Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature.

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.

Langerhans cell histiocytosis (eosinophilic granuloma) of the skull mimicking nummular headache. Report of two cases.

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.

Spontaneous and complete regeneration of a vertebra plana after surgical curettage of an eosinophilic granuloma.

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.

Eosinophilic Granuloma of Skull with Fluid Level and Epidural Hematoma: A Case Report and Review of the Literature.

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.

Pandora's box: eosinophilic granuloma at the cerebellopontine angle-should we open it?

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.

The surgical strategy for eosinophilic granuloma of the pediatric cervical spine complicated with neurologic deficit and/or spinal instability.

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.

Traumatic ulcerative granuloma with stromal eosinophilia around mini dental implants without the protection of a denture base.

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.

Ependymosarcoma with eosinophilic granular cells.

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.

Eosinophilic granuloma of the sternum in a child treated with closed biopsy.

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.

[Surgical treatment combined with oral administration of indomethacin for eosinophilic granuloma of the skull: report of a pediatric case].

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.

Eosinophilic granuloma of the clavicle in an 11-year-old Chinese girl: A case report.

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.

Operative case of Langerhans'cell histiocytosis of the skull with dural invasion. An immunohystochemical study of ki-67 expression of eosinophilic granuloma: case report and review of the literature.

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.

Misdiagnosed widespread eosinophilic granuloma of the mandible.

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.

Eosinophilic granuloma of the temporal bone in an adult: controversies in the management.

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 of the Liver Mimicking Metastatic Liver Tumor.

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: resolution of lesion after biopsy.

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.

Traumatic ulcerative granuloma with stromal eosinophilia. A case report and short literature review.

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.

Eosinophilic granuloma of bilateral humerus with unusual diagnostic process.

A 15-year-old boy presented with a right shoulder pain and the inability to use his right upper limb after carrying a heavy object. Physical examination and local imaging revealed a benign tumor or tumor-like lesion in the upper ends of both humeri and a pathologic fracture in the right humerus. An initial biopsy was reported as fibroblastic osteosarcoma. But the CT-guided biopsy was diagnosed as eosinophilic granuloma (EG). Bone curretage and grafting confirmed diagnosis of EG. This case illustrates the importance of combining a clinical impression with imaging and pathology in bone and soft tissue tumors.