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Gastric cancer following a liver transplantation for glycogen storage disease type Ia (von Gierke disease): A case report.

Xiao H, Bian J, Zhang L, Wang Z, Ding A - Oncol Lett (2014)

Bottom Line: Thus, a total gastrectomy with lymph node dissection was performed and the tumor was histologically determined to be a poorly differentiated adenocarcinoma (histopathological stage, pT4N1M0).The patient recovered well and was discharged on postoperative day 10 without any complications.To the best of our knowledge, this is the first case of de novo gastric cancer in a patient with GSD-Ia to be reported.

View Article: PubMed Central - PubMed

Affiliation: Department of General Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu 210006, P.R. China.

ABSTRACT
Glycogen storage disease type Ia (GSD-Ia; also termed von Gierke disease) is an inherited metabolic disorder resulting from a glucose-6-phosphatase deficiency. Liver transplantation is considered to be the most effective treatment for GSD-Ia patients. In the present study, the case of a patient with GSD-Ia who received a liver transplantation at 17 years of age is presented. During the 12 years following transplantation, the patient's quality of life markedly improved. However, recently, the patient was diagnosed with de novo gastric cancer following a biopsy. Thus, a total gastrectomy with lymph node dissection was performed and the tumor was histologically determined to be a poorly differentiated adenocarcinoma (histopathological stage, pT4N1M0). The patient recovered well and was discharged on postoperative day 10 without any complications. To the best of our knowledge, this is the first case of de novo gastric cancer in a patient with GSD-Ia to be reported.

No MeSH data available.


Related in: MedlinePlus

(A) Surgical findings revealed that the gastric posterior wall was extensively rigid and the margins were irregular (white arrow). (B) Histopathological examination of the resected tissue indicated poorly differentiated adenocarcinoma and a number of signet-ring cell carcinoma cells (white arrows) (stain, hematoxylin and eosin; magnification, ×100).
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f2-ol-08-06-2803: (A) Surgical findings revealed that the gastric posterior wall was extensively rigid and the margins were irregular (white arrow). (B) Histopathological examination of the resected tissue indicated poorly differentiated adenocarcinoma and a number of signet-ring cell carcinoma cells (white arrows) (stain, hematoxylin and eosin; magnification, ×100).

Mentions: In June 2001, a 29-year-old male underwent a modified piggy-back liver transplantation for von Gierke disease. Following the surgery, the patient’s quality of life markedly improved. Tacrolimus (3 mg, every 12 h for one year), mycophenolate mofetil (1000 mg, every 12 h for six months), and prednisone (5 mg, every 12 h for two months) were administered for immunosuppression following surgery and currently, the patient receives 50 mg Ciclosporin in the morning and 75 mg at night, daily. In August 2013, the patient was admitted to Nanjing First Hospital (Nanjing, China) due to melena that had been occurring for three days. The melena disappeared following conservative treatment, which included hydration with water, glucose, electrolytes and amino acids, fasting and the administration of Losec, a proton-pump inhibitor. A gastric endoscopy was subsequently performed and revealed a deep ulcer with a necrotic gray area in the base, which was located in the posterior wall of the fundus region of the stomach, accompanied by edema in the immediately adjacent mucosa. Histological examination of the biopsied specimen revealed signet-ring cells in the muscularis propria (Fig. 1A) and immunochemical staining was positive for epithelial membrane antigen (Fig. 1B) and cytokeratin (Fig. 1C) expression, which indicated the diagnosis of signet-ring cell carcinoma. Enhanced computed tomography scanning and magnetic resonance imaging of the upper abdomen revealed a thickened stomach wall. In addition, pre-operative serological examination revealed that the α-fetoprotein (3.46 ng/ml; normal range, <20 ng/ml), carcinoembryonic antigen (2.37 ng/ml; normal range, <10 ng/ml) and carbohydrate antigen 19-9 (12.58 U/ml; normal range, <37 U/ml) levels were normal. Following the diagnosis of de novo gastric cancer, surgery was performed in September 2013. During the laparotomy, a broad region of the gastric posterior wall was identified to be rigid with irregular margins (Fig. 2A). The tumor measured ~9.5×8.5 cm in diameter. Subsequently, a total gastrectomy with lymph node dissection was performed. Reconstruction was performed using the Rous-en-Y esophagojejunostomy method and a naso-intestinal feeding tube was inserted into the distal bowel. Following surgery, routine nutritional support and immunosuppressive medications were administered. Histopathological examination of the resected stomach tissue revealed poorly differentiated adenocarcinoma with a number of partially signet-ring carcinoma cells (Fig. 2B); the invasion penetrated the wall via the serosa with perigastric lymph node metastasis (1/11 lymph nodes removed from the lesser curvature and 0/4 lymph nodes from the greater curvature were pathologically confirmed to be metastatic). The tumor was staged as pT4N1M0 with no distant metastasis. The patient recovered well and was discharged on postoperative day 10 without any complications.


Gastric cancer following a liver transplantation for glycogen storage disease type Ia (von Gierke disease): A case report.

Xiao H, Bian J, Zhang L, Wang Z, Ding A - Oncol Lett (2014)

(A) Surgical findings revealed that the gastric posterior wall was extensively rigid and the margins were irregular (white arrow). (B) Histopathological examination of the resected tissue indicated poorly differentiated adenocarcinoma and a number of signet-ring cell carcinoma cells (white arrows) (stain, hematoxylin and eosin; magnification, ×100).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4214470&req=5

f2-ol-08-06-2803: (A) Surgical findings revealed that the gastric posterior wall was extensively rigid and the margins were irregular (white arrow). (B) Histopathological examination of the resected tissue indicated poorly differentiated adenocarcinoma and a number of signet-ring cell carcinoma cells (white arrows) (stain, hematoxylin and eosin; magnification, ×100).
Mentions: In June 2001, a 29-year-old male underwent a modified piggy-back liver transplantation for von Gierke disease. Following the surgery, the patient’s quality of life markedly improved. Tacrolimus (3 mg, every 12 h for one year), mycophenolate mofetil (1000 mg, every 12 h for six months), and prednisone (5 mg, every 12 h for two months) were administered for immunosuppression following surgery and currently, the patient receives 50 mg Ciclosporin in the morning and 75 mg at night, daily. In August 2013, the patient was admitted to Nanjing First Hospital (Nanjing, China) due to melena that had been occurring for three days. The melena disappeared following conservative treatment, which included hydration with water, glucose, electrolytes and amino acids, fasting and the administration of Losec, a proton-pump inhibitor. A gastric endoscopy was subsequently performed and revealed a deep ulcer with a necrotic gray area in the base, which was located in the posterior wall of the fundus region of the stomach, accompanied by edema in the immediately adjacent mucosa. Histological examination of the biopsied specimen revealed signet-ring cells in the muscularis propria (Fig. 1A) and immunochemical staining was positive for epithelial membrane antigen (Fig. 1B) and cytokeratin (Fig. 1C) expression, which indicated the diagnosis of signet-ring cell carcinoma. Enhanced computed tomography scanning and magnetic resonance imaging of the upper abdomen revealed a thickened stomach wall. In addition, pre-operative serological examination revealed that the α-fetoprotein (3.46 ng/ml; normal range, <20 ng/ml), carcinoembryonic antigen (2.37 ng/ml; normal range, <10 ng/ml) and carbohydrate antigen 19-9 (12.58 U/ml; normal range, <37 U/ml) levels were normal. Following the diagnosis of de novo gastric cancer, surgery was performed in September 2013. During the laparotomy, a broad region of the gastric posterior wall was identified to be rigid with irregular margins (Fig. 2A). The tumor measured ~9.5×8.5 cm in diameter. Subsequently, a total gastrectomy with lymph node dissection was performed. Reconstruction was performed using the Rous-en-Y esophagojejunostomy method and a naso-intestinal feeding tube was inserted into the distal bowel. Following surgery, routine nutritional support and immunosuppressive medications were administered. Histopathological examination of the resected stomach tissue revealed poorly differentiated adenocarcinoma with a number of partially signet-ring carcinoma cells (Fig. 2B); the invasion penetrated the wall via the serosa with perigastric lymph node metastasis (1/11 lymph nodes removed from the lesser curvature and 0/4 lymph nodes from the greater curvature were pathologically confirmed to be metastatic). The tumor was staged as pT4N1M0 with no distant metastasis. The patient recovered well and was discharged on postoperative day 10 without any complications.

Bottom Line: Thus, a total gastrectomy with lymph node dissection was performed and the tumor was histologically determined to be a poorly differentiated adenocarcinoma (histopathological stage, pT4N1M0).The patient recovered well and was discharged on postoperative day 10 without any complications.To the best of our knowledge, this is the first case of de novo gastric cancer in a patient with GSD-Ia to be reported.

View Article: PubMed Central - PubMed

Affiliation: Department of General Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu 210006, P.R. China.

ABSTRACT
Glycogen storage disease type Ia (GSD-Ia; also termed von Gierke disease) is an inherited metabolic disorder resulting from a glucose-6-phosphatase deficiency. Liver transplantation is considered to be the most effective treatment for GSD-Ia patients. In the present study, the case of a patient with GSD-Ia who received a liver transplantation at 17 years of age is presented. During the 12 years following transplantation, the patient's quality of life markedly improved. However, recently, the patient was diagnosed with de novo gastric cancer following a biopsy. Thus, a total gastrectomy with lymph node dissection was performed and the tumor was histologically determined to be a poorly differentiated adenocarcinoma (histopathological stage, pT4N1M0). The patient recovered well and was discharged on postoperative day 10 without any complications. To the best of our knowledge, this is the first case of de novo gastric cancer in a patient with GSD-Ia to be reported.

No MeSH data available.


Related in: MedlinePlus