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PubMed1994

Alveolar soft part sarcoma of the uterus: case report and review of the literature.

A case of alveolar soft part sarcoma of the uterus is described. It presented as intermenstrual bleeding in a 47-year-old woman. This is a rare tumor, forming about 1% of soft tissue sarcomas. Several hundred cases have been described, mostly arising in the limbs. We believe this to be the 3rd case reported in the uterus and the 12th in the female genital tract. The other reported cases are reviewed, and further information about their outcome has been obtained from the authors.

Curettage↗
PubMedDate not supplied

Alveolar soft part sarcoma. Report of a case diagnosed by needle aspiration cytology and electron microscopy.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare tumor, accounting for less than 1% of all primary soft tissue malignant neoplasms. The tumor is a distinct clinicopathologic entity, but its histogenesis is controversial. CASE: The needle aspirate from a soft tissue tumor in a 15-year-old girl was stained with Diff-Quik and periodic acid--Schiff (PAS) with and without prior digestion with diastase. Minute tumor tissue fragments were processed for transmission electron microscopic examination (EM). Clustered malignant cells with ill-defined, frothy or vacuolated cytoplasm showed a positive reaction with PAS and PAS with prior diastase digestion and oval nuclei containing macronucleoli. Intracytoplasmic, membrane-bound, rhomboid and rod-shaped crystals with a regular lattice pattern with 50-80 A periodicity were detected by EM study of aspirated tumor cells. CONCLUSION: Light and EM studies of aspirated tumor cells showed diagnostic features of the tumor.

Adolescent↗
PubMed1993

Alveolar soft part sarcoma of the uterine cervix.

A rare case of an alveolar soft part sarcoma of the uterine cervix in an 8 year old girl is presented. The patient was admitted because of genital bleeding lasting for 7 months. A polypoid tumor, 2 x 1.5 cm in diameter, was found in her external uterine os and was surgically resected. Microscopically, the tumor consisted of a uniform sheet of tumor cells in the cytoplasm which contained granules and which were stained with periodic acid-Schiff, both before and after the diastase digestion. Alveolar arrangement of the tumor cells was manifested with reticulin silver impregnation. Dense, membrane bound granules were evident at an ultrastructural level in the cytoplasm of the tumor cells. An immunohistochemical examination demonstrated a positive reaction for anti-desmin, anti-myoglobin, anti-HHF35 and anti-neuron specific enolase in the cytoplasm.

Child↗
PubMed2001

The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25.

Alveolar soft part sarcoma (ASPS) is an unusual tumor with highly characteristic histopathology and ultrastructure, controversial histogenesis, and enigmatic clinical behavior. Recent cytogenetic studies have identified a recurrent der(17) due to a non-reciprocal t(X;17)(p11.2;q25) in this sarcoma. To define the interval containing the Xp11.2 break, we first performed FISH on ASPS cases using YAC probes for OATL1 (Xp11.23) and OATL2 (Xp11.21), and cosmid probes from the intervening genomic region. This localized the breakpoint to a 160 kb interval. The prime candidate within this previously fully sequenced region was TFE3, a transcription factor gene known to be fused to translocation partners on 1 and X in some papillary renal cell carcinomas. Southern blotting using a TFE3 genomic probe identified non-germline bands in several ASPS cases, consistent with rearrangement and possible fusion of TFE3 with a gene on 17q25. Amplification of the 5' portion of cDNAs containing the 3' portion of TFE3 in two different ASPS cases identified a novel sequence, designated ASPL, fused in-frame to TFE3 exon 4 (type 1 fusion) or exon 3 (type 2 fusion). Reverse transcriptase PCR using a forward primer from ASPL and a TFE3 exon 4 reverse primer detected an ASPL-TFE3 fusion transcript in all ASPS cases (12/12: 9 type 1, 3 type 2), establishing the utility of this assay in the diagnosis of ASPS. Using appropriate primers, the reciprocal fusion transcript, TFE3-ASPL, was detected in only one of 12 cases, consistent with the non-reciprocal nature of the translocation in most cases, and supporting ASPL-TFE3 as its oncogenically significant fusion product. ASPL maps to chromosome 17, is ubiquitously expressed, and matches numerous ESTs (Unigene cluster Hs.84128) but no named genes. The ASPL cDNA open reading frame encodes a predicted protein of 476 amino acids that contains within its carboxy-terminal portion of a UBX-like domain that shows significant similarity to predicted proteins of unknown function in several model organisms. The ASPL-TFE3 fusion replaces the N-terminal portion of TFE3 by the fused ASPL sequences, while retaining the TFE3 DNA-binding domain, implicating transcriptional deregulation in the pathogenesis of this tumor, consistent with the biology of several other translocation-associated sarcomas. Oncogene (2001) 20, 48 - 57.

Adolescent↗
PubMed2000

Alveolar soft part sarcoma: MR and angiographic findings.

OBJECTIVE: To present the MR and angiographic findings of alveolar soft part sarcoma (ASPS). DESIGN AND PATIENTS: MR examinations (12 tumors of 10 patients) of ASPS performed at multiple hospitals were retrospectively reviewed. The tumors were found in the thigh (n=4), lower leg (n=4), femur (n=2, local metastasis), scalp (n=1) and arm (n=1). The MR signal characteristics including signal intensity, homogeneity and signal void of lesions and bony invasion including direct invasion or local metastasis were evaluated. Angiographic findings (n=4) and post-embolotherapy follow-up MR imaging (n=2) findings were also assessed. RESULTS: Local bony metastasis was found in two cases. Seven tumors showed heterogeneous high signal intensity on T - and T2-weighted images with good enhancement. One tumor had a very high signal on T1-weighted images. Eight tumors (67%) showed numerous signal voids in or near the tumors. All four angiographic studies showed numerous enlarged vessels, arteriovenous shunts and delayed washout. Two cases mimicked arteriovenous malformations on angiographic studies but MR images demonstrated solid soft tissue components as well as tortuous vessels. CONCLUSIONS: High signal on T1 -weighted image and numerous signal voids are highly suggestive of ASPS, although they are not universal as has been suggested and arteriovenous malformation should be included in the differential diagnosis. Local bony metastases in ASPS were seen in two cases and should be carefully investigated.

Adolescent↗
PubMed1977

[Treatment of alveolar soft part sarcoma (author's transl)].

Till now few reported cases of alveolar soft part sarcoma of the skeletal musculature which was classified for the first time by Christopherson in 1952 could not make plain a uniforme treatment. Radioresistance was also described as unsuccessful chemotherapy as early or excessiv surgical proceeding. Our own unusual observation of a case of this very rare malignant mesenchymal tumor may give a direction for a sensible therapy.

Adult↗
PubMed1990

Alveolar soft part sarcoma. Immunohistochemical evidence for muscle cell differentiation.

Seven cases of alveolar soft part sarcomas (ASPSs) were studied immunohistochemically for the presence of a number of differentiation markers in an attempt to define the cellular nature of this tumor. Desmin-positive tumor cells were found in three and muscle actin-positive cells in four cases when studied in formaldehyde-fixed and paraffin-embedded material. In one case studied in frozen sections, focal desmin positivity but no other intermediate filaments were found. Immunostaining for synaptophysin, a general neuroendocrine marker, was negative in all cases. All tumors were positive with a monoclonal antibody NK1C3, which consistently stains melanomas, and three cases showed significant numbers of S-100 protein-positive tumor cells, but immunostaining with HMB-45, a melanoma-specific monoclonal antibody, was negative in all cases. However, several rhabdomyosarcomas studied for NK1C3 and S-100 protein for comparison were also at least focally positive. Electron microscopic examination, performed in three cases, showed uniform paucity of all kinds of filaments in the tumor cells of ASPS, and it specifically failed to reveal any signs of smooth or striated muscle cell differentiation. Thus, the results of the present study do not unequivocally define the nature of ASPS but speak against its paraganglionic character and present evidence for muscle cell differentiation.

Actins↗
PubMed1989

Alveolar soft part sarcoma of the uterine cervix.

We describe two cases of alveolar soft part sarcoma (ASPS) that occurred as a primary lesion in the uterine cervix. In both cases, the tumor exhibited the typical histologic features of ASPS. In one case, the material for immunohistochemical staining and electron microscopy was available, and the findings of these studies were consistent with the diagnosis of ASPS. A review of the literature disclosed seven previous cases of ASPS occurring in the female genital tract. The tumor was located in the uterine cervix in only three cases. Although ASPS most commonly involves the soft tissues of the extremities and the head and neck region, it can also occur in rather unusual locations such as the female genital tract. Pathologists should be aware of these unexpected occurrences.

Actins↗
PubMed2004

[Detection of ASPL-TFE3 fusion gene by reverse transcriptase polymerase chain reaction in paraffin-embedded tumor tissues of alveolar soft part sarcoma].

OBJECTIVE: To investigate the significance of detecting chimeric mRNA resulting from t(X;17)(p11.2;q25) in paraffin-embedded tumor tissues of alveolar soft part sarcoma (ASPS). METHODS: Formalin-fixed, paraffin-embedded tumor tissues from 8 cases of alveolar soft part sarcoma and 15 cases of controls (including 6 alveolar rhabdomyosarcomas, 6 renal cell carcinomas, 2 paragangliomas and 1 granular cell myoblastoma) were retrieved from the archival materials. ASPL-TFE3 fusion transcripts were analyzed in all samples by reverse transcriptase-polymerase chain reaction (RT-PCR). The quality of the mRNA was assessed using the house-keeping gene beta-actin. RESULTS: ASPL-TFE3 fusion transcripts were detected in 6 of the 8 ASPS cases (4 being type 2 and 2 being type 1). The remaining 2 cases were negative for both beta-actin and ASPL-TFE3. No ASPL-TFE3 mRNA expression was detected in all the controls. PAX3/7-FKHR fusion transcripts were also detected in 4 of the 6 alveolar rhabdomyosarcoma samples. CONCLUSIONS: The expression of ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues can serve as an useful molecular marker in the diagnosis of ASPS. It may also be helpful in elucidating the underlying pathogenesis of ASPS in subsequent retrospective studies.

Adolescent↗
PubMed2000

Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare malignant tumor and little is known about its clinical features and management. We report on a series of 19 pediatric patients managed over 20 years. PATIENTS AND METHODS: Primary conservative surgery was performed in all patients and was radical in nine, non-radical in three; seven patients underwent biopsy alone (3 unresectable tumors, 4 metastatic disease). In two cases radical surgery was performed after primary chemotherapy. Radiotherapy was delivered to 8 patients, chemotherapy to 15. RESULTS: After a median follow-up of 74 months, the five-year survival was 80% for the whole series, 91% for patients with localized disease, 100% for patients with tumor < or = 5 cm, and 31% for those > 5 cm; 16 of 19 patients were alive (12 of 12 with grossly-resected tumor in first continuous remission). Chemotherapy achieved two partial remission among seven evaluable patients. CONCLUSIONS: Pediatric ASPS has a more favorable prognosis than its adult counterpart. In this series, tumor size correlates with metastatic disease at onset and is the major factor influencing survival. Surgery is the mainstay of therapy. The effectiveness of adjuvant therapy remains to be established, though radiotherapy may be advisable in cases of inadequate surgery.

Adolescent↗