Brain Metastasis of Alveolar Soft Part Sarcoma treated Successfully with Hypothermia: A Case Report

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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Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report

World Journal of Clinical Cases ◽

10.12998/wjcc.v10.i1.296 ◽

2022 ◽

Vol 10 (1) ◽

pp. 296-303

Author(s):

Jun-Yu Chen ◽

Bo Cen ◽

Fei Hu ◽

Yong Qiu ◽

Guo-Min Xiao ◽

...

Keyword(s):

Case Report ◽

Clinical Characteristics ◽

Soft Part ◽

Alveolar Soft Part Sarcoma

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Spontaneous regression in alveolar soft part sarcoma: case report and literature review

World Journal of Surgical Oncology ◽

10.1186/1477-7819-7-53 ◽

2009 ◽

Vol 7 (1) ◽

Cited By ~ 13

Author(s):

Mohammed N. BaniHani ◽

Abdel Rahman A. Al Manasra

Keyword(s):

Case Report ◽

Literature Review ◽

Spontaneous Regression ◽

Soft Part ◽

Alveolar Soft Part Sarcoma

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Alveolar soft-part sarcoma. A case report.

The Journal of Bone and Joint Surgery (American) ◽

10.2106/00004623-198365060-00019 ◽

1983 ◽

Vol 65 (6) ◽

pp. 841-842 ◽

Cited By ~ 1

Author(s):

A E Brodsky ◽

M D Dennis ◽

W R Sassard

Keyword(s):

Case Report ◽

Soft Part ◽

Alveolar Soft Part Sarcoma

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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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