Adult ; Histiocytosis, Langerhans-Cell ; diagnosis ; Humans ; Male ; Pancreas ; pathology ; Parathyroid Glands ; pathology

Child, Preschool ; Diagnosis, Differential ; Histiocytosis, Langerhans-Cell ; complications ; diagnosis ; physiopathology ; Humans ; Male ; Thyroid Gland ; pathology ; Thyroid Nodule ; diagnosis ; etiology ; pathology

Diagnosis, Differential ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; surgery ; Humans ; Infant ; Male ; S100 Proteins ; metabolism ; Xanthogranuloma, Juvenile ; metabolism ; pathology

OBJECTIVETo improve the recognition of the clinical features and results of laboratory examination for isolated pulmonary Langerhans cell histiocytosis (PLCH) in children.

METHODThe information of one case with isolated PLCH was analyzed and reports of 11 cases with isolated PLCH were reviewed.

RESULTThe patient we report is only 2 years old with 1 month of course of disease, manifesting with prominent pulmonary involvement: cough and short of breath; CT scan of the chest showed punctiform, nodular and reticular high density opacities involving all lobes of both lungs. Biopsy of the lung tissue showed expression of CD1a, CD68, S-100, consistent with the diagnosis of LCH. He received prednisolone, VP16 and Vindesine with good response. Ten of 11 cases of isolated PLCH reported before manifesting with cough and dyspnea, CT scan of the chest showed interstitial lung changes (5/8), cystic changes (5/8), small nodules (2/8) and pneumothorax (2/8). Langerhans cells were found in 9 cases on lung biopsy, part of biopsy lung tissues were stained with anti-CD1a, the alveolar lavage fluid of the other 2 cases were stained with S-100 and anti-CD1a.

CONCLUSIONIsolated PLCH is rarely reported in children. It manifested with prominent pulmonary involvement: cough and short of breath, and CT scan of the chest showed interstitial lung changes, small nodules or cysts involving the lung, Langerhans cell could be found in lung biopsy, and the immunohistochemical staining in lung biopsy lung and alveolar lavage fluid stained with S-100 and anti-CD1a antibodies.

Biopsy ; Bronchoalveolar Lavage Fluid ; Child, Preschool ; Histiocytosis, Langerhans-Cell ; diagnosis ; pathology ; Humans ; Lung ; pathology ; Male ; Retrospective Studies

Antigens, CD ; metabolism ; Antigens, CD1 ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Infant ; Langerhans Cells ; pathology ; Lymph Nodes ; pathology ; Lymphohistiocytosis, Hemophagocytic ; pathology ; Male ; S100 Proteins ; metabolism

Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis.
Adolescent ; Brain ; Diagnosis ; Diagnosis, Differential ; Dura Mater ; Female ; Histiocytosis, Langerhans-Cell* ; Humans ; Langerhans Cells ; Magnetic Resonance Imaging* ; Meningioma* ; Pathology ; Skull ; Tail

OBJECTIVETo investigate the clinicopathologic features, diagnosis, differential diagnosis of multiple Rosai-Dorfman disease (RDD).

METHODSSeven cases of multiple RDD were treated in Fujian Provincal Hospital and Fuzhou General Hospital of Nanjing Military Command of Chinese PLA. The disease was analyzed, focusing on the process of diagnosis, the treatment and follow-up. Histopathology, immunohistochemical profiles and relative literature were reviewed to reveal the characteristics of this disease.

RESULTSThe seven cases, occurred in 3 women and 4 men from 18 to 60 years of age (median 45.71 years), with masses measured of 0.8-6.0 cm (average size of 3.0 cm). Masses located in, left subcutaneous arm, thyroid, paratrachea, left maxilla, right subcutaneous cheek, left subcutaneous chest wall, right subcutaneous inguina, bilateral subcutaneous neck, right tibia, right thigh skin, right frontal lobe of brain, cerebral parafalx and bilateral lymph nodes of the neck, respectively. Among the cases, from the first case to the sixth case were extranodal tissue, and the seventh case was located in lymph nodes. Cases showed progressive increase of the mass. Histologically, all lesions of seven cases were similar with nodular structures presenting with alternating hyper- and hypo-cellular areas. The hypo-cellular areas revealed lymph-sinustoid structure characterized by variable numbers of large histiocytes, which had an abundant cytoplasm, pale to eosinophilic in appearance, phagocytozed intact lymphocytes or emperipolesis. While hyper-cellular areas revealed the infiltration of lymphocytes, plasma cells, neutrophils and numerous collagen fiber. Two cases also revealed the infiltration of lymphoid follicles. Immunohistochemically, the large histiocytes were strongly positive for S-100, CD163 and CD68 protein.

CONCLUSIONSMultiple RDD is rare. In clinic and pathology, it needs to be differentiated from granulomatous diseases, IgG4-related sclerotic diseases, inflammatory myofibroblastic tumor, fibrohistiocytoma, Langerhans cell histiocytosis, and so on. The primary approach of treatment for multiple RDD is complete surgical excision and its prognosis is good.

Adult ; Brain Diseases ; pathology ; Diagnosis, Differential ; Female ; Histiocytosis, Langerhans-Cell ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Lymph Nodes ; pathology ; Lymphatic Diseases ; pathology ; Male ; Middle Aged ; Skin Diseases ; pathology ; Thigh ; Young Adult

Antigens, CD1 ; metabolism ; Child, Preschool ; Cholangitis, Sclerosing ; diagnosis ; etiology ; metabolism ; Diagnosis, Differential ; Female ; Histiocytosis, Langerhans-Cell ; complications ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Liver ; metabolism ; pathology ; S100 Proteins ; metabolism

OBJECTIVE: To study the diagnosis and treatment for Langerhans cell histiocytosis (LCH) of head and face. METHOD: Clinical data of 19 cases suffering from LCH of head and face verified by pathology was retrospectively analyzed. The clinical presentation, diagnosis and treatment were discussed. RESULT: Of all the 19 cases, 5 patients had an involvement of the scalp or skull, 5 had the maxilla involvement and 9 temporal bone. Among the 19 patients, type I stage I was 8 cases(all adults), type II stage I 7 cases (all children), type ILL stage II 2 (1 adult, 1 child), stage III 1 and stage IV 1 (both child). Their main clinical manifestations included scalp mass, defect of skull, swelling or mass of facial maxilla and of ear or temporal bone, otorrhea, granuloma of auricular canal, and so on. Fifteen cases were cured by surgical curettage combined with radiotherapy and 4 by surgical curettage combined with chemotherapy. One patient had the sequelae of diabetes insipidus. CONCLUSION Diagnosis of LCH should be based on synthetical analyses of clinical manifestations, features of imaging and histopathology. Surgical curettage, radiotherapy and chemotherapy are effective treatment regimens. The prognosis is generally good if the patient gets correct diagnosis and timely rational treatment.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Face ; pathology ; Female ; Head ; pathology ; Histiocytosis, Langerhans-Cell ; diagnosis ; pathology ; therapy ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

A 31-year-old man presented with a dry cough and exertional dyspnea. The chest X-ray showed multiple nodular opacities throughout the entire lung field. Chest computed tomography (CT) revealed variable-sized nodules with a peribronchiolar or centrilobular distribution, some of which revealed thick-walled cavitary change. Based on the chest CT findings, it was initially assumed that metastatic lung nodules with hematogenous spread were present; therefore, we performed an open lung biopsy. On microscopic examination, several compact cellular interstitial infiltrates composed of Langerhans' cells, eosinophils, and lymphocytes were observed. Immunochemically, the Langerhans' cells showed strong cytoplasmic staining for S-100 protein. Based on these findings, the patient was diagnosed with Langerhans' cell histiocytosis of the lung. High-resolution CT of the chest is a useful, sensitive tool in the diagnosis of pulmonary Langerhans' cell histiocytosis (PLCH). A typical radiologic finding of PLCH is irregularly shaped cysts. The radiological finding in this case of nodular opacities throughout the lung fields only without cysts is rare in PLCH. We report a case of PLCH with atypical multiple nodules mimicking hematogenous metastatic lung nodules.
Adult ; Diagnosis, Differential ; Histiocytosis, Langerhans-Cell/*diagnosis/etiology/pathology/radiography ; Humans ; Lung Neoplasms/*diagnosis/*secondary ; Male ; Radiography, Thoracic ; Tomography, X-Ray Computed