Bronchial Carcinoid Tumour in Pregnancy

RL Cornell, J Wilkens, TK Cooper, GJ Beattie, T Sethi

Corresponding Author
Rachel L Cornell
Department of Obstetrics and Gynaecology
Simpson Centre for Reproductive Health
Royal Infirmary of Edinburgh
Little France Crescent
Edinburgh
EH16 4SA

Telephone: 07766312646
Email: RachelCornell_505@hotmail.com

Keywords
Carcinoid tumour, Pregnancy


Summary

Breathlessness is a commonly encountered symptom in pregnancy. The differential diagnosis for acute breathlessness in pregnancy is broad and possibilities range from physiological changes and psychological causes to acute respiratory disorders, which include pulmonary embolism (PE), acute asthma, infection and pneumothorax. It is rare to find a malignancy as the underlying cause of breathlessness.

We report a case of severe breathlessness and chest pain in a 26-year-old woman presenting at 36 weeks gestation. A chest radiograph was normal but a computertomogram (CT) identified a mass, which was subsequently demonstrated to be a malignant bronchial carcinoid tumour. Although abdominal carcinoid tumours have been reported in the literature, to our knowledge, this is the first reported pulmonary carcinoid tumour, presenting during pregnancy.

Case Report

A 26-year-old, primigravid woman, presented at 36 weeks gestation with acute breathlessness at rest. This was exacerbated by exertion and associated with right-sided chest pain. She was a non-smoker with no medical or family history.

On examination, she was apyrexial and normotensive but tachycardic. Her respiratory rate was 20 bpm and oxygen saturations were 97% on air. Respiratory examination revealed symmetrical expansion and expiratory monophonic wheeze, audible throughout her chest. Calves were non-tender and systemic examination was unremarkable. Regular fetal movements and daily cardiotocographs were reassuring.

Haematology and biochemistry investigations were normal. Arterial blood gas demonstrated H+ of 36.4nmol/l, pO2 of 16.5kPa, pCO2 of 3.72kPa and BE of –4.6 on 4L O2. Chest X-ray was normal. ECG demonstrated a sinus tachycardia.

Presentation was suggestive of acute asthma but symptoms persisted despite treatment with oxygen, salbutamol nebulisers and steroids. Findings were not typical of PE but as other pathologies had been excluded, treatment dose heparin was commenced. A ventilation-perfusion scan was inconclusive so a pulmonary angiography (CTPA) was arranged. This excluded PE but demonstrated an atypical endobronchial mass, see Figure 1.

 

As the patient was now 37 weeks pregnant, caesarean section was undertaken to permit further investigation. Under spinal anaesthesia, a healthy male infant was born.

Further imaging demonstrated a left main bronchus mass, enlarged hilar lymph nodes, peribronchial thickening and upper lobe atelectasis. Abdominal and pelvic CTs were normal. At bronchoscopy, a solid mass obstructing 80% of the lumen was seen. No biopsy was taken due to its vascularity and washings consisted of epithelial and inflammatory cells only. No malignant cells were identified.

The patient was referred to thoracic surgery and underwent sleeve resection. Histology of the specimen demonstrated typical characteristics of a carcinoid tumour, with no lymphatic or vascular invasion. The tumour was staged as T1 N0 Mx.

Discussion

A carcinoid tumour is a tumour of the neuroendocrine system. There has been a six-fold increase in the incidence of these tumours between 1973 and 20021. The male:female sex ratio for carcinoids is almost equal1 but two thirds of individuals with bronchial carcinoid are female2. The mean age of diagnosis is 57 years2.

74% of carcinoids originate in the gastrointestinal tract and only 25% occur in the respiratory tract4. Pulmonary carcinoids are rare, constituting 1-5% of all lung cancers3. They display a spectrum of clinical behaviour and histological differentiation. Classification is based on histological features and subdivides them into typical carcinoids, atypical carcinoids and neuroendocrine carcinomas4.

Bronchial carcinoids characteristically involve central airways and growth is often slow. It may be several years before symptoms appear and diagnosis is made. Presenting symptoms may include cough, hemoptysis, pain, dyspnoea or obstructive pneumonia. Localised wheeze and new-onset "asthma" have also been described5. Up to 51% of patients are however asymptomatic and diagnosis is incidental5.

Severe breathlessness is uncommon with subtotal obstruction of one bronchus. Physiological breathlessness, which is common in pregnancy, may have been exaggerated by the co-existing tumour. Without pregnancy, this tumour may have been asymptomatic and diagnosis may have been delayed for many years.

Surgery is the treatment of choice for pulmonary carcinoids. Complete excision is usually possible as only 15% of tumours demonstrate metastases. Patients with typical bronchial carcinoids have an excellent prognosis, with 5-, 10- and 15-year survivals of 92.4%, 88.3% and 76.4% respectively6.

Few cases of bronchial carcinoid tumours have been reported in the literature. There are several reports of abdominal carcinoid tumours during pregnancy7, most of which are incidental findings, with no implications for mother or fetus. Presentation of a bronchial carcinoid during pregnancy has never been reported.

The effects of carcinoid tumours on pregnancy are unclear, as is the effect of pregnancy on the tumour. There are reports of clinical improvement in carcinoids during pregnancy, with recurrence of tumour symptoms postpartum8,9. However, there are also reports documenting poor obstetric outcomes in the presence of carcinoid tumours. Examples include spontaneous miscarriage10,11, unexplained intra-uterine death and placental abruption7. Causes of these complications are not clearly understood, but interaction of the carcinoid tumours with the biochemical and hormonal systems of pregnancy may be responsible. Although the number of cases is small, it appears that carcinoid hepatic metastases and carcinoid syndrome may be associated with an increased rate of fetal death8.

Zimmer et al demonstrated that corticosteroid administration prevented carcinoid crises12. Clinical improvement during pregnancy may be explained by the increase in cortisol production in pregnancy.

Hormonal products, such as serotonin, ACTH and chromogranin, can be demonstrated within neurosecretory granules in all carcinoids. One animal study demonstrated an increase in uterine vascular resistance with serotonin administration, which led to changes in placental function, resulting in intra-uterine growth retardation13. This mechanism may be responsible for poor obstetric outcome.

Viale et al demonstrated progesterone receptors on typical bronchial carcinoid tumours14. Acute presentation in pregnancy may result from receptor stimulation by elevated progesterone concentrations and subsequent accelerated growth. Immunohistochemistry failed to demonstrate progesterone receptors in this case.

Our patient presented at 36 weeks gestation with acute dyspnoea. Step-wise investigation allowed exclusion of common causes and revealed the unusual diagnosis of a bronchial carcinoid tumour.

Presentation of life-threatening maternal conditions during pregnancy forces difficult decisions to be made. The health of the mother and unborn child must be considered when opting for treatment prior to, or following, delivery. Implications include delayed treatment and disease progression, pregnancy complications secondary to simultaneous treatment, and complications of premature delivery, miscarriage or termination.

 

References

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