Chest wall sarcomas

Abstract

Aim: To evaluate initial symptoms and clinical features, diagnosis and delay in osteosarcoma (OS), Ewing sarcoma (EWS) and chest wall chondrosarcoma (CS). To assess treatment and identify prognostic factors in chest wall CS.

Patients: Initial symptoms and clinical features were analyzed from the records of the very first visit to a doctor often a general practitioner, for symptoms related to the bone sarcoma. Study I All patients (< 30 yrs) diagnosed in Sweden (1983-95) with OS or EWS, at all skeletal sites (149 patients). Study II All patients (< 30 yrs) diagnosed (1981-2000) with EWS of the chest wall (26 patients). Study III and IV All patients diagnosed in Sweden (1980-2002) with CS of the chest wall (106 patients).

Clinical findings: The most common initial symptom in OS and EWS was pain related to strain, reported by 85 % of the OS and 64 % of the EWS. Pain at night was reported by approximately 20 % of the OS and EWS. In the chest wall, pain at night was reported by only 1/26 of the EWS and none of the CS. Only 32 % of the chest wall CS patients complained about pain. A palpable mass was the most important clinical finding noted in 34 to 69 % of the patients in the different series.

Diagnosis: The initial symptoms in OS and EWS were non-specific and generated many differential diagnoses. A tumor was suspected in approximately 25 % of the cases at the first medical visit. In chest wall CS the doctor suspected a malignancy at the first visit in 83 %. Initial plain x-rays showed pathological findings in 91 % of the OS but only in 57 % of the EWS and 66 % of the chest wall CS. FNAB of CS gave a correct diagnosis for 26 % when done outside a sarcoma center but for 94 % at sarcoma centers.

Treatment: 23/26 of the EWS of the rib were treated surgically. Twelve had correct preoperative chemotherapy and only 9 were handled at a sarcoma center. 55/106 of the chest wall CS were treated at a sarcoma center, 42 at non-specialty hospitals and 9 had palliative treatment only. Chest wall CS treated at a sarcoma center resulted in 7 % intralesional resections compared to 52 % for those treated at non-specialty hospitals.

Prognosis: The estimated 10-year survival rate for EWS of the rib was 0.54 and patients treated according to correct protocols had a better outcome (p<0.05). The 10-year survival rate was 0.67 for chest wall CS treated in a curative intend. Patients operated with wide surgical margins had a survival rate of 0.96 compared to 0.46 after an intralesional resection. The difference in surgical margins achieved at sarcoma centers and at non-specialty centers resulted in higher survival rates at sarcoma centers; 0.75 compared with 0.59 at non-specialty centers.

Delay: Doctor s delay was longer in EWS than in OS (4 compared to 2 months). OS with metastasis at diagnosis had longer doctor s delay and also EWS with metastasis at diagnosis had longer delay but the difference was not significant. Chest wall CS were associated with a wide spread of both patient s and doctor s delay. A normal interpreted x-ray and falsely normal cytological diagnosis were associated with longer doctor s delay. Total delay was significantly longer in patients who died of the chest wall CS.

Conclusions: The initial symptoms of sarcomas are often pain related to strain. A palpable mass was discovered in many patients already at the first visit. Plain x-rays often missed sarcomas of the rib, spine and pelvis. FNAB is unreliable when done outside a sarcoma center. Patients with chest wall sarcomas should be referred to sarcoma centers for diagnosis and treatment to improve outcome.