RESUMEN
Mixed tumors occur most commonly in major salivary glands. They may rarely appear in other sites, such as skin, breast, and lung. There have been rare reports of similar tumors occurring in bone. We report on a patient with a primary mixed tumor of bone. No other primary site has become apparent on follow-up of 5 years. Although mixed tumors detected by bone biopsy are likely to be metastatic, they may rarely represent primary bone neoplasms.
Asunto(s)
Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/patología , Tumor Mixto Maligno/diagnóstico por imagen , Tumor Mixto Maligno/patología , Huesos Tarsianos/diagnóstico por imagen , Huesos Tarsianos/patología , Adulto , Biopsia , Femenino , Humanos , RadiografíaRESUMEN
A 53-year-old woman had extraskeletal osteosarcoma that developed from a soft tissue bony mass present on the volar aspect of the left wrist for 4 years. Initially, the bony mass was soft and movable, but during the first year it became hard and fixed. The patient had no history of trauma. Because the lesion did not grow or cause any symptoms, the patient did not come to the hospital until 4 years after she first noticed the lesion. Radiologically, the bony mass had features characteristic of mature myositis ossificans, showing "eggshell" ossification. A nonmineralized soft tissue mass occurred between the surface of the radius and the bony shell. Histologically, a high-grade osteosarcoma was present between the surface of the radius and the well-differentiated bone tissue, which included fatty and hematopoietic marrow. All the findings indicated that our patient had an extremely rare case of malignant transformation of myositis ossificans.
Asunto(s)
Antebrazo , Miositis Osificante/complicaciones , Osteosarcoma/complicaciones , Neoplasias de los Tejidos Blandos/complicaciones , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Miositis Osificante/diagnóstico , Miositis Osificante/diagnóstico por imagen , Osteosarcoma/diagnóstico , Osteosarcoma/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
In a study of the clinical, radiographic, and pathological features of chondromyxoid fibroma, the tumor was slightly more common in men, usually in the second decade of life. Almost half of the tumors involved the long bones, although the ilium and the small bones were also common sites. Roentgenograms showed a sharply marginated, lobulated, lucent defect in the metaphysis. The tumor involved the medullary bone in an eccentric fashion, and the cortex was thinned and expanded. Periosteal reaction and soft tissue extension were uncommon. Mineralization was identified in 13% of the lesions. Histologically, the tumors were almost always arranged in lobules, which were prominent (macrolobular) or somewhat indistinct (microlobular). The tumor cells were spindle-shaped or stellate and arranged in a myxoid matrix. Calcification was seen in more than one third of the cases but was rarely prominent. Hyaline cartilage and chondroblastoma-like areas were not uncommon. Approximately 18% of tumors showed bizarre nuclei. Permeation of bony trabeculae was uncommon. Treatment was conservative surgical removal; approximately one fourth of the patients had recurrence.
Asunto(s)
Neoplasias Óseas/patología , Condroblastoma/patología , Adolescente , Adulto , Distribución por Edad , Anciano , Anciano de 80 o más Años , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/terapia , Niño , Condroblastoma/diagnóstico por imagen , Condroblastoma/terapia , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Distribución por Sexo , Tomografía Computarizada por Rayos XRESUMEN
Malignant pigmented villonodular synovitis (PVNS) is a rare lesion whose existence may be debatable. We studied eight cases that we consider to be examples of malignant PVNS. The three male and five female patients were aged 12 to 79 years. The knee was involved in three cases; the ankle in two; and the cheek, dorsum of the foot, and thigh in one each. Four patients had swelling for 6 months to 17 years before presentation. Three cases of malignant PVNS were secondary, arising in patients in whom PVNS had been documented previously, and five cases were primary, with histologic features similar to those of the secondary ones. Important histologic features of malignancy were (a) a nodular, solid infiltrative pattern of the lesion; (b) large, plump, round or oval cells with deep eosino philic cytoplasm and indistinct borders; (c) large nuclei with prominent nucleoli; and (d) necrotic areas. Atypical mitoses were occasionally seen. Four patients died with pulmonary metastasis (two also had metastasis to inguinal lymph nodes). Four patients are alive from 3 1/2 to 5 years after the last surgical treatment. The malignant nature of this lesion, the histologic architecture similar to that of PVNS, and the fibrohistiocytic appearance of the cells suggest that malignant PVNS is an entity.
Asunto(s)
Tumores de Células Gigantes/patología , Articulaciones , Neoplasias de Tejido Conjuntivo/patología , Sinovitis Pigmentada Vellonodular/patología , Tendones , Adulto , Anciano , Anatomía Transversal , Niño , Diagnóstico Diferencial , Resultado Fatal , Femenino , Citometría de Flujo , Estudios de Seguimiento , Tumores de Células Gigantes/etiología , Tumores de Células Gigantes/cirugía , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Neoplasias de Tejido Conjuntivo/etiología , Neoplasias de Tejido Conjuntivo/cirugía , Sinovitis Pigmentada Vellonodular/etiología , Sinovitis Pigmentada Vellonodular/cirugía , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: Computed radiography of the musculoskeletal system has the potential to become a powerful tool in the practice of diagnostic radiology. It addresses many of the geographic and film-distribution concerns facing diagnostic imaging. We undertook this study to compare and document the quality of computed radiographs and conventional screen-film images before widespread implementation. MATERIALS AND METHODS: We evaluated clinical images using direct comparison. Bilateral hand images from 50 patients were scored independently by six musculoskeletal radiologists. In each case one hand was imaged with a conventional screen-film technique and the other with computed radiography. Images were masked to eliminate as much bias as possible. The numeric scores assigned to the images by the observers were analyzed using Student's t test. RESULTS: Computed radiographs were judged with statistical significance to be better than conventional screen-film images in all features judged by the observers, including bone cortex, bone trabeculae, corticomedullary junction, distal phalangeal tuft, soft tissues, fat planes, bone-soft-tissue interface, and overall contrast and density. CONCLUSION: The statistically significant determination that the image quality of computed radiographs is at least as good as screen-film images allows confident use of computed radiography and enables radiologists to take advantage of its many other practical capabilities related to image distribution, storage, cost, and geographic coverage without sacrificing image quality.
Asunto(s)
Mano/diagnóstico por imagen , Adulto , Anciano , Artritis Reumatoide/diagnóstico por imagen , Femenino , Humanos , Masculino , Persona de Mediana Edad , Osteoartritis/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Pantallas Intensificadoras de Rayos XRESUMEN
BACKGROUND: Osteoma of the skull and facial bones is not uncommon, is usually asymptomatic, and may be associated with Gardner's syndrome. Osteomas involving other bones are unusual. METHODS: The authors describe 14 cases (eight men and six women from age 21-66 years) of parosteal osteoma of bones other than of the skull and face. RESULTS: Thirteen lesions involved long bones, and one involved the clavicle. A long-standing (18 months to 31 years) mass was reported in seven patients. Symptoms of pain, a mass, or both were present in all except three patients who were asymptomatic. Lesions ranged from 2.5 to 20 cm in greatest dimension. Histologically, they blended with the cortex, did not infiltrate the medullary cavity, and consisted of dense sclerotic lamellar bone with haversian systems, similar to the architecture of normal cortical bone. There was no spindle cell proliferation. Nine patients underwent resection, four had biopsy, and one had debulking. With a follow-up of 1-23 years, no recurrence or metastasis was reported. CONCLUSIONS: Parosteal osteoma must be distinguished from parosteal osteosarcoma, a low grade malignant neoplasm. If radiographs do not identify areas of radiolucency and sections do not contain spindle cells, the diagnosis of parosteal osteoma is justified.
Asunto(s)
Neoplasias Óseas/patología , Osteoma/patología , Adulto , Anciano , Neoplasias Óseas/diagnóstico por imagen , Clavícula/diagnóstico por imagen , Clavícula/patología , Clavícula/cirugía , Diagnóstico Diferencial , Supervivencia sin Enfermedad , Femenino , Neoplasias Femorales/diagnóstico por imagen , Neoplasias Femorales/patología , Neoplasias Femorales/cirugía , Peroné/diagnóstico por imagen , Peroné/patología , Peroné/cirugía , Estudios de Seguimiento , Osteón/patología , Humanos , Húmero/diagnóstico por imagen , Húmero/patología , Húmero/cirugía , Masculino , Persona de Mediana Edad , Osteoma/diagnóstico por imagen , Osteosarcoma Yuxtacortical/diagnóstico , Osteosclerosis/patología , Radiografía , Tibia/diagnóstico por imagen , Tibia/patología , Tibia/cirugíaRESUMEN
Oncogenic osteomalacia is an unusual and rare clinicopathologic syndrome characterized by mesenchymal tumors that apparently produce osteomalacia and biochemical abnormalities consisting of hypophosphatemia, normocalcemia, and increased levels of alkaline phosphatase. We collected from the Mayo Clinic files and from our consultation files the records for 17 cases of osteomalacia associated with bone lesions. There were five cases of fibrous dysplasia, three of hemangiopericytoma, and two of phosphaturic mesenchymal tumor. There was one case each of osteosarcoma, chondroblastoma, chondromyxoid fibroma, malignant fibrous histiocytoma, giant cell tumor, metaphyseal fibrous defect, and hemangioma. In this study we can figure out that the most common characteristic histologic features of our cases were hemangiopericytomatous vascular proliferation, fine lace-like stromal calcification, and stromal giant cells. In most of the cases, the clinical and biochemical symptoms and signs resolved soon after complete resection of the lesion. When the lesion recurred or metastasized, the symptoms and signs also recurred.
Asunto(s)
Neoplasias Óseas/complicaciones , Displasia Fibrosa Ósea/complicaciones , Osteomalacia/etiología , Adolescente , Adulto , Anciano , Neoplasias Óseas/clasificación , Neoplasias Óseas/diagnóstico , Niño , Preescolar , Femenino , Displasia Fibrosa Ósea/clasificación , Displasia Fibrosa Ósea/diagnóstico , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Osteomalacia/patologíaRESUMEN
The records of 226 patients (sixty-seven who were managed at our institution and 159 who were identified from the consultation files) who had had a parosteal osteosarcoma were reviewed. The criteria for diagnosis were that, roentgenographically, the lesion had arisen from the surface of the bone and that, histologically, the tumor was well differentiated (Grade 1 or 2); it was characterized by well formed osteoid within a spindle-cell stroma; and, when there was medullary involvement, less than 25 per cent of the medullary cavity was affected. Dedifferentiation was more common (16 per cent of the patients) than previously reported and was associated with a poor prognosis. Cross-sectional imaging studies demonstrated medullary involvement in 22 per cent of the patients, an unmineralized soft-tissue mass peripheral to the mineral component in 51 per cent, and adjacent soft-tissue invasion in 46 per cent. In contrast to the findings in our previous studies, medullary involvement was not a poor prognostic factor. At an average of thirteen years (range, two to forty-one years), eleven of the sixty-seven patients who were managed at our institution died of the tumor; ten of these patients had a dedifferentiated tumor. Statistical analysis of the thirty-nine patients who had had the primary treatment at our institution revealed that incomplete resection was associated with an increased risk of local recurrence and that dedifferentiation markedly increased the risk of metastasis.
Asunto(s)
Neoplasias Óseas/patología , Osteosarcoma Yuxtacortical/patología , Adolescente , Adulto , Anciano , Neoplasias Óseas/mortalidad , Neoplasias Óseas/cirugía , Diferenciación Celular , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Invasividad Neoplásica , Metástasis de la Neoplasia , Recurrencia Local de Neoplasia , Osteosarcoma Yuxtacortical/mortalidad , Osteosarcoma Yuxtacortical/cirugía , Pronóstico , Factores de Riesgo , Análisis de Supervivencia , Tasa de SupervivenciaRESUMEN
Massive osteolysis is rare, peculiar, and enigmatic. The clinical, radiographic, and histologic features were reviewed in 11 cases. The patients were generally young (mean age, 20.7 years), and males outnumbered females. More than half the patients had involvement of either the femur or the ribs alone or including the vertebrae. On the basis of radiographic studies, the disease process is capable of originating either in bone or adjacent soft tissues. Histologically, the authors were unable to identify pathognomonic features. Other than excision of all involved tissue, surgical treatment was generally ineffective. Radiation therapy may be successful in eradicating the disease in selected cases.
Asunto(s)
Osteólisis Esencial/diagnóstico por imagen , Adolescente , Adulto , Preescolar , Terapia Combinada , Femenino , Fracturas Espontáneas/diagnóstico por imagen , Fracturas Espontáneas/patología , Humanos , Masculino , Osteólisis Esencial/patología , Osteólisis Esencial/terapia , RadiografíaRESUMEN
BACKGROUND: The incidence of osteosarcoma of the hand is about 0.18% of all osteosarcomas. METHODS: This study describes the clinicopathologic profile of 12 patients (3 Mayo Clinic and 9 consultation) with osteosarcoma of the hand (13 tumors). RESULTS: The patients (six male patients, five female, one unknown) were from 16 to 81 years of age (average, 45). Seven tumors were in phalanges and six in metacarpals. Nine tumors were classified as conventional, one as low-grade intraosseous, one as osteoblastoma-like, one as high-grade surface, and one as periosteal osteosarcoma. Recurrent disease developed in all patients in whom the initial operation resulted in intralesional excision or a marginal margin but not in the five patients in whom the initial operation achieved wide margins. Only one patient died of metastatic disease. CONCLUSIONS: The findings in this review support the conclusion that surgical treatment with a wide margin provides good results in patients with osteosarcoma of the hand.
Asunto(s)
Neoplasias Óseas/patología , Mano/patología , Osteosarcoma/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/cirugía , Femenino , Mano/diagnóstico por imagen , Mano/cirugía , Humanos , Masculino , Recurrencia Local de Neoplasia/cirugía , Osteosarcoma/diagnóstico por imagen , Osteosarcoma/cirugía , Pronóstico , Radiografía , Estudios RetrospectivosRESUMEN
We studied 12 cases of a rare primary bone neoplasm--fibrocartilaginous mesenchymoma--including five from the original report on this condition. The seven male and five female patients were 9 to 25 years old. The metaphyses of the long bones were the most common site, with the fibula accounting for a third of all cases. Histologically, the lesion contained spindle cells, bone trabeculae, and islands of cartilage. At least some of the cartilage was in the form of plates that resembled epiphyseal plates. Intralesional excision led to a high rate of recurrence, but no metastasis or death was related to the tumor. Our results indicate that the entity described by Dahlin and co-authors is histologically distinct and that its behavior does not merit the term malignant.
Asunto(s)
Neoplasias Óseas/patología , Mesenquimoma/patología , Adolescente , Adulto , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/epidemiología , Cartílago/patología , Niño , Femenino , Estudios de Seguimiento , Humanos , Masculino , Mesenquimoma/diagnóstico por imagen , Mesenquimoma/epidemiología , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Giant cell tumors (GCT) usually involve the epiphyses of long bones and only rarely involve the metaphysis or diaphysis without epiphyseal extension. METHODS: This report presents the clinical and pathologic features of 14 patients with metaphyseal and diaphyseal GCT. Of these tumors, 10 were metaphyseal; 2, metadiaphyseal; and 2, diaphyseal. RESULTS: The sites of involvement included the proximal tibia in six patients, distal radius in three, proximal fibula in one, distal fibula in one, distal ulna in one, proximal humerus in one, and distal femur in one. Radiographically, the tumors were lucent, and the majority were sharply marginated without sclerosis. By contrast with conventional epiphyseal GCT, which generally appear in the mature skeleton, a large proportion (50%) of the GCT in this series were in patients who had open epiphyseal growth plates. Despite the unusual clinical presentation, the behavior of metaphyseal and diaphyseal GCT was similar to that of the typical epiphyseal GCT. There was a 43% recurrence rate after curettage in this series. CONCLUSIONS: It is important to distinguish this subset of GCT from other giant cell-rich lesions more common in these sites, including aneurysmal bone cyst, osteosarcoma, and nonossifying fibroma.
Asunto(s)
Neoplasias Óseas , Tumores de Células Gigantes , Adolescente , Adulto , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/patología , Neoplasias Óseas/cirugía , Niño , Femenino , Neoplasias Femorales/diagnóstico por imagen , Neoplasias Femorales/patología , Neoplasias Femorales/cirugía , Peroné , Tumores de Células Gigantes/diagnóstico por imagen , Tumores de Células Gigantes/patología , Tumores de Células Gigantes/cirugía , Humanos , Húmero , Masculino , Persona de Mediana Edad , Radiografía , Radio (Anatomía) , Tibia , CúbitoAsunto(s)
Neoplasias Óseas/etiología , Ilion , Neoplasias Inducidas por Radiación , Neoplasias Primarias Secundarias/etiología , Osteosarcoma/etiología , Adenocarcinoma/radioterapia , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/patología , Radioisótopos de Cobalto/efectos adversos , Neoplasias del Colon/radioterapia , Femenino , Humanos , Ilion/patología , Persona de Mediana Edad , Neoplasias Inducidas por Radiación/patología , Neoplasias Primarias Secundarias/patología , Osteosarcoma/diagnóstico por imagen , Osteosarcoma/patología , RadiografíaRESUMEN
BACKGROUND: Most giant cell tumors (GCT) occur at the ends of long bones. There is little information about GCT of the skull bones. METHODS: The authors reviewed the Mayo Clinic files, which contained 546 cases of GCT, and their own consultation files, which contained approximately 1500 cases. RESULTS: Eleven tumors occurred in the sphenoid bone with extension to the surrounding bones and structures in 8 patients. One tumor (in Paget's disease) occurred in the frontal bone, one tumor was in the occipital bone, and one tumor was in the temporal bone. There were 4 men and 11 women whose ages ranged from 8 to 78 years, with a mean of 36.5 years. Radiographic findings were not suggestive of a specific diagnosis, although the features were those of an aggressive lesion. Histologically, the tumors had features typical of GCT. However, a prominent spindle cell component was seen in five tumors. The initial treatment in all patients but one was intralesional excision that was as complete as possible. The last patient had a wide excision and had soft tissue recurrence at 1 year. This was excised and she was free of disease at 2.7 years. Three patients died, one in the immediate postoperative period and the other two at 1.6 and 4 years with progression of tumor. One patient had postoperative radiation therapy and was without evidence of disease for 2 years when he was lost to follow-up. The remaining 10 patients all had postoperative radiation therapy; 6 patients were alive without disease from 4 to 34 years. However, one of these six patients had a recurrence that was treated surgically with additional radiation. Four patients were alive with tumor from 2.1 to 26 years at the time of this report. CONCLUSIONS: GCT of the skull bones is rare but should be distinguished from giant cell reparative granuloma because of the tendency for progression. Surgical ablation (as complete as possible) and postoperative radiation therapy seem to be the treatment of choice for GCT of the skull bones.
Asunto(s)
Tumores de Células Gigantes/patología , Neoplasias Craneales/patología , Adolescente , Adulto , Anciano , Niño , Terapia Combinada , Femenino , Tumores de Células Gigantes/diagnóstico por imagen , Tumores de Células Gigantes/terapia , Granuloma de Células Gigantes/patología , Humanos , Masculino , Persona de Mediana Edad , Radiografía , Neoplasias Craneales/diagnóstico por imagen , Neoplasias Craneales/terapiaAsunto(s)
Neoplasias Óseas/secundario , Melanoma/secundario , Neoplasias Cutáneas/patología , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/cirugía , Humanos , Masculino , Melanoma/diagnóstico por imagen , Melanoma/cirugía , Persona de Mediana Edad , Radiografía , Radio (Anatomía)/diagnóstico por imagen , Radio (Anatomía)/cirugíaRESUMEN
Tumors that metastasize are considered "malignant" by definition. However, benign giant-cell tumor of bone is an exception because of the potential for histologically benign pulmonary metastases, a fact seldom emphasized in the radiologic literature. We therefore report our experience with 13 cases of pulmonary metastasis among 475 patients (prevalence, 3%) in whom benign giant-cell tumor of bone was diagnosed before 1990 at our institution. Five (38%) of the 13 primary bone tumors were located in the distal radius. Local recurrence at the site of the primary bone tumor tumor occurred in seven patients (54%) before pulmonary metastases developed. The mean interval from the diagnosis of the primary bone tumor to the onset of pulmonary metastasis was 3.8 years, with a maximum of 10.7 years. Fifty-four percent of the patients (7/13) had pulmonary metastases 3 years after diagnosis of the primary bone lesion, and 92% (12/13) had pulmonary metastases 7.5 years after diagnosis. Overall mortality rate directly due to giant-cell tumor and its metastases was 23%. On chest radiographs and CT scans, pulmonary metastases appeared as rounded, nodular opacities of homogeneous density, ranging from 0.5 cm to 8.0 cm in diameter. Peripheral regions of the lungs were involved in 85% of the cases and basilar regions in 62%. Our study shows that benign giant-cell tumor of bone can produce pulmonary metastases, that metastases most often occurred with recurrent local disease and distal radial lesions, that the prognosis was relatively favorable, and that such metastases had no distinguishing radiologic features.
Asunto(s)
Neoplasias Óseas/patología , Tumores de Células Gigantes/secundario , Neoplasias Pulmonares/secundario , Adulto , Neoplasias Óseas/epidemiología , Femenino , Tumores de Células Gigantes/diagnóstico por imagen , Tumores de Células Gigantes/epidemiología , Humanos , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/epidemiología , Masculino , Prevalencia , Pronóstico , Radiografía , Estudios Retrospectivos , Factores de TiempoRESUMEN
Between January 1990 and January 1991, 87 patients (100 shoulder arthroplasties) were radiographically examined with both standard and fluoroscopically positioned views. The sets of radiographs were compared and assessed for acceptability (the ability to interpret the interfaces), presence or absence of radiolucent zones, and differences between size and location of radiolucent zones. Fifty-six of the standard radiographs were unacceptable because of obliquity of the x-ray beam protection; only three localized views were unacceptable (p < 0.0001). Nineteen of the unacceptable standard views had radiolucent zones detected on the comparable fluoroscopic views. Forty-four radiographic sets were acceptable for evaluation. Of these, 27 sets had no differences; standard views showed the interface changes better in one, and localized views were better in 76 (p < 0.001). Thus in 68 of 100 total shoulder arthroplasties evaluated, clinical information that was not seen on standard radiographs was identified on fluoroscopic views. We conclude that fluoroscopic positioning is superior to standard radiographs in detecting implant-bone interface changes.
RESUMEN
Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. This study reviews 27 cases of desmoplastic fibroma, consisting of 9 from the Mayo Clinic files and 18 from our consultation files. There was a male predominance, and 74% of the patients were in the first 3 decades of life. The most frequent sites of involvement were the metaphysis of long bones and the mandible. Radiographically, the tumors were lucent, expansile lesions with well-defined margins. Histologically, they contained slender spindle cells and various amounts of collagen fibers. En bloc resection is the treatment of choice because a high incidence of recurrence was noticed after lesional curettage.
Asunto(s)
Neoplasias Óseas/patología , Fibroma/patología , Adolescente , Adulto , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/cirugía , Nucléolo Celular/ultraestructura , Núcleo Celular/ultraestructura , Niño , Preescolar , Colágeno , Femenino , Fibroblastos/patología , Fibroma/diagnóstico por imagen , Fibroma/cirugía , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Pronóstico , RadiografíaRESUMEN
Congenital fibromatosis is a rare and benign myofibroblastic tumor that may occur in either a solitary or multicentric form. The soft-tissue form of this entity is well recognized. This report analyzes the clinical and pathologic features of 14 cases of congenital fibromatosis in which the tumor existed as a solitary lesion in bone. Most of the tumors (71%) were in patients who were 2 years old or younger. All but one tumor involved the craniofacial bones. Radiographically, they were purely lucent lesions in which a sclerotic rim was visible in each skull lesion. The myofibroblastic appearance closely resembled the microscopic features seen in the soft-tissue counterpart. For appropriate surgical management, it is imperative to recognize that this tumor occurs as a solitary lesion in bone and that it is distinct from other, more aggressive fibrous lesions.