[Malignant rhabdoid tumor of the lung].
BACKGROUND
Rhabdoid tumours usually develop in brain and spinal cord or kidney; they are highly malignant neoplasms that typically arise in infancy and early childhood. However, rare cases of pulmonary localization have been described, particularly among young adults.


METHODS
A 26-year-old man, smoker, had a right apical lung mass associated with a Pancoast syndrome leading to haemoptysis. There was also a tumour of the left thigh and scalp. Histological samples taken at these three locations were in favour of an undifferentiated carcinoma. The lack of nuclear integrase interactor 1 expression, and immunohistochemical appearance supported the diagnosis of rhabdoid tumour. Despite treatment, unfavourable progression confirmed this hypothesis, doubling time was less than six weeks with development of multiple metastases resulted in death within only three months after diagnosis.


CONCLUSIONS
The lack of expression of integrase interactor 1 should suggest the diagnosis of rhabdoid tumour, especially when there is quick progression. The prognosis of these tumours remains poor and therapeutic options are limited.
Service de pneumologie, hôpitaux de Brabois, CHU de Nancy, 54500 Vandœuvre-lès-Nancy, France. Electronic address: m.zysman@chu-nancy.fr.
M
M
Zysman
Service de pneumologie, hôpitaux de Brabois, CHU de Nancy, 54500 Vandœuvre-lès-Nancy, France.
C
C
Clement-Duchene
Service d'anatomopathologie, hôpitaux de Brabois, CHU de Nancy, 54500 Vandœuvre-lès-Nancy, France.
C
C
Bastien
Service de pneumologie, hôpitaux de Brabois, CHU de Nancy, 54500 Vandœuvre-lès-Nancy, France.
P
P
Vaillant
Service de pneumologie, hôpitaux de Brabois, CHU de Nancy, 54500 Vandœuvre-lès-Nancy, France.
Y
Y
Martinet
D000328:Adult; D017809:Fatal Outcome; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D009362:Neoplasm Metastasis; D013902:Radiography, Thoracic; D035583:Rare Diseases; D018335:Rhabdoid Tumor / Q000000981:diagnostic imaging / Q000473:pathology*
Integrase interactor 1; Rare lung tumors; Rhabdoid tumour; Tumeur rhabdoïde; Tumeurs pulmonaires rares