GIST With Nephrotic Syndrome as a Paraneoplastic Syndrome
GIST With Nephrotic Syndrome as a Paraneoplastic Syndrome
A 69-year-old Asian woman was admitted to our hospital because of diminished appetite. She had no previous medical history and was not complaining of fever and, therefore, she was not taking oral non-steroid anti-inflammatory drugs. Computed tomography revealed a gastric tumor measuring 56 × 55mm, accompanied by calcification (Figure 1). Angiography via her left gastric artery showed staining of the tumor (Figure 2), and endoscopic ultrasonography demonstrated a heterogeneous lesion 60mm in diameter, arising in the posterior wall of the stomach (Figure 3). Laboratory examinations revealed hypoproteinemia (4.8g/dL; normal range, 6.5 to 8.0), hypoalbuminemia (2.2g/dL; normal range, 3.8 to 5.3), and severe proteinuria (8.47g/dL; normal range, 0 to 0.15), and her protein creatinine ratio was 14.3g/g and amount of urine for 24 hours was 1200mL, clinically suggesting nephrotic syndrome. Therefore, to avoid an invasive inspection, a kidney biopsy was not carried out. There were no neurologic symptoms or skin disease.
(Enlarge Image)
Figure 1.
Computed tomographic findings of the tumor. A computed tomographic scan showing a large tumor measuring 56 × 55mm accompanied by calcification. An arrow indicates the tumor; the arrow head indicates the stomach.
(Enlarge Image)
Figure 2.
Angiographic findings of the tumor. Angiography via the left gastric artery showed staining of the tumor.
(Enlarge Image)
Figure 3.
Endoscopic ultrasonographic findings of the tumor. Endoscopic ultrasonography demonstrated a heterogeneous lesion 60mm in diameter, arising in the posterior wall of the stomach.
Despite albumin administration, her hypoalbuminemia did not improve preoperatively. She was given a diagnosis of GIST of the stomach accompanied by nephrotic syndrome, and partial gastrectomy was performed. Macroscopically, a well-demarcated, hard, whitish submucosal tumor arose from the gastric wall (Figure 4a); histological and immunohistochemical examinations revealed a spindle-cell tumor positive for CD34 and c-kit (Figure 4b, 4c), and a GIST was diagnosed.
(Enlarge Image)
Figure 4.
Pathological findings of the tumor. (a) Macroscopically, the tumor appeared to be gray-whitish and hard and arose from the gastric wall. (b,c) Both CD34 and c-kit were positive on immunohistochemical staining.
Her postoperative course was uneventful. One week after her operation, her serum albumin (3.3g/dL) and urinary protein (1.71g/dL) levels remarkably improved after removal of the tumor. She remains well with no recurrence of the tumor or nephrotic syndrome (her recent urinary protein level: 1.19mg/mL) 2 years after surgery.
Case Presentation
A 69-year-old Asian woman was admitted to our hospital because of diminished appetite. She had no previous medical history and was not complaining of fever and, therefore, she was not taking oral non-steroid anti-inflammatory drugs. Computed tomography revealed a gastric tumor measuring 56 × 55mm, accompanied by calcification (Figure 1). Angiography via her left gastric artery showed staining of the tumor (Figure 2), and endoscopic ultrasonography demonstrated a heterogeneous lesion 60mm in diameter, arising in the posterior wall of the stomach (Figure 3). Laboratory examinations revealed hypoproteinemia (4.8g/dL; normal range, 6.5 to 8.0), hypoalbuminemia (2.2g/dL; normal range, 3.8 to 5.3), and severe proteinuria (8.47g/dL; normal range, 0 to 0.15), and her protein creatinine ratio was 14.3g/g and amount of urine for 24 hours was 1200mL, clinically suggesting nephrotic syndrome. Therefore, to avoid an invasive inspection, a kidney biopsy was not carried out. There were no neurologic symptoms or skin disease.
(Enlarge Image)
Figure 1.
Computed tomographic findings of the tumor. A computed tomographic scan showing a large tumor measuring 56 × 55mm accompanied by calcification. An arrow indicates the tumor; the arrow head indicates the stomach.
(Enlarge Image)
Figure 2.
Angiographic findings of the tumor. Angiography via the left gastric artery showed staining of the tumor.
(Enlarge Image)
Figure 3.
Endoscopic ultrasonographic findings of the tumor. Endoscopic ultrasonography demonstrated a heterogeneous lesion 60mm in diameter, arising in the posterior wall of the stomach.
Despite albumin administration, her hypoalbuminemia did not improve preoperatively. She was given a diagnosis of GIST of the stomach accompanied by nephrotic syndrome, and partial gastrectomy was performed. Macroscopically, a well-demarcated, hard, whitish submucosal tumor arose from the gastric wall (Figure 4a); histological and immunohistochemical examinations revealed a spindle-cell tumor positive for CD34 and c-kit (Figure 4b, 4c), and a GIST was diagnosed.
(Enlarge Image)
Figure 4.
Pathological findings of the tumor. (a) Macroscopically, the tumor appeared to be gray-whitish and hard and arose from the gastric wall. (b,c) Both CD34 and c-kit were positive on immunohistochemical staining.
Her postoperative course was uneventful. One week after her operation, her serum albumin (3.3g/dL) and urinary protein (1.71g/dL) levels remarkably improved after removal of the tumor. She remains well with no recurrence of the tumor or nephrotic syndrome (her recent urinary protein level: 1.19mg/mL) 2 years after surgery.
