Published in:
Open Access
01-12-2016 | Case report
A case of lymphangioleiomyomatosis associated with endometrial cancer and severe systemic lupus erythematosus
Authors:
Kensuke Suzuki, Kazunori Nagasaka, Katsutoshi Oda, Hiroyuki Abe, Daichi Maeda, Yoko Matsumoto, Takahide Arimoto, Kei Kawana, Masashi Fukayama, Yutaka Osuga, Tomoyuki Fujii
Published in:
BMC Cancer
|
Issue 1/2016
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Abstract
Background
Lymphangioleiomyomatosis (LAM) is a rare idiopathic disorder that occurs in women of childbearing age, and consists of a diffuse proliferation of abnormal smooth muscle cells along the thoracic and abdominal lymphogenous route.
Case presentation
We experienced a case of a 47-yo woman with recent history of systemic lupus erythematosus (SLE) diagnosed with endometrial cancer, initially suspected to have metastasized to pelvic and para-aortic lymph nodes based on preoperative diagnostic imaging. Subsequent pathological diagnosis revealed stage IB endometrial cancer without evidence of lymph node involvement. Instead, enlarged pelvic and para-aortic lymph nodes were found to be due to extrapulmonary LAM, from a primary lesion found inside the uterine myometrium. SLE improved after surgery.
Conclusion
This is the first reported case of comorbid endometrial cancer, SLE, and aggressive LAM metastasizing to regional lymph nodes, and strengthens the clinical evidence for a common role of mTOR pathway hyperactivity and estrogen responsiveness in the pathophysiology of metastasizing lesions of the genital tract.