Intrathoracic chemodectoma with multiple localisations.

Abstract

a mediastinal tumour was discovered in an asymptomatic woman with a history of vomiting and an oesophageal anomaly which had not been treated. A tumour of the anterosuperior mediastinum in relation to the aortic arch was extirpated and proved to be a chemodectoma or non-chromaffin paraganglioma.At subsequent follow-ups the mediastinum was never normal and the heart size progressively increased, the oesophageal anomaly remaining unchanged. A small opacity appeared in the left lung in 1974. An operation performed in 1975 revealed an osteochondroma in the lung, pericarditis, and an intrapericardial chemodectoma. The oesophagus was not explored.An intrathoracic chemodectoma is rare. The importance of angiography in its diagnosis is emphasised. Malignant degeneration is seldom observed. Therapy is surgical, the tumour being radioresistant. The possibility to be considered in our patient was either relapse of the tumour with degeneration or multiple localisations of the tumour.

Gastric Stromal Sarcoma, Pulmonary Chondroma, and Extra-adrenal Paraganglioma (Carney Triad): Natural History, Adrenocortical Component, and Possible Familial Occurrence

“…Seven patients (9%) had esophageal tumors that were excised in 4 patients (leiomyoma in 3, stromal tumor in 1). One unresected tumor had been present for 37 years, 14 and another had not changed in size for more than 6 years. 37 The tumors were asymptomatic except in 1 case.…”

Lung abscess complicating chondromas in Carney's syndrome

“…In some cases, they have grown slowly without giving rise to complications over a follow-up period of as long as 30 yrs [5]. In two cases, the chondromas have caused narrowing of airways with distal infection, but the development of a lung abscess, as in the present patient, has not previously been reported [6,7]. …”

“…The syndrome has been incomplete in 74% of reported cases, with only two components being present [1][2][3][4][5][6][7][8][9][10]. The aetiology is unknown as these tumours do not share a common embryological origin, and no familial or genetic basis has been found.…”

“…In 11 cases the chondromas have been part of a complete triad of tumours, and in 25 cases they have occurred with a gastric leiomyosarcoma [1][2][3][4][5][6][7][8][9][10]. Typically, there is a long interval between diagnosis of the different components of the syndrome.…”

“…Bourke* aa 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.…”