Repeated Resection for Recurrent Metastatic Solid Pseudopapillary Neoplasm of the Pancreas.

BACKGROUND Solid pseudopapillary neoplasm (SPN) accounts for 1.0% to 2.0% of all pancreatic neoplasms. SPN generally has good prognosis after surgery; however, 10% to 15% of patients have local recurrence or distant metastasis. There have been a few reports of successful surgical resection of isolated recurrent tumors after radical resection and sporadic reports of multiple metastasectomies. Herein, we present a case of recurrent SPN treated by repeated surgeries. CASE REPORT A 49-year-old woman was referred to our hospital with jaundice and right upper abdominal pain. Computed tomography (CT) scanning revealed a 73×43-mm heterogeneous mass in the pancreatic head. We performed a pancreatoduodenectomy and diagnosed SPN. The patient was discharged without any complications and was followed up by CT once every 6 to 12 months. Six years later, a 15×15-mm tumor was detected in Couinaud segment VI of the liver. A liver biopsy showed a pathological match to the pancreatic tumor. We performed a partial hepatectomy, and the pathology report confirmed metastatic SPN. At 8 and 10 years after the initial surgery, the patient underwent further partial hepatectomies for confirmed solitary liver metastases of SPN. The Ki-67 index increased for each metastasis identified (initial tumor, 1.88%; 6 years, 7.38%; 8 years, 5.53%; 10 years, 11.22%). No further masses were detected, and the patient survived more than 10 years following surgery. CONCLUSIONS Despite histological transformation to high-grade malignant disease, repeated aggressive surgical resection led to long-term survival in our patient with SPN.

A case of Williams syndrome with suspected coexisting ectopic aldosterone-producing tumor in the liver.

SUMMARY: A 31-year-old man with Williams syndrome (WS) was referred to our hospital because of a 9-year history of hypertension, hypokalemia, and high plasma aldosterone concentration to renin activity ratio. A diagnosis of primary aldosteronism (PA) was clinically confirmed but an abdominal CT scan showed no abnormal findings in his adrenal glands. However, a 13-mm hypervascular tumor in the posterosuperior segment of the right hepatic lobe was detected. Adrenal venous sampling (AVS) subsequently revealed the presence of an extended tributary of the right adrenal vein to the liver surrounding the tumor. Segmental AVS further demonstrated a high plasma aldosterone concentration (PAC) in the right superior tributary vein draining the tumor. Laparoscopic partial hepatectomy was performed. The resected tumor histologically separated from the liver was composed of clear cells, immunohistochemically positive for aldesterone synthase (CYP11B2), and subsequently diagnosed as aldosterone-producing adrenal adenoma. After surgery, his blood pressure, serum potassium level, plasma renin activity and PAC were normalized. To the best of our knowledge, this is the first report of WS associated with PA. WS harbors a high prevalence of hypertension and therefore PA should be considered when managing the patients with WS and hypertension. In this case, the CT findings alone could not differentiate the adrenal rest tumor. Our case, therefore, highlights the usefulness of segmental AVS to distinguish adrenal tumors from hepatic adrenal rest tumors. LEARNING POINTS: Williams syndrome (WS) is a rare genetic disorder, characterized by a constellation of medical and cognitive findings, with a hallmark feature of generalized arteriopathy presenting as stenoses of elastic arteries and hypertension. WS is a disease with a high frequency of hypertension but the renin-aldosterone system in WS cases has not been studied at all. If a patient with WS had hypertension and severe hypokalemia, low PRA and high ARR, the coexistence of primary aldosteronism (PA) should be considered. Adrenal rest tumors are thought to arise from aberrant adrenal tissues and are a rare cause of PA. Hepatic adrenal rest tumor (HART) should be considered in the differential diagnosis when detecting a mass in the right hepatic lobe. Segmental adrenal venous sampling could contribute to distinguish adrenal tumors from HART.

The Oncologic Outcomes of Inferior Mesenteric Artery-Preserving Laparoscopic Lymph Node Dissection for Upper-Rectal or Sigmoid Colon Cancer.

BACKGROUND: The standard procedure of lymph node (LN) dissection for rectal or sigmoid colon cancer remains controversial. Many laparoscopic surgeons cut the inferior mesenteric artery (IMA) at the root; however, this may cause bowel ischemia around the site of anastomosis. We performed D3 or D2 LN dissection with the preservation of the IMA. The aim of this study was to evaluate the oncologic outcomes of IMA-preserving LN dissection. PATIENTS AND METHODS: We retrospectively analyzed 142 consecutive patients with upper-rectal or sigmoid colon cancer who underwent laparoscopic LN dissection with the preservation of the IMA. The number of LNs dissected and the overall and disease-free survival rates were retrospectively evaluated. RESULTS: Laparoscopic D3 (n = 92) or D2 (n = 50) LN dissection were performed with the preservation of the IMA. The left colic artery was preserved in all cases, the superior rectal artery was also preserved in 30 cases. The mean number of LNs dissected was 19.6 in the D3 group and 12.9 in the D2 group. The median operative time was 230 minutes, and the median volume of blood loss was 17 mL. There was no treatment-related mortality. The estimated 5-year disease-free survival rates of the stage I, II, and III patients were 93%, 83%, and 74%, respectively. No patients developed LN recurrence. CONCLUSIONS: Laparoscopic surgery with IMA-preserving LN dissection is an oncologically acceptable treatment strategy in patients with upper-rectal or sigmoid colon cancer.

A laparoscopic approach is associated with a decreased incidence of SSI in patients undergoing palliative surgery for malignant bowel obstruction.

BACKGROUND: Several authors have reported on the utility of a laparoscopic approach for the palliation of malignant bowel obstruction (MBO); however, the advantages of laparoscopic surgery for MBO have not yet been confirmed. METHODS: We retrospectively reviewed the medical records of patients who underwent palliative surgery for MBO between 2007 and 2015. Laparoscopic procedures have been performed when technically possible since 2014. Successful palliation was defined as the ability to tolerate solid food (TSF) for at least 2 weeks. RESULTS: Twenty-two patients underwent laparoscopic palliative surgery, and 171 patients underwent conventional open palliative surgery to relieve the symptoms of MBO. Laparoscopic palliative surgery was performed for patients with MBO due to colorectal cancer (n = 12), uterine cancer (n = 3), and other types of cancers (including gastric, prostate, and renal cancer). The following laparoscopic procedures were performed: stoma placement (n = 18), palliative resection (n = 3) and bypass (n = 2). The median operative time was 100 min and the median operative blood loss was 9 ml. The laparoscopic palliative operation allowed 91% (20/22) of the patients to consume a solid diet for more than 2 weeks, and be discharged from hospital. There were no significant differences between laparoscopic surgery and open surgery with regard to the ability to TSF or the postoperative mortality rate. The postoperative morbidity (Clavien-Dindo Grade ≥ II) rates in the laparoscopic and open surgery groups were 14% and 32%, respectively. Laparoscopic surgery led to a significantly lower rate of postoperative surgical site infection (SSI) in comparison to open surgery (4.5% vs 32%; P = 0.0053). CONCLUSION: A laparoscopic approach in palliative surgery for MBO was safe and feasible, and was associated with a lower incidence of SSIs. By minimizing the postoperative morbidity rate, the laparoscopic approach may provide significant benefits to patients with MBO who have a limited life expectancy.

[Distal gastrectomy for initially unresectable gastric cancer with pyloric stenosis following gastrojejunostomy and S-1 plus CDDP chemotherapy].

We report a case of advanced gastric cancer with pyloric stenosis, in which a curative resection was performed following gastrojejunostomy and S-1 based chemotherapy. A 46-year-old female presenting with vomiting was diagnosed with unresectable gastric cancer with pyloric stenosis after a detailed examination. She underwent laparotomy, which revealed a T4 tumor invading the pancreas head and involving the gallbladder. A gastrojejunostomy was performed. After the operation, chemotherapy of S-1(100mg/body, days 1-21)plus cisplatin(85mg/body, day 8)was administered. After 4 courses, the tumor size was markedly reduced upon imaging examinations. Six months after gastrojejunostomy, distal gastrectomy was curatively performed. The pathological findings were type 3, por1, pT4a(SE), pN1, M0, pStage III A. After 5 courses of S-1(100mg/day, days 1-28)as adjuvant chemotherapy, she had a recurrence at a lymph node behind the pancreas head. Despite irinotecan+cisplatin following docetaxel therapy, she had no effective benefits and died from the cancer 17 months after the first operation. The prognosis of unresectable gastric cancer with pyloric stenosis is not promising; however, gastrojejunostomy following S-1-based chemotherapy could lead such patients to curative resection and a longer survival time.

Induction of donor-specific tolerance by adenovirus-mediated CD40Ig gene therapy in rat liver transplantation.

BACKGROUND: Blockade of CD40-CD40 ligand (CD154) costimulatory pathway with anti-CD154 antibody (Ab) prolongs allograft survival in experimental organ transplantations; however, repeated agent administration is needed to provide an adequate immunosuppression. Seeking for simple and effective approach to interfere this signaling, we applied adenovirus-mediated gene therapy by encoding CD40Ig gene (AdCD40Ig). METHODS: Liver graft from ACI (RT1av1) rat was transplanted orthotopically into LEW (RT1l) rat, and AdCD40Ig was given to animals via the penile vein immediately after grafting (n=6). RESULTS: A single treatment with AdCD40Ig at 1x10(9) plaque forming units induced specific expression of CD40Ig gene on allograft liver, produced substantial amount of the protein in the sera, and allowed indefinite graft survival. Whereas, LEW recipients given no treatment or control adenovirus vector (AdLacZ) promptly rejected ACI liver. In addition, AdCD40Ig-treated, long-term survivors accepted skin graft from the donor strain but not the third party graft. Histopathology revealed that liver structure of the long-term surviving animals was completely preserved in normal with no infiltration of mononuclear cells. CONCLUSION: Blockade of CD40-CD154 pathway by CD40Ig gene therapy is a potent alloantigen-specific immunosuppressive strategy to induce permanent acceptance of liver allograft and would be a new therapeutic candidate in a clinical liver transplantation.