A Giant Solid-Cystic Gastric Inflammatory Myofibroblastic Tumor: A Case Report and Literature Review.

An inflammatory myofibroblastic tumor (IMT) is a very rare tumor of mesenchymal origin with unclear etiopathogenesis, no unique diagnostic features, and no specific management protocol. It is often confused with inflammatory pseudotumor in literature, and the distinction needs further study. The average size, recurrence risk, and metastatic potential differ as per the site of origin. The abdomen is a very rare site for IMTs. Hepatic IMTs (H-IMTs) are reported to be solid tumors with sizes ranging from 1 cm to 20 cm in literature, and gastric IMTs (G-IMTs) range from 3 cm to 10 cm in size and can be solid-cystic. We report here a case of a 36-year-old gentleman with a 34x27x17 cm solid-cystic lesion in the lesser sac with loss of fat planes with stomach and left hemi-liver. The patient was managed by complete surgical resection of the lesion with wedge gastrectomy and wedge hepatectomy and recovered uneventfully. To our knowledge and based on our literature review, this case presents the largest reported and solid-cystic G-IMT with the involvement of left hemi-liver in a young gentleman and discusses its management as well as the relevant literature on this rare entity. This clinical presentation of G-IMT should be kept in the differential diagnosis in a relevant case presenting in the future. Immunohistochemistry is a must to establish the diagnosis, and surgical resection to negative margins is the management option of choice in resectable cases.

First jejunal vein, jejunal trunk, and pancreatico-duodenectomy: resolving the literature conundrum.

A detailed knowledge of the surgical anatomy of tributaries of the superior mesenteric vein, especially proximal jejunal venous anatomy (first jejunal vein and jejunal trunk), is a key prerequisite for performing a safe pancreatico-duodenectomy. However, the available literature on the anatomical course and surgical relevance of these vessels is scarce, the nomenclature across the articles is heterogeneous, and the resulting evidence is confusing to interpret. Standardized terminology and an in-depth review of these vessels with regard to their course, termination, vascular relations, and variations will help the surgeons in planning and performing this complex surgery safely, especially when a venous resection and reconstruction is planned in cases of borderline resectable pancreatic cancer. A uniform nomenclature and a unifying classification are proposed in this review for these two tributaries to help resolve the literature conundrum. This standardized terminology and anatomical description will assist the radiologists in reporting pancreatic protocol-computed tomography scans and surgeons in selecting the appropriate steps for the different anatomical orientations of these tributaries for the performance of safe pancreatic surgery. This will also help future researchers communicate in well-defined terms in reference to these tributaries so as to avoid confusion in future studies.

Anatomical Challenges during Pancreaticoduodenectomy for Adenocarcinoma Head of Pancreas in Presence of Intestinal Rotation Abnormalities: A Report of Two Cases.

Purpose Pancreaticoduodenenctomy is a complex surgery and the sequence of steps is affected by anatomical variations involving small intestine and major vascular structures. This article depicts our approach to two such cases and highlights the importance of identifying these variations preoperatively on imaging, so as to modify the surgery plan accordingly. Cases We report following two cases of pancreatic head adenocarcinoma (1) one with incomplete intestinal rotation with a replaced right hepatic artery and (2) one with intestinal nonrotation. In both cases, the small bowel was aggregated on the right side of the abdomen, making duodenal mobilization challenging. The surgical approach was modified to prevent injury to these vessels. A superior mesenteric artery (SMA)-first approach helped in early isolation of vascular structures especially when vascular anomaly was also present. Interbowel adhesiolysis, limited kocherisation, tracing all vessels to its origin before division, paracolic anastomotic limb after a longer jejunal limb resection in nonrotation cases, and modification in retropancreatic tunnel creation are few of the key surgical adaptations. Conclusion Asymptomatic Intestinal malrotation is rare in adults and must be identified on preoperative imaging. Resultant intestinal and vascular anatomical variations need meticulous surgical planning and modification of conventional surgical approach for safe performance of PD.

Pyrexia of Unknown Origin: An Atypical Presentation of Hepatic Hemangioma.

Pyrexia of unknown origin (PUO) has been a diagnostic challenge for decades. Hepatic hemangioma (HH) is not a common differential diagnosis of PUO. It is the most common benign hepatic tumor, commonly asymptomatic and incidentally detected, or can present with vague abdominal pain. PUO is a rare presenting feature. We describe a case of 38-year-old lady presenting with PUO. With no other identifiable source of fever despite exhaustive investigations, a giant hemangioma in right lobe of liver detected on abdominal ultrasonography was deemed to be the cause of PUO. The patient had sudden decrease in hemoglobin while undergoing workup, which on imaging showed a bleeding hemangioma and right hepatectomy was performed. Patient had an uneventful recovery and her PUO also resolved after surgery. HH should be considered a rare diagnosis of exclusion for PUO after a standard algorithmic approach does not reveal any other cause.

SURGICAL MANAGEMENT OF LARGE HEPATOCELLULAR CARCINOMA: THE FIRST SINGLE-CENTER STUDY FROM WESTERN INDIA.

BACKGROUND: Majority of patients with large size HCC (>10 cm) are not offered surgery as per Barcelona Clinic Liver Cancer (BCLC) criteria and hence, their outcomes are not well studied, especially from India, owing to a lower incidence. AIM: To analyze outcomes of surgery for large HCCs. METHODS: This retrospective observational study included all patients who underwent surgery for large HCC from January 2007 to December 2017. The entire perioperative and follow up data was collected and analyzed. RESULTS: Nineteen patients were included. Ten were non-cirrhotic; 16 were BCLC grade A; one BCLC grade B; and two were BCLC C. Two cirrhotic and three non-cirrhotic underwent preoperative sequential trans-arterial chemoembolization and portal vein embolization. Right hepatectomy was the most commonly done procedure. The postoperative 30-day mortality rate was 5% (1/19). Wound infection and postoperative ascites was seen in seven patients each. Postoperative liver failure was seen in five. Two cirrhotic and two non-cirrhotic patients had postoperative bile leak. The hospital stay was 11.9±5.4 days (median 12 days). Vascular invasion was present in four cirrhotic and five non-cirrhotic patients. The median follow-up was 32 months. Five patients died in the follow-up period. Seven had recurrence and median recurrence free survival was 18 months. The cumulative recurrence free survival was 88% and 54%, whereas the cumulative overall survival was 94% and 73% at one and three years respectively. Both were better in non-cirrhotic; however, the difference was not statistically significant. The recurrence free survival was better in patients without vascular invasion and the difference was statistically significant (p=0.011). CONCLUSION: Large HCC is not a contraindication for surgery. Vascular invasion if present, adversely affects survival. Proper case selection can provide the most favorable survival with minimal morbidity.

Multimodality Management of Ruptured Large Hepatocellular Carcinoma and Its Recurrence: Rupture at Presentation Should Not Rupture Hope of Long-Term Survival.

A 59-year-old gentleman with a history of aortic valve replacement presented with spontaneously ruptured hepatocellular carcinoma in right lobe of a hepatitis C virus (HCV)-related chronic liver disease with hemoperitoneum. This acute emergency was managed by transarterial embolization. Right trisectionectomy with preservation of segment IVB after augmentation of future liver remnant by transarterial chemoembolization followed by portal vein embolization was subsequently performed. Sustained virological response to HCV was attained after surgery using sofosbuvir-based regimen. He had a delayed operative bed recurrence 1.5 years later with pulmonary metastatic disease which was managed by operative bed metastasectomy with mesh reconstruction of diaphragm and sorafenib. He is on sorafenib since past 3 years and doing well at 4.5-years follow-up since the first presentation, with significant regression of pulmonary disease and no other disease elsewhere, which highlights that where there is hope, there is a way.

Surgical management of large hepatocellular carcinoma: the first single-center study from western india / Manejo cirúrgico de grandes carcinomas hepatocelulares: primeiro estudo de um único centro da índia ocidental

ABSTRACT Background: Majority of patients with large size HCC (>10 cm) are not offered surgery as per Barcelona Clinic Liver Cancer (BCLC) criteria and hence, their outcomes are not well studied, especially from India, owing to a lower incidence. Aim: To analyze outcomes of surgery for large HCCs. Methods: This retrospective observational study included all patients who underwent surgery for large HCC from January 2007 to December 2017. The entire perioperative and follow up data was collected and analyzed. Results: Nineteen patients were included. Ten were non-cirrhotic; 16 were BCLC grade A; one BCLC grade B; and two were BCLC C. Two cirrhotic and three non-cirrhotic underwent preoperative sequential trans-arterial chemoembolization and portal vein embolization. Right hepatectomy was the most commonly done procedure. The postoperative 30-day mortality rate was 5% (1/19). Wound infection and postoperative ascites was seen in seven patients each. Postoperative liver failure was seen in five. Two cirrhotic and two non-cirrhotic patients had postoperative bile leak. The hospital stay was 11.9±5.4 days (median 12 days). Vascular invasion was present in four cirrhotic and five non-cirrhotic patients. The median follow-up was 32 months. Five patients died in the follow-up period. Seven had recurrence and median recurrence free survival was 18 months. The cumulative recurrence free survival was 88% and 54%, whereas the cumulative overall survival was 94% and 73% at one and three years respectively. Both were better in non-cirrhotic; however, the difference was not statistically significant. The recurrence free survival was better in patients without vascular invasion and the difference was statistically significant (p=0.011). Conclusion: Large HCC is not a contraindication for surgery. Vascular invasion if present, adversely affects survival. Proper case selection can provide the most favorable survival with minimal morbidity.

RESUMO Racional: A maioria dos pacientes com CHC de grande porte (>10 cm) não tem indicação cirúrgica conforme os critérios do Barcelona Clinic Liver Cancer (BCLC) e, portanto, seus resultados não são bem estudados, principalmente na Índia, devido a uma menor incidência. Objetivo: Analisar os resultados da cirurgia para HCCs de grande porte. Métodos: Este estudo observacional retrospectivo incluiu todos os pacientes submetidos à cirurgia para grandes CHC de janeiro de 2007 a dezembro de 2017. Todos os dados perioperatórios e de acompanhamento foram coletados e analisados. Resultados: Dezenove pacientes foram incluídos. Dez não eram cirróticos; 16 eram BCLC grau A; um BCLC grau B; e dois eram BCLC C. Dois cirróticos e três não-cirróticos foram submetidos à quimioembolização transarterial sequencial pré-operatória e embolização da veia porta. Hepatectomia direita foi o procedimento mais comumente realizado. A taxa de mortalidade pós-operatória em 30 dias foi de 5% (1/19). Infecção da ferida e ascite pós-operatória foram observadas em sete pacientes cada. Insuficiência hepática pós-operatória foi observada em cinco. Dois pacientes cirróticos e dois não cirróticos apresentaram vazamento de bile no pós-operatório. O tempo de internação foi de 11,9±5,4 dias (mediana de 12 dias). A invasão vascular estava presente em quatro pacientes cirróticos e cinco não cirróticos. O acompanhamento médio foi de 32 meses. Cinco pacientes morreram no período de acompanhamento. Sete tiveram recorrência e sobrevida mediana livre de recorrência foi de 18 meses. A sobrevida livre de recorrência cumulativa foi de 88% e 54%, enquanto a sobrevida global cumulativa foi de 94% e 73% em um e três anos, respectivamente. Ambos eram melhores em não-cirróticos; no entanto, a diferença não foi estatisticamente significante. A sobrevida livre de recidiva foi melhor nos pacientes sem invasão vascular e a diferença foi estatisticamente significante (p=0,011). Conclusão: CHC grande não é contraindicação para cirurgia. Invasão vascular, se presente, afeta adversamente a sobrevida. Seleção adequada de casos pode fornecer sobrevida mais favorável com morbidade mínima.

Late postcholecystectomy Mirizzi syndrome due to a sessile gall bladder remnant calculus managed by laparoscopic completion cholecystectomy: a feasible surgical option.

Postcholecystectomy Mirizzi syndrome (PCMS) is an uncommon entity that can occur due to cystic duct stump calculus, gall bladder remnant calculus or migrated surgical clip. It can be classified into early PCMS or late PCMS. It is often misdiagnosed and the management depends on the site of impaction of stone or clip. Endoscopy can be performed for cystic duct stump calculus. However, surgery is the treatment for remnant gall bladder calculus. Role of laparoscopic management is controversial. We present here a case of a 48-year-old woman with late PCMS due to an impacted calculus in a sessile gall bladder remnant following a subtotal cholecystectomy, managed with laparoscopic completion cholecystectomy, review the literature, provide tips for safe laparoscopy for PCMS and summarise our algorithmic approach to the management of the postcholecystectomy syndrome.

Intrapancreatic accessory spleen: an enigmatic entity.

Solitary hypervascular lesion in the distal body/tail of pancreas in a patient with non-specific abdominal symptoms is a diagnostic challenge. Neuroendocrine neoplasm (NEN) and metastasis from renal cell carcinoma are the most common differentials and intrapancreatic accessory spleen (IPAS) is the rarest of its differential diagnosis. We present, here, a case of a 56-year-old man with a space-occupying lesion in body/tail of pancreas that was preoperatively diagnosed as a NEN based on elevated chromogranin levels and hyperenhancing lesion on contrast-enhanced CT scan. He underwent a spleen-preserving distal pancreatectomy. The final histopathology revealed an IPAS.

Diagnosis and Management of Pancreatic Adenocarcinoma in the Background of Chronic Pancreatitis: Core Issues.

BACKGROUND: The incidence of pancreatic adenocarcinoma (PDAC) in patients with chronic pancreatitis (CP) is as high as 5%. It is a commonly encountered diagnostic challenge in patients with CP on long-term follow-up. SUMMARY: This review consolidates the existing literature on assessment of PDAC in background of CP, its evaluation through the available investigations, surgical management, and prognostication. Recent change in symptomatology of an otherwise stable CP should raise a suspicion of malignancy. Endoscopic ultrasound (EUS) is more specific and sensitive in establishing the diagnosis of PDAC compared to cross-sectional imaging (computed tomography/magnetic resonance imaging). Intraoperative assessment with careful palpation coupled with careful clinical judgment helps in differentiating between an inflammatory mass and pancreatic cancer. Confirmation can be obtained with either preoperative EUS-guided fine needle cytology/core biopsy or intraoperative core biopsy under ultrasound guidance. However, despite complete evaluation with above options, 1-6% patients often show malignancy on final histopathological examination. Key Messages: Diagnosis of PDAC in CP needs a high index of suspicion. Cross-sectional imaging has poor negative predictive value. CA 19-9 with MUC5AC combination may become an ideal investigation. EUS with core biopsy/frozen section has a good sensitivity and specificity and low false negative results. Complete radical resection should be the aim to get long-term survival.

An outcome analysis of videoscopic assisted retroperitoneal debridement in infected pancreatic necrosis: a single centre experience.

OBJECTIVES: Infected pancreatic necrosis (IPN) is a dreadful complication of moderately severe and severe acute necrotising pancreatitis (ANP). Videoscopic assisted retroperitoneal debridement (VARD) is a minimally invasive surgical option for predominantly left sided, posterior and laterally located disease in patients not responding to conservative and percutaneous options. This study aimed to present an outcome analysis of VARD in the management of IPN at our tertiary care centre. MATERIAL AND METHODS: The present retrospective analysis of prospectively entered data included 22 patients diagnosed as ANP with IPN from January 2015 to December 2017. These patients were admitted in the surgical gastroenterology unit of our tertiary care centre. The outcome of these patients managed with VARD was evaluated. RESULTS: The aetiology of ANP was idiopathic, and gallstones were found in 7 patients each and alcohol in 8. Twelve patients were managed with a single VARD procedure; whereas, 10 required a re-debridement due to suboptimal improvement. Eighteen out of 22 patients survived whereas 4 succumbed to major postoperative bleeding/severe sepsis and multiorgan failure (Mortality 18.2%). Hospital stay after the index procedure was between 6 to 11 weeks. CONCLUSION: VARD is a safe and effective surgical option for the management of IPN that worsens or fails to respond to conservative and percutaneous drainage options after a minimum of 4 weeks of moderately severe and severe ANP. It decreases postoperative morbidity and mortality and avoids major laparotomy, and hence, it can be considered in a selected group of patients.

Autoimmune Pancreatitis Type II: First Report from India.

Autoimmune Pancreatitis (AIP) presents in two forms - Type I or lymphoplasmacytic sclerosing pancreatitis and Type II or idiopathic ducto-centric pancreatitis (IDCP). AIP II is rare in south Asia and, especially so, in India. Most patients have either Idiopathic Chronic Pancreatitis (ICP) or alcohol related chronic pancreatitis. AIP Type I has been described in India. We herein report a patient who had features of ICP on imaging, for whom surgery was performed to relieve chronic pancreatic pain. However, the pathologic features revealed AIP Type II or IDCP.

Is Revision Surgery Justified for Symptomatic Pancreatico-enteric Anastomotic Stenosis in Long-term Survivors Following Pancreaticoduodenectomy for Malignancy?

BACKGROUND: Pancreatico-enteric anastomotic (PEA) stenosis is one of the late complications following pancreaticoduodenectomy (PD) and reported for benign diseases. Literature for PEA stenosis following PD for malignancy is very limited due to low survival. MATERIAL AND METHODS: Patients undergoing surgery for symptomatic, recurrent, obstructive pancreatitis due to PEA stenosis following PD for malignancy were retrospectively identified from the authors' prospective database between January 1997 and December 2014. RESULTS: Six patients with median age 56.5 years underwent revision surgery for PEA stenosis during this time period. At primary PD, all were node negative with T1/T2 disease. The primary PEA were pancreatico-jejunostomy (PJ) (n = 5) and pancreatico-gastrostomy (n = 1). Median time to develop symptoms was 62 months. At revision surgery, a Roux-en-Y longitudinal PJ (n = 5) and an end-to-side PJ (n = 1) were done. With a median follow-up of 36 months, pain relief was excellent (n = 5) to average (n = 1). CONCLUSION: With improving long-term survival in patients undergoing PD for malignancy more such patients will be identified in future. Patients with symptomatic PEA stenosis following PD for malignancy can be managed surgically, with excellent outcomes in centers of expertise in pancreatic surgery.

Brunneroma: A Rare Cause of Duodeno-duodenal Intussusception.

Brunner gland hamartoma (brunneroma) is a rare benign tumor of the duodenum. It is usually asymptomatic and detected incidentally by endoscopy or other imaging modality. The definitive diagnosis is based on histopathological findings. These may mimic tumors of other natures, such as gastrointestinal stromal tumors (GIST), carcinoids, lipomas, and leiomyomas. Here, we present a case of duodenal polyp presenting with abdominal pain and obstructive symptoms that caused duodenal intussusception. It was surgically removed and found to be a brunneroma on histopathology. HOW TO CITE THIS ARTICLE: Patankar AM, Wadhwa AM, Bajaj A, Ingule A, Wagle P. Brunneroma: A Rare Cause of Duodeno-duodenal Intussusception. Euroasian J Hepato-Gastroenterol 2016;6(1):84-88.

Real-time ultrasound: Key factor in identifying celiac artery compression syndrome.

The median arcuate ligament syndrome (MALS) or celiac artery compression syndrome (CACS) is a rare entity, presenting clinically with postprandial abdominal pain and weight loss. The diagnosis is made on computed tomography (CT) angiography, which reveals extrinsic compression of the proximal part of the celiac artery by the median arcuate ligament, producing a characteristic hooked appearance. We report a case of the celiac artery compression syndrome, diagnosed by Doppler USG evaluation.

Synchronous hepatocellular carcinoma with renal cell carcinoma: a case report and review of literature of multiple synchronous primary malignancies.

Multiple synchronous primary malignancies have been reported since the 19th century. A number of proposed theories as to the predisposing factors have been discussed. The criteria to diagnose multiple primary malignancies have been revised by Warren and Gates. We hereby present a case of an asymptomatic individual detected with a synchronous hepatocellular carcinoma and a renal cell carcinoma, its presentation, diagnosis, and the management. The occurrence of synchronous hepatocellular carcinoma with renal cell carcinoma is very rare and only a few cases have been reported. Synchronous extrahepatic primary malignancies have been reported in a few studies across the world though with a varied incidence rate. The occurrence seems to be in the older age group without gender differentiation. The extrahepatic malignancies were more common in cirrhotic livers though the overall survival does not differ between patients with hepatocellular carcinoma alone and hepatocellular carcinoma with synchronous extrahepatic malignancies.