1732P - Primary pulmonary sarcoma: A EURACAN project

Background

Primary pulmonary sarcoma (PPS) is a very rare entity accounting for less than 1% of all pulmonary malignancies. Data on PPS are scarce and rely mostly on small case series. EURopean reference Network on rare Adult solid CANcers (EURACAN) is a wide network enabling physicians to exchange on clinical practice and research on rare adult solid cancers. We aimed at using EURACAN to assess outcome of patients with PPS.

Methods

Through EURACAN, a working group for PPS was created. A retrospective database was built including patient and tumor characteristics, staging work-up, treatment and outcome. Potential prognostic factors were assessed by univariable analysis and compared using logrank test. Statistically significant parameters were evaluated by multivariable analysis to identify independent survival predictors. Independent predictors and their coefficient were used to build the PPS score. For each patient, the score was correlated to overall survival (OS). OS was calculated from the date of diagnosis until death or last follow-up. P-value ≤ 0.05 was considered significant.

Results

16 centers from 9 European countries (F: n=49, 31%; B: n=29, 19%; I: n=26, 17%; D: n=23, 15%, A: n=9, 6%; P: n=6, 4%; CZ: n=5, 3%, E: n=5, 3%; PL: n=3, 2%) shared data on 155 patients (82 male, 53%) with PPS. Median age was 55 (8 to 89) years. Median tumor size was 79 mm (6 to 300). 58 tumors were classified M1 (37%). 104 patients underwent curative-intended treatment including surgery (67%). There were 32 histological subtypes, most commonly synovial sarcoma (n=45, 29%), leiomyosarcoma (n=19, 12%) and epithelioid hemangioendothelioma (n=16, 10%). Median follow-up was 24 months. Median OS was 3 years with a 5- and 10-year OS of 40% and 24%. Multivariable analysis identified male sex (p=0.018), older age (p<0.001), larger tumor size (p=0.003) and presence of metastases (p<0.001) as negative prognostic factors for OS. Coefficients for sex, age, tumor size and presence of metastasis were used to build PPS score. An increase in PPS score was correlated to a statistically significant decrease in OS (p<0.001).

Conclusions

The PPS working group within EURACAN allowed the creation of the largest European database on PPS. The PPS score correlated to OS and could be useful in the management of patients with PPS after further external validation.

Clinical trial identification

Editorial acknowledgement

Funding

Has not received any funding.

Disclosure

All authors have declared no conflicts of interest.