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Introduction: Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited., Methods: Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG., Results: In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination., Conclusion: Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Rationale: Eosinophilic granuloma (EG) - the most common form of Langerhans cell histiocytosis - occurs rarely, and manifestations with only rib and clavicle involvement are extremely rare. EG symptoms often include pain, swelling, and soft tissue mass. The clinical diagnosis of bone EG is complex, and the differential diagnosis includes Ewing sarcoma, tuberculosis, multiple myeloma, lymphoma, primary bone malignancy, and other osteolytic lesions., Patients Concern: The patient was an 11-year-old female who found a subcutaneous mass at the junction of the right clavicle and sternum 2 days before presenting at the clinic without apparent triggers. Initially, we considered a subcutaneous cyst or inflammatory mass. Color ultrasound and computed tomography examination revealed osteomyelitis. Finally, the patient was diagnosed with EG after a pathological tissue biopsy, and the child recovered after surgery and anti-infective treatment., Diagnosis: The patient underwent surgery to remove the tumor at a specialist hospital and was diagnosed with EG by pathological examination., Intervention: The patient went to a specialist hospital for surgery to remove the mass and underwent anti-infective treatment., Outcomes: The patient recovered after surgical resection and antibiotic treatment., Lessons: In this report, we emphasize that the clinical presentation of EG in children is not specific. Furthermore, examining age, history, presence of symptoms, and the number of sites is essential to make a correct diagnosis, and a histological examination is necessary to confirm the diagnosis., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

We herein report a case of coagulation necrosis with granulation and eosinophilic infiltration of the liver. A 37-year-old woman was diagnosed with a new mass lesion in the liver 1 month after breast cancer surgery and admitted for a further examination. Because the tumor occurred immediately after surgery, it was considered essential to determine whether or not it was a metastatic liver tumor from breast cancer. A percutaneous liver tumor biopsy revealed eosinophilic granuloma of the liver, which is considered to have a high possibility of visceral larva migrans with suspected gnathostomiasis infection. A detailed medical history and histological diagnosis are important for making a differential diagnosis.

Eosinophilic granuloma (EG) is a rare bony disease deriving from abnormal proliferation of histiocytes, and is the most common form of presentation of Langerhans cell histiocytosis. EG predominantly affects the axial skeleton. However, when localised in the head and neck district, mandibular lesions account for the majority of cases. Mandibular lesions can mimic other pathological conditions, making biopsy fundamental for differential diagnosis. Treatment depends on the severity of the disease, ranging from pharmacological treatment to surgical approach. However, EG is also reported to possibly undergo spontaneous resolution. In this case report, we describe a rare case of EG with particularly aggressive behaviour in a young patient. Initially, local pharmacological treatment with intralesional administration of corticosteroids lead to worsening of the symptoms, increase in lesion's dimensions, and disruption of the cortical bone. A more invasive therapeutic approach involving radical surgery was then performed, with consequent resolution of the disease., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Spinal eosinophilic granulomas (EGs) are uncommon tumors, constituting <1% of all bone tumors. They are mostly seen in the pediatric age group, whereas adult onset is rare. The cervical spine is an infrequent location for EG. The literature is sparse regarding the clinical and management aspects of these lesions, especially in adults., Methods: A literature review was performed by searching online databases to analyze all the reported cases of adult-onset cervical EG., Results: Sixty-two cases were identified and analyzed. The male/female ratio was 3.1:1. The mean age at presentation was 32.8 years (range, 18-71 years). Neck pain, limb weakness, and restriction of movement were the most frequent symptoms. The C2 vertebra was the most frequently involved. The vertebral body was involved in >80% of patients and only 2 had vertebra plana morphology. The cervical lesions involved single vertebra in 42 patients (82%), whereas 9 patients (18%) had multiple vertebral involvement. Fifteen patients were managed conservatively, of whom 7 failed and required surgery later; 40 patients underwent definitive surgery and 17 received adjuvant treatment. Posterior-only surgeries were performed in 5 patients, anterior-only in 20, and circumferential in 14. The mean follow-up duration was 68 months (range, 12-268 months). The outcome was good to excellent in all, except 1, irrespective of type of treatment received., Conclusions: Cervical spine EG is rare in adults and usually presents as an osteolytic lesion involving the vertebral body; vertebra plana is very rare. Surgery is typically reserved for patients with severe neurologic deficits and bony instability. The outcome seems good in most cases., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Histiocytoses are a group of heterogeneous diseases that encompass Langerhans cell histiocytosis and non-Langerhans cell histiocytosis. Cutaneous plane xanthoma is a non-Langerhans cell histiocytic disorder characterized by the presence of yellow-orange plaques on the face, neck, upper trunk and extremities. It can appear in association with several systemic diseases (including dyslipidemias, paraproteinemias, cardiovascular diseases and lymphoproliferative disorders), but is rarely connected with Langerhans cell histiocytoses. Eosinophilic granuloma is one of the clinical entities of Langerhans cell histiocytoses, characterized by skeletal lesions and occurring prominently in children. Mixed histiocytosis, the concomitant occurrence of Langerhans cell histiocytosis and non-Langerhans cell histiocytosis in a single patient, is exceptional. We herein report a case of eosinophilic granuloma in an adult Chinese man who also developed plane xanthoma on his scalp and face, and we also include a published work review of the comorbid cases of eosinophilic granuloma and non-Langerhans cell histiocytosis. To the best of our knowledge, this is the first report on the mixed histiocytosis of cutaneous plane xanthoma and eosinophilic granuloma in China., (© 2017 Japanese Dermatological Association.)

Background Nummular headache is a rare, recently described topographic headache defined by the circumscribed coin-shaped area of pain. It is classified as a primary headache. There is debate about whether it is due to a peripheral or central disturbance, and its relationship to migraine. Case reports We report two patients with presumed nummular headache secondary to Langerhans cell histiocytosis, both with resolution of their headaches after surgical resection. Conclusion Imaging in patients with clinical features of nummular headache is recommended, as this and other cases highlight that it may be symptomatic. There are no distinguishing clinical features to separate nummular headache from secondary mimics, and treatment of the underlying cause may be curative.

Competing Interests: There are no conflicts of interest

Introduction: Eosinophilic angiocentric fibrosis (EAF) is a slowly progressive, benign disease involving the mucosa of the upper airways or, more rarely, the orbit. It belongs to the spectrum of IgG4-related disease., Case Report: The authors report the case of a 61-year-old man who presented with orbital involvement (visual loss, pain, proptosis, and eyelid oedema), headache and nasal obstruction. Imaging revealed a right ethmoido-orbital mass infiltrating the periorbital fat and enveloping the optic nerve. Histological examination concluded on a diagnosis of EAF in the presence of perivascular infiltration by inflammatory cells, predominantly eosinophils, and zones of "onion skin" fibrosis. Immunohistochemistry attributed these lesions to IgG4-related disease. Initial treatment with corticosteroids followed by dapsone failed to control the disease and resulted in severe steroid dependence. Surgical ethmoidectomy with resection of the lamina papyracea was performed to allow displacement of the eyeball into the nasal cavity in the event of another episode., Discussion: The combination of surgery and rituximab achieved lasting pain relief with no recurrence of exophthalmos., (Copyright © 2017. Published by Elsevier Masson SAS.)

Purpose: The eosinophilic granuloma is a unifocal or multifocal Langerhans cell histiocytosis characterized by an expanding proliferation of Langerhans cells in bones. Skeletal LCH is a rare condition, and vertebral regeneration in cases of vertebral body collapse is even rarer. We report the case of a girl with spontaneous complete healing., Methods Ad Results: A 3-year-old girl was referred for nighttime back pain, with no fever and no neurologic signs. Within a few days, she developed sudden painful restriction of all spine movements. X-ray and computed tomography (CT) of the spine showed reduced T7 vertebral body height (vertebra plana).The patient underwent T7 curettage and the histopathological exam was suggestive of LCH. Two additional skull lesions were found and therefore she underwent chemotherapy. After 7 years of follow-up, total vertebral reconstruction was observed., Conclusions: Despite the rarity of the condition and despite the rarity of vertebral body lesion resolution, total vertebral body reconstruction was observed over a 7-year period. Long-term follow-up is necessary for a better understanding of the final outcome of patients with EG.

Spinal involvement occurs frequently in cases of eosinophilic granuloma (EG), but surgical treatment is limited primarily to those with spinal instability. Involvement of the cervical spine is rare, but primarily occurs in the vertebral bodies, and is normally amenable to anterior corpectomy and spinal reconstruction. The authors describe a 27-year-old man with pathologically proven EG who presented with complete destruction of the C-1 lateral mass requiring spinal stabilization. A titanium expandable cage was used to reconstruct the weight-bearing column from the occipital condyle to the superior articular surface of C-2 from a posterior approach, with preservation of the traversing vertebral artery. To the authors' knowledge, this is the first reported instance of reconstruction of the C-1 lateral mass using an expandable metal cage, which facilitated preservation of the vertebral artery.

Intracranial Langerhans cell histiocytosis commonly presents as skull lesions in children. An intratumoral cyst with fluid level and epidural hematoma occurring with eosinophilic granuloma is very rare. We report a 15-year-old boy who presented with a spontaneous epidural hematoma which was the result of a temporal eosinophilic granuloma. Multiple explanations for epidural hematoma in such cases have been discussed. Intratumoral hemorrhage followed by cyst formation and rupture may explain the pathophysiology of epidural hemorrhage formation., (© 2016 S. Karger AG, Basel.)

Background: Various therapeutic approaches have been proposed for the treatment of pediatric patients with eosinophilic granuloma (EG) of the cervical spine. Our aim was to discuss and present our experience with the individualized surgical intervention of pediatric cervical EG complicated with neurologic deficits and/or spinal instability., Methods: We retrospectively analyzed the clinical data of 19 children who were diagnosed with cervical EG comor spinal/or spinal instability (evaluated by the Spinal Instability Neoplastic Score, SINS ≥ 7) and treated surgically in our institution., Results: Lesions involved C1-2 in 7 patients and C3-7 in 12 patients. Anterior tumor resection combined with posterior pedicle screw fixation, anterior approach of excision and instrumentation, and posterior tumor resection combined with pedicle screws instrumentation were selected according to the different locations of tumors. Frankel scale and Oucher scale improved significantly after surgery. There was no morphologic alteration of the neck at follow-up., Conclusions: Surgery can significantly improve the neurologic status and symptoms. Surgical decision-making must be individually tailored to minimize the influence of surgery on spine growth.

The coracoid base is a very rare location for tumors. It is difficult to diagnose and approach, but easy to dismiss. In this case, the tumor (eosinophilic granuloma of the scapula) was located at the base of the coracoid, and the posterior cortex was eroded by the tumor. Accessing this lesion through the deltopectoral approach with coracoid osteotomy without penetrating the supposed tumor extending posterior soft tissue will be discussed. This approach gives a wider and safer access to the surgeon than the posterior approach. This report presents a customized solution with the deltopectoral approach, and the chevron-type osteotomy to access the coracoid base and tension band wiring to fix the osteotomy side.

Background: Langerhans cell histiocytosis (LCH) is a disorder of immature LCH cells, eosinophils, macrophages, lymphocytes, and multinucleated giant cells. Eosinophilic granuloma (EG) is a focal form of LCH that presents mostly in the skull, femur, vertebrae, pelvis, mandible, and ribs. Intracranial presentation of EG is very rare in the literature., Case Description: A 17-year-old boy visited our clinic with headache, dizziness, and tinnitus that were present for 2 months. Brain MRI depicted a lesion at the right cerebellopontine angle. The lesion was hypointense on T1-weighted and hyperintense on T2-weighted brain MR images. The lesion enhanced homogenously after I.V. contrast material administration. Pre-operative diagnoses were vestibular schwannoma and meningioma. Surgery was planned. Retrosigmoid approach was preferred in the surgery. The lesion was excised partially. Pathological analysis depicted cell infiltration composed of eosinophils besides histiocytes, plasma cells, and lymphocytes in different amounts. CD1a was positive yet S100 was negative. Final diagnosis was eosinophilic granuloma. Post-operative course was uneventful. The patient was referred to pediatric oncology unit, and steroid therapy was initiated. Post-operative follow-up brain MRIs showed that the lesion had regressed further than immediate post-operative images by only steroid use. In long-term follow-up, new lesions appeared on the patient's skin in multiple locations and in the sclera of his left eye. At the last follow-up (3 years post-operatively) skin and scleral lesions were noticed to have regressed spontaneously and the intracranial structures were tumor free., Discussion and Conclusion: To the best of our knowledge, EG at the cerebellopontine angle has not been presented in the literature. What makes our case further unique is its negativity for S-100 antigen. Eosinophilic granuloma should be kept in differential diagnosis of mass lesions presented at the cerebellopontine angle, especially in children and young adults with high eosinophils and lymphocytes in their peripheral blood. Sole steroid trials could be conveyed in suspicious cases before any further intervention. If the lesions do not regress or enlarge with time, surgery should be considered. However, long-term follow-up of these patients is necessary since natural history of the disease has not been defined, yet.

Background: The craniocervical junction is a complex anatomic location that contains the occipital bone, atlas, axis, and important complex ligamentous structures. The stability of this region is ensured only with the help of ligaments., Case Description: A 6-year-old boy was admitted to our clinic for neck pain. Computed tomography and magnetic resonance imaging revealed a lytic bone lesion involving the C2 vertebral body and pedicle without odontoid tip. The tumor was resected using an anterior retropharyngeal approach and a wide marginal resection method. The odontoid tip and alar ligaments were protected, and the costal autografts were located between the C1-odontoid tip and the C3 body. The costal graft was stabilized in the C3 body with a miniplate. Then, C1-C3 posterior fixation with fusion was performed. The craniocervical junction was not considered unstable because the occipital bone was not involved in the fusion. Histologic examination confirmed the diagnosis of eosinophilic granuloma. Fusion was detected on a 1-year postoperative cervical computed tomography scan., Conclusions: The occiput should not be involved in the fusion area when the alar ligaments are preserved during surgery for a C2 lesion., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Eosinophilic granuloma (EG) is the most common and benign form of the spectrum of disorders referred to as Langerhans cell histiocytosis (LCH). Langerhans cell histiocytosis is primarily regarded as a pediatric disease, with few adult cases of multifocal EG of bone reported. We report a case of multifocal EG in a 48-year-old woman, who presented with right knee pain. Radiographs showed a small lytic lesion in the medial femoral condyle. Diagnosis was confirmed by ultrasound-guided biopsy. She had had a previous EG lesion excised from her skull. Whole-body bone scan demonstrated a new skull lesion in the right diploic space, which was confirmed by magnetic resonance imaging. The patient underwent curettage, bone grafting, and prophylactic internal fixation of the right distal femur lesion. The skull lesion was treated with repeat craniectomy. Two years later, she developed a new lesion in the right distal femoral metaphysis, which was treated with intralesional corticosteroid injections. Now, more than 1 year later, the patient is pain-free with no evidence of new or recurrent disease. Because multifocal EG is a rare diagnosis in adults, appropriate clinical suspicion, in combination with radiographic findings and histologic examination, is essential for correct diagnosis and treatment.

Background Context: Benign tumors of the vertebrae are generally an uncommon cause for surgery. Complete removal of these tumors requires in most cases extensive surgical technique that consists of generous surgical exposure followed by laminectomy, facetectomy, and sometimes even an instrumented fusion., Purpose: The aim was to describe our experience in performing resection of benign vertebral tumors, using a minimally invasive surgical (MIS) approach., Study Design: This was a retrospective review of case records., Patient Sample: Patients who underwent MIS, resection of benign vertebral tumors., Outcome Measures: Complete neurologic examination and pain evaluation, as measured by the visual analog scale (VAS). Secondary outcomes included postoperative spinal instability assessment and surgical margins examinations., Methods: Patients were evaluated preoperatively and postoperatively at 1, 3, and 6 months intervals clinically and radiographically using plain radiographs and postoperative computed tomography (CT) scans. Final pathologic report, operative time, blood loss, complications, and hospital length of stay were also recorded., Results: Between 2009 and 2013, 14 patients underwent MIS, resection of benign vertebral tumors at our institution. Mean follow-up time was 4 years. There were eight men and six women with a mean age of 27 years (range 16-68 years). For tumors located in the posterior elements, a direct posterior approach was used. Tumors located at the pedicle of the vertebra were excised using a transpedicular approach, and tumors protruding into the foramen were excised using the transforaminal approach. The transcanal approach was used when decompression of the thecal sac or nerve root was required, and the retroperitoneal transpsoas approach was used for tumors located in the vertebral body. Complete removal of these tumors was achieved in all cases, and was verified by a follow-up CT scan. Pathology revealed osteoid osteoma in five patients, osteoblastoma in three patients. Eosinophilic granuloma, fibrous dysplasia, and fibroid adenoma were found in one case each. Average VAS pain score improved from 7.7 (7-9) to 2.8 (0-7) after surgery., Conclusions: Minimally invasive techniques are a valuable choice for the treatment of benign osseous tumors of the spine. A larger, long-term study is in progress. In the meantime, we suggest surgeons experienced with both open and MIS surgery should consider these techniques., (Copyright © 2015 Elsevier Inc. All rights reserved.)

A 43-year-old Japanese woman visited for a hepatic tumor incidentally found. We suspected eosinophilic granuloma of the liver (EGL) due to visceral larva migrans (VLM). However, neither past history nor medical interview indicated a risk of parasitosis. Blood testing revealed eosinophilia, serum examination showed normal results for immunoglobulin E, and enzyme-linked immunosorbent assay yielded negative for Toxocara and Anisakis. Gastric and colonic endoscopy revealed normal features. Several imagings showed central necrosis of the tumor. After informed consent, laparoscopic resection was performed. Histopathological examination showed EGL without parasites. No recurrence had occurred postoperatively. Most reports documented that EGL are caused by VLM. However, parasites are not always demonstrable on serum, histopathological, or immunochemical examinations. When acting as allergens to induce type I responses, microscopic agents other than parasites in the intestinal tract could induce eosinophilic inflammation in the liver. Accumulation of more cases should help clarify other pathogeneses for EGL.

Objective: To explore the clinical efficacy and complications of treating sternal tumors by resection and titanium mesh thoracic reconstruction., Methods: This retrospective analysis of eight patients with sternal tumors treated in the Department of Orthopedic Surgery at the First Affiliated Hospital of Zhengzhou University from January 2008 to June 2012 included five men and three women aged 37-66 years (mean, 50.4 years). The histological diagnoses were chondrosarcoma (two cases), osteosarcoma (one), malignant fibrous histiocytoma (two), eosinophilic granuloma (one) and sternal metastasis from breast cancer (two). The tumors were invading the manubrium sterni (three cases), manubrium sterni and body (three) and sternal body (two). All patients underwent needle or incisional biopsy prior to sternal tumor resection and titanium mesh thoracic reconstruction., Results: All patients were followed for 9 months to 4 years. There were no intraoperative complications or operative or postoperative deaths. One patient developed a deep wound hematoma 1 week postoperatively; incisional drainage and debridement resulting in healing within 2 weeks. There was no loosening or exsertion of the titanium mesh and no patients developed respiratory complications or thoracic deformity. One patient with malignant fibrous histiocytoma died of lung metastases 9 months postoperatively, another with malignant fibrous histiocytoma died of liver metastases 14 months postoperatively; the remaining patients survived without tumor recurrence., Conclusion: Titanium mesh chest reconstruction after sternal tumor resection has the advantages of simplifying the procedure, achieving a good shape and having few complications. Titanium mesh is an ideal material for reconstruction of the sternum., (© 2015 Chinese Orthopaedic Association and Wiley Publishing Asia Pty Ltd.)

This is a report of a case of an unusual oral lesion after the placement of mini implants for the retention of a mandibular overdenture. A patient received four 2-mm-wide dental implants in the anterior mandible and had her mandibular denture relined with a soft material. After 3 months, she was not wearing her mandibular denture, and two nodular ulcerated lesions were observed near the mini implants. The lesions ceased following excision and regular denture wearing. Clinical and microscopic examination led to the diagnosis of traumatic ulcerative granuloma with stromal eosinophilia (TUGSE). TUGSE is rare lesion with a benign course that may occur following injury of the oral mucosa by mini implants under certain circumstances., (© 2014 by the American College of Prosthodontists.)

Stroke in children is relatively uncommon. We describe an 8-year-old boy diagnosed with primary eosinophilic granuloma (EG) of the frontal bone. After excision of the EG, the postoperative course was eventful. The patient had an acute right middle cerebral artery (MCA) infarct and had been comatose with a diminished Glasgow Coma Scale (GCS) score of 5. Urgent decompressive hemicraniectomy with duraplasty was performed. The postoperative course after the second operation was uneventful. Hematological tests revealed a diagnosis of factor V Leiden. The patient was discharged with left hemiparesis and GCS of 15. To the best of our knowledge, no such clinical picture of MCA infarction after EG excision has been described before. Neurosurgeons should be aware of inherited thrombophilias, such as factor V Leiden, if the postoperative clinical course worsens because of cerebral artery thrombosis. Also, decompressive hemicraniectomy could be life saving and should be performed urgently without any hesitation.

Background Context: Eosinophilic granuloma (EG) involving the spine is uncommon in adults. Atlantoaxial instability (AAI) secondary to EG of the axis in adults is an extremely rare clinical condition that can give rise to severe neurologic morbidity or mortality if not treated appropriately. There have been no previous reports on the condition in adults., Purpose: To present the outcome and clinical experience for the management of AAI secondary to EG of the axis in adults., Study Design: A retrospective review study., Patient Sample: All adult patients with AAI secondary to EG of the axis who were admitted to the spine service at the study institution between January 1999 and April 2012., Outcome Measures: Clinical symptoms, neurologic status, radiologic manifestations, treatment, outcome, and/or complications were recorded and analyzed., Methods: Six consecutive adults who presented clinical and radiographic manifestations of AAI secondary to EG of axis were treated and monitored. All patients were treated surgically with anterior tumor resection and posterior reconstruction of spinal stability. Oral steroid therapy was administered after surgery as adjuvant therapy., Results: The mean duration of follow-up was 77 months (range, 37-140 months). The most common radiographic feature was osteolytic destruction of the vertebral body of the axis. All patients had favorable recoveries, with osseous fusion. There were no surgery-related postoperative complications, and neither recurrence nor spinal deformity had occurred by the final follow-up examination., Conclusions: Surgical intervention via anterior tumor resection and posterior reconstruction was found to be safe and effective for treating AAI secondary to EG in adults, in terms of recovering neurologic function, improving symptom relief, and reducing the risks resulting from osteolytic destruction. Surgical treatment plus oral steroid therapy can produce beneficial results and definitive local control during the follow-up period., (Copyright © 2014 Elsevier Inc. All rights reserved.)

A 13-year-old girl presented headache for 5 d upon admission to hospital. An initial CT revealed 3 lesions located in her skull, the sizes of which were 2.5 cm×3.2 cm,1.2 cm×1.0 cm,0.3 cm×0.3 cm, respectively. The largest lesion was resected by surgery and confirmed as eosinophilic granuloma by pathology. After surgery, she took oral indomethacin 25 mg b·i·d for 3 months and tolerated it well. CT scan was performed 3 months and 1 year later, and the results showed that the unresected lesions shrank progressively and the defected bones were regenerated and healed one year later after operation.

Langerhans cell histiocytosis is a rare neoplastic proliferative disorder of the Langerhans cells. The clinical course is variable, ranging from a low symptomatic single bone lesion to fatal multiple organ involvement. Rarely, the sternum can be the first and single location of the disease. We report on a 12-year-old boy who presented with an aggressive lytic lesion of the proximal sternum associated with local pain and afternoon fever. Histopathological analysis of the closed biopsy specimen indicated eosinophilic granuloma of bone/Langerhans cell histiocytosis. Soon after the biopsy procedure the pain and fever subsided. Computed tomography at 2 months showed healing of the lytic lesion. The patient received no other type of treatment. At 2 year follow up he was symptom and disease free., (© 2014 The Authors. Pediatrics International © 2014 Japan Pediatric Society.)

Eosinophilic granuloma (EG) is a rare histiocytic disorder resulting from clonal proliferation of Langerhans cells. It accounts for less than 1% of all osseous neoplasms and has a predilection for involving the axial skeleton. Although suspicion of the disease may arise from clinical features and radiographic demonstration of destructive bone lesions, it is still difficult to make a correct diagnosis without proper pathological evaluation. This is more evident when common differentials mimicking EG, both clinically and radiologically, need to be ruled out. This report describes a case of unifocal EG of the mandible occurring in a 4-year-old boy whose initial presentation led to confusion between osteomyelitis, primary bone tumour and lymphoma. A final diagnosis of EG was established after histopathological examination of the biopsy specimen.

Ependymosarcoma is a new entity of malignant gliomas composed of ependymal and sarcomatous components. Were port a rare case of ependymosarcoma with eosinophlic cells which occurred to the right trigon of the lateral ventricle.A 62-year-old man complained of headaches over a 2-month period. A hard, gray mass was found in the right trigon of the lateral ventricle during the operation.Although he received radiation and chemotherapy, the patient died due to tumor disseminating through the whole brain within 7 months after the operation. The histological examination revealed that the anaplastic glial components intermingled with the sarcomatous components. Immunohistochemically, sarcomatous cells were positive for α smooth muscle actin and desmin. However, anaplasticglial cells were not positive for these markers. In addition, Masson trichrome stain showed a plethora of collagen fibers between sarcomatous cells, but no collagen fibers were produced by the glial tumor cells. Solid focal papillary lesions of the glial tumor showed dot-like epithelial membrane antigen and diffuse cytoplasmic D2-40 immunoreactivity. Based on the above findings, these anaplastic glial tumor cells should show focal ependymal differentiation, and sarcomatous cells show myofibroblastic differentiation. In addition, almost 10%of the tumor cells in the neoplasm showed bright eosinophilic granules in the cytoplasm. These cytoplasmic eosinophilic granules and bundles were negative on PAS staining. Intracytoplasmic eosinophilic granules of tumor cells were strongly positive for αB-crystallin, HSP 27 and GFAP, respectively. These findings suggest that the clinicopathological characteristics of the present case should be consistent with the criterion of ependymosarcoma by Rodriguez et al.

Object: Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up., Methods: The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010., Results: Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2-18 years). Chordomas received proton or LINAC irradiation, and as of 4-15 years of follow-up, no recurrence has been noted., Conclusions: Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.

Background: Eosinophilic angiocentric fibrosis is a chronic, idiopathic disorder that usually involves the upper respiratory tract and features progressive submucosal perivascular fibrosis of unknown etiology. To our knowledge, only 5 cases of eosinophilic angiocentric fibrosis with primary orbital involvement have been reported., Methods and Results: We report the case of a 46-year-old man with right proptosis and lateral globe displacement caused by a primary eosinophilic angiocentric fibrosis extending from the orbit into the anterior ethmoid. The nasal extension of the lesion helped in establishing the correct diagnosis., Conclusion: Physicians involved in the treatment of orbital pathologies should be familiar with this entity, because it may manifest as an intraorbital mass growing primarily or secondly into the orbit. The clinical manifestations of eosinophilic angiocentric fibrosis with orbital involvement often mimic other more common ophthalmological diseases. Biopsies are necessary for diagnosis and treatment planning, although cures are usually of palliative effect., (Copyright © 2013 Wiley Periodicals, Inc.)

Eosinophilic granuloma is most common in children. In this paper we describe two children with a history of local swelling and pain in the humeral area who showed pathological fracture of the humerus. Needle biopsies confirmed the diagnosis of eosinophilic granuloma. Surgical procedures were performed in both patients. Both patients showed total remission after wide resection combined with segmental nonvascularized fibular graft and elastic nail. Both patients are currently free of disease after 4-year follow-up. There are several treatment modalities in eosinophilic granulomas such as radiotherapy, chemotherapy, local or systemic steroids, curettage, bone grafting and internal fixation. Although good results have been reported with nonsurgical treatment, surgery is a more effective treatment option in selected cases. In this paper we describe two children with massive solitary eosinophilic granuloma of the humerus who were successfully treated with segment resection and fibular bloc graft.

Object: Solitary eosinophilic granuloma (EG) of the calvaria is most commonly treated with surgical excision. The authors hypothesize that many solitary EGs will resolve without intervention, and observation may be a reasonable option. This study was undertaken to investigate that hypothesis., Methods: The authors reviewed their institutional records and identified 14 cases of solitary calvarial EG. In 6 cases the patients underwent resection based on family and/or neurosurgeon preferences. A strategy of nonoperative management (purposeful observation) was chosen for the other 8 cases. The authors report the clinical course and imaging results in these 8 cases., Results: One of the 8 patients underwent surgery 2 months after presentation because of slight enlargement of the lesion and increasing pain. After a median follow-up period of 1 year (range 6-19 months), none of the other patients had required surgery. Five of these 7 patients had pain at presentation. Pain resolved completely in all 5. The remaining 2 remained asymptomatic. Complete resolution of pain was reported in the 5 patients who had pain at presentation. There was complete clinical resolution of the palpable soft-tissue lesion in all 7 cases. Complete radiographic resolution of the lesion was observed in 5 cases and near-complete resolution in the remaining 2., Conclusions: Observation is a safe and reasonable approach in the management of solitary calvarial EG and may prevent unnecessary surgical interventions.

The authors report a 12-mo-old girl with calcified cerebral cryptococcal granuloma. She was admitted with a 6-mo history of seizures. Laboratory examinations showed no abnormal findings. Electroencephalography revealed bilateral slow wave activity, greater in the right occipital region. CT showed an irregular calcified focus with small surrounding low density in the right parieto-occipital region. MRI demonstrated mixed signals without edema and visible flow-voids. The clinical symptoms mimicked intracranial vascular malformations. The diagnosis of cerebral cryptococcal granuloma was made by histopathology. Partial resection of the lesion with post-operatively antifungal and anticonvulsant therapy offered the satisfactory result. Cerebral cryptococcal granuloma is extremely rare, especially in infants. Calcification is indeed unusual. Cerebral cryptococcal granuloma should be included in the differential diagnosis of intracranial mass with calcification in infants.

Eosinophilic granuloma is a rare disease belonging to the Langerhans' cell histiocytosis group. It accounts for only 1% of all tumor-like lesions of bone. A 6-year-old girl presented with proptosis, and a mass and pain in her right eye. Cranial computerized tomography and magnetic resonance imaging revealed a mass in her right orbital area. The patient was operated through a right frontotemporal craniotomy and orbitozygomatic osteotomy. The tumour was totally removed. The postoperative course was uneventful. Histopathological examination revealed a diagnosis of eosinophilic granuloma with typical Langerhans cells and eosinophylic leucocyte infiltration. We report an orbital eosinophilic granuloma case, which is rarely seen, and discuss its clinical and pathological features.

Eosinophilic granuloma is a rare, benign, lesion characterized by uncontrolled proliferation of Langerhan's cells. It is commonly found in a bone and has been described in almost every bone in the body. It usually affects children and is seldom found in adults. A case of 60-year-old adult male with an eosinophilic granuloma of the right temporal bone with infiltration of the temporal and infratemporal fossa and osteolysis of the squama of the temporal bone is presented. Diagnostic procedure and course of treatment are described. Controversies in the management and different approaches in therapy of such lesion are discussed because there is no agreed treatment protocol established.

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.

The patient was a 16-year-old girl complaining about progressive swelling on the right side of her face, right headache, and right orbital proptosis. A CT scan revealed a lytic mass centered in the greater wing of the right sphenoid bone with extension into the orbit, middle cranial fossa, and infratemporal fossa. The patient underwent a subtemporal-preauricular infratemporal fossa approach, which allowed complete resection of the tumor, with a low morbidity. The pathological diagnosis was eosinophilic granuloma. At 36 months after the initial treatment the patient is free of disease. We conclude that lesions of this complex anatomic area are a challenge to biopsy and treat and they could be treated only with radical surgery with low morbidity, at centers with expertise in skull base surgery.

Eosinophilic granuloma (EG), Letterer-Siwe disease and Hand-Schuller-Christian disease are collectively called Langherans-cell histiocytosis (LCH). While the latter two are systemic diseases, the former is a localized form of histiocytosis. Solitary EG of the skull are rare lesions characterized by a natural history not well defined yet. In this context, we report a case of a 23-year-old male suffering for a recurrent and progressive right parietal headache. On computed tomography (CT) it was observed an ostelytic lesion which on magnetic resonance imaging (MRI) appeared as an hyperintense soft mass on both T1 and T2 weighted images. The lesion showed a marked and heterogeneous enhancement after gadolinium administration. The surgical excision was complete and the severe headache disappeared. Immunohistochemical analysis of the specimen indicated an eosinophilic granuloma characterized by Ki-67 nuclear antigen expression with a labeling index of 20%. In the pertinent literature we have found two aggressive cases of EG showing the Ki-67 expression with a respectively 6.2% (occipital bone granuloma) and 10% (parietal bone granuloma) labeling index. That high proliferative activity suggests a local Langherans' cell proliferation along with an exuberant inflammatory response and also explains the aggressive clinical course and the rapid expansion of the lesion observed in some rare cases of solitary EG. This is the third case-report of calvarial EG characterized by Ki-67 nuclear antigen expression.

Langerhans cell histiocytosis is a collective term used to describe a group of enigmatic proliferative disorders. Three disease variants include eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian syndrome. Eosinophilic granuloma is reported to be the most benign type of the triad. In this report, a 7-year-old child with an eosinophilic granuloma of the left side of her mandible is presented. There was a painless, hard, tender mass arising from the left side of the molar region. Panoramic radiographs showed a radiolucent area at the molar region. The lesion was resolved 2 weeks after the biopsy. The 1.5 years of radiographic and clinical follow-up was satisfactory.

We introduce a case of 53-year-old female with rapidly developing tongue ulceration clinically mimicking squamous cell carcinoma of the oral mucosa. After a microscopic examination traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) was diagnosed. In a short literature review, we characterize this entity, analyse its aetiology and nature. Differential diagnosis is also discussed.

Langerhans cell histiocytosis (LCH) comprises a group of disorders, the common feature of which is Langerhans cell proliferation. The clinical presentation is highly varied. The severity and prognosis of the disease are dependent on the type and extent of organ involvement. This paper reports a rare case of a four-month-old white male with unifocal LCH limited exclusively to the mandible, discussing the diagnosis, radiographic and immunohistochemical aspects, treatment and monitoring multidisciplinary of the case., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

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.