1/30. Rosai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report.Rosai-Dorfman disease (RDD) is a rare idiopathic histoproliferative disease affecting the systemic lymph nodes. Although an extranodal lesion has also been recognized, central nervous system involvement is extremely rare. To the authors' knowledge, only 20 cases of intracranial lesions have been reported previously. Intracranial RDD is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis. The authors treated a large frontal lobe tumor associated with multiple meningeal nodules in a 67-year-old patient presenting with diplopia and headache. Radiological examination indicated that the mass was an inflammatory lesion rather than a meningioma. Microscopically the lesion consisted of mixed inflammatory infiltrate with marked emperipolesis, which is characteristic of RDD. A review of the literature and a discussion of the differential diagnosis of this rare lesion are also presented.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/30. 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 = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/30. Primary intracerebral rosai-dorfman disease: a case report.A 45-year-old woman presented with an isolated, contrast-enhancing brain lesion in white matter of the right frontal lobe, preoperatively thought to be either a primary brain neoplasm or metastasis. The lesion was demonstrated by histology and immunohistochemistry to be Rosai-Dorfman disease. central nervous system (CNS) manifestations of this disease are rare. There have been 27 cases of intracranial involvement reported previously. All of them have been dural-based, where the disease clinically and radiologically resembles meningioma. To our knowledge, this is the first case of an isolated intraparenchymal CNS lesion without dural attachment, where the clinical and radiological features resembled an intraparenchymal glial neoplasm, lymphoma or metastatic tumor.- - - - - - - - - - ranking = 0.2313357654789keywords = nervous system, brain (Clic here for more details about this article) |
4/30. A report of intracranial Rosai-Dorfman disease with literature review.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), is a rare benign histiocytic proliferative disorder. Over 650 cases have been reported since 1969. To the best of our knowledge, there have been only 31 cases with central nervous system involvement reported in the literature. Intracranial disease usually presents clinically and radiologically as a "meningioma". It can be misdiagnosed as a nonspecific inflammatory process because of the atypical histologic features of Rosai-Dorfman disease occurring in a non-nodal location. Familiarity with such atypical histologic features and appropriate use of immunohistochemical stains is required for a definitive diagnosis of central nervous system Rosai-Dorfman disease. We report such an intracranial lesion with other extranodal sites of involvement with a 5-year follow up and a review of previously reported cases. Ann Diagn Pathol 5:96-102, 2001.- - - - - - - - - - ranking = 2keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/30. Meningeal Rosai-Dorfman disease: report of three cases and literature review.Rosai-Dorfman disease is a well-recognized clinicopathological entity, which in rare cases affects the central nervous system, where it mimics meningioma. We describe three cases and review the literature. Histological and immunohistochemical confirmation is essential for definitive diagnosis. In addition to emperipolesis (lymphophagocytosis), reactivity for S-100 and CD68 and nonreactivity for CD-la immunostaining are characteristic features of this histioproliferative disease. In contrast to meningioma, this tumor usually occurs in young males and infiltrates the brain parenchyma.- - - - - - - - - - ranking = 1.0028962991443keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
6/30. Isolated Rosai-Dorfman disease presenting as peripheral mononeuropathy and clinically mimicking a neurogenic tumor: case report.BACKGROUND: Rosai-Dorfman disease is a rare idiopathic disorder of proliferative histiocytes affecting the lymph nodes. It usually manifests as bilateral cervical lymphadenopathy and fever with concurrent polyhyperglobulinemia. Cases involving the nervous system are quite rare; most CNS lesions are located intracranially or arise from the spinal dura or leptomeninges. To our knowledge, there has been no previous report of isolated Rosai-Dorfman disease presenting as peripheral mononeuropathy.CASE REPORT: We report a 43-year-old female with isolated extranodal Rosai-Dorfman disease in the medial aspect of the right upper arm, which presented as aberrant ulnar neuropathy caused by a mass encasing the right basilic vein and the medial anteriobrachial cutaneous branch of the right ulnar nerve. Preoperative diagnosis was a neurogenic tumor. The patient underwent excision of the mass, and pathologic examination confirmed the diagnosis of Rosai-Dorfman disease.CONCLUSION: An unusual case of extranodal isolated Rosai-Dorfman disease, presenting as peripheral mononeuropathy, is reported. Clinically, it simulated a neurogenic tumor, extending the etiologic spectrum of entrapment neuropathy of the peripheral nerve.- - - - - - - - - - ranking = 0.22554316719029keywords = nervous system (Clic here for more details about this article) |
7/30. Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma.Rosai-Dorfman Disease (RDD) is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, manifestation in the central nervous system (CNS) is extremely rare, particularly in the brain parenchyma. A 39-year-old male presented with an isolated well-circumscribed brain mass in the right temporal lobe, preoperatively thought to be a meningioma. histology and immunohistochemistry confirmed that the lesion was RDD. The intraparenchymal brain location of RDD appears to have a benign course. Although the adjuvant therapy is a treatment of choice, surgical resection seems to be the appropriate treatment modality. From the clinical point of view RDD might be an important intracerebral entity because it may mimic other lesions, particularly other histiocytic disorders.- - - - - - - - - - ranking = 1.0202740940101keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
8/30. lung cancer and Rosai-Dorfman's disease. A clinicopathological study.CASE REPORT: A 60-year-old female patient underwent craniotomy for a cerebral lesion in the frontoparietal lobe. Histologically, it turned out to be a metastasis from an adenocarcinoma. The primary tumor was found in the upper lobe of the left lung. The patient had whole brain radiation therapy only, the lung tumor was not treated. 4 years later, she presented with enlarged cervical lymph nodes. A biopsy showed dilated sinuses filled with histiocytes, but no tumor cells. The diagnosis of sinus histiocytosis with massive lymphadenopathy was made (Rosai-Dorfman's disease). Without any treatment, the swelling resolved completely a few weeks later. Similar episodes were observed several times in the following years. The patient died more than 7 years after the diagnosis of a metastasizing lung cancer due to pneumonia. CONCLUSION: In a patient with a pulmonary neoplasm and suspected supraclavicular lymph node spread, Rosai-Dorfman's syndrome should be considered as a rare differential diagnosis.- - - - - - - - - - ranking = 0.0028962991443041keywords = brain (Clic here for more details about this article) |
9/30. May 2003: 57-year-old-woman with acute loss of strength in her right upper extremity and slurred speech.The May 2003 COM. A 57-year-old woman presented with slurring of her speech and right arm weakness. Her past medical history included idiopathic hypertrophic subendocardial stenosis (IHSS), arthritis, asthma, congestive heart failure, hypertension and NIDDM. Neurological examination showed persistent word finding difficulty but her motor and sensory function had essentially returned to normal. Extensive laboratory studies were unrevealing. Imaging studies showed a meningeal lesion over the left posterior parietal lobe and the findings suggested an infectious or inflammatory process. A biopsy of the involved dura and meninges was performed and revealed leptomeningeal Rosai-Dorfman disease. emperipolesis was noted. The finding of emperipolesis is characteristic of Rosai-Dorfman disease of the leptomeninges, but in 30% of cases, this feature will not be identified. Large pale histiocytes of Rosai-Dorfman disease are immunoreactive for S-100 protein and KP1, but negative for CD1a. The differential diagnosis of a chronic inflammatory infiltrate containing numerous, large histiocytes includes granulomatous diseases such as Wegener graulomatosis and sarcoid, hodgkin disease, and Langerhans histiocytosis. CNS Rosai-Dorfman most commonly involves patients between 20- and 40-years-old, with a slight male predominance. Approximately 75% of cases are intracranial, whereas 20% involve the spine. Over 90% of CNS Rosai-Dorfman cases involve the leptomeninges and are seen by neuroimaging as a dural-based, contrast-enhancing masses that often elicit vasogenic edema in the underlying brain. Thus, clinically and radiologically, the disease is thought to represent meningioma. Leptomeningeal Rosai-Dorfman disease is considered a benign condition and in most cases surgical resection is the treatment of choice. Although the number of cases in the literature is small, disease progression following surgical resection is uncommon. Little is known regarding the pathogenesis of Rosai-Dorfman disease. Most have suggested that it represents either an autoimmune disease or a reaction to an infectious agent that has yet to be discovered. Currently it is best considered a benign, idiopathic histiocytosis.- - - - - - - - - - ranking = 0.0028962991443041keywords = brain (Clic here for more details about this article) |
10/30. Crush cytology of Rosai-Dorfman disease of the central nervous system. A report of 2 cases.BACKGROUND: Rosai-Dorfman disease (RDD) of the central nervous system (CNS) is rare. There is no previous report on intraoperative crush cytology of this disorder. CASES: A 70-year-old man presented with headache and was found to have 2 intracranial extraaxial masses. A 62-year-old man presented with cauda equina syndrome secondary to a mass lesion in the sacral canal. The crush smears, prepared intraoperatively from a small portion of the biopsy specimen in each case, showed scattered lymphoid aggregates in a lighter background with a loose admixture of lymphocytes, plasma cells, usual histiocytes and RDD histiocytes. The RDD histiocytes contained single but sometimes multiple nuclei that were larger and more hyperchromatic than those of the usual histiocytes. Many RDD histiocytes had voluminous, pale pink cytoplasm. Lymphophagocytosis by RDD histiocytes was observed. The findings in the crush smears reflected the histology of the lesions. The lymphophagocytosis and nuclear details in the RDD histiocytes were more visible in the crush smears than in the frozen sections. CONCLUSION: Crush cytology appears useful, alone or in conjunction with frozen sections, in the intraoperative diagnosis of CNS RDD.- - - - - - - - - - ranking = 5keywords = central nervous system, nervous system (Clic here for more details about this article) |
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