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Results for “Sarcoma, Alveolar Soft Part”

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PubMed1993

Immunophenotype and ultrastructure of alveolar soft part sarcoma.

Nine cases of alveolar soft part sarcoma were studied in terms of their immunoreactivity towards intermediate filaments and muscle markers, and their ultrastructure with regard to muscle features and the cytoplasmic crystals characteristic of this tumour. Inconsistent expression of alpha-smooth muscle actin, vimentin and desmin were found. No unambiguous muscle cell characteristics were identified by electron microscopy, although multiple subplasmalemmal densities with overlying lamina were emphasised as a typical but not specific muscle feature. Classical alveolar soft part sarcoma crystals at the ultrastructural level were absent throughout, but pseudo-crystals with a pre-crystalline finely filamentous content were numerous in one case. The results were integrated into a comprehensive comparison with the literature. It is clear that immunohistochemical evidence of striated muscle differentiation is detectable in some but not all cases of ASPS. The detection of Myo D1 protein in the small numbers of ASPSs so far studied for this gene product raises the possibility that in the majority of ASPSs this is the only evidence of early striated muscle differentiation and that only in the minority is it able to induce desmin production. Study of a substantial series for Myo D1 is required to explore this possibility.

Actins↗

PubMedDate not supplied

Crystal-deficient alveolar soft part sarcoma.

Classically, ultrastructural examination of alveolar soft part sarcoma reveals large, dramatic, rhomboid to needlelike crystals with a characteristic substructure. In this study of four cases of alveolar soft part sarcoma, only two exhibited large crystals, which were rare. All four cases, however, exhibited round, electron-dense granules, and in the two cases without large crystals these granules rarely exhibited elongation with the characteristic substructure of alveolar soft part sarcoma that permits definitive diagnosis. Two of these cases had been previously studied at other institutions, where crystals were not identified ultrastructurally and electron microscopy was considered noncontributory. Large crystals, then, may be rare or absent in alveolar soft part sarcoma. Careful search may be necessary to find granules with the characteristic periodic substructure.

Adult↗

PubMed1999

Immunohistochemical profile of myogenin and MyoD1 does not support skeletal muscle lineage in alveolar soft part sarcoma.

BACKGROUND: The histogenesis of alveolar soft part sarcoma remains elusive. Myogenic origin is favored, although conflicting data on immunohistochemical demonstration of muscle-associated markers exist. Myogenin and MyoD1, transcription factors of the myogenic determination family, have crucial roles in commitment and differentiation of mesenchymal progenitor cells to myogenic lineage and in maintenance of skeletal muscle phenotype. Their immunohistochemical detection is specific in characterization of rhabdomyosarcoma. METHODS: Antibodies for myogenin, MyoD1, desmin, and muscle-specific actin were employed on a large series of cases (n = 19) of formalin-fixed, paraffin-embedded alveolar soft part sarcoma. RESULTS: Minimal scattered nuclear staining was seen with myogenin. All cases had pronounced, nonspecific granular cytoplasmic immunostaining with MyoD1; nuclei were negative. All tumors were negative for desmin and muscle-specific actin. Ultrastructural study in 10 cases failed to reveal features of skeletal muscle differentiation. CONCLUSIONS: Cytoplasmic staining with MyoD1 in alveolar soft part sarcoma may correspond to cross-reactivity with an undetermined cytoplasmic antigen. The lack of immunostaining with myogenin, MyoD1, desmin, and muscle-specific actin provides evidence against a myogenic origin for alveolar soft part sarcoma.

Actins↗

PubMedDate not supplied

Alveolar soft part sarcoma of the tongue.

Alveolar soft part sarcoma (ASPS) is a rare malignancy; only 39 cases have been reported in the head and neck region. A 19-year-old woman is presented here who had ASPS of the tongue. Surgical resection was followed by irradiation therapy. She is tumor-free 2 years after treatment.

Adult↗

PubMedDate not supplied

Smooth tubular aggregates associated with plasmalemmal invagination in alveolar soft part sarcoma.

The ultrastructure of four alveolar soft part sarcomas was examined to search for ultrastructural signs of myogenic or neural origin. A new ultrastructural structure, an unusual tubular structure, was found in two of four cases. The structure appeared as a large, smooth, tubular aggregate in the cytoplasm of some tumor cells but did not show a honeycomb arrangement of tubules. The aggregate was composed of long, serpentine, branching, smooth, irregularly arranged tubules without ribosomes that ran in various directions. The aggregates intermingled with small amounts of cytoplasmic organelles. Because the aggregated tubules were at times continuous with cell membranes, it was shown that they were the complex extensions or invaginations of cell membranes. Neither myelin-axon complexes nor myofilaments, including Z band material, were seen in any case. There was a possibility that the smooth tubular aggregate was a T-tubule-like structure, suggesting that the tumors were derived from skeletal muscle cells.

Cell Membrane↗

PubMed1985

Alveolar soft part sarcoma of the uterine cervix.

Alveolar soft part sarcoma is a histologically distinctive neoplasm of uncertain histogenesis. Since its initial description in 1952, more than 200 cases have been reported. The extremities are most often the sites of involvement; the tongue, bones, and the orbit have been less commonly involved. The present paper describes a case of alveolar soft part sarcoma which was present only within the uterine cervix of a 37-year-old woman. Histologically, the tumor cells were arranged in the characteristic alveolar pattern; diagnostic PAS-positive diastase-resistant needle-shaped crystals were observed within the cytoplasm of the tumor cells. After the initial biopsy, the patient underwent a radical hysterectomy and pelvic lymph node dissection. Although no residual tumor was found within the cervix, a microscopic focus of tumor was detected in an obturator lymph node. The patient is at present clinically free of disease.

Adult↗

PubMed2002

Cytologic features of alveolar soft part sarcoma: report of three cases.

Alveolar soft part sarcoma (ASPS) is a rare, high-grade, epithelial-like sarcoma that shows characteristic histopathologic findings. Although a chromosomal anomaly that seems specific has been recently described, its diagnosis is based on histologic and ultrastructural features. The tumor shows no specific immunohistologic findings. Cytologic features of three cases of ASPS are presented. Preoperative fine-needle aspiration (FNA) of the primary soft tissue tumor was performed in two cases. In another two, mediastinal and pulmonary and subcutaneous metastatic lesions were aspirated. In all cases the cytologic image was identical with numerous, dissociated, large neoplastic cells with round-to-plasmocytoid morphology. Cytoplasmic fragility and granularity with abundant, atypical, naked nuclei were present. In one case, FNA material was available for ultrastructural studies. It disclosed the characteristic cytoplasmic crystalline structures. A specific cytologic diagnosis of ASPS was given in all cases. In conclusion, ASPS is a rare neoplastic entity that shows a characteristic cytologic image. When accompanied by an adequate clinical context it permits specific preoperative recognition. While immunocytologic studies are helpful to exclude other neoplasms, ultrastructure may result in an exact diagnosis.

Adult↗

PubMed2003

Chemotherapy in alveolar soft part sarcomas. What do we know?

Alveolar soft part sarcoma (ASPS) is a rare tumour. Published series about treatment and outcome are scarce. Conclusive data about the response to chemotherapy are not available. The aim of this study was to analyse the efficacy of palliative chemotherapeutic treatment options and the incidence and mode of presentation of brain metastases. We retrospectively analysed our own sarcoma data-base and reviewed the literature. From our registry containing 757 patients, we identified 8 patients with ASPS. From the literature, 47 cases of adult patients and 13 children with sufficient data about chemotherapy were identified. Response to first-line chemotherapy in 68 patients was: complete remission (CR) 4%, partial remission (PR) 3%, stable disease (SD) 41%, progressive disease (PD) 51%. 285 patients with stage IV disease were evaluable for the analysis of metastatic sites. The incidence of brain metastases was 30.5% (87/285). Brain metastases were detected at a median interval of 48 months (range 0-396 months) after the primary diagnosis. Median survival after the diagnosis of brain metastases was 12 months. The median survival for patients with stage IV disease treated by chemotherapy was 36+ months (range 10-132 months) (31 patients evaluable) with a median follow-up of 46 months (range 10-135 months). ASPS shows a high incidence of brain metastases, at least 3 times higher than that of other soft tissue sarcomas. Chemotherapeutic regimens used for the treatment of other soft tissue sarcomas lack efficacy in ASPS. Staging investigations for ASPS should routinely include imaging of the brain. ASPS patients should not be treated with chemotherapy outside of controlled clinical trials. New targets for specific biologically-directed therapies need to be developed.

Adult↗

PubMed2005

Alveolar soft part sarcoma: a rare and enigmatic entity.

UNLABELLED: Alveolar soft part sarcoma is a rare malignant tumor with unusual clinical behavior. Treatment of alveolar soft part sarcoma has been difficult to evaluate because of the small numbers of cases seen, but it seemed that although treatment of the primary tumor in alveolar soft part sarcoma often is successful, treatment of metastatic tumors is unsuccessful. A review of outcome after treatment of primary and metastatic disease in the 15 patients in our database with alveolar soft part sarcoma was done in order to evaluate this issue. Nine of 15 patients presented with metastatic disease and one further patient developed metastases. Treatment of primary tumors involved surgical excision in all but one patient and radiation in all patients. Adjuvant chemotherapy was administered to one patient with localized disease and to six patients with metastatic disease. There were no local recurrences. Treatment of metastatic tumors involved chemotherapy in seven patients, metastectomy in three patients, and radiation in two patients. All instances of the metastatic disease either recurred or progressed. Overall survival was 75% at 5 years and 40% at 10 years with a mean survival of 6.5 years, despite the high number of patients with metastatic disease. Current treatment results in good local control of primary tumors, but poor control of metastatic tumors. New approaches to treatment of metastatic alveolar soft part sarcoma must be investigated and applied. LEVEL OF EVIDENCE: Therapeutic study, Level IV-1 (case series). See the Guidelines for Authors for a complete description of levels of evidence.

Adolescent↗

PubMed1988

Alveolar soft part sarcoma. A cytologic and immunohistochemical study.

Alveolar soft part sarcoma is an uncommon soft tissue tumor that has seldom been studied by cytologic methods. The cytomorphologic features of two cases of this sarcoma are described. To enhance diagnostic accuracy in a suspected alveolar soft part sarcoma, the authors present the differential diagnosis and the application of immunocytochemical procedures to cytologic specimens.

Adult↗

PubMed1991

Alveolar soft part sarcoma of the pulmonary vein.

Alveolar soft part sarcoma of the lung seen in a 42-year-old female is reported. In the partial pneumonectomy specimen, there was a 3 x 2.5 cm tumor arising from the pulmonary vein at the level of the right lung hilus, with tumor thrombus formation. The transition between the tumor and venous smooth muscle layer was microscopically confirmed. At autopsy, performed 18 months after surgery, metastases were noted in the left lung and brain. No primary focus was identified in the soft tissue. The alveolus-forming clear tumor cells contained diastase-resistant periodic acid-Schiff-reactive granules. Immunohistochemically, granular cytoplasmic reactivities with monoclonal antibodies against pan-actin and alpha-sarcomeric actin were demonstrated, whereas other muscle markers such as desmin, alpha-smooth muscle actin, myoglobin, fast skeletal myosin, and the mm-isozyme of creatine kinase were negative. Ultrastructurally, crystallized structures were occasionally identified in the membrane-bound, electron lucent granules, which often filled the tumor cell cytoplasm. The muscle cell nature of the neoplasm is discussed.

Adult↗

PubMed1998

Alveolar soft part sarcoma.

We report a case of alveolar soft part sarcoma (ASPS) of the thigh with lung metastases in a 22-year-old woman. The findings of digital subtraction angiography (DSA) and MRI contributed to the diagnosis of ASPS. Especially dynamic contrast-enhanced MRI was useful for evaluating the effect of chemoembolotherapy.

Adult↗

PubMed2005

Alveolar soft part sarcoma of the head and neck.

Alveolar soft part sarcoma (ASPS) of the head and neck region has been a rarely reported entity. These lesions have a high propensity for distant metastasis. A retrospective study of the medical records at our institute, revealed thirty-eight cases of ASPS. Six of these were of primary head and neck origin. The article highlights the aggressive nature of the tumour and the need to arrive at a consensus on the treatment protocol.

Adolescent↗

PubMed2000

Alveolar soft part sarcoma: the role of prognostic markers.

Alveolar soft part sarcoma (ASPS) is a rare malignant neoplasm characterized by slow growth and indolent behavior. The role of proliferative markers and tumor suppressor genes is unknown in these tumors. To investigate their potential role in diagnosis and prognosis, we studied 13 cases of primary ASPS and 14 metastases and correlated them with clinicopathologic parameters. Immunohistochemistry was performed with anti-p53 and anti-Ki-67 antibodies. Polymerase chain reaction after tumor microdissection was performed to search for possible loss of heterozygosity in chromosomes 1p, 9p, 17q, 22q, and TP53 to identify possible changes that may clarify the histogenesis of these tumors. Four of five (80%) primary ASPS cases were positive for Ki-67 and all of them developed later metastases. One patient whose tumor did not stain for Ki-67 remained free of disease for 9 years. Eleven of 13 (85%) metastases were positive for Ki-67; however, there was no correlation with final outcome. All the primary ASPS cases analyzed for p53 yielded negative results, but two (15%) of 13 metastases were weakly positive. There was no correlation of these markers with prognosis or clinicopathologic parameters. No loss of heterozygosity was found except in one of nine (11%) informative metastases for D1S165 (at 1p36). Our preliminary data suggest that Ki-67-positive immunostaining may be a prognostic indicator for the development of metastases in ASPS.

Adolescent↗

PubMed1998

DNA copy number changes in alveolar soft part sarcoma: a comparative genomic hybridization study.

Alveolar soft part sarcoma (ASPS) is a rare, histologically distinctive soft tissue sarcoma typically occurring in children and young adults. Although the tumor often shows focal expression of muscle markers, its relationship with rhabdomyosarcoma is not established. The genetic background of ASPS is poorly understood. This study was undertaken to analyze the DNA copy number changes in 13 cases of ASPS using comparative genomic hybridization (CGH) on formaldehyde-fixed, paraffin-embedded tissue sections. Four ASPS cases showed DNA copy number changes. Gains were more common than losses. Gains observed in more than one case included 1q, 8q, 12q and 16p. Although these findings do not show consistent DNA copy number changes in ASPS, they give preliminary clues to genomic areas that might be important in the pathogenesis of ASPS.

Adolescent↗

PubMed1993

Alveolar soft part sarcoma of the tongue: a case report.

Alveolar soft part sarcoma of the tongue is a rare malignancy. Until 1988, only 23 cases have been documented in the English literature. In this report, a 21 year old man with typical features of alveolar soft part sarcoma of the tongue is presented. Treatment was wide surgical resection with reconstruction using a pectoralis major myocutaneous flap.

Adult↗

PubMedDate not supplied

Fine needle aspiration cytology and core biopsy in the diagnosis of alveolar soft part sarcoma presenting with lung metastases. A case report.

BACKGROUND: Alveolar soft part sarcoma is a rare soft tissue tumor of uncertain origin usually affecting young adults. This neoplasm has early metastatic potential. Its cytologic features, particularly when presenting with metastases, have rarely been described. CASE: A 23-year-old male presented with shortness of breath and scapular pain. Routine chest roentgenograms revealed multiple lung nodules. Malignancy was established by percutaneous fluoroscopically guided fine needle aspiration on a lung nodule. Possible metastatic alveolar soft part sarcoma was suggested by cytology among few considerations in the differential diagnosis. Alveolar soft part sarcoma was confirmed by lung core biopsy and further supported by immunohistochemistry and electron microscopy. Tumor cells expressed muscle-specific actin and myoglobin, and contained diastase-resistant inclusions with periodic acid-Schiff stain. Ultrastructurally, peculiar, elongated intracytoplasmic crystalline bodies typical of this neoplasm were identified. A meticulous clinical search led to finding the primary tumor deeply located in the right posterior thigh. CONCLUSION: Aspiration cytology is a reliable, cost-efficient technique in the diagnostic workup of masses suspicious for malignancy.

Adult↗

PubMed1999

Alveolar soft part sarcoma: a review and update.

Alveolar soft-part sarcoma (ASPS) is one of the most unusual of the soft tissue sarcomas. Although it most commonly arises in the fascial planes or skeletal muscles of the lower extremities in adults, and in the head and neck region in children, it has also been documented in extraskeletal muscle locations, especially the female genital tract. Despite extensive investigation, the histogenesis of ASPS continues to be an unsettled issue a half century after its description. The most significant morphologic features are the organoid pattern and the ultrastructural demonstration of a secretory activity that ends in the formation of the characteristic crystals. Early suggestions that it was related to paragangliomas or had a Schwann cell derivation have been discounted. More recently, a possible skeletal muscle origin has been favored based on the immunohistochemical demonstration of various muscle-associated proteins, especially desmin. However, a report of immunoreactivity for the myogenic regulatory protein MyoD1 has not been confirmed in subsequent studies. Although some immunohistochemical studies have recently indicated that ASPS may have muscle differentiation, there is presently no conclusive evidence that it represents a unique type of muscle-derived tumor.

Adolescent↗