Lung abscess complicating chondromas in Carney's syndrome

Abstract

In 1977, CARNEY et al. [1] described an unusual syndrome characterized by the concomitant or successive occurrence of a combination of three disparate tumours in young females: gastric leiomyosarcoma, pulmonary chondroma and catecholamine-secreting paraganglioma. The pulmonary chondromas may be multiple and bilateral, and are often found as incidental lesions on chest radiography during investigation of other components of the syndrome [1][2][3][4][5][6][7][8][9][10]. They tend to grow slowly over many years, but are benign and do not usually give rise to complications. We describe a case of Carney's syndrome, in which the chondromas were complicated by the development of a lung abscess requiring surgical resection. Case reportA 26 yr old female presented with a 3-week history of right pleuritic pain, sweating and rigors. She had a pyrexia of 38.5°C and crackles were audible over the right chest posteriorly. Chest radiography showed a nodule in the right upper lobe and a cavitating lesion with an air-fluid level in the apical segment of the right lower lobe distal to a lobulated mass ( fig. 1). Computed tomography (CT) demonstrated a further nodule in the left lower lobe and showed that the nodules were solid, well-circumscribed lesions containing a rim of calcification ( fig. 2). Eleven years previously, the patient had undergone a partial gastrectomy for a malignant leiomyosarcoma of the stomach, having presented with anaemia of 35 g·L -1 . A chest radiograph at that time was normal.Investigations showed: a white cell count of 21.2×10 9 cells·L -1 (95% neutrophils); haemoglobin level of 77 g·L -1 ; and erythrocyte sedimentation rate of 105 mm in the first hour. Bronchoscopy revealed extrinsic compression of the posterior wall of bronchus intermedius, and transbronchial biopsies from the apical segment of the right lower lobe showed groups of chondrocytes and chondroid matrix indicative of a chondroma, with no evidence of malignancy ( fig. 3). Transthoracic needle aspiration of the cavitating lesion yielded pus, but no organisms were isolated on culture. On upper gastointestinal endoscopy, there was no evidence of recurrence of the leiomyosarcoma. Urinary catecholamine levels were normal, and CT and an iodine-131 metaiodobenzylguanidine (MIBG) scan produced no evidence of a paraganglioma. The patient's blood group was We describe a young woman with Carney's syndrome, who developed a lung abscess, due to obstruction of a bronchus by a chondroma, 11 yrs after having had a partial gastrectomy for a leiomyosarcoma. Eur Respir J 1998; 11: 1409-1411.

Giant Pulmonary Chondromas as a Component of Carney’s Triad

“…Протекают бессимптомно, крайне редко у пациентов возникают жалобы на кровохарканье и боли в грудной клетке [9]. Описан случай формирования абсцесса с типичной клинической картиной на фоне обструкции бронхов хондромой [10]. Макроскопически имеют включения кальция и ограничены фиброзной капсулой [11].…”