Ischemic Cholangiopathy: Insight From an Explant Liver Allograft Affected by Severe Post-transplant Hypoxemia Due to Hepatopulmonary Syndrome: A Case Report.

Increased biliary complications in hepatopulmonary syndrome (HPS) have been hypothesized due to post-transplant hypoxemia. Supporting this hypothesis, we report histopathological findings from an explant liver allograft where the recipient suffered severe and prolonged post-operative hypoxemia. A 4-year-old child underwent liver transplantation (LT) for decompensated chronic liver disease complicated by severe HPS. The post-operative period was complicated by severe prolonged hypoxemia. HPS resolved completely 6 months after LT only to recur 3 months later due to graft dysfunction. The child underwent retransplantation 8 months after the first LT. The explant liver showed bile duct loss along with ulceration and fibrosis of large hilar bile ducts biliary, suggestive of ischemic cholangiopathy. Based on the histopathology findings, we suggest that severe prolonged hypoxemia during post-transplant period could cause ischemic cholangiopathy, which can lead to biliary complications.

Evaluation of biologic potential and risk stratification for predicting disease-free survival after resection of primary gastrointestinal stromal tumor: A multivariate clinicopathological study.

BACKGROUND AND OBJECTIVES: Gastrointestinal stromal tumor (GIST) is mesenchymal neoplasms of the gastrointestinal tract, which express CD117, a c-kit proto-oncogene protein and show gain of function mutation of c-kit gene. Apart from the presence of metastasis, the criteria to differentiate benign and malignant GISTs are not well-defined. Although a variety of prognostic factors have been investigated, no method has yet proven sufficient to enable reliable determination of malignancy in all cases. This study was planned to risk stratify the GIST cases with respect to the various clinicopathological features and to identify prognostic factors in GIST. MATERIALS AND METHODS: n histological and immunohistochemical analysis, 121 cases of GIST were identified. MIB-1 (Ki-67) labeling index (LI) was performed in 60 cases. Follow-up data was available for 93 patients. A P < 0.05 was taken as significant. RESULTS: Larger tumor size, high mitotic activity and Ki-67 LI of >10% were identified as significant predictors of disease-free survival in univariate analysis (P < 0.0001). Other factors of statistically significant value were a high cellularity (P < 0.0027), nuclear pleomorphism (P = 0.0002), epithelioid cell type (P = 0.0098), presence of tumor necrosis (P < 0.01), presence of skeinoid fibers (P = 0.042), S-100 negativity (P = 0.025). Extra-gastrointestinal GIST and metastasis were more frequently associated with progressive disease (PD) as compared with GIST (P < 0.0004), (P < 0.0001). On multivariate analysis size (P = 0.0025), Ki-67 labeling index (P = 0.0186) and mitotic count (P = 0.0375) emerged as independent prognostic predictors of PD. CONCLUSION: This study suggests that GIST in Asian population may have a different phenotype with some predilection to nodal metastasis. Of all the features studied, tumor size and mitotic index are the best prognosticators in GIST with the addition of Ki-67 LI, wherever available.

Quality assurance in quality of life assessment--measuring the validity of the King's Health Questionnaire.

INTRODUCTION AND HYPOTHESIS: The King's Health Questionnaire (KHQ) is a disease-specific, self-administered questionnaire designed to assess the impact of urinary incontinence on quality of life (QOL) in women. To our knowledge there are no data on women's perception of completing the KHQ. Do they feel the KHQ to be useful and valuable or do they feel it to be too burdensome to be used in clinical practice? Therefore, we designed this study to evaluate patients' perception of the KHQ using QQ-10. The QQ-10 is a validated tool designed to measure patient's views on questionnaires. METHODS: This was a prospective observational study conducted at a tertiary referral teaching hospital. Patients were recruited from a one-stop urodynamics clinic. The study participants were asked to complete QQ-10 to give their views regarding KHQ. This produces two responses: positive value (communication, relevance, ease of use, comprehensiveness, enjoyableness, willingness to repeat) and negative burden (over-long, embarrassing, complicated, and upsetting). Mean scores and standard deviation for positive and negative responses were calculated RESULTS: The KHQ was found to have a high mean value (73; range 13-100) and a low mean burden (25; range 0-81) regarding responses to individual QQ-10 items. This was reinforced by the positive comments provided in the text boxes. CONCLUSIONS: Women perceived the KHQ as a valuable tool in their assessment without being bothersome.

Long-term follow-up after colpocleisis: regret, bowel, and bladder function.

INTRODUCTION AND HYPOTHESIS: Colpocleisis is not a popular option amongst surgeons, possibly due to concern about long-term regret. This study assessed a cohort of women who underwent colpocleisis 2-5 years previously to determine the regret rate, the effect on quality of life (QOL) and bladder and bowel function. METHODS: This was a longitudinal study of patients who underwent colpocleisis at least 2 years previously. Participants were asked to complete the Prolapse QOL (P-QOL), International Consultation on Incontinence-Urinary Incontinence (ICIQ-UI Short Form) and Colorectal Anal Distress Inventory questionnaires (CRADI). Two additional questions were asked: (1) Do you regret having vaginal closure surgery for prolapse? (never, sometimes, often, all the time), and (2) Would you recommend this surgery to a relative or friend who is not sexually active? (yes, no.) RESULTS: Thirty-four women were identified. Six had died by the time of follow-up, and five declined to answer the questionnaires because of ill health. Twenty-three women responded (67 %). Mean age was 78.68 years. One woman regretted having had the surgery, as the colpocleisis had failed. Twenty-one women (91.3 %) would recommend this surgery; one would not (4.3 %), and another was not sure (4.5 %). Low P-QOL [8 (0-37) ± 9.41), ICIQ-UI (7 (0-17) ± 5.44) and CRADI (10; 0-28 ± 8.13] scores suggest a positive impact on QOL, bladder and bowel function. CONCLUSION: In this cohort, colpocleisis produced a good outcome with low regret rate (4.3 %), good QOL and minimal effect on bladder and bowel function at 2-5 years.

Surgical management of hepatic arterioportal fistula in a neonate.

Congenital arterioportal fistulae in the liver are rare malformations which can lead to portal hypertension. We report a hepatic arterioportal fistula in a neonate who presented with intestinal hypoperfusion. Computerised tomography angiography showed a fistulous communication between the left hepatic artery and portal vein with hypoperfusion of small and large bowel. A formal left hepatectomy was done followed by clinical improvement and reduction in portal venous pressures. The case and the literature pertaining to it are discussed.

Cytomorphology of gastrointestinal stromal tumors and extra-gastrointestinal stromal tumors: A comprehensive morphologic study.

BACKGROUND: The term gastrointestinal stromal tumors (GIST) is used to refer to those mesenchymal neoplasms of the gastrointestinal tract (GIT) which express CD117, a c-kit proto-oncogene protein. AIMS: To study the cytological features of GIST and extra-gastrointestinal stromal tumors (EGIST), to correlate them with histology and to determine cytological indicators of malignancy. MATERIALS AND METHODS: Cytological smears from patients diagnosed as GIST/EGIST on histology were retrieved. From Jan 2000 to July 2010, 26 GIST (13 primary, 12 metastatic, one recurrent) and seven EGIST (5 primary, one metastatic, one recurrent) cytologic samples from 27 patients were identified. RESULTS: The patients included 20 males and 7 females with a mean age of 50.6 years. Tumor sites included stomach (5), duodenum (5), ileum (2), ileocecal (1), rectum (1), liver (9), retroperitoneum (5), mesentery (1), subcutaneous nodule (1), supra-penile lump (1), ascitic (1) and pleural fluids (1). The smears were cellular with cohesive to loosely cohesive thinly spread irregularly outlined cell clusters held together by thin calibre vessels. The tumor cells were mild to moderately pleomorphic, spindle to epithelioid with variable chromatin pattern and variable cytoplasm. Cellular dyscohesion, nuclear pleomorphism, intranuclear pseudoinclusions, prominent nucleoli, mitosis and necrosis were more prominent in malignant, metastatic and recurrent tumors. CONCLUSIONS: GISTs show a wide spectrum of cytological features and the presence of mitosis, necrosis and nuclear pleomorphism can help in prediction of malignant behavior. Further, cytology is a very useful screening modality in patients of GIST and EGIST to detect early recurrence and metastasis at follow-up.

Diagnosis of adrenal histoplasmosis by fine needle aspiration cytology: an analysis based on five cases.

BACKGROUND: There is limited description in the literature of the initial diagnosis of adrenal histoplasmosis by fine needle aspiration cytology (FNAC). OBJECTIVE: To study the aetiology, clinical features and morphological differential diagnosis of adrenal histoplasmosis diagnosed initially by FNAC. METHODS: Five cases diagnosed by ultrasound-guided FNAC from 2008 to 2010 were found in the laboratory records and reviewed. Clinical features and cytomorphological features were correlated. RESULTS: All the patients were male and were aged 45-83 years (mean 60 years). Three patients had a history of contact with birds, one was diabetic and all were negative for human immunodeficiency virus (HIV). The commonest symptom was weight loss. On cytological smears, uniform round to oval (about 2-4 µm in diameter) budding yeasts were seen intracellularly (within histiocytes) as well as extracellularly. In three cases, an inflammatory response in the form of epithelioid cell granulomas along with multinucleated giant cells was seen. CONCLUSIONS: Adrenal histoplasmosis does occur in immunocompetent persons living in areas not endemic for the disease. Although it is not common, it needs to be considered in the differential diagnosis of bilateral adrenal enlargement. FNAC is a simple, safe technique to establish the initial diagnosis. Correct diagnosis and treatment leads to a favourable outcome.

Adenomyoma of ampulla: a rare cause of obstructive jaundice.

Adenomyoma is a rare benign lesion occurring commonly in the fundus of the gallbladder in the biliary tract. Ampullary adenomyoma is rarer still, presenting as obstructive jaundice due to its location at the ampulla and may mimic malignancy on clinical and radiological examination. Endoscopic biopsy may not be always diagnostic if the lesion is deep seated. Histologically these lesions show a combination of diverse benign epithelial and mesenchymal elements, which should not be confused with an invasive tumor because of its arrangement. We report three cases of adenomyoma located at the ampulla presenting as obstructive jaundice. The diagnosis was confirmed on pacreaticoduodenectomy resection specimens.

Glioblastoma with melanotic differentiation.

A 54-year-old male presented with the history of headache and vomiting. MRI of the head showed right posterior temporal mass which was surgically excised. Histopathological examination revealed features of glioblastoma with pigmented cells. The pigment was demonstrated to be melanin which was confirmed by special stains and immunohistochemistry. This is the first description of glioblastoma with melanotic differentiation reported in the literature. The relevant literature is briefly reviewed.