Development of Hepatocellular Carcinoma after Achieving Sustained HCVVirologic Response and Regression of Cirrhosis

Hepatocellular carcinoma (HCC) is the fastest growing cause of cancer mortality. Interferon/ribavirin and direct acting antiviral (DAA) therapy have successfully treated HCV infection and may halt the progression of fibrosis. It is generally believed that HCC primarily occurs in the background of HCV- related cirrhosis; theoretically, achieving sustained viral response (SVR) could decrease the incidence of HCC by slowing down fibrosing process; hence preventing progression of cirrhosis. Here, we present a case of 64-year-old female who was successfully treated for HCV infection with interferon in 1994. Patient achieved sustained viral response (SVR) and has been in remission for 23 years. In June 2017, she presented with abdominal pain and CT scan revealed a liver mass which was subsequently biopsied and proved to be HCC. Patient was treated with partial hepatectomy and histologic examination of the resected liver mass revealed well-differentiated HCC; non-neoplastic liver demonstrated features of regression of cirrhosis/fibrosis with fibrous expansion of few portal tracts and scattered very fine curvilinear fibrous septa; no portal/lobular inflammation was present. The patient developed HCC 23 years after achieving SVR. Significant regression of cirrhosis/fibrosis in the non-neoplastic liver argues against the usual course of HCC development in cirrhotic/advanced fibrotic setting, and suggests the possibility of an alternative phenomenon, e.g., latency of HCV and/or oncologic potential of HCV at the genomic level. This rare event raises certain questions which have not been properly investigated in the course of HCC development, such as: 1) Relationship between regression of cirrhosis/fibrosis and developing HCC; 2) If absence and regression of cirrhosis/fibrosis carries the same value; 3) Necessity of updating the surveillance criteria in patient with SVR lacking cirrhosis; and 4) Oncologic potential of HCV acquired at the genomic level; these questions/issues are currently under investigation.https://scholarlycommons.henryford.com/merf2020caserpt/1084/thumbnail.jp

Systemic Sclerosis with Gastrointestinal manifestations: a unique presentation

Systemic sclerosis is a chronic autoimmune disease of unknown etiology. Disease course usually begins with Raynaud’s phenomenon followed by skin sclerosis and internal organ involvement. Diagnosis is made based on the clinical symptoms, presence of antibodies and endoscopy with biopsy. With this background, we present a case of a 60-year female who was followed by GI clinic for treatment resistant dyspepsia, bloating and nausea. Past medical history was significant for fibromyalgia, Raynaud’s disease and transient ischemic attack. For evaluation of dyspepsia, she underwent endoscopy with gastric and esophageal biopsies, which were superficial and showed only non-specific chronic inflammation. Further, 24 hour pH monitoring results were unhelpful in making a definitive diagnosis. Esophageal manometry showed a hypertensive lower esophageal sphincter for which she underwent Heller’s myotomy. Her symptoms, however, persisted and she was scheduled to undergo laparoscopy and open gastric wall biopsy. Stomach biopsy revealed gastric mucosa showing focal vascular ectasia. There was significantly increased fibrosis involving the muscularis mucosae and propria (Figure A), highlighted by trichrome stain (Figure B-D). Following biopsy results, Scl-70 Ab test was performed and it turned out to be positive (42, Nunits). Thus, supporting the diagnosis of systemic sclerosis. Full thickness gastrointestinal tract biopsies of systemic sclerosis cases are rarely seen in routine surgical pathology practice. This case is unique because of the complexity of clinical presentation requiring open gastric wall biopsy but demonstrates the value of pathologic evaluation for diagnosis of rare autoimmune disorders such as systemic sclerosis.https://scholarlycommons.henryford.com/merf2020caserpt/1013/thumbnail.jp

Successful Treatment of Hereditary Hemorrhagic Telangiectasia with Octreotide

Hereditary hemorrhagic telangiectasia (HHT) is a disorder characterized by telangiectasias and arteriovenous malformations. We present a case report of a 74-year-old man diagnosed with HHT having a favorable response to a somatostatin analogue for treatment. This patient had been suffering from chronic anemia from recurrent gastrointestinal bleeding, requiring oral/intravenous iron replacement, frequent endoscopic ablations, and blood transfusions. Due to insufficient treatment, he was started on subcutaneous octreotide, with significant improvement as evidenced by a steady increase in the hemoglobin level, decreased endoscopic interventions, and decreased blood transfusions, making this the first case of HHT successfully treated with octreotide

Successful Treatment of Hereditary Hemorrhagic Telangiectasia with Octreotide

Hereditary hemorrhagic telangiectasia (HHT) is a disorder characterized by telangiectasias and arteriovenous malformations. We present a case report of a 74-year-old man diagnosed with HHT having a favorable response to a somatostatin analogue for treatment. This patient had been suffering from chronic anemia from recurrent gastrointestinal bleeding, requiring oral/intravenous iron replacement, frequent endoscopic ablations, and blood transfusions. Due to insufficient treatment, he was started on subcutaneous octreotide, with significant improvement as evidenced by a steady increase in the hemoglobin level, decreased endoscopic interventions, and decreased blood transfusions, making this the first case of HHT successfully treated with octreotide

Appendiceal intussusception presenting as a caecal mass.

INTRODUCTION: The differential diagnosis of caecal mass is broad and the inclusion of appendiceal pathologies is an important element. CASE REPORT: We report a 37-year-old woman with recurrent right iliac fossa pain. Computed tomography scan revealed a caecal mass suggesting complete inversion or intussusception of the appendix, which was confirmed by pathologic microscopic examination. This case report discusses appendiceal intussusception with emphasis on diagnosis and treatment options. DISCUSSION: Appendiceal intussusception is a rare entity and the complete type typically presents as a polypoid lesion located at the appendiceal orifice in the caecum. It is imperative to include this entity in the differential diagnosis of caecal mass, especially during colonoscopy, as the removal of this polypoid lesion can result in a devastating caecal perforation or haemorrhage

Correlation of clinicopathological features and treatment outcome of therapeutic apheresis in refractory liver allograft rejection

Background: Until recently donor specific antibody (DSA) interference with graft function has not been considered a cause for liver graft loss secondary to rejection, and thus medical management has been the standard therapeutic approach, with the exception of hyperacute liver rejection when retransplantation is required. Current literature has shown that persistent elevation of donor specific antibodies (DSAs) is associated with intractable allograft liver transplant rejection and continued liver injury, and therapeutic apheresis intervention is being applied more often, as to treat other solid organ transplant rejection. We retrospectively reviewed the correlation of the laboratory findings and histopathological features in this liver allograft rejection cohort treated with plasmapheresis (TPE) and or Extracorporeal Photopheresis (ECP). Design: Six liver transplant (LT) patients who were refractory to standard anti-rejection treatment were treated with the therapeutic apheresis (TPE/ECP) following our institutional protocol: three patients have completed treatment and 3 are still undergoing treatment. Five patients received TPE along with ECP and one patient only received ECP. The treatment regimen is: TPE 6 treatments, each followed by IVIG; ECP 4 treatment cycles (TC) weekly + 4 TC biweekly + 4 TC monthly. Each TC involves two consecutive days of ECP. The TPE, IVIG and ECP interventions have been tolerated well and without intervention related complications. Antirejection treatment response was monitored by serial liver enzymes (AST, ALT, Total Bilirubin(T. bili) & ALP), DSAs and liver biopsy. Results: Results are summarized in table 1. Serial liver enzymes (AST, ALT, Total Bilirubin & ALP) and liver biopsy on histological examination were correlated and showed improvement and/or resolution of the rejection episode. Sequential liver biopsies showed resolving histologic parameters of rejectionConclusions: In our cohort of patients with refractory liver allograft rejection, we observed that TPE/ECP could be used as an effective additional treatment modality, which alleviates rejection as evidenced by clinic-pathologic parameters. (Table presented)

Appendiceal endosalpingiosis: clinical presentation and imaging appearance of a rare condition of the appendix.

Endosalpingiosis rarely affects the appendix but can be mistaken for acute appendicitis or appendiceal tumors. The medical literature regarding appendiceal endosalpingiosis is sparse; consisting of only four case reports which are primarily focused on the histopathology but provide little radiologic correlation. Endosalpingiosis is a rare condition characterized by the presence of benign fallopian tubal-like glandular epithelium derived from Mullerian ducts, usually affecting the serosal surfaces of the pelvis and peritoneum. It is histologically differentiated from endometriosis as endosalpingiosis lacks endometrial stroma. Endosalpingiosis tends to affect older women and has been associated with ovarian serous tumors of low malignant potential. After a retrospective review of a pathology database, we present pathologically proven cases of appendiceal endosalpingiosis with correlative imaging. We discuss the clinical presentation, illustrate the CT and MRI appearance, histologic characteristics, and review the current medical literature of appendiceal endosalpingiosis

Appendiceal endosalpingiosis: clinical presentation and imaging appearance of a rare condition of the appendix

Endosalpingiosis rarely affects the appendix but can be mistaken for acute appendicitis or appendiceal tumors. The medical literature regarding appendiceal endosalpingiosis is sparse; consisting of only four case reports which are primarily focused on the histopathology but provide little radiologic correlation. Endosalpingiosis is a rare condition characterized by the presence of benign fallopian tubal-like glandular epithelium derived from Mullerian ducts, usually affecting the serosal surfaces of the pelvis and peritoneum. It is histologically differentiated from endometriosis as endosalpingiosis lacks endometrial stroma. Endosalpingiosis tends to affect older women and has been associated with ovarian serous tumors of low malignant potential. After a retrospective review of a pathology database, we present pathologically proven cases of appendiceal endosalpingiosis with correlative imaging. We discuss the clinical presentation, illustrate the CT and MRI appearance, histologic characteristics, and review the current medical literature of appendiceal endosalpingiosis

Reducing Culture Reporting Errors in the Microbiology Laboratory

Introduction: To date, there are few papers that characterize the types of errors in microbiology laboratories, and there is scant research demonstrating the effects of interventions on microbiology lab errors. This study aims to categorize the types of culture reporting errors found in a microbiology lab as well as document the error rates before and after interventions designed to reduce errors and eradicate a blame culture. Methods: To increase the amount of errors reported, we moved from a self-reporting system to an automatic reporting system. Errors were categorized into 5 different types: Gram stain (misinterpretations), identification (incorrect analysis), set up labeling (incorrect patient labels on culture plates), procedural (not followed procedures), and miscellaneous (an organism followed criteria for a certain group, but was eventually identified as different). Error rates were tracked according to technologist, and technologists were given real-time feedback by a manager. Furthermore, error rate was monitored in the daily quality management meeting. Technologists attended a year-end review with a manager in order to improve their performance. To maintain these changes, policies were developed to monitor technologist error rate. If a certain number of errors per month is reached, that technologist is required to undergo re-training by either a manager or senior technologist. If a technologist fails to correct any error properly, they are also required to re-train. Results: In 2013, we recorded 0.5 errors per 1000 tests. By 2018, we recorded only 0.1 errors per 1000 tests, an 80% decrease in errors per 1000 tests. The yearly culture volume from 2013 to 2018 increased by 32%, while the yearly error rate went from 0.05% per year to 0.01% per year, a statistically significant decrease (p=0.0007). Conclusion: This study supports the effectiveness of the changes implemented to decrease errors in culture reporting. By tracking and correcting errors in real time, technologists were educated on error prevention. Laboratory safety became a priority to all technologists in addition to managers through daily error prevention and monitoring.https://scholarlycommons.henryford.com/merf2019qi/1015/thumbnail.jp

Endometrial clear cell carcinoma with metastasis to the common hepatic duct: A rare aetiology of obstructive jaundice, diagnosed by biliary cytology brushing specimen

Clear cell carcinoma is an aggressive subtype of uterine carcinoma. Metastases can be local and/or distant but metastasis to the biliary tree resulting in obstructive jaundice is extremely rare. This is the first report of endometrial carcinoma of clear cell type with metastasis to the common hepatic duct, causing malignant biliary stricture and obstructive jaundice in a young woman, diagnosed on a biliary cytology brushing specimen