[Eosinophilic granuloma of the mandible--a case report]. / Eosinophiles Granulom des Unterkiefers. Fallbericht.

BACKGROUND: Langerhans cell histiocytosis is characterized by a clonal proliferation of Langerhans cells. The clinical manifestation varies from a localized lesion (eosinophilic granuloma) to a systemic disease. The diagnosis can only be confirmed histopathologically. A comprehensive staging is necessary to determine the extent of the disease and to establish an adequate therapy. CASE REPORT: We report on a 27 years old patient who was referred to our clinic with the diagnosis of an osteomyelitis of the mandibular angle and a pathological fracture after extraction of tooth 38 one month before. Curettage and primary bone grafting were performed. In the histological examination of the specimen infiltrates of a Langerhans cell histiocytosis were found. The clinical and radiological staging demonstrated a solitary mandibular lesion (eosinophilic granuloma). After wound healing a low-dose radiotherapy with 6 Gray was performed. Two years after completion of the therapy the patient is asymptomatic and does not show any evidence of recurrence. CONCLUSION: Langerhans cell histiocytosis has to be included in the differential diagnosis of osteolytic lesions of the mandible. A low-dose radiotherapy is a reasonable and well-tolerated treatment option.

[Multifocal Langerhans cell histiocytosis of bone: late revelation in a 76-year-old woman]. / Histiocytose langerhansienne osseuse multifocale: révélation tardive chez une femme de 76 ans.

Langerhans cell histiocytosis or histiocytosis X has a variable course from a self-limited eosinophilic granuloma to an aggressive disseminated disease. It mainly affects children. We report a 76-year-old woman with multifocal bone histiocytosis X, involving the rachis, an iliac bone and the skull. The diagnosis has been established by histological exam. Outcome was favourable after chemotherapy.

Primary solitary eosinophilic granuloma of the brain and postoperative radiotherapy: a clinical case.

A case of solitary eosinophilic granuloma (EG) of the brain in a 53-year-old woman is presented. The tumor developed in the anterior cranial fossa causing destruction of the skull basis and the retrobulbar space, accompanied by vision impairment. The tumor was partially excised and the patient was irradiated postoperatively with 40 Gy 6 MV x-rays, followed by 10 cycles of chemotherapy. Follow-up computed tomography (CT) showed no response at 30 Gy and no progression during chemotherapy. Attention is drawn to cases of EG indolent in growth but resistant to radiation and chemotherapy.

[Unifocal eosinophilic granuloma of the temporal bone]. / Granuloma eosinófilo unifocal de hueso temporal.

We present a case of a twelve year old child with a eosinophilic granuloma of the temporal bone. The eosinophilic granuloma is the most frecuent and most benign form of the histiocytosis of the Langerhans cells. The frecuency of the othological manifestations of this condition varies between 15-60 percent and radiologically, the images are characterized by litho-lesions with sharp edges. The diagnosis is histological and the treatment includes surgical intervention accompanied by inter-lesion corticoid-therapy and/or radiotherapy.

Eosinophilic granuloma of the sacrum treated with radiation therapy: a case report.

BACKGROUND CONTEXT: Eosinophilic granulomas (EGs) of the sacrum have been reported in fewer than 10 patients. Treatment algorithms for these tumors remain poorly defined; there are no reports of treating solitary sacral EG with radiation therapy (RT). PURPOSE: This study aimed to describe the presentation, treatment, and outcome of sacral EG in an adult patient with intractable pain and radiculopathy, treated in a novel fashion with RT. STUDY DESIGN/SETTING: The study design was a case report from a tertiary cancer referral center. METHODS: Patient records, imaging, and pathology were reviewed. RESULTS: A 35-year-old man received 20 Gy of radiation to his S1 EG lesion. He subsequently developed vertebra plana of S1 causing symptomatic L5-S1 stenosis, but 15 months after RT treatment was free of pain or tumor recurrence. CONCLUSION: Radiation therapy is an effective treatment option for sacral EG causing severe axial pain and neural impingement.

Radicular compression in multifocal eosinophilic granuloma. Successful treatment with radiotherapy.

A 7-year-old boy with previously diagnosed multifocal eosinophilic granuloma displayed signs and symptoms of a cervical radiculopathy. After myelograpic demonstration of extradural compression, corticosteroid and radiation therapy produced rapid and complete resolution of his symptoms. We suggest that more discrete classification of the "xanthomatoses" of the neurologic literature may provide the basis for choosing nonsurgical intervention in selected cases.

Adverse effects of pregnancy on multifocal eosinophilic granuloma.

An unusual case of a patient diagnosed as having the histiocytic proliferative disorder characterized by the lesion eosinophilic granuloma (histiocytosis X) is presented. Fifteen-year follow-up revealed a progression from a solitary eosinophilic granuloma of the bone to multifocal involvement of many body structures including the vulva, vagina, and cervix. This progression occurred after nine asymptomatic years and was temporally related to the patient's first pregnancy. After successful treatment and remission, rapid appearance and growth of observable genital lesions were noted during a second pregnancy. Pregnancy termination in the first trimester was advised due to signs of dissemination and local symptoms.

Eosinophilic granuloma. Presence of OKT6-positive cells and good response to intralesional steroid.

A patient with eosinophilic granuloma, with extensive oral mucous membrane involvement, was treated with intralesional triamcinolone acetonide injections. Although radiation therapy had failed, this treatment resulted in remission. The biopsy specimen from the patient's lesion contained a predominance of OKT6-positive cells, which were shown by electron microscopic analysis to be mostly indeterminate cells and a small percentage of Langerhans' cells, with characteristic L-cell granules; there was also 5% eosinophils. Many L-cell granules were being produced between cell membranes of the same as well as neighboring L cells. It was thought that in this condition, as in other proliferative conditions of L cells, cell membranes are extremely adhesive and that such adhesion areas of L cells form L-cell granules. This occurs in a similar fashion as the adhesive areas of epithelial cells form desmosomes.

Solitary eosinophilic granuloma in the frontal lobe: case report.

A rare case of a solitary eosinophilic granuloma in the brain is reported. The mass, located in the right frontal lobe, mimicked a glioma not only grossly, but also by neuroimaging. The lesion was confirmed to be an eosinophilic granuloma by electron microscopy and immunohistochemical staining for S-100 protein and HLA-DR.

Focal eosinophilic granuloma of the temporal lobe. Case report.

The authors describe the clinical, pathological, and therapeutic aspects of a case of focal temporal lobe eosinophilic granuloma, presenting with otitis media and meningitis, and evolving subsequently into a temporal lobe mass. This triad, otitis media, meningitis, focal cerebral lesion with systemic manifestations of inflammation, eosinophilia of blood and cerebrospinal fluid, should suggest eosinophilic granuloma in the differential diagnosis. The lesion is sensitive to relatively small doses of radiation.

Percutaneous vertebroplasty with acrylic cement in the treatment of a Langerhans cell vertebral histiocytosis.

A 25-year-old man developed multiple eosinophilic granuloma of bone including vertebral and sacral localization. Radiotherapy was initially administered. One year later, a relapse occurred in another vertebrae which was previously irradiated. Percutaneous vertebroplasty was, for the first time to our knowledge performed, in this indication, with a good clinical result with follow-up now for one year. We emphasize that such treatment is permissible only in symptomatic, progressive lesions, with threatened decompensation of spinal stability. This technique should be used only on an adult.

Neoplasms of the shoulder girdle.

The author has briefly discussed radiation therapy, immunotherapy, and chemotherapy in the treatment of some of the more commonly encountered disease entities affecting the shoulder girdle. Specific operations and their indications are discussed. However, utilizing physical and x-ray examination, arteriography, lymphangiography, and bone scan information, the surgeon may decide that the routine operation is not indicated for a particular tumor problem. He should be able to be flexible and to modify the operation according to the specific presenting problem and not be bound by routine fixed rules. In this way the patient can be assured of the very best treatment.

Positive 'fistula sign' with intact ear-drum in a patient with eosinophil granuloma of the mastoid.

A case is presented of a positive 'fistula sign' in a patient with an intact ear-drum and no history of previous ear-disease. The cause proved to be an eosinophil granuloma of the temporal bone which responded well to radiotherapy.

Eosinophilic granuloma of the orbit: a paradox of aggressive destruction responsive to minimal intervention.

PURPOSE: To describe the findings and outcomes in eosinophilic granuloma (unifocal Langerhans cell histiocytosis [LCH]) of the orbit, and to explain the paradox of aggressive bone destruction responsive to minimal intervention. METHODS: Retrospective, consecutive, interventional case series of patients treated from 1985 through 2001. Minimum inclusion criteria were demonstration of CD1a positivity or Birbeck granules, treatment by a single surgeon, systemic evaluation by a pediatric oncologist, and follow-up of 12 months. A pathogenetic construct was assembled from general LCH concepts and the specific orbital findings. RESULTS: Seven patients met study criteria. All were male, 2 to 16 years of age. All had eyelid or forehead swelling and osteolytic defects, with symptoms of 2 to 6 weeks' duration. All underwent incisional biopsy, with frozen-section examination suggestive of LCH in 6 of 7 cases. The 2 earliest patients received low-dose irradiation after simple biopsy. The 5 most recent patients had subtotal curettage at the time of biopsy; 4 of 5 received simultaneous intralesional corticosteroid injection. In all cases, systemic evaluation showed no other focus of LCH, reossification was timely, and no local recurrence or additional focus was noted in follow-up of 1 to 17 years. CONCLUSIONS: Transient immune dysfunction may provoke the cytokine-mediated proliferation of pathologic Langerhans cells within the hematopoietic marrow of the anterolateral frontal bone. These cells cause osteolysis through elaboration of interleukin-1 and prostaglandin E2. Corticosteroids can inhibit the mediators. We recommend incisional biopsy, frozen-section provisional diagnosis, subtotal curettage, intralesional corticosteroid instillation, postoperative systemic evaluation, and long-term follow-up.

Multifocal eosinophilic granuloma--a case report.

A 1.5 year old Kuwaiti child male presented with a massive bilateral ulcerative lesion of palate and gingiva with massive submandibular lymphadenopathy which were proven histologically to be due to an eosinophilic granuloma. Bone survey was normal. The patient was treated by chemotherapy and irradiation locally with dramatic response and no relapse occurred in more than 18 months of follow up.

[Radiotherapy in Langerhans-cell histiocytosis. 2 case reports and review of the literature]. / Strahlentherapie bei der Langerhanszell-Histiozytose. Zwei Einzelfallberichte--Literaturübersicht.

The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. We present the results of two female adults with eosinophilic granuloma of bone who underwent conventionally fractionated radiation therapy with total doses of 7 x 1.8 Gy and 7 x 2.0 Gy in four different sites. After observation periods raging from three months to six years local control of the disease was achieved in all treated locations. A review of 18 previously published studies include a total of 310 sites of eosinophilic granuloma of bone in 216 patients. It was demonstrated in 13 studies that the patients had complete relief of symptoms. An average of 94.3% had local control of the symptoms. Furthermore, in 12 studies for a total of 344 cases with involvement of other organs local control was reported in an average of 64.8% (range: 14.3-100%). Based on our own observations and on the literature review we conclude that low dose radiation therapy plays an important role in the management of localised Langerhans' cell histiocytosis. In order to minimise the risk of radiation induced neoplasms an accurate and precise radiation technique is required.

[Histiocytosis X (Langerhans cell histiocytosis) of temporal bone. Role of scintigraphy in disease progress evaluation]. / Histiocytosis X (Langerhans cell histiocytosis) kosci skroniowej. Znaczenie scyntygrafii w ocenie zaawansowania choroby.

The authors present a case report of a 7-year-old girl with Langerhans cell histiocytosis of temporal bone. Edema and bone destruction involved zygomatic process of temporal bone and temporal fossa without ear occupation. The role of picturing diagnosis in evaluation of lesion extent with special importance of scintigraphy is discussed. Treatment methods of Langerhans cell histiocytosis lesions in temporal bone are presented. In our case locally Depo-Medrol injection was given and radiation was left as last resort management.

[Oral histiocytosis X with severe upper maxillary destruction]. / Histiocitosis X oral con severa destrucción del maxilar superior.

Histiocytosis X is a group of disorders of the reticuloendothelial system that have different clinical features and common histological characteristics, including abnormal proliferation of Langerhans cells. Eosinophilic granuloma is a localized form of histiocytosis affecting one or more bones. A case report is made of a patient diagnosed as histiocytosis X whose first manifestation was the appearance of an orosinusal communication. The lesions were located in the maxillary bones and produced large osteolytic lesions. No other bones were affected. In spite of surgery and radiotherapy, the disease recurred at 12 months.

[Clinical problems of radiotherapy in pediatric oncology]. / Klinicheskie problemy radiatsionnoi detskoi onkologii.

The main principles of radiation therapy as well as the role of ratio Integral Dose/Total Dose in radiation treatment of malignant tumors in children are considered. Indications for radiation therapy of nephroblastomas and peculiarities of radiation treatment of haemangiomas are described. Expedience is proved of radiation of only the liver part affected by neuroblastoma. Tactics of radiation therapy of eosinophilic granuloma of bones in analyzed. Possibility of conservative therapy including radiation component in treatment of retinoblastoma in children is discussed.