1/97. 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 = 1keywords = histiocytosis (Clic here for more details about this article) |
2/97. Punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis: report of four new cases.Four new cases of punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis (LCH) are added to the four previously reported cases. All cases were shown on CT scans; plain films were rarely diagnostic. Pathologic correlation remains elusive since the usual biopsies of the LCH have been on skin or bone biopsies. A single prior pathologic study of the thymus in untreated LCH showed microscopic calcospherites. The thymic punctate calcific densities in patients with LCH may represent further accretion so that the calcospherites become macroscopic. The finding of such punctate calcific densities in an enlarged thymus of an infant with skin or bone or lung disease is strongly suggestive of LCH.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
3/97. 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 = 1.2keywords = histiocytosis (Clic here for more details about this article) |
4/97. Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma involving the same lymph node: a report of four cases and review of the literature.Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a disorder of unknown cause. Rarely, patients with SHML also have malignant lymphoma, usually involving anatomic sites different from those involved by SHML We report four patients in whom SHML and malignant lymphoma were identified in the same lymph node biopsy specimen. The SHML in each case was present as a small focus, less than 1 cm. Immunohistochemical studies showed that the abnormal histiocytes were positive for S-100 and negative for CD1a. The malignant lymphomas included two cases of follicular lymphoma and two cases of Hodgkin's disease, nodular lymphocyte predominant type. The presence of SHML in these patients did not impact clinical decisions, and there was no evidence of SHML elsewhere. Thus, the presence of focal SHML associated with malignant lymphoma in these cases was an incidental histologic finding that seems not to have had any clinical significance.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
5/97. Sinus histiocytosis with massive lymphadenopathy.Five cases of "sinus histiocytosis with massive lymphadenopathy" are reported. The clinical features and histopathological appearances are described. The majority were African children with massive cervical lymphadenopathy. Histologically, the lymphnode sinuses are widely dilated by mature histiocytes, with phagocytosis of lymphocytes and other hematopoietic cells by the proliferating histiocytes. The diagnostic difficulties are discussed. These are the first cases recognized in zambia.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
6/97. Marginal corneal infiltrates: a possible new manifestation of sinus histiocytosis with massive lymphadenopathy.PURPOSE: To describe unilateral deep marginal corneal infiltrates and chronic anterior uveitis in a young patient with sinus histiocytosis and massive lymphadenopathy. methods: A 20-year-old man with a painless enlargement of his cervical lymph nodes was followed for five years after debulking of these nodes on the right side for right chronic anterior uveitis and deep marginal corneal infiltrates. RESULTS: Histological sections of cervical lymph node confirmed the diagnosis of sinus histiocytosis with the typical emperipolesis. The uveitis partially responded to topical corticosteroid treatment but tended to recur upon its withdrawal. The marginal corneal infiltrates resolved gradually over a period of five years. CONCLUSIONS: Marginal corneal infiltrates and uveitis may presumably be associated with sinus histiocytosis. patients with sinus histiocytosis should also be screened for rare ocular involvement, such as uveitis and corneal infiltrates.- - - - - - - - - - ranking = 1.6keywords = histiocytosis (Clic here for more details about this article) |
7/97. Langerhans cell histiocytosis infiltration in cerebrospinal fluid: a case report.Langerhans cell histiocytosis (LCH) is a disease of unknown etiology characterized by a proliferation of histiocytic cells resembling the integumentary cells bearing the name of langerhans cells. LCH can be unifocal or multifocal, with one- or many-organ involvement. We present a case of LCH diagnosed in the cerebrospinal fluid of a patient with generalized lymphadenopathy and central nervous system involvement.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
8/97. Rosai-Dorfman disease of the parotid gland: cytologic and histopathologic findings with immunohistochemical correlation.Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown origin and a distinct clinicopathologic entity also known as sinus histiocytosis with massive lymphadenopathy. The disease can involve extranodal tissues and rarely can present as salivary gland enlargement without significant lymphadenopathy. Involvement of the extranodal head and neck sites appears to be more common in patients with immunologic abnormalities. The disease was first described in 4 patients in 1969, and with later descriptions of more patients, the disease was established as a well-defined clinicopathologic entity. The characteristic pathologic feature of this disease is proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrate, consisting of moderately abundant plasma cells and lymphocytes. Fine-needle aspiration biopsy can be helpful in establishing the correct diagnosis, since surgical treatment is not necessary other than obtaining tissue for definitive diagnosis. We describe cytologic, histopathologic, and immunohistochemical features of a case of Rosai-Dorfman disease that involved a major salivary gland without significant lymphadenopathy in a 48-year-old patient with systemic lupus erythematosus. We also briefly discuss possible causes and pathogenesis and review the literature.- - - - - - - - - - ranking = 0.2keywords = histiocytosis (Clic here for more details about this article) |
9/97. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review.Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites. It usually shows a prolonged clinical course with occasional exacerbation and remission phases. We describe the long-term follow-up of a case of SHML that showed typical clinical features and in which various therapeutic strategies were attempted. Chemotherapy and alpha-interferon (IFN) were ineffective; surgery was ultimately required with satisfactory results. From an extensive literature review we found different treatment strategies in SHML in the 80 cases published between 1969 and 2000. Spontaneous resolution of adenopathies is frequently observed: 32 out of 40 cases which did not receive chemotherapy, radiotherapy, or surgery were healthy at the time of publication. radiotherapy alone showed conflicting results: 3 complete remissions (CR) were obtained in the 9 patients treated. Surgical debulking when required was effective--8/9 CR--while chemotherapy showed generally negative results. IFN has been previously employed in only one case. In conclusion, clinical observation without treatment is advisable when possible. In the presence of vital organ compression and/or extranodal localization with important clinical signs, surgical debulking may be necessary. radiotherapy has shown limited efficacy, while chemotherapy is in general ineffective. More experience is needed to evaluate the role of IFN.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
10/97. Lipogranulomatous lymphadenopathy as a potential source of error in fine needle aspiration cytology. A case report.BACKGROUND: Lipogranulomatosis refers to the presence, in lymph nodes and spleen, of lipid material arising from endogenous sources, such as tumors, hematomas, cholesterol deposits, fat embolism and fat necrosis. The histologic image is similar to that seen in lymph nodes after lymphography, with vacuolized histiocytes; round, empty spaces; and numerous multinucleated giant cells (MGC). CASE: A 27-year-old woman was referred to our department for fine needle aspiration of bilateral inguinal lymph nodes. One year before, a giant cell tumor of the sacrum was surgically excised at another medical center. The aspirated material showed a normal lymphoid cell background in addition to several MGC and ill-defined cellular groups of mononuclear, histiocytelike cells admixed with mature lymphocytes. After the cytologic report was issued both nodes were surgically excised. The most remarkable histologic finding was the presence of sinus histiocytosis and multifocal subcapsular and sinusoidal lipogranulomas with numerous MGC. CONCLUSION: Lipogranulomatous lymphadenopathy is a rare pathologic condition and may be responsible for smears with numerous MGC. This entity should be considered when evaluating lymph node aspirates from patients with neoplasia, particularly that with a giant cell component.- - - - - - - - - - ranking = 0.2keywords = histiocytosis (Clic here for more details about this article) |
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