Successful use of three-dimensional conformal radiotherapy as adjuvant treatment for alveolar soft part sarcoma. A case report / Uso exitoso de la radioterapia conformada tridimensional como tratamiento adyuvante del sarcoma alveolar de partes blandas. Reporte de un caso

Abstract Introduction: Alveolar soft part sarcoma is a very rare and aggressive type of sarcoma. Although its histology and genetic characteristics have been identified, the benefits of adjuvant radiotherapy for its treatment are still being studied. Case presentation: In November 2007, a 21-year-old woman presented with a primary tumor in the right thigh, with histological and immunohistochemical confirmation of an alveolar soft part sarcoma, which was totally resected in December 2007. Also, the large size of the mass suggested an unfavorable evolution. Two years after the first surgery, two metastatic tumors were detected in the right lung, which were completely resected separately. Two years later, the patient had two independent relapse events, five months apart: a mass in the right tight, and a metastatic tumor in the adrenal gland, together with a relapse in the tight. All tumors were successfully resected. In June 2014, after the last local relapse, adjuvant radiotherapy was started because of the risk of thigh amputation. At the end of treatment, the patient's general condition was good. Currently, at age 34, the patient is monitored through periodic evaluations, showing disease regression and stabilization. Conclusions: Currently, it is known that radiation not only produces cytotoxic effects on the target region but also induces an immune system-mediated systemic response with potential antimetastatic properties. Emerging radiobiological paradigms should be considered, particularly since they could explain some encouraging and unexpected results, such as those described in this case.

Resumen Introducción. El sarcoma alveolar de partes blandas es un raro y agresivo tipo de sarcoma. Aunque se han identificado sus características histológicas y genéticas, todavía se están estudiando los beneficios de la radioterapia adyuvante en su tratamiento. Presentación del caso. En noviembre de 2007, una mujer de 21 años se presentó con un tumor primario en el muslo derecho, con confirmación histológica e inmunohistoquímica de sarcoma alveolar de partes blandas y que fue completamente removido en diciembre de 2007. La masa mostró un gran tamaño, sugiriendo una evolución desfavorable. Dos años después de la primera cirugía, se detectaron dos tumores metastásicos en el pulmón derecho, que también fueron removidos, de forma separada. Dos años después, la paciente tuvo dos relapsos, separados por cinco meses: una masa en el muslo derecho, y un tumor metastásico en la glándula suprarrenal junto con una recaída en el muslo. Todos los tumores fueron extirpados con éxito. En junio de 2014, después de la última recaída local, el muslo estaba en riesgo de ser amputado, por lo que se decidió iniciar radioterapia adyuvante. Al final del tratamiento, la condición general de la paciente fue buena. Actualmente, ya con 34 años, es evaluada periódicamente, mostrando regresión y estabilización de la enfermedad. Conclusiones. Actualmente, se sabe que la radiación no solo produce efectos citotóxicos en la región objetivo, sino que también induce una respuesta sistémica mediada por el sistema inmune, con propiedades potencialmente antimetastásicas. En este sentido, se sugiere considerar los paradigmas radiobiológicos emergentes, ya que estos podrían explicar algunos resultados alentadores e inesperados como los descritos en este caso.

Alveolar soft part sarcoma of extremity

Alveolar soft-part sarcoma (ASPS) is an extremely rare connective tissue tumor, predominantly seen in adolescents and young adults, with a female preponderance. Alveolar soft-part sarcoma (ASPS) is a slow growing tumor, but with high likelihood of metastasis, leading to high mortality. A classical histopathological feature of an alveolar pattern from the biopsy of the lesion favors the diagnosis. We report a case of 14 years old male patient who presented with a history of single painless swelling over thigh for which surgical excision was done. Histopathology was suggestive of Alveolar soft-part sarcoma (ASPS). There was no evidence of distant metastases. He was treated with external beam radiotherapy in view of vascular invasion.

Alveolar soft part sarcoma in children: a clinicopathological study of 13 cases / 中华病理学杂志

Objective@#To investigate the clinicopathological manifestations, molecular genetic, diagnostic histology and differential diagnosis of alveolar soft part sarcoma (ASPS) in children.@*Methods@#A total of 13 cases of ASPS diagnosed at Beijing Children′s Hospital from August 2009 to November 2018 were collected. HE staining, histochemical staining for PAS and D-PAS, immunohistochemical (IHC) staining for TFE3, INI1 and CD68 and florescence in situ hybridization (FISH) for TFE3 gene translocation were performed.@*Results@#There were four males and nine females, age ranged from 1 year and 2 months to 13 years and 8 months (mean 7.8 years); and four patients were under 5 years old. Histologically, the tumors showed a distinctive and characteristic nested or organoid growth pattern (11 cases) or solid, diffuse growth (2 cases). The tumor cells possessed abundant eosinophilic, or glycogen-rich and clear to vacuolated cytoplasm. The chromatin was relatively dispersed, with prominent and pleomorphic nucleoli; mitotic figures were rare. Vascular invasion was frequently seen. IHC staining showed specific nuclear TFE3 staining. The tumor cells were also positive for INI1,CD68 and vimentin; but were negative for MyoD1, Myogenin, CK and S-100 protein. Seven cases showed PAS and D-PAS staining, with fuchsia acicular or rod-shaped crystals in tumor cytoplasm. Nine cases showed TFE3 break-apart signals by FISH.@*Conclusions@#ASPS is a rare soft tissue sarcoma in children. Compared with ASPA in adults, it has both similarities and unique clinicopathologic characteristics. The diagnosis needs to be confirmed by combining clinical, pathologic, IHC and genetic testing.

Alveolar soft part sarcoma in children: a clinicopathological study of 13 cases / 中华病理学杂志

To investigate the clinicopathological manifestations, molecular genetic, diagnostic histology and differential diagnosis of alveolar soft part sarcoma (ASPS) in children. A total of 13 cases of ASPS diagnosed at Beijing Children's Hospital from August 2009 to November 2018 were collected. HE staining, histochemical staining for PAS and D-PAS, immunohistochemical (IHC) staining for TFE3, INI1 and CD68 and florescence in situ hybridization (FISH) for TFE3 gene translocation were performed. There were four males and nine females, age ranged from 1 year and 2 months to 13 years and 8 months (mean 7.8 years); and four patients were under 5 years old. Histologically, the tumors showed a distinctive and characteristic nested or organoid growth pattern (11 cases) or solid, diffuse growth (2 cases). The tumor cells possessed abundant eosinophilic, or glycogen-rich and clear to vacuolated cytoplasm. The chromatin was relatively dispersed, with prominent and pleomorphic nucleoli; mitotic figures were rare. Vascular invasion was frequently seen. IHC staining showed specific nuclear TFE3 staining. The tumor cells were also positive for INI1,CD68 and vimentin; but were negative for MyoD1, Myogenin, CK and S-100 protein. Seven cases showed PAS and D-PAS staining, with fuchsia acicular or rod-shaped crystals in tumor cytoplasm. Nine cases showed TFE3 break-apart signals by FISH. ASPS is a rare soft tissue sarcoma in children. Compared with ASPA in adults, it has both similarities and unique clinicopathologic characteristics. The diagnosis needs to be confirmed by combining clinical, pathologic, IHC and genetic testing.

Progress of primary intracranial alveolar soft part sarcoma / 肿瘤研究与临床

Alveolar soft part sarcoma (ASPS) is a kind of extremely rare malignant soft tissue sarcoma, which was first discovered and defined by American scholars in 1952. Its main manifestation is painless and slow growth mass, and distant metastasis often occurs in lung, bone, brain, liver with poor prognosis. In recent years, with the accumulation of the number of cases and the development of related technology, the diagnosis and treatment of ASPS have been improved greatly. However, the primary intracranial ASPS is still rare, this paper reviews the progress of primary intracranial ASPS, in order to provide help for its clinical diagnosis and treatment.

Secondary Malignancies Developing After Acute Lymphoblastic Leukemia And Its TreatmentWith Constitutional Mismatch Repair Deficiency Syndrome In Siblings.

Background & Objectives: Acute Lymphoblastic Leukemia (ALL) is the most common childhood malignancy, with an annual rate of 3 to 4 cases per 1,00,000 children. ALL patients are treated with chemotherapeutic agents and cranial irradiation. Long-term sequelae of treatment are impaired intellectual and psychomotor functioning, neuroendocrine abnormalitites, impaired reproductive capacity, cardiotoxicity and second malignant neoplasms are being reported with increased frequency in the survivors. Among second neoplasms observed after treatment of ALL, central nervous system (CNS) tumors in patients treated with cranial irradiation are the most common. Others are Lymphoma, Acute Myeloid Leukemia (AML) and Thyroid Cancer. Methodology & Results: We are reporting four cases ,who developed ALL at the age of 8,6,4 and 25 years respectively(among that case 1 & case 2 are siblings) and were treated with chemotherapy and cranial irradiation. They developed Astrocytoma Grade-4 , Alveolar Soft-Part Sarcoma(ASPS) , Anaplastic Large Cell Medulloblastoma & Ewing’s Sarcoma at, 12 , 15, 8, 12 & 26 years of age respectively. Conclusion: Oncologists should make sure about the radiation doses before starting treatment and they should keep CMMR-D as a possibility in pediatric patients with siblings having h/o malignancy.

Durable Response to Pazopanib in a Patient with Metastatic Alveolar Soft Part Sarcoma / 이화의대지

Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma, and frequently, metastases are found at diagnosis. In patients with metastatic or unresected ASPS, systemic treatment is extremely limited, because conventional chemotherapeutic agents have not been effective in most cases. A novel agent inhibiting angiogenesis, pazopanib, has been proven to be effective for metastatic soft tissue sarcoma in a second-line setting. However, the efficacy of pazopanib in ASPS has not yet been reported. A 22-year-old man presented with right calf ASPS and multiple lung metastases. Pazopanib as a second-line treatment showed significant tumor response. To the best of our knowledge, this is the first report of the effectiveness of pazopanib in ASPS.

Retrospective research of prognosis factors involved surgical treatment for alveolar soft part sarcoma / 中华骨科杂志

Objective To explore clinical characteristics of alveolar soft part sarcoma(ASPS) and prognosis factors related to surgical treatment.Methods This study retrospectively analyzed 29 ASPS consecutive cases between Sep.1982 to Sep.2010,including 18 males and 11 females,with average 24.4 and median 21 years old (ranging 9 to 58).26 patients with surgery were enrolled in this study,with 23 cases primarily ocurred in soft tissue and 3 cases in bone.There were 17 cases with painless mass (65.4％,17/26) and 9 cases with pain (36.4％,9/26).Demographics,tumor size,stage,surgical margin,adjuvant chemotherapy provided,local recurrence,metastatic rate and overall survival probability were evaluated.We used multivariate analysis of logistic regression and Cox regression for local recurrence and survival rate respectively,and univariate analysis for tumor size,surgical margin,adjuvant chemotherapy and local recurrence.Results According to the Enneking classification,we distinguished stage Ⅱ 14 cases and stage Ⅲ 12 cases.Marginal excision was obtained in 9 cases as well as wide resection in 17 patients.The average and median follow-up time was 45.9 and 31 months (ranging 5 to 226) respectively,12 cases eventually survived (46.2％).The 5-year survival rates for stage Ⅱ and Ⅲ cases were 79.5％ and 23.4％ respectively.We found significant difference for local recurrence with surgical margin.However,there was no significant difference for prevention of metastatic progression with or without adjuvant chemotherapy.For tumor size,5-year survival rate of ＞ 5 cm and ＜ 5 cm group were 40.7％ and 80.0％ respectively.Multivariate analysis of logistic regression showed the surgical marginal was the only significant risk factor for local recurrence,while Cox regression showed both stage and tumor size were independent prognostic indicators for survival.Conclusion Although presenting as a slowly growing and painless mass,ASPS is an aggressive tumor with high risk of metastasis.Prognosis of ASPS is basically related to the characteristics of tumor size,surgical stage and quality of surgery.With limited improvement of adjuvant chemotherapy for metastasis control and survival,new agents are eagerly needed to complement surgery to eradicate this disease.

Clinicopathological study of the alveolar soft part sarcoma with 16 cases reports / 临床与实验病理学杂志

Purpose To investigate the clinicopathological changes, immuniohistochemistry and molecular genetics phenotypic charac-teristics of the alveolar soft part sarcoma ( ASPS) . Methods 16 cases of ASPS were studied with clinicopathological, cytochemistry technique and immunohistochemical staining, two cases of ASPS were studied by FISH. Results There were 6 males and 10 females with the age 8~58 years (median age 31. 7 years). The tumors were located at limbs, shoulder and back, tongue, vocal cords, lung, cervix, and ureter. The clinical manifestations of the patients was a slowly growing mass. Histopathologically the tumor showed typical organ-like or acinar-like structure with sinus-like blood vessels and the fibrous septa formation. Sometime the clear or abundant eosino-philic granular cytoplasm of the tumors were obvious. The tumor cells had a crystalline substance formation by PAS staining. The tumor cells were positive for TFE3 and Cathepsin K by immunohistochemical staining. The ASPL-TFE3 gene fusion detection of tumor cells were present. Conclusion ASPS often located on the limbs of young patients. It may misdiagnosed as malignant epithelial tumors, primary or metastatic adenocarcinoma and paraganglioma when the tumor locate on a rare anatomical parts or an organs, such as tongue, vocal cords, cervix, ureter, etc. It is valueable that the typical alveolar-like structure of the tumor and the expression for TFE3 and Cathepsin K for the pathological diagnosis of ASPS. It is an important indicator that the ASPL-TFE3 gene fusion detection by FISH for the tumor.

Alveolar Soft Part Sarcoma of the Uterine Cervix: A Case Report and Review of the Literature

Alveolar soft part sarcoma (ASPS) of the uterine cervix is a rare malignancy, and 21 cases have been reported the literature from every language (including our case). Herein, we describe a 17-yearold female patient who presented with active vaginal bleeding. Pelvic examination revealed a 1.6 x1.0x0.5-cm-sized soft mass protruding from the uterine cervix. The final pathological diagnosis was ASPS of the uterine cervix. Immunohistochemically, tumor cells were strongly nuclear positive for transcription factor E3. The patient remained disease free for 24 months without adjuvant therapy. The prognosis of ASPS in the cervix is considerably better than that of ASPS in soft tissues due to early clinical detection, small size, and resectability. ASPS should be considered in the differential diagnosis of an unusual epithelioid neoplasm showing organoid appearance with mild cytologic atypia and no/rare mitotic figures, particularly in young women. Pathologists should be aware of those unusual locations where ASPS may originate.

Alveolar Soft Part Sarcoma Arising from the Kidney: Imaging and Clinical Features

Alveolar soft part sarcoma (ASPS) is an extremely rare malignant soft tissue sarcoma primarily affecting young patients. It usually occurs in the lower extremities, although it can occur in soft tissue anywhere in the body. However, to our knowledge, there has been no case of primary ASPS originating from the kidney in the literature. We herein present the imaging and clinical features of an ASPS which occurred in a 16-year-old male presented as a palpable mass in the left side of the abdomen.

Alveolar Soft Part Sarcoma: Clinical Presentation, Treatment and Outcome in a Series of 19 Patients

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that usually affects young patients. Because of the rarity of the disease, most reports relating to ASPS are in the form of case reports or small series. METHODS: We performed a retrospective study to evaluate the clinicopathologic features, treatment, outcome and pattern of treatment failure in a consecutive series of patients with localized or metastatic ASPS between 1996 and 2011. Demographics, tumor sizes, sites and extent of disease, treatments provided, progression-free survival, and overall survival were evaluated. RESULTS: A total of 19 patients were identified. The clinical assumptive diagnosis of the first medical examination doctor was benign soft tissue tumor in 5 cases (26%) and benign hemangioma in 4 cases (21%), delaying treatment. The most common location of primary tumor was the thigh. The median diameter of the mass was 55 mm (range, 10 to 130 mm). An R0 resection was obtained in 11 cases. Adjuvant radiotherapy was delivered in 8 cases; postoperative systemic chemotherapy was delivered in 10 cases. Eight out of 15 patients (53%) exhibited metastases either at presentation or later. Median overall follow-up was 54 months. CONCLUSIONS: The treatment principle for alveolar soft tissue sarcoma is massive resection, and when the surgical margin is questionable, radiation treatment can be added for prevention of local recurrence. Also, due to discovery of metastases and local recurrence, even after 5 years of general treatment, outpatient department follow-up is needed, and we need to keep in mind that lung, intracranial, and bone metastases are common.

Imaging features and pathological findings of alveolar soft part sarcoma / 中华放射学杂志

Objective To explore the correlation between imaging features and pathological findings of alveolar soft part sarcoma (ASPS).Methods The clinical data,radiographic images and pathological characteristics of seven patients with histopathologically proved ASPS from 2003 to 2009 were retrospectively reviewed.Digital radiography (DR),plain CT,pre and post-contrast MR and DSA were performed in all patients.HE staining and periodic acid-Schiff (PAS) staining were used in pathological examination and CD34 were marked in immunehistochemical study.Results All patients were females.Their age ranged from 16 to 37 years with median of 24 years.Two tumors were located in pelvics,three were located in thighs,and two were located in legs.DR and CT plain scan features of ASPS were non-specific.MR demonstrated the tumors with large diameter and well defined margin locating in the deep tissues.Tumors were hyperintense on T2-weighted and T1-weighted images with many flow void vessels and persistent remarkable enhancement.There were numerous vessels with arteriovenous fistula on DSA.Pathological examination revealed solid nests of several polygonal tumor cells arranged in an alveolar pattern and separated by thinwalled,sinusoidal vascular channels.Tumor cell were noted on periodic acid-Schiff (PAS) stains.And the endothelial cells of sinusoidal vascular channels were positive on CD34.Conclusions As a malignant tumor which predominantly developed in deep location of trunk or extremities in young females,numerous void signal and hyperintensity on T1-weighted were the features of ASPS on MR.Numerous vessels could also be confirmed on DSA.These image findings were correlated closely with the pathological manifestations.

A Case of Alveolar Soft Part Sarcoma of the Pleura

Alveolar soft part sarcoma (ASPS) is a rare malignant soft-tissue neoplasm of unknown histogenesis. The two main sites of occurrence are the lower extremities in adults and the head and neck in children. We report the first case of pleural ASPS occurring in a 58-yr-old man who presented with progressive dyspnea. A computed tomographic scan of the thorax revealed a large enhancing pleural mass with pleural effusion in the left hemithorax. Wide excision of the pleural mass was performed. Histologically, the tumor consisted of organoid nests of large polygonal cells, the cytoplasm of which had eosinophilic and D-PAS positive granules. Immunohistochemical staining showed that the tumor cell nuclei were positive for transcription factor 3 (TFE3). The pleural ASPS with multiple bone metastases recurred 1 yr after surgery and the patient died of acute pulmonary embolism 1.5 yr after diagnosis.

A Case of Alveolar Soft Part Sarcoma in Floor of Mouth Mimicking Ranula / 대한이비인후과학회지

Alveolar soft part sarcoma (ASPS) is a rare, aggressive mesenchymal malignancy that comprises of approximately 1% of all soft tissue sarcomas. About 60 cases of ASPS arising in the head and neck have been reported, with most of them occuring in the orbit and tongue. An ASPS found in the mouth floor is extremely rare, with only one single case reported in english literature. Because of its rarity and slow growing feature, it can be misdiagnosed as a benign oral cavity mass or a vascular neoplasm. We herein report a new case of mouth floor ASPS, which mimicked a ranula in a child. In our patient of 12 year-old girl, tumor was located at the floor of mouth. She was successfully treated with surgical excision without adjuvant radiation or chemotherapy.

Clinical analysis of twenty-eight patients with alveolar soft part sarcoma / 中国医师进修杂志

Objective To study the clinical features of alveolar soft part sarcoma (ASPS) and assess its prognostic factors.Methods The clinical data of 28 ASPS patients from January 2006 to January 2009 were analyzed retropectively,and were followed up to get their prognosis.The survival analysis and risk factors were analyzed and compared.Results Two cases with soft tissue primary ASPS got recurrence after the first resection,the recurrence rate was 8.0％ (2/25).After re-resection,no postoperative recurrence occurred.Three cases with bone primary ASPS did not have postoperative local tumor recurrence,but 2 cases appeared tumor progression and got pulmonary metastasis.All the patients had poor overall prognosis,11 cases survived without tumor,8 cases survived with tumor,9 cases died.The median survival time was 38.5 months,5-year survival rate was 46.4％ (13/28),5-yeax survival rate of male patients was 50.0％ (5/10),female patients was 44.4％(8/18),5-year survival rate between male and female patients had no statistical significance (P =0.260).The 5-year survival rate of patients without distant metastasis was 81.8％ (9/11),while the rate of patients with distant metastasis (pulmonary,brain or bone metastasis) was only 23.5％(4/17),and the rates had significant difference (P =0.036).Age,gender had no significant relation with the prognosis,while tumor size,location and distant metastasis were the important factors affecting the prognosis.Conclusions ASPS commonly occurs in young persons.The hips and thighs are the most common sites ofsofte tissue primary ASPS.Prognosis has relation with distant metastasis,tumor size and part or thorough resection.The risk of recurrency and metastasis can be decreased through assessing disease condition as soon as possible,developing a reasonable treatment plan and wide resection of the primary tumor.

Alveolar Soft Part Sarcoma Metastasized to Both the Skull and the Brain

Alveolar soft part sarcoma (ASPS) with skull and brain metastases is extremely rare. A 53-year-old patient diagnosed as skull metastasis of ASPS visited our clinic complaining of an outgrowing scalp mass in spite of radiation therapy. Past medical history revealed that the patient had been diagnosed and treated for ASPS of the thigh 4 years ago. Magnetic resonance imaging revealed a hyperintense ovoid mass on the T2-weighted image, an isointense on the T1-weighted image, and a homogeneous enhanced mass with gadolinium. Another small-sized enhanced mass with mild peritumoral swelling was found at the deep white matter of the left frontal lobe. A gross total resection of the skull lesion with cranioplasty was performed for the surgical defect. A histologic examination of the specimens revealed metastatic ASPS involving the skull. Surgery with a total removal of the lesions may be effective for improving a patient's symptoms especially from neurological dysfunction.

Alveolar soft-part sarcoma of tongue.

Alveolar soft-part sarcoma is a clinically and morphologically distinct soft-tissue sarcoma of adolescent and young adult patients. Though immunohistochemical stains implicate a myogenic origin, the histogenesis of this tumor has not yet been established. Its high vascular nature leads to dissemination of the tumor cells into the bloodstream and metastasis. It comprises less than 1% of all soft-tissue sarcomas and less than 0.1% of sarcomas of the head and neck, preferably involving the orbit (48%) and tongue (25%). Lingual involvement is very rare and only about 31 cases have been reported in English literature. Their deceivingly indolent clinical courses often lead to misdiagnosis and delayed treatment. The reported case indicates its asymptomatic nature and microscopic similarity to granular cell tumor, which is the common benign tumor of the tongue.

Cytologic Findings of Alveolar Soft Part Sarcoma Presenting with Multiple Pulmonary Masses: A Case Report with Review of Literature

Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor of uncertain origin, and it has a strong propensity for metastasis to the lungs, bones and brain. We report upon an unusual case of ASPS, presenting as multiple lung nodules with no other detectable primary site, in a 44-year-old man. A fine needle aspiration of the nodules yielded scattered, discohesive cells, each containing an eccentrically displaced nucleus and prominent nucleolus, on a granular background. Tumor cells with numerous bared nuclei, and occasional sheets of epithelioid cells were also found. Under the cytological diagnosis of an unclassified epithelioid malignant tumor, resection of the lung nodules was performed. The histologic findings were consistent with ASPS, showing positive TFE3-nuclear immunoreactivity. There is limited literature concerning cytological findings associated with pulmonary ASPS: especially in cases where the primary site is unknown. Here, we present a cytological review of pulmonary ASPS, investigating the significance of TFE3 staining in the diagnosis of ASPS.

Alveolar soft part sarcoma: a clinicopathological analysis of 21 cases / 临床与实验病理学杂志

Purpose To investigate the clinical and pathological features and differential diagnosis of alveolar soft part sarcoma (ASPS).Methods The clinical data were retrospectively analyzed in 21 cases of ASPS. Histopathological and immunohistochemical changes of the tumors were also observed in the paraffin-embedded tissue samples.Results In 21 cases, 11 were male and 10 females. Ages of the patients ranged from 4 to 56 years old,with mean 25.9 years old.The lesions were mainly located in the deep soft tissues of legs.Microscopically,tumor cells with granular cytoplasm arranged in alveolar or solid structures,and were separated by sinusoidal vessels. There existed crystals in the cytoplasm of tumor cells after PAS staining.Immunohistochemically,10 cases were positive for MyoD1, 4 positive for desmin,9 positive for S-100,11 positive for NSE, and 11 positive for Vim. All of ASPS were negative for AE1/AE3,CK, EMA, SMA, MSA and Syn.Conclusions ASPS is a rare malignant neoplasm in adolescents and young adults. It is prone to early hematogenous metastases and a high recurrence rate after conservative surgical excision. Therefore, the prognosis of ASPS is poor. Careful analysis of the clinicopathological features and immunohistochemisitry will be helpful to reach an accurate diagnosis.