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

OBJECTIVE: To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis (LCH) of the temporal bone in children. METHOD: Seven children with LCH of the temporal bone n our hospital were retrospectively summed up from April 2009 to April 2014. The patients were followed up 1-5 years, their clinical manifestations, imaging findings, diagnosis, therapy and prognosis were studied. Correlation between clinical classifications and prognosis was also analyzed. RESULT: Among the 7 patients, 4 were boys and 3 were girls. 5 cases belonged to the single system group and 2 cases belonged to the multisystem group. The most common clinical characters were temporal tumor, otorrhea, otalgia, hearing loss and granulation of external auditory canal. CT of the temporal bones showed extensive osteolytic destructions with diffuse soft tissure density, without border sclerotization. The cases were. received different therapies. Followed up for 1-5 years, 4 cases were regressive, 1 case kept stable, while 2 cases showed progressive. The two boys then received standard treatment combined steroids with vinblastine. The prognosis in the multisystem group was significantly different from the single system group (P < 0.05). CONCLUSION The clinical manifestations of LCH vary a lot. The diagnosis is based on histological and immunophenotypic examination of lesion tissue. The main therapy includs surgery, chemotherapy and radiotherapy. The prognosis of the single system group is much better than the multisystem group.
Child ; Combined Modality Therapy ; Deafness ; etiology ; Ear Canal ; pathology ; Ear Diseases ; etiology ; Female ; Histiocytosis, Langerhans-Cell ; diagnosis ; pathology ; therapy ; Humans ; Male ; Prognosis ; Retrospective Studies ; Steroids ; therapeutic use ; Temporal Bone ; pathology

OBJECTIVETo study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T).

METHODSThe clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature.

RESULTSThe age of patients ranged from 20 to 68 years. The sites of involvement included lymph node, tonsil and soft tissue. Histologically, the tumor cells were arranged in sheets, fascicles or whorls and intimately admixed with abundant lymphocytes and plasma cells. They were oval to spindly in shape and contained pale eosinophilic cytoplasm, oval nuclei and distinct nucleoli.Immunohistochemical study showed that the tumor cells were positive for S-100 protein and CD68.

CONCLUSIONSIDCS/T is a rare malignant tumor with poor prognosis. It carries distinctive histologic pattern and immunophenotype. The entity needs to be distinguished from follicular dendritic cell sarcoma/tumor, anaplastic large cell lymphoma and other spindle cell sarcomas in occurring soft tissue.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Lymph Nodes ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Middle Aged ; Neck ; S100 Proteins ; metabolism ; Sarcoma ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; Thigh ; Tonsillar Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

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

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

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

Purpose of this study was to analyse the characteristics of clinical, iconographical, pathological and treatment methods of Langerhans cell histiocytosis (LCH), so as to improve the diagnosis and treatment level of this disease. The clinical data of 35 LCH patients were studied retrospectively. These patients were divided into 2 groups according to age <14 years old and ≥ 14 years old. The clinical symptoms were analysed and the signs, imageology and pathology manifestation and treatment results were evaluated. The results showed that LCH clinical manifestations were diverse and complex. Surgical treatment for patients with single system involvement of LCH was better than that of multi-system involvement of LCH (MS-LCH). For the latter, combined chemotherapy effects was better. After 3-year follow-up, 1-year OS was 94% ± 4%, 2-years OS was 91% ± 5%, 3-year OS was 86% ± 7%. 3 years OS of group <14 years old and ≥ 14 years old was 94% ± 6% and 81% ± 10% respectively. The OS of former was better than that of the later, but because a small number of cases, this difference was not statistically significant. It is concluded that LCH is easy to be misdiagnosed, the pathological biopsy is the gold standard of LCH diagnosis. The PET-CT can be of great help in identifying stages and finding lesion areas of the disease. Pulmonary Langerhans cell histiocytosis (PLCH) is more common in adult. Combined chemotherapy can improve the prognosis of the patients. The treatment methods should be choosed according to the stage and classification of disease.
Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Histiocytosis, Langerhans-Cell ; diagnosis ; drug therapy ; pathology ; Humans ; Infant ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Young Adult

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

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

OBJECTIVESTo observe the clinicopathologic features of Langerhans cell histiocytosis (LCH), and to evaluate the values of langerin, CD1a and S-100 protein expression in diagnosis of the tumor.

METHODSTotal 258 cases of Langerhans cell histiocytosis in the past 18 years (from 1992 to 2008) were collected, morphologic review and immunohistochemical staining were performed.

RESULTSIn all 258 cases, the ages of patients older than 16 years or younger than 2 years were 126 (48.8%) and 37 (14.3%), respectively, in the remaining 95 (36.8%) of the cases, the age of the patients ranged from 2 to 16 years. For all of 258 cases, there were 364 diseased sites. Bony lesions accounted for 77.2% (281 cases), especially the skull (112 cases, 39.9%), followed by lymph node (25 cases, 6.9%) and skin (14 cases, 3.8%). Clinically, unisystem or unifocal disease was predominant (201 cases, 77.9%), followed by unisystem and multifocal disease (21 cases, 8.1%), multi-system disease (26 cases, 10.1%), isolated pulmonary LCH (2 cases, 0.8%), and unclassified (8 cases, 3.1%). Histologically, variable number of Langerhans cells was present in 265 samples of 258 cases. Multinucleated giant cells were found in 166 (62.6%) of the samples. Eosinophils were the major infiltrating non-neoplastic cells, and eosinophilic abscess was seen in 57 cases (21.5%). Coagulative necrosis and dead bone were detected in 29 (10.9%) and 124 (46.8%) of the cases, respectively. Immunohistochemically, the expression of S-100 protein, CD1a and langerin was 99.1% (209/211), 100% (206/206) and 98.5% (193/196), respectively, and the sensitivity of them had no statistical difference.

CONCLUSIONSIn this group of LCH cases, the ratio of adult patients is high, but the proportion of multi-organ lesion is low. No significant difference of the sensitivity is found among langerin, CD1a and S-100 expression in diagnosis of LCH.

Adolescent ; Adult ; Antigens, CD ; metabolism ; Antigens, CD1 ; metabolism ; Child ; Child, Preschool ; Diagnosis, Differential ; Eosinophils ; pathology ; Female ; Follow-Up Studies ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Infant ; Langerhans Cells ; pathology ; Lectins, C-Type ; metabolism ; Lymph Nodes ; pathology ; Male ; Mannose-Binding Lectins ; metabolism ; Middle Aged ; S100 Proteins ; metabolism ; Skin ; pathology ; Survival Rate ; Young Adult