Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients

Abstract Context Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor most commonly occurring in deep intramuscular plane of lower extremities of adolescents and young adults. It is a highly vascular, slow growing tumor with malignant potential having lung as the most common site of metastases at the time of presentation. Aims The aim is to review the imaging findings of ASPS and determine characteristic imaging features of this rare tumor. Materials and Methods Sixteen patients having histopathological diagnosis and preoperative imaging of ASPS attending the dedicated sarcoma clinic at our institute were included in the study. The demographic, clinical, and imaging data were retrieved from the case records and then evaluated for characteristic imaging features which may raise suspicion of ASPS. Results The patients ranged from 3 to 72 years of age and with a slight male preponderance. Of the eight CECTs evaluated, 62.5% tumors showed well-defined lobulated margins, 87.5% cases showed intense enhancement with presence of feeder vessels. On CEMRI of 10 patients, 70% had well circumscribed lobulated margins with intense enhancement and tortuous flow voids in most of them. All cases showed T2 hyperintense signal. Fourteen of 16 (87.5%) patients had metastatic disease with lung as the most common site (92.8%). Conclusion ASPS is a rare soft tissue sarcoma seen in children and young adults. Imaging may mimic a vascular malformation due to the presence of tortuous feeders. Misdiagnosis at an early stage may lead to later metastatic presentation of the disease, thus emphasizing the need to suspect it on imaging.

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Alveolar soft part sarcoma from soft tissue and bone. A single reference hospital experience

10.26226/morressier.5b4709866f4cb30010952082 ◽

2018 ◽

Author(s):

Ignacio Ruz-Caracuel ◽

Itziar de La Peña Navarro

Keyword(s):

Soft Tissue ◽

Soft Part ◽

Alveolar Soft Part Sarcoma ◽

Hospital Experience ◽

Reference Hospital

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Imaging features of hemangioma in long tubular bones

BMC Musculoskeletal Disorders ◽

10.1186/s12891-020-03882-2 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Lei Cao ◽

Jin-Xu Wen ◽

Shu-Man Han ◽

Hui-Zhao Wu ◽

Zhi-Gang Peng ◽

...

Keyword(s):

Soft Tissue ◽

Plain Radiograph ◽

Ground Glass Opacity ◽

Plain Radiography ◽

Long Bone ◽

Imaging Features ◽

Mri Imaging ◽

Soft Tissue Density ◽

Tissue Density ◽

Hyperintense Signal

Abstract Background To investigate the imaging features of hemangiomas in long tabular bones for better diagnosis. Methods Twenty-four patients with long bone hemangiomas confirmed by pathology were enrolled. Nineteen patients had plain radiography, fourteen patients had computed tomography (CT) and eleven had magnetic resonance imaging (MRI). The hemangioma was divided into medullary [13], periosteal [6] and intracortical type [5]. Results Among 19 patients with plain radiography, eleven patients were medullary, three periosteal, and five intracortical. In the medullary type, the lesion was primarily osteolytic, including five cases with irregular and unclear rims and one lesion having osteosclerotic and unclear rims. In three patients with the periosteal type, the lesion had clear rims with involvement of the cortical bone in the form of bone defect, including two cases with local thickened bone periosteum and one case having expansile periosteum. Five intracortical hemangiomas had intracortical osteolytic lesions with clear margins. Among 14 patients with CT imaging, 8 cases were medullary, three periosteal, and three intracortical. Among 8 medullary hemangiomas, one had ground glass opacity, and seven had osteolytic, expansile lesions like soft tissue density with no calcification. In three periosteal cases, the lesion was osteolytic with thickened periosteum and narrowed medullary cavity. In three intracortical hemangiomas, the lesion was of even soft tissue density with no calcification. Among 11 patients with MRI imaging, seven were medullary, two periosteal, and two intracortical. Among 7 medullary lesions, six were of hypointense signal on T1WI and hyperintensesignal on T2 WI. In two periosteal cases, the periosteum was thickened, with one case being of equal signal, and the other having no signal. Two intracortical hemangiomas were both of slightly low signal on T1WI but hyperintense signal on T2WI. Conclusions The long bone hemangiomas had characteristic cystic honeycomb-like presentations in plain radiograph. CT and MRI imagings are helpful for diagnosis of hemangiomas in long bone.

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A case of multiple synchronously diagnosed brain metastases from alveolar soft part sarcoma without concurrent lung involvement

Surgical Neurology International ◽

10.25259/sni_554_2020 ◽

2021 ◽

Vol 12 ◽

pp. 111

Author(s):

Mark A. Damante ◽

Kristin M. Huntoon ◽

Joshua D. Palmer ◽

David A. Liebner ◽

James Bradley Elder

Keyword(s):

Soft Tissue ◽

Brain Metastasis ◽

Lung Metastasis ◽

Soft Tissue Mass ◽

Soft Part ◽

Primary Lesion ◽

Alveolar Soft Part Sarcoma ◽

Lung Involvement ◽

Tissue Mass ◽

Intracranial Involvement

Background: Alveolar soft part sarcoma (ASPS) is a rare soft-tissue sarcoma with a propensity for early hematogenous dissemination to the lungs and frequent brain metastasis. The development of lung metastasis almost invariably precedes intracranial involvement. There are no previously reported cases in which a patient was synchronously diagnosed with ASPS and multiple brain metastasis without lung involvement. Case Description: A 29-year-old gentleman was found to have three intracranial lesions following the onset of generalized seizures. Staging studies identified a soft-tissue mass in the left thigh and an adjacent femoral lesion. Biopsy of the soft-tissue mass was consistent with ASPS. The patient then underwent neoadjuvant stereotactic radiotherapy to all three brain lesions, followed by en bloc resection of the dominant lesion. The patient was then started on a programmed death-ligand 1 (PD-L1) inhibitor. Subsequent surgical resection of the primary lesion and femur metastasis demonstrates a histopathologic complete response of the bony metastasis and partial response of the primary lesion. At present, the patient has received 14 cycles of atezolizumab without recurrence of the primary or bony lesions and the irradiated intracranial disease has remained stable without recurrence of the resected dominant lesion. Conclusion: While intracranial involvement is relatively common in ASPS, a case with multiple, synchronously diagnosed brain metastasis without concurrent lung metastasis has not been described. The presented case discusses the safety and efficacy of aggressive management of intracranial disease in the setting of atezolizumab. Prospective evaluation of the efficacy of checkpoint inhibitors and the prognostic value of PD-L1 expression in ASPS with brain metastasis are necessary.

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Alveolar soft part sarcoma in children and young adults: A report of 69 cases

Pediatric Blood & Cancer ◽

10.1002/pbc.26953 ◽

2018 ◽

Vol 65 (5) ◽

pp. e26953 ◽

Cited By ~ 20

Author(s):

Ricardo J. Flores ◽

Douglas J. Harrison ◽

Noah C. Federman ◽

Wayne L. Furman ◽

Winston W. Huh ◽

...

Keyword(s):

Young Adults ◽

Soft Part ◽

Alveolar Soft Part Sarcoma ◽

Children And Young Adults

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Alveolar Soft-Part Sarcoma in Olfactory Cleft: A Case Report and Clinicopathological Review

Ear Nose & Throat Journal ◽

10.1177/0145561320955134 ◽

2020 ◽

pp. 014556132095513

Author(s):

Saud Alromaih ◽

Saleh Alqaryan ◽

Saleh Alabood ◽

Somaya Alabaishi ◽

Abdulrazag Ajlan ◽

...

Keyword(s):

Case Report ◽

Soft Tissue ◽

Malignant Disease ◽

Clinical Presentation ◽

Soft Part ◽

Soft Tissue Sarcomas ◽

Alveolar Soft Part Sarcoma ◽

Olfactory Cleft

Sinonasal alveolar soft-part sarcoma (ASPS) is a rare malignant disease that comprises 0.4% to 1.2% of all soft-tissue sarcomas. Alveolar soft-part sarcoma is usually difficult to diagnose because it has many clinical and pathological mimickers. In this case study, ASPS occurred in the olfactory cleft, representing the second case in this location in the literature. This article presents the clinical presentation, radiologic, and histopathological case, and reviews the literature regarding the differentials.

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