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PubMed1991

[A case of effective preoperative chemotherapy in alveolar soft part sarcoma].

Alveolar soft part sarcoma is primarily treated by surgery, and reports of effective chemotherapy have been infrequent. We experienced a patient with alveolar soft part sarcoma in the left thigh, in whom preoperative chemotherapy with cisplatinum (CDDP) and adriamycin (ADM) was effective. This patient was a 13-year-old male who had an elastic hard mass with the size of his fist in the medial thigh. Plain X-ray revealed bone destruction with periosteal reactions in the distal diaphysis of the femur. Biopsy specimen showed alveolar growth of relatively large cells with eosinophilic or clear cytoplasm. PAS staining was positive in microgranular patterns in part of the cells, but no crystalline structure were observed. A diagnosis of alveolar soft part sarcoma was made, and chemotherapy was started. After administration of 150 mg of CDDP intraarterially twice and 50 mg of ADM intravenously 5 times, effectiveness of chemotherapy was estimated by physical and radiological findings. In the resected specimen, the margins of the soft part tumor showed shell-like ossification, suggesting the subperiosteal or intraosteal origin. Histology of the removed specimen showed that the tumor was mostly replaced by fibrous tissues, indicating the effectiveness of the preoperative chemotherapy. This disease has a relatively long course, however, the present case is considered to be another exceptional case that responded to preoperative chemotherapy.

Adolescent↗

PubMed2003

Possible association between tumor-suppressor gene mutations and hMSH2/hMLH1 inactivation in alveolar soft part sarcoma.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor of unknown origin and pathogenesis. We clinicopathologically analyzed 16 cases of ASPS and screened for the genetic alterations of various tumor-suppressor genes and oncogenes, including p53, adenomatous polyposis coli (APC), E-cadherin, and beta-catenin, in 11 cases of ASPS. We also examined the expression of hMSH2/hMLH1 of DNA mismatch repair genes by immunohistochemistry, and promoter hypermethylation of these DNA mismatch repair genes by methylation-specific polymerase chain reaction (MS-PCR) to elucidate any possible association between mutation status of these genes and inactivation of the hMSH2/hMLH1 genes. Furthermore, microsatellite instability (MSI) analysis and loss of heterozygosity (LOH) on chromosome 5q analysis were used for some cases of ASPS where DNA derived from normal tissue was available. The 5-year overall survival rate for all of the patients in this study was 68.6%. The 5-year overall survival rates for patients presenting with localized ASPS and for patients with distant metastases were 83.3% and 47.6%, respectively. The high nuclear grade of tumor cells was a significantly adverse prognostic factor (P = 0.0085). Single-strand conformation polymorphism analysis followed by DNA direct sequencing revealed 4 point mutations of the p53 gene in 3 of 11 cases (27.3%), composed of 3 missense mutations and 1 silent mutation. In addition, 1 case with the E-cadherin missense mutation and 1 case with the APC missense mutations were observed, respectively. None of the cases harbored mutation of exon 3 of the beta-catenin gene. Loss of expression of the hMSH2 and hMLH1 genes was observed in 2 (18.2%) and 3 (27.3%) of 11 cases, respectively. All 3 cases with loss of hMLH1 gene expression harbored mutations of the p53 gene. There was a statistically significant correlation between the genetic alteration positive in these tumor-suppressor genes and loss of hMLH1 gene expression (P = 0.024). Methylation-specific PCR did not reveal hypermethylation of the hMSH2/hMLH1 promoter region in any of the cases examined. Three of 8 (37.5%) ASPS cases showed low MSI, and 2 of these 3 cases showed immunohistochemical lack of expression for either hMSH2 or hMLH1. LOH on 5q was present in 2 of 6 (33.3%) informative cases, and both cases showed LOH on the D5S346 marker, a microsatellite marker near the APC locus. Thus, inactivation of hMSH2/hMLH1 of DNA mismatch repair genes seems to have an important role to play in the mutagenesis of the tumor-suppressor genes in ASPS.

Adaptor Proteins, Signal Transducing↗

PubMed1980

Alveolar soft part sarcoma.

Alveolar soft part sarcoma is a neoplasm that usually presents as a slow-growing mass located in an extremity and is most often initially discovered by the patient, who then seeks medical evaluation (1, 2). Local pain or symptoms produced by metastases as the initial manifestations of this neoplasm are unusual (2). A case is presented in which CT demonstrated multiple metastases to the central nervous system that produced headaches as the first symptoms in a patient with alveolar soft part sarcoma.

Adolescent↗

PubMed1977

Lymphography in alveolar soft part sarcoma.

Alveolar soft part sarcoma is a rare tumour found in musculofasculofascial planes, most often in the lower limbs of young adults. The tumour shows a characteristic orderly cellular arrangement and the few mitoses belie the often rapid progress of the disease. Cytoplasmic crystals unique to this tumour may be demonstrated. The need for thorough investigation of the patient as an initial staging procedure and to provide a prognostic forecast is emphasised. Isotope scanning of the bones, brain and liver is indicated. Two cases are reported which demonstrate the value of lymphography. The nodal features found were: 1. Enlargement of a lymph node of normal interal architecture. 2. Almost complete nodal replacement by tumour. 3. Pseudolymphomatous appearances. Follow up films are required, after an interval, to show the progress of the disease or response to therapy.

Adult↗

PubMed1976

Alveolar soft part sarcoma.

Alveolar soft part sarcoma is a rare, highly malignant tumor. It usually arises in the skeletal muscles in the extremities of patients in their second or third decade with a female preponderance of 2:1. Three additional cases, including the angiographic findings, have been presented. The literature pertaining to this subject also has been reviewed.

Adolescent↗

PubMed1995

[Two cases with metastatic intracerebral alveolar soft part sarcoma].

The alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor which usually occurs in the lower extremity of young girls. The incidence of metastasis is said to be highest in the lung (38%), and second highest in the bone and brain (33%). This report describes two cases of metastatic intracerebral alveolar soft part sarcoma, originating in the lower extremity. A female patient noticed a painless swelling in her right leg at the age of 11, and 10 years later she underwent total removal of the tumor. The diagnosis was alveolar soft part sarcoma. At 37 she was admitted to our service with a diagnosis of cerebral metastasis in the left frontal lobe. Since then she has undergone surgical removal 4 times for recurrent cerebral metastasis and twice for lung metastasis. Now she is 55 years old and doing well except for mild left hemiparesis. She survives without cerebral or general metastasis 44 years following the onset of the sarcoma in her right leg and 18 years following the onset of the metastatic brain tumor. A 30-year-old man, who noticed a painless swelling in his left thigh in January 1991, underwent total removal of the tumor and the diagnosis was alveolar soft part sarcoma. He was admitted to our service with no neurological deficits in October 1992, but a CT scan showed a metastatic brain tumor in the left parieto-occipital region. Total removal of this metastatic brain tumor was successfully performed in November 1992. However, he died because of multiple brain and lung metastasis in February, 1994.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1994

Clinical presentation of alveolar soft-part sarcoma.

Alveolar soft-part sarcomas are rare and seldom considered in the differential diagnosis of a soft-tissue mass. Thus, early clinical recognition can be elusive. The authors have identified several clinical and radiographic features of alveolar soft-part sarcoma, emphasizing the importance of magnetic resonance imaging in the preoperative diagnostic and staging workup. Accurate diagnosis and treatment of this unusual tumor requires clinical suspicion and clinicopathologic correlation with appropriate radiographic studies. If the clinical or radiographic interpretation is equivocal, early biopsy is essential to differentiate alveolar soft-part sarcoma from arteriovenous malformation.

Adult↗

PubMed1989

The radiologic manifestations of alveolar soft-part sarcoma.

Alveolar soft-part sarcoma is a rare soft-tissue tumor of unknown cellular origin that is characterized histologically by its organized "pseudoalveolar" pattern. The radiologic findings in 11 patients with this neoplasm were reviewed. The six men and five women were 16-48 years old (mean, 27 years). Nine patients had untreated primary tumors (thigh, four; forearm, two; and buttock, rectus abdominis muscle, and infratemporal fossa, one each) and two had locally recurrent masses (one each in the retroperitoneum and retrocrural space). All patients were evaluated by conventional radiography, two by sonography, eight by CT, five by angiography, and three by MR. Conventional radiographs showed the soft-tissue mass in only four patients; four lesions caused destruction of adjacent bone and two had soft-tissue calcification. Unenhanced CT showed low-attenuation lesions in four of five patients. The lesions were hypervascular on contrast-enhanced CT or angiography in each of nine patients studied. Prominent draining veins were shown by CT or angiography in five patients. Three lesions had a prolonged capillary stain on angiography. Alveolar soft-part sarcoma should be considered in the differential diagnosis of a hypervascular soft-tissue mass, particularly in the thigh of a young adult.

Adolescent↗

PubMed1981

Pulmonary resection and chemotherapy for metastatic alveolar soft-part sarcoma.

Alveolar soft-part sarcoma (ASPS) is an unusual tumor of soft tissues; it has invariably ended in death from disseminated disease, and the lung has been the most common site of metastasis. We present a patient with ASPS with bilateral pulmonary metastases who achieved a complete response after bilateral thoracotomies with removal of all gross disease and after combination chemotherapy including vincristine, actinomycin D, cyclophosphamide, and doxorubicin. The patient has now been followed for five years since the appearance of the metastases and has been off therapy for the past 34 months. He shows no evidence of disease. We suggest that multimodality therapy is a reasonable approach in patients with ASPS and pulmonary metastases and that such therapy has the potential for improving survival.

Adolescent↗

PubMed1991

Fine-needle aspiration cytology of alveolar soft-part sarcoma.

Alveolar soft-part sarcoma (ASPS) is a rare soft-tissue tumor. Few cases have been reported in the aspiration cytology literature. We discuss the fine-needle aspiration (FNA) cytologic features of ASPS in four patients. The smears were characterized by single cells and clusters of cells associated with thin-walled vasculature, resulting in a distinct pseudoalveolar pattern. The cells had abundant granular cytoplasm and large round nuclei with prominent central nucleoli. Binucleated and occasional multinucleated cells were present. Numerous stripped tumor nuclei were a consistent feature. Periodic acid-Schiff (PAS)-positive diastase-resistant granules were demonstrable within the intact and fragmented cytoplasm. Electron microscopy of aspirated material identified granules and crystals with the characteristic lattice pattern. To conclude, the FNA cytologic features of ASPS are characteristic. Confirmation by special stains and electron microscopy of FNA material is possible. The differential diagnostic considerations include renal cell carcinoma, paraganglioma, granular cell tumor, clear cell sarcoma, and epithelioid sarcoma.

Adult↗

PubMed2006

MRI of alveolar soft-part sarcoma.

Alveolar soft-part sarcoma (ASPS) is rare. We present a case of a 30-year-old woman with a 6-month history of a palpable mass in her left thigh. MRI showed an 8-cm mass and a satellite nodule at the left gluteus muscle. The main tumor exhibited an isointense signal on T1-weighted images and high-signal-intensity areas with low-signal-intensity scanty solid components on T2-weighted images. MR angiography showed dilated and tortuous veins around the tumor. Histologic findings were compatible with ASPS.

Adult↗

PubMed1982

Alveolar soft-part sarcoma.

Alveolar soft-part sarcoma is a slow-growing but nevertheless malignant soft tissue tumour arising in muscle. It occurs most frequently in young women and often presents as a painless swelling in an upper or lower limb. The swelling, which may have been present for a considerable time, is firm and appears to be well circumscribed but it may recur locally after excision. Blood-borne metastases to the lung are common and this is frequently the mode of presentation. Lymphatic metastases are unusual. The tumour is composed of nests of large cells and the histological appearances may resemble those of a renal adenocarcinoma (hypernephroma). Six new cases of this very rare tumour are presented and the published reports are reviewed.

Adult↗

PubMed1995

Successful treatment for solitary brain metastasis from alveolar soft part sarcoma.

Following pulmonary metastases, alveolar soft part sarcoma has an unexplained predisposition to metastasize to the brain. Herein, a case of alveolar soft part sarcoma solitarily metastatic to the brain is described. A 23-year old female underwent the resection of alveolar soft part sarcoma from the right thigh. Three years after the surgery, a brain tumor was resected and pathology proved it was a metastatic alveolar soft part sarcoma. She is currently alive and well 6 years after brain surgery. Our report suggests that surgical resection for solitary brain metastasis from alveolar soft part sarcoma is an effective treatment modality.

Adult↗

PubMed1999

Primary alveolar soft part sarcoma of bone.

AIMS: Alveolar soft part sarcoma is a distinct, rare soft tissue tumour occurring primarily within the skeletal muscles or musculofascial planes in young adults. Primary involvement of bone is extremely rare. We report on six patients with alveolar soft part sarcoma occurring primarily in bone. METHODS AND RESULTS: Thorough clinical and radiographic examinations were done to rule out any other primary site. The patients were four women and two men aged 17-35 years (mean, 24.5 years). The primary site of the tumour was the femur in three patients, the ilium in one and the fibula in two. In one of the patients with fibular involvement, the tibia was also involved by direct extension. Of the long bone lesions, three were centred in the metaphysis and one in the diaphysis. Radiographically, all of the lesions demonstrated an osteolytic pattern of bone destruction with ill-defined margins and a wide zone of transition between the lesion and adjacent normal bone. Microscopically, all tumours showed the typical histological pattern of alveolar soft part sarcoma. Diastase-resistant, periodic acid-Schiff-positive crystalline structures were identified within the cytoplasm and confirmed ultrastructurally. Immunohistochemically, a keratin stain was negative in all cases; there was positive staining for MyoD1 in the cytoplasm but not the nuclei. Distant metastasis developed in four patients; one died. CONCLUSION: Alveolar soft part sarcoma arising in bone is extraordinarily rare but should be considered in the differential diagnosis of metastatic hypernephroma in a young patient.

Adolescent↗

PubMed1996

[A case of gastrointestinal bleeding from metastatic alveolar soft part sarcoma of the jejunum].

Alveolar soft part sarcoma (ASPS) is a rare malignant tumor. It is often seen in the extremities of youngsters, and frequently metastasizes to the lung, brain, and bones. We experienced a case of ASPS which presented with gastrointestinal (GI)bleeding and was diagnosed as metasatic ASPS of the jejunum. We could not find in the literature a single case of ASPS which metastasized to the intestine and caused GI bleeding. We feel that it is important to consider the possibility that metastasis to the intestine can cause GI bleeding in a patient with a history of ASPS.

Adult↗

PubMed2006

Alveolar soft part sarcoma of the larynx.

Alveolar soft part sarcoma (ASPS) is a rare malignancy. It has generally a poor prognosis. Survival depends on the presence of metastases. Approximately, one-fourth of the cases are encountered in the head and neck region, mostly in the orbits and tongue. Surgery is accepted as the most effective treatment, radiotherapy and chemotherapy may be used as adjuvant treatments. Since it is a highly vascular tumor, profuse bleeding may occur during surgery. In this article, we report a case of ASPS occurring in the larynx, an extremely rare location for this rather unusual tumor. To our knowledge, only three cases of laryngeal ASPS have been previously reported in the English literature.

Adult↗

PubMedDate not supplied

Alveolar soft part sarcoma of the tongue.

Alveolar soft part sarcoma is a rare malignancy. To our knowledge, 13 cases in the head and neck have been reported previously. It occurs more commonly in the extremities, where it is associated with a poor prognosis. Its clinical course resembles that of adenoid cystic adenocarcinoma in that late metastases often cause death in 10 to 15 years. A 5-year-old girl with alveolar soft part sarcoma was successfully treated with partial glossectomy and is free of disease at two-year follow-up.

Child, Preschool↗

PubMed1988

Scintigraphic evaluation of two cases with alveolar soft part sarcoma.

Two cases of alveolar soft part sarcoma were examined with gallium-67 citrate (67Ga), technetium-99m-methylenediphosphate (99mTc-MDP) and technetium-99m(V)-dimercaptosuccinic acid (Tc(V)-DMS) to compare the sensitivity of these three radiopharmaceuticals. All scintigrams were positive with primary tumor, and images with Tc(V)-DMS were the best. Skull metastasis could be also detected by all agents, but scintigrams with lung metastases were negative. In scintigraphic evaluation of alveolar soft part sarcoma, Tc(V)-DMS may be a better agent than 67Ga or 99mTc-MDP.

Adult↗