2016
DOI: 10.2169/internalmedicine.55.6199
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Intimal Sarcoma of the Pulmonary Artery Treated with Pazopanib

Abstract: Intimal sarcoma is a rare disease with a poor prognosis. We herein report the case of a 71-year-old man with intimal sarcoma of the pulmonary artery treated with pazopanib. The tumor showed regression after 1 month of treatment. Hand-foot syndrome led to cessation of pazopanib, which triggered a disease flare. Pazopanib should be considered in patients with intimal sarcoma of the pulmonary artery that is unresectable or recurrent after surgery or cytotoxic chemotherapy. We must be careful about drug cessation,… Show more

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Cited by 17 publications
(21 citation statements)
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“…The previous data available are confined to case reports and small retrospective series, do not always report MDM2 status, and suggest a poor activity of chemotherapy in InS, with few anecdotal responses observed . In our series, which included only confirmed MDM2‐positive cases, InS showed sensitivity to anthracycline‐based regimens, possibly greater than expected in other soft tissue sarcomas and to what has been reported previously by Van Dievel and Penel, who observed no responses over 5 InS patients each .…”
Section: Discussionsupporting
confidence: 47%
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“…The previous data available are confined to case reports and small retrospective series, do not always report MDM2 status, and suggest a poor activity of chemotherapy in InS, with few anecdotal responses observed . In our series, which included only confirmed MDM2‐positive cases, InS showed sensitivity to anthracycline‐based regimens, possibly greater than expected in other soft tissue sarcomas and to what has been reported previously by Van Dievel and Penel, who observed no responses over 5 InS patients each .…”
Section: Discussionsupporting
confidence: 47%
“…In contrast to angiosarcoma, another sarcoma potentially arising from the heart, a low rwORR (8%) and a limited mPFS (3.2 months) were observed with gemcitabine‐based chemotherapy . Similarly, the activity of pazopanib previously suggested by Kollar (2 RECIST PR/2 patients) and Funatsu (1 PR/1 patient) was limited (rwORR, 8%; mPFS, 3.7 months) . Of note, if anthracyclines were mostly used as an upfront treatment, gemcitabine and pazopanib were mainly used as a further line of treatment.…”
Section: Discussionmentioning
confidence: 94%
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“…With regard to other novel agents for pulmonary intimal artery sarcoma, Funatsu et al described a case of pulmonary artery intimal sarcoma showing partial response to pazopanib [13], a multitarget tyrosine kinase inhibitor active in advanced or metastatic soft tissue sarcoma [14]. In addition, there have been case reports showing good response of pulmonary artery intimal sarcoma to vinorelbine-based regimens [15, 16].…”
Section: Discussionmentioning
confidence: 99%