A case of lymphangioleiomyomatosis with diffuse large B-cell lymphoma: Usefulness of FDG-PET

• Published in: Respiratory medicine case reports 2020-01, Vol.29, p.100999, Article 100999
• Main Authors: Orimo, Mami, Kondo, Mitsuko, Takeyama, Kiyoshi, Masui, Kenta, Tanaka, Junji, Tagaya, Etsuko
• Format: Article
• Language: English
• Subjects: Case Report; Diffuse large B-cell lymphoma (DLBCL); FDG-PET; Lymphangioleiomyomatosis (LAM)
• ISSN: 2213-0071; 2213-0071
• DOI: 10.1016/j.rmcr.2020.100999

Lymphangioleiomyomatosis (LAM) is characterized by cystic lung disease, abdominal tumor and involvement of the axial lymph nodes. We report a very rare case of LAM with malignant lymphoma. A 51-year-old female had medical history of recurrent pneumothorax and nephrectomy for a left renal angiomyolipoma and was diagnosed with LAM by video-assisted thoracoscopic surgery at the age of 30. She presented with left neck mass. Computed tomography and magnetic response imaging showed multiple enlarged cervical lymph nodes. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed abnormal uptake in the mass. We suspected a malignant tumor or extrapulmonary lesion of LAM, and performed surgical biopsy. Pathologically, diffuse large B-cell lymphoma was diagnosed, but LAM was not detected. After she received six cycles of R–CHOP chemotherapy, FDG-PET turned negative and complete metabolic response was achieved. As LAM is reported to be silent for FDG-PET, unusual uptake of FDG is useful for identifying other neoplasms. For this case, FDG-PET was useful for the differential diagnosis and therapeutic evaluation.