Alveolar soft part sarcoma: review of nine cases including two cases with unusual histology

Jong, Roland; Kandel, Rita A.; Fornasier, Victor; Bell, Robert S.; Bédard, Yvan C.

doi:10.1046/j.1365-2559.1998.00322.x

Cited by 32 publications

(12 citation statements)

References 22 publications

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“…4,20 For example, the patient with ASPS in his renal hilum (Table 1, case 12) was initially diagnosed as presenting with paraganglioma, on the basis of morphologic feature and location. However, in many cases, the diagnosis of ASPS can become an issue when tumors lack the typical morphologic patterns, especially the tumors occurring in rare locations.…”

Section: Discussionmentioning

confidence: 99%

Alveolar Soft Part Sarcoma: A Bimarker Diagnostic Strategy Using TFE3 Immunoassay and ASPL-TFE3 Fusion Transcripts in Paraffin-Embedded Tumor Tissues

Pang

Chang²,

Zou³

et al. 2008

Diagnostic Molecular Pathology

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Alveolar soft part sarcoma (ASPS) is a malignancy with low incidence, but with poor prognosis if misdiagnosed. Immunohistochemical assay using TFE3 antibody has been shown to be a sensitive technique for ASPS diagnosis. A specific chromosomal translocation, t(X;17)(p11.2;q25), results in the ASPL-TFE3 fusion gene: it is detectable using reverse-transcription polymerase chain reaction (RT-PCR) in frozen tumor tissues of ASPS. However, the diagnostic usefulness of these markers has not been investigated in Chinese ASPS patients. Here, we report the first systematic study applying TFE3 immunoassay and ASPL-TFE3 fusion transcript detection to archival paraffin-embedded tissues in a large Chinese ASPS patient population. Sixteen patients had been diagnosed with ASPS (age, 3 to 58 y; 3 male patients and 13 female patients). Their tumors presented predominantly in the extremities (8/16), and were often located in the region of the orbit when affecting infants and children (3/16). Others had tumors in the chest wall, breast, and right pubis, respectively. One patient exhibited a tumor in the renal hilum, a location that had not been previously reported. Two patients had tumor metastases in the lung and the brain. ASPS tumors showed the best immunoreactivity to the TFE3 antibody (16/16). However, their immunoreactivity to other antibodies, including myoglobin (13/16), actin (10/16), desmin (2/16), and vimentin (2/16), was of various degrees. Positive staining was observed for the neural markers, NSE (9/16) and CgA (7/16), respectively. Using a strategy of RT-PCR, followed by a nested PCR with a different primer set, we were able to detect the expression of the chimeric ASPL-TFE3 mRNA in 11 of the 16 ASPS tumors. Of these 11, 7were type 1 ASPL-TFE3 and 4 were type 2 ASPL-TFE3, including the tumor located in the renal hilum. No expression of ASPL-TFE3 fusion transcripts was detectable in all 38 control tumors. Our results demonstrate that the ''bimarker strategy,'' a combination of TFE3 immunostaining and ASPL-TFE3 chimeric transcript detection, might have sufficient sensitivity and specificity in diagnosing most of the ASPSs. As both diagnostic techniques can be applied to widely available archival paraffinembedded tissues, the usefulness of the strategy is largely implicated in routine pathology laboratories.

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Section: Discussionmentioning

confidence: 99%

Alveolar Soft Part Sarcoma: A Bimarker Diagnostic Strategy Using TFE3 Immunoassay and ASPL-TFE3 Fusion Transcripts in Paraffin-Embedded Tumor Tissues

Pang

Chang²,

Zou³

et al. 2008

Diagnostic Molecular Pathology

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“…Evans some years ago was one of the first to draw attention to the ''atypical'' histologic features, which included nuclear hyperchromatism, pleomorphism, vague nesting or ''alveolar'' pattern, an attenuated eosinophilic cytoplasm, and increased mitotic activity [20]. More recently, Jong and associates described 2 examples of ASPS, one of which had pseudoglandular profiles and the other had a spindle cell pattern [21]. Our case had several uncommon and even unusual features, including a diffuse, predominantly nonalveolar or nonorganoid pattern of formless sheets of cells.…”

Section: Discussionmentioning

confidence: 99%

Primary Cardiac Alveolar Soft Part Sarcoma. A Report of the First Observed Case with Molecular Diagnostics Corroboration

et al. 2008

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A case of primary alveolar soft part sarcoma (ASPS) of the heart is reported in an 11-year-old female as 1 of 16 cases of ASPS presenting in the first 2 decades of life in our institutional 17-year review period. The classic alveolar or organoid pattern was inconspicuous as compared to a more diffuse or formless pattern consisting of a population of uniform round cells with abundant eosinophilic cytoplasm, but in addition there was a second, minor population of gigantiform tumor cells with a variety of unusual shapes. Scattered tumor cells contained dense eosinophilic condensations in the cytoplasm. Other unusual features for ASPS in our case included a lymphohistocytic reaction and zonal necrosis. Immunohistochemistry revealed nuclear reactivity for TFE3, and the ASPL-TFE3 fusion transcript was identified by reverse-transcriptase polymerase chain reaction. The only other examples of ASPS involving the heart were 3 cases in the literature of metastatic disease from tumors arising in the soft tissues. This initial case of primary cardiac ASPS joins the list of other types of sarcomas in children that have been reported as primary neoplasms of the heart.

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“…Die auf den Nachweis von Desmin und MyoD1-Regulator-Protein gestützte Hypothese eines myogenen Ursprungs konnte durch neuere Untersuchungen nicht bestätigt werden [8,12,15,16,22]. Die auf den Nachweis von Desmin und MyoD1-Regulator-Protein gestützte Hypothese eines myogenen Ursprungs konnte durch neuere Untersuchungen nicht bestätigt werden [8,12,15,16,22].…”

Section: Kasuistikunclassified