1/30. Reconstruction of the mandibular condyle using transport distraction osteogenesis.Transport distraction osteogenesis was used to reconstruct the articulation of the temporomandibular joint in two patients. Patient 1 underwent a gap arthroplasty for release of a bony ankylosis. Patient 2 had degenerative joint disease after tumor resection. Both patients had painful, limited mouth opening and facial asymmetry as a result of condylar shortening. A reverse-L osteotomy was performed from the sigmoid notch to the posterior border of the mandible. An external transport distraction device was applied, and the segment was advanced superiorly, 1.0 mm per day, until contact was made with the glenoid fossa. After distraction, the bone was found to have remodeled, inducing a new cortical layer on the articular surface. Additionally, a pseudodisk was generated as a result of the transport distraction. The two patients tolerated the procedure well, returning to pain-free normal mouth opening and being able to masticate regular diets.- - - - - - - - - - ranking = 1keywords = tract (Clic here for more details about this article) |
2/30. Ocular and cerebral involvement in familial lymphohistiocytosis.A 5-month-old infant girl died of familial lymphohistiocytosis. The central nervous system showed widespread perivascular infiltration of the cerebral pia and arachnoid, the cerebral white matter and deep gray matter, the cerebellum, and brain stem by lymphocytes, benign appearing histiocytes, and macrophages with erythro-and lymphophagocytosis. The eyes had mild infiltration of the anterior uveal tract, moderate involvement of the inner retina, and marked infiltration of the optic nerves by identical cells.- - - - - - - - - - ranking = 0.125keywords = tract (Clic here for more details about this article) |
3/30. Langerhans' cell histiocytosis arising at the site of basal cell carcinoma excision.Langerhans' cell histiocytosis (LCH) may be associated with a wide range of neoplastic disorders. However, the combination of a LCH and a basal cell carcinoma (BCC) of the skin still represents a highly unusual condition. In this publication, we report the case of a 48-year-old woman who developed a localized LCH involving the area of a previously excised BCC. Although the exact pathogenesis of this peculiar association remains yet to be elucidated, the hypothesis of an exaggerated Langerhans' cell reaction in response to the underlying BCC appears to be attractive.- - - - - - - - - - ranking = 0.125keywords = tract (Clic here for more details about this article) |
4/30. Digestive tract involvement with exudative enteropathy in Langerhans cell histiocytosis.Protein-losing enteropathy was observed in two children with Langerhans' cell histiocytosis (LCH). One patient was an infant with congenital cutaneous lesions; the second child had sigmoid and lymph node infiltration. Electron microscopy and immunohistochemistry confirmed, in both, infiltration of duodenum, skin, and liver by LCH. Gastrointestinal involvement by LCH seldom produces prominent clinical manifestations but indicates widespread multisystem disease. Immunohistochemical and/or ultrastructural features allow definitive diagnosis from mucosal biopsy specimens. review of the literature of gastrointestinal infiltration by LCH emphasizes its poor prognosis, especially when associated with organ dysfunction.- - - - - - - - - - ranking = 0.5keywords = tract (Clic here for more details about this article) |
5/30. Vulvar Langerhans cell histiocytosis: a case report and review of the literature.BACKGROUND: Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Only, seven cases of primary vulvar LCH have been previously reported in the medical literature. We describe an additional case of LCH in which the disease was confined to the vulva. CASE: A 33-year-old gravida 0, para 0 Ethiopian woman presented with a nodular lesion on her left vulva. The lesion was biopsied, and the results were consistent with LCH. A metastatic workup did not reveal any evidence of disease beyond the vulva. The patient was initially treated with radiotherapy to the vulva. She was diagnosed with recurrent disease in the vulva 21 months after the completion of radiotherapy. At that time, she underwent a wide local excision. Five months later, we found a lesion on her right labium majus that was consistent with a recurrence. The patient's vulva was treated with a higher dose of radiotherapy than it had been the first time. Six months later the patient again experienced a local recurrence. She underwent a wide radical vulvar excision of diffuse bilateral lesions and was free of disease for approximately 3 months, after which she experienced another recurrence and underwent treatment with thalidomide. Within 2 months of starting thalidomide therapy, the patient experienced resolution of her symptoms and of her vulvar lesions. CONCLUSIONS: Primary LCH of the vulva is very rare. Its etiology and pathophysiology, as well as the most effective modes of therapy, remain elusive. We propose that thalidomide is a useful alternative for patients with this disease.- - - - - - - - - - ranking = 0.125keywords = tract (Clic here for more details about this article) |
6/30. Disseminated Langerhans cell histiocytosis in identical twins unresponsive to recombinant human alpha-interferon and total body irradiation.Monozygotic twin boys presented at 1 year of age with seborrheic skin rash, otorrhea, and hepatosplenomegaly. skin biopsy confirmed Langerhans cell histiocytosis. Treatment with conventional antineoplastic drugs and with calf thymus extract was ineffective. The disease remained refractory to recombinant human alpha-interferon and to low-dose total body irradiation, and the children died between 3 and 3 1/2 years of age.- - - - - - - - - - ranking = 0.125keywords = tract (Clic here for more details about this article) |
7/30. CD56/NCAM-positive langerhans cell sarcoma: a clinicopathologic study of 4 cases.This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive langerhans cell sarcoma (LCS). Three of the patients were elderly, between 59 and 62 years of age at presentation, and the other was 35 years old. The presenting symptoms included fever, bone pain, and weakness. The patients shared some clinical findings, such as multiorgan involvement of lymph nodes, skin, lung, bone marrow, and spleen. LCS carries a poor prognosis, and 3 of the patients died of the disease within several years of presentation despite multiagent chemotherapy and radiotherapy. Of special interest is that all of the cases showed CD56 expression on the tumor cells in addition to expression of CD1a, S100beta, and langerin, the presence of which suggests derivation from langerhans cells. For control, CD56 was also examined in 8 cases of Langerhans cell histiocytosis (LCH), a single-system unifocal or multifocal disease, and the results of staining of the tumor cells were negative. Our findings indicated that CD56 may be a clinically relevant biologic marker for predicting an intractable course of Langerhans cell neoplasms, although it is often difficult to draw a definite morphologically-based distinction between LCS and LCH.- - - - - - - - - - ranking = 0.125keywords = tract (Clic here for more details about this article) |
8/30. Primary langerhans cell histiocytosis of the vulva.Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 12 cases of primary vulvar LCH have previously been published. We describe an additional case of LCH in which the disease was confined to the vulva. A 64-year-old woman was admitted to our hospital with a nodular mass on her left labium minus, and complete surgical excision was performed. On histological and immunohistochemical examination, the tumor fulfilled the criteria of LCH. A metastatic workup did not reveal any evidence of disease beyond the vulva. The patient received local radiotherapy, and 22 months later she is in excellent condition without local recurrence or metastatic disease.- - - - - - - - - - ranking = 0.125keywords = tract (Clic here for more details about this article) |
9/30. Anterior uveitis and iris nodules that are associated with Langerhans cell histiocytosis.PURPOSE: To describe a case of Langerhans cell histiocytosis (LCH) that involved the anterior uveal tract. DESIGN: Interventional case report. methods: A retrospective review was conducted on a patient with iris nodules and anterior uveitis in the setting of LCH. visual acuity and clinical findings that were noted on slit lamp biomicroscopy were extracted. RESULTS: An 18-year-old male patient with unilateral anterior segment inflammation and iris nodules experienced visual improvement from 20/200 to 20/25 after treatment with a 5-day course of topical corticosteroids. Regression of the iris nodules and anterior segment inflammation was also noted. bone marrow aspirate confirmed recurrent, active LCH. CONCLUSION: The clinician should include LCH in the differential diagnosis when faced with anterior segment inflammation in conjunction with iris nodules. Additionally, LCH can be treated successfully with topical corticosteroid therapy.- - - - - - - - - - ranking = 0.25keywords = tract (Clic here for more details about this article) |
10/30. liver transplantation in an adult with sclerosing cholangitis due to Langerhans cell histiocytosis.Sclerosing cholangitis due to Langerhans cell histiocytosis (LCH) is a rare cause of end-stage liver disease, seen mainly in children. Only a few adult cases have been reported worldwide. liver transplantation may be a viable treatment option for what is otherwise an irreversible condition. We describe a 65-year-old female with LCH who developed severe sclerosing cholangitis with jaundice, intractable pruritus and peritoneal disease. She underwent orthotopic liver transplantation with complete amelioration of symptoms and remained well 14 months following her operation. Explant histology confirmed LCH involvement with an associated extensive sclerosing cholangitis. Symptomatic LCH cholangiopathy is an emerging indication for liver transplantation in adults.- - - - - - - - - - ranking = 0.125keywords = tract (Clic here for more details about this article) |
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