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RESEARCH PRODUCT

Advanced epithelioid haemangioendotelioma: Fever, pain, and pleural effusion predict a worse outcome.

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e22540 Background: Epithelioid hemangioendothelioma (EHE) is an exceedingly rare soft tissue sarcoma subtype. EHE often presents as a multifocal/ multivisceral disease and its clinical behavior is highly unpredictable from indolent to very aggressive forms. A common choice in advanced patients is a close, active surveillance (AS), considering a treatment only in case of disease progression. Our retrospective study aimed to identify clinical features associated with a more aggressive behavior. Methods: Patients affected by advanced EHE treated in 6 centers of the Italian Rare Cancer Network were retrospectively reviewed. Diagnosis was confirmed by a sarcoma expert pathologist and molecular analysis was performed. Baseline clinical features were evaluated, including the presence of systemic symptoms (fever, weight loss, anorexia) and/or tumor related pain. Number of organs involved (1 vs > 1 and 1-2 vs > 2, mitotic index (< 2 vs ≥= 2 mitosis/10 high power fields - HPF). Results: 74 patients were identified (M:F = 42:32; mean age = 44 yrs - range = 16-81, median number of mitosis/10 HPF = 2 - range 0/8, systemic simptoms Y:N = 30:44; fever Y:N = 21:53; tumor related pain Y:N = 23:51; pleural effusion Y:N = 11:63). All patients had metastatic disease; 29 showed the involvement of one organ while 45 had multivisceral disease (18 of them > 2 viscera). At the time of diagnosis, all patients were naive from any systemic therapy and underwent exclusive AS. At a median FU of 32 months (range: 3-212), 48 (65%) patients progressed while 26 (35%) remained stable. No spontaneous regressions were observed. Median PFS was 30 months (range 2-115). Median OS was 54 months (range 3-151). Age, gender, mitotic index, number of involved organs, anorexia and weight loss did not correlate with median PFS (m-PFS). Fever correlated with shorter m-PFS (8 vs 45 months, P= 0.004), as well as pain (10 vs 42 months, P= 0.02) and the presence of pleural effusion (5 vs 46 months, P= 0.0001). In addition, fever and pleural effusion correlated with a shorter m-OS, respectively 29 vs 60 months (P = 0.03) and 11 vs 77 months (P < 0.001). The presence of pain corresponded to a not significant worse m-OS (39 vs 54 months, P= 0.16). Conclusions: Despite the small number of cases, this retrospective study suggests that in advanced EHE patients the presence of fever and/or tumor related pain and/or of pleural effusion can be associated with a more aggressive clinical behavior. Pleural effusion seems to be associated with the worse outcome, with a m-OS of 12 months. These data are worth confirming on a larger patient population.