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

Involvement of the axis by solitary eosinophilic granuloma is rare. We report a 12-year-old girl who presented with a unifocal eosinophilic granuloma of C2 that resulted in a pathologic fracture and atlanto-axial subluxation. She had no other skeletal or systemic manifestations of histiocytosis X. She was successfully treated with posterior spinal fusion and transoral excisional biopsy. Because solitary lesions may mimic malignant tumors, biopsy is advised in atypical cases. The prognosis for solitary eosinophilic granuloma is almost always favorable in terms of symptomatic improvement and restoration of vertebral height.
 ...
PMID:Atlanto-axial instability secondary to solitary eosinophilic granuloma of C2 in a 12-year-old girl. 213 58

Fifty-two patients with histologically proven histiocytosis X and intrathoracic and/or bone lesions have been evaluated. In the group, bone lesions appear clearly prevalent (51/52 patients) mainly as localized monostotic disease (31/51). Flat bones were more frequently involved, with special evidence for skull and ribs. Short as well as long bones were only seldom compromised, almost only in polyostotic and generalized disease. On the basis of our experience the most suggestive radiological findings in bone to diagnose an histiocytosis X were the following: preferential involvement of a flat bone, osteolytic pattern, sharp borders (72.9%), rare evidence of pathological fracture (20%) and periostitis (10%). On the contrary, the involvement of the neighbouring soft tissues (38.8%) appears to be significant.
 ...
PMID:[Histiocytosis X: radiologic evaluation of 52 patients]. 660 26

We report a case of Langerhans cell histiocytosis (histiocytosis X) in a 36-year-old female with an euthyroid nodular goitre as the rare initial presentation. She subsequently developed a pathological fracture of the lumbar spine which was initially thought to be due to malignant infiltration, possibly from metastatic thyroid carcinoma. Open vertebral biopsy and total thyroidectomy were performed. Histological features of histiocytic proliferation with erythrophagocytosis were found in both the thyroid gland and in the involved vertebral body. The diagnosis of histiocytosis X was made on the basis of the positive immunoperoxidase staining of the histiocytic cells with S-100 and peanut agglutinin (PNA) as well as the clinical picture. Despite systemic chemotherapy with low dose corticosteroids and vinblastine, diabetes insipidus with a suprasellar mass occurred three years post thyroidectomy. Successful management of this uncommon disease is difficult to conduct and evaluate at the present time.
 ...
PMID:Langerhans cell histiocytosis presenting as a goitre: a case report. 825 67

A 13-year-old Ethiopian female patient who initially presented with localized pain and minimal soft tissue swelling later developed pathological fracture of the left femnur. The patient was initially treated as a case of osteomyelitis until the diagnosis of Langerhans cell histiocytosis (histiocytosis X, eosiniphilic granuloma) was made by bone biopsy. The clinical presentation of the case and brief review of the literature is discussed.
 ...
PMID:Unifocal langerhans cell histiocytosis presenting with pathological fracture. 1764 63

The goal of therapy in Langerhans cell histiocytosis (LCH) is to decrease the activity and proliferation of histiocytes, lymphocytes and macrophages that cause the disease. Patients with disease that is localized to skin, bone and lymph node (defined as "nonrisk" organs) generally have a good prognosis and require minimal treatment. However, patients with lesions in "risk" organs (liver, spleen, lung, bone marrow) have a worse overall prognosis regarding mortality and morbidity. Likewise, patients with LCH in the central nervous system (CNS), vertebrae, facial bones or bones of the anterior or middle cranial fossa are at higher risk for morbidity and recurrent disease. LCH in the orbit, mastoid or temporal skull regions are classified as "CNS risk" because of an increased frequency of developing diabetes insipidus and other endocrine abnormalities or parenchymal brain lesions. Outcomes of patients with LCH in only one bone in "nonrisk" locations are generally benign, and the disease responds well to several treatment modalities including observation, surgical excision, steroid injection or radiation therapy. The last is generally reserved for a single vertebral lesion or when a risk of pathologic fracture exists in the greater trochanter. The greatest challenge in treatment of LCH is patients with multisystem disease. Patients with persistent or worsening disease in risk organs by the end of the first 6-12 weeks of therapy have significantly decreased overall survival regardless of treatment. Additionally, optimal treatment for patients with late-onset CNS symptoms and adults with LCH remain to be defined. In this article we will review the evolution of multicenter and international treatment studies as well as the current Histiocyte Society research treatment protocol, LCH-III. We also review experiences with a variety of agents that have been used to treat LCH outside of clinical trials. Since LCH is a rare disease in children and adults, these patients should be enrolled on clinical trials whenever possible to advance our knowledge of the optimal therapeutic interventions and long-term outcomes.
 ...
PMID:Langerhans cell histiocytosis: a review of past, current and future therapies. 1794 Jun 39

Eosinophilic granuloma is the most common expression of Langerhans cell histiocytosis and corresponds with typical bone lesions. This disease is of importance to dental professionals because early clinical signs can occur in the jaw and can cause extensive destruction of the periodontal tissues. Pathological fracture is an unusual finding. The purpose of this case report is to describe a case of eosinophilic granuloma in a 28-year-old black man who initially appeared with intense pain from a pathological mandibular fracture.
 ...
PMID:Eosinophilic granuloma in the jaws. 1794 36

Growth of benign tumours and tumour-like lesions of bone results in osteolysis which may cause pathological fracture. Bisphosphonates are anti-osteolytic agents which have proved effective in the treatment of number of osteolytic conditions. In this study we report the results of treatment with the aminobisphosphonate, pamidronate, of three benign osteolytic tumours of bone, two cases of fibrous dysplasia and one of Langerhans cell histiocytosis. In all three cases there was clinical and radiological improvement following treatment. Radiologically, bone lesions did not exhibit progressive enlargement. Two cases of fibrous dysplasia also showed features suggestive of increased bone formation. These findings indicate that bisphosphonates are likely to be useful in controlling the osteolysis of benign tumours/tumour-like lesions of bone, particularly in those cases where surgical intervention is not possible or multifocal lesions are present.
 ...
PMID:Bisphosphonate treatment of benign multifocal and unifocal osteolytic tumours of bone. 1852 67

Localized Langerhans cell histiocytosis (LLCH), formerly known as eosinophilic granuloma, mainly affects the skull, mandible, vertebrae, and ribs in children and the long bones of adults. Symptoms range from none to pain, swelling, and tenderness over the site of the lesion. General malaise and fever occasionally are present. Radiographically, lesions appear as radiolucent areas with well demarcated borders. LLCH may resolve spontaneously after biopsy in a period of months to years. However, if features include continuous pain, decrease of function, pathologic fractures, migration and resorption of teeth, or rapid progression, then active treatment needs to be considered. Treatment approaches include surgery, radiotherapy, chemotherapy, and intralesional injection of corticosteroids. In children with mandibular LLCH, 1 dose of methyprednisolone succinate injection has proven to be adequate. However, injections have not been performed in cases involving pathologic fracture. We report a new case of LLCH of the mandible that caused a pathologic fracture in an adult patient. Repeated intralesional corticosteroid injections resulted in fracture line disappearance within 14 months and lesion healing by the end of the 36-month follow-up.
 ...
PMID:Treatment of localized Langerhans' cell histiocytosis of the mandible with intralesional steroid injection: report of a case. 2045 43

Eosinophilic granuloma is the most common expression of Langerhans cell histiocytosis and corresponds with typical bone lesions. Early clinical signs can occur in the mandible and can cause extensive destruction of the periodontal tissues. Pathologic fracture is an unusual finding. A case of misdiagnosed eosinophilic granuloma in a 45-year-old man treated with free fibula flap and implant-supported overdenture prosthesis is reported. Free fibula flap with dental implants is a safe and reliable method for comprehensive functional and aesthetic mandibular defect reconstruction.
 ...
PMID:Misdiagnosed widespread eosinophilic granuloma of the mandible. 2280 Nov 84

Langerhans Cell Histiocytosis (LCH) is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient's lesion was treated by curettage, bone grafting, and internal fixation, due to the presence of pathologic fracture. At the 2-year followup, the patient was asymptomatic, and the lesion showed complete resolution without recurrence. The case report highlights the characteristic features of Langerhans Cell Histiocytosis in an unusual location, the knowledge of which would help avoid delayed or missed diagnosis in the future.
...
PMID:Langerhans cell histiocytosis of the clavicle in a 13-year-old boy. 2451 64


1 2 Next >>