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.

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.

Tumours and tumour-like lesions of the hip in the paediatric age: a review of the Rizzoli experience.

Bone tumours and tumour-like lesions of the hip in children are rare. Signs and symptoms of these tumours are generally nonspecific. Delay of diagnosis is not uncommon. A high index of suspicion in young patients presenting with persistent pain and without history of trauma, that is unresolved with conservative therapy should prompt further investigation, including radiographs or computed tomography scan of the pelvis. In the experience of the Istituto Rizzoli, in patients less than 14 years (mean 9 years, ranged from 6 months to 14 years), 752 tumours and tumours-like lesions occurred in the pelvis or proximal femur, involving the hip. Tumour-like lesions accounted for 322 cases (simple bone cyst in 255, eosinophilic granuloma in 43, aneurismal bone cyst in 34), benign tumours for 340 cases (osteoid osteoma in 229, fibrous dysplasia in 63, exostosis in 48) and malignant tumours for 80 cases (Ewing's sarcoma in 53 and osteosarcoma in 27). The epidemiology, pathology, clinical presentation, and radiograph findings are discussed for each of these tumours.Treatment of these tumours differs from observation or minimally invasive treatment for most pseudotumoural lesions, intralesional excision or termoablation for benign bone tumours and wide resection for malignant bone tumours. In this latter group, chemotherapy is required and often administered pre- and postoperatively.

Eosinophilic granuloma manifesting as painless cervical lymphadenopathy in a patient positive for human immunodeficiency virus 1.

Eosinophilic granuloma is rarely reported within lymph nodes. Furthermore, it is even more rarely reported in the setting of human immunodeficiency virus (HIV) infection. No definitive etiologic association exists between Langerhans cell histiocytosis (LCH) and HIV. However, their potential relationship underscores the significance of cytokines and their influence on biological niches required for Langerhans development and homeostasis.

Gastric eosinophilic granuloma in China: case series.

AIM: Eosinophilic granulomas are benign localized lesions in the stomach that are distinct from eosinophilic gastritis. The aim of this study was to identify the potential etiological factors and clinical features of gastric eosinophilic granulomas (GEG). METHODS: Clinical manifestations, histopathological features, diagnosis and treatments of 48 hospitalized cases of GEG were analyzed retrospectively. A modified Giemsa staining was employed to detect Helicobacter pylori in 23 of these 48 patients. RESULTS: There was a significant gender difference among patients with GEG with male patients significantly more affected than female patients. The final diagnosis was made after operating the patients (the misdiagnosis level was rather high before resection). Lymphoid follicles were found in 68.6% of the tissues surrounding the lesion. H. pylori infection was detected in 69.6% of patients. In 11 patients eosinophilia in the peripheral blood was observed. CONCLUSION: The results suggest that H. pylori infection, estrogen status and local allergic reactions may be associated with the development of GEG.

Non-pruritic granuloma in Norwegian forest cats.

The eosinophilic granuloma complex is a group of skin disorders common in cats. This paper describes the clinical, haematological and histopathological features of 17 related Norwegian forest cats, six of which had a linear granuloma on the caudal thigh, three of which also had a granuloma on the lower lip, and one of which had a granuloma in combination with an indolent ulcer. The high prevalence of the disease in this population is suggestive of a genetic background.

Qualitative diagnosis of calvarial metastasis by neural network and logistic regression.

RATIONALE AND OBJECTIVES: To simplify the diagnostic features used by an artificial neural network compared with logistic regression (LR) in the diagnosis of calvarial metastasis with computed tomography and analyze their accuracy. MATERIALS AND METHODS: Twenty-one of 167 patients with calvarial lesions were found to have metastasis. Clinical and computed tomography data were used for LR and neural network models. Both models were tested with the leave-one-out method. The final results of each model were compared using the area under receiver operating characteristic curve (Az). RESULTS: The neural network identified metastasis significantly more successfully than LR with an Az of 0.9324 +/- 0.0386 versus 0.9192 +/- 0.0373, P = .01. The most important features selected by the LR and neural network were age and edge definition. CONCLUSION: Neural networks offer wide possibilities over statistics for the study of calvarial metastases other than their minimum clinical and radiologic features for diagnosis.

Langerhans cell histiocytosis of bone in children and adolescents.

The purpose of this paper was to analyze the orthopaedic aspects of Langerhans cell histiocytosis and to assess the outcome after treatment. Fifty-six patients with Langerhans cell histiocytosis involving the bone were retrospectively studied. Three groups were identified with regard to the type of bone involvement. Group I patients (n = 26) had a solitary bone lesion, with no systemic involvement. No treatment was given in 22 patients and curettage was done in 4 patients. At last follow-up, complete or partial healing was observed in 25 patients. Group II patients (n = 13) had multiple bone lesions, with no systemic involvement. Clinical and radiographic pictures as well as the outcome after treatment were similar to those in group I. Group III patients (n = 17) had bone and systemic involvement. Chemotherapy was used in all group III patients, with adjuvant radiotherapy in seven patients. Recurrence or new lesions were observed in 11 patients. Complications of treatment were observed in 14 patients. Lesions in group I and II patients had a considerable potential for spontaneous healing. Group III patients had the worst prognosis. Current chemotherapy protocols have a high morbidity and produce a variable response.

[Eosinophilic ulcer of the oral mucosa: 11 cases]. / Ulcère éosinophilique de la muqueuse buccale: 11 cas.

BACKGROUND: The eosinophilic ulcer is a lesion of the oral mucosa, that has been infrequently described in the literature. This is a benign and self-limiting lesion of unknown origin. CASE REPORTS: In this article, eleven new cases of eosinophilic ulcer of the oral mucosa are presented. The clinical, histologic and evolutive features are reviewed. DISCUSSION: Recurrent trauma is clearly involved in the pathogenesis of this entity.

Eosinophilic granuloma of the cervical spine.

STUDY DESIGN: A meta-analysis was performed based on 53 cases of cervical eosinophilic granuloma reported in the literature and 1 in an adult treated by the authors. OBJECTIVE: To stress the clinical and radiologic differences between cervical and thoracolumbar spinal eosinophilic granuloma and to point out differences between adults and children with cervical eosinophilic granuloma to avoid false diagnosis. SUMMARY OF BACKGROUND DATA: Until now, cervical eosinophilic granuloma has been reported in 43 children and 9 adults. In 1 case the age is unknown. In previous studies, differences between adults and children with cervical eosinophilic granuloma have not been analyzed, nor has cervical eosinophilic granuloma been compared with thoracolumbar eosinophilic granuloma. METHODS: All reported cases of cervical eosinophilic granuloma were analyzed concerning age and sex distribution, clinical and radiologic presentation, therapy, and outcome. The authors' case in a 46-year-old patient is discussed. RESULTS: The presenting symptoms of cervical eosinophilic granuloma are usually pain and restricted range of motion. In contrast to eosinophilic granuloma of the thoracic spine and lumbar spine, the neurologic symptoms are less frequent, and the first radiographic sign is an osteolytic lesion. Vertebra plana is a rare sign in cervical eosinophilic granuloma. In children, the middle cervical spine is most often affected, whereas in adults it is the second vertebra. The outcome of the patients has been good in most cases, independently of treatment. CONCLUSION: In most cases of cervical eosinophilic granuloma, immobilization is an adequate therapy. If the process continuous to progress, radiotherapy is recommended. Surgical treatment should be reserved for cases with instability or neurologic defects.

Eosinophilic granuloma of the liver: a characteristic lesion with relationship to visceral larva migrans.

Children with the clinical syndrome of visceral larva migrans as a result of Toxocara species have typical lesions in the liver and other viscera, consisting of palisading granulomas that contain numerous eosinophils and often Charcot-Leyden crystals; recognizable parasites are uncommon. Similar eosinophilic granulomas that are found incidentally in adults often cause diagnostic problems. To define better the clinical, laboratory, and pathologic features of these lesions, we reviewed 43 cases of hepatic eosinophilic granuloma (excluding cases of Langerhans' cell histiocytosis) collected in the files of the AFIP over a period of 31 years. The eosinophilic granulomas were found in patients of all ages (range 12 months to 77 years); 30% were younger than 20 years. There were 26 male and 17 female patients. Most patients (26 of 43; 60%) were asymptomatic, and the lesions were discovered incidentally. Others had fever (20%) or abdominal pain (20%). The granulomas were typically multiple (61%), with central necrosis surrounded by a mixed inflammatory infiltrate with numerous eosinophils and variable numbers of neutrophils. lymphocytes, and a palisade of epithelioid histiocytes and/or giant cells. Charcot-Leyden crystals were present in 19 cases (44%). Remnants of parasites (eight Toxocara sp., two Capillaria sp.) were identified in the tissue in 10 patients. There was a positive serologic test for Toxocara sp. in five additional cases. Immunohistochemical staining using polyclonal antiserum against Toxocara canis larvae demonstrated positivity in macrophages in eight of 13 cases tested. We conclude that identification of an eosinophilic granuloma in the liver should suggest the diagnosis of visceral larva migrans and prompt a search for the causative organism with serial sectioning of the block and serologic tests for Toxocara and other causative parasites.

An outbreak of trematode-induced granulomas of the conjunctiva.

PURPOSE: To describe the epidemiologic, clinical, and histopathologic features of trematode granulomas of the conjunctiva, eyelid, and anterior chamber in pediatric patients. DESIGN: Prospective noncomparative case series. PARTICIPANTS: Forty-one children from a southern Indian village with conjunctival granulomas. METHODS: The village of Sellananthal was selected for a field visit after analysis of earlier hospital-based allergic conjunctival granuloma cases. Children with ocular diseases were examined, and histories of exposure to assumed risk factors and clinical findings were evaluated. Selected patients were brought to the base hospital for excisional biopsy. Serial sections obtained from the excised nodules were examined for the presence of a parasite. MAIN OUTCOME MEASURES: Histopathologic examination of excised conjunctival lesions or response of lesions to local medical therapy. RESULTS: In this year-long prospective study, 41 children (16 years or younger; 38 boys and 3 girls) with clinical features of allergic conjunctival granulomas were examined. Thirty-four patients were from a single village located in the southern Indian state of Tamil Nadu; the remaining 7 were from various parts of the same state. All children swam in their village's freshwater pond. Twenty patients with nodules less than 5 mm in diameter received medical treatment; 13 with larger nodules underwent surgical excision of the lesions. Nine of these 13 cases revealed a zonal granulomatous inflammation admixed with eosinophilic leukocytes; 4 of these 9 displayed fragments of the tegument and internal structures of a trematode and Splendore-Hoeppli phenomenon. The remaining 4 of the 13 cases revealed nongranulomatous inflammation made up of lymphocytes, histiocytes, and eosinophils. Eight patients refused surgical treatment. CONCLUSIONS: In southern India, one cause of allergic conjunctival granulomas in children seems to be trematode infection. The clustering of cases in a single village and exposure to a village freshwater pond indicate the need for an epidemiologic investigation and study of the parasite's life cycle. Sporadic cases from other parts of the state with similar histories of exposure to their local pond or river water suggest a widespread distribution of the etiologic agent.

Treatment of Langerhans-cell histiocytosis in children. Experience at the Children's Hospital of Nancy.

Forty children who had Langerhans-cell histiocytosis were followed for an average of six years (range, excluding patients who died of the disease, two to fifteen years). The patients were divided into two diagnostic groups: those who had localized disease (involving one bone or more only) and those who had multifocal disease (an osseous lesion and a soft-tissue mass, a skin rash, diabetes insipidus, or generalized disease). Methods of treatment included curettage, bone-grafting, chemotherapy, local or systemic corticosteroids, and radiotherapy. Nineteen of the thirty patients who had localized disease had a complete response to the therapy, four had a partial response, and seven had no response. Twenty-one of these thirty patients had not had a recurrence by the time of the latest follow-up examination; nine had a local recurrence within four years after the initial therapy but had no additional recurrences after treatment of the local recurrence. No recurrence occurred more than four years after the time that the initial diagnosis had been made. Five of the ten patients who had multifocal disease had a complete response to the therapy, two had a partial response, and three had no response. Six patients had a recurrence; four did not. Two patients died of the disease. As a result of this study, we recommend the avoidance of intensive measures of treatment, if possible, and we advise long-term follow-up of these patients.

Histiocitosis X pulmonar primaria (granuloma eosinofílico pulmonar)

La histiocitosis X pulmonar primaria es una patología infrecuente que afecta predominantemente a personas entre los 20 y los 40 años, usualmente con historia de tabaquismo. Presentamos un grupo de tres pacientes con esta entidad que se estudiaron en el Hospital Santa Clara en los últimos 20 años. Los síntomas más frecuentes fueron tos con expectoración mucosa y disnea. En todos se documentó historia de tabaquismo. La radiografía de tórax mostró infiltrado intersticial reticulonodular difuso, con volumen pulmonar normal y signos de hipertensión pulmonar precapilar. Todos se presentaron con hipoxemia severa y cor pulmonale. La histopatología confirmó el diagnóstico de histiocitosis X pulmonar primaria.

[X-ray morphology of eosinophilic granuloma other than skull and spine]. / Röntgenmorphologie des eosinophilen Granuloms ausserhalb von Schädel und Wirbelsäule.

The radiological findings in 39 patients with a manifestation of eosinophilic granuloma (e.g.) other than skull and spine were evaluated. The localisation was found in the femur (26%), clavicula (20%) and ribs (20%). The lesions were located in 81% in the diaphysis and in 9.5% either in the epi-metaphysis or epi-metadiaphysis. Associated periosteal reactions were observed in 38% of the patients, and were solid in 25%, lamellar in 10%, and in 3% complex. The majority of the tumours were classified as Lodwick IB lesions (41%). Lodwick IC lesions were seen in 13%, Lodwick II lesions in 25.5% and Lodwick III lesions in 20.5% of the patients. In patients below the age of 20 years the eosinophilic granuloma is characterised by a Lodwick IB lesion without or with a solid periosteal reaction, which allows differentiation from Ewing's sarcoma or osteomyelitis. Contrariwise, in patients above 20 years of age the e.g. appears with a higher Lodwick grade. Hence, differentiation, X-ray morphology, between e.g., metastasis, lymphoma, and multiple myeloma does not seem possible.

Eosinophilic granuloma of the left ischium.

A case of eosinophilic granuloma in an unusual location (ischium) is presented. Eosinophilic granuloma, a benign lesion of bone, has many radiographic appearances that vary with location. The flat bone of the skull and pelvis are common sites of involvement. Although the iliac wings and pubic rami are not uncommon sites for such a lesion, the ischium is a rare site. This occurrence prompted us to report this case.

Primary bone tumours of the thoracic skeleton: an audit of the Leeds regional bone tumour registry.

An audit of the Leeds regional bone tumour registry found that primary bone tumours of the thoracic skeleton constituted 90 of the 2004 cases (4.5%). Thirty seven per cent occurred in the ribs, 32% in the scapulae, 11% in the thoracic vertebrae, 11% in the sternum, and 9% in the clavicles. Malignant tumours were more common than benign (54 v 36) and occurred in an older population (mean ages 47 and 31 years). The scapula was the most common site for malignant lesions and the ribs the most common site for benign tumours. Chondrosarcoma was the commonest tumour in older patients, fibrous dysplasia and plasmacytoma in the middle age group, and eosinophilic granuloma in children. Presenting symptoms were a poor guide to whether the lesion was malignant or not. This and the small proportion of correct preoperative diagnoses indicate the need for early biopsy. Bone tumour registries provide a valuable source of cumulative information about uncommon tumours and facilitate accurate diagnosis, teaching, and research.

Tumores óseos. Estadística en relación a sexo, edad y sitio. Informe de 1351 casos / Bone tumors. Statistic in relationship to sex, age and location. Report of 1351 cases

Se presenta una serie de 1351 casos de tumores óseos primarios benignos y malignos, metastásicos, tumores malignos de tejidos blancos que invadieron hueso y lesiones seudotumorales atendidos en ocho años, de 1982 a 1989 en el Hospital de Ortopedia Magdalena de las Salinas del IMSS. La relación del sexo masculino y femenino fue de 1.3:1; la población pediátrica correspondió a 422 pacientes, de los cuales 91.5% cursaron con lesiones malignas y 8.5% con tumores malignos; 929 enfermos fueron adultos, y de ellos, 75.6% tuvieron lesiones benignas y 24.4% tumores malignos. Los segmentos anatómicos afectados con mayor frecuencia fueron: la extremidad pélvida con 754 casos y la extremidad torácica con 301. Se presenta el registro detallado en las variables analizadas. Los datos encontrados en nuestro estudio son similares a los informacos en las "series clásicas".