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Clinical Activity of Pazopanib in Metastatic Extraosseous Ewing Sarcoma.

Attia S, Okuno SH, Robinson SI, Webber NP, Indelicato DJ, Jones RL, Bagaria SP, Jones RL, Sherman C, Kozak KR, Cortese CM, McFarland T, Trent JC, Maki RG - Rare Tumors (2015)

Bottom Line: We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor.To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma.This national multi-institutional study is ongoing.

View Article: PubMed Central - PubMed

Affiliation: Mayo Clinic , Jacksonville, FL, USA.

ABSTRACT
We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor. To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma. This case provides rationale for adding a Ewing sarcoma arm to SARC024, a phase II study of regorafenib, another multi-targeted kinase inhibitor, in patients with liposarcoma, osteosarcoma and Ewing and Ewing-like sarcomas (NCT02048371). This national multi-institutional study is ongoing.

No MeSH data available.


Related in: MedlinePlus

(A) Low and (B) high power view of Hematoxylin & Eosin stain of S2 nerve root tumor showing primitive neuroectodermal tumor/Ewing’s sarcoma; (C) CD99 immunostain was positive. Chromogranin, synaptophysin, keratin (AE1/AE3), actin, desmin and melan-A were negative. Low power view of (D) Hematoxylin & Eosin and (E) CD99 immunostains of a transbronchial lung nodule biopsy confirming Ewing sarcoma metastasis.
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fig001: (A) Low and (B) high power view of Hematoxylin & Eosin stain of S2 nerve root tumor showing primitive neuroectodermal tumor/Ewing’s sarcoma; (C) CD99 immunostain was positive. Chromogranin, synaptophysin, keratin (AE1/AE3), actin, desmin and melan-A were negative. Low power view of (D) Hematoxylin & Eosin and (E) CD99 immunostains of a transbronchial lung nodule biopsy confirming Ewing sarcoma metastasis.

Mentions: In 2001, a 57-year-old male was diagnosed with primary systemic amyloidosis and received high dose melphalan with autologous stem cell transplantation, placing him in remission. In 2005, he developed intermittent right lower extremity radiculopathy. Magnetic resonance imaging (MRI) of his pelvis at an outside hospital was reportedly normal. His symptoms progressed and, in 2008, MRI revealed a right S2 nerve root mass radiographically consistent with a benign nerve sheath tumor. He was observed. The mass had enlarged on MRI a year later. Resection demonstrated a 2.2-cm poorly differentiated malignant neoplasm consistent with primitive neuroectodermal tumor/Ewing sarcoma (Figure 1A-C). Staging scans, including whole body positron emission tomography (PET) and high resolution computed tomography (CT) scan of the chest, abdomen and pelvis, revealed no signs of metastasis. A bone marrow biopsy was not performed.


Clinical Activity of Pazopanib in Metastatic Extraosseous Ewing Sarcoma.

Attia S, Okuno SH, Robinson SI, Webber NP, Indelicato DJ, Jones RL, Bagaria SP, Jones RL, Sherman C, Kozak KR, Cortese CM, McFarland T, Trent JC, Maki RG - Rare Tumors (2015)

(A) Low and (B) high power view of Hematoxylin & Eosin stain of S2 nerve root tumor showing primitive neuroectodermal tumor/Ewing’s sarcoma; (C) CD99 immunostain was positive. Chromogranin, synaptophysin, keratin (AE1/AE3), actin, desmin and melan-A were negative. Low power view of (D) Hematoxylin & Eosin and (E) CD99 immunostains of a transbronchial lung nodule biopsy confirming Ewing sarcoma metastasis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508650&req=5

fig001: (A) Low and (B) high power view of Hematoxylin & Eosin stain of S2 nerve root tumor showing primitive neuroectodermal tumor/Ewing’s sarcoma; (C) CD99 immunostain was positive. Chromogranin, synaptophysin, keratin (AE1/AE3), actin, desmin and melan-A were negative. Low power view of (D) Hematoxylin & Eosin and (E) CD99 immunostains of a transbronchial lung nodule biopsy confirming Ewing sarcoma metastasis.
Mentions: In 2001, a 57-year-old male was diagnosed with primary systemic amyloidosis and received high dose melphalan with autologous stem cell transplantation, placing him in remission. In 2005, he developed intermittent right lower extremity radiculopathy. Magnetic resonance imaging (MRI) of his pelvis at an outside hospital was reportedly normal. His symptoms progressed and, in 2008, MRI revealed a right S2 nerve root mass radiographically consistent with a benign nerve sheath tumor. He was observed. The mass had enlarged on MRI a year later. Resection demonstrated a 2.2-cm poorly differentiated malignant neoplasm consistent with primitive neuroectodermal tumor/Ewing sarcoma (Figure 1A-C). Staging scans, including whole body positron emission tomography (PET) and high resolution computed tomography (CT) scan of the chest, abdomen and pelvis, revealed no signs of metastasis. A bone marrow biopsy was not performed.

Bottom Line: We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor.To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma.This national multi-institutional study is ongoing.

View Article: PubMed Central - PubMed

Affiliation: Mayo Clinic , Jacksonville, FL, USA.

ABSTRACT
We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor. To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma. This case provides rationale for adding a Ewing sarcoma arm to SARC024, a phase II study of regorafenib, another multi-targeted kinase inhibitor, in patients with liposarcoma, osteosarcoma and Ewing and Ewing-like sarcomas (NCT02048371). This national multi-institutional study is ongoing.

No MeSH data available.


Related in: MedlinePlus