Indometacina como tratamiento de granuloma eosinófilo multifocal / Indomethacin as a treatment for multifocal Eosinophilic granuloma

Granuloma eosinófilo es la variante más frecuente de histiocitosis de células de Langerhans. La mayoría de las lesiones ocurren en cráneo, costillas, columna vertebral o huesos largos, y pueden ser únicas o múltiples. El tratamiento depende del lugar de la afectación y del número de lesiones. Las opciones terapéuticas incluyen un agente único con prednisona, la combinación de vinblastina y prednisona, curetaje de las lesiones óseas o instilación intralesional de esteroides. Indometacina parece ser efectiva como tratamiento de lesiones de histiocitosis de células de Langerhans del hueso en niños y es bien tolerada. Presentamos el caso de un paciente varón de 4 años de edad con afectación de 2 huesos del cuerpo, cráneo y vértebra, tratado con curetaje de la lesión craneal e indometacina oral durante 19 meses, con completa curación de las lesiones y sin recurrencia 4 meses después de suspenderla. Concluimos que indometacina parece ser efectiva en el tratamiento de lesiones óseas de histiocitosis de células de Langerhans en niños, evitando otras terapias más agresivas. (AU)

Eosinophilic granuloma of the bone is the most common variant of Langerhans cell histiocytosis. Most of the lesions occur in the skull, ribs, spine or long bones and may be single or multiple. Therapy is generally chosen based on the site involved and the number of lesions. Treatment options include single agent with prednisone, the combination of vinblastine and prednisone, curettage of bone lesions or intralesional steroids injection. Indomethacin seems to be effective in treating isolated Langerhans cell histiocytosis of the bone in children and is generally well-tolerated. We present the case of a 4-year-old boy with involvement of 2 bones, skull and vertebra, treated with curettage of the skull and indomethacin for 19 months. There was complete healing of the lesions at the end of the treatment and no evidence of recurrence 4 months post-treatment. We conclude that indomethacin seems to be effective in the treatment of Langerhans cell histiocytosis of the bone in children, avoiding more aggressive therapies. (AU)

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.

Acquired butterfly vertebra as a sequela of eosinophilic granuloma.

Butterfly vertebras are an abnormal embryological formation of the spinal bodies that occur because of a lack of fusion of the chondrification centers of the vertebral bodies. Langerhans cell histiocytosis is an entity that frequently involves vertebral bodies resulting in flat vertebras, and recovery of the vertebral body height is a very unusual finding. We present a case report of a pediatric patient with a thoracic acquired butterfly vertebra which occurred secondary to a Langerhans cell histiocytosis involvement. It is extremely rare to find vertebra plana that regains its complete height but is even more infrequent to evidence of a butterfly vertebra deformity that is not congenital.

Granuloma eosinofílico cervical en el adolescente: reporte de un caso y revisión de la literatura / Cervical eosinophilic granuloma in adolescents: case report and literature review

INTRODUCCIÓN El granuloma eosinofílico (GE) es una patología infrecuente, sobre todo en adultos, que puede afectar la columna cervical. A pesar de la vasta literatura, esta enfermedad afecta principalmente a la población infantil, y no hay un consenso sobre el manejo en adultos. Con el objetivo de aportar conocimiento respecto a esta patología poco frecuente, se presenta un caso clínico de GE cervical en un paciente de 16 años, a quien se trató de manera conservadora, con buenos resultados y retorno completo a sus actividades. CASO CLÍNICO Un hombre de 16 años, seleccionado de rugby, consultó por dolor cervical axial persistente y nocturno de 6 semanas de evolución, sin trauma evidente. Al examen, destacó dolor a la compresión axial sin compromiso neurológico asociado. Los exámenes de tomografía computarizada (TC) y resonancia magnética (RM) revelaron lesión lítica en el cuerpo de C3 de características agresivas, de presentación monostótica en tomografía por emisión de positrones-tomografía computada (TEP-TC) compatible con tumor primario vertebral. Se decidió realizar biopsia percutánea bajo TC, para definir el diagnóstico y manejo adecuado, la cual fue compatible con células de Langerhans. Al no presentar clínica ni imagenología de inestabilidad ósea evidente o compromiso neurológico, se manejó con tratamiento conservador, inmovilización cervical, analgesia oral, y seguimiento estrecho. A los cuatro meses de evolución, se presentó con una TC con cambios reparativos del cuerpo vertebral y sin dolor, y logró retomar sus actividad habituales. CONCLUSIONES El diagnóstico de GE es infrecuente a esta edad, y se debe plantear entre diagnósticos diferenciales de lesiones líticas agresivas primarias vertebrales. Es necesario el uso de imágenes, y la biopsia vertebral es fundamental para confirmar el diagnóstico. Su manejo va a depender de la sintomatología, del compromiso de estructuras vecinas, y de la estabilidad de la vértebra afectada. El manejo conservador con seguimiento clínico e imagenológico es una opción viable.

INTRODUCTION Eosinophilic granuloma (EG) is a rare, tumor-like lesion, infrequently affecting the cervical spine, particularly in adults. Although vastly described in literature, this pathology mainly affects children, and there is still no consensus on its treatment in older patients. With the goal of contributing to increase the knowledge regarding this infrequent pathology, we present a case of a C3 eosinophilic granuloma in a 16-year-old patient, who was treated conservatively, with good results, including complete return to his previous activities. CLINICAL CASE a 16-year-old male, elite rugby player, presented with a history of persistent neck pain, mainly at night, with no previous trauma. Upon physical examination, he reported neck pain with axial compression of the head, without neurological impairment. Both computed tomography (CT) and magnetic resonance imaging (MRI) scan revealed an aggressive lytic lesion in the C3 vertebral body, a with monostotic presentation on positron emission tomography-computed tomography (PET-CT) compatible with a primary spine tumor. A CT-guided percutaneous biopsy was obtained to establish the diagnosis and provide the proper management. The results were compatible with Langerhans cells. As he presented no symptoms or imaging findings of evident bone instability, as well as no neurological impairment, the patient was treated conservatively, with a cervical brace, oral pain medication and close followup. A CT obtained after four months of treatment showed reparative changes of the C3 vertebral body; at this point, the patient reported no neck pain, so he was able to return to his previous activities. CONCLUSIONS Although an EG is rare at this age, it should be considered in the differential diagnosis of primary vertebral aggressive lytic lesions. Imaging and a vertebral biopsy are paramount to confirm the diagnosis. The treatment modality depends on the symptoms, the involvement of adjacent structures, and the stability of the affected vertebra. Conservative management including clinical and imaging followup is a viable option.

Multifocal eosinophilic granuloma of the jaws with long-term follow-up: a case report.

Eosinophilic granuloma, a rare disease, has various clinical manifestations and no specific X-rays features and is thus easily misdiagnosed. This paper reports a case of multifocal eosinophilic granuloma of jaw with long-term follow-up. The patient initially presented with periodontal tissue destruction.The diagnosis, treatment and prognosis of multifocal eosinophilic granuloma of jaw were discussed in combination with the literature to alert this disease in clinical practice.

Recurrent eosinophilic granuloma involving maxilla and mandible in an adult male: an unusual case report.

Langerhans' cell histiocytosis (LCH) is a disease spectrum characterized by the intense infiltration/accumulation of Langerhans' cells in the affected tissues. Eosinophilic granuloma is the mildest form of LCH which mainly affects the skull, mandible, ribs and femur in children and young adults but rarely occurs in the maxilla. Here, we describe an unusual case of eosinophilic granuloma affecting the tissues adjacent to tooth #25 and tooth #46 (FDI numbering system) in a 35-year-old male. Although lesions underwent clinical remission after local radiotherapy, a new lesion appeared 11 months after initial therapy in tooth #18, indicating recurrence of eosinophilic granuloma. Subsequently, the patient was treated with corticosteroid therapy, achieved remission and is disease free after 3 years of follow-up.

Multifocal eosinophilic granuloma of the jaws with long-term follow-up: a case report / 华西口腔医学杂志

Eosinophilic granuloma, a rare disease, has various clinical manifestations and no specific X-rays features and is thus easily misdiagnosed. This paper reports a case of multifocal eosinophilic granuloma of jaw with long-term follow-up. The patient initially presented with periodontal tissue destruction.The diagnosis, treatment and prognosis of multifocal eosinophilic granuloma of jaw were discussed in combination with the literature to alert this disease in clinical practice.

Vertebra Plana in Children May Result from Etiologies Other Than Eosinophilic Granuloma.

BACKGROUND: Vertebra plana in children is a diagnostic dilemma for orthopaedic surgeons. This radiographic finding sometimes has been said to be pathognomonic for eosinophilic granuloma (Langerhans cell histiocytosis); however, vertebra plana may also be caused by a range of other conditions. We sought to determine whether vertebra plana can be associated with malignancies other than eosinophilic granuloma. QUESTIONS/PURPOSES: (1) To report the underlying diagnoses for children with vertebra plana and determine how frequently these patients were found to have eosinophilic granuloma as opposed to an underlying malignant process, (2) to evaluate the occurrence of nondiagnostic results on biopsy, and (3) to determine whether the presenting characteristics of spinal lesions were associated with the ultimate clinical diagnosis. METHODS: As part of a retrospective review, our institutional electronic medical record was searched for all patients younger than 18 years between 1976 and 2017 whose clinical record included the term vertebra plana. Patients with trauma were excluded. Twenty-seven patients met the inclusion criteria (mean [range] age 9 years [0 to 18]; 12 girls). To address our first research purpose about the underlying diagnoses of patients with vertebra plana, we reviewed the final clinical diagnosis. To address our second research purpose about the utility of biopsy, we reviewed which patients underwent a biopsy and whether it had been diagnostic. To address our third research purpose about the radiographic criteria, we classified the radiographs and compared this to the clinical diagnosis. Vertebral collapse was described as less than 50% collapse, 50% to 100% collapse, symmetrical, and asymmetrical. The location of each lesion was noted. RESULTS: Twelve of 27 patients had a diagnosis of eosinophilic granuloma. Six of 27 had other neoplastic etiologies, including acute lymphoblastic leukemia, primary germ cell tumor, giant cell tumor, rhabdomyosarcoma and teratoma. Seventeen of 27 patients underwent biopsy to confirm the diagnosis; six biopsies were consistent with eosinophilic granuloma, six for other etiologies, and five were nondiagnostic. With the limited patient numbers available, there was no difference in the frequency of less than 50% loss of height or 50% to 100%, or symmetric and asymmetric loss of height, and location of the lesion among patients with eosinophilic granuloma and those with other diagnoses. CONCLUSIONS: Eosinophilic granuloma or Langerhans cell histiocytosis is a common cause of vertebra plana, but other causes must be considered in children presenting with this radiographic finding. Six of 27 of patients presenting to our center with vertebra plana had an underlying neoplasm other than eosinophilic granuloma. With the limited patient numbers available, pain, spinal location, and the degree and symmetry of collapse were not associated with a diagnosis of eosinophilic granuloma in this series. Thus, patients presenting with vertebral plana and back pain need a comprehensive work-up and potentially tissue biopsy to determine diagnosis and appropriate treatment. LEVEL OF EVIDENCE: Level IV, diagnostic study.

Long-term Follow-up of Eosinophilic Granulomas of the Axial and Appendicular Skeleton Managed With Biopsy Alone.

BACKGROUND: This study aimed to evaluate the long-term clinical and radiologic follow-up results of eosinophilic granulomas (EGs) of the axial and appendicular skeleton managed with biopsy alone. METHODS: Fifty-five patients with unifocal osseous EGs of the axial and appendicular skeleton were followed after biopsy. Patients were divided into 2 groups on the basis of localization of the lesions. In group 1, there were 32 (58.2%) children with extremity long bone involvement: femur, tibia, humerus, ulna, and radius. Group 2 included 23 (41.8%) patients with lesions located in other appendicular and axial skeleton bones: pelvis, scapula, clavicle, lumbar, and thoracic vertebrae. After confirming the diagnosis by a closed biopsy, no further surgical intervention was performed. Clinical recovery included regression of the localized symptoms, mainly pain resolution. Functional improvement was assessed by Musculoskeletal Tumor Society (MSTS) scoring. Radiologic healing was defined as ossification of the entire lesion with cortical thickening in long and flat bones, and restoration of vertebral body height in the spine. Complications, including local recurrence, were determined. RESULTS: The patients comprised 28 boys and 27 girls with an average age of 9.2 years (range, 3 to 16 y). The average follow-up was 76 months (range, 28 to 132 mo). The median time from biopsy to clinical recovery was 17 days [95% confidence interval (CI), 13.3-20.6] and 36 days (95% Cl, 32.8-39.1) in group 1 and group 2, respectively. MSTS scores increased progressively till the end of 12 months in both groups. The median time from biopsy to radiologic healing was 16 months (95% CI, 11.5-20.4) and 42 months (95% Cl, 39.3-44.6) in group 1 and group 2, respectively. Both clinical recovery (P=0.021) and radiologic healing (P=0.009) were significantly faster in group 1 compared with group 2. No major complication was seen after biopsy. All lesions regressed without a local recurrence. CONCLUSIONS: Unifocal osseous EGs have spontaneous healing potential and confirming the diagnosis by biopsy is enough to obtain good clinical and radiologic results without any additional surgical intervention. TYPE OF STUDY: This was a therapeutic study. LEVEL OF EVIDENCE: Level IV.

Canine eosinophilic pulmonary granulomatosis: case report and literature review.

We describe and illustrate herein a case of eosinophilic pulmonary granulomatosis (EPG) in a 16-mo-old, castrated male, Great Pyrenees crossbred dog. EPG appears to differ from eosinophilic pneumonias and eosinophilic bronchopneumopathy in dogs by the presence of nodules and masses in the lungs composed of eosinophils, macrophages, and various combinations of lymphocytes, plasma cells, neutrophils, and mast cells within fibrous tissue. Specific information from this dog was added to the information from the limited number of other dogs diagnosed with EPG reported in the veterinary literature, and the information analyzed. EPG appears to have no breed or sex predilection and tends to be a disease of younger dogs, often ≤ 3 y of age. Antemortem imaging of nodules and masses in the lungs, combined with cytologic evidence of eosinophils in the lesions, is highly suggestive of EPG, and the diagnosis can be confirmed postmortem. Concurrent eosinophilia and basophilia can be features of EPG, but their diagnostic value requires further investigation, as does the possible causal association with Dirofilaria immitis infection.

Eosinophilic bronchitis, eosinophilic granuloma, and eosinophilic bronchopneumopathy in 75 dogs (2006-2016).

BACKGROUND: Eosinophilic lung disease is a poorly understood inflammatory airway disease that results in substantial morbidity. OBJECTIVE: To describe clinical findings in dogs with eosinophilic lung disease defined on the basis of radiographic, bronchoscopic, and bronchoalveolar lavage fluid (BAL) analysis. Categories included eosinophilic bronchitis (EB), eosinophilic granuloma (EG), and eosinophilic bronchopneumopathy (EBP). ANIMALS: Seventy-five client owned dogs. METHODS: Medical records were retrospectively reviewed for dogs with idiopathic BAL fluid eosinophilia. Information abstracted included duration and nature of clinical signs, bronchoscopic findings, and laboratory data. Thoracic radiographs were evaluated for the pattern of infiltrate, bronchiectasis, and lymphadenomegaly. RESULTS: Thoracic radiographs were normal or demonstrated a bronchial pattern in 31 dogs assigned a diagnosis of EB. Nine dogs had intraluminal mass lesions and were bronchoscopically diagnosed with EG. The remaining 35 dogs were categorized as having EBP based on radiographic changes, yellow green mucus in the airways, mucosal changes, and airway collapse. Age and duration of cough did not differ among groups. Dogs with EB were less likely to have bronchiectasis or peripheral eosinophilia, had lower total nucleated cell count in BAL fluid, and lower percentage of eosinophils in BAL fluid compared to dogs in the other 2 groups. In contrast to previous reports, prolonged survival (>55 months) was documented in dogs with EG. CONCLUSIONS AND CLINICAL IMPORTANCE: Dogs with eosinophilic lung disease can be categorized based on imaging, bronchoscopic and BAL fluid cytologic findings. Further studies are needed to establish response to treatment in these groups.

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.

Incidence and radiological pattern of eosinophilic granuloma: a retrospective study in a Chinese tertiary hospital.

BACKGROUND: The incidence and radiological patterns of eosinophilic granuloma (EG) in China is not clear. We described the incidence, presentation, and imaging characteristics of Chinese EG patients in a tertiary hospital. METHODS: A retrospective chart review was performed from January 2004 to October 2017 at a single tertiary general hospital. Seventy-six patients were pathologically identified as EG. Besides, 60 patients with preoperative imaging diagnosis of "EG" were analyzed to reveal the radiological patterns and their diagnostic power. RESULTS: Fifty-three male and 23 female EG patients with a mean age of 18.1 ± 16.7 years (range 1-58 years) were retrospectively included. Significant differences were observed in gender (male to female = 2.3:1) and age (the highest incidence at the age of 0~5 years) for EG. EG predominantly involved the skeletal system: flat bones (31.43%) > irregular bones (24.76%) > long bones (22.86%) > other organs (20.95%). No obvious relationships between season, biochemical markers, and EG incidence were observed. The common presenting symptoms were pain followed with local mass, and most patients underwent surgical resection. Among 60 imagingly diagnosed "EG" patients from April 2009 to October 2017, only 22 were with histological confirmation. The correct diagnosis rates were 37.1% (13 out of 35), 16.7% (5 out of 30), and 22.2% (8 out of 36) for plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI), respectively. CONCLUSIONS: Chinese EG has a varied presentation, age distribution, and gender difference. EG diagnosis is still based on biopsy or histopathology instead of imaging techniques.

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.

18F-FDG PET/CT for the Evaluation of Primary Eosinophilic Granuloma of the Hypothalamus.

A 21-y-old man who presented with polyuria and polydipsia was discovered to have diabetes insipidus due to eosinophilic granuloma of the hypothalamus. 18F-FDG PET/CT, which was performed as a metastatic work-up, revealed an intensely 18F-FDG-avid hypothalamic mass and no other sites of disease.

Langerhans' cell histiocytosis with neurological injuries diagnosed from a single cutaneous lesion.

Histiocytoses are rare diseases caused by the proliferation of histiocytes. The pathogenesis remains unknown and the highest incidence occurs in pediatric patients. The clinical presentations can be varied, in multiple organs and systems, and the skin lesions are not always present. Evolution is unpredictable and treatment depends on the extent and severity of the disease. It is described the case of a patient with various neurological symptoms, extensively investigated, who had its was diagnosed with histiocytosis from a single skin lesion. This report highlights the importance of Dermatology in assisting the investigation of difficult cases in medical practice.