PMC4501452_01
Chest CT and pelvic MRI. A and B. Chest CT demonstrates diffusely distributed small cystic lesions with reticular opacity. C and D. T2-weighted (C) and diffusion-weighted (D) MRI show two large tumors, one of which (arrow) is located in the myometrium and the other (arrowhead) is located adjacent to the pelvic wall. The diffusion-weighted MRI reveals that the two tumors have different patterns. The retroperitoneal tumor(arrowhead) is higher intensity than the uterine tumor. E. A sagittal section of pelvic MRI shows a tumor (arrow) in the myometrium of the uterus (asterisk) with a very strong contrasting effect.
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Chest CT demonstrated diffusely distributed small cystic lesions, reticular opacity and micronodular lesions (Fig. 1A, B). Transabdominal and transvaginal ultrasound examinations showed a suspected leiomyoma of the uterus, but during systemic surveillance, pelvic MR imaging showed two large tumors with a very strong contrasting effect in the myometrium of the uterine corpus and retroperitoneal space (Fig. 1C-E).
Histopathological findings of the surgical biopsy of the lung. A and B. Hematoxylin-Eosin staining of the lung biopsy samples shows diffusely distributed small cystic lesions. Some cyst walls exhibit the accumulation of spindle-shaped cells. (B: x100). C-F. Immunohistochemistry demonstrates positive staining for alphaSMA, HMB45, estrogen receptor (ER) and D2-40. (C and F: x100, D and E: x400).
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The immunohistochemical examination of the LAM cells was positive for both estrogen and progesterone receptors(ER, PR, respectively) (Fig. 2A-F).
Histopathological findings of the resected uterine and retroperitoneal tumors. A. A gross examination shows the uterine tumor resected via total hysterectomy with BSO. The tumor is in the right posterior wall of the uterus (arrow). The surface of the retroperitoneal tumor is rugged (inset). B. Low magnification view of the uterine tumor (H-E stain). C and D. High magnification view of the uterine tumor (H-E (C) and D2-40 (D) staining). (x100). E and F. High magnification view of the myometrium showing H-E (E) and D2-40 (F) staining. There are LAM cell clusters in the myometrium and slit-like lesions (D2-40-positive) which mean lymphatic vessels. (x100). G and H. High magnification view of the uterine tumor showing LAM cell infiltration in fat tissue (H-E (G: x200) and D2-40 (H: x400) staining). I. High magnification view of LAM cell cluster(LCC) enveloped by D2-40 positive lymphatic endothelial cells. LCC is composed by LAM cells with HMB-45 positive staining(inset). J, K, L and M. Low magnification view of the uterine tumor showing LAM cell infiltration (ER, PR, HMB45 and alphaSMA staining, x100). N, O, P and Q. Low magnification view of the retroperitoneal tumor showing LAM cell infiltration (ER, PR, HMB-45 and alphaSMA staining, x100).
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The size of tumor in the myometrium of the uterine corpus was 45 x 45 x 25 mm, while that of the retroperitoneal tumor was 70 x 60 x 35 mm (Fig. 3A). Macroscopically, well-circumscribed, yellowish and fragile tumors with cleft-like lesions were observed from the myometrium of the uterine corpus to the serous membrane borderline (Fig. 3B). Accordingly, these tumors were diagnosed as uterine and retroperitoneal LAM lesions (Fig. 3C-Q).
Clinical course Bronchodilators were initially introduced without any improvements in the patient's symptoms or pulmonary function. Uterine tumor resection concomitant with bilateral salpingo-oophorectomy (BSO) slightly reduced the decline in FEV1. However, home oxygen therapy was administered following tumorectomy, and treatment with sirolimus was initiated. An apparent improvement in the FEV1 was observed following the introduction of sirolimus treatment. No obvious regrowth of the uterine tumors has since been noted. The serum KL-6 level fluctuated during the sirolimus treatment.
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As a consequence, the dyspnea and FEV1 slope recovered (Fig. 4).