ABSTRACT
Disruption of intestinal microbial communities appears to underlie many human illnesses, but the mechanisms that promote this dysbiosis and its adverse consequences are poorly understood. In patients who received allogeneic hematopoietic cell transplantation (allo-HCT), we describe a high incidence of enterococcal expansion, which was associated with graft-versus-host disease (GVHD) and mortality. We found that Enterococcus also expands in the mouse gastrointestinal tract after allo-HCT and exacerbates disease severity in gnotobiotic models. Enterococcus growth is dependent on the disaccharide lactose, and dietary lactose depletion attenuates Enterococcus outgrowth and reduces the severity of GVHD in mice. Allo-HCT patients carrying lactose-nonabsorber genotypes showed compromised clearance of postantibiotic Enterococcus domination. We report lactose as a common nutrient that drives expansion of a commensal bacterium that exacerbates an intestinal and systemic inflammatory disease.
Subject(s)
Enterococcus/growth & development , Gastrointestinal Microbiome , Graft vs Host Disease/microbiology , Hematopoietic Stem Cell Transplantation , Lactose/metabolism , Aged , Animals , Dysbiosis , Enterococcus/genetics , Enterococcus/metabolism , Feces/microbiology , Female , Gastrointestinal Microbiome/genetics , Humans , Intestines/microbiology , Male , Mice , Microbiota , Middle Aged , RNA, Ribosomal, 16S , Sequence Analysis, RNA , Transplantation, HomologousABSTRACT
INTRODUCTION: Cherubism is a rare familial fibro-osseous lesion usually observed in children. The diagnosis is clinical and radiological. Outcome is generally favorable, but with a risk of ophthalmologic complications, especially lower eyelid retraction, proptosis, diplopia, displacement of eyeball and loss of visual acuity. Clinicians must be aware of these complications for proper detection and specific treatment. OBSERVATION: We report a case of cherubism observed in a 10-year-old boy who presented exophthalmia with reduced visual acuity on the right due to reduction of the volume of the orbital cavity and compression of the optic nerve. Partial maxillectomy was required for decompression. DISCUSSION: Cherubism can lead to various types of ophthalmologic complications. Exophthalmos and loss of visual acuity due to compression of the optic nerve are the most common. Surgery is generally not required, but can become crucial in selected patients.
Subject(s)
Cherubism/complications , Cherubism/surgery , Exophthalmos/etiology , Maxilla/surgery , Nerve Compression Syndromes/etiology , Optic Nerve Diseases/etiology , Child , Decompression, Surgical , Exophthalmos/surgery , Humans , Male , Nerve Compression Syndromes/surgery , Optic Nerve Diseases/surgery , Oral Surgical Procedures , Vision Disorders/etiology , Vision Disorders/surgery , Visual AcuityABSTRACT
Bezold's abscess and sigmoid sinus thrombosis are both classic complications of otitis media. However, since the introduction of antibiotics both have become very rare. Very few cases of these complications occurring concomitantly have been reported. We describe the clinical case of a 45 year old male with a 10 year history of neglected right ear cholesteatoma. The patient presented to our department with a 10 day history of purulent right otorrhea, otalgia, increasing headach, pyrexia and suppurating right parapharyngeal mass. CT scan confirmed right middle ear cholesteatoma associated with ipsilateral Bezold abscess and and sigmoid sinus thrombosis. Treatment was by mastoidectomy, broad spectrum intravenous antibiotics and anticoagulation. The patient made a full uncomplicated recovery. The association between chronic middle ear disease, sigmoid sinus thrombosis and Bezold abscess are discussed.
Subject(s)
Abscess/diagnostic imaging , Sinus Thrombosis, Intracranial/diagnostic imaging , Sinus Thrombosis, Intracranial/etiology , Abscess/complications , Abscess/surgery , Cholesteatoma, Middle Ear/complications , Cholesteatoma, Middle Ear/diagnostic imaging , Cholesteatoma, Middle Ear/surgery , Humans , Male , Middle Aged , Otitis Media, Suppurative/complications , Otitis Media, Suppurative/diagnostic imaging , Otitis Media, Suppurative/surgery , Otorhinolaryngologic Surgical Procedures/methods , Sinus Thrombosis, Intracranial/surgery , Tomography, X-Ray ComputedABSTRACT
INTRODUCTION: Osteomyelitis of the temporomandibular joint is a rare complication of invasive external otitis, often occurring after inappropriate initial treatment of the causal affection. OBSERVATION: A 65-year-old diabetic man was treated for invasive otitis externa complicated by destructive osteomyelitis of the ipsilateral temporomandibular joint. The diagnosis was suspected clinically and confirmed by imaging. The course was favorable with medical treatment. DISCUSSION: Osteomyelitis is an extremely unusual complication of invasive otitis externa. Diffusion of the infection toward the temporomandibular joint can be secondary to an osteitis of the bony canal or spread via congenital dehiscences of the external auditory canal. Pain, limitation of mouth opening and auricular swelling are the main clinical signs. Treatment is essentially medical, based on adapted antibiotic therapy.
Subject(s)
Osteomyelitis/etiology , Otitis Externa/complications , Temporomandibular Joint Disorders/etiology , Aged , Anti-Bacterial Agents/therapeutic use , Ciprofloxacin/therapeutic use , Gentamicins/therapeutic use , Humans , Male , Necrosis , Osteomyelitis/drug therapy , Otitis Externa/drug therapy , Otitis Externa/pathology , Radiography, Panoramic , Temporomandibular Joint Disorders/drug therapyABSTRACT
OBJECTIVES: Langerhans cell histiocytosis is a proliferating cell disease that may take various forms characterised by bone, skin lymph nodes and visceral lesions. Eosinophilic granuloma is a localised form of histiocytosis X, or Langerhans' cells histiocytosis, a benign form with unknown aetiology. At the head and neck level, it is usually located on the temporal, and usually occur in association with multifocal disease; however, isolated lesions may occur in the mastoid bone alone. METHODS: The author's present a case of a patient with unifocal eosinophilic granuloma limited to the mastoid treated in ENT and radiotherapy departments. RESULTS: CT scan demonstrated an osteolytic lesion of the mastoid. The biopsy and immunohistochemical study confirmed the diagnosis. A surgery followed by a radiotherapy (20 Gy) have stabilised the situation. There is no recurrency after one year. CONCLUSION: Unifocal eosinophilic granuloma of the temporal bone is a benign lesion of langerhans' cell histiocytosis. Its diagnosis is difficult when the disease remains isolated. Its treatment depend on its extension and its risks. The excellent prognosis does not dispense on regulary follow up.
Subject(s)
Bone Diseases/diagnosis , Eosinophilic Granuloma/diagnosis , Mastoid/pathology , Adolescent , Biopsy , Female , Follow-Up Studies , Humans , Immunohistochemistry , Osteolysis/diagnosis , Temporal Bone/pathology , Tomography, X-Ray ComputedABSTRACT
INTRODUCTION: Osteoma of the nasosinus cavities, particularly the frontoethmoidal cavities, is often a fortuitous radiographic discovery. Potential complications include ophthalmological, sinusal, and endocranial disorders. MATERIAL AND METHODS: Nine patients (five men and four women), mean age 24.3 years (18-43) presenting orbital osteoma were managed from 1994 to 1999. RESULTS: Clinical signs were dominated by ophthalmological disorders, basically non-reducible exophthalmia (n = 8). Two patients had permanent unilateral nasal obstruction and one had acute sinusitis. The bony orbital tumefaction was located in the superior medial angle in five patients. Radiography (Blondeau view) demonstrated an opacity in the sinus with an orbital extension. Computed tomography, performed in all patients, identified the lesion location: ethmoidofrontal (n = 4), anterior ethmoidal (n = 2), frontal (n = 2), ethmoidomaxillary (n = 1). There were no endocranial extensions. Surgery was undertaken for eight patients generally via the transfacial approach (n = 7); a bicoronal approach was used for one patient. En bloc resection (n = 4) or fragmentation (n = 4) was performed. There was one postoperative complication: regressive diplopy. These patients have been followed regularly with clinical and radiographic explorations and have been free of relapse for 2 to 6 years. DISCUSSION: Frontoethmoidal osteoma is a relatively rare benign tumor. Severity depends on the orbital and endocranial extension. Imaging provides the positive diagnosis and guides the surgical approach. Therapeutic indications depend on the tumor size, its course, and the development of complications. We observed total relapse free outcome after complete resection.
Subject(s)
Orbital Neoplasms/surgery , Osteoma/surgery , Adolescent , Adult , Diplopia/etiology , Ethmoid Sinus/surgery , Exophthalmos/etiology , Female , Follow-Up Studies , Frontal Sinus/surgery , Humans , Male , Maxillary Sinus/surgery , Nasal Obstruction/etiology , Orbital Neoplasms/complications , Orbital Neoplasms/diagnosis , Osteoma/complications , Osteoma/diagnosis , Paranasal Sinus Neoplasms/diagnosis , Paranasal Sinus Neoplasms/surgery , Postoperative Complications , Sinusitis/etiology , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
UNLABELLED: Laryngeal papillomatosis is well-known as one part of benign lesions of the larynx in children under 15 years. Cases of papillomatosis are extremely rare in adults. OBJECTIVES: To report our experience about 4 cases. MATERIAL AND METHOD: It concerns 3 females and 1 male, who are in good health and without any disease in relation of upper respiratory tract or viruses. For all patients there is no familiar viral disease (especially cutaneous or genital). The age of patients is between 28 and 72 years. All clinical signs at the diagnosis aren't specific and are observed in other laryngeal diseases. Direct micro-laryngoscopy is mandatory in order to establish the right diagnosis and to perform the first stage of local treatment. Micro-instruments are used to remove these benign nipple-like without CO2 laser or local medicines or injections. RESULTS: Our study mentions a frequent association of gastro-oesophageal reflux (3 cases). The average follow-up is 5 years and half and in this period, there is no malignant evolution. CONCLUSION: The authors also review the literature and in particular the potential cause of laryngeal papillomatosis and new ideas on treatment of this unusual condition.
Subject(s)
Laryngeal Neoplasms/pathology , Laryngeal Neoplasms/surgery , Microsurgery/methods , Papilloma/pathology , Papilloma/surgery , Adult , Age of Onset , Aged , Female , Gastroesophageal Reflux/etiology , Humans , Laryngeal Neoplasms/complications , Laryngoscopy , Male , Middle Aged , Papilloma/complicationsABSTRACT
Hemangiopericytoma is a slow growing insidious potentially malignant vascular tumor that can occur anywhere in the body. Primary localization in the infratemporalis fossa, an area deep in the face, is uncommon and difficult to diagnose and treat. We present two cases of hemangiopericytoma observed in the infratemporalis fossa and recall the epidemiological and clinical features of these lesions, discussion diagnostic and therapeutic options.
Subject(s)
Hemangiopericytoma/pathology , Skull Base Neoplasms/pathology , Adolescent , Carotid Artery, Internal/diagnostic imaging , Cranial Fossa, Middle/diagnostic imaging , Cranial Fossa, Middle/pathology , Cranial Fossa, Middle/surgery , Fatal Outcome , Female , Hemangiopericytoma/diagnostic imaging , Hemangiopericytoma/surgery , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Skull Base Neoplasms/diagnostic imaging , Skull Base Neoplasms/surgery , Tomography, X-Ray ComputedABSTRACT
The classification of superficial vascular anomalies of the face recognizes two major categories: hemangiomas and vascular malformations. A multidisciplinary approach to these malformations is necessary. MRI of venous vascular malformations, pulsed Doppler and arteriography of arteriovenous malformations are key examinations in the exploration strategy for superficial vascular malformations. Hemangiomas always regress. Surgery is generally needed only for late sequelae although in some cases early surgery is beneficial. Percutaneous sclerotherapy and surgery are indicated for venous malformations in order to minimize or improve skin, muscle and bone distorsions. Arteriovenous malformations are the most dangerous vascular anomalies of the face. Superselective arterial embolization prepares the surgical excision of the nidus, complete resection is essential. We review 6 cases of superficial vascular malformations of the face treated surgically and discuss the diagnosis features and the treatment modalities of these vascular anomalies.
Subject(s)
Arteriovenous Malformations/surgery , Face/blood supply , Facial Neoplasms/surgery , Hemangioma/surgery , Adolescent , Adult , Cheek/blood supply , Child, Preschool , Embolization, Therapeutic , Female , Humans , Jugular Veins/abnormalities , Jugular Veins/surgery , Lip/blood supply , Lip Neoplasms/surgery , MaleABSTRACT
Extranodal non-Hodgkin lymphoma of the head and neck is usually seen in the Waldeyer's ring. Localizations in the infratemporalis fossa are rare and to date, only seven cases have been reported. The clinical and radiographic features of non-Hodgkin lymphoma is non-specific. Positive diagnosis requires a pathologic examination, generally guided by immunohistochemistry results. We report a case and discuss the epidemiological, diagnostic, therapeutic and prognostic features of these tumors.
Subject(s)
Cranial Fossa, Middle/pathology , Lymphoma, B-Cell/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Skull Base Neoplasms/pathology , Aged , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chemotherapy, Adjuvant , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Female , Humans , Immunohistochemistry , Lymphoma, B-Cell/drug therapy , Lymphoma, B-Cell/radiotherapy , Lymphoma, B-Cell/surgery , Lymphoma, Large B-Cell, Diffuse/drug therapy , Lymphoma, Large B-Cell, Diffuse/radiotherapy , Lymphoma, Large B-Cell, Diffuse/surgery , Magnetic Resonance Imaging , Prednisone/administration & dosage , Prognosis , Radiotherapy, Adjuvant , Skull Base Neoplasms/drug therapy , Skull Base Neoplasms/radiotherapy , Skull Base Neoplasms/surgery , Tomography, X-Ray Computed , Vincristine/administration & dosageABSTRACT
Chondrosarcoma is a malignant cartilaginous tumor rarely found in the jaw bone. Tumor progression is slow with strong propensity to local recurrence after surgical removal. The prognosis is poor and depends on the degree of differentiation and the quality of the resection. We report a case of mandibular chondrosarcoma that exhibited particularly massive extension to soft tissue. We discuss the epidemiology, diagnosis, treatment and follow-up of these tumors.
Subject(s)
Chondrosarcoma/pathology , Mandibular Neoplasms/pathology , Aged , Chondrosarcoma/diagnostic imaging , Chondrosarcoma/surgery , Fatal Outcome , Female , Humans , Mandibular Neoplasms/diagnostic imaging , Mandibular Neoplasms/surgery , Prognosis , Tomography, X-Ray ComputedABSTRACT
Because tumor necrosis factor (TNF)-alpha can upregulate alveolar fluid clearance (AFC) in pneumonia or septic peritonitis, the mechanisms responsible for the TNF-alpha-mediated increase in epithelial fluid transport were studied. In rats, 5 microg of TNF-alpha in the alveolar instillate increased AFC by 67%. This increase was inhibited by amiloride but not by propranolol. We also tested a triple-mutant TNF-alpha that is deficient in the lectinlike tip portion of the molecule responsible for its membrane conductance effect; the mutant also has decreased binding affinity to both TNF-alpha receptors. The triple-mutant TNF-alpha did not increase AFC. Perfusion of human A549 cells, patched in the whole cell mode, with TNF-alpha (120 ng/ml) resulted in a sustained increase in Na(+) currents from 82 +/- 9 to 549 +/- 146 pA (P < 0.005; n = 6). The TNF-alpha-elicited Na(+) current was inhibited by amiloride, and there was no change when A549 cells were perfused with the triple-mutant TNF-alpha or after preincubation with blocking antibodies to the two TNF-alpha receptors before perfusion with TNF-alpha. In conclusion, although TNF- alpha can initiate acute inflammation and edema formation in the lung, TNF-alpha can also increase AFC by an amiloride-sensitive, cAMP-independent mechanism that enhances the resolution of alveolar edema in pathological conditions by either binding to its receptors or activating Na(+) channels by means of its lectinlike domain.
Subject(s)
Amiloride/administration & dosage , Pulmonary Alveoli/drug effects , Respiratory Mucosa/drug effects , Sodium/metabolism , Tumor Necrosis Factor-alpha/administration & dosage , Adrenergic beta-Agonists/administration & dosage , Adrenergic beta-Antagonists/administration & dosage , Amino Acid Substitution , Animals , Antibodies, Blocking/pharmacology , Antigens, CD/metabolism , Biological Transport/drug effects , Biological Transport/physiology , Cell Line , Humans , Instillation, Drug , Male , Membrane Potentials/drug effects , Mutation , Patch-Clamp Techniques , Propranolol/administration & dosage , Pulmonary Alveoli/metabolism , Rats , Rats, Sprague-Dawley , Receptors, Tumor Necrosis Factor/antagonists & inhibitors , Receptors, Tumor Necrosis Factor/metabolism , Receptors, Tumor Necrosis Factor, Type I , Receptors, Tumor Necrosis Factor, Type II , Respiratory Mucosa/metabolism , Sodium Channels/drug effects , Sodium Channels/metabolism , Tumor Necrosis Factor-alpha/geneticsABSTRACT
An unusual case of massive osteolysis destroying the left side of the mandible, the maxilla, the orbit, the cranium bones and the upper cervical spine is reported. The evolution of this disease was observed over a period of 4 years. The literature was reviewed, only 39 cases have been found involving the maxillo-facial bones. In our case, attempts at surgery was without success. However, further radiotherapy with 35 Gy controlled the progression of this osteolysis.
Subject(s)
Cervical Vertebrae/pathology , Facial Bones/pathology , Osteolysis, Essential/diagnosis , Skull/pathology , Spinal Diseases/diagnosis , Adolescent , Humans , Male , Mandibular Diseases/diagnosis , Maxillary Diseases/diagnosis , Occipital Bone/pathology , Orbital Diseases/diagnosis , Osteolysis, Essential/radiotherapy , Osteolysis, Essential/surgery , Temporal Bone/pathologyABSTRACT
There is considerable interest in identifying the basic mechanisms by which dexamethasone alters ion transport across the adult alveolar epithelium. Herein, we incubated synchronized A549 cells, a human alveolar epithelial cell line, with dexamethasone (1 microM) for 24-48 h. When normalized to HPRT (a housekeeping gene), A549 beta- and gamma-subunit mRNA levels for the human amiloride-sensitive epithelial sodium channel (hENaC), assessed by RT-PCR, increased by 1.6- and 17-fold respectively, compared with control values (P < 0.05). These changes were abolished by actinomycin D, indicating transcriptional regulation. Western blotting studies revealed that dexamethasone also increased expression of beta- and gamma-hENaC protein levels. In contrast, alpha-hENaC mRNA increased by onefold (P > 0.05) and alpha-hENaC protein level was unchanged. Incubation of A549 cells with dexamethasone increased their whole cell amiloride-sensitive sodium currents twofold and decreased the K(0.5) for amiloride from 833 +/- 69 to 22 +/- 5.4 nM (mean +/- SE; P < 0.01). Single channel recordings in the cell-attached mode showed that dexamethasone treatment increased single channel open time and open probability threefold and decreased channel conductance from 8.63 +/- 0.036 to 4. 4 +/- 0.027 pS (mean +/- SE; P < 0.01). We concluded that dexamethasone modulates the amiloride-sensitive Na(+) channels by differentially regulating the expression of beta- and gamma-subunits at the mRNA and protein levels in the human A549 cell line, with little effect on alpha-hENaC subunit.
Subject(s)
Anti-Inflammatory Agents/pharmacology , Dexamethasone/pharmacology , Lung/metabolism , Sodium Channels/drug effects , Sodium Channels/physiology , Administration, Topical , Cell Line , Electric Conductivity , Electrophysiology , Epithelial Sodium Channels , Glucocorticoids , Humans , Lung/cytology , Lung/physiology , RNA, Messenger/metabolism , Reverse Transcriptase Polymerase Chain Reaction , Sodium Channels/geneticsABSTRACT
Amiloride-sensitive Na(+) channels, present in fetal and adult alveolar epithelial type II (ATII) cells, play a critical role in the reabsorption of fetal fluid shortly after birth and in limiting the extent of alveolar edema across the adult lung. Because of the difficulty in isolating and culturing ATII cells, there is considerable interest in characterizing the properties of ion channels and their response to injury of ATII cell-like cell lines such as A549 that derive from a human alveolar cell carcinoma. A549 cells were shown to contain alpha-, beta-, and gamma-epithelial Na(+) channel mRNAs. In the whole cell mode of the patch-clamp technique (bath, 145 mM Na(+); pipette, 145 mM K(+)), A549 cells exhibited inward Na(+) currents reversibly inhibited by amiloride, with an inhibition constant of 0.83 microM. Ion substitution studies showed that these channels were moderately selective for Na(+) (Na(+)-to-K(+) permeability ratio = 6:1). Inward Na(+) currents were activated by forskolin (10 microM) and inhibited by nitric oxide (300 nM) and cGMP. Recordings in cell-attached mode revealed the presence of an amiloride-sensitive Na(+) channel with a unitary conductance of 8.6 +/- 0.04 (SE) pS. Channel activity was increased by forskolin and decreased by nitric oxide and the cGMP analog 8-bromo-cGMP. These data demonstrate that A549 cells contain amiloride-sensitive Na(+) channels with biophysical properties similar to those of ATII cells.
Subject(s)
Sodium Channels/physiology , Amiloride/pharmacology , Biophysical Phenomena , Biophysics , Cyclic AMP/pharmacology , Cyclic GMP/pharmacology , Electric Conductivity , Epithelial Sodium Channels , Extracellular Space/metabolism , Gene Expression , Humans , Nitric Oxide/pharmacology , Osmolar Concentration , Sodium/metabolism , Sodium Channels/drug effects , Sodium Channels/genetics , Tumor Cells, CulturedABSTRACT
Existing evidence supports the presence of active transport of Na(+) across the mammalian alveolar epithelium and its upregulation by agents that increase cytoplasmic cAMP levels. However, there is controversy regarding the mechanisms responsible for this upregulation. Herein we present the results of various patch-clamp studies indicating the presence of 25- to 27-pS, amiloride-sensitive, moderately selective Na(+) channels (Na(+)-to-K(+) permeability ratio = 7:1) located on the apical membranes of rat alveolar type II (ATII) cells maintained in primary culture. The addition of terbutaline to the bath solution increased the open probability of single channels present in cell-attached patches of ATII cells without affecting their conductance. A similar increase in open probability was seen after the addition of protein kinase A, ATP, and Mg(2+) to the cytoplasmic side of inside-out patches. Measurement of short-circuit currents across confluent monolayers of rat or rabbit ATII cells indicates that terbutaline and 8-(4-chlorophenylthio)-cAMP increase vectorial Na(+) transport and activate Cl(-) channels. Currently, there is a controversy as to whether the cAMP-induced increase in Na(+) transport is due solely to hyperpolarization of the cytoplasmic side of the ATII cell membrane due to Cl(-) influx or whether it results from simultaneous stimulation of both Cl(-) and Na(+) conductive pathways. Additional studies are needed to resolve this issue.
Subject(s)
Cyclic AMP/physiology , Pulmonary Alveoli/metabolism , Sodium/metabolism , Animals , Biological Transport/physiology , Cell Line , Humans , Pulmonary Alveoli/cytology , Rats/metabolismABSTRACT
Gorham's disease also called idiopathic massive osteolysis represents a rare affection with unknown aetiology. This disease was described in different bones of the body but its location on maxillo-facial skeleton is particular by the number of cases in the world (only 32), by morphological, functional consequences and the prognosis. The authors describe a new case of male teenager (17 years old) who had a massive osteolysis of mandibular ramus, upper maxillary, malaire, sphenoid, temporal and occipital left bone. The authors give precision about epidemiological data, methods of diagnosis and therapeutic procedures.
Subject(s)
Facial Bones , Mandibular Diseases/diagnosis , Maxillary Diseases/diagnosis , Osteolysis, Essential/diagnosis , Skull , Adolescent , Facial Bones/pathology , Follow-Up Studies , Humans , Image Processing, Computer-Assisted , Imaging, Three-Dimensional , Magnetic Resonance Imaging , Male , Mandibular Diseases/radiotherapy , Maxillary Diseases/radiotherapy , Osteolysis, Essential/pathology , Osteolysis, Essential/radiotherapy , Skull/pathology , Tomography, X-Ray ComputedABSTRACT
We report 12 cases of osteosarcoma (10 located in the mandible and 2 in the maxilla). There were 8 men and 4 women who were older than usually observed in osteosarcoma at other sites (8 of 121 patients were over 30 and the average age was 34 years). Clinical signs were variable. Bone tumefaction was the most frequent sign, with a diameter greater than 5 cm in most cases (10 out of 12). Surgery alone was used for 8 patients and radiosurgery for 3. Follow-up is available for 8 patients. All presented local recurrence with extensive tumors progression. All died within an interval of 3 to 30 months despite palliative surgery and/or radiotherapy or chemotherapy.