Ewha Med J.  2016 Jul;39(3):89-92. 10.12771/emj.2016.39.3.89.

Durable Response to Pazopanib in a Patient with Metastatic Alveolar Soft Part Sarcoma

• Han J
• Na II
• Jung MW
• Lee SH
• An JW
• Koh JS

• KMID: 2344898
• DOI: http://doi.org/10.12771/emj.2016.39.3.89

Abstract

Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma, and frequently, metastases are found at diagnosis. In patients with metastatic or unresected ASPS, systemic treatment is extremely limited, because conventional chemotherapeutic agents have not been effective in most cases. A novel agent inhibiting angiogenesis, pazopanib, has been proven to be effective for metastatic soft tissue sarcoma in a second-line setting. However, the efficacy of pazopanib in ASPS has not yet been reported. A 22-year-old man presented with right calf ASPS and multiple lung metastases. Pazopanib as a second-line treatment showed significant tumor response. To the best of our knowledge, this is the first report of the effectiveness of pazopanib in ASPS.

Keyword

Alveolar soft part sarcoma; Neoplasm metastasis; Pazopanib

MeSH Terms

Diagnosis
Humans
Lung
Neoplasm Metastasis
Sarcoma
Sarcoma, Alveolar Soft Part*
Viperidae
Young Adult

Figure

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  Fig. 1 PET image. It positron emission tomography shows hypermetabolic mass (arrow) in the right calf.

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  Fig. 2 Light microscopic findings. The tumor cells have plump eosinophilic granular cytoplasm and atypical nuclei with prominent nucleoli (H&E, ×400).

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  Fig. 3 Chest CT images. (A) Prior to pazopanib treatment, chest computed tomography (CT) in May 2015 shows multiple small metastatic nodules in both lungs. (B) Follow-up chest CT after 2 months of pazopanib treatment demonstrates that multiple small metastatic nodules in both lungs decreased in size. (C) Follow-up chest CT after 8 months of pazopanib treatment shows durable response in chemotherapy.

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