[A Case of Malignant Peripheral Nerve Sheath Tumor of the Anus Resected by Robot Assisted-APR].

An 81-year-old female visited a local hospital with complaints of anal pain. A tumor was found on the right side of her anus, and the histopathological diagnosis was a non-epithelial malignant tumor. Therefore, the patient was referred to our hospital. Result of imaging inspection revealed that the tumor had invaded the lower rectum, but had not distantly metastasized. Based on the findings of another biopsy, the patient was diagnosed with a malignant peripheral nerve sheath tumor (MPNST). Robot-assisted abdominoperineal resection(D1)was performed, and the lesion was resected without any pathological remnants. During the postoperative period, the patient developed perineal wound infection. Subsequently, the patient was discharged from the hospital on postoperative day 10. At the 6-month postoperative follow-up, no recurrence was noted. Most MPNSTs occur in the limbs, trunk, and neck. MPNST in the primary gastrointestinal tract or in the vicinity of the gastrointestinal tract is relatively rare, and in principle, combined resection of the intestinal tract is required for surgical treatment. Here, we report a case of MPNST that occurred near the anus and infiltrated to the lower rectum and was completely resected by robot-assisted abdominoperineal resection.

Late hemorrhage after pancreatoduodenectomy and heavy ion beam therapy.

The patient was a 57-year-old man diagnosed with cancer of the pancreatic head. After treatment by heavy ion beam therapy, pylorus-preserving pancreatoduodenectomy was performed. The tumor was pT3, pN0, pM0, stage IIA. Sixteen months after the surgery, the patient was admitted to the hospital because he was vomiting blood. Hemorrhaging caused by failure of the cut end of the gastroduodenal artery into the elevated jejunum was confirmed by angiogram, and the hemorrhaging could be stopped by a transcatheter arterial embolization operation. Twenty-four months after surgery, the patient was readmitted because he was once again vomiting blood. Hemorrhaging from the elevated jejunum was suspected by hemorrhagic scintigram, but the source could not be identified on further examination, and the choice of treatment was difficult. The patient died on the 9th day after admittance to the hospital. Even on examination at autopsy, the source of the hemorrhaging could not be identified. No recurrence of cancer could be found. This has proven to be a perplexing case, in that hemorrhaging from the end of the routinely cut gastroduodenal artery occurred 16 and 24 months after heavy ion beam therapy and pylorus-preserving pancreatoduodenectomy for pancreatic cancer.

Repeated pancreatectomy after pancreatoduodenectomy.

BACKGROUND: When pancreatic duct dilatation is found in the patient having undergone pancreatoduodenectomy (PD), observation is chosen in most cases. Similarly, recurrent tumor in the remnant pancreas of invasive ductal carcinoma (IDC) of the pancreas is seldom indicated for resection. We have aggressively performed repeated pancreatectomy for these cases and obtained good results. METHODS: Repeated pancreatectomy after PD was performed for three types of circumstances: (1) pancreatodigestive anastomotic stricture; (2) neoplasm after intraductal papillary mucinous neoplasm (IPMN); and (3) recurrence of IDC of the pancreas. RESULTS: Resection of anastomosis and reanastomosis was performed for pancreatodigestive stricture in four patients. Symptoms derived from pancreatitis in three patients resolved by the second operation and did not recur during follow-up. None of the four patients required pancreatic enzyme substitution because of clinically overt malabsorption, and the defecation frequency of the four patients was within twice a day. Mild diabetes mellitus has been identified in only one patient who had diabetes mellitus before the second surgery. Completion pancreatectomy and pancreatic tail resection was performed for recurrence in two patients and IDC in one patient, respectively, after PD for IPMN. Intrapancreatic recurrences of IPMN in two patients existed in the main pancreatic ducts. As CT revealed pancreatic duct dilatation but not intraductal tumors, recurrences were not correctly diagnosed before the second operation. Completion pancreatectomy was performed for recurrence of IDC in two patients. One patient who underwent completion pancreatectomy for recurrence of IDC survived 66/44 months after the first/second operation. CONCLUSION: Repeated pancreatectomy should be performed for patients with pancreatodigestive anastomotic stricture to preserve remnant pancreatic function and for patients with neoplasm or pancreatic duct dilatation after PD for IPMN, and repeated pancreatectomy for recurrence of IDC might be indicated for selected patients.

[Trend in the management of pancreatic adenocarcinoma--Japan vs. US and Europe].

Pancreatic adenocarcinoma remains to have poor prognosis. Most of all patients have locally advanced disease with or without distant disease when diagnosed. Current rationale for the treatment of pancreatic adenocarcinoma in the US and European countries consists of the following formula: (1) accurate staging by improved imaging. (2) a balanced-resection which means not too extensive not too limited. (3) centralized treatment in high-volume center with minimal surgical mortality. (4) surgery alone is not enough for cure and need more radical adjuvant or neoadjuvant therapy. On the other hands, Japanese surgeons had challenged to improve outcome by radical resections but did not show their advantages in terms of survival benefit as shown in recent randomized controlled trials. Now we should look back to surgical role and think 'who can benefit by surgical resection'. The efficacy of "Japanese" radical resection including vascular resection or pancreatic nerve plexus resection should be evaluated, although the devise of novel diagnostic modalities and more effective adjuvant or neoadjuvant therapy are crucial to improve prognosis of this disease.

A case of peribiliary cysts accompanying bile duct carcinoma.

A rare case of peribiliary cysts accompanying bile duct carcinoma is presented. A 54-year-old man was diagnosed as having lower bile duct carcinoma and peribiliary cysts by diagnostic imaging. He underwent pylorus preserving pancreatoduodenectomy. As for the peribiliary cysts, a course of observation was taken. Over surgery due to misdiagnosis of patients with biliary malignancy accompanied by peribiliary cysts should be avoided.

The lethal toxicity of pancreatic ascites fluid in severe acute necrotizing pancreatitis.

BACKGROUND/AIMS: The pathogenesis and the molecular mechanisms of the development and progression of the acute pancreatitis (AP) are not clearly understood. Ascites fluid is known to be important in the clinical progression of AP. We present the lethal toxicity of human pancreatic ascites fluid for experimental pancreatitis, with the therapeutic course of severe necrotizing AP. METHODOLOGY: The material was in a 33-year-old male admitted with epigastric pain. An abdominal CT revealed that his pancreas was swollen and contained pancreatic fluid collection extending to the pelvic cavity. He had complicated acute renal failure, sepsis, and DIC, and received hemodialysis, and continuous arterial infusion therapy (CAI). The peripancreatic infection was acquired, and percutaneous interventional radiology (IVR) was performed for the abscess drainage. The drained liquid around the pancreas contained high molecular cytokines, protease, and bacterial contamination. To evaluate the toxicity of the ascites fluid, we gave it intraperitoneally to rats in which pancreatitis had been induced and rats that had undergone a sham operation; these rats died immediately. The consistent irrigation and drainage of the abscess was administered and the patient's general condition improved. At this time, we gave the drained material intraperitoneally to other rats with induced pancreatitis and sham operation, but all these rats survived. RESULTS: These experimental results suggested that pancreatitis-associated ascites fluid contained a lethal toxicity. For curing this disease, elimination of these potential toxic mediators was essential. Our intensive IVR-based therapy improved the patient's prognosis. CONCLUSIONS: Downregulating this inflammatory process leads to a decrease in the mortality of severe acute pancreatitis.

MPR-hCT imaging of the pancreatic fluid pathway to Grey-Turner's and Cullen's sign in acute pancreatitis.

Subcutaneous manifestations (Grey Turner's sign and Cullen's sign) of severe acute pancreatitis (SAP) are often discussed but rarely observed in a daily clinic setting. This paper will demonstrate the anatomic pathways followed by the extravasated pancreatic enzymes and how their effects lead to these ecchymoses by multiplanar reformation (MPR) images obtained by helical computed tomography (hCT). A 34-year-old female was admitted with SAP. A hCT scan revealed a swollen pancreas and cholecystolithiasis. The fluid collection around the pancreas extended to the pelvic cavity, infiltrating subcutaneous tissue in the left anterior and lateral abdominal wall. She was treated with interventional endoscopy (IVE) and continuous arterial infusion (CAI) therapy immediately following admission, she survived the SAP and these ecchymoses resolved within 7 days of presentation. MPR images obtained by hCT revealed that, the infiltration of the extra-pancreatic fluid collection between the leaves of the anterior renal fascia through the anterior and posterior pararenal space had reached into a relationship with the subcutaneous tissues in the left flank at the clinical site of discoloration as the pathway of extension to Grey Turner's sign, and also revealed anterior extension from the inflamed gastrohepatic ligament and across the falciform ligament to Cullen's sign.

Exceptional ultra-late recurrence of papilla Vater carcinoma after pancreaticoduodenectomy: two cases at 17 and 25 years.

Curative resection does not always equate with long-term survival. Cancer of the papilla Vater can remain clinically quiescent for decades prior to regional or distant recurrence. Nevertheless, late and ultra-late recurrence (respectively 10 and 15 years after initial treatment) are exceptional events. This protracted disease-free interval challenges the concept of a "cure" for cancer of the papilla Vater. In the first case reported here, a 74-year-old female underwent pancreaticoduodenectomy in 1985 for cancer of the papilla Vater revealed histologically as a well-differentiated papillotubular adenocarcinoma, stage IA (UICC classification). Multiple hepatic recurrences were found 17 years after the operation; hepatic biopsy showed histologically well to moderately differentiated papillotubular adenocarcinoma. She died about 17.5 years after the original operation. The second case is that of an 82-year-old female who underwent pancreaticoduodenectomy in 1974 for cancer of the papilla Vater, histologically a well-differentiated adenocarcinoma, stage IA. In the 25.5 years after the operation, the cancer recurred at the choledochoduodenal anastomosis and involved the liver hilus. Autopsy showed histologically well to moderately differentiated adenocarcinoma at the locations indicated, together with lung and lymph node metastases. The ultra-late recurrences (>15 years) in these cases are highly exceptional and, to our knowledge, this is the first report of such recurrences in cancer of the papilla Vater. It can occur in any patient, with or without identifiable risk factors. Because cancer of the papilla Vater can recur in many prognostically favorable cases after prolonged disease-free intervals, the possibility of delayed recurrence should not be ignored.

Chromosomal variations within aneuploid cancer lines.

Aneuploid cancers exhibit a wide spectrum of clinical aggressiveness, possibly because of varying chromosome compositions. To test this, karyotypes from the diploid CCD-34Lu fibroblast and the aneuploid A549 and SUIT-2 cancer lines underwent fluorescence in situ hybridization (FISH) and DAPI counterstaining. The number of DAPI-stained and FISH-identified chromosomes, 1-22, X,Y, as well as structural abnormalities, were counted and compared using the chi(2), Mann-Whitney rank sum test and the Levene's equality of variance. Virtually all of the evaluable diploid CCD-34Lu karyotypes had 46 chromosomes with two normal-appearing homologues. The aneuploid chromosome numbers per karyotype were highly variable, averaging 62 and 72 for the A549 and SUIT-2 lines, respectively. However, the A549 chromosome numbers were more narrowly distributed than the SUIT-2 karyotype chromosome numbers. Furthermore, 25% of the A549 chromosomes had structural abnormalities compared to only 7% of the SUIT-2 chromosomes. The chromosomal compositions of the aneuploid A549 and SUIT-2 cancer lines are widely divergent, suggesting that diverse genetic alterations, rather than chance, may govern the chromosome makeups of aneuploid cancers.