Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Laminectomy, performed on a 46-year-old woman because of weakness and paresthesias of the lower limbs, showed multiple discrete yellow nodules on the nerve roots of the cauda equina. One of these lesions was excised and proved to be a granuloma consistent with the diagnosis of histiocytosis X.
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PMID:Histiocytosis X of the cauda equina. 108 94

A 14-year-old boy with Langerhans cell histiocytosis of the spinal cord who presented with progressive enlarged bilateral inguinal masses, difficulty in urination, walking and paresthesia in the left lower extremity and perineum is reported. Radiographic studies and computerized axial tomography of T12 to L2 spines showed destruction of the vertebral body of L1 and left-sided paravertebral soft tissue mass extending into the vertebral canal with complete block of the dural sac at the level of L1 spine. The operative finding was an extradural mass at the anterolateral aspect of L1 to L2 spines. After surgical removal of the lesion and chemotherapy with vincristine, prednisolone, 6-MP and methotrexate for 15 months, his neurological deficit improved and there has been no evidence of recurrent disease during his two years follow-up. Langerhans cell histiocytosis is a possible cause of spinal cord compression or cauda equina syndrome and should be considered after excluding other more common causes.
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PMID:Langerhans cell histiocytosis of the spinal cord: a case report. 208 19

A 64-year-old male farmer presented with a rapidly progressive swelling of the left mandible since 6 months. The swelling was firm to hard, diffuse, nontender, obliterating the vestibule with paresthesia of lower lip. The cone beam computed tomography imaging revealed an ill-defined, moth-eaten radiolucency with destruction of the buccal and lingual cortical plates. The rapid growth and aggressive behavior of the lesion coupled with guidance from the patient's previous reports from the incisional biopsy and fine needle aspiration cytology warranted a mandibular resection. Microscopic examination showed an encapsulated lesion situated in the connective tissue containing a mixture of proliferating spindle-shaped cells arranged in fascicles and round cells infiltrating into the connective tissue stroma and bone. The neoplastic cells exhibited atypical features such as pleomorphism, hyperchromatism and increased mitotic figures with noncleaved nuclei. A working diagnosis of a spindle-cell sarcoma was arrived at with various differentials provided such as fibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, Langerhans cell histiocytosis and lymphoma and stating the need for immunohistochemistry to subtype the tumor. The neoplastic cells were negative for Van Gieson's stain and Masson's trichrome. Immunohistochemical analysis performed using desmin, smooth muscle actin, S-100 and CD1a in a bid to determine the phenotype of the tumor and rule out the previously stated differentials were all negative for the lesion. Lymphoid markers such as leukocyte common antigen and CD20 (cluster differentiation marker for B-cells) showed positivity in spindle-shaped cells as well as round cells indicating the tumor to be a lymphoproliferative lesion of B-cell type. A final diagnosis of "spindle-cell variant of non-Hodgkin's lymphoma" was rendered based on the immunohistochemical profile.
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PMID:A rare spindle-cell variant of non-Hodgkin's lymphoma of the mandible. 2719 75