Statistics from Altmetric.com
A 41-year-old woman presented in December 1997 with dry cough with streaky haemoptysis of 15 days duration. She had had a goitre for the last 25 years and had been diagnosed as having thyrotoxicosis on clinical and biochemical grounds (triiodothyronine (T3) 2.8 ng/ml and thyroxine (T4) 220 ng/ml) in September 1995. She had received carbimazole for 6 months and remained asymptomatic thereafter.
On examination, her body mass index (BMI) was 24.1 kg/m2. She had no pallor, lymphadenopathy, clubbing, oedema or hyperkeratosis. Her pulse was 96 beats/min regular, blood pressure 110/70 mmHg. She denied a history of fever, weight loss, loss of appetite, or recent increase in goitre size, but had been dysphagic to solids for 2–3 days. She was a non-smoker and never consumed alcohol. She had a firm, grade III (WHO) non-tender multinodular goitre with retrosternal extension. Systemic examinations were normal except for diffuse bilateral rhonchi. Her haemogram and serum biochemistry, including thyroid hormone profile (T3 0.86 ng/ml, T4 78 ng/ml, thyroid-stimulating hormone 0.3 μU/ml) were normal. Ultrasonography of thyroid confirmed the multinodular goitre with retrosternal extension with some areas of necrosis and specks of calcification. Ultrasound-guided fine needle aspiration cytology (FNAC) revealed follicular cells with nuclear grooving and psammoma bodies. Her radiological profile is shown in figure 1. Bronchoscopy could not be completed because of laryngeal spasm. Computed tomography (CT)-guided FNAC from pulmonary lesions was inconclusive.
- What is shown in figure 1?
- What is your diagnosis?
- What would be the approach to confirm the diagnosis?
Figure 1 shows bilateral multiple nodular opacities in lung fields on chest X-ray, further substantiated on high-resolution CT. Cervical X-ray shows a large goitre with retrosternal extension and calcification.
With the available clinical and radiological profile, a diagnosis of euthyroid multinodular goitre with retrosternal extension and metastatic lesions in the lung can be made. Further work-up to determine the primary source, whether from thyroid or elsewhere, is required.
In the presence of a long-standing euthyroid goitre, the possibility of thyroid carcinoma with pulmonary metastases is high. However, multinodularity and absence of cervical lymphadenopathy in the face of pulmonary metastases decreases this probability.1The presence of nuclear grooving and psammoma bodies can also be seen in long-standing multinodular goitre.2 3
The clue to the primary site of malignancy came from the widening of the prevertebral space in the cricopharyngeal region and the presence of malignant keratinizing squamous cells in the sputum. Barium swallow showed a narrowed segment with irregular mucosa in the upper third of the oesophagus (figure 2). Fibre-optic endoscopy revealed a growth in the upper cervical oesophagus. Brush cytology smears from the growth confirmed squamous cell carcinoma. Her disease was classified as stage IV (any T, any N & M1) according to the TNM staging system. She received cisplatin and etoposide cyclically (3 days treatment at 4-week intervals) and palliative radiation therapy.
Patients with oesophageal carcinoma with metastatic disease usually present with dysphagia, odynophagia, weight loss and marked emaciation.4 Our patient had maintained reasonably good health (BMI 24.1 kg/m2, haemoglobin 10.6 g/dl) at presentation. She experienced transient dysphagia to solids, attributable to compression from the multinodular goitre. Dysphagia, however, is not a presenting manifestation of upper cervical oesophageal carcinoma as this segment of the oesophagus is capacious.
Squamous cell carcinoma of the oesophagus, oropharyngeal leucoplakia and palmo-plantar tylosis are genetically linked. However, except for a positive family history of dysphagia in two of her first degree relatives, there were no indications for this syndrome in our patient.5 The relative frequencies of squamous cell carcinoma in the upper, middle and lower portions of oesophagus are 17%, 55% and 33%, respectively. Based on gross appearance, the fungating variety accounts for 60%, ulcerative type 25%, and infiltrative type 15%.5
Chronic cough in squamous cell carcinoma of the oesophagus occurs due to aspiration, tracheo-oesophageal fistula, or pulmonary metastases. In view of ready access to blood vessels, haematogenous metastases to lungs, liver, kidneys and bone are common.5
when metastatic pulmonary lesions are seen in a patient with long-standing euthyroid nodular goitre without enlarged lymph nodes in the neck, a non-thyroidal primary source needs to be considered
nuclear grooving and psammoma bodies, which are considered a cytological hallmark of papillary carcinoma, can also be present in long-standing multinodular goitre
upper oesophageal carcinoma with lung metastases may present with subtle symptomatology
Chemotherapy combined with palliative radiation yields better results than radiation therapy alone in patients with localised carcinoma of the oesophagus.5 Only about 20% of lesions are resectable but surgical intervention does not lead to increased survival except when the lymph nodes are not involved. The overall prognosis is poor, with 5-year survival rate less than 5%.5
Euthyroid multinodular goitre and squamous cell carcinoma of oesophagus with pulmonary metastases.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.