ABSTRACT
Globular hepatic amyloid (GHA) is rare, and its clinical significance remains unclear. Recently, leukocyte chemotactic factor-associated amyloidosis (ALECT2) has been reported to involve the liver, showing a globular pattern. We reviewed 70 consecutive cases of hepatic amyloidosis to determine the prevalence and morphology of hepatic amyloid subtypes, especially ALECT2 and its association with GHA. Each case was reviewed for amyloid subtype (immunohistochemistry and/or mass spectrometry), its pattern (linear or globular), and distribution (vascular, perisinusoidal, or stromal). In addition, 24 cases of confirmed hepatic ALECT2 on mass spectrometry from our consultation files were also reviewed. LECT2 immunostaining was performed in 49 cases. Of the 70 cases, immunoglobulin light chain (AL) type was most common with 41 cases (59%), followed by transthyretin (ATTR) 15 cases (22%), 3 cases each of fibrinogen A (AFib) (4%), serum amyloid A (AA) (4%), and ALECT2 (4%), 2 cases of apolipoproteins (AApoA1) (3%), and 3 cases (4%) were unclassified. Three of our 70 cases (4%), with ALECT2, and all 24 cases (100%) of mass spectrometry-confirmed hepatic ALECT2 showed only GHA deposits in the hepatic sinusoids and portal tracts. Three (4%) other cases of AL type showed a focal globular pattern admixed with prominent linear amyloid. None of the other amyloid subtypes showed GHA. LECT2 immunostain was positive in all 27 cases (100%) of ALECT2 and negative in the other 22 non-ALECT2 cases (100%) (14 AL, 5 ATTR, 1 AA, 1 AFib, 1 AApoA1). Pure GHA is uncommon (4%) but is highly specific for ALECT2, and LECT2 immunostain is helpful in confirming this amyloid type.
Subject(s)
Amyloid/analysis , Amyloidosis/diagnosis , Intercellular Signaling Peptides and Proteins/analysis , Liver Diseases/diagnosis , Liver/chemistry , Adult , Aged , Aged, 80 and over , Amyloidosis/classification , Amyloidosis/epidemiology , Amyloidosis/metabolism , Biomarkers/analysis , Female , Humans , Immunohistochemistry , Liver Diseases/classification , Liver Diseases/epidemiology , Liver Diseases/metabolism , Male , Middle Aged , Minnesota/epidemiology , Predictive Value of Tests , Prevalence , Prognosis , Retrospective StudiesABSTRACT
Morular metaplasia has been exceptionally described in the stomach. We report the formation of morules in a case of sporadic fundic gland polyposis with low grade dysplasia in a 47 year old woman that underwent total gastrectomy. The immunophenotype of the morules was CD10, CDX2, Beta-catenin nuclear positive; p63, Ki67, chromogranin, synaptophysin, Ck20 negative. The presence of morules in a neoplasm know to be associated with the disruption of wnt/ß-catenin pathway is discussed. The report provides the first description of the immunophenotype of morules in the stomach and hypothesizes the biological similitude of fundic gland polyposis to other tumors characterized by morules formation and wnt/ß-catenin pathway dysfunction.
Subject(s)
Polyps/pathology , Stomach Neoplasms/pathology , Biomarkers/analysis , Comorbidity , Cough/complications , Dermatomyositis/epidemiology , Female , Gastroesophageal Reflux/complications , Humans , Immunohistochemistry , Metaplasia/pathology , Middle Aged , Polyps/complications , Stomach Neoplasms/complicationsABSTRACT
Hibernoma is a rare tumor containing prominent brown adipocytes that resemble normal brown fat. Brown fat (versus white fat) is predominantly found in hibernating mammals and infants. Brown fat adipocytes contain a higher number of small lipid droplets and a much denser concentration of mitochondria. The tumor can occur in a variety of locations however the extremities, followed by the head and neck, have been the most common sights. All variants of hibernoma described have followed a benign course with the majority presenting as a small, lobulated, nontender lesions. We present a case of a giant hibernoma arising from the pleura which invaded the intra and extra-thoracic chest.
