The course of neurofibromatosis type 1 on immunosuppression after lung transplantation: report of 2 cases.
We describe 2 patients with neurofibromatosis type 1 (NF1) who underwent lung transplantation. Case 1 reports a patient with NF1 with no complications 5 years after bilateral lung transplantation. Case 2 details a patient with NF1 diagnosed with both post-transplant lymphoproliferative disorder (PTLD) and massive intra-abdominal sarcoma consistent with a malignant nerve sheath tumor 9 months after lung transplantation. There was no clinical evidence of sarcoma preceding or immediately following lung transplant as demonstrated by a normal abdominal sonogram 3 months post-transplantation. Although an increased risk for cancer has been documented in NF1, the rapid malignant degeneration of a neurofibroma following transplantation raises concern about immunosuppression and transplantation candidacy among individuals with NF1.
Merlo
Christian A
CA
Division of Pulmonary and Critical Care Medicine, The Johns Hopkins Hospital, Baltimore, Maryland, USA. cmerlo@jhmi.edu
Studer
Sean M
SM
Conte
John V
JV
Yang
Stephen C
SC
Sonnett
Joshua
J
Orens
Jonathan B
JB
D000008:Abdominal Neoplasms / Q000209:etiology*; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications*; D012509:Sarcoma / Q000209:etiology*