ln an attempt to clarify the dual origin histiocytes and to reclassify histiocytic proliferative disorders according to their immunohistochemical properties, normal histiocytes and histiocytes in selected proliferative disorders were stained using the peroxidase-antiperoxidase method for lysozyme, 1-antichymotrypsin and for S-100 protein. The proliferated histocytes of cosinophilic granutoma and Letterer-siwe disease were strongly immunoreactive for S-100 protein. In histiocytic medullary reticulosis (HMR) and in histiocytic lymphoma, all three markers were found within the tumor cells. ln fibrous histiocytoma and in juvenile xanthogranuloma, only a few weakly immunoreactive cells for S-100 protein were observed. lnflammatory malignant fibrous histiocytoma(MFH) (Xanthosarcoma) and xanthoma were immunoreactive for 1-antichymotrypsin and lysozyme respectively. ln MFH of the storiform -pleomorphic type and in atypical fibroxanthoma, stains using all of the histiocytic markers were negative. These results suggest that eosinophilic granuloma. Letterer-Siew disease, fibroxanthoma and juvenile xanthogranloma are proliferative disorder of T-zone histiocytes; HMR and histiocytic lymphoma are those of pluripotential stem cells capable of dual histiocytic differentiation; xanthoma and xanthosarcoma are monocytic proliferative disease; and MFH of the storiform-pleomorphic type and atypical fibroxanthoma are not true histiocytic diseases.
Histiocytes/*metabolism ; Human ; Immunohistochemistry ; Reticuloendotheliosis/classification/*metabolism/pathology ; S100 Proteins/metabolism ; Support, Non-U.S. Gov't

OBJECTIVETo analyze the clinicopathologic and immunohistochemical features of nodular histiocytic/mesothelial hyperplasia (NHMH) and to improve the knowledge of this disease.

METHODSSeven cases of NHMH were collected and the clinicopathologic and immunohistochemical data were analyzed with review of the literature.

RESULTSSeven male patients aged from 1.5 to 5.0 years (mean 2.8). The main clinical symptom was an inguinal mass.Grossly, main pathological changes were the mural nodule or free nodule in lumen, with diameter of 0.1-0.5 cm.Histologically, the tumor cell morphology was relatively single, cohesive polygonal or oval cells which were arranged in solid sheets or nests, usually with ovoid or deeply grooved nuclei and a moderate amount of pale pink cytoplasm in the nodular collection area. The nuclei had delicate chromatin and no obvious atypia, and mitosis was incidentally found. A few scattered lymphocytes were found in the stroma. The cyst wall was lined by a single layer of mesothelial cells.Immunohistochemically, the most cells in nodular lesion were strongly positive for the histiocytic marker CD68, vimentin and α1-antichymotrypsin, while lining mesothelial cells on the wall were positive for calretinin, MC, WT1, CK5/6, CKpan and EMA.

CONCLUSIONSNHMH is a rare and benign tumor-like lesion, and easy to be misdiagnozed, which should be distinguished from neuroendocrine tumors, Langerhans cell histiocytosis, seminoma, mesothelioma and so on. The correct diagnosis of this lesion depends on the clinical characteristics, morphology and immunohistochemistry.

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Calbindin 2 ; metabolism ; Child, Preschool ; Diagnosis, Differential ; Epithelium ; metabolism ; pathology ; surgery ; Histiocytes ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Hyperplasia ; metabolism ; pathology ; surgery ; Infant ; Leukocyte Common Antigens ; metabolism ; Male ; Mesothelioma ; metabolism ; pathology ; Mucin-1 ; metabolism ; Neuroendocrine Tumors ; metabolism ; pathology ; Seminoma ; metabolism ; pathology ; Vimentin ; metabolism ; WT1 Proteins ; metabolism ; alpha 1-Antichymotrypsin ; metabolism

Niemann-Pick type C is an inborn error of metabolism that affects lipid degradation and storage, which is characterized by hepatosplenomegaly and progressive neurological symptoms. A 7-month-old girl with jaundice was presented cholestasis and hepatosplenomegaly. Laboratory study showed elevated acid phosphatase, angiotensin converting enzyme and mild decrease of cholesterol. Characteristic foamy cell and sea-blue histiocytes in bone marrow biopsy consistent with Niemann-Pick disease. Niemann-Pick type C was suspected by past medical history and findings of physical examination. Therefore, molecular analysis was performed and found mutations of NPC1 gene. We report the first Korean case of type C Niemann-Pick disease confirmed by mutation analysis.
Acid Phosphatase ; Biopsy ; Bone Marrow ; Cholestasis* ; Cholesterol ; Diagnosis* ; Female ; Histiocytes ; Humans ; Infant ; Jaundice ; Metabolism ; Niemann-Pick Diseases ; Peptidyl-Dipeptidase A ; Physical Examination

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder characterized by a nonneoplastic proliferation of distinctive histiocyte cells within lymph node sinuses and lymphatics in extranodal sites. SHML occurs worldwide and is primarily a disease of childhood and early adulthood. A 26-yr-old man presented with painless palpable lymph node in cervical area. Radiographic studies revealed pleural effusion with lymphadenopathy and calcification in mediastinum. The cervical lymph node biopsy showed dilated sinuses filled with histiocytes with clear cytoplasm. The cells stained positive with CD68 and S-100. These cytologic and immunohistochemical findings were considered consistent with the diagnosis of SHML.
Adult ; Antigens, CD/metabolism ; Antigens, Differentiation, Myelomonocytic/metabolism ; Histiocytes/pathology ; Histiocytosis, Sinus/*diagnosis/metabolism/pathology ; Humans ; Lymph Nodes/pathology ; Male ; Neck ; Pleural Effusion/*radiography ; S100 Proteins/metabolism ; Tomography, X-Ray Computed

Aged ; Antigens, CD ; metabolism ; Antigens, CD20 ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; CD3 Complex ; metabolism ; CD79 Antigens ; metabolism ; Diagnosis, Differential ; Histiocytes ; immunology ; pathology ; Hodgkin Disease ; immunology ; pathology ; Humans ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell ; immunology ; pathology ; Lymphoma, Large B-Cell, Diffuse ; immunology ; pathology ; Male ; T-Lymphocytes ; immunology ; pathology

Xanthogranulomatous inflammation is an uncommon form of chronic granulomatous inflammation that is characterized by the presence of lipid-filled histiocytes with lymphocytes, plasma cells and neutrophils. It is best known to occur in the kidney. Other organs in which xanthogranulomatous inflammation has been reported are the gallbladder, stomach, anorectal area, bone, urinary bladder, testis, epididymis and female genital tract. Only a few cases of xanthogranulomatous oophoritis have been reported to date. Infection has been thought to be the main etiologic factor in the pathogenesis of xanthogranulomatous lesion. The pathogenesis of xanthogranulomatous inflammation remains still unclear. Other proposed causes are abnormality in lipid metabolism, endometriosis and ineffective clearance of bacteria by phagocytes. We describe a case of a xanthogranulomatous oophoritis in a 23-year-old virgin which associates with hemorrhagic ovarian cyst and minimal association of pelvic inflammatory disease.
Bacteria ; Endometriosis ; Epididymis ; Female ; Gallbladder ; Histiocytes ; Humans ; Inflammation ; Kidney ; Lipid Metabolism ; Lymphocytes ; Male ; Neutrophils ; Oophoritis* ; Ovarian Cysts ; Pelvic Inflammatory Disease ; Phagocytes ; Plasma Cells ; Stomach ; Testis ; Urinary Bladder ; Young Adult