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Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocytic cells that rarely affects adults. Different therapeutic options have been used in the treatment of this condition depending on the severity of the disease. However, the management of LCH is often controversial when a resection with clean margins is not achieved. We present a 37-year-old male who was diagnosed with LCH affecting the middle third of the clavicle. The patient was subjected to a wide resection of the lesion creating a large bone defect. The reconstruction was then performed using a 3-D printed porous pseudo-prosthesis (Ti6AL4V) which was fixed using an anatomical clavicular plate (Depuy-Synthes, Raynham, MA, USA) and locking screws. The implant trabeculae were filled with bioactive bone graft substitute (GlassBONETM, Noraker, France). After two years of follow-up the patient was pain free, had a full range of movement, and scored 100 on the Constant Scale and 2.5 points on the disabilities of the arm, shoulder and hand score (DASH). Moreover, in this case, the implantation of the 3-D printed pseudo-prosthesis avoided the use of adjuvant therapies such as radiotherapy. The use of a 3-D printed pseudo-prosthesis achieved an excellent clinical and functional outcome in the treatment of a large bone defect, following a resection of a LCH of the clavicle. 3-D printed pseudo-prostheses could be useful instruments for the treatment of bone defects following large bone resections in musculoskeletal tumors.

Athough few case reports of human fascioliasis have been reported from different parts of India, there is no case reported from Kashmir valley till date. Here in we report two cases of human fascioliasis. The both patients presented with fever, marked eosinophilia and liver leisions on imaging. Hepatobiliary imaging showed vague features like mild biliary dilatation and liver leisions representing burrows. Liver biopsy in one of the patients revealed eosinophilic granuloma. Both patients were diagnosed definitively with endoscopic retrograde cholangio-pancreatography (ERCP) by demonstrating live adult fasciola worms. Any patient presenting with fever, marked eosinophilia and liver leisions on imaging should be evaluated for fascioliasis.

OBJECTIVE: Spinal eosinophilic granulomas (EGs) are uncommon tumors, constituting 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, 12e 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.

Langerhans cell histiocytosis (LCH) is a rare idiopathic disorder of the reticulo-endothelial system with abnormal proliferation of the bone marrow derived from Langerhans cells.LCH mainly affects children and young adults, with a slight male predilection. It is clinically divided into three groups namely Letter-Siwe disease (multiple multi organ affecting LCH at very young age), Hand-Schuler-Christian disease (LCH of bone involvement exophthalmos and diabetes insipidus), and Eosinophilic Granuloma (LCH of bone, solitary or multiple).Eosinophilic Granuloma (EG) is the most benign and common form of the Langerhans cell histiocytosis, it is a rare proliferative disease that can affect single or multiple organs.It is an intraosseous destructive lesion characterized by the presence of vast numbers of eosinophils and histiocytes. It can be localized or multifocal.Most frequent signs and symptoms are pain, swelling, ulceration, gingival necrosis, alveolar bone destruction with mobility and loss of teeth.The diagnosis is confirmed by immunohistochemistry using either the anti-CD1a or anti-S100 protein antibody.The severity and prognosis depend on the type and extension of the organ involved. The prognosis of patients with a single EG lesion is generally good.The literature reveals a lack of consensus about treatment, it varies depending on the size, number, localization of the lesions, and patient's general conditions.This article describes an unusual case of eosinophilic granuloma with maxillary involvement diagnosed in a young woman of 29 years old, treated in the oral surgery department of the dental consultation and treatment center in Casablanca.Although lesions underwent clinical remission after surgery and corticosteroid, a new lesion appeared 18 months after initial therapy, indicating recurrence of eosinophilic granuloma.

Clinical case A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively. The lesion responded to treatment. Discussion The orbital involvement of Langerhans cell histiocytosis, despite its low incidence, should be considered in the examination of acute peri-orbital swelling. It usually presents as an osteolytic lesion, and it is confirmed with a histological examination and immunohistochemical techniques for CD1a and S100. An interdisciplinary approach is recommended to rule out multifocal or multisystemic diseases, as well as to develop an appropriate treatment strategy.

Tongue worms utilise herbivorous mammals as intermediate hosts and reside in the nasopharynx of carnivores as their definitive hosts. A recent study in south eastern Australia showed an unexpectedly high infection (67%) of wild dogs with these parasites. The present study aimed at determining the pathogenicity of the parasite in both definitive (dog) and intermediate (cattle) hosts by histopathology. The definitive host showed multifocal haemorrhage of the interstitium of the nasal mucosa, multifocal mucosal erosion, congestion and haemorrhage, with haemosiderin laden macrophages present in those foci and distortion and destruction of the nasal mucosa. Histopathologic examination of lymph nodes from an infected cow showed diffuse eosinophilic granulomatous necrotising lymphadenitis and perinodal panniculitis with intralesional parasitic remnants and comparatively large numbers of eosinophils. A large, ~300-500 μm diameter, area of necrosis was also observed in one lymph node. This is the first time a study has been undertaken in Australia to determine the pathogenicity of tongue worms in both their definitive and intermediate hosts. This is a preliminary study and to properly estimate the health impact of infection with this pathogenic parasites on Australian production and companion animals more studies are necessary.

Introduction: Langerhans cell histiocytosis (LCH) is a rare uni or multisystem disorder associated with extreme production of immunoreactive Langerhans cells. Although this disorder has been reported in all age groups, spinal involvement especially thoracolumbar spine is seldom reported in toddlers. Case presentation: We present a one (1) year, four (4) months female child with a history of recurrent fever, irritability and severe anemia of one (1) month duration. CT-scan and MRI revealed a collapsed twelfth thoracic vertebra (T12) body height greater than 95% with a huge intradural soft tissue mass. T12 cortectomy via posterior thoracolumbar approach was used to decompress the soft tissue part followed by interbody fusion with titanium cage filled with autologous iliac crest bone graft, fixation using plates and screws. Conclusion: We are of the view that surgical decompression of spinal LHC lesions with interbody fusion with titanium cage filled with autologous iliac crest bone graft and fixation using plates and screws is very crucial in relieving neurological deficits. However, the patient will need repeated surgeries as she advances with age in a timely manner to avert any neurological deficit that may occur. Keywords: Langerhans cell histiocytosis (LCH), Letterer-Siwe disease, Hand-Schüller-Christian disease, Eosinophilic granuloma

BACKGROUND: In a recent phase III trial (NCT02020889) 53% of mepolizumab-treated versus 19% of placebo-treated patients with eosinophilic granulomatosis with polyangiitis (EGPA) achieved protocol-defined remission. OBJECTIVE: We sought to investigate post hoc the clinical benefit of mepolizumab in patients with EGPA using a comprehensive definition of benefit encompassing remission, oral glucocorticoid (OGC) dose reduction, and EGPA relapses. METHODS: The randomized, placebo-controlled, double-blind, parallel-group trial recruited patients with relapsing/refractory EGPA receiving stable OGCs (prednisolone/prednisone, ≥7.5-50 mg/d) for 4 or more weeks. Patients received 300 mg of subcutaneous mepolizumab or placebo every 4 weeks for 52 weeks. Clinical benefit was defined post hoc as follows: remission at any time (2 definitions used), 50% or greater OGC dose reduction during weeks 48 to 52, or no EGPA relapses. The 2 remission definitions were Birmingham Vasculitis Activity Score of 0 plus OGC dose of 4 mg/d or less (remission 1/clinical benefit 1) or 7.5 mg/d or less (remission 2/clinical benefit 2). Clinical benefit was assessed in all patients and among subgroups with a baseline blood eosinophil count of less than 150 cells/μL, baseline OGC dosage of greater than 20 mg/d, or weight of greater than 85 kg. RESULTS: With mepolizumab versus placebo, 78% versus 32% of patients experienced clinical benefit 1, and 87% versus 53% of patients experienced clinical benefit 2 (both P < .001). Significantly more patients experienced clinical benefit 1 with mepolizumab versus placebo in the blood eosinophil count less than 150 cells/μL subgroup (72% vs 43%, P = .033) and weight greater than 85 kg subgroup (68% vs 23%, P = .005); in the OGC greater than 20 mg/d subgroup, results were not significant but favored mepolizumab (60% vs 36%, P = .395). CONCLUSION: When a comprehensive definition of clinical benefit was applied to data from a randomized controlled trial, 78% to 87% of patients with EGPA experienced benefit with mepolizumab.

A mare presented with lameness and a large swelling palmarolateral and proximal to the right carpus. Radiography revealed mineral opacity, bone remodeling, and soft tissue swelling. At surgery, fibrous tissue was removed from the subcutaneous area and sulfur granules were removed from near the carpal canal. Histopathology revealed eosinophilic inflammation. Habronemiasis was the primary differential, although no larvae were found. The swelling resolved, and the mare was sound 1 year after initial presentation.

Background Context Eosinophilic granulomas (EGs) of the sacrum have been reported in fewer than 10 patients. Treatment algorithms for these tumors remain poorly defined; there are no reports of treating solitary sacral EG with radiation therapy (RT). Purpose This study aimed to describe the presentation, treatment, and outcome of sacral EG in an adult patient with intractable pain and radiculopathy, treated in a novel fashion with RT. Study Design/Setting The study design was a case report from a tertiary cancer referral center. Methods Patient records, imaging, and pathology were reviewed. Results A 35-year-old man received 20 Gy of radiation to his S1 EG lesion. He subsequently developed vertebra plana of S1 causing symptomatic L5–S1 stenosis, but 15 months after RT treatment was free of pain or tumor recurrence. Conclusion Radiation therapy is an effective treatment option for sacral EG causing severe axial pain and neural impingement.

The thoracic cage provides the structural support that makes respiration possible, provides protection to vital organs such as the lungs, heart, liver, and spleen, and serves as an anchor point for the upper extremities. Neoplasms of the bony thorax are not an uncommon incidental finding at both radiography and cross-sectional imaging. Some tumors have a characteristic appearance and it is important that an accurate differential diagnosis be provided. Misidentification could lead to unnecessary imaging or procedures with associated cost, morbidity, and mortality. The purpose of this article is to serve as a quick review of bone tumors commonly encountered in the thorax and that every radiologist should know. Please note that there are also several non-neoplastic osseous lesions that may mimic bone tumors such as osteomyelitis and eosinophilic granuloma; however, these entities are beyond the scope of this review and would not be discussed.

Background context Benign tumors of the vertebrae are generally an uncommon cause for surgery. Complete removal of these tumors requires in most cases extensive surgical technique that consists of generous surgical exposure followed by laminectomy, facetectomy, and sometimes even an instrumented fusion. Purpose The aim was to describe our experience in performing resection of benign vertebral tumors, using a minimally invasive surgical (MIS) approach. Study design This was a retrospective review of case records. Patient sample Patients who underwent MIS, resection of benign vertebral tumors. Outcome measures Complete neurologic examination and pain evaluation, as measured by the visual analog scale (VAS). Secondary outcomes included postoperative spinal instability assessment and surgical margins examinations. Methods Patients were evaluated preoperatively and postoperatively at 1, 3, and 6 months intervals clinically and radiographically using plain radiographs and postoperative computed tomography (CT) scans. Final pathologic report, operative time, blood loss, complications, and hospital length of stay were also recorded. Results Between 2009 and 2013, 14 patients underwent MIS, resection of benign vertebral tumors at our institution. Mean follow-up time was 4 years. There were eight men and six women with a mean age of 27 years (range 16–68 years). For tumors located in the posterior elements, a direct posterior approach was used. Tumors located at the pedicle of the vertebra were excised using a transpedicular approach, and tumors protruding into the foramen were excised using the transforaminal approach. The transcanal approach was used when decompression of the thecal sac or nerve root was required, and the retroperitoneal transpsoas approach was used for tumors located in the vertebral body. Complete removal of these tumors was achieved in all cases, and was verified by a follow-up CT scan. Pathology revealed osteoid osteoma in five patients, osteoblastoma in three patients. Eosinophilic granuloma, fibrous dysplasia, and fibroid adenoma were found in one case each. Average VAS pain score improved from 7.7 (7–9) to 2.8 (0–7) after surgery. Conclusions Minimally invasive techniques are a valuable choice for the treatment of benign osseous tumors of the spine. A larger, long-term study is in progress. In the meantime, we suggest surgeons experienced with both open and MIS surgery should consider these techniques.

Background context Eosinophilic granuloma (EG) involving the spine is uncommon in adults. Atlantoaxial instability (AAI) secondary to EG of the axis in adults is an extremely rare clinical condition that can give rise to severe neurologic morbidity or mortality if not treated appropriately. There have been no previous reports on the condition in adults. Purpose To present the outcome and clinical experience for the management of AAI secondary to EG of the axis in adults. Study design A retrospective review study. Patient sample All adult patients with AAI secondary to EG of the axis who were admitted to the spine service at the study institution between January 1999 and April 2012. Outcome measures Clinical symptoms, neurologic status, radiologic manifestations, treatment, outcome, and/or complications were recorded and analyzed. Methods Six consecutive adults who presented clinical and radiographic manifestations of AAI secondary to EG of axis were treated and monitored. All patients were treated surgically with anterior tumor resection and posterior reconstruction of spinal stability. Oral steroid therapy was administered after surgery as adjuvant therapy. Results The mean duration of follow-up was 77 months (range, 37–140 months). The most common radiographic feature was osteolytic destruction of the vertebral body of the axis. All patients had favorable recoveries, with osseous fusion. There were no surgery-related postoperative complications, and neither recurrence nor spinal deformity had occurred by the final follow-up examination. Conclusions Surgical intervention via anterior tumor resection and posterior reconstruction was found to be safe and effective for treating AAI secondary to EG in adults, in terms of recovering neurologic function, improving symptom relief, and reducing the risks resulting from osteolytic destruction. Surgical treatment plus oral steroid therapy can produce beneficial results and definitive local control during the follow-up period.

compressed the left bronchial tree. The tumor completely surrounded large blood vessels and the heart, as confirmed by echocardiography. The systolic function of the heart was preserved. Flexible fiberoptic bronchoscopy (FFB) under sedation determined that the lumen of the left bronchial tree was narrowed to one-third of its normal size due to external compression. The type of the tumor was not determined by transtracheal and ultrasound-guided transthoracic puncture, and the patient presented for biopsy. The patient was ASA class IV due to position and extension of the tumor. The high risk of the procedure including TPVB was explained to the patient, and she signed an informed consent for both surgery and the nerve block. Methylprednisolone, pantoprazole, cefazolin, and 5 mg of oral diazepam were administered preoperatively. It was elected to use TPVB repeated at each dermatome level. With the patient in a sitting position, 8 mL of 2% lidocaine was administered subcutaneously. Using a 10-cm long 22-gauge Tuohy spinal needle and a loss of resistance technique, single punctures of the 4 left paravertebral spaces from T2-T5 were performed. After careful aspiration, 5 mL of 0.5% bupivacaine per segment was administered. 10-13 The onset of sensory loss occurred approximately 25 minutes after the injections. Before starting the incision, 0.05 mg of alfentanil and 1 mg of midazolam were administered intravenously, and 100 mg of 2% lidocaine was injected under the skin. The patient was lying on her back but in the right semilateral position, and the surgical access was an anterior mediastinotomy carried out through the third left intercostal space. A 5-cm transverse parasternal skin incision, just lateral to the sternal border, removing the underlying costal cartilage, was used. Electrocautery was used to divide intercostal muscles, and after removing costal cartilage, the internal mammary vessels were ligated. Abundant biopsy specimens were taken. Excellent analgesic effect was achieved. During the operation, the patient was awake, did not experience pain, and was hemodynamically stable and spontaneously breathing. After surgery, she was observed for 24 hours. The pathohistologic examination was unable to determine the precise type of the tumor, and the procedure was repeated 5 days later. Under TPVB, the wound was reopened, and several large biopsy specimens were taken. The postoperative course was uneventful after both procedures. The pathologic diagnosis was eosinophilic granuloma.

Mycetoma is a chronic granulomatous infection of skin and subcutaneous tissue, which may involve bone. The disease is caused by true fungi (eumycetoma) or filamentous bacteria (actinomycetoma). Both types of organism are important soil saprophytes, and therefore infection is normally acquired by traumatic inoculation of the skin by contaminated material. Clinically, mycetomas are characterized by tumefaction, draining sinuses and discharging grains. The grains are aggregates of fungal hyphae or bacterial filaments, and grain size, colour and consistency provide the initial clue to the causative organism. To our knowledge, this probably is the first case report of oral eumycetoma of infancy. Review of literature shows only ten cases of eumycetoma involving the head and neck, including the present case. Histological examinations of the biopsies were the cornerstone in the diagnosis of mycetoma. The distinction between eumycetoma and actinomycetoma is essential for treatment. Direct microscopic examination of the grain and histopathological examination, using histochemical staining will distinguish between the two. Treatment consists of long courses of antifungals and antibacterials agents, often combined with surgery.

Information pertaining to parasitic fauna and parasitic diseases in Manipuri ponies in India is not available. Moreover, no systematic studies have been undertaken on cutaneous habronemosis in Manipuri ponies which is a common skin problem of Manipuri ponies as reported by pony owners. Keeping in the view of the importance of parasitic infections in veterinary health coverage particularly in Manipuri ponies, the present study was planned. A survey of natural cases of cutaneous habronemosis followed by molecular confirmation of species involved and treatments were done. Out of 200 ponies examined, nine cases (4.5%) of cutaneous habronemosis was recorded. Gross examination revealed raised and ulcerated wounds with necrotic tissues covered with yellowish-tan granulation. Histopathological study revealed eosinophilic granuloma and in the center of the granuloma with necrotic debris. Remnants of the Hebronema larvae with infiltrating neutrophils surrounded by proliferating fibrous tissue with numerous eosinophils, macrophages and lymphocytes were also observed. Molecular detection of Habronema sp. was confirmed by semi-nested PCR. Sequence analysis revealed larvae of H. muscae was the common spirurid species responsible for producing cutaneous habronemosis in Manipuri ponies. Subsequently, sequence submitted to NCBI GenBank and accession number obtained (MH038181). Surgical removal of necrotic tissue, ivermectin injection along with antibiotics successfully cured all the lesions in infected ponies.Results confirmed occurrence of cutaneous habronemosis in Manipuri ponies in India.

Backround: This retrospective study aimed to determine the frequency of bone tumours of the clavicle and their histopathological, anatomical and epidemiological characteristics in a large case series. Methods: The records of 327 lesions of the clavicle collected from 1976 to 2018 in our bone tumour registry and institute of pathology were reviewed. Following data were evaluated: age, gender, side, radiological assessment, tumour location within the clavicle, and histopathological findings. Results: Bone tumours were detected in 113 patients with a mean age of 40 years. The lateral third of the clavicle was most frequently involved. Analysis revealed 22 benign, 31 intermediate, and 60 malignant tumours. Eosinophilic granuloma was the most commonly found neoplasm (18.6%), followed by bone metastases (15.0%), Plasma cell myeloma (8.8%), Ewing sarcoma (8.8%), and Osteosarcoma (8.0%). 53% of the tumours were malignant. Mean age was 51 years in the malignant tumour group and 28 years in patients with a benign/intermediate lesion (p

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. Herein, we describe the clinical and histologic spectrum of LCH of the orbit on the basis of five cases. One patient exhibited multifocal unisystem disease, the other four patients presented with a localized process. The typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunostaining. Transmission electron microscopy exhibited characteristic intracytoplasmic Birbeck granules. The different ophthalmic manifestations of LCH and treatment strategies are reviewed in the context of previously reported cases. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH has to be included in the differential diagnosis in tumors of the ocular adnexae, in particular in young children.

Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is a rare disorder in which bone marrow– derived pathologic cells infiltrate and destroy tissue. It has traditionally been recognized to present as Letterer-Siwe disease (acute disseminated multisystem disease occurring in infants), Hand-Schuller-Christian disease (chronic disseminated disease arising in children), and eosinophilic granuloma (disease limited to bones, occurring in adolescents and young adults). 1 This schema remains useful, but it is an oversimplification because infants can have single-system self-healing skin disease and adults can have multisystem life-threatening disease. 2,3 We report the case of a 22-month-old boy with disease simultaneously presenting in the maxilla and mandible.

Langerhans’ cell histiocytosis is a collective term used to describe a group of enigmatic, proliferative disorders. The natural history of the disease varies from a slow, benign, localized, symptomatic bony or soft-tissue lesion, to a rapidly, progressive, widespread, multiple-organ disorder which is often fatal. Eosinophilic granuloma (EG) accounts for 60–70% of all cases of Langerhans’ cell histiocytosis and can present as solitary (50–75%) or multifocal defects in bone. It occasionally presents as a localized soft-tissue lesion. Multiple treatment options are available but the response is unpredictable. However, no studies show complete resolution following a conservative modality of treatment in the management of EG of condyle and ramus. We describe a case, treated with intralesional injections of triamcinolone acetonide (Tricort 40 mg/ml, 6 injections at weekly intervals over 6 weeks), leading to complete resolution of the lesion, along with a brief mention of the review of literature.

Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100,000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology.

Primary bone tumours of the growing spine are rare and most are benign. However there is a potential for local aggressiveness and malignancies do occur. Surgical resection is the mainstay of treatment and is usually very challenging due to the complexity of local anatomy and proximity to neurovascular structures. In this review we discuss the overall approach to primary bone tumours of the growing spine, focussing on the most common subtypes of benign and malignant tumours seen in this subset.

The eosinophilic granuloma of bone is the most common type of histiocytic disorder involving the orbital area. Imaging data typically show bony defects with an intra-orbital soft-tissue extension. Surgical debulking is most often required. Rosai-Dorfman disease is commonly associated with uni- or bilateral orbital locations. The other histiocytic disorders are very rare, but some have a poor prognosis such as Erdheim-Chester disease.

Summary Bilateral temporal bone involvement of eosinophilic granuloma is extremely rare. It has been estimated that approximately 22 cases have been reported so far in the world literature. In many cases, the symptoms are misinterpreted as otitis media, cholesteatoma, or mastoiditis. We report a case of bilateral temporal eosinophilic granuloma with otic capsule involvement, who was initially misdiagnosed and treated for non-specific otitis media. After mastoidectomy and chemotherapy with vinblastine and prednisone the patient's bony lesions of the temporal bone incompletely reossified and labyrinthine function was not satisfactory. Children with otitis media or mastoiditis with no response to medical treatment should be evaluated with Computerized tomography (CT) scanning of the temporal bone.

Summary Introduction Eosinophilic granuloma in the jawbones can be confused with aggressive periodontitis or more rarely with inflammatory lesions of dental origin. We had for objective to analyze the various elements of clinical, radiological, and pathological differential diagnosis for this rare lesion through a clinical observation. Case report A 26-year-old male patient consulted for loose teeth and gingivitis. The initial diagnosis was aggressive periodontitis. The ineffective periodontal treatment suggested another diagnosis, eosinophilic granuloma, requiring tooth extraction and curettage. A histological analysis confirmed the diagnosis. Bone scintigraphy revealed a second focus on the left maxilla. Complementary chemotherapy was efficient on the mandibular site but failed to prevent worsening on the maxilla, which was treated surgically. Thirty months after, the patient's condition was stable. Discussion The diagnosis of eosinophilic granuloma is difficult and relies on histology and immunolabelling with protein S100 and antigen CD1a. Treatment is surgery and conservative in case of isolated lesions.

Objectives To present a series of eight cases of rare sacral tumors that represent a wide spectrum of the aggressive disease involving the sacrum and to discuss the efficacy of CT-guided biopsy in these cases. Material and methods We reviewed all cases of sacral tumors biopsied under CT guidance in the last two years. Prior CT and MR images were analyzed before biopsy, and 3 mm CT slices of the lesion were obtained to determine the location of the target area and the path of the needle. All biopsies were performed using 11 G needles and 15 G coaxial needles. Results Eight sacral lesions were biopsied under CT guidance, and a diagnosis was reached in four. In three of these, the diagnosis (chordoma, plasmocytoma, and metastasis from melanoma) was reached in a single session. In the fourth case, three sessions were necessary to reach the diagnosis of Ewing's sarcoma. For various reasons, the remaining four cases required surgical biopsy to reach the diagnoses of chondrosarcoma, eosinophilic granuloma, and primary bone lymphoma in two patients. The diagnostic efficacy of CT-guided biopsy was 36%. Conclusion Our results suggest that CT-guided biopsy is less useful in the characterization of primary tumors than in metastases. The diagnosis of primary bone lymphomas is especially complicated. Repeating the procedure after inconclusive results probably will not provide additional information, and it is recommendable to perform surgical biopsy in these cases.

Langerhans cell histiocytosis is a systemic disease resulting from the oligoclonal proliferation of Langerhans cells, occurring most commonly in children and young adults. The focal form of the disease, also known as eosinophilic granuloma, most frequently involves the calvaria. We present two cases of calvarial eosinophilic granulomas that were surgically removed. These tumors are reputed to have an excellent prognosis, even if local recurrences and systemic dissemination can occur during the follow-up. The authors discuss the pathogenesis and the evolutive profile but also the therapeutic management of solitary eosinophilic granuloma of the calvaria.

Vertebra plana is a concept introduced by Calve in 1925 to name a process characterized by the collapse of a single vertebra with no involvement of the discs, widening of the intervertebral space of at least one third compared with normal width, and an increase in the density of the collapsed vertebra. Because of its benign and self-limiting nature, this entity was originally thought to be an osteochondritis. Later publications did not corroborate this view and connected this entity initially with eosinophilic granuloma and subsequently with additional malignant infiltrating tumoral processes, in particular with Ewing's sarcoma and lymphomas, thus posing difficulties for its management. The initial concept was extended in 1990 to crushed and wedged vertebrae, including developmental abnormalities such as bone dysplasias. The present article provides a general review of vertebra plana, including the concept, etiology and pathogenesis of related processes, clinical findings and diagnosis. We also discuss the debate on the benign or malignant nature of vertebra plana and on the need for an immediate biopsy versus observation, as well as the therapeutic orientations.

Langerhans cell histiocytosis (LCH) is an uncommon disease encompassing three clinically different entities: eosinophilic granuloma, Hand-Schüller-Christian disease, and Abt-Letterer-Siewe disease. Despite usually being a multisystemic disease affecting numerous different organs, involvement of the thyroid gland is extremely rare, and only a few cases in adults have been described in the literature. Herein, we present the case of a 28-year-old male patient presenting with LCH involving the skin, the skeletal system, and the thyroid gland. Fine needle aspiration (FNA) of the thyroid was performed and showed the typical Langerhans cells (LC) with foamy cytoplasm and slender nuclei with longitudinal grooves against a background of inflammatory cells with only a few eosinophilic granulocytes. Immunocytochemically, the LC showed positive staining with antibodies against CD1a and Langerin, a recently detected glycoprotein exclusively expressed in LC. Langerin is the major protein that makes up the so-called Birbeck granules, the electronmicroscopical hallmark of LC. Since LCH involvement of the thyroid is occasionally mistaken for papillary thyroid carcinoma cells, we propose that application of Langerin in combination with CD1a is a helpful diagnostic adjunct for the correct assessment of LCH affecting the thyroid gland.

Purpose: The present study presents 22 cases of pediatric patients harboring an eosinophilic granuloma (EG) of the skull. Methods: Twenty-two patients (13 males, 9 females; mean age, 7.5 years; range, 3-14 years) with a suspected diagnosis of a cranial EG were enrolled in the study. They all had been preoperatively evaluated by skull x-ray and computed tomography, whereas 10 patients additionally underwent magnetic resonance imaging. To rule out a multifocal disease, scintigraphy was performed in all cases preoperatively. Surgical excision of the lesions was performed, and EG was proven histopathologically. Results: There was a male predominance. Frontal bone was the most common affected bone. One patient had a multifocal disease. Total excision of the lesion was performed in 19 of 22 patients. No patient received postoperative radiotherapy. In the remaining 3 cases because of the periorbital localization of the EG and the subsequent risk of disfigurement, only a biopsy was performed. These patients were treated with sulfamethoxazole and trimethoprim for 6 months. All 3 lesions were resolved. The follow-up examinations ranged from 6 months to 17 years, with a mean follow-up of 6.2 years, and no tumor recurrence was noted. Conclusions: We conclude that EG is a benign disease. At the time of diagnosis, a bone scan should be performed to rule out a multifocal disease. Surgical resection is the treatment of choice. Nevertheless, for lesions in which excision can cause cosmetic defects, administration of sulfamethoxazole and trimethoprim after biopsy appeared to be an effective alternative treatment.

Spontaneous healing of large bone defects in the mandibles of 33 patients was studied. Standard postoperative clinical and radiographic examinations were performed immediately after surgery and after 2, 6 and 12 months. They were analysed using a novel relative bone densitometry method and indexes of relative bone healing were established. Spontaneous bone regeneration occurred in all patients clinically, and the computer analysis of radiographs showed that the mean final bone density in the bone defects was 88% of the bone density of the surrounding healthy bone. In the cases of smaller defects (the greatest diameter on panoramic radiographs was 20-30 mm) the final bone density was 97%, while the larger defects finally healed with 84% of the bone density of the surrounding bone. Increased patient age had a negative influence on healing and the shape of the bone defects was more important for healing than their volume. Spontaneous bone healing occurred even in large bone defects in the mandible, therefore this simple treatment with low economic and biological costs should be the treatment of choice, taking into account the patient's age, surgical principles and time of rehabilitation.

Summary The clinical spectrum of Langerhans cell histiocytosis (LCH) can be very diverse. Pathology can range from a single bony lesion to systemic disease with multi-organ dysfunction. Up to 61% of patients with LCH have otologic involvement and in 5–25% it might be the initial form of presentation [A.J. Goldsmith, D. Myssiorek, E. Valderrama, M. Patel, Unifocal Langerhans’ cell histiocytosis (eosinophilic granuloma) of the petrous apex, Arch. Otolaryngol. Head Neck Surg. 119 (1993) 113–116; R. Hermans, B. De Foer, M.H. Smet, J. Leysen, Eosinophilic granuloma of the head and neck: CT and MRI features in three cases, Pediatr. Radiol. 24 (1994) 33–36; T.V. McCaffrey, T.J. McDonald, Histiocytosis X of the ear and temporal bone: review of 22 cases, Laryngoscope 89 (1979) 1735–1742]. The otologic findings of LCH are similar to otomastoiditis and therefore LCH should be considered as a possible differential diagnosis in very young patients presenting with signs and symptoms suggestive of chronic otitis media.

Langerhans' histiocytosis or histiocytosis X is a rare intrinsically benign disease producing a destructive tumor with a variable clinical presentation and an often unpredictable clinical course. Focal forms such as eosinophilic granuloma of the bone only require minimal care but the gravity of multisystem forms causing organic dysfunction sometimes require aggressive chemotherapy. Bone involvement is generally observed in children mostly boys. Both sporadic and chronic forms are noted. We report a case observed in a 17-year-old adolescent who presented an exceptional association of bony destruction of the pelvis with extended asymptomatic pulmonary involvement. The lung disease led to the initial diagnosis and optimal surgical, pathological and radiological management.

Purpose To report 3 patients with adult-onset orbital Langerhans cell histiocytosis (LCH). Design Retrospective interventional case reports. Participants Three adult patients with orbital LCH. Methods Case reports from 2 different clinics and review of the scientific literature. Main Outcome Measures Clinical course and radiologic and histologic findings. Results Three healthy patients, aged 20 to 36 (mean, 27) years, presented with biopsy-proven unilateral orbital LCH. The initial presenting complaints were mainly orbital pain and headache with or without orbital swelling and minimal effect on ocular movements. There was radiologic evidence of disease centered on the greater wing of sphenoid bone in all cases. Biopsy via lateral canthotomy was performed with LCH confirmed based on histologic and immunohistochemical findings. There was no evidence of disease elsewhere on systemic investigations. One patient had curettage and intralesional corticosteroids, 1 patient had a craniotomy for more complete resection of the lesion, and the third patient was referred for radiotherapy. All patients achieved complete resolution of symptoms and signs. None of the patients demonstrated any evidence of distant or recurrent disease at follow-up. Conclusions Orbital LCH in adults is a rare, usually unifocal, and relatively benign disease most commonly affecting the greater wing of sphenoid bone, where there is still active bone marrow in young adults. There is no consensus on cause or therapy, but our reported cases show that several treatment modalities are effective.

Resume L'histiocytose de Langerhans est une pathologie rare de l'enfant et de l'adulte jeune qui provoque aussi bien des lesions focales que des atteintes de plusieurs organes mimant une vascularite ou une hemopathie maligne, par proliferation et accumulation tissulaire d'histiocytes. L'atteinte osseuse ou granulome eosinophile est la plus frequente. Les circonstances de decouverte de telles lesions sont le plus souvent la douleur, une tumefaction, une fracture, fievre et parfois otorrhee, hypoacousie ou exophtalmie en cas d'atteinte des os de la face. Les radiculalgies sont rarement decrites meme en cas d'atteinte du squelette axial. Nous reportons quatre cas de radiculagies liees a une histiocytose langerhansienne : deux cruralgies tronquees associees a une atteinte cotyloidienne chez deux hommes de 25 et 34 ans, ainsi que deux lombosciatiques chez une jeune femme presentant une atteinte corporeale de L5 et chez un homme de 41 ans chez qui existe une localisation sacree.

Eosinophilic granuloma (EG) is the localized and mildest form of histiocytosis-X group of diseases. In this study, 22 patients of pathologically proven EG involving the jaws were retrospectively studied concerning the clinical and radiographic presentation. The clinical and radiographic presentation of the 22 cases was quite varied, and the accuracy of clinical and radiographic diagnosis was low. However, the clinical and radiographic presentation correlated with the lesion site, and accordingly all the jaw lesions could be divided into three types (Alveolar Type, Intraosseous Type, and Mixed Type). Our classification based on the lesion sites reveals the correlation between the clinical and radiographic presentation of EG and the lesion sites, and may be useful in the identification of lesions of EG in the jaws.

Summary Langerhans’ cell histiocytosis (LCHs) or histiocytosis X is an idiopathic disorder characterized by a proliferation of histiocytes. It is generally considered to include a spectrum of disorders as eosinophilic granuloma (EG), Hand–Schuller–Christian (HSC) disease and Letterer–Siwe (LS) disease which vary in the prognostic result. EG is the most benign of them. The histiocytic lesions can occur in visceral organ(s) or bone(s) that can lead to presentation of the disease with different clinical manifestations including otologic manifestations. The presence of otorrhea or atypical otitis media, granulation tissue, aural polyps, or a postauricular mass should alert the otolaryngologist to the possibility of LCH. We presented a case of otologic histiocytosis with the temporal bone involvement which mimics as chronic infectious ear disease.

Objectives To report a patient with a rare case of orbital eosinophilic angiocentric fibrosis (EAF) and to review the literature. Design Interventional case report. Methods A 61-year-old man presented with a 6-week history of right periorbital edema and painless proptosis. Examination revealed a nonaxial proptosis, lateral globe displacement, and mild limitation in right eye adduction. Main Outcome Measures Clinical course and radiological and histological findings. Results Orbital imaging revealed a right medial orbital mass with involvement of middle ethmoidal air cells. An orbital biopsy of the mass demonstrated an inflammatory infiltrate with a marked eosinophilic component, onion skinning of vessels, and surrounding fibrosis. The diagnosis of orbital EAF was made. There was no response to a 3-month treatment course with systemic steroids, but the patient did not want any further surgical interventions. Conclusion Although orbital EAF is rare, ophthalmologists need to be aware of this entity, as it may invade the orbit from the sinonasal tract or present as a localized orbital mass. The presence of even minimal sinus involvement and the characteristic histopathology are useful in establishing the correct diagnosis.

Resume L'histiocytose langerhansienne est une affection rare en pediatrie. Elle a un spectre clinique tres divers, allant du simple granulome eosinophile a la forme grave multiviscerale. Observation. – Nous rapportons une observation de granulome eosinophile chez un enfant âge de huit ans revele par un torticolis. Le bilan radiologique realise a evoque le diagnostic. La confirmation a ete anatomopathologique. L'evolution a ete spontanement favorable avec un recul de 2,5 ans. Conclusion. – Un torticolis doit etre explore et surveille. Le granulome eosinophile peut etre une causee qui est rare chez l'enfant et d'evolution souvent spontanement favorable.

Background Only case reports or studies of small series of patients have focused on granuloma faciale (GF). Objective We sought to describe the clinicopathologic characteristics of GF in a large series of patients. Methods We conducted a retrospective analysis of 66 patients and 73 skin specimens. Results GF mostly presented as reddish plaques or nodules in middle-aged adults. One third of patients had multiple sites involved and 5 patients had extrafacial lesions. A clinical diagnosis of GF was made in only 10 cases; sarcoidosis, lymphoma, lupus, and basal cell carcinoma were the main differential diagnoses. The most frequent histopathologic features were the presence of a grenz zone, neutrophils, and telangiectases. Vascular changes were frequent, although necrotizing vasculitis appeared to be rare. There was often an association of acute and chronic inflammatory patterns, suggesting that GF follows a chronic history with reccurrent acute phases, rather than distinct successive acute and chronic stages. Limitations In this retrospective study, a reliable analysis of the outcome of patients could not be performed. Results of direct immunofluorescence tests and laboratory investigations were lacking in many cases. Conclusion GF is often clinically misdiagnosed; its morphologic spectrum is broader than usually described and includes a lack of eosinophils, the presence of vascular changes with rare vessel wall necrosis, and associated acute and chronic inflammatory patterns.

But L’histiocytose a cellules de Langerhans est une pathologie rare qui represente moins de 1 % de la pathologie tumorale de l’orbite. Methodes Le cas presente ici est celui d’un garcon de 9 ans qui a presente une tumefaction inflammatoire de l’angle supero-externe de l’orbite gauche. Une tomodensitometrie a ete realisee revelant une masse tissulaire intraorbitaire a l’origine d’une osteolyse du toit de l’orbite avec extension intracrânienne. Resultats Une biopsie transpalpebrale de la tumeur realisee a mis en evidence une proliferation d’histiocytes derivant des cellules de langerhans avec, a l’etude immunohistochimique, positivite a la proteine S100 et de l’anticorps anti-CD1a, permettant la confirmation du diagnostic. Devant une augmentation rapide du volume tumoral, accompagnee d’une menace de compression du globe oculaire et de l’extension intracrânienne, il a ete instituee une chimiotherapie (vinblastine) associee a une corticotherapie. L’evolution a ete favorable. Apres un recul de deux ans, on n’a observe ni dissemination, ni recidive. Conclusion Le cas de ce patient, suivi au service d’ophtalmologie du CHU de Rabat, permet de decrire les manifestations cliniques, anathomopathologiques de l’histiocytose a cellules de Langerhans dans sa localisation orbitaire.

Langerhans' cell histiocytosis (LCH) can involve virtually any site and organ of the body, either as an isolated lesion or as a widespread systemic disease. Bone can be involved as a part of generalised disease or as a separate entity, formerly known as eosinophilic granuloma. The clinical cause of localised LCH (LLCH) to bone is generally benign. Solitary bone lesions may resolve spontaneously after diagnostic biopsy in a period of months to years. If treatment is indicated, as in larger lesions with pain, swelling and risk for spontaneous fracture, the disease can be controlled by chemotherapy or local measures such as surgical curettage, low-dose radiotherapy and intralesional injection of corticosteroids. There are no controlled studies in the literature comparing outcomes of these treatment modalities because of low incidence of the disease and general tendency to regress. Three patients (female, 28 months; male, 9 years; male, 15 years) with LLCH of the mandible were treated in an one stage procedure with intralesional injection of 80, 40 and 80 mg methylpredisolone succinate, respectively, as the primary form of treatment. Patients were seen for clinical and radiological evolution 1, 3, 6, 9 and 12 months after treatment, and yearly thereafter. The overall outcome was excellent. The lesions showed clinically and radiologically complete remission approximately 6 months after treatment. There were no complications nor morbidity of the treatment. After a follow-up period of 12 months, the patients are well with no evidence of residual disease on radiographs. Local injection of corticosteroids should be the initial choice of treatment of LLCH of the mandible, because of the favourable treatment outcome in this disease and possible complications and lasting effects of surgery, radiotherapy and chemotherapy.

Objective To discuss a case of unifocal Langerhans cell histiocytosis (eosinophilic granuloma) presenting as an aggressive osseous destructive process. Clinical Features A 6-year-old girl had thigh pain of insidious onset that would wake her at night. Orthopedic testing reproduced local pain, and imaging revealed an osteolytic process in the femoral diaphysis with laminated periosteal reaction. Intervention and Outcome The patient was referred for further evaluation; unifocal Langerhans cell histiocytosis was found at biopsy and spontaneous healing subsequently reported. Conclusions Unifocal Langerhans cell histiocytosis may present with deceptive clinical and imaging findings, of which clinicians should be aware.

Background Granuloma faciale is an uncommon disease of unknown etiology that is often misdiagnosed clinically and by general pathologists. Objective To describe the clinicopathological features of a series of patients with granuloma faciale. Methods Eleven patients diagnosed with granuloma faciale between 1990 and 2002 were included in the study. Results Granuloma faciale was diagnosed in 11 patients (9 male and 2 female, mean age 53.45 years). All of them presented facial cutaneous lesions and two of them also developed extrafacial lesions. Histologically, in 8 cases the infiltrate was limited to the upper half of the dermis. Two specimens showed fibrinoid necrosis. Concentric fibrosis around small blood vessels was demonstrated in 5 patients. Conclusion The presence of abundant fibrosis in 5 of our patients similar to that observed in erythema elevatum diutinum suggests that granuloma faciale and erythema elevatum diutinum may be produced by similar or the same pathogenic mechanism.

Introduction: Langerhans’ cell histiocytosis is a collective term used to describe a group of enigmatic proliferative disorders. The natural history of the disease varies from a slow, benign, localized symptomatic bony or soft tissue lesion, to a rapidly progressive widespread multiple organ disorder which is often fatal. Eosinophilic granuloma accounts for 60–70% of all cases of Langerhans’ cell histiocytosis and can present as solitary (50–75%) or multifocal defects in bone. It occasionally presents as a localized soft tissue lesion. There are multiple treatment options but the response is unpredictable. Aims: We present three separate cases, of the maxillofacial skeleton where the lesions of eosinophilic granuloma resolved following incisional biopsy only. Patients and results: Three patients presented with solitary lesions of the maxillofacial skeleton. All were diagnosed as Langerhans’ cell histiocytosis following open curettage, which also resulted in resolution of the lesions. Follow-up has thus far been disease free. Conclusion: For some solitary Langerhans’ cell histiocytosis lesions, simple curettage is the only treatment required. The paper discusses the need to confirm the solitary nature of the disease and the need for follow-up. Reviewing the literature on the disease, the authors suggest that perhaps cellular immaturity holds the cells of the lesion in a disease state until pushed to maturity by the trauma of open curettage surgery, resulting in a complete resolution of the disease.

We present five cases of nodular histiocytic/mesothelial hyperplasia (two peritoneal, two pulmonary, and one pericardial) with identical microscopic features. All the lesions were biphasic and composed of cohesive monotonous epithelioid clusters of polygonal or oval cells with round or deeply grooved nuclei in association with darker cuboidal cells. Because of the increased cellularity and monotonous histologic pattern with some degree of cytologic atypia, neoplastic processes were seriously considered in the differential diagnoses. The majority of the cells marked as histiocytes by immunostain. A few scattered individual cells or small epithelial cell clusters were confirmed by calretinin stain to be mesothelial cells. The histologic patterns of the current lesions, irrespective of the location, were identical to nodular histiocytic/mesothelial hyperplasia. Histiocytic proliferations can be erroneously confused with primary mesothelial lesions or neoplasms such as granulosa cell tumor, eosinophilic granuloma, chronic myelogenous leukemia, and carcinoma. The purpose of this article is to describe the clinicopathologic features of nodular histiocytic/mesothelial hyperplasia and help familiarize pathologists with this lesion to prevent an erroneous diagnosis, particularly when it occurs in locations where mesothelial cells are not normally present.