ABSTRACT
Dr. Louis Dehner has contributed significantly to our understanding of testicular germ cell tumors in children. This review article considers the molecular biology, clinical attributes, pathologic findings, and prognosis of those lesions.
Subject(s)
Neoplasms, Germ Cell and Embryonal , Testicular Neoplasms , Humans , Infant , Infant, Newborn , MaleSubject(s)
Carcinoma, Signet Ring Cell/complications , Cholangitis/etiology , Duodenal Neoplasms/complications , Stomach Neoplasms/complications , Aged , Bile Ducts/immunology , Biomarkers/analysis , Biopsy , Carcinoma, Signet Ring Cell/diagnosis , Cholangiopancreatography, Endoscopic Retrograde , Cholangiopancreatography, Magnetic Resonance , Cholangitis/diagnosis , Cholangitis/immunology , Duodenal Neoplasms/diagnosis , Fatal Outcome , Humans , Immunoglobulin G/analysis , Immunohistochemistry , Male , Plasma Cells/immunology , Stomach Neoplasms/diagnosis , Tomography, X-Ray ComputedABSTRACT
Netherton syndrome (NS) is a rare autosomal recessive skin disease with severe skin inflammation and scaling, a specific hair shaft defect (trichorrhexis invaginata or bamboo hair), and severe atopic manifestations including atopic dermatitis and hay fever with high serum immunoglobulin E levels and hypereosinophilia. NS is caused by loss-of-function mutations in serine protease inhibitor of Kazal-type 5 (SPINK5) encoding lympho-epithelial Kazal-type-related inhibitor (LEKTI) expressed in the stratified epithelia. We report the first case of penile squamous cell carcinoma in a patient with NS.
Subject(s)
Carcinoma, Squamous Cell/complications , Netherton Syndrome/complications , Penile Neoplasms/complications , Adult , Carcinoma, Squamous Cell/pathology , Humans , Male , Penile Neoplasms/pathologyABSTRACT
AIMS: An increased amount of submucosal (SM) fat in the colon on imaging is considered to be characteristic of inflammatory bowel disease (IBD); however, a recent study in patients without IBD reported a correlation between colonic SM fat deposition and body weight (BW). The aim of this study was to perform a morphometric investigation of SM thickness in areas of fat deposition in the terminal ileum (TI), ileocaecal valve (ICV), and colonic sections, to determine whether there are variations by site, and whether it shows a correlation with BW, body mass index (BMI), or age. METHODS AND RESULTS: Representative samples of TI, ICV and colonic sections were collected prospectively from 115 autopsy cases without IBD. All of the study subjects were male (Veterans Hospital). SM thickness was measured in areas of fat deposition. Correlation analysis was performed between SM thickness and BW, BMI, and age. Fat deposition was common; however, with the exception of the ICV, it was neither consistent nor prominent, and it did not show a statistical correlation with BW, BMI, or age. CONCLUSIONS: SM fat deposition is common but not uniform or conspicuous in the TI or colon. In contrast to extravisceral intra-abdominal fat, it does not show a correlation with BW or BMI, and is not associated with ageing. As all study subjects were male, gender-dependent variability cannot be excluded.
Subject(s)
Adiposity , Body Mass Index , Body Weight , Ileocecal Valve/anatomy & histology , Ileum/anatomy & histology , Adult , Age Factors , Aged , Aged, 80 and over , Anthropometry , Autopsy , Colon/anatomy & histology , Humans , Inflammatory Bowel Diseases/pathology , Male , Middle AgedABSTRACT
OBJECTIVE: The number of islets available (yield) is an important predictor of insulin independence after islet autotransplantation (IAT) done at the time of total pancreatectomy to treat painful chronic pancreatitis. The aim of this study was to correlate histopathologic findings with islet yield and graft function. METHODS: Pancreatic histopathology was examined in 105 adults who underwent pancreatectomy and IAT; postoperative insulin use was known in 53 cases. Histologic degree of fibrosis, acinar atrophy, inflammation, and nesidioblastosis were scored by a surgical pathologist. The correlation of histopathology with islet yield and graft function was evaluated. RESULTS: Patients received a median of 2968 islet equivalents per kilogram. Fibrosis and acinar atrophy correlated negatively with islet yield (P < G 0.001, r =0.67), as did inflammation (P < G 0.001, r =0.43). There was a positive correlation of islet yield (P < G 0.0001, r = 0.64) and a negative correlation of fibrosis (P = 0.006, r = 0.43) and acinar atrophy (P = 0.006, r = 0.42) with islet graft function. CONCLUSION: More severe histopathologic changes were associated with a lower islet yield and lower likelihood of insulin independence. Total pancreatectomy and IAT should not be delayed in patients with painful chronic pancreatitis refractory to medical therapy; otherwise progressive damage to the pancreas may limit islet yield and increase the risk of diabetes.
