Multimodal Treatment of Metastatic Rectal Cancer in a Young Patient: Case Report and Literature Review.

Metastatic colorectal cancer requires a multidisciplinary and individualized approach. Herein, we reported the case of a young woman diagnosed with metastatic rectal cancer who received an individualized multimodal treatment strategy that resulted in a remarkable survival. There were several particular aspects of this case, such as the early onset of the disease, the successful use of conversion therapy, the application of liquid biopsy to guide treatment, and the specific nature of the bone metastasis. To offer more insights for navigating such challenges in patients with metastatic colorectal cancer, we have conducted a literature review to find more data related to the particularities of this case. The incidence of early onset colorectal cancer is on the rise. Data suggests that it differs from older-onset colorectal cancer in terms of its pathological, epidemiological, anatomical, metabolic, and biological characteristics. Conversion therapy and surgical intervention provide an opportunity for cure and improve outcomes in metastatic colorectal cancer. It is important to approach each case individually, as every patient with limited liver disease should be considered as a candidate for secondary resection. Moreover, liquid biopsy has an important role in the individualized management of metastatic colorectal cancer patients, as it offers additional information for treatment decisions.

Pancreatic Adenocarcinoma: Imaging Modalities and the Role of Artificial Intelligence in Analyzing CT and MRI Images.

Pancreatic ductal adenocarcinoma (PDAC) stands out as the predominant malignant neoplasm affecting the pancreas, characterized by a poor prognosis, in most cases patients being diagnosed in a nonresectable stage. Image-based artificial intelligence (AI) models implemented in tumor detection, segmentation, and classification could improve diagnosis with better treatment options and increased survival. This review included papers published in the last five years and describes the current trends in AI algorithms used in PDAC. We analyzed the applications of AI in the detection of PDAC, segmentation of the lesion, and classification algorithms used in differential diagnosis, prognosis, and histopathological and genomic prediction. The results show a lack of multi-institutional collaboration and stresses the need for bigger datasets in order for AI models to be implemented in a clinically relevant manner.

Liver Transplant in Patients with Hepatocarcinoma: Imaging Guidelines and Future Perspectives Using Artificial Intelligence.

Hepatocellular carcinoma is the most common primary malignant hepatic tumor and occurs most often in the setting of chronic liver disease. Liver transplantation is a curative treatment option and is an ideal solution because it solves the chronic underlying liver disorder while removing the malignant lesion. However, due to organ shortages, this treatment can only be applied to carefully selected patients according to clinical guidelines. Artificial intelligence is an emerging technology with multiple applications in medicine with a predilection for domains that work with medical imaging, like radiology. With the help of these technologies, laborious tasks can be automated, and new lesion imaging criteria can be developed based on pixel-level analysis. Our objectives are to review the developing AI applications that could be implemented to better stratify liver transplant candidates. The papers analysed applied AI for liver segmentation, evaluation of steatosis, sarcopenia assessment, lesion detection, segmentation, and characterization. A liver transplant is an optimal treatment for patients with hepatocellular carcinoma in the setting of chronic liver disease. Furthermore, AI could provide solutions for improving the management of liver transplant candidates to improve survival.

Case report: High-risk acute promyelocytic leukemia and COVID-19-related myocarditis one patient, two cytokine storms.

Acute promyelocytic leukemia (APL) is a unique, highly curable subtype of acute myeloid leukemia, owing to the therapeutic advances of the last decades which led to high complete remission rates and excellent long-term survival. Nevertheless, it remains associated with high early mortality rates. Early death is the major cause of treatment failure in APL and is mainly attributed to coagulopathy, differentiation syndrome, and less commonly, infectious events. Timely recognition of each complication plays a crucial role in the management of patients diagnosed with APL. Coronavirus Infectious Disease 2019 (COVID-19) has shown great heterogeneity in patient presentation. Clinical manifestations range from asymptomatic disease to severe forms, mainly characterized by a hyperinflammatory syndrome leading to acute respiratory distress and multiorgan failure. Patients with acute leukemia and concomitant COVID-19-related hyperinflammatory syndrome have particularly poor outcomes. We hereby report the case of a 28-year-old male patient who was diagnosed with high-risk APL, with severe associated coagulopathy at presentation. He was treated with chemotherapy according to the AIDA regimen. The first week of induction therapy was complicated by a differentiation syndrome manifesting as fever not attributable to infection and respiratory distress with pulmonary infiltrates, resolved after ATRA discontinuation and corticotherapy. On the fourth week of treatment, he tested positive for acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with minor pulmonary involvement. Clinical manifestations over the following days included tachycardia and hypotension, associated with elevated inflammatory markers and cardiac biomarkers (troponin I x58 upper NV). Cardiovascular magnetic resonance imaging was consistent with myocarditis. COVID-19-associated myocarditis was successfully treated with methylprednisolone, intravenous immunoglobulins and Anakinra. Differentiation syndrome and COVID-19-associated myocarditis are two life-threatening complications that adversely impact survival. However, early recognition and prompt treatment initiation can improve clinical outcomes, as was the case of our patient.

Parenchyma Sparing ALPPS - Ultrasound Guided Partition Through Segment 4 to Maximize Resectability (with video).

Background: Associating liver partition and portal vein ligation (ALPPS) has evolved as a treatment strategy for patients with liver tumors who are not amenable for upfront hepatectomy because of an insufficient future liver remnant (FLR). Aim of this study was to test the applicability of ultrasound guided parenchyma sparing surgery to ALPPS concept, by non-anatomically shifting the plane of transection in favor of FLR, resulting in a new technical variant of ALPPS, entitled parenchyma sparing ALPPS (psALPPS). Materials and Methods: Patients who could not safely undergo right trisectionectomy ALPPS because of insufficient FLR were considered eligible for psALPPS, consisting in liver partition through segment 4 using ultrasound guidance. Results: Between April 2017 and April 2021, five patients with median age of 68 years (range: 66-78), four male and one female, underwent psALPPS for colorectal liver metastases (N=2), intrahepatic cholangiocarcinoma (N=2), and hepatocellular carcinoma (N=1). Standardized FLR (sFLR) for segments 2-3 before stage 1 surgery would have been a median of 11.6%. PsALPPS could double the sFLR at stage 1 resulting in an increase of ps-sFLR from a median of 22.7% (at stage 1) to 34.0% (at stage 2) after a median interstage interval of 15 days. All patients tolerated surgery well and no major complications were recorded. Conclusions: Applying the principles of parenchyma sparing surgery to ALPPS offers the advantage to maximize FLR and simultaneously reduce ischemic injury of segment 4 compared to conventional ALPPS. In this way, psALPPS may markedly increase resectability while reducing morbidity. Video version: https://www.revistachirurgia.ro/pdfs/?EntryID=922974&art=2021-parenchyma-sparing-ALPPS-ultrasound-guided-partition.pdf

Parenchyma Sparing ALPPS Ultrasound Guided Partition Through Segment 4 to Maximize Resectability (with video).

Background: Associating liver partition and portal vein ligation (ALPPS) has evolved as a treatment strategy for patients with liver tumors who are not amenable for upfront hepatectomy because of an insufficient future liver remnant (FLR). Aim of this study was to test the applicability of ultrasound guided parenchyma sparing surgery to ALPPS concept, by non-anatomically shifting the plane of transection in favor of FLR, resulting in a new technical variant of ALPPS, entitled parenchyma sparing ALPPS (psALPPS). Materials and Methods: Patients who could not safely undergo right trisectionectomy ALPPS because of insufficient FLR were considered eligible for psALPPS, consisting in liver partition through segment 4 using ultrasound guidance. Results: Between April 2017 and April 2021, five patients with median age of 68 years (range: 66-78), four male and one female, underwent psALPPS for colorectal liver metastases (N=2), intrahepatic cholangiocarcinoma (N=2), and hepatocellular carcinoma (N=1). Standardized FLR (sFLR) for segments 2-3 before stage 1 surgery would have been a median of 11.6%. PsALPPS could double the sFLR at stage 1 resulting in an increase of ps-sFLR from a median of 22.7% (at stage 1) to 34.0% (at stage 2) after a median interstage interval of 15 days. All patients tolerated surgery well and no major complications were recorded. Conclusions: Applying the principles of parenchyma sparing surgery to ALPPS offers the advantage to maximize FLR and simultaneously reduce ischemic injury of segment 4 compared to conventional ALPPS. In this way, psALPPS may markedly increase resectability while reducing morbidity. Video: https://www.revistachirurgia.ro/pdfs/?EntryID=922974&art=2021-parenchyma-sparing-ALPPS-ultrasound-guided-partition.pdf

Definitive Surgery for Liver Trauma in a Tertiary HPB Center (with video).

Background: Surgery for severe liver trauma remains challenging even for HPB surgeons, mainly due the hemodynamical instability, involvement of major vascular and biliary elements, impaired background liver and frequent anatomical variants. In this setting, despite conservative policy, major liver resection is still required in selected cases. Also salvage liver transplantation may be needed. Our study aims to analyze the results after definitive surgery for hepatic injury (HI) in a tertiary HPB center. Methods: Sixty-six patients with HI were admitted and treated in our center between June 2000 and June 2021. The median age was 29 years (mean 35, range 10-76). The male/female ratio was 50/16. According to the American Association for the Surgery of Trauma (AAST) system, HIs were grade II in one patient (1.5%), grade III in 11 pts (16.7%), grade IV in 25 pts (37.9%), and grade V in 29 pts (43.9%); no patient had grade I or VI HI. Results: Fifty-two pts (78.8%) benefitted from surgery and 14 pts (21.2%) from non-operative treatment (NOT). Perihepatic packing was previously performed in 38 pts (73.1%). Surgery consisted in hepatic resections (HR) in 51 pts (77.3%) and liver transplantation in one patient (1.5%). The rate of major HR was 51.9% (27 HRs). The overall major morbidity and mortality rates were 33.3% (20 pts) and 13.6% (9 pts), respectively. For surgery, the major complication rate was 35.3% (18 pts), while for major and minor HR were 40.7% (11 pts) and 29.2% (7 pts), respectively; the mortality rate was 15.7% (8 pts). After NOT, the major morbidity and mortality rates were 14.3% (2 pts) and 7.1% (1 pt), respectively. Conclusions: Hepatic resections, especially major ones and/or involving vascular and biliary reconstructions, as well as non-operative treatment for severe hepatic injuries, are to be carried out in tertiary HPB centers, thus minimizing the morbidity and mortality rates, while having the liver transplantation as salvage option.

En-Bloc Complete Segment 1 Resection and Left Hepatectomy for Klatskin Tumor.

video width="640" height="480" controls controlsList="nodownload" poster="https://www.revistachirurgia.ro/pdfs/video/Complete_segment_resection_hepatectomy_klatskin_tumor.jpg" style="margin-top: -20px;" source src="https://www.revistachirurgia.ro/pdfs/video/Complete_segment_resection_hepatectomy_klatskin_tumor.mp4" type="video/mp4" Your browser does not support the video tag. /video We present the case of a 37-year-old male patient with no significant history, hospitalized in our center for painless jaundice with sudden onset. CT contrast portal phase imaging revealed a tumor located at the main biliary confluence, in the proximity of the main portal bifurcation, with no venous or arterial vascular invasion, nor extrahepatic metastases. MRCP diagnosed a Bismuth- Corlette type Klatskin IIIb tumor. For complete assessment of biliary involvement and surgical planning, in addition to the Bismuth-Corlette classification, we took into consideration the presence of infiltration of the bile ducts for segment 1, documented at MRCP. Therefore, a left hepatectomy with en-bloc complete segment 1 resection, along with the main bile ducts and hilar lymphadenectomy, was considered best suited for achieving curative resection in this case. After an uneventful postoperative course, the patient was discharged in the 12th postoperative day. Currently, the patient is disease-free after 84 months. We consider that the long-term recurrence free survival was favored by the complete segment 1 resection. Therefore, for complete assessment of biliary involvement in Klatskin tumor, we recommend that in addition to Bismuth-Corlette classification, infiltration of the bile duct for segment 1 should always be evaluated. If present, the entire segment 1 should be removed for best oncological results.

Anticoagulation Therapy for Portal Vein Thrombosis in Patients with Cirrhosis in a Tertiary Center Experience.

BACKGROUND AND AIMS: The evidence regarding the use of anticoagulant (AC) agents in portal vein thrombosis (PVT) is increasing and, most patients undergo chronic treatment with low molecular weight heparin (LMWH) or vitamin K antagonists (VKA). Nevertheless, there are no clear data about who should receive antithrombotic therapy, when to initiate it, how long and what dose should be used for this set of patients. The aim of the study was to assess the outcome of patients with cirrhosis and portal vein thrombosis who received AC therapy, in terms of thrombus regression, bleeding events and survival rates. METHODS: This observational and retrospective study included 107 cirrhotic patients diagnosed with PVT in a single tertiary center between 2010-2019. 54 received low molecular weight heparin or vitamin K antagonist (AC treatment group) and 53 were untreated. All patients were periodically follow-up to assess the evolution of PVT (regression, progression, stable thrombus) and potential occurrence of bleeding events. RESULTS: The regression of portal vein thrombosis was significantly higher in the AC treatment group (OR=2.430; 95% CI=1.11-6.167; p=0.026), more than 50% of on-treatment patients experiencing regression of the thrombus. However, bleeding events were significantly more frequent in the AC treatment group (18.5% vs. 7.5%) and the risk of bleeding was associated with thrombocytes less than 50x103/mm3 (OR=8.266; 95%CI: 2.310-39.211; p=0.002). Survival was better in the AC treatment group (68.4% vs 48.7% at 5 years and 92.7% vs 77.8% at 1 year, p=0.038) and was lower in patients that experienced bleeding events (37.22% survival at 5 years, mean time survival 44 months, p=0.008). CONCLUSIONS: In our cohort of cirrhotic patients with PVT more than 50% of patients receiving AC therapy presented regression of the thrombus; most of them obtained partial recanalization. The bleeding complication rate was higher than expected, reaching 18%. The overall mortality was lower in the treated group.

Liver Resection for Metastases from Gastrointestinal Stromal Tumors: Does it Improve Long-Term Survival?

BACKGROUND AIMS: The emergence of tyrosine kinase inhibitors, radically altered the management of GISTs and sparked controversy regarding the role of hepatic resection for metastatic tumors. This study aims to identify whether there is improvement in the overall survival of patients with gastrointestinal liver metastases, undergoing hepatic resection in the context of multimodal treatment strategy, as to those approached only by systemic therapy. Methods: Using a retrospective database, we identified 57 patients treated at our center over a 12-year period: Group A (n=31) underwent hepatic resection alongside systemic therapies, and B (n=26) only systemic therapies. In order to obtain a more robust sample, needed for the survival analysis, we performed a propensity score matching and a bootstrapping selection with Jackknife correction for errors; thus, we created an extended sample of 1000 virtual patients. Results: The overall survival measured in all patients was 47 months (95%CI:34-60); significantly higher for group A (56 months, 95%CI:37-75) compared to group B (38 months, 95%CI:19-56), (p=0.007, Log Rank test). Multivariate analysis identified one risk factor: the presence synchronous liver metastases upon diagnosis of primary. Conclusions: Liver resection following TKI therapy is the current mainstay of treatment strategy for potential cure and prolonged survival, in appropriately selected patients evaluated in an multidisciplinary tumor board.

Resection of Giant Liver Metastasis of Gastrointestinal Stromal Tumor Using Intraoperative Ultrasound Guidance.

We present the case of a 37-year-old patient with a surgical history of a gastrointestinal stromal tumor with jejunal location, AFIP classification 6a, hospitalized in our center for synchronous liver metastases. The oncological assessment performed after 12 months from surgery for primary tumor, during which Imatinib was administered, reveals stable disease. CT scan showed a single very large centrally located liver metastasis, 14 cm in diameter, involving segments V and VIII IV, IV and VII, compressing the main portal bifurcation, right hepatic vein, umbilical (scizural) vein and left hepatic vein, invading the middle hepatic vein. We considered it feasible to apply the concept of R1 vascular resection, performing a limited, non-anatomical, ultrasound-guided central hepatectomy, allowing detachment of the tumor from the right hepatic vein and from the umbilical vein. Thus, we sacrificed only the ventral portal pedicles of segments V and VIII and partially preserved these segments to avoid the risk of post-resection liver failure.Currently, the patient is disease-free after 53 months, supporting the concept of ultrasound-guided R1 vascular resection, in the context of systemic therapy with tyrosine kinase inhibitors for metastases of stromal gastrointestinal tumors. (video article https://www.revistachirurgia.ro/pdfs/video/Liver-Resection-Metastases-Stromal-Tumors-2283.mp4).

Tuberous sclerosis with negative genetic testing and multiple cerebral cavernomas: A new association (Case report).

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with multisystemic involvement usually resulting from mutations in the tuberous sclerosis 1 (TSC1) or TSC2 genes. However, 10 to 25% of patients do not exhibit these mutations. Cerebral cavernous malformations (CCMs) are capillary-venous malformations that can be asymptomatic or cause variable neurological manifestations, including seizures. Familial CCMs are recognized. In both conditions, specific dermatological lesions are associated. We present the case of a 31-year-old female with TSC diagnosed at the age of 18 years who presented with negative genetic testing. She was admitted to our department in 2019 for a sudden increased frequency of focal seizures. Patient examination revealed multiple facial and intraoral angiofibroma, diplopia, right hemihypoesthesia, brisk deep tendon reflexes, and distal leg paresthesia. VideoEEG indicated a frontal paramedian epileptogenic focus. Cerebral magnetic resonance imaging (MRI) and angioMRI identified multiple fronto-parietal cortical tubers, as well as multiple CCMs, with evidence of bleeding in one. Under antiepileptic drug (AED) and mTOR inhibitor treatment, the seizure frequency significantly improved in a short period of time. This is the first reported case of tuberous sclerosis with negative genetic testing associated with multiple cerebral cavernoma. Such complex patients require multidisciplinary management and detailed genetic testing for increasing knowledge on neuro-cutaneous disorders.

Is Long-Term Survival in Metastases from Neuroendocrine Neoplasms Improved by Liver Resection?

Background and Objectives: Although many of the neuroendocrine neoplasms (NEN) have a typically prolonged natural history compared with other gastrointestinal tract cancers, at least 40% of patients develop liver metastases. This study aims to identify whether liver resection improves the overall survival of patients with liver metastases from NEN. Materials and Methods: We conducted a retrospective study at "Fundeni" Clinical Institute over a time period of 15 years; we thereby identified a series of 93 patients treated for NEN with liver metastases, which we further divided into 2 groups as follows: A (45 patients) had been subjected to liver resection complemented by systemic therapies, and B (48 patients) underwent systemic therapy alone. To reduce the patient selection bias we performed at first a propensity score matching. This was followed by a bootstrapping selection with Jackknife error correction, with the purpose of getting a statistically illustrative sample. Results: The overall survival of the matched virtual cohort under study was 41 months (95% CI 37-45). Group A virtual matched patients showed a higher survival rate (52 mo., 95% CI: 45-59) than B (31 mo., 95% CI: 27-35), (p < 0.001, Log-Rank test). Upon multivariate analysis, seven independent factors were identified to have an influence on survival: location (midgut) and primary tumor grading (G3), absence of concomitant LM, number (2-4), location (unilobar), grading (G3) of LM, and 25-50% hepatic involvement at the time of the metastatic disease diagnosis. Conclusions: Hepatic resection is nowadays the main treatment providing potential cure and prolonged survival, for patients with NEN when integrated in a multimodal strategy based on systemic therapy.

Somatostatinoma and Neurofibromatosis Type 1-A Case Report and Review of the Literature.

Somatostatinomas are rare neuroendocrine tumors (NET) that arise in the gastrointestinal (GI) tract. Because of their insidious growth, they are usually asymptomatic until late stages, presenting as malignant disease. We report the case of a 50-year-old woman who presented with epigastric abdominal pain, diarrhea and significant weight loss in the last two years. On clinical examination the patient met the criteria for neurofibromatosis type 1 (NF1). Abdominal CT and MRI revealed an infiltrative duodenal mass, with pancreatic invasion, locoregional enlarged lymph nodes and disseminated hepatic nodules. Microscopy and immunohistochemistry uncovered a neuroendocrine tumor, staining positive for chromogranin A (CgA), synaptophysin and somatostatin, with a Ki67 = 1%. Somatostatin receptors (SSTRs) type 2 were negative and SSTRs type 5 were positive in less than 50% of tumoral cells. Our patient was classified as a T3N1M1 stage IV metastatic duodenal grade 1 somatostatinoma and treatment with somatostatin analogues and chemotherapy with capecitabine and temozolomide was started, with so far abdominal imaging follow-up showing stable disease. When a patient is diagnosed with a rare NET, such as a somatostatinoma, it is of utmost importance to determine if it is a sporadic tumor or just a feature of a genetic disorder.

Surgical treatment for severe liver injuries: a single-center experience.

BACKGROUND: The liver is one of the most frequently injured organs in abdominal trauma. The advancements in diagnosis and interventional therapy shifted the management of liver trauma towards a non-operative management (NOM). Nevertheless, in severe liver injuries (LI), surgical treatment often involving liver resection (LR) and rarely liver transplantation (LT) remains the main option. The present paper analyses a single center experience in a referral HPB center on a series of patients with high-grade liver trauma. METHODS: Forty-five patients with severe LI, that benefitted from NOM (6 pts), LRs (38 pts), and LT (1 pt) performed in our center between June 2000 and June 2019, were included in a combined prospective and retrospective study. The median age of the patients was 29 years (median 33, range 10-76), and the male/female ratio of 33/12. Almost all cases had blunt trauma, except 2 with stab wound (4.4%). RESULTS: LIs classified according to the American Association for the Surgery of Trauma (AAST) system were 13.3% (grade III), 44.2% (grade IV), and 42.2% (grade V); none were grade I, II or VI. The rate of major LR was 56.4% (22 LRs). The median operative time was 200 minutes (mean 236; range 150-420). The median blood loss was 750 ml (mean 940; range 500-6500). Overall and major complication rates were 100% (45 pts) and 33.3% (15 pts), respectively. Overall mortality rate was 15.6% (7 pts). CONCLUSIONS: Severe liver trauma, often involving complex liver resections, should be managed in a referral HPB center, thus obtaining the best results in terms of morbidity and mortality.

First Report of a pCys194Arg Notch 3 Mutation in a Romanian CADASIL Patient with Transient Ischemic Attacks and Patent Foramen Ovale - Case Report and Brief Review.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary disease caused by mutations in NOTCH3 gene, characterized by accumulation of a toxic protein in the small and medium size arterioles. Clinical manifestations of CADASIL include lacunar infarcts or, less frequently, large artery ischemic strokes, transient ischemic attacks, dementia, migraine and psychiatric disorders. Brain magnetic resonance imaging (MRI) usually shows multiple lacunar infarcts, diffuse leukoencephalopathy and cerebral microbleeds. The authors report the case of a 39-year-old Romanian woman who presented two transient ischemic attacks manifested with aphasia, headache and mild cognitive impairment. Brain MRI showed multiple isolated and confluent bilateral supratentorial hyperintense fluid-attenuated inversion recovery (FLAIR) and apparent diffusion coefficient (ADC) areas involving the subcortical and deep white matter, but also lenticular and caudate regions and normal aspects of the brain arteries on magnetic resonance angiography (MR-angiography). Differential diagnosis with other disorders affecting small cerebral vessels was performed. Transesophageal echocardiography showed presence of patent foramen ovale (PFO), with right-to-left shunt and contrast passage at Valsalva maneuver. Genetic testing revealed a pCys194Arg heterozygous mutation with C580T>C nucleotide's change on exon 4 of NOTCH 3 gene. The authors discuss the association of CADASIL to PFO and mild cognitive impairment as well as ongoing research for a therapeutic strategy.

Lymphocytes B population profile in a case of multiple sclerosis presenting with sudden sensorineural hearing loss caused by a demyelinating pontine lesion.

Sudden sensorineural hearing loss (SSHL) is a rare manifestation of multiple sclerosis, typically appearing in the early stages of the disease, especially in female subjects. SSHL is produced by the involvement of auditory tract, vestibulocochlear nerve and possibly cochlear structures and rarely due to a single lesion. The authors report the case of a young woman in which the onset of multiple sclerosis presented with SSHL caused by a pontine lesion. Oligoclonal bands in the cerebrospinal fluid (CSF) were absent at the disease onset and appeared during disease progression. Immunophenotyping of cells showed low cellularity of CD19+ cells in the CSF and expression of CD38+ on the majority of CD19+, CD20+ B cells in the peripheral blood, suggesting that many of them were mature B lymphocytes.

The Role of Histopathology and Immunohistochemistry in the Diagnosis of Neuroendocrine Biliary Duct Tumor.

Neuroendocrine tumors of the biliary tract are rare entities developed form Kulchitsky cells which undergo a process of malignant transformation. However, the differential diagnostic between neuroendocrine tumors of the biliary tract and hilar cholangiocarcinoma is very difficult to be established during the preoperative workup; therefore, most patients are submitted to surgery with radical intent and the final diagnostic remains to be confirmed through histopathological and immunohistochemistry studies of the specimen of resection. We present the case of a 60 year old patient who was submitted to en bloc extended right hepatectomy (including segment IV and caudate lobe) with extra hepatic biliary tree resection and left hepatic duct preservation, left cholangiojejunostomy (Roux-En-Y technique), celiac and common hepatic arteries lymphadenectomy and segmental portal vein resection with end-to-end anastomosis for a tumor of the biliary carrefour extended to the right biliary duct and invading the portal vein. The histopathological and immunohistochemistry studies confirmed the presence of a grade 1 neuroendocrine tumor, the staining being positive for Chromogranin A, Neuron-Specific Enolase (NSE) and Ki-67 (1% nuclear positive). At 24 months follow-up the patient is free of recurrent disease.

Retrohepatic Gallbladder Masquerading as Hydatid Cyst in a Patient with Right Liver Agenesis.

Agenesis of the right liver is a rare congenital anomaly which can be associated with an ectopic gallbladder. Hereby, it is presented the case of a 39-year-old man investigated for right upper quadrant abdominal pain and diagnosed at computed tomography with a cystic liver mass initially considered as hydatid cyst. At laparotomy, it was discovered agenesis of the right liver and the presumed hydatid cyst was a retrohepatic gallbladder with lithiasis. Cholecystectomy was performed with an uneventful outcome. Reassessment of the computed tomography images by an experienced radiologist confirmed the intraoperative diagnosis. Although agenesis of the right liver with retrohepatic gallbladder is an exceptional appearance, surgeons should be aware of this anomaly because it can raise challenging issues of diagnosis and surgical planning during cholecystectomy.