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.

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.

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.

Cone Beam CT study of a case of eosinophilic granuloma of the mandible in a young patient.

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.

Eosinophilic Granuloma of the Cervical Spine in Adults: A Review.

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.

Multidisciplinary rehabilitation of eosinophilic granuloma with bone graft surgery and a modified implant-supported hybrid prosthesis: A case report with a 6-year follow-up.

Eosinophilic granuloma is the most common form of Langerhans cell histiocytosis and corresponds with bone lesions characterized by pain, rapid growth, and high tendency of recurrence after inadequate curettage. It is a rare disease that is difficult to diagnose clinically and radiographically because it mimics other odontogenic cysts and tumors. In this report, the reconstruction of an osseous defect with an iliac graft and a modified implant-supported hybrid prosthesis after surgical excision of an eosinophilic granuloma in the mandible of a 27-year-old male patient was described. The patient was satisfied with the functional and esthetic results of the implant-supported restoration and a 6-year follow-up showed no sign of recurrence.

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.

Mixed histiocytosis: A case report and published work review.

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.

Right ethmoid eosinophilic angiocentric fibrosis with orbital extension.

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.

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.

Reconstruction of the C-1 lateral mass with a titanium expandable cage after resection of eosinophilic granuloma in an adult patient.

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.

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.

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.

A Novel Surgical Approach to Coracoid-based Eosinophilic Granuloma and the Technical Trick.

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.

Short Segment Stabilization by Protecting the Alar Ligaments in a Case of Eosinophilic Granuloma Involving the C2 Spine.

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.

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.

Multifocal Langerhans Cell Histiocytosis in an Adult.

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.