally contain vimentin and the differentiated tumor cells contain myoglobin and rhabdomyosarcoma has previously been reported as being positive for NSE. The production of external lamina material peripherally in the tumor cell nests and around vessels in the vascular septa was demonstrated both ultrastructurally and by immunohistochemistry using antibodies against collagen IV and laminin. The cytologic appearance in smears obtained by fine-needle aspiration from a case of the pleomorphic variant showed some resemblance to that of a carcinoma. The seven tumors with an ordinary cell appearance were found to show a diploid DNA-distribution at a quantitative analysis performed on paraffin sections, while the three tumors wholly or partly of the pleomorphic type showed an additional tetraploid peak.

Adolescent↗
PubMed2003

Alveolar soft part sarcoma in Japan: multi-institutional study of 57 patients from the Japanese Musculoskeletal Oncology Group.

OBJECTIVE: The clinical features and the management of alveolar soft part sarcoma (ASPS) are not well known. The efficacy of chemotherapy for soft tissue sarcoma, including high-dose ifosfamide and cisplatin, has not been established yet. Some reports suggest ASPS may occur primarily in bone. METHODS: We report on a series of 57 patients with ASPS over 20 years. Their ages ranged from 7 to 75 years (mean 25). RESULTS: There were 37 females and 20 males. Thirteen lesions (23%) showed bone involvement at the primary site, and 6 of them were diagnosed as bone tumors at presentation. Thirty-seven patients had distant metastases at presentation. Tumor size, bone involvement at the primary site and the presence of metastases at presentation were prognostic indicators (p < 0.05). Marginal excision with radiotherapy or wide excision without radiotherapy achieved good local control. Chemotherapy was performed in 47 patients with different regimens. Two patients treated with intra-arterial chemotherapy regimens responded partially, but intravenous chemotherapy with high-dose ifosfamide or cisplatin failed. CONCLUSIONS: ASPS can present primarily as a bone tumor. No advantage of chemotherapy with high-dose ifosfamide or cisplatin could be demonstrated.

Adolescent↗
PubMed2002

Alveolar soft part sarcoma. a report of 15 cases.

The aim of this study was to evaluate the presentation, course and treatment outcome of 15 patients with this rare type of sarcoma. The files of the patients were retrospectively analysed. Overall survival was calculated according to the Kaplan-Meier method. There were 15 patients, 8 male and 7 female. The mean age at diagnosis was 29 years for men and 24 years for women. The median survival was 48 months with an overall 5-year survival of 38%. 5 patients had haematogenic metastases at the time of diagnosis. For the remaining 10 patients with localised disease, the median survival was 48 months and the 5-year survival 48%. The median disease-free survival for these patients was 12 months with a 5-year disease free survival of 40%. After the occurrence of haematogenic metastases, patients survived a median period of 8 months (range 0-45 months). 5 patients are still free of disease after a median period of 234 months (12-295 months). Alveolar soft part sarcoma is found especially in young adults. When diagnosed, it is often metastasised with a poor prognosis. However, when radically resected, long-term survival is possible.

Adolescent↗
PubMed1984

Alveolar soft part sarcoma. An elaboration of a three-dimensional configuration of the crystalloids by digital image processing.

As an initial step to elucidate the nature of the unique characteristics of the crystalloids of alveolar soft part sarcoma, a three-dimensional model of the crystalloids was prepared by digital image analysis of electron micrographs by computer. It was revealed that occult periodicities are present at two intervals, 60 A and 380 A, in the filamentous structure of the crystalloid; and it was also revealed by the observation of each cross-section that two globular substances with a diameter of 60 A are arranged in a dumbbell pattern in each filamentous structure. The model prepared based on these data showed the double strands crossing each other at intervals of 380 A, each of which consists of successive arrangement of the globular substances with a diameter of 60 A. This structure is clearly similar to that of actin. The similarities and differences between these results and the well-known studies of the organization of naturally occurring actin bundles are discussed.

Adult↗
PubMed1977

Alveolar soft part sarcoma. Report of a case.

In 1967 an 18-year-old woman was operated on because of a tumour in her right thigh, of which she had been aware for one year. Histological examination led to a diagnosis of alveolar soft part sarcoma. Re-excision of the area was performed but not amputation of the extremity. Ten years later the woman is in good health. She is married and has two children. The patient must be kept under observation for years and should local recurrence arise, amputation will probably be necessary.

Adolescent↗
PubMed1991

Alveolar soft part sarcoma: an analysis of 8 cases. Review of the literature.

Eight cases of alveolar soft part carcinoma (ASPS) are presented. The average age of the patients was 28 years. There were 6 women and 2 men. The left sided and right sided tumours were equal in number. Local recurrence was not observed in any of the patient treated, but lung metastases occurred in 6 of them. Three patients died with disease the average 35 months after treatment. Histologically in one case the uniform sheets of cells were observed without alveolar arrangement. The presence of beta glucuronidase (BG), acid phosphatase (AP) and non specific esterase (NE) in tumor cells was seen in one case studied. Immunohistochemical and ultrastructural examination confirm the thesis, that ASPS consist a distinct clinico-morphologic entity, the histogenesis of which remains uncertain.

Adolescent↗
PubMedDate not supplied

Membrane-bound cytoplasmic crystals, similar to those in alveolar soft part sarcoma, in a human muscle spindle.

Membrane-bound cytoplasmic crystals were found in the intrafusal fibers of a muscle spindle from a patient with neurogenic atrophy. The crystals have a periodicity of 10 nm and an intersecting axis angle of approximately 80 degrees. This makes the crystals similar to those described in alveolar soft part sarcoma (ASPS). Because the crystals in ASPS may not be quite as specific as previously believed, and because similar crystals have been described in various other neoplasms, the present findings should not be taken as evidence for a muscle spindle derivation for ASPS.

Crystallization↗
PubMedDate not supplied

Alveolar soft part sarcoma. A case report with correlation of fine needle aspiration and ultrastructural cytologic features.

Tissue from an alveolar soft part.sarcoma (ASPS) was studied, and a comparison was made between the morphologic features found on light microscopy histology, fine needle aspiration (FNA) cytology and electron microscopy. The characteristic findings seen in FNA of ASPS (alveolar aggregates, capillary networks, eccentric nuclei with prominent nucleoli, intranuclear vacuoles, cytoplasmic zonation, intracellular and exocytosed magenta granules, sharply defined and thickened cell border) are supported by the ultrastructural findings and, although not totally specific, can serve as sensitive markers in order to raise the suspicion of this rare neoplasm in the appropriate clinical setting. A differential diagnostic discussion is included, supporting the above conclusion.

Adolescent↗
PubMed2000

APC and beta-catenin in alveolar soft part sarcoma (ASPS)--immunohistochemical and molecular genetic analysis.

Apart from its role in cell-adhesion, beta-catenin is regarded as an oncoprotein, the cytoplasmic level of which is regulated by APC as a tumor suppressor protein. Changes of chromosome 5q, the region that includes the APC-gene, are known to be important in the pathogenesis of fibromatosis; however, little is known about the significance of APC and beta-catenin in other mesenchymal tumors. Therefore, we used immunohistochemistry and DNA-analysis to investigate four cases of alveolar soft-part sarcoma (ASPS) as a mesenchymal tumor with a distinct histologic appearance. In three cases of ASPS the APC-gene product was found to have strong nuclear expression and only faint cytoplasmic staining. Beta-catenin showed a partly membranous, partly strong intracytoplasmic expression. No gene mutations for APC and beta-catenin were detected in any of the four cases. These investigations suggest that, apart from their function in carcinogenesis and fibromatoses, APC and beta-catenin play a role in the pathogenesis of soft tissue tumors such as ASPS. The significance of a striking nuclear accumulation of non-mutated, virtually functionally active APC-tumor suppressor protein has not yet been investigated. A nuclear function of APC in ASPS in down-regulating nuclear transcription processes linked to overexpression of beta-catenin, as is known in colorectal carcinogenesis, may be hypothesized.

Adenomatous Polyposis Coli Protein↗
PubMed2006

Sarcomas often express constitutive nitric oxide synthases (NOS) but infrequently inducible NOS.

Nitric oxide (NO) has a dual action in tumors, with both pro-tumor and anti-tumor activities. NO is produced by nitric oxide synthases (NOS). There are three enzyme isoforms: two of them are constitutively produced (neuronal or brain NOS and endothelial NOS), and one is an inducible form (iNOS). NOS expression has been shown in several epithelial tumors, but there is no report addressing NOS expression in sarcomas. The authors evaluated the expression of NOS in 97 cases of various sarcomas spotted in duplicate in a tissue array paraffin block. Eighty-four of the 97 tumor specimens (86.6%) expressed nNOS, and most of them showed a strong expression of the isoenzyme. Only chondrosarcomas and liposarcomas had significant numbers of negative cases, and all pleomorphic sarcomas, alveolar soft part sarcomas, angiosarcomas, gastrointestinal stromal tumors, and synovial sarcomas showed some degree of positivity. Forty-three cases (44.4%) showed eNOS immunostaining, but only 15.5% showed a strong signal, with emphasis on angiosarcomas, chondrosarcomas, alveolar soft part sarcomas, and synovial sarcoma. Strong expression of iNOS was observed in only 9 cases (9.3%), with weak expression in another 26 cases (26.8%). Strong expression of iNOS was found in malignant peripheral nerve sheet tumors, liposarcomas, pleomorphic sarcomas, fibrosarcomas, chondrosarcomas, and synovial sarcomas. Apparently alveolar soft part sarcomas are unusual in their capacity of expression of NOS isoforms, and in a very peculiar pattern. In conclusion, sarcomas in general commonly express constitutive NOS, and only a few types of sarcomas can express iNOS, the isoenzymes capable of releasing large amounts of NO. More comprehensive studies should be performed to better understand the clinical importance of NOS expression and NO production in sarcomas.

Humans↗