Le sarcome alvéolaire de la cuisse chez un jeune adulte: à propos d'un cas à l'hôpital Skyborne de Bukavu et revue de la littérature / NA

Les auteurs rapportent l'un des types rares des cancers, le sarcome alvéolaire; survenu chez un sujet masculin âgé de 32 ans, qui consulte pour une importante tuméfaction de la face antérieure de la racine de la cuisse gauche et dont les bilans biologiques et radiologiques n'avaient rien montré des particuliers. L'étude histologique avait posé le diagnostic d'un sarcome alvéolaire. Une exérèse radicale avait été pratiquée et le patient avait été mis sous chimiothérapie avec bonne tolérance. Le patient a été revu tous le trois mois pendant trois ans, puis une fois tous les six mois pendant la 4eme année sans qu'aucune récidive ni localisation métastasique n'ait été objectivée, avant que nous constations une récidive avec métastase pulmonaire à la 5eme année, ce qui emporta notre patient.

The authors report one of the rare types of cancers, alveolar sarcoma; occurred in a male subject aged 32 years, who consults for a large swelling of the anterior face of the root of the left thigh and whose biological and radiological assessments had shown nothing particular. The histological study diagnosed alveolar sarcoma. Radical excision was performed and the patient was put on chemotherapy with good tolerance. The patient was reviewed every three months for three years, then once every six months during the fourth year without any recurrence or metastatic localization was objectified, before we found a recurrence with pulmonary metastasis in the 5th year, which took away our patient.

Manifestaciones paraneoplásicas a propósito de un caso con sarcoma de partes blandas y metástasis ósea / Paraneoplastic manifestations regarding a case with soft tissue sarcoma and bone metastases

Introducción: El sarcoma de tejidos blandos (STB) constituye un grupo variado de más de 60 neoplasias que solo suponen 1 por ciento del total de los tumores. Objetivo: Describir el caso de un paciente con sarcoma de partes blandas con metástasis ósea y manifestaciones paraneoplásicas. Caso clínico: Se presenta el caso de un paciente masculino de 55 años de edad con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que refiere que hace 50 días inició con decaimiento marcado, luego de permanecer 1 mes con este síntoma inició con dolor en la columna en la parte más baja de la espalda, conjuntamente iniciando con un cuadro febril. Además, tuvo pérdida de la fuerza principalmente en las piernas, y hablaba cosas incoherentes sin sentido, gran inapetencia, con pérdida de peso de 50 lb en 20 días. Ante cuadro de pérdida de la conciencia y de la fuerza principalmente en las piernas se decidió su remisión hacia el Hospital Clínico Quirúrgico Lucía Íñiguez Landín de Holguín donde se concluyó como sarcoma de partes blandas con manifestaciones paraneoplásicas y metástasis ósea. Conclusiones: El caso presentado de sarcoma de partes blandas pretende aportar información sobre esta enfermedad tan infrecuente en nuestro medio(AU)

Introduction: Soft tissue sarcoma (STB) constitutes a varied group of more than 60 malignancies that only accounts for 1% of all tumors. Objective: To report a case of a patient with soft tissue sarcoma with bone metastases and paraneoplastic manifestations. Clinical case report: We report the case of a 55-year-old male patient with a history of type 2 diabetes mellitus and hypertension, who stated that 50 days ago he began to feel markedly listless. And after a month he began to suffer pain in the spine, in the lower back, and have a fever. In addition he had loss of strength mainly in the legs, and spoke inconsistently, nonsense, great loss of appetite, with weight loss of 50 lbs in 20 days. Given the condition of loss of consciousness and strength, mainly in the legs, it was decided to refer him to Lucía Iñiguez Landín Surgical Clinical Hospital in Holguín, where a soft tissue sarcoma with paraneoplastic manifestations and bone metastases was diagnosed. Conclusions: The report of this case of soft tissue sarcoma aims to provide information about this disease, which is so rare in our setting(AU)

Perfil epidemiológico de los pacientes con diagnóstico de sarcoma de partes blandas de 0 ­ 18 años en el Hospital Nacional de Niños Benjamín Bloom en el período de tiempo de enero del 2010 a diciembre del 2015 / Epidemiological profile of patients with a diagnosis of soft tissue sarcoma aged 0 - 18 years at the Benjamin Bloom National Children's Hospital in the period of time from January 2010 to December 2015

Los sarcomas de partes blandas (SPB) son un grupo heterogéneo de tumores poco frecuentes constituidos por una amplia variedad de subtipos histológicos, que representan el 7 % de los tumores malignos. Sus tasas de curación han mejorado significativamente en los últimos 30 años gracias a grupos cooperativos internacionales que desarrollan enfoques de tratamiento multidisciplinarios y adaptados al riesgo. Aproximadamente el 70% de los pacientes con SPB localizado puede curarse ahora pero su resultado se correlaciona directamente con su grupo de riesgo según lo determinado por varios factores pronósticos como histología, tamaño tumoral, invasividad local y distante, sitio del tumor y edad del paciente. El Hospital Nacional de Niños Benjamín Bloom (HNNBB), es el único hospital nacional de referencia para la atención en el departamento de Oncología del cáncer pediátrico por esta razón el propósito de esta investigación ha sido describir el perfil epidemiológico de los niños con diagnóstico de sarcoma de partes blandas de 0 ­ 18 años en el HNNBB en un período de cinco años. Cuál es el perfil epidemiológico de los niños de 0 a 18 años con sarcomas departes blandas en el Hospital Nacional de niños Benjamín Bloom

TFE3-Expressing Perivascular Epithelioid Cell Tumor of the Breast

Perivascular epithelioid cell tumor (PEComa) is a very rare mesenchymal tumor with a distinctive morphology and immunophenotype. PEComas usually harbor TSC2 alterations, although TFE3 translocations, which occur in MiT family translocation renal cell carcinoma and alveolar soft part sarcoma, are also possible. We recently experienced a case of PEComa with TFE3 expression arising in the breast. An 18-year-old female patient presented with a right breast mass. Histologically, the tumor consisted of epithelioid cells with alveolar structure and showed a diffuse strong expression of HMB45 and TFE3. TSC2 was preserved. Melan A and smooth muscle actin were negative. To our knowledge, this is the first Korean case of PEComa of the breast that intriguingly presented with TFE3 expression.

Factores que influyen negativamente en el control local de los sarcomas, estudio retrospectivo / Factors that negatively influence local control of sarcomas, retrospective study

Introducción: El rol de la cirugía y la adyuvancia con quimio y radio terapia influyen en la supervivencia y el control local de los sarcomas de partes blandas, por lo que el objetivo del presente estudio fue describir la asociación estadística en un grupo de pacientes con sarcomas de partes blandas tomando en cuenta el tamaño tumoral y la presencia de bordes libres. Métodos: El presente estudio retrospectivo, fue realizado desde enero 2008 a diciembre 2016 en el hospital Solca-Guayaquil, se incluyeron todos los casos de sarcoma de partes blandas. Las variables fueron el diagnóstico histopatológico, el tamaño tumoral, los bordes quirúrgicos, la manipulación previa, la supervivencia y la presencia de recidiva local y/o a distancia. Resultados: Fueron 156 casos, 84 hombres (54 %), de 41 a 60 años 49 casos (31 %). Liposarcoma 40 casos (25.6 %), Leimiosarcoma 25 casos (16 %), Sarcoma pleomórfico 19 casos (12.2%). Localizados en el miembro inferior 70 casos (44.9 %). El tamaño del sarcoma de 9.1 a 16 cm tuvo un Odds Ratio de 3.18 (IC95 1.26 a 7.98) P =0.01. La supervivencia fue igual entre los pacientes con y sin bordes libres Mantel-Cox P =0.26. La adyuvancia en los pacientes sin metástasis fue en 51 casos (53.1 %) y en los pacientes con metástasis fue de 48 (80 %) P<0.001. La adyuvancia en los pacientes sin recidiva fue en 47 casos (54.7 %), en los pacientes con recidiva 52 casos (74.3 %) P =0.013. La manipulación en el grupo sin recidiva fue de 9 casos (10.5 %), en el grupo con recidiva 20 casos (28.6 %) P =0.006. Conclusión: En el presente estudio los factores que influyen negativamente en el control local y sistémico de los sarcomas fueron: los tumores de tamaño de 9.1 a 16 cm, el tratamiento de Adyuvancia con Quimioterapia o Radioterapia y la manipulación previa.

Introduction: The role of surgery and adjuvance with chemo and radio therapy influence the survival and local control of soft tissue sarcomas, so the aim of the present study was to describe the statistical association in a group of patients with sarcomas of soft tissues taking into account the tumor size and the presence of free edges. Methods: The present retrospective study was carried out from January 2008 to December 2016 at the SOLCA-Guayaquil hospital, including all cases of soft tissue sarcoma. The variables were histopathological diagnosis, tumor size, surgical margins, previous manipulation, survival and the presence of local and / or distant recurrence. Results: There were 156 cases, 84 men (54 %), 41 to 60 years 49 cases (31%). Liposarcoma 40 cases (25.6 %), Leimiosarcoma 25 cases (16 %), pleomophic sarcoma 19 cases (12.2 %). Located in the lower limb 70 cases (44.9 %). The size of the sarcoma from 9.1 to 16 cm had an Odds Ratio of 3.18 (IC95 1.26 to 7.98) P = 0.01. Survival was equal between patients with and without free edges MantelCox P = 0.26. Adjuvance in patients without metastasis was 51 cases (53.1 %) and in patients with metastasis was 48 (80 %) P <0.001. Adjuvance in patients without recurrence was 47 cases (54.7 %), in patients with recurrence 52 cases (74.3 %) P = 0.013. The manipulation in the group without recurrence was 9 cases (10.5 %), in the group with recurrence 20 cases (28.6 %) P = 0.006. Conclusion: In the present study the factors that negatively influence the local and systemic control of sarcomas were: tumors of size from 9.1 to 16 cm, Adjuvant treatment with Chemotherapy or Radiotherapy and previous manipulation.

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.

Alveolar soft part sarcoma: a clinicopathologic analysis of 48 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of alveolar soft part sarcoma (ASPS).</p><p><b>METHODS</b>The clinical data and pathologic features of 48 cases of ASPS were evaluated. Immunohistochemical study, PAS staining and fluorescence in-situ hybridization (FISH) were carried out in selected examples. Relevant literature was reviewed.</p><p><b>RESULTS</b>Amongst the 48 cases studied, there were 17 males and 31 females, with male-to-female ratio of 1.0∶1.8. The age of patients ranged from 2 to 60 years (median=26 years). The tumor was most commonly located in deep soft tissue, especially that of lower extremities. Histologically, the tumor cells were arranged in alveolar or solid patterns and separated by sinusoidal vessels. They were large and contained abundant eosinophilic granules or crystals in cytoplasm. The nuclei were round to polygonal and vesicular, often with prominent nucleoli. Intravascular tumor extension was common. Some cases showed necrosis, hemorrhage and cystic changes. Immunohistochemical study showed that the tumor cells were positive for TFE3 (100%, 33/33). FISH assay was carried out in 4 cases and all of them had TFE3-ASPL gene fusion.</p><p><b>CONCLUSIONS</b>ASPS is a rare malignant neoplasm, often occurs in young patients. TFE3 is a useful immunohistochemical marker for diagnosis. The diagnosis is further confirmed by other markers.</p>

Alveolar soft part sarcoma of the larynx: a case report / 临床耳鼻咽喉头颈外科杂志

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcomas, this sarcoma occurs rarely in the larynx. Herein, we describe an unusual case of ASPS occurring in the larynx. The patient was a 46-year-old woman who presented with a more than 2-year history of right pyriform fossa mass and progressive hoarseness for three months. The endoscopic examination of the larynx revealed a mass in the right pyriform fossa with smooth surface and well-defined margin. Computed tomography demonstrated a heterogeneous tumor in the right pyriform fossa, and involving the paralaryngeal space, measuring approximately 2.1 cm x 1.7 cm x 2.6 cm, which was resected via lateral neck approach. Histopathology and immunohistochemistry revealed an ASPS. To ours knowledge, this is the first reported case of ASPS arising in pyriform fossa.

Dermatofibrosarcoma protuberans / Dermatofibrosarcoma protuberans

El dermatofibrosarcoma protuberans es un sarcoma de partes blandas, de malignidad intermedia y lento crecimiento. Afecta adultos jóvenes y a todas las etnias, aunque algunos trabajos observan cierta predilección por la etnia negra. La localizaciónhabitual es en tronco, extremidades proximales y en menor medida encabeza y cuello. El tratamiento de elección es la exéresis quirúrgica con preferencia por la cirugía micrográfica de Mohs. Presentamos seis pacientes con diagnóstico clínico e histopatológico de dermatofibrosarcoma protuberans, 2 mujeres y 4 varones de entre 24 a 52 años. Hasta el momento, 5 de ellos recibieron tratamientoquirúrgico (técnica micrográfica de Mohs en 4 pacientes y cirugía convencional en el restante). No se observaron recidivas en un período de seguimiento promediode 20 meses...

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: 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.

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.

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.

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.

Treatment and prognosis of stage IV alveolar soft part sarcoma / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To analyze the clinical features, treatment and prognosis of Stage IV alveolar soft part sarcoma.</p><p><b>METHODS</b>To analyze the clinical and pathological features, therapeutic methods and follow-up results in 21 patients with stage IV alveolar soft part sarcoma. There were 11 males and 10 females, in the age of 26-57 years (average 37.0 years old). All the 21 patients had metastasis: nine cases had multiple pulmonary metastasis, three cases had multiple pulmonary and brain metastasis, two cases had multiple brain metastasis, two cases had multiple pulmonary and bone metastasis, two cases had single pulmonary metastasis, one case had single bone metastasis, one case had single brain metastasis and one case had single soft tissue metastasis. Eight patients were treated by surgical operation, including five cases of complete resection for the primary and (or) metastatic tumor and 3 cases of palliative operation for the primary tumor. All patients received chemotherapy, including seven cases of CAVD regimen and 14 cases of MAID regimen treatment. One patient with single bone metastasis and five patients with multiple brain metastasis received post-operative whole brain radiation therapy.</p><p><b>RESULTS</b>All the eight patients with surgical operation had healing by first intention, and pathological examination showed that seven patients achieved R0 surgical margin and one case with R2 status. One patient with single brain metastasis had recurrence after operation. The toxic and adverse reactions of all patients treated with chemotherapy were tolerable. Among them, 17 cases had stable disease and 4 cases had disease progression after chemotherapy. The disease control rate (DCR) was 81.0%. The DCR of patients with CAVD regimen chemotherapy was 85.7% and that of patients treated with MAID regimen was 78.6% (P = 0.862). All patients were followed up for 8 - 86 months (average 32.4 months). The median survival time of all patients was 32.6 months. The 2-year survival rate was 55.1% and the 5-year survival rate was 21.8%. The median survival time in the patients with complete resection was 60.0 months, and that in patients with palliative operation was 27.0, showing a significant difference between them (P = 0.048). The median progression-free survival in patients with complete excision was 57.2 months and that in patients with palliative operation or without operation was 19.6 months, with a significant difference (P = 0.029). The median survival time in patients who received CAVD regimen chemotherapy was 30.0 months, and that in patients with MAID regimen was 51.0 months, with a non-significant difference (P = 0.511). The median progression-free time in patients with CAVD regimen chemotherapy was 13.0 months, and that in patients with MAID regimen was 38.0 months, also with a non-significant difference (P = 0.066).</p><p><b>CONCLUSIONS</b>Alveolar soft part sarcomas are rarely seen and highly malignant tumors, and the prognosis of stage IV ASPS is poor. Complete resection of all tumors is the key of successful treatment of Stage IV ASPS, and the site and number of tumor metastasis are important factors affecting prognosis. The curative effects of radiotherapy and chemotherapy for ASPS need to be further investigated.</p>

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.

Recurrent Alveolar Soft-Part Sarcoma With Concurrent Involvement of the Orbit and Multiple Sites of the Body

PURPOSE: To report a rare case of recurrent alveolar soft-part sarcoma (ASPS) with concurrent involvement of the orbit and multiple sites of the body that was removed successfully by surgery. CASE SUMMARY: A 37-year-old woman presented with diplopia at the left lateral gaze and proptosis of the left eye. Two years earlier, the patient had a mass excision of the right gluteus maximus and the left orbit, and ASPS was histopathologically diagnosed at that time. In addition, the patient had been treated with chemotherapy and radiation therapy. On radiologic examination, recurrent tumor of the left orbit was found and surgically removed. The histopathologic examination showed that tumor cells were arranged in an alveolar pattern divided by fibrous septa and contained abundant granules in the cytoplasm, typically consistent with ASPS. Postoperatively, the symptoms of diplopia and proptosis improved. CONCLUSIONS: ASPS can occur and recur in the orbit as well as systemically. In addition, at the time of surgical removal of ASPS in the orbit, the surgeon should be particularly cautious of massive bleeding.