Diagnosis and outcome of primary solid thoracic tumours in a high tuberculosis prevalent setting

[摘要] Background and aimPrimary solid thoracic neoplasms (PSTT) have a diverse pathological spectrum, varying prognosis and survival rates and are unusual in children. Early recognition, diagnosis and treatment is key to a good outcome. The aim was to describe the incidence, diagnostic challenges and outcome of PSTT, determine patient demographics, histological spectrum and treatment modalities.Methodology32 year retrospective review including all children under 16 years diagnosed with and treated for a thoracic mass at Tygerberg Children's Hospital between 1983 and 2015. Patients with pulmonary metastasis, benign pulmonary tumours and cystic lesions were excluded.ResultsWe identified 59 patients, 22 PSST and 37 haematological malignancies (22 lymphoma, 15 leukaemia) that presented with a thoracic mass and were recognised, diagnosed and treated in a tertiary care hospital situated in a middle income country with a high prevalence of HIV and TB. We found the incidence of PSTT to be 0.09 per 100 000 children per year and the median age at presentation was 3.29 (IQR1.4 – 7.02) with a 55% female predominance. Presenting complaints and symptomatology are very non-specific and variable, ranging from cough (55%), tachypnoea (36%), mass (36%), chest pain (23%) and fever (23%). One patient was asymptomatic and none had haemoptysis. Diagnosis was delayed in 36% of cases due to incorrect initial diagnosis and treatment for tuberculosis (23%) and pneumonia (13%). In all cases the CXR suggested a pulmonary abnormality, however invasive testing was required to make an accurate diagnosis, aided by biopsy (68%), surgery (32%) and bronchoscopy (5%). The histological spectrum of PSTT included 6 cases of neuroblastoma (27%), 3 of rhabdomyosarcoma (13%), 3 ganglioneuroblastomas (13%), 2 each of Pleuropulmonary blastoma, Kaposi sarcoma and Ewing's sarcomas (9%) and 1 each of infantile fibrosarcoma, myoepithelioma, undifferentiated sarcoma and osteogenic sarcoma. The stage at diagnosis included 69% early and 31% advanced stage disease. Management included chemotherapy (82%), radiotherapy (23%) and surgery (64%). Overall survival was 64%. Outcome was better with surgical management (83%) compared to non-surgical management (57%).ConclusionIn this first study reporting on PSTT from a middle income country, we demonstrated that there are numerous challenges in making the diagnosis, and patients are often misdiagnosed as TB or pneumonia. In spite of these challenges the outcome of children with PSTT remains comparable to children living in highly developed countries. Children with pneumonia and TB not responding to treatment, need to be referred for further evaluation to establish an underlying cause, which could include PSTT. These patients all presented with non-specific symptomatology, and diagnosis requires a high index of suspicion. All children with PSTT had abnormal, but not diagnostic chest X-rays and diagnosis was confirmed by invasive testing.

[发布机构] Stellenbosch University