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A case of alveolar soft-part sarcoma was studied. It illustrates the use of touch imprint preparations on fresh tissue sections examined while frozen. The technique is simple to use and can be applied to a wide variety of neoplastic and inflammatory lesions. Touch imprints substantially contribute to accurate histopathologic interpretation of lesions submitted for frozen-section diagnosis.
Alveolar soft-part sarcoma (ASPS) is a rare, distinctive sarcoma, typically occurring in young patients. Although it displays a relatively indolent clinical course, the ultimate prognosis is poor and is often characterised by late metastases. Recently, our understanding of the genetic events underlying the pathogenesis of ASPS has greatly increased. The historical, histopathological, ultrastructural, immunohistochemical and genetic aspects of ASPS are reviewed in this article.
Alveolar soft-part sarcoma is a rare soft tissue sarcoma of young adults with unknown histogenesis, and the organ most frequently involved in metastasis is the lung. We report the CT findings of three patients of pulmonary metastases of alveolar soft-part sarcoma, which manifested as clearly enhanced pulmonary nodules or masses. On enhanced scans, some of the masses were seen to contain dilated and tortuous intratumoral vessels.
A case of alveolar soft-part sarcoma with multiple cerebral metastases in addition to multiple lung and bone metastases is reported. This is a rare tumor which thus far has only been recognized as a malignant soft tissue tumor with great uncertainty. A 24-year-old man with a chief complaint of left frontal headache was admitted to our unit on September 26, 1994. A tumor of the right forearm had been surgically removed 9 years previously, and surgery had been followed by two courses of chemotherapy with vincristine, cyclophosphamide and actinomycin-D and subsequent irradiation for bone metastasis. The chemotherapy, however, did not have any clear effect. Neurological examination at the time revealed only bilateral papilledema, but CT and MRI scan of the brain showed a round mass in the left occipital lobe with homogeneous enhancement and another small mass was detected in the right occipital lobe. A cerebral angiogram showed a vascular tumor. The preoperative diagnosis was multiple cerebral metastases of alveolar soft part sarcoma. Left occipital craniotomy was performed on October 12, 1994, and the tumor was completely removed by lobectomy. Specimens of the tumor showed typical histological features of alveolar soft-part sarcoma. Whole-brain radiotherapy was administered postoperatively, and the patient is well neurologically without right homonymous hemianopsia. The clinical features of alveolar soft-part sarcoma are discussed. Only a few cases have been reported in the literature, but the most frequent site of the primary lesion has been the lower extremities, and cerebral metastasis has not been rare. All efforts should be focused on discovering this tumor in the early stage because surgical removal can yield good results, and radiotherapy is thought to be effective in terms of patient "quality of life".
Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumour found in young adults that usually arises in skeletal muscle and occurs most frequently in the lower limbs. Radiological and pathological findings of ASPS in a 34-year-old man who presented with increasing shortness of breath over a period of four to six weeks with peripheral blood eosinophilia, hypoxemia and a significant arteriovenous shunt are reported. The present article is the fourth report of eosinophilia in association with sarcoma, and the first involving ASPS.
Alveolar soft-part sarcoma (ASPS) is a very rare malignant tumor, usually of the extremities, and is seen most often in adolescents and young adults. Surgical excision of the primary and pulmonary metastases has resulted in prolonged survival in some patients, but adjuvant radiation and/or chemotherapy are generally thought to be ineffective. The authors describe a 13-year-old patient with ASPS of an extremity who presented with multiple bilateral pulmonary metastases at diagnosis. Following intensive multiagent chemotherapy, pulmonary metastases showed in vivo evidence of tumor death. The patient has remained disease-free for 10 years following treatment.
Approximately 25% of all alveolar soft-part sarcomas have their origin in the head, where the tongue and orbit are sites of predilection. The neoplasms possess characteristic light and electron-optic features, yet their histogenesis remains unclear. Recurrences, early metastases, and lethality over a prolonged period are pertinent biologic findings.
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Alveolar soft-part sarcoma (ASPS) is a rare tumour. Certain distinctive clinical and radiological features suggest the correct diagnosis. There is moderate predilection for young women. ASPS almost always arises in skeletal muscle and occurs most frequently in the lower limbs. There is often a long clinical history and a large mass at presentation. Two young females with ASPS presented with very vascular tumours in the thigh, with prominent intra- and extra-tumoural blood vessels. The imaging findings and the existing literature are reviewed.
Alveolar soft-part sarcoma is a rare, slow-growing, malignant tumour which metastasizes frequently to the lungs. Treatment with radiotherapy or chemotherapy is largely ineffective, and most patients die within 4 years of developing metastases. We report an unusual patient who survived 9 years after detection of pulmonary metastases. Initial treatment with cytotoxic chemotherapy was ineffective. However, during subsequent treatment with Chinese herbs when she developed galactorrhoea and amenorrhoea (probably due to drug-induced hyperprolactinaemia), her lung metastases regressed, and it is likely that this contributed to her prolonged survival. We hypothesize that alveolar soft-part sarcoma may be a hormone-sensitive tumour, and hormonal manipulation may be an alternative form of treatment which is worth considering.
Alveolar soft part sarcoma occurs mostly in the deep soft tissues. An unusual case of primary pulmonary alveolar soft part sarcoma is reported. A 39-year-old woman presented with thoracic pain revealing the tumor. The left lower lobe was surgically resected. The microscopic features of this tumor, including characteristic alveolar pattern and the PAS-positive crystals were typical of alveolar soft part sarcoma. Immunohistochemically, granular cytoplasmic reactivities were observed with antibodies against vimentin, myoglobin, methionine-enkephalin, S100 protein and neuron-specific-enolase. Electron microscopic study demonstrated numerous crystallized structures in the tumor cell cytoplasm. This is the third case of pulmonary alveolar soft part sarcoma, one arising from the pulmonary vein. The histogenesis of alveolar soft part sarcoma is still debated. Our case does not allow distinction between myogenic or neural origin of this tumor.
AIMS: Primary alveolar soft part sarcomas originating within the thoracic cavity are rare. The cases herein described highlight the ubiquitous distribution of this neoplasm and the importance of considering this tumour in the differential diagnosis of mediastinal tumours. METHODS AND RESULTS: Two cases of alveolar soft-part sarcoma of the mediastinum are presented. The patients are two men of 22 and 23 years of age. Clinically, the patients presented with symptoms of chest pain. One tumour was in the anterior mediastinum while the second tumour was in the posterior mediastinum. Even though the bulk of the tumours were in mediastinal locations, both patients had pulmonary metastases at the time of diagnosis. Histologically, both tumours showed the classic morphology of alveolar soft part sarcoma, i.e. a proliferation of large polygonal cells with round to oval nuclei, prominent nucleoli and moderate amounts of eosinophilic cytoplasm arranged in a prominent alveolar pattern. Periodic acid-Schiff stains showed the characteristic diastase-resistant intracytoplasmic crystals. Immunohistochemical studies showed focal myoglobin reactivity in one case, while cytokeratin, vimentin, S100 protein, chromogranin, synaptophysin, neurofilaments, leu-enkephalin, desmin, smooth muscle actin and muscle-specific actin were negative in both cases. The patient with the anterior mediastinal tumour died of disease 14 months after diagnosis, and the patient with the posterior mediastinal mass remained well for at least 15 years and was later lost to follow-up. CONCLUSIONS: As has been observed in other anatomic areas, namely soft tissues, alveolar soft part sarcomas may follow an uncertain natural history. Interestingly, in our cases, the tumour in the anterior mediastinum followed a fatal course raising the possibility that the anatomic location of the tumour may have play a role in the behaviour of the tumour.
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In 1952, Christopherson et al. proposed that alveolar soft-part sarcoma (ASPS) was a distinct entity with unique clinical and pathological features. Since their report, its histogenesis has not been determined. In order to clarify the histogenesis of ASPS, a study using reverse transcription polymerase chain reaction using cDNAs from MyoD1 and myogenin, and the actin filament from human-skeletal-muscle-related mRNAs has been performed in 5 cases of ASPS. The expression of MyoD1 and myogenin was determined in 5 and 2 cases of the 5 cases, respectively. Moreover, expression of the many mRNAs from the actin filament of skeletal muscle was also found in ASPS. According to these findings, it is now postulated that ASPS is of skeletal muscle origin.
The incidence, distribution, time of appearance, and radiologic findings of bone metastases from soft tissue sarcomas, exclusive of lymphomas, were evaluated in 320 patients with soft tissue sarcomas. Thirty patients (9.4%) had evidence of 58 bone metastases. Five of 30 patients presented with metastases, and 25 of 30 patients developed metastases up to 66 months after presentation with a mean time interval of 21.3 months. The incidence of skeletal metastases differed among histologic subtypes of sarcomas; alveolar soft part sarcoma (5 of 8), dedifferentiated liposarcoma (2 of 4), angiosarcoma (2 of 4), and rhabdomyosarcoma (5 of 16) tended to show a higher incidence of bone metastases. The sarcomas metastasized to the regional bones close to the primary tumor in 16 (53%) of 30 patients and to the axial bones in 18 (60%). On conventional radiographs, the osseous metastases demonstrated predominantly osteolytic changes, and evidence of pathological fracture was observed in 31% of 58 metastases.
Based on the data of the Surveillance, Epidemiology and End Results Section of the National Cancer Institute (SEER) program, soft tissue and bone sarcomas account for about 1% of all new malignancies diagnosed in the United States each year. However, there are numerous different histologic types, and any given type of sarcoma is extremely rare. Determining the incidence of sarcomas by age and type is difficult due to the limited data reported. The SEER program collects data regarding age but only limited data on histology, while most series reported in the literature include either adults or pediatric patients, but rarely both. In an effort to estimate the frequency and absolute numbers of different sarcomas in the adolescent and young adult population, the University of Texas M. D. Anderson Cancer Center (MDACC) tumor registry was queried for all soft tissue sarcomas from 1990 through 2003, and all bone sarcomas from 1990 through 2002. Based on this query, an overview of sarcomas that occur predominantly in the adolescent and young adult (AYA) population is presented. These sarcomas include rhabdomyosarcoma, synovial sarcoma, neurogenic sarcoma, epithelioid sarcomas, alveolar soft parts sarcoma, Ewing sarcoma, and osteosarcoma. Using the percentages for occurrence of each histologic type determined from the MDACC database, and the SEER estimate of overall sarcoma incidence, an estimate of the number of new cases in 2004 for the predominant histologic types occurring in the AYA population are presented. Also reviewed are the chromosomal translocations that occur frequently in sarcomas presenting in the AYA population.
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