Background. Multifocal micronodular pneumocyte hyperplasia (MMPH) and pulmonary lymphangiomyomatosis (LAM) are complications of tuberous sclerosis complex (TSC). We herein report a case of pulmonary adenocarcinoma in a patient with TSC and MMPH, along with a review of the literature.
Case. A 69-year-old female who had been diagnosed with TSC at 13 years of age was admitted to our hospital for an abnormal chest shadow, which had initially been noticed when she was 21 years old. Computed tomography showed a tumor shadow with a spicular structure in the right upper lung lobe (S
3) and multiple small nodules in the whole lung field. An FDG-PET scan showed fluorodeoxyglucose accumulation in the S
3 nodule of interest, but not in other nodules. Because she was diagnosed with lung cancer of stage cT1aN0M0 (IA), we performed an S
3 segmentectomy. The specimen was found to be adenocarcinoma. Many micronodules were seen in the remaining specimen, most of which were compatible with MMPH, but several adenocarcinomas with a lepidic pattern were also detected. Currently, three years post-surgery, she has shown no signs of recurrence, but as her serum CEA level has been gradually increasing, so we will continue strict observation.
Conclusion. Patients with the
TSC gene may be at risk for pulmonary adenocarcinoma. Further studies are needed to determine whether this is the case.
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