Alveolar soft tissue sarcoma: a report of 50 cases at a single institution

Zhao, Pengyuan; Li, Huixiang; Ren, Hongzheng

doi:10.1080/00015458.2022.2049060

Cited by 3 publications

(5 citation statements)

References 28 publications

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“…Fourteen articles (case series or original research) focusing on ASPS imaging were found, with a total of 151 patients included [2,3,[16][17][18][19][20][21][22][23][24][25][26][27][28]. We included studies with tumor location both at the extremities and in any other body region.…”

Section: Literature Review Resultsmentioning

confidence: 99%

“…ASPS is an ultra-rare subtype of STS of uncertain histogenesis, predominantly involving deep soft tissue of the extremities and mainly affecting the young population [30]. Chemotherapy is ineffective in the treatment of this neoplasm, while radiotherapy may play a role in reducing the risk of local recurrences after surgery [2]. Partial response to tyrosine kinase inhibitors, alone or in combination with checkpoint inhibitors or trabectedin, has been reported [31][32][33].…”

Section: Discussionmentioning

confidence: 99%

“…Alveolar soft part sarcoma (ASPS) is an extremely rare soft-tissue sarcoma (STS) subtype, first described by Christopherson et al (1952) [1]. It accounts for less than 1% of all STSs, usually affecting the extremities of children and young adults [2]. It is a very aggressive tumor characterized by poor prognosis, as well as a scarce response to radiation therapy and cytotoxic chemotherapy [2][3][4].…”

Section: Introduction 1general Characteristics and Epidemiologymentioning

confidence: 99%

“…It accounts for less than 1% of all STSs, usually affecting the extremities of children and young adults [2]. It is a very aggressive tumor characterized by poor prognosis, as well as a scarce response to radiation therapy and cytotoxic chemotherapy [2][3][4]. Surgical resection is the standard treatment based on complete resection with wide margins.…”

Section: Introduction 1general Characteristics and Epidemiologymentioning

confidence: 99%

“…Surgical resection is the standard treatment based on complete resection with wide margins. Median survival time is about 3 years in metastatic patients at baseline versus 11 years in patients with locally advanced tumors only [2,3,5].…”

Section: Introduction 1general Characteristics and Epidemiologymentioning

confidence: 99%

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Imaging Features of Alveolar Soft Part Sarcoma: Single Institution Experience and Literature Review

Spinnato,

Papalexis,

Colangeli

et al. 2023

Clinics and Practice

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Alveolar soft part sarcoma (ASPS) is an extremely rare and aggressive soft-tissue sarcoma (STS) subtype with poor prognosis and limited response to radiation therapy and chemotherapy. Prompt recognition and referral to sarcoma centers for appropriate management are crucial for patients’ survival. The purpose of this study was to report ASPS pre-treatment imaging features and to examine the existing literature on this topic. Twelve patients (7 women, 5 men—mean age 27.1 ± 10.7 years) were included from our single-center experience. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) available were reviewed according to an analysis grid incorporating features from the latest research on STS. Clinical, histological, and outcome data were collected. MRI was available in 10 patients (83.3%), US in 7 patients (58.3%), and CT in 3 patients (25%). Mean longest tumor diameter was 7.6 ± 2.9 cm, and all tumors were deeply seated. Large peritumoral feeding vessels were systematically found and identified on ultrasonography (7/7), MRI (10/10), and CT (3/3). US revealed a well-defined heterogeneous hypoechoic pattern, with abundant flow signals in all patients (7/7). In all patients, MRI showed mildly high signal intensity (SI) on T1-WI and high SI on T2-WI and peritumoral edema. Moreover, flow-voids (due to arteriosus high-flow) into the peritumoral/intratumoral feeding vessels were detected in the MRI fluid-sensitive sequences of all patients. At baseline, whole-body contrast-enhanced CT revealed metastases in 8/12 (66.7%) patients. A pre-treatment longest diameter > 5 cm was significantly associated with distant metastases at diagnosis (p = 0.01). A maximum diameter > 5 cm represents a risk of metastatic disease at diagnosis (odds ratio = 45.0000 (95% CI: 1.4908—1358.3585), p = 0.0285). In the comprehensive literature review, we found 14 articles (case series or original research) focusing on ASPS imaging, with a total of 151 patients included. Merging our experience with the data from the existing literature, we conclude that the hallmark of ASPS imaging at presentation are the following characteristics: deep location, a slight hyperintense MRI SI on T1-WI and a hyperintense SI on T2-WI, numerous MRI flow voids, high internal vascularization, and large peritumoral feeding vessels.

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