Changes to This Summary (12/14/2011)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Cellular and Histopathologic Classification ¹

Revised Table 1 ² to include chromosomal aberrations and genes involved with the inflammatory myofibroblastic tumor (IMT) histology (cited Jain et al. as reference 9).

Added Alaggio et al. as reference 13 ³ and Williams et al. as reference 14 ⁴.

Added text ⁴ to state that in another series of 33 patients, overall survival was 68% at 5 years and 53% at 10 years from diagnosis; survival was better for smaller tumors and completely resected tumors (cited Pennacchioli et al. as reference 19 and level of evidence 3iiA).

Added text ⁵ to state that a case report described a partial response in a patient with recurrent inflammatory myofibroblastic tumor who was treated with crizotinib, an ATP-competitive inhibitor of the ALK and MET tyrosine kinases (cited Butrynski et al. as reference 40).

Revised text ⁶ to state that inactivation of either TSC1 (9q34) or TSC2 (16p13.3) results in stimulation of the mTOR pathway, providing the basis for the treatment of non-surgically curable PEComas with mTOR inhibitors.

Added text ⁷ to state that a subsequent retrospective study performed by the Polish and German Cooperative Paediatric Soft Tissue Sarcoma Study Groups identified four chemotherapy responses in ten children treated; anti-angiogenesis therapy may prove useful in the treatment of this group of neoplasms (cited Bien et al. and Park et al. as references 71 and 74, respectively).

Stage Information ⁸

Expanded text ⁹ on Group II nonmetastatic disease to include subgroups IIA, IIB, and IIC.

Treatment of Nonmetastatic Childhood Soft Tissue Sarcoma ¹⁰

Added text ¹¹ to state that in one series, clear cell sarcoma demonstrated a propensity to metastasize to regional lymph nodes (cited Blazer et al. as reference 38).