Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rosai-Dorfman disease is a benign lymphohistiocytosis that often involve lymph nodes and present as massive lymphadenopathy with sinus histiocytosis. The disease is rarely associated with intracranial involvement. Herein, we report a 33-years-old man with recent onset of unconsciousness. According to his past medical history, he was suffering from frontal headache, ataxia and dizziness with no sensory or motor defect since August 2010. At initial work up, MRI showed infiltrating mass in the left parietal region. Microscopically, the mass consisted of infiltration of abundant lymphoplasma cells, neutrophils and some histiocytes scattered in fibrotic background. Emperipolesis (lymphocytophagocytosis) of histiocytic cells made the diagnosis of Rosai-Dorfman disease. Rosai-Dorfman disease should be added in the list of differential diagnosis for a dural mass mimicking meningioma or cerebral mass mimicking glioma, therefore, immunohistochemical staining for EMA, S100 and CD1a should be performed to rule out the differential diagnosis.
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PMID:Intracranial ROSAI-DORFMAN Disease. 2326 85

Histiocytic sarcoma is a rare malignancy with only 10 reports confirmed primarily involving the CNS. The diagnosis is dependent on the finding of malignant cells with histiocytic morphology and immunophenotype. The authors report a case of pathologically proven HS of the CNS. A 16-year-old boy presented with headaches, emesis, and altered sensorium. Noncontrast head CT scanning demonstrated a left parietal mass consistent with a tumor. Surgery was undertaken. Intraoperative findings revealed green-yellow exudates consistent with an abscess. Cultures were obtained and broad-spectrum antibiotics were started. The patient subsequently underwent multiple surgical procedures, including drainage and debulking of abscesses and hemicraniectomy. Two months after initial presentation, the patient's diagnosis of histiocytic sarcoma was confirmed. Pathological examination demonstrated necrotizing inflammation with preponderant neutrophil infiltration, variably atypical mononuclear and multinucleate histiocytes, and numerous mitoses. Additional immunohistochemistry studies confirmed immunoreactivity for CD68, CD45, CD45RO, and CD15 and were negative for CD3, CD20, melanoma cocktail, CD30, CD1a, CD34, HMB-45, and melan-A. Once the diagnosis of histiocytic sarcoma was confirmed, antibiotics were stopped and radiation therapy was undertaken. Despite treatment, the patient's neurological status continued to decline and the patient died 126 days after initial presentation. This case represents a rare confirmed example of CNS histiocytic sarcoma. A profound inflammatory infiltrate seen on pathology and green exudates seen intraoperatively make the condition difficult to distinguish from an abscess. Immunohistochemistry showing a histiocytic origin and negative for myeloid, dendritic, or other lymphoid markers is essential for the diagnosis. Further research is needed to establish consensus on treatment.
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PMID:Primary histiocytic sarcoma of the brain mimicking cerebral abscess. 2388 56

Due to the rarity of skull Langerhans cell histiocytosis (LCH), correlations between abnormalities on magnetic resonance imaging (MRI) and pathological findings have not been fully evaluated. Only a few reports have as yet compared MRI of LCH infiltration and histological diagnostic features. A 22-year-old man with an unremarkable past medical history presented with headache followed by a nodular mass in the right parietal area. Gadolinium (Gd)-enhanced T1-weighted images showed a "mushroom-shaped" extension, extending from the subcutaneous to the intracranial space. The galeal tissue, the dura mater, and the cranium adjacent to the lesion showed enhancement on Gd-enhanced T1-weighted MRI. Histologically, diagnosis was LCH, with immunohistochemical positivity for S-100, CD68, and CD1a, and showed a MIB-1 labeling index of 29.5%. By immunostaining, we confirmed LCH cells to be present in the galea with Gd enhancement and the skull without Gd enhancement on MRI. LCH cells might exist in the marginal cranium, if MRI shows only slight intensity change without Gd enhancement. Further evaluation to determine pathologically proven extent of LCH and its relation with neuroradiological diagnosis as well as clinical outcome is needed to understand heterogeneous response to surgical and/or nonsurgical treatment in LCH.
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PMID:Comparison of magnetic resonance imaging with invasive histological findings of Langerhans cell histiocytosis. 2460 97

We report a 58-year-old man who presented with swelling and redness in his left eye, headache, and blurred vision. A contrast-enhanced CT of the orbits revealed bilateral orbital masses. Whole-body PET/CT showed bilateral retrobulbar hypermetabolic soft tissue lesions, multiple areas of soft tissue involvement, and osseous lesions in bilateral lower extremities. An open surgical biopsy of the left orbital mass revealed xanthomatous non-Langerhans histiocytic infiltrate with Touton giant cells, positive for CD68 but negative for CD1a, establishing a diagnosis of Erdheim-Chester disease.
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PMID:Visualization of orbital involvement of Erdheim-Chester disease on PET/CT. 2487 84

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a reactive condition of unknown etiology, characterised by a proliferation of histiocytes exhibiting emperipolesis of lymphocytes and plasma cells. It usually presents as bilateral painless cervical lymphadenopathy. Extranodal RDD without nodal disease is seen in 23% of the cases. Intracranial RDD occurs in less than 5% of the total number of patients with extranodal disease. Isolated intracranial RDD in the absence of nodal disease is exceptional. We report a case of Rosai-Dorfman disease with isolated intracranial involvement. A 38-year-old man came with long-standing headache and the magnetic resonance imaging (MRI) scan showed features of meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive but CD1a and epithelial membrane antigen negative.
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PMID:Isolated intracranial rosai-dorfman disease involving the meninges: report of a rare case. 2564 May 69

Intracranial fibrous histiocytomas are rare; Benign Fibrous Histiocytoma (BFH) being uncommon than its malignant counterpart. BFH comprises fibroblasts and histiocytes without any nuclear pleomorphism or atypia. We present a case of a 42-year-old male who had swelling over the occipital region for the past five years, which progressively increased in size. He developed headache, dizziness, and gait disturbance over the last six months. Computed tomographic scan revealed a posterior fossa space-occupying lesion. Fine-needle aspiration cytology from the swelling revealed spindled fibroblasts along with histiocytes and multinucleated giant cells. Later, histopathology showed presence of spindle-shaped cells in storiform pattern admixed with histiocytes and giant cells. The giant cells and histiocytes were immunopositive for CD68 and spindled cells were positive for vimentin, but immunonegative for CD34, epithelial membrane antigen, CD1a and S100. The final diagnosis was intracranial BFH. We present this case because of its extreme rarity and unusual location.
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PMID:Benign Fibrous Histiocytoma: An Uncommon Presentation. 2889 10

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease (RDD), was first described in 1969 as a reactive condition of unknown etiology that is characterized by a proliferation of histiocytes exhibiting emperipolesis of both lymphocytes and plasma cells. It usually presents with painless cervical lymphadenopathy either with or without extranodal manifestations. Intracranial involvement of this disease is extremely rare. Intracranial RDD occurs in <5% of all patients with extranodal disease. Here, we report a case of RDD with isolated intracranial involvement. A 67-year-old male presented with a long-standing headache, retro-ocular pain, and progressive visual loss of the left eye. Magnetic resonance imaging showed features of optic nerve meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive; however, a CD1a and epithelial membrane antigen were negative.
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PMID:A Rare Case of Isolated Intracranial Rosai-Dorfman Disease Mimicking Optic Nerve Meningioma: A Case Report and Literature Review. 3028 62

The association between Rosai-Dorfman Disease (RDD) and cancer was reported for the first time in 1984. However, there are still a small number of reports of this association. We describe a 60-year-old woman who presented with a focal onset motor seizure followed by tonic-clonic generalization and persistent headache. Magnetic Resonance Imaging disclosed an irregular hyperintense lesion in T2 and vasogenic edema in the left parietal region. Immunohistochemical analysis of a biopsy fragment was positive for protein S-100 and CD68 and negative for CD1a, compatible with the diagnosis of RDD. She previously had breast cancer six years earlier and had used tamoxifen for two years and anostrozol for three years after diagnosis of cancer. RDD has been already associated with different cancers, such as Hodgkin's lymphoma, non-Hodgkin's lymphoma, follicular lymphoma, melanoma, adenocarcinoma and small cell lung cancer. As far as we know, this is the first report of an association between breast cancer and RDD.
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PMID:Isolated central nervous system Rosai-Dorfman disease and breast cancer: an unusual presentation. 3029 95


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