1/164. Sinus histiocytosis presenting as bilateral epibulbar masses.PURPOSE: To report a case of sinus histiocytosis presenting as multiple, bilateral epibulbar masses. methods: We examined a 4-year-old child with a 2-month history of gradually enlarging, salmon-colored epibulbar masses. Excisional biopsies and a systemic evaluation were performed. RESULTS: Systemic evaluation was normal; no lymphadenopathy was documented. Histopathologic evaluation disclosed features consistent with sinus histiocytosis. flow cytometry and gene rearrangement studies confirmed the benign nature of the disease. No tumor recurrence was noted. CONCLUSION: Sinus histiocytosis may present as isolated epibulbar masses that may be treated with surgical excision.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
2/164. Sinus histiocytosis with massive lymphadenopathy: a case of multiple dural involvement.An exceptional case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) arising from the meninges in a 60-year-old Japanese man is presented. Computerized tomographic scans and magnetic resonance images demonstrated well-circumscribed tumorous lesions that were homogeneously enhanced with contrast medium. Systemic examination revealed no abnormalities except for a cervical lymphadenopathy and diabetes mellitus. Microscopic examination of the resected specimens showed proliferated histiocytosis and infiltration of plasma cells and lymphocytes. The histology was characterized by the presence of histiocytes demonstrating lymphophagocytosis and immunoreactivity for S-100 protein staining. Immunohistochemical studies and electron microscopy were useful in confirming the diagnosis. The clinical and histopathological features of this disease are discussed.- - - - - - - - - - ranking = 0.85714285714286keywords = histiocytosis (Clic here for more details about this article) |
3/164. Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: first case report.Rosai-Dorfman disease is a histiocytic proliferative disorder which primarily affects lymph nodes. Extranodal involvement occurs in about one half of the patients and the head and neck area represents the region most commonly involved. We present the case of a 48 year-old female who was found with a pancreatic mass during evaluation for abdominal pain. She underwent a distal pancreatectomy and splenectomy. Her pathology showed sinus histiocytosis with massive lymphadenopathy (SHML) involving the pancreas and lymph nodes and focally the spleen. The histiocytes characteristically contained one or more viable lymphocytes in the cytoplasm. The lymphocytes had penetrated the cytoplasm in a process known as "emperipolesis", where the lymphocytes continued to have free movement in the histiocyte. The histiocytic cells were positive with S-100 protein and CD68. Rosai-Dorfman disease (SHML) can affect the peripancreatic lymph nodes with possible secondary pancreatic involvement and present as a pancreatic mass.- - - - - - - - - - ranking = 0.71428571428571keywords = histiocytosis (Clic here for more details about this article) |
4/164. Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin.Sinus histiocytosis with massive lymphadenopathy (SHML) is an idiopathic proliferation of unique histiocytes that have vesicular nuclei and voluminous pale cytoplasm, often with emperipolesis. Pure cutaneous involvement is very rare. We describe a patient with SHML limited to the skin whose lesion has spontaneously regressed. A 35-year-old Korean male visited the Department of dermatology due to facial rash for 2 months. A 3 x 3.5 cm-sized well-demarcated dark erythematous nontender plaque was noted on the right cheek. skin biopsy showed dense, nodular infiltrates of histiocytes with abundant cytoplasm and vesicular nuclei rimmed by lymphoplasma cell aggregates throughout the upper and mid-dermis. The histiocytes were immunohistochemically positive for S-100 protein and CD68, but negative for CD1a. Laboratory tests and a thorough physical examination revealed no abnormalities. These findings suggested that this was a case of SHML clinically limited to the skin. The skin lesion was initially resistant to steroid therapy, but began to regress 10 months after the onset without further treatment.- - - - - - - - - - ranking = 0.71428571428571keywords = histiocytosis (Clic here for more details about this article) |
5/164. radiation response of Rosai-Dorfman disease presenting with involvement of the orbits.The purpose of this study was to review an interesting case of recurrent orbital sinus histiocytosis. The patient initially failed surgery, chemotherapy, and steroid therapy, only to have a durable response to low-dose radiation therapy of the orbits, lasting 6 and 11 years, respectively. Because there are few documented responses to radiotherapy, we present a case report in conjunction with the clinical, radiographic, and histopathologic information as well as a literature review of similar cases.- - - - - - - - - - ranking = 0.14285714285714keywords = histiocytosis (Clic here for more details about this article) |
6/164. Sinus histiocytosis (Rosai-Dorfman disease) of the suprasellar region: MR imaging findings--a case report.Sinus histiocytosis with massive lymphadenopathy (SHML) is an uncommon disorder that typically manifests as systemic symptoms and lymphadenopathy. Extranodal, intracranial disease is uncommon. The authors report on a 15-year-old adolescent girl who had a suprasellar mass at magnetic resonance imaging. biopsy results demonstrated lymphophagocytosis consistent with a diagnosis of SHML. The clinical, radiologic, and histologic aspects of the disease are discussed.- - - - - - - - - - ranking = 0.71428571428571keywords = histiocytosis (Clic here for more details about this article) |
7/164. Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder.AIMS: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Immunophenotypically the histiocytes of SHML express intensively the S100 protein and in addition a panel of macrophage-associated antigens. Their exact relationship to either monocytes/macrophages or immune accessory dendritic cells is, however, still controversial. methods AND RESULTS: In this report recurrent nodal and extranodal manifestations of SHML of a 70-year-old patient were analysed by differential phenotyping using a panel of monoclonal and polyclonal antibodies to macrophage and immune accessory dendritic cell related antigens and by applying nonradioactive in-situ hybridization. CONCLUSIONS: We conclude that stimulation of monocytes/macrophages via macrophage colony stimulating factor (M-CSF) leading to immune suppressive macrophages represents a main mechanism for the pathogenesis of SHML. The study further provides evidence for the monocyte/macrophage but not dendritic cell differentiation of SHML histiocytes.- - - - - - - - - - ranking = 0.71428571428571keywords = histiocytosis (Clic here for more details about this article) |
8/164. Cytology of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign, self-limiting condition of unknown etiology, which generally presents as massive bilateral cervical lymphadenopathy. It is important to distinguish SHML from other causes of histiocytosis because of the different treatment modalities. This study was carried out to assess the utility of fine-needle aspiration cytology (FNAC) findings in SHML and to distinguish if from other reactive lymphadenopathies. The lymph nodes in 4 patients (3 male and 1 female) presenting with massive bilateral cervical lymphadenopathy were aspirated. All presented with persistent bilateral cervical lymphadenopathy, polymorphnuclear leukocytosis, and raised erythrocyte sedimentation rate (ESR). Smears showed a reactive lymphoid population consisting of mature lymphocytes, plasma cells, a few polymorphs, and many histiocytes showing emperipolesis. Based on the cytologic and clinical findings, a diagnosis of SHML was made. Histopathology confirmed the diagnosis in all cases. A conclusive diagnosis of SHML can be based on cytology, provided that the cytologic findings are interpreted in the appropriate clinical context. biopsy can be avoided in these patients. Diagn. Cytopathol. 2000;22:181-185.- - - - - - - - - - ranking = 0.85714285714286keywords = histiocytosis (Clic here for more details about this article) |
9/164. Isolated cerebellar involvement in Rosai-Dorfman disease: case report.OBJECTIVE AND IMPORTANCE: Sinus histiocytosis or Rosai-Dorfman disease (RDD) is a rare but well-recognized disorder characterized by an unusual proliferation of histiocytic cells. Intracranial localization is a rare manifestation of RDD. Only three cases of localization in the posterior fossa have been reported in the literature. The present report describes the first case, to our knowledge, of cerebellar localization of RDD. CLINICAL PRESENTATION: A 67-year-old woman was admitted to our institution with a 5-month history of cerebellar ataxia. Her medical history was unremarkable. The patient was alert and cooperative. No cranial nerve deficits were evident; Romberg positivity to the left side was recorded. No cutaneous abnormalities, lymphadenopathy, or hepatosplenomegaly were revealed by physical examination. Routine hematological and biochemical studies were normal except for the erythrocyte sedimentation rate, which was elevated. Radiologically, the lesion appeared as a well-defined and avascular mass in the right cerebellar lobe. meningioma was considered the most likely diagnosis. TECHNIQUE: The patient underwent a suboccipital craniotomy with complete excision of the lesion. Microscopic examination of the operative specimen revealed the presence of a mixed cellular population with predominant mature histiocytes. A peculiar feature was the presence of lymphocytes and monocytes within the cytoplasm of histiocytes (emperipolesis). Immunohistochemical study of the histiocytes revealed strong positivity for S-100, CD-68 antigen, and vimentin. CONCLUSION: Involvement of the central nervous system in RDD appears to have a benign prognosis, especially in the absence of nodal diseases. Surgery is essential for diagnosis, and, when total removal is achieved, the outcome is generally good without risk of recurrence.- - - - - - - - - - ranking = 0.14285714285714keywords = histiocytosis (Clic here for more details about this article) |
10/164. Rosai-Dorfman disease of the breast: a mimic of breast malignancy.Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is primarily a nodal-based, idiopathic, benign proliferative disorder of histiocytes with 43% of these cases also involving extranodal sites. The breast is an unusual site of occurrence of RDD. We report two cases of this exceptional event. The first represents an intramammary nodal Rosai-Dorfman disease, while the second is an extranodal disease with sole involvement of the breast. In both, the possibility of malignancy was raised. Histological examination of the two breast lesions revealed sheets of characteristic large histiocytes displaying emperipolesis, a microscopic hallmark of this disease. Immunohistochemical and ultrastructural studies were also performed; the former showed cytoplasmic staining of histiocytes for S-100 protein, while histiocytes that engulfed lymphocytes and plasma cells were identified on electron microscopy.- - - - - - - - - - ranking = 0.14285714285714keywords = histiocytosis (Clic here for more details about this article) |
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