Objective To investigate the clinical and magnetic resonance imaging(MRI) manifestations of Rosai-Dorfman disease(RDD) in central nervous system. Method The clinical and MRI data of 5 cases of RDD in central nervous system confirmed by pathology in the PLA General Hospital were analyzed retrospectively. Results The 5 cases included 4 males and 1 female,aged(39.8±21.7) years on average.Among them,4 cases were located in the intracranial area and 1 case in the thoracic spinal canal.The lesion showed isointense signal on T₁ weighted image and iso,slight-hypo,and slight-hyperintense signals on T₂ weighted image,and it presented intensively homogeneous enhancement in contrast-enhanced MRI.Two cases showed compressed brain area with edema around the left parietal and left frontotemporal dura,thickening and enhancement in the adjacent dura,and dural tail sign.Three cases presented bone destruction in adjacent diploe and thoracic vertebrae.One case showcased slight-hypo perfusion of the left parietal dura in arterial spin labeling. Conclusions RDD lesion usually appears as iso,slight hypo and slight hyper-intense signals on T₂ weighted image and presents intensively homogeneous enhancement in contrast-enhanced MRI.The disease may involve the adjacent bone and the lesion shows slight hypo-perfusion on perfusion images.The MRI manifestations of RDD are characteristic,which are helpful for preoperative diagnosis and evaluation of RDD.
Central Nervous System/pathology* ; Female ; Head ; Histiocytosis, Sinus/pathology* ; Humans ; Magnetic Resonance Imaging/methods* ; Male ; Retrospective Studies

Brain Diseases ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Skull ; pathology

OBJECTIVETo study the prevalence of IgG4-positive plasma cells in Rosai-Dorfman disease and to assess the association between Rosai-Dorfman disease and IgG4-related sclerosing disease (IgG4-SD).

METHODSThe clinicopathologic features of 12 tissue samples of Rosai-Dorfman disease (11 extranodal and one nodal) from nine patients were reviewed. The degree of fibrosis and occlusive phlebitis was studied by HE staining. The expression of IgG4 and IgG in plasma cells were studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSNine tissue samples showed different degree of fibrosis (four tissue samples were mild, one tissue sample was moderate and four tissue samples were severe) and two tissue samples showed occlusive phlebitis in the lesional tissue. Immunohistochemical study showed marked infiltration by IgG4-positive plasma cells (> 50 per high-power field) in four tissue samples, moderate infiltration (30 to 50 per high-power field) in two tissue samples, mild (10 to 29 per high-power field) in three cases and negative infiltration (< 10 per high-power field) in three tissue samples (P < 0.01). Three tissue samples fulfilled the diagnostic criteria of IgG4-SD (> 50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including one tissue sample each of Rosai-Dorfman disease in the left facial skin, above the left eye socket, and in the right parotid.

CONCLUSIONSSome cases of Rosai-Dorfman disease fulfill the diagnostic criteria and show the histologic features of IgG4-SD. They may represent members of the IgG4-SD spectrum. The detection of IgG4-positive plasma cells in the lesional tissues of Rosai-Dorfman disease may have clinical pathological significance.

Fibrosis ; Histiocytosis, Sinus ; diagnosis ; immunology ; Humans ; Immunoglobulin G ; chemistry ; Immunohistochemistry ; Phlebitis ; pathology ; Plasma Cells ; chemistry

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

Adrenal Cortex Hormones ; therapeutic use ; Adult ; Antitubercular Agents ; therapeutic use ; Female ; Histamine Antagonists ; therapeutic use ; Histiocytosis, Sinus ; diagnosis ; drug therapy ; Humans ; Hyperplasia ; diagnosis ; drug therapy ; Langerhans Cells ; drug effects ; pathology ; Prednisone ; therapeutic use ; Young Adult

Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytosis, Sinus ; pathology ; surgery ; Humans ; Immunoglobulin G ; blood ; Paranasal Sinus Diseases ; pathology ; surgery ; Paranasal Sinuses ; pathology ; S100 Proteins ; metabolism ; Sclerosis ; pathology

OBJECTIVETo study the prevalence of IgG4-positive plasma cells in inflammatory disease of nasal cavity and paranasal sinuses and its association with IgG4-related sclerosing disease (IgG4-SD).

METHODSThe expression of IgG4 and IgG in plasma cells of 103 cases diagnosed as inflammatory disease of nasal cavity and paranasal sinuses with dense lymphoplasmacytic infiltrate was studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSImmunohistochemical study showed marked infiltration by IgG4-positive plasma cells (>50 per high-power field) in 28 cases, moderate infiltration (30 to 50 per high-power field) in 23 cases, mild (10 to 29 per high-power field) in 30 cases and negative (<10 per high-power field) in 22 cases (P < 0.05). Twenty-two cases studied fulfilled the diagnostic criteria of IgG4-SD (>50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including 3 cases of chronic sinusitis (3/20), 3 cases of nasal polyps (3/18), 3 cases of inflammatory pseudotumor (3/17), 4 cases of fungal sinusitis (4/20), 1 case of rhinoscleroma (1/12), 7 cases of Wegener's granulomatosis (7/11) and 1 case of Rosai-Dorfman disease (1/2).

CONCLUSIONInflammatory disease of nasal cavity and paranasal sinuses fulfilling the diagnostic criteria IgG4-SD is not uncommon. Definitive diagnosis of IgG4-SD requires correlation with other clinical and laboratory findings. Some cases of unexplained inflammatory disease of nasal cavity and paranasal sinus may represent a member of the IgG4-SD spectrum. IgG4 carries diagnostic value in differential diagnosis of inflammatory disease occurring in nasal cavity and paranasal sinuses.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; metabolism ; pathology ; Granulomatosis with Polyangiitis ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; Humans ; Immunoglobulin G ; metabolism ; Immunohistochemistry ; Male ; Middle Aged ; Nasal Cavity ; immunology ; pathology ; Nasal Polyps ; metabolism ; pathology ; Nose Diseases ; immunology ; pathology ; Paranasal Sinus Diseases ; immunology ; pathology ; Paranasal Sinuses ; immunology ; pathology ; Plasma Cells ; immunology ; Rhinoscleroma ; metabolism ; pathology ; Sinusitis ; metabolism ; pathology ; Young Adult

Although Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites, the nasal cavity and pharyngeal cavity are rarely involved additional. In this report, a 22-year old man with multiple subcutaneous lumps and submucosal lumps in bilateral nasal cavity and pharyngeal cavity is presented, which was diagnosed by nasopharyngeal endoscope,computed tomography scan and biopsy. The subcutaneous lumps were ablated and prednisone was accepted for three months as preventive therapy.
Histiocytosis, Sinus ; pathology ; Humans ; Male ; Nasal Cavity ; pathology ; Pharynx ; pathology ; Subcutaneous Tissue ; pathology ; Young Adult

OBJECTIVETo study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract.

METHODSThe clinical, pathologic and immunohistochemical features of 10 cases of RDD were evaluated.

RESULTSAmong the 10 cases studied, there were 3 males and 7 females. The age of patients ranged from 20 to 61 years old (mean 38 years). The lesion arose in the nasal cavity (7 cases), nasopharynx (2 cases) or hard palate to trachea (1 case). Most of the patients presented with nasal obstruction, rhinorrhagia or tumor mass in the nasal/nasopharyngeal regions. CT scan often showed the presence of soft tissue lesion without bone destruction. Histologically, extranodal RDD was characterized by light-staining bands alternating with dark-staining bands. The light-staining bands were formed by aggregates of large round or polygonal histiocytes with emperipoiesis. The dark-staining bands were formed by abundant lymphoplasmacytic infiltrates. Immunohistochemical study showed that the histiocytes strongly expressed S-100 protein and partially expressed CD68. Six patients had no recurrence after surgical resection.

CONCLUSIONSExtranodal RDD of the upper respiratory tract is a rare disorder of histiocytic proliferation, which usually involves the nasal cavity and paranasal sinuses. RDD can easily mimic rhinoscleroma, mainly due to the overlapping morphologic appearance. Immunohistochemical study is helpful in the differential diagnosis.

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; diagnostic imaging ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Nasal Cavity ; pathology ; Nasopharyngeal Diseases ; diagnostic imaging ; metabolism ; pathology ; surgery ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Nose Diseases ; diagnostic imaging ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism ; Tomography, X-Ray Computed ; Young Adult

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytosis, Sinus ; diagnostic imaging ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Nose Diseases ; diagnostic imaging ; metabolism ; pathology ; surgery ; Nose Neoplasms ; metabolism ; pathology ; Plasmacytoma ; metabolism ; pathology ; Recurrence ; Rhinoscleroma ; metabolism ; pathology ; S100 Proteins ; metabolism ; Tomography, X-Ray Computed ; Young Adult