Surgical complications and technical failure of simultaneous pancreas-kidney transplantation: A 22-year experience from a single center.

Simultaneous pancreas-kidney transplantation (SPKT) is the best treatment for selected individuals with type 1 diabetes mellitus and end-stage renal disease. Despite advances in surgical techniques, donor and recipient selection, and immunosuppressive therapies, SPKT remains a complex procedure with associated surgical complications and adverse consequences. We conducted a retrospective study that included 263 SPKT procedures performed between May 2000, and December 2022. A total of 65 patients (25%) required at least one relaparotomy, resulting in an all-cause relaparotomy rate of 2.04 events per 100 in-hospital days. Lower donor body mass index was identified as an independent factor associated with reoperation (OR .815; 95% CI:  .725-.917, p = .001). Technical failure (TF) occurred in 9.9% of cases, primarily attributed to pancreas graft thrombosis, intra-abdominal infections, bleeding, and anastomotic leaks. Independent predictors of TF at 90 days included donor age above 36 years (HR 2.513; 95% CI 1.162-5.434), previous peritoneal dialysis (HR 2.503; 95% CI 1.149-5.451), and specific pancreas graft reinterventions. The findings highlight the importance of carefully considering donor and recipient factors in SPKT. The incidence of TF in our study population aligns with the recent series. Continuous efforts should focus on identifying and mitigating potential risk factors to enhance SPKT outcomes, thereby reducing post-transplant complications.

Intraductal papillary neoplasms of the bile ducts: a comparative study of a rare disease in Europe and Nagoya, Japan.

BACKGROUND: Intraductal papillary neoplasm of the bile ducts (IPNB) is a rare disease in Western countries. The aim of this study was to compare tumor characteristics, management strategies, and outcomes between Western and Eastern patients who underwent surgical resection for IPNB. METHODS: A multi-institutional retrospective series of patients with IPNB undergoing surgery between January 2010 and December 2020 was gathered under the auspices of the European-African Hepato-Pancreato-Biliary Association (E-AHPBA), and at Nagoya University Hospital, Japan. RESULTS: A total of 85 patients (51% male; median age 66 years) from 28 E-AHPBA centers were compared to 91 patients (64% male; median age 71 years) from Nagoya. Patients in Europe had more multiple lesions (23% vs 2%, P < .001), less invasive carcinoma (42% vs 85%, P < .001), and more intrahepatic tumors (52% vs 24%, P < .001) than in Nagoya. Patients in Europe experienced less 90-day grade >3 Clavien-Dindo complications (33% vs 68%, P < .001), but higher 90-day mortality rate (7.0% vs 0%, P = .03). R0 resections (81% vs 82%) were similar. Overall survival, excluding 90-day postoperative deaths, was similar in both regions. DISCUSSION: Despite performing more extensive resections, the low perioperative mortality rate observed in Nagoya was probably influenced by a combination of patient-, tumor-, and surgery-related factors.

Hepatic angiomyolipoma, misdiagnosed as hepatocellular carcinoma.

Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumor, and hepatic PEComa is even rarer. Its preoperative diagnosis is difficult, given the absence of specific clinical manifestations, often constituting an accidental finding, and the lack of a gold standard for identification using imaging studies. Instead, the diagnosis of hepatic PEComa is based on morphological and immunohistochemical features. We describe a case of an asymptomatic hepatic PEComa, angiomyolipoma type, which appeared in a middle-aged woman with chronic liver disease, during her follow-up and screening. Given the patient's context, human immunodeficiency virus-positive with chronic hepatitis C, and the similarities between the two tumors, the hepatic lesion was interpreted as hepatocellular carcinoma. The patient underwent surgical excision of the tumor, and the positive immunohistochemical staining for human melanoma black 45 and Melan A made the definitive diagnosis. In the absence of aggressiveness tumor markers, surveillance was decided. We also provide a literature review of these tumors.

Intraductal papillary neoplasms of the bile duct: a European retrospective multicenter observational study (EUR-IPNB study).

BACKGROUND/PURPOSE: Intraductal papillary neoplasm of the bile duct (IPNB) is a rare disease in Western countries. The main aim of this study was to characterize current surgical strategies and outcomes in the mainly European participating centers. METHODS: A multi-institutional retrospective series of patients with a diagnosis of IPNB undergoing surgery between 1 January 2010 and 31 December 2020 was gathered under the auspices of the European-African Hepato-Pancreato-Biliary Association. The textbook outcome (TO) was defined as a non-prolonged length of hospital stay plus the absence of any Clavien-Dindo grade at least III complications, readmission, or mortality within 90 postoperative days. RESULTS: A total of 28 centers contributed 85 patients who underwent surgery for IPNB. The median age was 66 years (55-72), 49.4% were women, and 87.1% were Caucasian. Open surgery was performed in 72 patients (84.7%) and laparoscopic in 13 (15.3%). TO was achieved in 54.1% of patients, reaching 63.8% after liver resection and 32.0% after pancreas resection. Median overall survival was 5.72 years, with 5-year overall survival of 63% (95% CI: 50-82). Overall survival was better in patients with Charlson comorbidity score 4 or less versus more than 4 ( P =0.016), intrahepatic versus extrahepatic tumor ( P =0.027), single versus multiple tumors ( P =0.007), those who underwent hepatic versus pancreatic resection ( P =0.017), or achieved versus failed TO ( P =0.029). Multivariable Cox regression analysis showed that not achieving TO (HR: 4.20; 95% CI: 1.11-15.94; P =0.03) was an independent prognostic factor of poor overall survival. CONCLUSIONS: Patients undergoing liver resection for IPNB were more likely to achieve a TO outcome than those requiring a pancreatic resection. Comorbidity, tumor location, and tumor multiplicity influenced overall survival. TO was an independent prognostic factor of overall survival.

Primary retroperitoneal PEComa: an incidental finding.

Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell 'sugar' tumours of the lung are well described, relatively little is known about other members of the PEComa family. We describe a case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, which appeared in a previously healthy middle-aged woman as an incidental finding, in a CT scan performed in the context of spontaneous pneumothorax. The patient underwent surgical excision of the tumour and the histopathological and immunohistochemical analysis of the surgical specimen made the definitive diagnosis. Although rare, reports of isolated retroperitoneal lymphangioleiomyoma and primary retroperitoneal PEComas NOS (not otherwise specified) are described in the literature, normally associated with pulmonary LAM. The patient's pulmonary imaging was normal. Short-term re-examination did not detect any recurrence. We also provide a literature review of this rare group of tumours.

Ciliated hepatic foregut cyst: A case report and review of literature.

INTRODUCTION: Ciliated hepatic foregut cyst (CHFC) is a rare cystic lesion that arises from the embryonic foregut with approximately 100 cases reported. Most commonly identified in segment IV of the liver, CHFC is typically asymptomatic and incidentally found on abdominal imaging. It is important to consider this entity in the differential diagnosis of atypical liver lesions since CHFC carries a risk of transformation into squamous cell carcinoma. A suspicion of CHFC is therefore an indication for surgical resection. CASE PRESENTATION: A 62-year-old male presented to surgery consultation for further evaluation of a hepatic cyst incidentally found on abdominal ultrasound. The patient was completely asymptomatic. Both abdominal computerized tomography and magnetic resonance imaging scan confirmed a 4 cm subcapsular cyst in segment IVa. Additional workup was unremarkable. Considering the diagnostic doubt the patient underwent laparoscopic cyst enucleation. Histology revealed a ciliated pseudostratified epithelium consistent with a CHFC. CLINICAL DISCUSSION: CHFC is a rare diagnostic entity that should be considered in the differential diagnosis of cystic hepatic lesions, particularly those located in segment IV of the liver. Since it is frequently asymptomatic, CHFC is usually found incidentally during surgery or imaging studies. Diagnosis of CHFC preoperatively is difficult due to the lack of specific radiographic findings. Moreover, metaplasia and squamous carcinoma can occur. Therefore complete surgical excision is the recommended treatment. CONCLUSION: Despite its rarity, CHFC carries a risk of malignant transformation. Accurate diagnosis is mandatory and surgical excision is recommended even in asymptomatic CHFC.

Perianal Paget Disease: Different Entities With the Same Name.

Extramammary Paget disease (EMPD) is a rare form of cancer that involves skin areas rich in apocrine glands. The common sites of occurrence include the vulva, perianal region, perineum, and axilla. Perianal Paget disease (PPD) is a subset of EMPD, which arises in the perianal skin. The disease commonly presents with a thickened plaque-like lesion with erythema or white scaly appearance. It is generally classified into two categories based on the origin of the tumor cells: (1) primary PPD if the tumor arises from intraepidermal cells and (2) secondary PPD if cancer originates from the underlying colorectal or urinary tract neoplasm. Due to its rarity, only a few sporadic case reports have been published in the literature, and treatment methods are yet to be standardized. In light of this, we report two PPD cases with different etiopathogenesis and staging: one involved only the perianal skin without regional or metastatic disease, and was not accompanied by visceral adenocarcinomas although there was a previous history of sigmoid adenocarcinoma; the other was probably secondary to an anal canal tumor spreading with disseminated disease involving the perianal and perineum area with bilateral inguinal, pelvic lymph node, and liver metastasis. The treatment plans and the outcomes of both cases were necessarily different from each other.

Hydatid Cyst Diagnosed by Endoscopic Ultrasound.

A 69-year-old female with unremarkable past history underwent endoscopy for dyspepsia. She denied weight loss or anorexia. Upper endoscopy revealed a bulge in the lesser curvature and posterior wall of the stomach with 4-5 cm. Endoscopic ultrasound was performed which showed a heterogeneous lesion, anechogenic in the major part, with a floating membrane inside, the greatest diameter of 90.8 × 17.2 mm, originated in the left liver lobe. Surgical resection was performed. Pathologic examination revealed a cystic lesion with an acellular thick fibrous wall, surrounded by a conspicuous inflammatory reaction. The cyst wall revealed a characteristic lamellar pattern of the fibers. In the internal surface of the lesion, there were remains of membranous structures, amidst which a vestigial Protoscolex was noted. In the presented case, a floating membrane was observed, which is a pathognomonic feature, establishing the diagnosis of hydatid cyst type 3. Fine needle aspiration guided by ultrasound was not performed due to the certainty in the diagnosis. To the authors' knowledge, these are the first images by endoscopic ultrasound of hydatid cyst of liver presented as a bulge in the stomach with pathognomonic features, which allowed the definitive diagnosis with no need for further diagnostic tests.

Advanced Glycation End Products Evolution after Pancreas-Kidney Transplantation: Plasmatic and Cutaneous Assessments.

Diabetes mellitus leads to increased Advanced Glycation End Products (AGE) production, which has been associated with secondary diabetic complications. Type 1 diabetic patients undergoing pancreas-kidney transplantation (SPKT) can restore normoglycemia and renal function, eventually decreasing AGE accumulation. We aimed to prospectively study AGE evolution after SPKT. Circulating AGE were assessed in 20 patients, at time 0 (T0), 3 months (T3), 6 months (T6), and 12 months (T12) after successful SPKT. Global AGE and carboxymethyllysine (CML) were analyzed, as well as advanced oxidation protein products (AOPP). Skin biopsies were obtained at T0 and T12. Immunohistochemistry with anti-AGE antibody evaluated skin AGE deposition. AGE mean values were 16.8 ± 6.4 µg/mL at T0; 17.1 ± 3.8 µg/mL at T3; 17.5 ± 5.6 µg/mL at T6; and 16.0 ± 5.2 µg/mL at T12. CML mean values were 0.94 ± 0.36 ng/mL at T0; 1.11 ± 0.48 ng/mL at T3; 0.99 ± 0.42 ng/mL at T6; and 0.78 ± 0.38 ng/mL at T12. AOPP mean values were 130.1 ± 76.8 µMol/L at T0; 137.3 ± 110.6 µMol/L at T3; 116.4 ± 51.2 µMol/L at T6; and 106.4 ± 57.9 µMol/L at T12. CML variation was significant (P = 0.022); AOPP variation was nearly significant (P = 0.076). Skin biopsies evolved mostly from a cytoplasmic diffuse to a peripheral interkeratinocytic immunoreaction pattern; in 7 cases, a reduction in AGE immunoreaction intensity was evident at T12. In conclusion, glycoxidation markers decrease, plasmatic and on tissues, may start early after SPKT. Studies with prolonged follow-up may confirm these data.

Perforated duodenal diverticulum: Surgical treatment and literature review.

INTRODUCTION: Duodenum is the second most frequent location for a diverticulum in the digestive tract. Complications are rare and perforation was only reported in less than 200 cases. PRESENTATION OF CASE: A 79-year-old female was admitted to Emergency Department with abdominal pain and vomiting for the last 24h. A CT scan was performed and moderated extra-luminal air was identified. During surgery a fourth portion perforated duodenal diverticulum was diagnosed and duodenal resection was performed. DISCUSSION: First reported in 1710, the incidence of duodenal diverticula can be as high as 22%. Nevertheless complications are extremely rare and include haemorrhage, inflammation, compression of surrounding organs, neoplastic progression, cholestasis and perforation. As perforations are often retroperitoneal, symptoms are nonspecific and rarely include peritoneal irritation, making clinical diagnose a challenge. CT scan will usually present extra-luminal retroperitoneal air and mesenteric fat stranding, providing clues for the diagnosis. Although non-operative treatment has been reported in selected patients, standard treatment is surgery and alternatives are diverse including diverticulectomy or duodenopancreatectomy. CONCLUSION: Perforated diverticula of the fourth portion of the duodenum are extremely rare and current evidence still supports surgery as the primary treatment modality.