Alveolar soft part sarcoma in childhood and adolescence: Report of three cases and review of literature

Abstract

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma with unique tumor characteristics, which is rare in children. Herein, we present the immunophenotype, treatment, and prognosis of three children with ASPS from The Second Xiangya Hospital of Central South University, and 51 children with ASPS have been reported in the previous literature, along with a focused review of the clinical features, pathological features, differential diagnosis, treatment, and prognosis of ASPS in pediatric patients.

The dynamic contrast enhanced-magnetic resonance imaging and diffusion-weighted imaging features of alveolar soft part sarcoma

“…Compared with CT, MRI is the preferred imaging modality to assess this lesion due to its advantages of multisequence imaging capability and excellent soft tissue contrast. Previous studies have reported that, compared with adjacent muscles, ASPS exhibited isointensity or a slightly high signal intensity on T1WI, which was believed to be associated with the slow flow of blood in or around the tumors, and heterogenous hyperintensity on T2WI, which might be related to bleeding, necrosis, and scar formation in the tumor tissue ( 5 , 7 , 14 ). Furthermore, researchers have also noted that intra- and extra-tumoral vascular signal voids are frequently observed on MRI, which is attributed to tortuous and dilated vessels with rapid blood flow washout ( 22 ).…”

“…Therefore, it is difficult to make an accurate diagnosis due to the limited experience with clinical characteristics. Previous studies have shown that ASPS mainly affects adolescents and young adults between the ages of 15 and 35 years ( 13 , 14 ). There is a slight female predominance in patients under the age of 30 years, whereas the opposite phenomenon has been found in patients above 30 ( 13 ).…”

“…There is a slight female predominance in patients under the age of 30 years, whereas the opposite phenomenon has been found in patients above 30 ( 13 ). ASPS can occur in all body parts, mostly in the deep soft tissues of the thighs and buttocks in adults, whereas it is mainly found in the head and neck in infants and children, especially in the orbit and tongue ( 14 , 15 ). In agreement with previous observations, our study showed that the median patient age at diagnosis was 23.50±6.36 years (9–49 years), and a majority (21/34) of the patients were aged less than 30 years.…”

“…Long-term survival of patients without metastases at presentation is longer than those presenting with single or multiple sites of metastasis. Currently, radical surgical resection is the only validated standard treatment, as the views on adjuvant chemotherapy and auxiliary radiotherapy are controversial ( 14 , 16 , 18 ). Thus, early diagnosis of ASPS with or without distant metastases is essential for therapeutic planning and improving prognosis.…”

Alveolar soft part sarcoma: Prolonged response to immune checkpoint inhibitors

“…It tends to be slowgrowing tumor detected at a low stage without metastatic spread, allowing complete surgical resection. 2 For patients with metastatic disease, the median survival is over 3 years with median progression-free survival between 4 and 12 months. [3][4][5] Chemotherapy does not have activity against ASPS, and for decades the standard of care was surgical resection including metastatectomy.…”