Bronchial Carcinoid Tumours in Children – A Review


Table 1: Number and Characteristics of Bronchial Carcinoid Tumours in Children in the Literature Authors


Number Age (years) Sex Symptoms (range; median) (M/F)


Wang, et al. 199312


*


Al-Qahtani, et al. 5 200315


Fauroux, et al. 11 200517


Rizzardi, et al. 15 200916


10–15; 13 9–15; 13 8–18; 15 17 10–21; 17


8/9 Atelectasis 59 % 11/6 Pneumonia 53 % Wheezing 47 %


3/2 -- 4/1


5/6 Pneumonia 70 % 7/4 Haemoptisis 35 %


11/4 Pneumonia 80 % 9/6 Cough 6.6 %


Haemoptisis 6.6 % Fever 6.6 %


*This paper includes patients up to 21 years of age.


Nevertheless, BCs are the most common primary lung neoplasms of childhood,14


neoplasms in paediatric populations.18,19


accounting for up to 80 % of malignant pulmonary Their incidence is apparently


increasing, although this is probably related to the introduction of more sensitive diagnostic tools as well as to an overall increased awareness of this disease among physicians.


Lung masses in children are approximately 10 times more likely to represent a benign developmental or reactive lesion than a neoplasm;13 common malformations forming solid and cystic masses of paediatric lung include bronchogenic cysts, segmental bronchial atresia, sequestration and congenital bronchial malformation. The vast majority of solid parenchymal lung masses in children represent inflammatory, infectious or reactive processes with a differential diagnosis including granulomatous inflammation (fungal, mycobacterial, parasitic, sarcoidosis and vasculitis), abscess, pneumonia, septic embolus, infarction and haematoma.20


A differential diagnosis should also be made with foreign bodies that are not uncommon in children.


To date, the aetiological factors predisposing infants and children to the development of pulmonary neoplasms are unknown. Diagnosis in the majority of cases occurs in adolescence with a mean age of 12–15 years,16,17


but diagnosis delay is highly variable and is


probably many months. The youngest case described in literature was at three years of age,21


in the adult or shows a prevalence of carcinoids in males but the operation and the histological


diagnosis were made three years later. In the few published series, the reported incidence is the same in both sexes15,17 population22


(male/female ratio 2.75).16 Clinical Presentation


Given the rarity of BC, clinical detection in children remains a challenge. In paediatric populations all BCs are centrally located in the bronchial tree. A tumour visualised in the bronchial tree by bronchoscopy or associated with atelectasis or obstructive pneumonia is commonly defined as ‘central’.


In contrast to the adult population, younger patients are all symptomatic and the most common symptom is obstructive pneumonia with recurrent pulmonary infections in the same location.12,16


Endoscopy plays a central role in the diagnosis and initial management of carcinoids, and a bronchial biopsy should be taken whenever possible. BC typically reveals as a smooth pink–reddish or yellow endobronchial mass often covered by intact mucosa.


In the past, major haemorrhage after biopsy was feared,26 but in


specialised centres with daily clinical practice, endobronchial biopsy of a carcinoid (despite historical dogma to the contrary)27


significantly increases the diagnostic yield without adding morbidity or mortality.16,24 Other symptoms


include persistent cough, haemoptysis, wheezing and recurrent fever. These symptoms are aspecific, but their recurrence in a child with obstructive pneumonia localised in the same lobe, persistent cough or wheezing not responsive to the usual therapy should alert the


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A careful endoscopic assessment is also of fundamental importance to determine the feasibility of surgery and to plan the best surgical treatment, with the main aim being trying to determine the feasibility of a bronchoplastic procedure. Moreover, in these central tumours obstructing the major bronchi, endoscopic debulking with or without laser allows physicians to look behind the tumour and evaluate its base of implant. It also permits physicians to treat airway obstruction and avoid recurrent pneumonia, which could irreversibly damage the lung parenchyma. Patients with a suspected BC should undergo a clinical evaluation. Blood tests include chromogranin A, NSE and serotonin. Urinary tests with 24-hour urinary excretion of


197


physician and suggest diagnostic procedures such as chest X-ray and bronchoscopy. Although many authors21,23


10/7 4/1 10/1 5/10 17/0 5/0 11/0 13/2 1 2 0 0 1 0 3 2


Site (R/L) Treatment Classic/ Histology n Recurrence Follow-up/Status Parenchyma-saving (TC/AC)


All alive (2 metastasis) Mean 6.5 years [0.5–34]


All alive


Mean 30 months [6–48] All alive


Mean 4.3 years [1–10] 1 dead (not for BC) 14 alive (1 metastasis) Mean 10 years [3–32]


have stressed the importance


of such symptoms (including asthma) for early diagnosis, many patients still undergo invasive diagnostic procedures too late, often after a symptomatic course of several months.


The characteristics of patients and clinical, surgical, pathological and follow-up data of the major recent reports in paediatric populations are summarised in Table 1.12,15–17


Carcinoid syndrome, presenting with


flushing, diarrhoea, palpitations and asthma-like symptoms, is caused by serotonin release from the tumour. Unlike its counterparts in the gut, classic carcinoid syndrome is rare in BC and is generally associated with metastatic disease. In fact, it is seen at presentation in only in 0.7 % of adult patients with BC24 rarely in paediatric patients.12,25


and is reported very Diagnosis and Imaging


Diagnosis may be delayed due to low clinical suspicion and to the atypical ways in which pulmonary carcinoid can present. Early diagnosis increases the likelihood of definitive management. It is hoped that an increased awareness of BC will result in earlier diagnosis and surgical resection with a good prognosis.21


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