Alveolar soft part sarcoma: MR and angiographic findings

Suh, Jin‐Suck; Cho, Jaemin; Lee, Sang Hoon; Shin, Kyoo-Ho; Yang, Woo Ick; Lee, Jeong Hoon; Cho, Jaehyun; Suh, Kyung Jin; Lee, Young Joon; Ryu, Kyung Nam

doi:10.1007/s002560000285

Cited by 77 publications

(68 citation statements)

References 15 publications

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“…The tumor exhibits a slight female preponderance in patients ≤30 years old, in contrast to a male preponderance in patients >30 years old. The average age of onset falls within the second decade of life (2,3).…”

Section: Discussionmentioning

confidence: 99%

Magnetic resonance imaging and computed tomography features of alveolar soft-part sarcoma in the right deltoid muscle: A case report

Sun

et al. 2016

Oncology Letters

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Abstract. Alveolar soft-part sarcoma (ASPS) is a rare, highly vascular malignant soft-tissue tumor that predominantly affects young adults. The tumor can occur in any bodily region, but is most frequently observed in the lower deep soft tissues of the extremities, and is rarely observed in the upper extremities. The present study reported a case of ASPS of the right deltoid muscle. The patient presented with a 3-year history of a mass on the right shoulder that exhibited rapid growth in the month prior to diagnosis. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed. The lesion mainly demonstrated isointense or mildly hyperintense signals compared with the muscle on the T1-weighted images of the MRI, and heterogeneous high signal intensity on the T2-weighted images. CT enhancement showed a homogeneous enhanced mass. The tumor was resected and submitted for histopathological examination. The diagnosis was verified as ASPS by microscopic examination and immunohistochemical analysis. No distant metastases were noted. No evidence of local tumor recurrence was seen at 6 weeks following the wide surgical excision. The CT scan revealed no metastatic nodules in either lung during the follow-up. ASPS should be considered as a possible diagnosis when a slow-growing, large mass is detected in young adults in the soft tissue of the extremities, with high signal intensity and numerous signal voids on T1-weighted images (T1WI) and T2WI, and strong contrast-enhancement. IntroductionAlveolar soft-part sarcoma (ASPS) is an infrequently encountered type of soft-tissue sarcoma that usually develops in the soft tissues of the extremities. The tumor accounts for <1% of soft-tissue sarcomas and predominantly affects teenagers and young adults (1). ASPS has a high propensity for metastasis and the lungs are the most frequent site of metastasis by the hematogenous route. Although prolonged survival is possible even in patients with metastasis, the long-term disease specific mortality rate is high. The prognosis of ASPS is poor due to the high frequency of metastatic disease. A large study indicated that the median survival time was 3 years if metastatic disease was present at diagnosis and 11 years without metastatic disease at presentation (1,2). ASPS is a rare, aggressive malignancy of uncertain histological origin, with a predisposition towards vascular invasion and distant metastasis. ASPS is most commonly observed in the extremities (1), but most commonly in the lower rather than the upper limbs. A previous study reported that ASPS is most commonly identified in the lower extremities (44%) and is rarely seen in the upper extremities (2).Magnetic resonance imaging (MRI) is the favored imaging modality for evaluation of this lesion due to its excellent soft tissue contrast, multiplanar imaging capability and lack of radiation exposure. Computed tomography (CT) enhancement and CT angiography clearly showed rich blood vessels within and around the tumor. The present study reports a case of ASPS of the right d...

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Section: Discussionmentioning

confidence: 99%

Magnetic resonance imaging and computed tomography features of alveolar soft-part sarcoma in the right deltoid muscle: A case report

Sun

et al. 2016

Oncology Letters

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“…MRI obtained using axial T1WI before and after contrast administration. For sites of involvement darker gray indicates higher frequency resulting in hypervascular lesions with prominent draining veins on angiography or MRA [39]. ASPS are usually heterogeneous, well-circumscribed lesions with high SI on T1WI and T2WI and intense inhomogeneous contrast enhancement [10].…”

Section: Epithelioid Sarcomamentioning

confidence: 99%

Soft tissue sarcomas at a glance: clinical, histological, and MR imaging features of malignant extremity soft tissue tumors

et al. 2009

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“…10 It also has an unusual pattern of metastatic spread for a sarcoma, with the brain being a common site of metastases. 1 While the radiological characteristics of primary ASPS have been described in the literature, 11,12 imaging features of its metastatic pattern with respect to distribution and morphology are not widely reported. The purpose of our study was to describe the imaging features of ASPS with emphasis on the metastatic pattern.…”

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confidence: 99%

Imaging features of primary and metastatic alveolar soft part sarcoma: single institute experience in 25 patients

Sood¹,

Baheti²,

Shinagare³

et al. 2014

BJR

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Objective: To describe imaging features of primary and metastatic alveolar soft part sarcoma (ASPS). Methods: In this institutional review board-approved and Health Insurance Portability and Accountability Actcompliant retrospective study, 25 patients (14 males; mean age, 25 years; range, 18-40 years) with pathologically proven ASPS seen at our institute between 1995 and 2013 were included. Imaging of primary tumours in 5 patients and follow-up imaging in 25 patients were reviewed by 2 radiologists in consensus. Clinical information was obtained from electronic medical records. Results: The most common sites for the primary tumour were extremities (17/25, 68%) and torso (6/25, 24%). Primary tumours (n 5 5) were well circumscribed, compared with skeletal muscle, were isodense on CT, hyperintense on T 1 and T 2 weighted images with intense post-contrast enhancement, prominent feeders on CT and flow voids on MRI. Metastases developed in 23/25 (92%) patients, 18 at presentation. The most common sites of metastases were the lungs (100%), lymph nodes (74%), bones (57%) and brain (43%). Visceral and nodal metastases were hypervascular. At the time of reporting the results, 15 patients have died, 6 are alive and 4 were lost to follow-up. Median survival was 74 months for those without brain metastases (n 5 8) and 60 months for those with brain metastases (n 5 7). Median survival was shorter for patients with metastases at presentation. Conclusion: ASPS most commonly involves the lower extremities of young adults, is hypervascular on imaging, often metastasizes at presentation, frequently to lung, nodes, bones and brain, and has an indolent course despite metastases. Brain metastases and high tumour burden (number of metastatic sites) at presentation decreased survival in our study. Advances in knowledge: ASPS has an unusual pattern of metastases to the brain and nodes in addition to lung and bones. It has an indolent course despite metastases.

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Alveolar soft part sarcoma: MR and angiographic findings

Cited by 77 publications

References 15 publications

Magnetic resonance imaging and computed tomography features of alveolar soft-part sarcoma in the right deltoid muscle: A case report

Magnetic resonance imaging and computed tomography features of alveolar soft-part sarcoma in the right deltoid muscle: A case report

Soft tissue sarcomas at a glance: clinical, histological, and MR imaging features of malignant extremity soft tissue tumors

Imaging features of primary and metastatic alveolar soft part sarcoma: single institute experience in 25 patients

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