Proliferation Of Langerhans Cells Research Articles

Langerhans Cell Histiocytosis in a 5-Year-Old Girl: A Case Report and Review of Literature

AbstractLangerhans cell histiocytosis (LCH) is an uncommon hematological disorder affecting infants and young children. LCH is a rare disorder of the reticuloendothelial system associated with proliferation of Langerhans cells and mature eosinophils. LCH can involve any bone, but the most common are pelvis, ribs, skull, long bones, vertebra, and facial bones. In the skull, frontal and parietal bones are commonly involved followed by the jaws, where mandible is more commonly affected than the maxilla. In this article, we report a case of LCH in a 5-year-old child involving the mandible. Swelling of one side of the face and aggressive periosteal reaction led to the diagnosis of monostotic LCH. The manuscript also summarizes the results of a literature search in PubMed of reported cases of LCH over the past 10 years.

High Prevalence of BRAF V600E Mutations in Langerhans Cell Histiocytosis of Head and Neck in Chinese Patients

Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation of Langerhans cells and has been classified as a hematolymphoid tumor. BRAF V600E mutation was found to be frequent in LCH; however, it has also been reported that Asia patients with LCH tend to show a lower rate of BRAF V600E mutation. In this study, we found LCH from the head and neck region often involved bone especially the posterior of the mandible and presented a high prevalence of BRAF V600E mutation in Chinese patients. Our findings also showed immunohistochemical detection correlated very well to DNA sequencing of BRAF alterations, which may be useful in the diagnosis of LCH, especially in cases with a low proportion of Langerhans cells, and BRAF inhibitors might be a treatment option for patients with LCH harboring BRAF V600E mutation.

CyclinD1 Is Useful to Differentiate Langerhans Cell Histiocytosis From Reactive Langerhans Cells.

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by clonal proliferation of neoplastic Langerhans cells (LCs). LC proliferation can also be seen in different reactive dermatosis. CyclinD1 is a downstream marker of mitogen-activated protein (MAP) kinase pathway, which is often activated in LCH. This study aimed to evaluate the role of cyclinD1 to differentiate reactive LC proliferation from LCH. All cases of cutaneous LCH diagnosed by biopsy in the past 3 years (n = 13) were immunostained with CD1a, p53, CD31, and cyclinD1. Seven cases each of discoid lupus erythematosus (DLE) and lichen planus (LP) were taken as control. Presence of p53, CD31, and cyclinD1-positive LCs (CD1a-positive) were compared in the dermis. In all LCH cases, dermal neoplastic LCs showed diffuse CD1a positivity and 12 cases (92.3%) showed variable (30%-70%) cyclinD1 expression. Weak p53 and CD31 expression were seen in 61.5% and 46.1% of LCH cases, respectively. In the control group, 5 cases of LP and 4 cases of DLE showed variable LC proliferation, highlighted by CD1a positivity. However, no case of reactive dermatosis showed cyclinD1 or p53 expression by the reactive LCs. Weak and patchy CD31 expression by the reactive LCs were found in 1 (25%) and 2 (40%) cases of DLE and LP, respectively. To conclude, cyclinD1 is frequently expressed in neoplastic LCs in LCH. It is an efficient marker to differentiate neoplastic from reactive LC proliferation, and can be used as a surrogate marker in LCH.

Langerhans cell histiocytosis with concurrent papillary thyroid microcarcinoma

Langerhans cell histiocytosis (LCH) is a rare disease, characterised by proliferation of Langerhans cells in various organs. It can present as localised or disseminated disease, with particular predilection for involvement of skin, bone, brain and lungs. Solitary thyroid gland involvement is exceptionally rare, with only a limited number of cases reported in the literature. Here we describe a case of a 45-year-old female with a known history of hypothyroidism, presenting with enlarged multinodular thyroid gland on clinical examination. Ultrasonography showed a 20 mm left lobe thyroid nodule in the lower pole, and pre-operative fine needle aspiration cytology assessment was classified as suspicious for malignancy (Bethesda category 5). The patient underwent left hemi-thyroidectomy with neck dissection. Microscopic examination revealed chronic lymphocytic thyroiditis with two foci of papillary thyroid microcarcinoma (7.5 mm and 0.6 mm) together with adjacent LCH spanning 3.5 mm. Although concomitant papillary thyroid carcinoma and LCH in thyroid is rare, it has recently been increasingly reported. This draws attention to possible association between the aetiology of both disorders, and necessitates thorough histological examination for concurrent papillary thyroid carcinoma and lymphocytic thyroiditis when diagnosis of LCH in thyroid gland is attained.

Cerebral Langerhans Cell Histiocytosis: A Case Report

Langerhans cells histiocytosis (LCH) is a disease caused by the proliferation and abnormal accumulation of Langerhans cells in different tissues and organs. It is a rare disease that still hides many of its mysteries as to its etiology and pathophysiology. The cerebral localization remains exceptional and dominated by hypothalamic-pituitary axis involvement. We report a case of a 20-year-old patient followed for acquired central diabetes insipidus with Panhypopituitarism and multiple dermatological lesions, who recently had visual acuity decline with progressive cerebellar syndrome in a state of apyrexia. Brain MRI showed multiples scattered plaques and nodular lesions involving the periventricular white matter, the basal ganglia, cerebellum, brainstem and cervical medulla. The two diagnoses suggested in this clinical presentation sarcoidosis with pituitary localization and cerebral Langerhans cells histiocytosis. A skin biopsy confirmed the diagnosis of langherans cell histiocytosis.

Langerhans cell histiocytosis in children: a case report and brief review of the literature

Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is a non-malignant disease involving clonal proliferation of Langerhans cells. It is an orphan disease affecting mainly the child and the young adult. Its etiology is still unknown, and its clinical spectrum is quite wide. Systemic treatment incorporating steroids and cytostatic drugs for at least one year has improved prognosis of multisystem LCH and represents the current standard of care. We report the case of a multifocal bone LCH in a 2-year-old girl affecting the frontal and parietal bones, associated with emphysematous bulla. The diagnosis was confirmed with histological examination. The evolution was favorable after treatment. Based on this observation, we will make a brief review of the literature to present the clinical, histological, radiological, therapeutic and evolutionary aspects of this orphan disease.

Histiocitosis de células de Langerhans de partes blandas y hueso con células gigantes multinucleadas y mutación BRAF V600E

Langerhans cell histiocytosis (LCH) is a heterogeneous disease characterized by proliferation of Langerhans cells and BRAF mutation in almost half of the cases. Bone involvement is common but large soft tissue disease is uncommon. We report a pediatric patient with a large tumor mass involving the left iliac bone and the adjacent soft tissue. The computed tomography scan showed an osteolytic lesion with soft tissue extension. Surgical curettage of the lesion was performed and the final histopathologic diagnosis was LCH with CD1a immunoreactivity in tumor cells. The molecular analysis revealed a BRAF V600E mutation. We discuss the histopathological and immunohistochemical differential diagnosis with histiocytosis other than LCH.

Lower Intestinal Langerhans Cell Histiocytosis Masquerading as Chronic Malabsorption Syndrome and Failure to Thrive in a Child: A Rare Case Presented with a Succinct Review of Recent Literature

AbstractLangerhans cell histiocytosis (LCH) encompasses a group of disorders characterized by neoplastic proliferation of Langerhans cells causing destruction of tissue, morbidity and mortality. The authors bring forth the case of a 2-year-old child presenting with chronic diarrhea and features of chronic malabsorption that revealed lesions over the ileocolonic mucosa. Histopathological examination with immunohistochemical expression for CD1a confirmed the diagnosis of LCH. Gastrointestinal LCH is quite uncommon, and review of the published English literature revealed about 50 reported cases. The importance of diagnosing this disease is echoed by the grim survival rate of 18 months from the time of diagnosis that is seen in around 60% of cases. Owing to its rarity and unfamiliarity of practicing physicians and pathologists of this disease at this site, the authors wish to discuss the potential pitfalls and differential diagnoses.

DILEMMA IN THE DIAGNOSIS OF COCCIDIOIDOMYCOSIS

SESSION TITLE: Chest Infections 3 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Coccidioidomycosis (CM) is a disease with broad spectrum of clinical manifestations that can affect immunocompromised as well as immunocompetent individuals. Patients can be asymptomatic or may have pneumonia like features. CM is endemic in several parts of the South-western US and South America. The incidence of CM is increasing all across the US and it poses several diagnostic challenges including delay in diagnosis and care. CASE PRESENTATION: A 30-year-old male with history of tobacco and marijuana smoking was admitted with fever, cough and intermittent hemoptysis for 3 weeks. He has history of travel to Aruba and Arizona 4 weeks prior to admission. He also has history of exposure to farm animals and used to hunt deer. He initially went to urgent care, diagnosed with pneumonia and was given Clarithromycin. However, his symptoms did not improve. On admission, exam showed minimal rales on both sides. CXR showed LLL opacity and bilateral hilar lymphadenopathy (LAD). CBC revealed eosinophilia 12.6%. He was started on Vancomycin, Zosyn and Azithromycin. Extensive work up was negative including tuberculosis, histoplasmosis, aspergillosis, coccidomycosis, legionella, brucellosis, blastomycosis, HIV. ACE level was negative. CT Thorax revealed cavitary lesion in the LLL with surrounding consolidation and multiple pulmonary nodules with surrounding ground glass opacity bilaterally. Significant LAD was seen in the mediastinum and in both pulmonary hila. Transbronchial biopsy showed focal proliferation of langerhans cells, CD1a reactive but negative S1-00 stain, consistent with Langerhans cell histiocytosis (PLCH) with dense eosinophilic infiltrate, however the eosinophilia was more extensive and not related to the positive CD1a areas which was thought to be secondary to chronic eosinophilic pneumonia. He was instructed to quit smoking and started corticosteroid 60 mg per day and Bactrim. However, his symptom did not improve and mediastinal LAD worsened. He had excisional biopsy of one of his mediastinal lymph nodes which showed suppurative necrotizing and non-necrotizing granulomatous inflammation. Numerous coccidioides fungal yeast were seen. He was started on Fluconazole and his symptoms improved. Repeat CXR showed resolution of opacities/cavity noted previously. DISCUSSION: Due to the versatile manifestation of this disease, the diagnosis is challenging, resulting in an extensive evaluation for malignancy and other infectious/granulomatous diseases. Serology can take weeks to months to develop, for this reason, obtaining fungal culture and tissue biopsy considered to be more valuable for diagnosis. Treatment involves Azole or Amphotericin B and duration depends on extent of infection and immune status of the patient. CONCLUSIONS: Physicians should have high suspicion for CM in patients with travel to endemic areas even if serology was negative and biopsy should be done to confirm the diagnosis. Reference #1: Wang Z, Wen S, Ying K. A case study of imported pulmonary coccidioidomycosis. Journal of Zhejiang University Science B. 2011;12(4):298-302. https://doi.org/10.1631/jzus.B1000261. Reference #2: Al-Daraji WI, Al-Mahmoud RMW, Ali MA. Disseminated Coccidioidomycosis: A Case Report from the United Kingdom. International Journal of Clinical and Experimental Pathology. 2009;2(5):494-497. DISCLOSURES: No relevant relationships by Housam Hegazy, source=Web Response No relevant relationships by Mili Shah, source=Web Response No relevant relationships by Bashar Sharma, source=Web Response No relevant relationships by Vikrant Tambe, source=Web Response

Spontaneous resolution of unifocal Langerhans cell histiocytosis of the skull: potential role of ultrasound in detection and imaging follow-up

Langerhans cell histiocytosis is a tumor-like condition characterized by idiopathic proliferation of Langerhans cells. The disease may involve the skeleton as well as other organs systems. Bone involvement may be solitary or multifocal. Unifocal osseous Langerhans cell histiocytosis may involve virtually any bone, with the calvarium being most frequently involved. Plain radiography, computed tomography and magnetic resonance imaging are the most used techniques for detection and characterization of the lesion. The use of ultrasound is less known, although it may be a valuable technique in detection and follow-up of superficially located lesions such as calvarial lesions. This case report describes an 8-year-old girl, in whom the lesion was initially detected by ultrasound. Furthermore, ultrasound was used to evaluate spontaneous resolution of the lesion. The knowledge of ultrasound characteristics may be important to avoid unnecessary radiation and gadolinium administration, particularly in a pediatric population.

Clinical Research of Pulmonary Langerhans Cell Histiocytosis in Children.

Background:Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis.Methods:A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors.Results:The median age of PLCH group was significantly lower than that of non-PLCH group (18.63 months vs. 43.4 months, P < 0.001). All PLCH children had other organ involvement and only 11 cases (9.4%) had respiratory symptoms. The most common radiologic finding was cystic lesions (29 cases, 24.8%). Pulmonary function abnormalities were dominated by obstructive ventilatory dysfunction (63 cases, 82.9%). The 5-year overall survival (OS) of PLCH children was 93.6% ± 2.3% and the event-free survival (EFS) was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases (13.2%) were due to progression or recurrence of lung damage. The 5-year OS of PLCH children with “risk organ” involvement was significantly lower than those without “risk organ” involvement (86.0% ± 4.9% vs. 100%, χ2 = 8.793, P = 0.003). The difference of EFS between two groups was also significant (43.7% ± 7.7% vs. 66.3% ± 6.5%, χ2 = 5.399, P = 0.020). The “risk organ” involvement had a significant impact on survival (hazard ratio = 1.9, P = 0.039).Conclusions:PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of “risk organ” involvement is the key point to improving EFS.

A case of langerhans cell histiocytosis associated with mantle cell lymphoma

Langerhans Cell Histiocytosis (LCH), which is caused by neoplastic clonal proliferation of Langerhans cells, may be associated with various malignant neoplasms, especially lymphomas, in rare cases. In literature some studies observed an unexpected number of second malignancies, both prior to and after the diagnosis of MCL. Here, we report a case with concurrent LCH and mantle cell lymphoma (MCL) in a same lymph node. To the best of our knowledge, this is the second reported case of LCH associated with mantle cell lymphoma in a lymph node.

CDKN2A/B Deletion and Double-hit Mutations of the MAPK Pathway Underlie the Aggressive Behavior of Langerhans Cell Tumors.

Langerhans cell histiocytosis (LCH) has a mostly favorable outcome, whereas Langerhans cell sarcoma (LCS) is an aggressive tumor. It is still unclear whether any specific molecular alterations could underlie the aggressive behavior of Langerhans cell proliferations. We used targeted next-generation sequencing and array-comparative genomic hybridization to profile 22 LCH samples from different patients together with 3 LCS samples corresponding to different relapses from the same patient. The third LCS relapse was a composite tumor including both B-cell chronic lymphocytic leukemia and LCS components. The 22 LCH samples were mostly of bone origin and showed classic histophenotypical features. Array-comparative genomic hybridization showed in all 3 LCS samples a similar homozygous somatic loss affecting the CDKN2A/B locus, whereas the 17 informative LCH samples did not show any detectable abnormality. In the 3 LCS samples, targeted next-generation sequencing of 495 cancer genes detected common mutations in KMT2D/MLL2 and in both MAP2K1 and NRAS genes, whereas BRAF was not mutated. A NOTCH1 mutation was acquired in 2 LCS samples. The composite LCS/B-cell chronic lymphocytic leukemia tumor showed the same genetic profile in its 2 components. LCH samples showed mutually exclusive mutations of BRAF (8/20) and MAP2K1 (4/19), but no mutation of KMT2D, NRAS nor NOTCH1. These results suggest that CDKN2A/B deletion and/or simultaneous mutations of MAP2K1 and NRAS may underlie the aggressive behavior of Langerhans cell tumors, and thus could be useful for the diagnosis of malignancy in histiocytic neoplasms. The MAPK pathway "double hit" profile provides a basis for targeted therapy in LCS patients.

A rare co-existence of langerhans cell histiocytosis and lichen planus

Langerhans cell histiocytosis (LCH) is the commonest of the histiocytic disorders. Owing to the relative rarity of the condition, it remains a disease in which the diagnosis is often delayed or missed. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cells in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Lichen planus presents as purple, polygonal, pruritic, papular eruption of unknown etiology affecting skin, mucous membranes and nails. Despite of thourough literature search, we could not find any association of lichen planus with LCH. Hereby, we report a case of five year old child with acute onset of polymorphic eruptions all over the body which on histopathology and Immunohistochemistry showed features of Langerhans cell histiocytosis and lichen planus on bilateral lower limb.

Langerhans Cell Histiocytosis in Temporal Bone: Case Report

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by uncontrolled proliferation of Langerhans cells. We report a case of a 5-years old girl with left post auricular swelling, otalagia and otorrhea of 2 month duration, High resolution computed tomography of temporal bone show destructive expanding tissue density mass. Apparent diffusion coefficient is 1.08×10³ mm²/s in diffusion weight magnetic resonance imaging .Post auricular incision revealed a pinkish, firm granulomatous mass occupied middle ear and mastoid Histopathological examination revealed a mixture of Langerhans histocytes and eosinophils. CD1 and S100 are positive in mmunohistochemistry.

Isolated Langerhans Cell Histiocytosis of Thyroid-Masquerading as a Thyroid Neoplasm - A Rare Case Report

Langerhans Cell Histiocytosis (LCH) is a rare neoplastic disease of unknown etiology characterized by oligoclonal proliferation of langerhans cells with a varying incidence rate of 4-5.4 per 1 million individuals. LCH occurs in localised and multifocal forms. LCH lesions were first described in the year 1893, they are composed of large histiocytes with abundant cytoplasm intermixed with lymphocytes and eosinophils. Diabetes insipidus is the most common endocrinological abnormality of LCH. Other manifestations such as hypothalamic/pituitary axis disturbance and anterior pituitary deficiency can occur resulting in secondary or tertiary hypothyroidism. Cases of LCH with isolated thyroid involvement are very rare. We present a case of LCH, in a 42-year-old female who had a nodular thyroid swelling since one and a half year with euthyroid status. Total thyroidectomy specimen was received with a clinical diagnosis of thyroid neoplasm. Histopathology revealed langerhans histiocytes both mononucleated and multinucleated with prominent nuclear grooves, accompanied by good number of eosinophils and lymphocytes. Histopathology diagnosis of LCH was given supported by IHC S-100 and CD 1a positivity. Since, it’s a rare entity it needs to be distinguished from its closer mimics non Hodgkin’s lymphoma and Rosai Dorfman’s disease. Treatment of choice is surgical resection, but there is no evidence of improved outcome on usage of adjuvant chemoradiotherapy for primary thyroid LCH post resection. So, more case reports and articles are recommended to acess treatment regimen for better outcome.

Cyclin D1 Is Expressed in Neoplastic Cells of Langerhans Cell Histiocytosis but Not Reactive Langerhans Cell Proliferations.

Langerhans cell histiocytosis (LCH) is characterized by frequent activating mutations involving the mitogen-activated protein kinase (MAPK) pathway. Therefore, downstream markers of MAPK pathway activation such as cyclin D1 may be useful as novel diagnostic markers of neoplasia in LCH. The goal of this study was to investigate cyclin D1 expression in LCH and reactive Langerhans cell accumulations using immunohistochemistry on archival tissue. All LCH cases tested (39/39) showed cyclin D1 expression in CD1a/Langerin cells. Most cases (22/39; 56%) showed strong cyclin D1 expression in the majority (≥50%) of lesional cells. Only a few cases (6/39; 15%) showed cyclin D1 expression in a small subset (<20%). Nearly all LCH cases (26/27; 96%) showed p-ERK expression by immunohistochemistry, parallel to cyclin D1 expression. CD1a Langerhans cells in all cases of florid dermatopathic lymphadenopathy and normal skin were negative for cyclin D1, as demonstrated by CD1a/cyclin D1 double staining. The majority of skin specimens (14/18; 78%) with dermatitis-related changes did not show cyclin D1 expression in the CD1a epidermal Langerhans cell aggregates. A minority (4/18; 22%) showed weak cyclin D1 staining in a small subset (5% to 10%) of CD1a Langerhans cells. We conclude that cyclin D1 is ubiquitously expressed in LCH, in keeping with the known near universal MAPK activation in this disease. Further, it is not significantly expressed in reactive Langerhans cell proliferations in lymph node or skin. Therefore, cyclin D1 immunohistochemistry may be useful in excluding non-neoplastic mimics of LCH.

A rare case of CD1a‐negative Langerhans cell histiocytosis of the central nervous system in a child

Key Clinical MessageLangerhans cell histiocytosis is a dendritic cell disorder with a wide spectrum of severity and presentations. Histopathology typically demonstrates a proliferation of Langerhans cells and a lymphohistiocytic inflammatory infiltrate with eosinophils. The diagnosis is supported by immunohistochemistry with the cell markers S100, CD1a, CD68, and Langerin [Blood, 126, 2015, 26 and N Engl J Med, 331, 1994, 154].

Langerhans cell histiocytosis associated with lymphoma: an incidental finding that is not associated with BRAF or MAP2K1 mutations

Langerhans cell histiocytosis is characterized by a localized or systemic proliferation of Langerhans cells. BRAF mutations have been reported in 40–70% of cases and MAP2K1 mutations have been found in BRAF-negative cases, supporting that Langerhans cell histiocytosis is a true neoplasm, at least in mutated cases. In a small subset of patients, Langerhans cell histiocytosis is detected incidentally in a biopsy involved by lymphoma. These lesions are usually minute and rarely have been assessed for mutations. We assessed for BRAF and MAP2K1 mutations in seven cases of Langerhans cell histiocytosis detected incidentally in biopsies involved by lymphoma. We performed immunohistochemical analysis for phosphorylated (p)-ERK. There were four men and three women (median age, 54 years; range, 28–84). The biopsies included lymph nodes (n=6) and chest wall (n=1). The lymphomas included five classical Hodgkin lymphoma, one mantle cell lymphoma, and one angioimmunoblastic T-cell lymphoma. All cases were negative for BRAF V600E and MAP2K1 mutations. Nevertheless, three of seven cases showed ERK activation as shown by expression of p-ERK. We performed mutation analysis using a panel of 134 commonly mutated genes (including BRAF and MAP2K1) by next-generation sequencing on three cases, including two cases positive for p-ERK by immunohistochemistry. No mutations were detected in any of the three cases assessed. Six patients received therapy appropriate for their lymphoma. With a median follow-up of 21 months (range, 6–89), no patients developed disseminated or recurrent Langerhans cell histiocytosis. We conclude that lymphoma-associated Langerhans cell histiocytosis is a clinically benign process that is not associated with BRAF V600E or MAP2K1 mutations and, as suggested by others, the designation Langerhans cell hyperplasia may be more appropriate. Nevertheless, the expression of p-ERK in three cases suggests that the RAS–RAF–MAP2K–ERK pathway is activated, perhaps by non-mutational mechanisms induced by the presence of lymphoma or lymphoma–microenvironment interactions.

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

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. 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 7years of follow-up, total vertebral reconstruction was observed. 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.