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.

[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.

Secondary malignant fibrous histiocytoma following refractory langerhans cell histiocytosis.

We describe a rare case of secondary malignant fibrous histiocytoma (MFH) following Langerhans cell histiocytosis (LCH). A 23-year-old Japanese male exhibited systemic lymphadenopathy, multiple lung tumors, and osteolytic changes in bilateral iliac bones in 1989. A biopsy specimen from the left iliac bone revealed an infiltration of S-100 protein-positive histiocyte-like cells intermingled with eosinophils, which confirmed the diagnosis of eosinophilic granuloma, a type of LCH. Although the patient was treated with prednisolone initially, the disease did not respond well and progressed gradually over time. The patient subsequently received multiple courses of chemotherapy and immunosuppressive therapy with many kinds of anticancer agents for 6 years. He also received radiotherapy totaling 136.8 Gy for lung tumors and osteolytic lesions of the pelvis. In 1997, because of the LCH refractoriness, biopsy was performed again from the right inguinal lymph node. Microscopic examinations demonstrated a mixture of spindle-shaped cells and histiocyte-like cells, which appeared to be in a storiform pattern. The tumor cells were immunohistologically positive for CD68 and vimentin, but negative for CD1a and S-100 protein. Therefore, the patient was diagnosed with MFH. Although chemotherapy was continued, the patient died of pneumonia during the neutropenic period following chemotherapy. Autopsy revealed systemic invasion of MFH and dissemination of mucormycosis. LCH was not detected histologically in any tissues.

[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.

Eosinophilic granuloma in the jaws.

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.

A case report of unifocal Langerhans' cell histiocytosis or eosinophilic granuloma.

A case of unifocal Langerhans' cell histiocytosis or eosinophilic granuloma in a child is reported where the frontal, zygomatic and maxillary bones of the left orbital wall are affected. As eosinophilic granuloma is a rare disease and the involvement of maxillary bone in orbital eosinophilic granuloma is not well documented in the literature, this case is reported for its unusual involvement of 3 bones at a single site. The diagnosis was established by clinical, radiological and histological findings. As no other system or site was involved and considering the osseous involvement of multiple bones at one site, local radiotherapy was preferred as the mode of treatment. The patient responded favourably to radiotherapy with reduction of proptosis and tumour mass but there was no visual recovery. In the absence of universal agreement over the mode of treatment, the result emphasises the probable benign nature of the tumour and the need to withhold more aggressive treatment modalities for extensive multisystem involvements.

[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.

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

Presentamos el caso de un niño de 12 años de edad con un granuloma eosinófilo del hueso temporal. El granuloma eosinófilo es la forma más benigna y frecuente de la histiocitosis de células Langerhans. La frecuencia de manifestaciones otológicas varía entre un 15-60% y radiológicamente las imágenes se caracterizan por lesiones líticas de bordes cortantes. El diagnóstico es necesariamente histológico y el tratamiento incluye resección quirúrgica acompañado de corticoterapia intralesional y/o radioterapia

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

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.

Solitary eosinophilic granuloma of the third metacarpal at pediatric age.

Langerhans cell histiocytosis involves disorders previously referred to as "histiocytosis X" (including eosinophilic granuloma of bone, Letterer-Siwe, and Hand-Schüller-Christian syndrome). Eosinophilic granuloma of the hand, especially if solitary, is extremely rare. This is the third pediatric case reported in the literature with solitary eosinophilic granuloma of the hand and the second with metacarpal involvement. The patient's lesion was curetted and the histologic examination showed eosinophilic granuloma. The patient received adjuvant radiotherapy because of continuing mild functional impairment and fracture risk. At 12-month follow-up near complete recovery of the lesion was observed. Radiologically, there was sclerosis and remodeling of the third metacarpal. The patient is disease-free at 26 months.

Patologías excepcionales en estructuras de cabeza y cuello / Exceptional pathologies in head and neck structures

De 4711 enfermos con lesiones patológicas de cabeza y cuello tratadas, 14 de ellas (0,29 por ciento) han sido, en nuestra experiencia, variedades únicas en sus respectivos órganos. Siete son presentadas en este artículo. (AU)

Patologías excepcionales en estructuras de cabeza y cuello / Exceptional pathologies in head and neck structures

De 4711 enfermos con lesiones patológicas de cabeza y cuello tratadas, 14 de ellas (0,29 por ciento) han sido, en nuestra experiencia, variedades únicas en sus respectivos órganos. Siete son presentadas en este artículo.

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.

[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.

[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.

[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.