Br. J. Dis. Chest (1988) 82, 414
NODULAR PULMONARY AMYLOIDOSIS: A CASE REPORT AND REVIEW OF LITERATURE S. HOLMES, J. B. DESAI AND R. N. SAPSFORD Department
Surgery, St Mary’s Hospital, London W2
Since 1974, 20 new cases of nodular pulmonary amyloidosis have been reported in the English literature. We present a new case and a review of the literature. INTRODUCTION The lung is frequently involved in generalized amyloidosis (1,2). Amyloid localized to the respiratory tract is, however, a rarer finding (3). Kramer and Som (4) reported 9.5 cases of primary tumour forming amyloidosis of the respiratory tract in 1935 and of these only four cases involved the lung parenchyma alone. Amyloid of the trachea and bronchi is the common form, leading to signs and symptoms of airway obstruction. In a review of cases (5) there was an appreciable mortality from asphyxia and respiratory insufficiency. Isolated parenchymal amyloid is rare and occurs in two forms, a diffuse type and a nodular type (6). These two types are markedly contrasting in their aetiology, course and prognosis. Diffuse pulmonary amyloid is usually associated with multiple myeloma or primary generalized amyloidosis, is very rare and leads fatally to respiratory failure (5). Nodular pulmonary amyloid, however, has a more benign natural history and a better prognosis. A full classification of amyloid of the lower respiratory tract has recently been revised by Thompson (7). Since a review by Saab et al. in 1974 there have been 20 new cases reported in the English literature, bringing the total to 57. Case Report
A 7Zyear-old Caucasianlady presented with epigastric pain. A chest radiograph at that time showed a small round opacity in the mid-zone of her right lung. It was approximately 1 cm in diameter, contained no calcification and there was no hilar lymphadenopathy. The patient was asymptomatic and in the past had been treated for mild hypertension and had had a benign uterine polyp removed 30 years previously. She was a non-smoker. Tomograms showed the lesion to be smooth and round and it was decided to observe any progress. Repeat tomograms taken 3 and 9 months later showed there to be no change in size. However, 20 months after the initial radiograph there was a small increase in sizeof the lesion and there were now two lesions in her right mid-zone (Fig. 1). Correspondence to: Mr J. B. Desai FRCS, Senior Registrar, Cardiothoracic Surgery, Middlesex Hospital, Mortimer Street, London Wl.
Fig. I. Chest X-ray on presentation showing a small opacity in the right mid-zone A percutaneous fine needle aspiration biopsy using a 22G needle under CT control was done and cytological examination of the aspirate showed that there were several clusters of glandular epithelia with morphological features suggestive of malignancy. Routine investigations, including haemoglobin, electrolytes, calcium, glucose, renal and liver function were all normal. Her ESR was 27. Her pulmonary function tests showed a minimal restrictive pattern with vital capacity of 1.8 litres (78% of predicted value) and FEVl of 1.4 litres (88% of predicted value). In view of the suspicion of malignancy a right thoracotomy was performed. Examination of the lung revealed four nodules; two in the upper lobe and one in each of the middle and lower lobes. There was no lymphadenopathy. These were removed in three wedge resections. Her postoperative course was uneventful. Histology showed the nodules to contain an amorphous eosinophilic material with an infiltrate of chronic inflammatory cells and the staining properties of amyloid. Subsequent laboratory investigations, including serum electrophoresis, immunoglobulins and urinalysis for Bence-Jones proteins have revealed no abnormalities. The patient was discharged home on the ninth postoperative day. When seen in the clinic 6 months later she was very well with no evidence of further nodule formation on chest radiograph.
DISCUSSION Since the first report of nodular amyloid formation in the lung parenchyma by Lesser (9) in 1877 there have been several reviews of cases, notably by Prowse in 1958 (6), Condon in 1964 (10) and Saab in 1974 (8). The incidence of new cases reported seems to be steady; 20 in the last 10 years compared with 17 in the previous decade before that. Since the review by Saab et al. the 20 new cases have shown a similarity in natural history with previous cases. The presentation of these neoplasms is still most commonly as an incidental finding on a routine chest radiograph. The nodules are typically asymptomatic,
vary in size and number, may be unilateral or bilateral and have no distinctive radiological features except that they often resemble malignant tumours of the lung. The average age at presentation is 63 with a range of 38-95. There is a roughly equal sex distribution. Amongst the newer cases there have been some other modes of presentation. Lee et al. (11) described a case that presented with haemoptysis and a chest radiograph revealed atelectasis and a lobar collapse. The patient later died of a pulmonary haemorrhage, although no autopsy was performed. Others presented with pleuritic chest pain (12), cough and one with the symptoms of a pancoast tumour (13). Another 86-year-old lady presented in respiratory failure due to massive nodular involvement of the lung parenchyma (14). Thompson et al. (15) reported a case of a solitary amyloid tumour affecting the parenchymal lung tissue, a main bronchus and also the draining lymph nodes causing hilar lymphadenopathy. These cases demonstrate that nodular pulmonary amyloidosis may not be as harmless a condition as previously reported. Owing to the radiographic appearances of these patients the majority have the diagnosis made by thoracotomy and open lung biopsy. Bierry (17) and Scott (18), however, report cases of lung masses diagnosed as pulmonary amyloid by percutaneous lung needle biopsy. The biopsies were unequivocal enough to restrict further investigations to those conditions associated with amyloid formation. Other cases, however (15,19,20), including our own, have had needle aspiration biopsy results that are equivocal or even suggestive of showing malignant cells. The case we have presented here is interesting in that it shows the development of a single nodule to multiple unilateral nodules. The time course for this change was 20 months between initial radiographic appearance and surgical excision, although for 16 months only one nodule was visible. Other patients have been followed up after the initial diagnosis and excision, and it has been observed that amyloid nodules redeveloped in the same or opposite lung, although many years later (20). CONCLUSIONS In conclusion nodular pulmonary amyloidosis is still a rare disease, but may not run such a benign course as previously thought. Indeed, some of the more recently recorded cases show multiple nodules, hilar lymph node involvement and appear associated with other conditions such as benign hypergammaglobulinaemia (21, 22) and Sjogren’s syndrome (23), thus having a morbidity and even mortality. It may be that all these cases are within a spectrum of disease forming a continuum between classifications. Thus, they may lie between an isolated, single, benign pulmonary nodule and diffuse alveolar amyloid which has the poorer prognosis. REFERENCES 1. Cohen AS. Amyloidosis. New Engl J Med 1967;277:522. 2. Weiss L. Isolated multiple nodular pulmonary amyloidosis. Am J Clin Path 1960;33:318-29. 3. Rubinow A, Celli B, Cohen A, Rigden B, Brody J. Localized amyloidosis of the lower respiratory tract. Am Rev Resp Dis 1978;118:603. 4. Kramer R, Som M. Local tumour-like deposits of amyloid in larynx. Arch Otolar 1935;21:32234.
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Date accepted 22 June 1987