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Inflammatory fibroid polyps (IFP) were described by Vanek 60 years ago as "submucosal granuloma with eosinophilic infiltration". IFP represent polypous proliferations of spindle cells in the submucosa and mucosa of the stomach, small bowel and colon with inflammatory infiltration. The lesions have been regarded as inflammatory and reactive. Recent data show that the spindle cells express PDGFRA, and the majority of IFP harbour activating PDGFRA mutations. Therefore, IFP represent true benign mesenchymal tumors of the gastrointestinal tract.

Langerhans cell histiocytosis is characterized by a clonal proliferation of Langerhans cells. The clinical manifestation varies from a localized lesion (eosinophilic granuloma) to a systemic disease. The diagnosis can only be confirmed histopathologically. A comprehensive staging is necessary to determine the extent of the disease and to establish an adequate therapy.We report on a 27 years old patient who was referred to our clinic with the diagnosis of an osteomyelitis of the mandibular angle and a pathological fracture after extraction of tooth 38 one month before. Curettage and primary bone grafting were performed. In the histological examination of the specimen infiltrates of a Langerhans cell histiocytosis were found. The clinical and radiological staging demonstrated a solitary mandibular lesion (eosinophilic granuloma). After wound healing a low-dose radiotherapy with 6 Gray was performed. Two years after completion of the therapy the patient is asymptomatic and does not show any evidence of recurrence.Langerhans cell histiocytosis has to be included in the differential diagnosis of osteolytic lesions of the mandible. A low-dose radiotherapy is a reasonable and well-tolerated treatment option.

Three patients with a rare extrafacial granuloma eosinophilicum are presented. Lesions were localized on the scalp, the upper back or the upper arm. Only one patient had a typical facial granuloma eosinophilicum at the same time. Diagnosis was established by histopathology. The histopathological findings vary with the age of the lesion. Early lesions are characterized by a vasculitis with many eosinophils separated by a Grenz zone from epidermis and follicular structures. With time the inflammation changes and hyalin fibrosis takes place.

Eosinophilic granuloma, Hand-Schüller-Christian disease an Letterer-Siwe disease are characterised by ideopathic proliferation of histiocytes producing focal or systemic manifestations. Definitive diagnosis of histiocytosis is made by histopathology including immunohistochemical detection of S-100 and CD1a antigens. In general these diseases are summarised under the term Langerhans-cell histiocytosis (LCH). The localised form of LCH, in which the disease is limited to bones, lymphatic nodes or the lung, is commonly referred to as eosinophilic granuloma. Surgical excision, radiotherapy and chemotherapy, either alone or in combination, are the main treatment options.We present the case of a nine-year old boy with an extended eosinophilic granuloma of left temporal bone. The patient was submitted to a chemotherapeutic protocol with glucocorticoids, vinblastine and etoposide. Until today a successful treatment and a complete remission for one year can be reported.Unifocal eosinophilic granuloma is usually treated by local excision and low-dose irradiation. However treatment with the chemotherapeutic protocol offered an excellent alternative avoiding extensive surgical destruction of the temporal bone in this case.

Cystic processes are changing the course of the inferior alveolar nerve in the mandible. This study evaluates the possibility of demonstrating the relationship between space-occupying processes and the course of the neurovascular bundle.Thirteen patients with cystic processes in the mandible (9 keratocystic lesions, 1 eosinophilic granuloma, 1 plasmocytoma, 2 adamantinomas) were examined by MRI (1.5-T magnet, 8-cm surface coil, PD-gradient-echo-sequences in sagittal and coronal orientation, without enhancement) and the results retrospectively evaluated.The entire course of the nerve could be delineated in all patients. In six patients with minor cystic processes, the nerve was identified in both sagittal and coronal orientation. In seven patients with major cystic lesions, only parts of the nerve were detected in either image orientation, but the nerve could be visualized in its entire length by evaluating coronal and sagittal images side by side.It is possible to delineate the inferior alveolar nerve in its entirety along pathologic mandibular lesions. For large cystic lesions, this requires the evaluation of both coronal and sagittal sections of multidirectional MRI.

The indications for surgical treatment of benign bone tumors and tumor-like lesions depend on the biological activity, clinical symptoms and anatomic location of the the lesion. Clinically silent lesions ("leave me alone Lesions") lacking any scintigraphic enhancement and without danger of pathologic fracture or malign transformation only need surveillance and do not require surgery.However, tumors becoming symptomatic, displaying progression after termination of skeletal growth or radiographic signs of malignancy should be scheduled for surgical intervention. For most benign tumors intralesional curettage and subsequent bone grafting is the treatment of choice, maintaining structural integrity and functional stability of the bone and adjacent joint. In case of uncertain histopathological diagnosis an open biopsy should be performed followed by definitive either marginal or en-bloc resection, preferably at a single procedure. Depending on the tumor-specific risk of recurrence, adjuvant measures as phenol instillation or cryotherapy using liquid nitrogen may additionally be applied. As for the unicameral juvenile bone cyst or osteoid osteoma alternative minimal invasive techniques like corticoid injection and CT-guided percutaneous eradication of the nidus have been successfully introduced into clinical practice. Depending on the activity of the tumor recurrent lesions are recommended to be treated by marginal or enbloc resection. The radical and aggressive mode of surgical therapy has to be balanced with the treatment-related morbidity, i.e. complication rate, possibilities for reconstructive stabilizations and resultant functional deficits,making the decision a responsible challenge to the orthopedic surgeon.

Idiopathic orbital pseudotumor is a rare disease. 10 % of the cases occur in children. In most of these juvenile cases, the swelling is bilateral, and histologically, eosinophilic granulocytes are found. In contrast to adults, children very rarely develop an associated systemic disorder. The disease is generally treated with steroids. Little is known about the natural course of idiopathic orbital pseudotumors in childhood.A 2S-year old girl presented with a unilateral swelling of the right upper lid. Four weeks later, a comparable swelling of the left side was revealed. We took biopsies of the lacrimal gland on both sides. Histology displayed an eosinophilic pseudotumor of the lacrimal gland. Steroid treatment was started, but after 4 weeks the parents refused to continue the treatment. During the following year, the bilateral swelling remained unchanged. Surprisingly, the bilateral swelling disappeared 3 years later and no systemic disorder had occured.Our case shows that spontaneous resolution of a juvenile eosinophilic orbital pseudotumor is possible. Treatment with steroids does not appear to be compulsory in all cases.

We report on a six year old female presented with a painful torticollis and a hemidysaesthesia caused by destruction of the third cervical vertebra and a paravertebral soft-tissue mass. At diagnostic routine finally a biopsy gives the diagnosis of Langerhans cell histiocytosis. In a second open approach the destructed vertebral body was replaced by a precisely adjusted autologous bone interponate and the patient was maintained in halo vest immobilisation. The outcome is described and an overview of the current literature is given.

Patients with Langerhans Cell Histiocytosis (LCH or Eosinophilic granuloma) were assessed from the orthopaedic point of view to give recommendations for the management of the disease.The results of 36 cases of histologically proven bony manifestations out of 48 treated cases were reviewed. A retrospective analysis of our treated cases with bony manifestations of LCH between 1970 and 1995 was performed.Twenty-two cases exhibited isolated bony manifestations, 18 were monoostotic and 4 were polyostotic. We treated 14 cases with multi-organ disease including bony manifestations of LCH. In the cases of exclusive bony manifestations reactivations were rare and usually occurred in other bones.In order to assure stability local control is the general goal of orthopaedic treatment. In isolated lesions control can be achieved by excochleation and filling with cancellous bone or prednisolon instillation. Multiple lesions should be treated primarily by systemic drugs and operative procedures are only necessary if severe local problems occur. Additionally, we recommend interdisciplinary cooperation between ortopedic surgeon, pediatrist and pathologist.

A 53 year old man presented with a giant variant of granuloma faciale, closely resembling rhinophyma. Therapeutic approaches with cryosurgery and dapsone were unsuccessful. Surgical reconstruction of the nasal skin resulted in an excellent and long lasting effect. We give a short overview of this relatively rare disease, describe an unusual manifestation and discuss the therapeutic possibilities. Surgical procedures seem to offer the best results, despite the inflammatory pathogenesis of the disease.

Six patients with granuloma faciale, including patients with multiple lesions, were treated successfully with cryosurgery. Granuloma faciale is known to be difficult to treat. Cryosurgery is an effective and minimally invasive therapy for this granulomatous inflammation of the skin. It should be considered as an alternative to dapsone.

The localized form of the Langerhans cell histiocytosis was referred to earlier as eosinophilic granuloma, which has the best prognosis of all histiocytosis syndromes concerning survival. The non-malignant proliferative disorder of the histiocytic system is still of unknown etiology. Characteristic radiographic signs are osteolytic lesions.We report on an otologic manifestation of a 20-year-old man's temporal bone, which developed three years after a successful treatment of an eosinophilic granuloma of the mandible.Surgery and low-dose irradiation led to a complete remission of the disease. Other manifestations of the disease were not detected until now.In the presence of chronic aural discharge, Langerhans cell histiocytosis must bei considered in differential diagnosis and a histopathological examination should be performed.

A 35-year-old woman from Georgia presented with multiple bilateral yellow nodules of the upper bulbar conjunctiva, accompanied by a severe injection of conjunctival vessels. The histologic examination of excisional biopsies showed the characteristic morphology of the Splendore-Hoeppli-phenomenon, which describes granulomas with a necrotized eosinophilic center, surrounded by a ring of epitheloid cells, giant cells and eosinophils. Although nematodes could be identified in only a few lesions, they should always be kept in mind as the causing agent of this condition. No cause of the lesions was found in our patient. The nodules may regress spontaneously. Topical application of corticosteroid eye drops, however, may give relief of symptoms, and sometimes excision of the granulomas will be necessary.

The present paper describes a case of eosinophilic granuloma of the lumbar spine. The lesion affected multiple spinal levels and was accompanied by an extensive paravertebral soft-tissue mass. HR-CT scans revealed additional pulmonary histiocytosis X.

A case of eosinophilic granuloma facial is presented in which the lesions have been successfully treated by cryotherapy, healing with slight pigmentation. Since this disease appears to be difficult to treat, a great many forms of therapy have been proposed in the literature. These will be discussed with the results presented.

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 gastroenteritis (EGE) is an etiologically obscure and rare inflammation which can affect all sections of the gastrointestinal tract from esophagus to rectum in a diffuse or segmentary manner. An infiltrate of eosinophilic granulocytes is found to varying degrees in all wall layers. The clinical symptoms depend on the site and extent of organ involvement. Diagnosis can only be established histologically. Peripheral eosinophilia is inconsistent and not diagnostic. ESR, leukocyte count, serum IgE, and RAST tests in foodstuffs may be normal or elevated. Two women patients are described with tumorous eosinophilic colitis of the cecum and colon ascendens, who underwent surgery for clinical acute abdomen. A further woman patient was hospitalized with bloody diarrhea and marked eosinophilia in the blood findings. Eosinophilic colitis was likewise found histologically in the mucosa which appeared with reddened patches in sigmoidoscopy. Eosinophilic colitis associated with eosinophilic gastroenteritis is rather rare and may therefore be overlooked. Our literature review contains only 64 such cases, in only 6 of which was the tumorous form found. Counting our own patients as well, eosinophilic colitis occurs somewhat more frequently in women (27 = 56%) than in men (21 = 44%). The clinical symptoms, possible causes and therapeutic approaches are discussed in the light of the literature.

The case of a ten-year old girl, presenting with pain and restricted movement of the left arm is reviewed. X-rays and bone scan suggested an Ewing's sarcoma of the left humerus, so that the child was referred to surgery. Surprisingly, the histological examination of the surgical specimen revealed a solitary eosinophilic granuloma of the bone.

Zusammenfassung Unter Verwendung histochemischer Nachweise fur Enzyme und Gewebsbestandteile sowie allgemeiner Farbemethoden wurden Untersuchungen an einem eosinophilen Granulom vorgenommen. Als gunstigste Methode erwies sich der Nachweis des Enzyms Phenoloxidase (EC 1.14.18.1). Mit dieser Methode ist eine schnelle und sichere Erfassung der Eosinophilen und damit ein Uberblick uber ihre Haufigkeit und Verteilung moglich. Elektronenmikroskopisch konnten die histiocytaren Zellen als Langerhans-Zellen identifiziert werden. Sie stehen in engem Kontakt mit den zahlreichen eosinophilen Granulocyten, deren Granula vielfaltige morphologische Veranderungen in ihrer Struktur aufweisen. Die Granula konnen offenbar auch zur Bildung neuer struktureller Zellkomponenten beitragen. Das eosinophile Granulom entspricht einer zellvermittelten immunologischen Abwehrreaktion, angenahert dem Typ einer verzogerten Uberempfindlichkeit.

The lacrimal caruncle of an 11-year-old female patient developed a 2 mm x 3 mm-sized growth with the primary appearance of an abscess. The process did not regress under local antibiotic therapy and was excised. Histological examination revealed histiocytosis X (eosinophilic granuloma).

Between 1957 and 1990 15 patients with solitary (stage Ia by Greenberger) and 2 patients with multiple (stage Ib) eosinophilic granuloma of the bone were treated by radiotherapy in 18 locations after previous surgery. The doses applied varied from 6 Gy to 42 Gy (median for patients under 18 years: 8 Gy, for patients over 18 years: 30 Gy). Median follow-up was 9 years (range, 0.3-33 years). Local control rate and survival were 100%. Three patients with granuloma developed a second granuloma within 13 years after primary diagnosis (two bone manifestations, one cutaneous manifestation). Long-term follow-up of patients with solitary eosinophilic granuloma is necessary.

By means of the proof for the enzyme phenol oxidase (PO; EC 1.14.18.1) was investigated, whether the eosinophilic granuloma correspond to a cell mediated immunological defence reaction. The method allowed to determine the eosinophils rapidly and surely and give an overview on the frequency and distribution of these cells. By electron microscopy was Langerhans-cells identified in close contact to many eosinophils. Their granula shows manifold changes in their structure. The eosinophilic granuloma is a cell mediated defence reaction approximate to the type of a delayed hypersensitivity.

A solitary eosinophilic granuloma in an 8 month-old infant is presented. The tumour, which had penetrated bone dura, but spared the brain tissue was radically excised. This case shows an unusually low age of presentation of this rare disease, and an unusual endocranial extension of the tumour. If an infantile eosinophilic granuloma is suspected, then computerized tomography should always be performed.

We report a woman with extensive polypoid sinusitis and bony destruction of the middle and posterior skull base. Histological examination of the specimen from the paranasal sinuses and adjacent skull base revealed polypoid eosinophilic sinusitis: malignancy could be excluded histologically and clinically. Plasma-CEA-levels were elevated to 85 micrograms/l with no typical source of CEA expression, and they fell after therapy. Immunocytochemistry revealed a remarkable expression of CEA in the surface epithelia of the specimen obtained from the paranasal sinuses. CEA-immunoscintigraphy showed an enhancement in the regions of the paranasal sinuses without expression elsewhere in the body. The authors discuss the possibility of using CEA as a marker for aggressive forms of chronic sinusitis and suggest radiotherapy is a successful treatment option in addition to surgery. The observation period of almost two years confirms the value of the chosen therapy.

The case report is about an unusual presacrale localisation of an eosinophil granuloma in a newborn girl. We discuss the differential diagnosis and difficulties in conventional histology and immunohistochemistry.

The contribution to the clinical picture of eosinophilic granuloma of the spine. This tumor-like, osteolytic bone lesion presents both diagnostic and therapeutic problems. Solitary eosinophilic granuloma of the spine is not common. Compared to the multiple manifestation of eosinophilic granuloma, which seldom spares the spine, vertebral involvement is rare in cases of solitary eosinophilic granuloma (about 10 p.c.). Spinal involvement of this disease is mainly characterized by an undramatic, uneventful clinical course, even in cases of extensive osteolytic bone defects, varying laboratory findings as well as partial or complete collapse of a vertebra, mostly in form of a true vertebrae plana. Open biopsy is recommended instead of needle aspiration biopsy in solitary eosinophilic granuloma of the spine in order to exclude Ewing's sarcoma, neuroblastoma, or bony manifestations of leukemia. In cases of solitary eosinophilic granuloma various therapeutic methods have been tried. Some authors have registered good results by means of prolonged immobilization, similar to fracture treatment. Others have used radiation therapy in moderate doses. We suggest operative treatment of solitary eosinophilic granuloma. Our procedure comprises open biopsy, frozen section examination, and curettage of the affected vertebral body, taking care not to destroy the epiphyseal plate or the intervertebral disc. Vertebral body replacement is achieved by means of a bone graft, taken from the iliac crest. The original height of the vertebra is thus restored and immediate stability of the involved area guaranteed. Undisturbed bone growth of the end plated of the vertebral body can be observed. This technique is to prevent disturbances of spinal growth and permanent deformities.(ABSTRACT TRUNCATED AT 250 WORDS)

We report on a patient with the diagnosis granuloma eosinophilicum faciale (GEF). Clinical and histological findings are described with special emphasis on immunhistological characteristics in lesional skin. A review on the literature is given.

Bone tumors in children show a large variety in Kind. Often there is no clear distinction between benign and malignant tumors. So in between according to Zollinger there are semimalignant tumors with potential malignity. Adequate excision of these tumors or resection enbloc inhibit the tumor recurrence and give a good prognosis to semimalignant tumors. 31 children with benign bone tumors were operated between 1972 and 1979. They have been followed up medically and radiologically from 1 to 7 years. Up to now we could neither find a recurrence nor a sign of malignant degeneration.

The Churg-Strauss syndrome is a disorder characterized by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. Only few patients are identified as having this syndrome. The three histological criteria are necrotizing vasculitis, tissue infiltration by eosinophils, and extravascular granulomas; they often do not coexist in one patient. To find a clinical approach to diagnosis, it is necessary to exclude other disorders with hypereosinophilia, granulomas, and vasculitis. In regard to this clinical viewpoint it seems that the syndrome is not as rare as may be assumed according to the relevant autopsy findings. Two cases are reported in which the Churg-Strauss syndrome developed together with rheumatoid arthritis; in one case it was likely triggered by treatment with D-penicillamin.