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

Smit, Carine (2017-12)

Thesis (MMed)--Stellenbosch University, 2017.

Thesis

Background and aim Primary 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. Methodology 32 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. Results We 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%). Conclusion In 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.

Agtergrond en doelstelling Primêre soliede torakale tumors (PSTT) het ‘n diverse patologiese spektrum, groot variasie in prognose en uitkoms en is buitengewoon in kinders. Vroeë herkenning, diagnose en behandeling is die grondslag van ‘n goeie uitkoms. Die doel is om die insidensie, diagnostiese uitdagings en uitkoms van PSTT te beskryf, asook om meer spesifiek te kyk na die demografie, histologiese spektrum en behandeling van pasiënte. Metodiek Hierdie is ‘n retrospektiewe oorsig wat strek oor 32 jaar, vanaf 1983 to 2015, van alle kinders jonger as 16 jaar gediagnoseer en behandel vir ‘n torakale massa by Tygerberg Kinderhospitaal. Pasiënte met pulmonale metastase, nie-kwaadaardige pulmonale tumors en sistiese letsels is uitgesluit. Resultate Ons het 59 pasiënte geïdentifiseer wat met ‘n torakale massa gepresenteer het, 22 met PSTT en 37 met hematologiese maligniteite (22 limfoom en 15 leukemie). Hulle is almal gediagnoseer en behandel in ‘n tersiêre vlak hospitaal geleë in ‘n middelinkomste land met ‘n hoë voorkoms van HIV en TB. Die insidensie van PSTT is 0.09 per 100 000 kinders per jaar en die mediane ouderdom ten tye van presentasie was 3.29 (interkwartielvariasiewydte 1.4 – 7.02) met ‘n 55% vroulike oorheersing. Die klagtes en simptome waarmee pasiënte gepresenteer het was baie nie-spesifiek en het gestrek van hoes (55%), tagipnee (36%), ‘n massa (36%), borskaspyn (23%) en koors (23%). Een pasiënt was asimptomaties en geen pasiënte het met hemoptiese gepresenteer nie. Die diagnose is vertraag in 36% van gevalle as gevolg van ‘n oorspronklike verkeerde diagnose en behandeling van tuberkulose (23%) en pneumonie (13%). In alle gevalle het ‘n borskas x-straal ‘n pulmonale abnormaliteit suggereer, maar verdere indringende toetse was nodig om die korrekte diagnose te maak, met die hulp van biopsies (68%), chirurgie (32%) en brongoskopie (5%). Die histologiese spektrum van PSTT het 6 gevalle van neuroblastoom (27%), 3 elk van rhabdomiosarkoom en ganglioneuroblastoom (13%), 2 elk van pleuropulmonale blastoom, Kaposi sarkoom en Ewing’s sarkoom (9%) en 1 elk van infantiele fibrosarkoom, mioepitilioom, ongedifferensieerde sarkoom en osteogene sarkoom (5%) ingesluit. Ten tye van diagnose het 69% van gevalle gepresenteer met vroeë stadium en 31% met gevorde stadium van siekte. Behandeling het chemoterapie (82%), bestraling (23%) en chirurgie (64%) ingesluit en 64% van pasiënte het oorleef. Uitkoms was beter met chirurgiese behandeling (83%) teenoor nie-chirurgiese behandeling (57%). Gevolgtrekking In hierdie eerste studie wat verslag lewer oor PSTT in ‘n middelinkomste land, het ons verskeie diagnostiese uitdagings uitgewys en aangetoon dat pasiënte maklik verkeerd gediagnoseer kan word met TB of pneumonie. Ten spyte van hierdie uitdagings, is die uitkoms van pasiënte met PSTT vergelykbaar met die van Eerstewêreldlande. Kinders met pneumonie of TB wat nie kliniese verbetering toon op behandeling nie, moet verwys word vir verdere ondersoeke om ‘n onderliggende oorsaak te bepaal, waarvan PSTT ‘n moontlikheid is. Hierdie pasiënte het almal gepresenteer met nie-spesifieke simptome en ‘n hoë indeks van suspisie is nodig om die diagnose te maak. Al die pasiënte het ‘n abnormale, maar nie diagnostiese, borskas x-straal gehad en die diagnose is bevestig deur indringende toetse.

Please refer to this item in SUNScholar by using the following persistent URL: http://hdl.handle.net/10019.1/102617
This item appears in the following collections: