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PubMed1993

[Intensive chemotherapy for alveolar soft part sarcoma with lung metastasis in a child].

Alveolar soft part sarcoma (ASPS) is one of the soft tissue sarcomas characterized by metastasis early in the course of the disease, regardless of slow tumor growth. The ultimate clinical outcome is, therefore, very poor. To date, the efficacy of combination chemotherapy for this tumor has not been confirmed. We present a case of ASPS in an 8-year-old boy with bilateral pulmonary metastasis who received systemic combination chemotherapy (CPM/IFO/ACD/ADR/CD DP/VP-16 as induction, and VCR/ACD as maintenance therapy) for eleven months. The result showed that despite the prevention of tumor progression, such intensive chemotherapy could not cure the disease. Besides refinement of chemotherapy regimens, immunotherapy might be considered for ASPS.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2000

Primary alveolar soft part sarcoma of the stomach: a case report and review.

Alveolar soft part sarcoma (ASPS) is a rare tumor typically located in skeletal muscles and muscolofascial planes. Isolated cases of ASPS have been described as arising in the viscera. We report a mesenchymal tumor of the stomach in a 54-year-old Italian woman without evidence of primary neoplasm elsewhere ten years following the initial diagnosis. The histologic, histochemical, immunohistochemical, and electron microscopic findings were all consistent with the diagnosis of ASPS and allowed differentiating it from morphologically similar and more common tumors, such as metastatic renal cell carcinoma and paraganglioma. The patient is alive and well ten years following the initial presentation.

Biomarkers, Tumor↗
PubMed2006

Alveolar soft part sarcoma in brain with cardiac metastasis: a case report.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm. The coexistence of ASPS with cardiac metastasis is quite rare, in particular. In general, the sarcoma is a malignant disease and grows very fast. However, the mean survival time of patients with ASPS is relatively long. Patients who are diagnosed with localized disease usually have a favorable prognosis, while those who present with widespread metastases usually have a poor prognosis and ultimately, succumb to their disease. The use of modern treatment modalities, such as combination of surgery, and radiotherapy, may significantly prolong the survival time in many patients. Because of the long-term period with symptomatic brain tumors in this disease, the patients should be treated even in the presence of multiple metastases in other organs.

Adult↗
PubMed1976

[A calvalial metastasis of alveolar soft part sarcoma (author's transl)].

The authors report and discuss a calvalial metastasis of alveolar soft part sarcoma which primarily developed in the left arm. A 13-year-old girl noted a painless tumor in the left upper arm at the age of 7 years. The tumor was excised and histologically alveolar soft part sarcoma. 5 years later, a rapid growing tumor was noted in the parietal area. The craniograms showed the left parietal bone defect. The chest X-ray showed a few large oval metastatic shadows. The left common carotid angiograms demonostrated high vascularities with irregular vessels in the arterial phase and a homogenous blush or stain in the venous phase. It was thought likely to meningioma. In selective external carotid angiograms, early arterial phase showed a few of vascular streaks in beads, fine tortuous vessels and early conspicuous filling of enlarged vein. Paradoxically, despite evidence of rapid shunting of blood from the lesion. "Wash out" of contrast media from the lesion was slow. Above mentioned angiographic appearance was consistent with that of cases reported by Rosenbaum, A.E., and was considered characteristic features of alveolar soft part sarcoma, meningiosarcoma and malignant melanoma. It must be emphasized that the slective angiography is important to differentiate angiographically alveolar soft part sarcoma, meningiosarcoma, malignant melanoma from meningioma or angioma. Radiotherapy is not effective to the pulmonary metastatic lesion. Selective angiography should be emphazied to be essential for differentiation of alveolar soft part sarcoma from angioma or meningioma.

Adolescent↗
PubMed1979

Alveolar soft part sarcoma: a report of two cases with some histochemical and ultrastructural observations.

In a survey of all malignant soft tissue tumors in the extremities and limb girdles in Finland between 1960 and 1969, only one alveolar soft part sarcoma was found among 246 tumors (0.4%). Another alveolar soft part sarcoma, diagnosed in 1976, was more thoroughly studied. There was evidence that the characteristic crystals of alveolar soft part sarcoma are formed from the dense granules. Both were PASM-positive at ultrastructural level. No monoamines were detected in the cells by formaldehyde-induced fluorescence. This is a further fact to nullify the theory of the paraganglionic origin of alveolar soft part sarcoma, but the question of the histogenesis of the tumor still remains open.

Adult↗
PubMed2004

Alveolar soft part sarcoma on the glabella.

OBJECTIVE: To present a paediatric case of alveolar soft part sarcoma on the glabella. CASE: A 10-year-old female patient with a slowly growing painful and pruritic mass between her brows. During surgical excision of the mass, it was seen that the mass was firmly adherent to the periosteum. Extended surgical removal with the underlying periosteum was carried out. The pathological result was alveolar soft part sarcoma which was immunohistochemically positive for vimentin and showed focal desmin positivity. The patient had no evidence of distant metastases on screening. CONCLUSION: Total surgical excision of the tumour in due time before metastases should be the mainstay of treatment. A high index of suspicion, both during diagnosis and surgery, should be maintained by the clinician.

Child↗
PubMed1986

Adenosine triphosphatase activity of crystalline inclusions in alveolar soft part sarcoma. An ultrahistochemical study of a case.

A case of alveolar soft part sarcoma was studied by light and electron microscopy and by electron microscopic enzyme histochemistry of adenosine triphosphatase (ATPase) and 5'nucleotidase(5'Nase). The tumor showed distinct alveolar pattern and diastase resistant PAS positive crystalline inclusions were found in the cytoplasm. Ultrastructurally, characteristic rhomboid crystals and dense granules were observed and they were positive for Mg++- and Ca++-ATPases but negative for 5'Nase. Tumor cell membrane also showed positive activity of ATPase in addition to 5'Nase. These results would support the myogenous derivation of alveolar soft part sarcoma.

5'-Nucleotidase↗
PubMed1998

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

AIM: Alveolar soft part sarcoma is a very rare tumour. Nine cases are reviewed in order to identify new aspects of this tumour. METHODS AND RESULTS: The clinical course, histological, immunohistochemical and ultrastructural features of nine cases of alveolar soft part sarcoma were reviewed. Proliferative activity and p53 protein accumulation were assessed immunohistochemically. The patients were aged between 18 and 70 years. In the cases with sufficient follow-up, survival was variable with two patients dying within 5 months and four alive at 4 years. Histologically all tumours had an alveolar component but one case also had a spindle component and another case had a pseudoglandular pattern. Six cases showed desmin immunoreactivity, one was muscle-specific actin positive, two were positive for S100 protein and three were positive for vimentin. MIB-1 immunostaining was seen in up to 35% of cells. Two cases showed p53 protein accumulation. CONCLUSIONS: There appeared to be no correlation between short term survival (4 years or less) and clinical presentation, adjuvant treatment, tumour size, histological grade, vascular invasion by tumour, proliferative index, or p53 protein accumulation. Although unusual, spindle cell or pseudoglandular components can be seen in alveolar soft part sarcoma.

Adult↗
PubMed1985

Alveolar soft part sarcoma of the vagina: an immunohistochemical and electron microscopic study.

Alveolar soft part sarcoma is a soft tissue neoplasm of unknown histogenesis which has a distinctive morphology. It is a relatively rare tumor with approximately 200 cases described in the literature. Only two cases have previously been reported as occurring in the vagina. The purpose of this paper is to report the third case of alveolar soft part sarcoma occurring in the vagina and to emphasize the role of electron microscopy in the differential diagnosis.

Adenocarcinoma↗
PubMedDate not supplied

Alveolar soft part sarcoma: histogenesis.

BACKGROUND: 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, however, the histogenesis has remained undetermined. MATERIALS AND METHODS: In order to investigate the histogenesis of ASPS, two cases were examined using the immunofluorescence antibody (IF) method, the avidin-biotin peroxidase complex (ABC) method, Western bloffing with an anti-actin polyclonal antibody, and reverse transcription polymerase chain reaction (RT-PCR). Moreover, a total of seven cases were examined using the ABC method involving eight antibodies related to skeletal muscle; RESULTS: The results obtained from the IF method suggested that the granules in the cytoplasm were probably actin. The results from the IF method, Western blotting and RT-PCR were compatible with the presence of alpha-actin from human skeletal muscle in ASPS, while the ABC method using eight muscle-related antibodies also suggested the origin of ASPS was skeletal muscle. CONCLUSION: It is postulated that the histogenesis of ASPS is of skeletal muscle origin.

Actins↗
PubMed1992

[Alveolar soft part sarcoma: a clinicopathologic and immunohistochemical study of 13 cases].

Thirteen cases of alveolar soft part sarcoma were studied clinicopathologically and by the PAP technique antisera against desmin, myoglobin, S-100 protein, neuron-specific enolase (NSE). We suggested, light microscopically, the main points for diagnosis and differential diagnosis of alveolar soft part sarcoma. Immunohistochemically, the tumor cells reacted positively for desmin (five cases), myoglobin (three cases) and NSE (four cases). No immunoreactivity for S-100 protein was observed. Similar results had been reported by others. No definite conclusion about histogenesis of alveolar soft part sarcoma could be drawn from the small number of cases in this study.

Adolescent↗
PubMed1990

[The histogenesis of alveolar soft part sarcoma: a clinicopathological analysis of 135 cases].

135 cases of alveolar soft part sarcoma (ASPS) are presented. It is the largest series of ASPS currently reported in the medical literature. Follow-up clinical data were available in 51 cases. 68 of the patients were male and 67 were female. The mean age of the patients when making the diagnosis was 35.5 years. Most of the tumors were located at the extremities (72.1%), particularly on the thigh and buttock (36.1%). The five year survival rate was 45.9%, the recurrence rate was 47.1%, and the metastatic rate was 35.3%. The most common sites of metastases were the lung, brain and liver. Microscopially, all the tumors gave a typical organoid structure. Electron microscopic study showed special crystals in the tumor cells. 33 cases of ASPS were examined immunohistochemically with 16 kinds of antibodies in order to clarify the histogenesis of alveolar soft part sarcoma. The presence of desmin, actin, myoglobin and myosin suggests the myogenic character of this tumor.

Adolescent↗
PubMed1989

Alveolar soft part sarcoma. An ultrastructural and immunocytochemical investigation of its histogenesis.

Fourteen alveolar soft part sarcomas were studied using electron microscopic analysis and a battery of immunocytochemical procedures in order to critically assess the extent to which these methods could explain the histogenesis of the tumor. All the tumors were stained with antibodies against keratin, vimentin, S-100 protein, Leu-7, neuron-specific enolase, desmin, muscle-specific actin, myoglobin, smooth muscle myosin, and fast and slow myosins. Eight tumors stained for vimentin, eight for desmin, eight for neuron-specific enolase, two for muscle-specific actin, and one for fast myosin. No reactivity was detected with the other antibodies. Electron microscopic study showed a consistent pattern of fine structure and clarified the mode of formation of the distinctive cytoplasmic crystals which were detected in all 14 tumors. Intermediate filaments were sparse and specific myofilaments were not detected. Alveolar soft part sarcoma has a myogenic phenotype and the cells show skeletal muscle differentiation but do not closely resemble any known prototypic normal cell.

Actins↗
PubMed1999

Alveolar soft part sarcoma of the tongue: report of an unusual pattern in a child.

A case of an unusual, primary alveolar soft part sarcoma of the tongue in a 3-year-old boy is presented. Alveolar soft part sarcoma is a rare malignant tumor that presents more frequently in the head and neck region of infants and children. To date, only six cases of lingual alveolar soft part sarcoma presenting in the first decade of life have been reported in the English literature. The patient has undergone surgical resection without adjuvant chemotherapy or radiation. Thus far, the patient does not manifest persistent or recurrent disease.

Biomarkers, Tumor↗
PubMedDate not supplied

Retroperitoneal sarcoma with features suggestive of alveolar soft part sarcoma.

A 72-year-old man presented with a 15 x 12 x 5 cm cystic and necrotic tumor involving the superior region of the stomach and the retroperitoneum down to the transverse colon and extending laterally from the left lobe of the liver to the spleen without involving either organ. Light microscopy showed the tumor cells to be arranged in an organoid pattern. Immunocytochemistry showed a strong reaction for desmin and vimentin. Electron microscopy demonstrated features suggestive of an alveolar soft part sarcoma.

Aged↗
PubMed1986

Alveolar soft part sarcoma, granular cell tumor, and paraganglioma. An immunohistochemical comparative study.

Five cases of alveolar soft part sarcoma, 5 cases of granular cell tumor, and 6 cases of paraganglioma were investigated immunohistochemically to examine the expression of tissue-specific intermediate filaments (cytokeratin, vimentin, desmin, and glial fibrillary acidic protein (GFAP], actin, myoglobin, and nervous tissue markers (S-100 protein, neuron-specific enolase, and Leu-7). In alveolar soft part sarcomas, some of the tumor cells were positive for desmin, but negative for nervous tissue markers. The tumor cells of granular cell tumors were stained with anti-S-100 protein antibody, but not with anti-neuron-specific enolase antibody. In contrast, the tumor cells of paragangliomas were positive for neuron-specific enolase, but not for S-100 protein except for stellate cells surrounding the tumor cell nests. This immunohistochemical approach was valuable for the differential diagnosis of these three tumors. Furthermore, the complete absence of cytokeratin in all of the tumor cells may be helpful in distinguishing these three tumors from metastatic carcinoma in soft tissue. The histogenesis of alveolar soft part sarcoma is a matter of controversy. The result that besides desmin actin was also demonstrated in some of the tumor cells may support the myogenic origin of this tumor.

Adult↗
PubMed1996

Alveolar soft part sarcoma metastatic to the breast.

Metastasis to the breast is uncommon, with an incidence of 0.5-3%. Alveolar soft part sarcoma is rare, accounting for < 1% of malignant soft tissue tumors, which are themselves unusual. Excluding contralateral breast and hematologic malignant disease, the primary lesion in most cases of metastasis to the breast is melanoma, small cell carcinoma of the lung, or ovarian carcinoma, although rhabdomyosarcoma is the most common primary tumor in children. We describe a 26-year-old woman with no history of malignant disease who presented with two masses in the right breast that clinical evaluations and ultrasonography indicated were fibroadenomas. Pathological studies after excisional biopsy, however, indicated alveolar soft part sarcoma. Subsequent computed tomography showed the primary tumor in the anterior left thigh and multiple bilateral lung metastases. Because of the presence of distant metastases, the patient was treated with chemotherapy.

Adult↗