Orbital Sarcoma Classification Correlates With Metastasis and Survival

By Will Boggs MD

March 05, 2020

NEW YORK (Reuters Health) - The T and N categories defined by the American Joint Committee on Cancer (AJCC) 8th edition correlate with metastasis and survival in patients with orbital sarcoma, according to new research.

"This was the first study to actually validate that the AJCC criteria for orbital sarcoma correlate with disease-specific survival outcomes," Dr. Bita Esmaeli of the University of Texas MD Anderson Cancer Center, in Houston, told Reuters Health by email.

The rarity of orbital sarcomas, which account for only about 5% of orbital tumors, has complicated the standardization of their staging and management. Most recommendations have been based on extrapolation from experience with sarcomas of the same histologic types in other anatomical sites, Dr. Esmaeli and colleagues note in JAMA Ophthalmology.

In their evaluation of the AJCC classification, the researchers focused on whether the T and N categories at presentation correlate with local recurrence (LR), lymph node metastasis (LNM), distant metastasis (DM) and death due to disease (DD).

The study included 73 patients with orbital sarcoma, all treated by Dr. Esmaeli between 2003 and 2018. Their median age was 21 years (range, 0-77 years).

Most patients underwent multimodal therapy, including chemotherapy plus irradiation (42%), surgery with curative intent plus chemotherapy plus irradiation (19%), and surgery with curative intent plus irradiation (14%).

During a median follow-up of 38.9 months, 16 patients had LR, seven had LNM, 14 had DM and 16 had DD.

The risk of LR, LNM, DM and DD increased significantly with T category, and the risk of LR, DM, and DD increased significantly with tumor size.

Disease category T3 or greater at presentation, in particular, seemed to be associated with higher risk for LR, DM and DD.

Each 1-cm increase in tumor size was associated with an 86% higher risk of LR, a 79% higher risk of DM and a 134% higher risk of DD, all significant results.

N1 versus N0 category was associated with significantly higher risks of DM and DD, but not with LR or LNM.

"We need to more carefully look for metastasis in patients who have greater than T2 orbital sarcomas and always consider additional adjuvant treatments (for example, radiation or drug therapy if possible) for larger orbital sarcomas," Dr. Esmaeli said.

"Careful monitoring and surveillance for regional nodal and systemic metastases should be considered in patients with T3 or higher disease at presentation and/or N1 disease at presentation (meaning patients who present with nodal metastasis as these are at higher risk of developing distant metastasis and death from disease)," she said.

SOURCE: https://bit.ly/3cvYXT8 JAMA Ophthalmology, online February 27, 2020.