[New syndrome: endocranial osteoma with osteomas of the skeleton, osteomas of the peripheral soft tissues & cicatrical histiocytofibroma].
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BACKGROUND: Cranial osteomas are regarded by some as very common; yet their classification, symptomatology, and management have been neglected. METHODS: We report on a giant enostotic convexity osteoma and have reviewed the medical literature. RESULTS: A new comprehensive classification for cranial osteomas is proposed: (1) intraparenchymal, (2) dural, (3) skull base, and (4) skull vault. The latter is in turn, subdivided into exostotic and enostotic variants. Three symptom producing enostotic convexity osteomas have been reported in the world literature. We also describe a giant enostotic skull vault osteoma and propose an original surgical technique used to successfully resect this unusual tumor. CONCLUSIONS: Most cranial osteomas are asymptomatic and need not be resected. Those that are symptomatic should be managed properly. Their excision, if nor properly performed, may lead to unforeseen cerebral complications.
The benign bone lesions--osteoma, osteoid osteoma, and osteoblastoma--are characterized as bone-forming because tumor cells produce osteoid or mature bone. Osteoma is a slow-growing lesion most commonly seen in the paranasal sinuses and in the calvaria. When it occurs in the long bones, it is invariably juxtacortical and may need to be differentiated from, among others, parosteal osteosarcoma, sessile osteochondroma, and a matured juxtacortical focus of myositis ossificans. Osteoid osteoma and osteoblastoma appear histologically very similar. Their clinical presentations and distribution in the skeleton, however, are distinct: osteoid osteoma is usually accompanied by nocturnal pain promptly relieved by salicylates; osteoblastoma arises predominantly in the axial skeleton, spinal lesions constituting one-third of reported cases. This review focuses on the application of the various imaging modalities in the diagnosis, differential diagnosis, and evaluation of these lesions. Their histopathology also is discussed, and their treatment briefly outlined.
We report the molecular cloning of two replication-competent osteoma-inducing murine leukemia viruses from the RFB osteoma virus stock (M. P. Finkel, C. A. Reilly, Jr., B. O. Biskis, and I. L. Greco, p. 353-366, in C. H. G. Price and F. G. M. Ross, ed., Bone--Certain Aspects of Neoplasia, 1973). Like the original RFB osteoma virus stock, viruses derived from the molecular RFB clones induced multiple osteomas in mice of the CBA/Ca strain. The cloned RFB viruses were indistinguishable by restriction enzyme analysis and by nucleotide sequence analysis of their long-terminal-repeat regions and showed close relatedness to the Akv murine leukemia virus.
Primary and secondary forms of ossification can be distinguished on the basis of the skin, with osteoma cutis occurring in primary forms. Three entities can be differentiated: solitary and generalized osteoma cutis and multiple miliary osteoma of the face. Clinically, multiple papules 2-3 mm in diameter are present, which histologically consist of bony trabeculae enclosing mature fat cells and, occasionally, marrow cells. We describe the clinical, radiological and histological features of a case of multiple miliary osteoma of the face in an otherwise healthy 55-year-old woman.
RFB virus is an ecotropic C-type retrovirus isolated from CF-1 mice, in which it is associated with induction of osteomas. Sequence analysis of the RFB provirus revealed no evidence for presence of an oncogene or a recombined env gene. RFB virus is a member of the murine leukemia virus (MuLV) group (RFB MuLV), sharing 97% nucleotide identity with the endogenous ecotropic provirus of AKR mice (Akv). Like Akv, expression of RFB MuLV mRNAs is inducible by dexamethasone treatment, indicating that FRB MuLV also shares transcriptional control signals with Akv. We assessed the pathogenic potential of RFB MuLV in NMRI mice, which, in contrast to CF-1 mice, do not contain endogenous ecotropic retroviruses. RFB MuLV induced osteomas, osteopetrosis, and lymphomas in newborn NMRI mice. Another CF-1 mouse-derived leukemia virus, FBJ MuLV, the helper virus of the FBJ osteosarcoma virus stock, as well as Akv, also induced osteomas, osteopetrosis, and lymphomas in NMRI mice similar to RFB MuLV. These findings indicate that endogenous retroviruses carry a pathogenic potential in hematopoietic tissues and in the skeleton.
The authors present a case of pathologically proved osteoid osteoma that was visualized randomly on an I-131 whole-body scan. Search of the medical literature did not reveal any mention of radioactive iodine uptake by osteoid osteomas. Therefore, the authors concluded that this pathology must be included in the differential diagnostic list of positive findings on I-131 scans.
A 57-year-old woman was admitted with a 3-month history of diplopia and exophthalmos in the right eye. Plain skull X-P and axial CT demonstrated two bony tumors. One involved her right orbit, frontal sinus and ethmoidal sinus, and the other was located in the left occipital bone. The right intraorbital tumor was removed almost totally via the superomedial orbital approach by means of microsurgical drilling. Histopathological examination revealed mature osteoma. The microsurgical drilling procedure was helpful in obtaining an optimal result from surgical treatment of the orbital osteoma.
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Recently endonasal surgery has been considered to be a valuable contribution in the management of paranasal sinus osteoma. A retrospective evaluation study of 34 frontoethmoidal osteomas (23 frontal and 11 ethmoidal osteomas) treated at a tertiary care facility from 1990 to 1999 is presented. Twenty three osteomas (68%) were resected endonasally. Eleven osteomas (32%) were removed using an osteoplastic frontal sinus approach with coronal incision. In 5 cases of huge osteomas originating at the anterior frontal sinus wall, reconstruction of the resected anterior-frontal sinus wall was achieved by autologous outer table grafts harvested from the parietal region. Endoscopic and radiological follow-up ranging from 1 to 32 months showed three incomplete endonasal osteoma resections. Complete osteoma removal was achieved via endonasal revision surgery in two of these cases, while the third small residual osteoma remains under observation. There was no case of osteoplastic osteoma removal where incomplete osteoma resection became obvious during follow-up. Ethmoidal osteomas without extrasinusal extension can be resected endonasally. The endonasal approach should be considered also for frontal sinus osteomas if (1) sufficient frontal sinus access can be achieved endonasally, (2) the osteoma is placed medially to a virtual sagittal plane through the lamina papyracea, and (3) the tumour base is at the inferior part of the posterior frontal sinus wall. We favour the osteoplastic frontal sinus approach with coronal incision if an external approach is required to achieve tumour resection with the best aesthetic results.
INTRODUCTION: Osteomas are benign tumours located within bones or developing on them (1). The incidence of osteomas is as follows: frontal, ethmoid and maxillary, while they are extremely rare in the sphenoid sinus (2). Most often they are localized on sutures, and extremely rarely on occipital squama (3). They are often asymptomatic, and can be accidentally detected, by radiographic examination (4). The main clinical symptom is headache of varying intensity and quality, and in most cases not proportional to the size of the osteoma, which ranges from the size of pepper bean to the size of a child's head (5). In addition to headache, there can be sensitivity to pressure in the region of the frontal sinus (6). On exteriorization they give the symptomatology of the organ on which they develop. Depending on the direction of osteoma exteriorization, various complications may occur (6,7,8,9,10). CASE DESCRIPTION: A male patient born 1958, was admitted to the Department of Infectious Diseases on Nov. 29, 1996 with high temperature and strong headaches. The patient had had a traffic accident in 1989. X-ray did not show any injuries of cranial bones, but a frontal sinus osteoma has already been diagnosed. He had been suffering from occasional headaches several years back. Symptoms of the disease started three days prior to admission, with increased body temperature and headaches which persisted in spite of prescribed analgesics. Seven days prior to onset of the disease, the patient had had a cold. When admitted, he was conscious, oriented, with well developed osteomuscular structure. He did not vomit or have photophobia; meningeal signs were negative; febrile. Laboratory blood findings were normal, except for higher sedimentation (SE + 27) and higher blood sugar (BS = 8.1 mmol/l). Due to permanent diffuse headaches a lumbar puncture was performed on Dec. 1, 1996. Laboratory and microscopic examinations showed that the patient had purulent meningitis (Table 1). X-rays of the cranium showed a frontal sinus osteoma, starting from the frontal ethmoid cells and filling the entire right frontal cavity. Electroencephalogram dated Dec. 3 shows no signs of focal or diffuse electro-cortical dysfunction. An otolaryngologist was consulted who recommended surgical extirpation of the osteoma. Axial computerized tomography of the structures of endocranium in 5 and 10 mm sections was done on an outpatient basis on Dec 17, 1996. The findings confirmed existence of frontal sinus osteoma on the right side (without lesions of the frontobasal brain structures) and calcification of fhalx cerebri. After appropriate pre-operative preparation, surgery was performed in general endotracheal anaesthesia on December 6, 1997: Trepanatio sinus frontalis sec. Tato, evacuation osteomatis et obliteratio sinus frontalis lateris dextri. It was found intraoperatively that the right frontal sinus was entirely filled with whitish-yellow osseous tissue. The osseous tumour was completely immobile in relation to the surrounding tissues and filled the entire cavity of the frontal sinus, descending into both front enthmoids towards the right posterior ethmoid and penetrating the upper orbit wall over a radius of about 5 x 10 mm. The osteoma was carefully extirpated from the location with a drill and it was found that its pressure had denuded the dura in the right region of the cranial cavity with the diameter of about 1 cm2. The osteoma was removed in three osseous fragments, total size 4 x 2.5 x 1.5 cm. Upon removal of the osteoma, the sinus walls were explored for possible fracture, due to the head injury from 1989. No signs of fracture were found. Total obliteration of the right frontal sinus was made, with a closure of the nasofrontal channel and osteoplastic reconstruction of the frontal sinus wall. The postoperative course was regular. CONCLUSION: This paper describes an osteoma of the frontal sinus in a 39-year-old patient. (ABSTRACT TRUNCATED)