Purpose: Patients with metastatic colorectal cancer (mCRC) with BRAF V600E mutation have a poor response to standard cytotoxic agents and extremely poor outcomes. Thus, the optimal indication for metastasectomy remains debatable, even when the metastatic region is resectable. The aims of this retrospective study were to examine the outcomes of BRAF V600E mCRC, identify risk factors for poor survival, and establish the optimal surgical indication for resection. Materials and Methods: The subjects were 14 patients with BRAF V600E mCRC who started treatment in our department between December 2016 and April 2020. No patients underwent surgery prior to introduction of systemic chemotherapy. Patients were divided into two groups based on the initial response to first-line chemotherapy: controlled disease (CD, n=8) for tumors that shrunk or stabilized from baseline, and uncontrolled disease (UD, n=6) for tumors that increased in size. Results: The median follow-up period was 39.7 months. The 2-year overall survival rate was 35.7% in all patients, and was higher in the CD group than in the UD group (50.0% vs. 16.7%, P=0.051). Six patients (42.9%) underwent curative metastasectomy, of whom 4 developed recurrence and one underwent re-resection. In the CD group, 2 of 3 patients who underwent metastasectomy were alive without cancer, while all 3 patients in the UD group developed recurrence after metastasectomy and 2 had early recurrence within 3 months. Conclusion: This study suggests that evaluation of the initial response to first-line chemotherapy may be a useful prognostic factor in BRAF V600E mCRC. Even if a tumor is resectable, the indication for metastasectomy should be carefully considered in cases with poor disease control in the initial response.

Immune checkpoint inhibitors (ICIs) may cause immune-related adverse events (irAEs) mediated by immune mechanisms. Here, we report a case of unresectable advanced esophageal cancer in which severe irAEs developed due to an ICI. The patient was a 79-year-old male who was diagnosed with unresectable advanced esophageal cancer and underwent immunotherapy as third-line treatment. After four courses of immunotherapy, the patient developed ICI-related tuberculosis (TB). Immunotherapy was discontinued and TB treatment was given priority. Six months later, nivolumab was resumed in parallel with TB treatment, but three weeks after resumption of immunotherapy, immune thrombocytopenic purpura developed, in addition to immune-related liver and renal damage. Steroid treatment for irAEs led to normalization of platelet counts and improvement of liver and renal damage after two weeks. The patient was discharged from hospital after one month of this treatment. After discharge, there was no evidence of tumor progression for three months after the last administration of nivolumab.

Small cell gastric neuroendocrine carcinoma (SCNEC) is extremely rare. Here, we report a case of stage IV SCNEC treated with chemotherapy followed by radical surgery, after which the patient had asymptomatic survival for a long period of time. The patient was a 74-year-old male with a symptom of anemia. Gastric endoscopy indicated type II cancer in the posterior wall of the lesser curvature spreading from the central portion of the gastric body to the pyloric antrum. Histology with specific staining of tissue was LCA(–), synaptophysin(+), CD56(+), and chromogranin A(–). A few cells stained very weakly for cytokeratin, but most were negative. Ki-67/MIB-1 proliferation index was as high as 84.6%. These results led to diagnosis of gastric SCNEC. CT indicated para-aortic lymph node metastasis, and the case was diagnosed as T3, N1, M1 stage IV based on the Japanese Classification of Gastric Carcinoma (15th edition). The patient received one course of irinotecan (CPT-II)+carboplatin (CBDCA) chemotherapy, but side effects occurred and the next five courses of chemotherapy were changed to etoposide (VP16)+CBDCA. Follow-up imaging indicated significant tumor reduction, and hence, total gastrectomy was performed. After surgery, histology indicated elimination of cancer lesions and complete remission was achieved.

There are few reports of perioperative management of patients with glycogen storage disease type Ia. We report a case of hepatic resection for hepatocellular adenoma associated with glycogen storage disease type Ia that responded to strict perioperative management. The patient was a 20-year-old female with multiple hepatocellular adenomas who was diagnosed with glycogen storage disease type Ia at 2 years old. At age 19, she was diagnosed with focal segmental glomerulosclerosis secondary to glycogenosis, and renal transplantation was planned. Before renal transplantation, she was referred to our department for resection of hepatocellular adenomas >5 cm. Imaging revealed a 17-cm diameter mass in the lateral segment, a 6.5-cm diameter mass in S8, and multiple masses of <3 cm in diameter scattered throughout the liver. Left hepatectomy and S8 partial hepatectomy were performed. Histopathological analysis showed partial positive expression of β-catenin, glutamine synthetase, and cyclase-associated protein 2, which are indicators of malignancy. Therefore, the possibility of malignancy could not be ruled out. In the perioperative period, the patient was monitored using arterial blood gas analysis, and blood glucose was controlled by continuous high-concentration glucose administration. Using this approach, we were able to manage the patient without occurrence of significant lactic acidosis or hypoglycemia.

A 60-year-old man presented to our hospital with anemia. He had a history of left nephrectomy due to renal cell carcinoma 10 years ago. Upper gastrointestinal endoscopy revealed a tumor in the second portion of the duodenum. CT showed a mass of 6 cm protruding from the head of the pancreas into the duodenum. CT from five years ago showed a tumor measuring 1 cm in the pancreas head, indicating that the tumor had increased in size over time. The diagnosis was pancreatic metastasis of renal cell carcinoma with duodenal invasion. Subtotal stomach-preserving pancreaticoduodenectomy was performed. Seven days after surgery, the patient had acute cholangitis and developed septic shock concomitant with acute adrenal insufficiency, although some time was required to suspect acute adrenal insufficiency from hypoglycemia of unknown cause. Steroid treatment was started and subsequently the patient showed relatively rapid improvement and recovery, and was discharged 25 days after surgery. After discharge, follow-up of adrenal function revealed latent chronic adrenal insufficiency. This case serves as a reminder that surgeons should be aware of the risk of development of acute adrenal insufficiency in a case with high physical stress. Since acute adrenal insufficiency is life-threatening, this condition should be suspected in a case with refractory hypoglycemia and rapid therapy should be initiated.

A 52-year-old woman visited our hospital with complaints of recurrent urinary infection. An examination showed that the sigmoid colon was connected to an abscess in the pelvis. Surgery was planned, but before the operation could be performed the patient was admitted to our hospital for an obstructive urinary infection. A renogram showed that the right kidney was non-functional. Thus, laparoscopic total nephroureterectomy and sigmoid colectomy were performed, and a scar in the sigmoid colon was found to be adhered firmly to the right ureter. The patient was discharged from hospital 10 days after the operation. A pathological examination showed scars on the right ureter and sigmoid colon, and the final diagnosis was ureterocolic fistula. We report this case as a rare example of ureterocolic fistula induced by colonic diverticulitis in a Japanese patient, and we also discuss similar reports from other countries.

A 25-year-old woman with abdominal pain and diarrhea visited her local doctor and was diagnosed with ulcerative colitis. The inflammation went into remission after administration of prednisolone, but flared four months later. She was referred to our hospital because she subsequently became pregnant. The patient was diagnosed with total-colon type ulcerative colitis, Matts grade 4, and did not respond to infliximab and granulocyte-removal therapy; therefore, we decided that surgery was indicated. To ensure the safety of the mother and child, a three-stage divided surgery was planned. At 14 weeks of gestation, laparoscopic subtotal colon resection, ileal stoma, and fixation of the sigmoid colon to the abdominal wall were performed. At 3 months after a normal delivery (41 weeks of gestation, birth weight 2,665 g), the patient underwent laparoscopic total resection of the remaining colon, ileal pouch-anal anastomosis, and reconstruction of the ileal stoma. Finally, the ileal stoma was closed 6 months later. In this case, laparoscopic surgery was chosen after consultation with anesthesiologists, and the technique was devised to ensure safe continuation of pregnancy.