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Background Alveolar soft part sarcoma (ASPS) is a rare histological subtype of soft-tissue sarcoma, which remains refractory to conventional cytotoxic chemotherapy. We aimed to characterize ASPS and investigate whether the oncological outcome has improved over the past decade. Methods One hundred and twenty patients with newly diagnosed ASPS from 2006 to 2017, identified from the Bone and Soft-Tissue Tumor Registry in Japan, were analyzed retrospectively. Results The study cohort comprised 34 (28%) patients with localized ASPS and 86 (72%) with metastatic disease at presentation. The 5-year disease-specific survival (DSS) was 68% for all patients and 86% and 62% for localized and metastatic disease, respectively (p = 0.019). Metastasis at presentation was the only adverse prognostic factor for DSS (hazard ratio [HR]: 7.65; p = 0.048). Patients who were > 25 years (80%; p = 0.023), had deep-seated tumors (75%; p = 0.002), and tumors > 5 cm (5-10 cm, 81%; > 10 cm, 81%; p < 0.001) were more likely to have metastases at presentation. In patients with localized ASPS, adjuvant chemotherapy or radiotherapy did not affect survival, and 13 patients (45%) developed distant metastases in the lung (n = 12, 92%) and brain (n = 2, 15%). In patients with metastatic ASPS (lung, n = 85 [99%]; bone, n = 12 [14%]; and brain n = 9 [11%]), surgery for the primary or metastatic site did not affect survival. Prolonged survival was seen in patients who received pazopanib treatment (p = 0.045), but not in those who received doxorubicin-based cytotoxic chemotherapy. Overall, improved DSS for metastatic ASPS has been observed since 2012 (5-year DSS, from 58 to 65%) when pazopanib was approved for advanced diseases, although without a statistically significant difference (p = 0.117). Conclusion The national study confirmed a unique feature of ASPS with frequent metastasis to the lung and brain but an indolent clinical course. An overall trend toward prolonged survival after the introduction of

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., ntroducción. 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.

Background Alveolar soft part sarcoma (ASPS) is a rare histological subtype of soft-tissue sarcoma, which remains refractory to conventional cytotoxic chemotherapy. We aimed to characterize ASPS and investigate whether the oncological outcome has improved over the past decade. Methods One hundred and twenty patients with newly diagnosed ASPS from 2006 to 2017, identified from the Bone and Soft-Tissue Tumor Registry in Japan, were analyzed retrospectively. Results The study cohort comprised 34 (28%) patients with localized ASPS and 86 (72%) with metastatic disease at presentation. The 5-year disease-specific survival (DSS) was 68% for all patients and 86% and 62% for localized and metastatic disease, respectively (p = 0.019). Metastasis at presentation was the only adverse prognostic factor for DSS (hazard ratio [HR]: 7.65; p = 0.048). Patients who were > 25 years (80%; p = 0.023), had deep-seated tumors (75%; p = 0.002), and tumors > 5 cm (5-10 cm, 81%; > 10 cm, 81%; p < 0.001) were more likely to have metastases at presentation. In patients with localized ASPS, adjuvant chemotherapy or radiotherapy did not affect survival, and 13 patients (45%) developed distant metastases in the lung (n = 12, 92%) and brain (n = 2, 15%). In patients with metastatic ASPS (lung, n = 85 [99%]; bone, n = 12 [14%]; and brain n = 9 [11%]), surgery for the primary or metastatic site did not affect survival. Prolonged survival was seen in patients who received pazopanib treatment (p = 0.045), but not in those who received doxorubicin-based cytotoxic chemotherapy. Overall, improved DSS for metastatic ASPS has been observed since 2012 (5-year DSS, from 58 to 65%) when pazopanib was approved for advanced diseases, although without a statistically significant difference (p = 0.117). Conclusion The national study confirmed a unique feature of ASPS with frequent metastasis to the lung and brain but an indolent clinical course. An overall trend toward prolonged survival after the introduction of

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., 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 is a rare soft tissue tumor with uncertain histogenesis. It is a slow growing tumor with a high rate of metastasis. The tumor is not easily identified as clinical symptoms are not pronounced. The retroperitoneum is a rare location of tumor, with a few cases published in literature. Surgical excision is the mainstay of treatment. Here we describe a rare case of a large retroperitoneal Alveolar soft part sarcoma in a young female with radiological and histopathological findings.

Alveolar soft part sarcoma is a rare soft tissue tumor with uncertain histogenesis. It is a slow growing tumor with a high rate of metastasis. The tumor is not easily identified as clinical symptoms are not pronounced. The retroperitoneum is a rare location of tumor, with a few cases published in literature. Surgical excision is the mainstay of treatment. Here we describe a rare case of a large retroperitoneal Alveolar soft part sarcoma in a young female with radiological and histopathological findings.