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Objective: To investigate the operative procedure and the effectiveness of eosinophilic granuloma (EG) of long bones in children., Methods: Between January 2005 and December 2009, 14 patients with EG of long bones were treated. There were 9 boys and 5 girls, aged from 1 to 13 years (mean, 6.5 years). The locations were femur in 5 cases, humerus in 4 cases, tibia in 2 cases, fibula in 1 case, and femur complicated with tibia in 2 cases. The disease duration was 7 days to 10 months (median, 2 months). X-ray films showed that osteolytic destruction had clear boundary, which did not involve the epiphyseal plate. Of 14 cases, 12 cases of tumor were treated by curettage, autologous iliac bone or combined artificial bone graft repair, and 2 cases were treated by resection, autologous iliac reconstruction, plate and screw fixation. Five cases complicated with pathological fracture underwent reduction and fixation., Results: All cases were diagnosed pathologically as having EG. All incisions healed by first intention. A total of 12 patients were followed up 1 to 4 years (mean, 2 years). The X-ray films showed tumor focus and pathological fracture healed within 3 to 4 months (mean, 3.5 months). Tibial lesion was found in 1 case of femoral tumor after 8 months, and was cured after reoperation. No recurrence occurred in other 11 cases. According to comprehensive assessing standard of X-ray film and joint function, the results of all cases were excellent., Conclusion: EG of long bones in children is more common in the femur and humerus. Tumor curettage and autologous iliac bone graft repair is an effective method, and postoperative prognosis is good. There may be multiple lesions, so long-term follow-up is needed.

Eosinophilic granuloma occurs almost exclusively in the diaphysis or metaphysis, when tubular bones are affected. The investigators present an extremely rare case of eosinophilic granuloma arising at the epiphysis of the femoral head in an 8-year-old boy. Plain radiographs and computed tomography showed a well-circumscribed radiolucent lesion, suggesting chondroblastoma or Brodie's abscess. However, the findings on magnetic resonance images were different from typical features of chondroblastoma or Brodie's abscess. The lesion was curetted. Histological diagnosis was eosinophilic granuloma. Differential diagnoses of a radiolucent lesion at the epiphysis in a child should include, though quite rare, eosinophilic granuloma.

Unlabelled: BACKROUNDS: Eosinophilic granuloma is one of the rarest causes of bone tumors, especially in adults. Eosinophilic granuloma is the commonest form of Langerhans cell histiocytosis and represents the unifocal osseous form of the disease which usually affects the skull and long bones. Eosinophilic granuloma, is a benign disease in which diagnosis and differential diagnosis presents more difficulties than treatment., Observation: We present a case of eosinophilic granuloma of the rib with long term follow-up of 14 years which was treated with a combination of surgery and chemotherapy., Conclusion: Prognosis of adult eosinophilic granuloma is excellent and the recurrence rate is limited. All available treatment options, including surgery, chemotherapy, corticosteroids, radiation, and even palliative treatment have very good results and in many cases the disease seems to heal spontaneously. However the disease, due to its rarity and unknown pathogenesis still remains an enigma for the clinical doctor.

Objective: To explore the imaging diagnosis of pelvic eosinophilic granuloma and improve diagnosis accuracy., Methods: A retrospective analysis of the plain film, CT and MRI was conducted in 24 cases of pathologically confirmed eosinophilic granuloma. All the patients received radiography, CT scan was performed in 17 cases and MRI was done 9 cases., Results: In the 24 cases of eosinophilic granuloma, 6 lesions were located in the pubis, 5 in the ischium and 13 in the iliac region. The lesion showed bony destruction or irregular bony destruction, with periosteal reaction and soft tissue mass., Conclusion: Pelvic eosinophilic granuloma has characteristic imaging features, and combination of the imaging findings can improve the diagnosing accuracy.

Langerhans cell histiocytosis is a disease of unknown aetiology which may be isolated or affect multiple organs and which frequently affects the head and neck, with cranial compromise being one of the most common manifestations in children over five years. We present the case of a three year old girl with no otologic history who came to our hospital with a clinic of subperiostic abscess, subsequently diagnosed as eosinophilic granuloma. We describe the treatment and clinical evolution of the case., (Copyright 2009 Elsevier España, S.L. All rights reserved.)

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 is the localized form of Langerhans' cell histiocytosis. There are several reports of magnetic resonance (MR) imaging of eosinophilic granuloma of the skull, however there are few reports about dural enhancement. We report an operative case of eosinophilic granuloma of the skull with dural invasion. A 42-year old man was admitted to our hospital. He was neurologically intact and there were no other osseous or soft tissue lesions. CT showed an osteolytic lesion in the left parietal bone. MR images showed the lesion as isointense on T1-weighted, and high intense on T2-weighted images. T1-weighted images with Gd-DTPA demonstrated the mass which was enhanced with dural surface and subgaleal tissue. The angiogram demonstrated a tumor stain fed by the left occipital artery. Bone scintigraphy demonstrated a solitary lesion showing peripheral uptake with a central defect. The patient underwent craniectomy with removal of the dura and a subgaleal lesion. Histological examination revealed characteristic eosinophilic granuloma with dural invasion. No recurrence of the lesion was demonstrated 6 months after surgery.

Background: The authors describe 9 cases of children with eosinophilic granuloma (EG) of the skull and report on their clinical manifestations, treatment, and prognosis., Method: Nine consecutive patients were diagnosed as EG of the skull and confirmed pathologically between 1996 and 2005. In the present study, multi- and single-system Langerhans' cell histiocytosis without skull involvement were excluded. Patients with EG of the skull were divided into two groups: (1) those with only a single bone lesion and those with (2) multiple bone lesions. Surgical removal was performed between 2 and 10 years of age (mean, 4.2 years)., Results: Eight (88.9%) of the study subjects were found to have a single bone lesion at diagnosis, and 1 had multiple bone lesions. Seven patients had a painless skull mass and 2 patients had a painful skull mass. Total removal was performed in all 9 patients. Eight patients received postoperative chemotherapy or indomethacin as adjuvant therapy. Of the 8 patients who received adjuvant therapy, 4 were treated with indomethacin and the remaining 4 received methotrexate-based chemotherapy. Eight patients did not experience EG recurrence, however, 1 patient developed additional lesions 2 years after surgical excision., Conclusions: EG of the skull is a clinicopathological entity with a good outcome. However, therapies and prognoses are dependent on age at diagnosis and the number of bony involvements.

Objectives: Langerhans cell histiocytosis is a proliferating cell disease that may take various forms characterised by bone, skin lymph nodes and visceral lesions. Eosinophilic granuloma is a localised form of histiocytosis X, or Langerhans' cells histiocytosis, a benign form with unknown aetiology. At the head and neck level, it is usually located on the temporal, and usually occur in association with multifocal disease; however, isolated lesions may occur in the mastoid bone alone., Methods: The author's present a case of a patient with unifocal eosinophilic granuloma limited to the mastoid treated in ENT and radiotherapy departments., Results: CT scan demonstrated an osteolytic lesion of the mastoid. The biopsy and immunohistochemical study confirmed the diagnosis. A surgery followed by a radiotherapy (20 Gy) have stabilised the situation. There is no recurrency after one year., Conclusion: Unifocal eosinophilic granuloma of the temporal bone is a benign lesion of langerhans' cell histiocytosis. Its diagnosis is difficult when the disease remains isolated. Its treatment depend on its extension and its risks. The excellent prognosis does not dispense on regulary follow up.

We reviewed the imaging of four pathologically proven calvarial eosinophil granulomas. The diameter of the lesions ranged from 13 to 40 mm; three were biconvex, but the other had a collar-stud appearance. Two lesions were in the frontal and two in the parietal bone. On bone-window CT, a bevelled edge was seen in three cases and button sequestration in one, but no sclerotic rim was shown. Although one lesion had a low-density area, the lesions were slightly denser than grey matter. They were isointense with grey or white matter on T1-weighted MRI and gave heterogeneous high signal on proton-density and T2-weighted images. All enhanced markedly, with a less strongly enhancing portion within them. A tail of dural enhancement and reactive change in the overlying galea or temporal muscle were seen in all cases.

We report a case of multifocal-monosystemic Langerhans cell histiocytosis (LCH), formerly usually referred to as eosinophilic granuloma (EG) of bone. The condition developed in a 36-year-old man. A notable infrequent thoracic spine location and two successive distinct costal lesions were observed. Both the first costal site and the vertebral location healed spontaneously; the second costal lesion underwent biopsy resection. The patient's disease course with an 8-year follow-up is discussed with reference to various treatment options, emphasising in selected cases a watchful conservative approach, in view of the widely documented potential for spontaneous healing.

A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion.

The imaging studies of nine histologically proven eosinophilic granulomas were reviewed. Radiographs and MRI studies were performed on all patients, with eight patients being examined by bone scintigraphy and six by CT. Matrix calcification, not evident on radiography, was demonstrated in two cases by CT. MRI proved superior to both the radiographs and CT in defining the medullary extent of the lesion and the surrounding soft tissue changes. In eight of nine cases, on STIR sequences, an endosteal rim of low signal intensity surrounding the main lesion was present and may be an early feature of healing. The degree of peritumoral oedema accompanying eosinophilic granuloma was less extensive than that seen in either Ewing's sarcoma or osteomyelitis. The presence of both a low signal endosteal rim and limited peritumoural oedema on STIR sequences may be a useful indicator to the diagnosis of underlying eosinophilic granuloma.

We report the radiological findings and more specifically the MRI features in three typical cases of Langerhans' cell histiocytosis of the head and neck. All three cases were of solitary eosinophilic granuloma of bone: two mandibular and one temporal bone lesion. Reports on the MRI features of head and neck eosinophilic granulomas are rare.

The findings in 11 patients with histologically proven eosinophilic granuloma (EG) examined by magnetic resonance imaging (MRI) are described. In contrast with the variable appearance of EG on conventional radiography and computed tomography (CT), relatively constant features--intermediate to high signal intensity on T1-weighting, high signal intensity on T2-weighting, marked enhancement--were found on MRI. MRI was superior to other imaging methods in demonstrating bone marrow involvement and any accompanying soft tissue mass or inflammation. Intermediate to high signal intensity on T1-weighting and marked contrast enhancement could not be "explained" by histological findings. Prediction of the evolutionary phase of EG by MRI remains questionable because of the phase I (proliferative) histology of all 11 lesions.

The appearance on magnetic resonance imaging (MRI) of 16 cases of pathologically proven eosinophilic granuloma were reviewed retrospectively and correlated with the radiographic appearance of the lesion. The most common MR appearance (ten cases) was a focal lesion, surrounded by an extensive, ill-defined bone marrow and soft tissue reaction with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, considered to represent bone marrow and soft tissue edema (the flare phenomenon). The MRI manifestations of eosinophilic granuloma, especially during the early stages, are nonspecific, and may stimulate an aggressive lesion such as osteomyelitis or Ewings sarcoma, or other benign bone tumors such as osteoid osteoma or chondroblastoma.

After plain radiography has been performed, magnetic resonance (MR) imaging is considered the modality of choice for the evaluation of suspected musculoskeletal lesions because of its exquisite sensitivity to changes in the signal intensity of marrow and soft tissue. That sensitivity, however, may lead to an overestimation of the aggressiveness and extent of some benign bone lesions, particularly in children. Such lesions include chondroblastoma, osteoid osteoma, eosinophilic granuloma, and stress fractures. Potentially misleading MR features commonly seen include prominent marrow edema, soft-tissue edema, and apparent mass effect adjacent to the bone lesion. Features that these lesions have in common that may explain the MR findings include associated inflammatory reactions caused by the lesions and their occurrence in childhood, when the periosteum is more loosely attached. Knowledge of the potential pitfalls encountered with MR imaging may help explain the discrepancy between the radiographic and MR appearances of these benign lesions and avoid misplaced reliance on MR imaging for a diagnosis. Radiography remains the single most valuable modality in determining a differential diagnosis for bone lesions.

The case of a ten-year old girl, presenting with pain and restricted movement of the left arm is reviewed. X-rays and bone scan suggested an Ewing's sarcoma of the left humerus, so that the child was referred to surgery. Surprisingly, the histological examination of the surgical specimen revealed a solitary eosinophilic granuloma of the bone.

Fine-needle aspiration (FNA) cytology of three cases of Langerhans' cell histiocytosis (eosinophilic granuloma [EG]) of bone in children (mean age--8.3 yr; range 5-11 yr) is presented. Two patients presented with vertebral lesions and the third had a femoral mass. Cytomorphologic features of EG were seen in all cases including Langerhans' cell histiocytes having oval to reniform shape nuclei with nuclear grooving and abundant pale cytoplasm. The background showed a polymorphic population of cells including neutrophils, lymphocytes, foamy histiocytes, and osteoclasts. Moderate numbers of eosinophils were seen in two cases, while eosinophils were sparse in the third case. Ancillary immunocytochemical (ICC) studies performed on the aspirated material demonstrated positive staining for S-100 protein (all three cases) and T-6 antigen (one case). Ultrastructural examination (EM) performed in one case demonstrated characteristic Birbeck granules in the histiocytes. A specific cytologic diagnosis was made in all cases, enabling proper chemotherapy in one case, surgical excision in another and spontaneous resolution in the third case. Our experience demonstrates that FNA cytology can make a definitive diagnosis of EG, especially when coupled with ancillary studies such as ICC and EM on the aspirated material.

The authors describe current opinions about origin, pathology, histology, diagnosis and treatment of the eosinophilic granuloma. Clinical course, diagnosis and treatment of the own case were discussed.

T6-antigen-bearing Langerhans' cells were detected in a touch imprint of eosinophilic granuloma involving a thoracic vertebra of an 11-year-old boy. It is interesting to note that in this localized form of histiocytosis X, no T6-antigen-bearing Langerhans' cells were found in the peripheral blood. Immunofluorescence detection of T6 antigenicity has not been described in this disease and may serve as a rapid diagnostic test.

Destructive bone lesions in long bones associated with onion like periosteal reaction was found in two girls. Diagnosis of Ewing sarcoma was followed by bone biopsy which indicated eosinophilic granuloma. The radiological appearances of osteomyelitis, tuberculosis, congenital syphilis, Caffey's disease and metastases of neuroblastoma are similar. Bone biopsy is mandatory for correct diagnosis.

This is a review of 48 patients registered in the University of British Columbia Bone Tumour Registry as having eosinophilic granuloma, with emphasis on the historical development, treatment and results. Comments on the place of scintigraphic imaging are included. Eosinophilic granuloma in its solitary form is a self-limiting disease requiring no treatment. It can, however, progress to multifocal disease or the historically important triad known as Hand-Schüller-Christian syndrome. There is some evidence that it may have an immunologic basis, but the authors have little new to offer with respect to its etiology.

Eosinophilic granuloma is frequently confused with infectious diseases or neoplastic conditions of the temporal bone. It is one of a spectrum of diseases classically known as histiocytosis X. Eosinophilic granuloma usually takes a benign clinical course; however, progression of disease has frequently been documented in the pediatric age group. Our interest in eosinophilic granuloma was generated by a 2-year-old white boy who presented with a large postauricular mass, aural discharge, and posterior-canal-wall granulation tissue. We present this patient's clinical course and a review of the literature concerning diagnosis, treatment, and prognosis of eosinophilic granuloma of the temporal bone.

Eosinophilic granuloma of bone is a rare skeletal manifestation of one of the spectrum of diseases known as histiocytosis X. These lesions often simulate other pathophysiologic processes (Ewing sarcoma, chronic osteomyelitis, Brodie abscess, and chondroblastoma) and may present significant diagnostic problems. Three additional cases of eosinophilic granuloma in an epiphysis are reviewed in this report. Transphyseal extension was present in all our cases and in five of 10 cases documented in the literature. Diagnosis demands accurate biopsy and histopathologic evaluation. Treatment and prognosis are individualized.

A 26 year-old male presented with a spontaneous pathological fracture of the right femur caused by an osteolytic lesion. A chest X-ray demonstrated the coexistence of interstitial pulmonary fibrosis. Fibrocaseous tuberculosis and widespread malignancy were ruled out by appropriate investigations. The differential diagnosis, as based on the histological examination of the bone tumour, rested between hyperparathyroidism complicated by pulmonary disease and eosinophilic granuloma with lung manifestations. Hyperparathyroidism was ruled out by the absence of clinical and biochemical evidence of a disturbance of calcium metabolism. The diagnosis of histiocytosis-X was established by the histological appearance of the lung biopsy.

Sixteen children (average age 7.4 years) with nontraumatic lesions of the clavicle were reviewed. There were six cases of neoplasms, six cases of infection, and four developmental anomalies. The patients with infections all had pain at presentation, with elevated white blood cell counts and erythrocyte sedimentation rates. Tissue biopsy was an important diagnostic tool.

The value of nuclear magnetic resonance (NMR) tomography in the skeleton is analysed in 27 patients who had been examined by a prototype NMR scanner (resistive magnet, 0.2 Tesla). Anatomical structures and detail can be seen using the spin-echo mode and brief sequences. There is marked contrast difference between normal and abnormal tissues. Pathological changes lead to marked alterations in relaxation times. Inflammatory conditions, tumours and aseptic bone necrosis are demonstrated with a high degree of sensitivity. Tomography in the three geometric planes, with any desired additional plane, shows the extent and localisation of bone abnormalities.

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.

The authors review and study, especially from the anatomo-pathological aspect, the different tumours and pseudo-tumoural dysplasias of the orbital bones taken from the collection of the Laboratory of Ocular Pathological Anatomy of the Hotel-Dieu in Paris. They demonstrate the particular frequency of osteomas and fibrous dysplasias and discuss the orbital predisposition of giant cell tumours of bone. Finally they underline the existence of vascular bone tumours and aneurysmal cysts.