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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The lacrimal caruncle of an 11-year-old female patient developed a 2 mm x 3 mm-sized growth with the primary appearance of an abscess. The process did not regress under local antibiotic therapy and was excised. Histological examination revealed histiocytosis X (eosinophilic granuloma).
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PMID:[Histiocytosis X (eosinophilic granuloma) of the caruncle]. 178 61

Advances in pathology allow for more specific diagnoses of orbital disease. The authors discuss the value of awareness of advances in cytology, histochemistry, immunohistochemistry and electron microscopy as applied to orbital disease. Modern cytologic technique can aid in clearer visualization of cellular detail with improved diagnosis of thin needle aspiration biopsies. Histochemistry offers an increasing range of methods for identification of cellular and extracellular substances such as amyloid, fibrin, neuroglia, and collagen. Immunohistochemistry allows for identification of an ever-increasing number of component antigens including immunoglobulins, myoglobins, keratin, glial fibre protein, etc. Electronmicroscopic technique including plastic embedding allow for specific identification of lesions based on subcellular components and characteristic nuclear, cytoplasmic, membrane, basement membrane and stromal components. The value of these methods has been demonstrated with case presentations of "small round cell tumors" of the adult and child. In addition, the pathologic diagnosis of several rare lesions of the orbit including neuroendocrine carcinoma, histiocytosis X, simultaneously occurring poorly differentiated mucoepidermoid carcinoma of lacrimal gland and adenocarcinoma of the prostate are demonstrated to underline the advances in technology. Emphasis is placed on the management of biopsy material to maximize diagnostic potential.
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PMID:Recent advances in pathology as applied to orbital biopsy. Practical considerations. 637 43

A 17-year-old boy presented with a left upper lid swelling, headaches and diplopia. An orbital computerized tomography (CT) scan showed a mass in the left lacrimal fossa eroding bone and extending into the temporalis fossa and intracranially. An urgent biopsy without curettage was carried out and showed Langerhans cell histiocytosis. He was otherwise well and no other lesion was found. He was therefore observed and reviewed regularly. During follow-up it was noted that the mass was reducing in size. Five months after the initial biopsy a further procedure, involving curettage and an intralesional steroid, was carried out at a tertiary referral center. Histology of the material obtained showed no remaining evidence of Langerhans cell histiocytosis. Spontaneous resolution of orbital Langerhans cell histiocytosis has been described clinically and radiologically. This is the first case of spontaneous resolution to be confirmed histologically.
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PMID:Biopsy-confirmed spontaneous resolution of orbital langerhans cell histiocytosis. 1576 15

We report a patient presenting with bilateral lacrimal gland involvement and perioptic nerve sheath lesions due to Langerhans cell histiocytosis (LCH) invasion. LCH is a rare multisystemic disease characterized by a clonal proliferation of Langerhans cells. All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem disease. Osteolytic orbital bone lesions with extension into the adjacent orbital soft tissues have been described. To our knowledge, lacrimal gland involvement has probably been described only once before. Perioptic nerve lesions are also very rare, having been described only three times before.
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PMID:Lacrimal gland and perioptic nerve lesions due to Langerhans cell histiocytosis (2007: 9b). 1800 8

Erdheim-Chester disease (ECD) is a granulomatous and infiltrative disorder of unknown etiology with proliferation of cholesterol-containing histiocytes and peculiar bone involvement. It is very similar to Langerhans cell histiocytosis (LCH) on histology but with a different immunohistochemical profile. This is the first report of intraocular involvement in this disease. MPSG, a 46 y.o. woman, presented with proptosis of the OD. She referred ulcerated lesions on the hard palate, symmetrical and bilateral osteosclerosis of the fibulae and tibiae and a nodule in the right breast (biopsy: xantomatous histiocytic infiltrate CD68+, S-100 and CD1a negative on immunohistochemistry compatible with ECD). MRI studies demonstrated an extraconal tumor in the juxta-bulbar temporal portion of the right orbit close to the lacrimal gland and hyperintense on T1. Vision was 20/20 OU, with numerous drusen in the posterior pole, similar to basal laminar drusen. Two regions of orange subretinal infiltrates that showed progressive staining on the angiogram were seen in the peripapillary region and also close to the fovea in the OD. Choroidal neovascular membranes were seen 2 years later in OU leading to severe visual loss in the OS and to a slight visual field loss in the OD, which retained 20/20 vision. This pioneer report depicts in vivo characteristics of histiocytic granulomas in ECD. Caution should be taken with patients with ECD as potentially blinding intraocular complications may arise.
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PMID:[Intraocular involvement in Erdheim-Chester disease--first report in the literature: case report]. 1815 16

Diseases of the posterior compartment and the orbit are characterised by histological findings, most of which can be reproduced clinically. Examples are the examination of calcifications in retinoblastoma by ultrasonography. In the present review, histological findings of tumour and other diseases of the posterior ocular compartment and the orbit are presented and correlated with the clinical pictures and imaging techniques: uveal melanoma, choroidal nevus, choroidal metastases, choroidal hemangioma, retinoblastoma, Coat's disease, sympathetic ophthalmia, pleomorphic adenoma (benign mixed tumour) of the lacrimal gland, dacryoadenitis, lymphoma, rhabdomyosarcoma, Langerhans cell histiocytosis, orbital metastases, and phthisical eyes. Histopathology is usually the gold standard for a definitive diagnosis. It is very important for residents and those in training to become familiar with clinico-pathological correlations as these provide insight in pathophysiological processes. Regarding ophthalmic surgery, ophthalmic pathology offers the possibility to study wound healing and complications. A close collaboration between clinicians and ocular pathologists allows for an optimised processing of the submitted tissue and diagnosis. Thus, pre- and postoperative care can also be improved. This outstanding knowledge that ophthalmologists have gained over the last decades and beyond, should be preserved and passed on to the next generations in order to maintain a high standard in ophthalmological care.
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PMID:[Clinico-pathological correlations: posterior compartment of the eye and orbit]. 2283 34

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type cells. Orbital LCH is infrequent, typically manifesting as an isolated lytic bony lesion with an adjacent soft tissue mass in a child. Isolated lacrimal gland involvement by LCH is extremely rare, with only 2 previously reported cases. The authors describe a 37-year-old woman with a 6-month history of painless right upper eyelid swelling and diffuse right lacrimal gland enlargement without bony changes on computed tomography scan. Excisional biopsy of the lacrimal gland demonstrated concurrent LCH, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, and increased IgG4-expressing plasma cells. Work-up was negative for systemic hematolymphoid malignancy and IgG4-related disease. This case illustrates the association between LCH, mucosa-associated lymphoid tissue lymphoma, and elevated IgG4 plasma cells in the lacrimal gland, and we review the emerging theories proposed to explain this phenomenon.
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PMID:Isolated Langerhans Cell Histiocytosis of the Lacrimal Gland in Conjunction With Mucosa-Associated Lymphoid Tissue Lymphoma and Elevated IgG4 Plasma Cells. 3121 41