Primitive neuroectodermal tumor of the chest wall in a patient with Jeune's syndrome and renal transplant.
Jeune's syndrome is a rare autosomal disorder characterized by osseous dysplasia, fetal respiratory distress, and renal failure in later life. We describe a 27-year-old man with Jeune's syndrome who underwent renal transplantation and 6 years later developed a sarcoma (primitive neuroectodermal tumor [PNET]) in the soft tissue of the chest wall, a principal site of dysplasia in this disorder.
Department of Medicine, Abington Memorial Hospital, PA 19001.
J
J
Redmond
M P
MP
Richter
H D
HD
Stein
H E
HE
Auerbach
R A
RA
Jubelier
J S
JS
Brooks
D000328:Adult; D001238:Asphyxia Neonatorum / Q000150:complications*; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D009380:Neoplasms, Nerve Tissue / Q000209:etiology / Q000473:pathology*; D010009:Osteochondrodysplasias / Q000150:complications; D012983:Soft Tissue Neoplasms / Q000209:etiology / Q000473:pathology*; D013577:Syndrome; D013899:Thoracic Neoplasms / Q000209:etiology / Q000473:pathology*; D013909:Thorax / Q000002:abnormalities*