PMC5767566_01
Radiological findings on admission. Chest radiograph revealing right pleural effusion.
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The chest radiograph revealed right pleural effusion with mild to moderate retention (Fig. 1), and the chest CT scan showed moderate accumulation of the right pleural effusion with multiple thin-walled cysts in both lung fields (Fig. 2A).
Computed tomography (CT) scans (1.25-mm slice) on admission (A, B) and after 2 months of sirolimus treatment (C, D). A: Chest CT scan revealing moderate accumulation of the right pleural effusion and multiple thin-walled cysts (arrows) in both lung fields. B: Enhanced abdominal CT scan demonstrating ascites and a solid tumor (arrowheads) in the right pelvis. C: Chest CT scan revealing that the multiple small cysts in both lungs are stable and pleural effusion retention is decreased. D: Enhanced abdominal CT scan showing that the quantity of ascites fluid and size of the tumor lesion are smaller.
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The enhanced abdominal CT scan demonstrated ascites and slightly heterogeneous tumorous lesions of the right pelvis (Fig. 2B). Two months later, the serum albumin level became within normal range, and the enhanced abdominal CT scan revealed that the quantity of ascites fluid and size of the tumor lesion were smaller (Fig. 2D). Additionally, the multiple small cysts in both lungs were stable on the chest CT scan (Fig. 2C).
Patient's clinical course.
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The patient's clinical course is presented in Fig. 3.
Photographs before and after treatment. A: Photograph of the chylous ascites. B: Photograph of chyluria. C: Photograph of the transparent urine.
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The ascites fluid was chylous ascites (Fig. 4A). Postoperatively, we noticed that chyluria (Fig. 4B) had worsened, and a diagnosis of nephrotic syndrome was made (daily urinary protein level: 11,258 mg/day; daily urinary albumin level: 4675.45 mg/day). After 10 days of treatment with sirolimus, her urine became transparent (Fig. 4C), and hypoalbuminemia improved dramatically.
Results of the histopathological examination. A: The cell that had a kind of circular nucleus from a uniform circle in stained acidophilic cytoplasm forms a strand from a vacuole nest and multiplies (hematoxylin-eosin stain [HE], x200). B: A part of the same type of cell became spindle-shaped and complex (HE, x200). C-E: The immunohistochemical stain is positive for smooth muscle actin (C), HMB45 (D), and MelanA (E).
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According to the histopathological examination, the cell that had a kind of circular nucleus from a uniform circle in acidophilic cytoplasm stained by hematoxylin-eosin formed a strand from a vacuole nest and multiplied (Fig. 5A). Additionally, a part of the same type of cell became spindle-shaped and was complex (Fig. 5B). The immunohistochemical stain was positive for smooth muscle actin (Fig. 5C), HMB45 (Fig. 5D), and MelanA (Fig. 5E), leading to the diagnosis of LAM.