Chronic mandibular hypomobility related to a unilateral accessory condyle. A case report.

BACKGROUND: Chronic mandibular hypomobility is characterized by a long-standing limitation of the mouth opening related to multiple etiologies, including elongation of the coronoid apophysis. Unlike the most frequent pathologies that cause coronoid elongation, such as hyperplasia, osteoma (OM), and osteochondroma (OC), the accessory mandibular condyle (AMC) is a rare entity. Clinical Presentation: The AMC shows a configuration similar to a normal mandibular condyle with an articular surface covered by fibrocartilage that articulates with the temporal wall of the zygomatic bone, and histologically, does not show neoplastic growth. The patient was treated with a coronoidectomy, removing the coronoid apophysis as well as the AMC. CLINICAL RELEVANCE: This case report presents a case of an AMC to describe the clinical, imaging, surgical, and histological characteristics, establishing its differential diagnosis with hyperplasia, OM, and OC of the coronoid apophysis.

Biopsia endoscópica de osteocondroma de fosa infratemporal / Endoscopic biopsy of infratemporal fossa osteochondroma

Presentamos el caso de una mujer de 13 años con un gran tumor de características óseas en la fosa infratemporal derecha, el cual fue biopsiado mediante un abordaje endoscópico transeptal transpterigoídeo. La biospia mostró un osteocondroma. Describimos el caso y discutimos sus aspectos relevantes.

We report the case of a 13-year-old woman with a large tumor with osseous appearance in her right infratemporal fossa, which was biopsied through an endoscopic transpterygoid approach. The biopsy showed an osteocondroma. We described the case and discuss its relevant aspects.

Osteocondroma escapular: un caso infrecuente / Scapular osteochondroma: an infrequent case

El osteocondroma de la escápula es un tumor beningno muy raro por su ubicación, que produce dolor y disfunción mecánica de la articulación cuando se establece en la superficie ventral de la escápula, afecta principalmente a los varones en los períodos del crecimiento óseo, entre 10 y 30 años de edad, representan el 14,4 % de todos los tumores de la escápula y el 49 % de los tumores benignos de la escápula. Surgen en las regiones metafisarias de los huesos largos (fémur, húmero, tibia), causando irritación mecánica que conduce a impotencia funcional, bursitis, e incluso fractura de la escápula, pocos casos en huesos planos han sido descritos. El diagnostico se realiza con la valoración física, clínica y exámenes complementarios, debiendo ser confirmado por el histopatológo previa biopsia. La indicación quirúrgica incluye la disfunción mecánica de una articulación o tendón en relación con el tamaño de la lesión, y del cambio sarcomatoso siendo el procedimiento de elección la escisión quirúrgica abierta. A continuación se describe el caso de una joven de 11 años a quien se diagnosticó un osteocondroma subescapular, previabiopsia escisional.

Osteochondroma of the scapulais a rare benign tumor in view of its location, which produces pain and mechanical dysfunction of the joint when it is on the ventral surface of the scapula, mainly affecting men during bone growth periods, betweenthe ages of 10 and 30, accounting for 14.4 % of allscapula tumors and 49 % of benign tumors of thescapula. They arise in the metaphyseal regions ofthe long bones (femur, humerus, tibia), causing mechanical irritation leading to functional impotence,bursitis, and fracture of the scapula. Few cases inflat bones have been described. Diagnosis is made with physical, clinical and complementary examinations, and must be confirmed by prior histopathology biopsy. The surgical indication includes mechanical dysfunction of a joint or tendon in relationto the size of the lesion, sarcomatous change being the procedure of choice in open surgical excision. The case of an 11-year-old girl who was diagnosed with a subscapular osteochondroma after previous excisional biopsy, is described below.

The clinical pathological significance of FRAT1 and ROR2 expression in cartilage tumors.

PURPOSE: Chondrosarcoma is a malignant bone tumor with poor prognosis. Surgical treatment is the first choice for chondrosarcomas. Chondrosarcoma is not sensitive to chemotherapy and radiotherapy. Identification of biological markers is important for the early diagnosis and targeted treatment of chondrosarcoma. This study investigated the protein expression and clinicopathological significance of ROR2 and FRAT1 in 59 chondrosarcomas and 33 osteochondromas. METHODS: ROR2 and FRAT1 protein expression in tissues was measured by immunohistochemistry. RESULTS: The percentage of positive ROR2 and FRAT1 expression was significantly higher in patients with chondrosarcoma than in patients with osteochondroma (P < 0.01). The percentage of positive ROR2 and FRAT1 expression was significantly lower in patients with histological grade I, AJCC stage I/II stage, Enneking stage I, non-metastatic and invasive chondrosarcoma than patients with histological grade III, AJCC stage III/IV, Enneking stage II + III, metastatic and invasive chondrosarcoma (P < 0.05 or P < 0.01). ROR2 expression was positively correlated with FRAT1 expression in chondrosarcoma. Kaplan-Meier survival analysis demonstrated that histological grade, AJCC stage, Enneking stage, metastasis, invasion, and ROR2 and FRAT1 expression significantly correlated with a short mean survival time of patients with chondrosarcoma (P < 0.05 or P < 0.01). Cox multivariate analysis showed that positive ROR2 and FRAT1 expression was an independent prognostic factor that negatively correlated with postoperative survival and positively correlated with mortality. CONCLUSION: Positive ROR2 and FRAT1 expression is associated with the progression and poor prognosis of chondrosarcoma.

Sinquinesis óculomotora: dos casos de pacientes excepcionales / Oculomotor synkinesis: two cases excepcional patients

Las sinquinesis son movimientos simultáneos o coordinados en secuencia de mivimientos de músculos suplidos por diferentes nervios o por ramas independientes del mismo nervio que ocurren luego de la recuperación de una lesión axonal periferica; son debidos a la dirección errónea ("misdirection") que toman algunos axones hacia otros músculos que no constituyen su objetivo o blanco; así, cuando el paciente intenta mover algunos músculos, ocurren contracciones involuntarias en otros, no esperadas anatómicamente. Es una suerte de recableado mal realizado y confuso. En las dos pacientes que constituyen nuestro informe, ocurrieron alteraciones óculomotoras excepcionales. En la primera de 60 años portadora de un aneurisma gigante del senocavernoso izquierdo, la sinquinesis nerviosa aberrante ocurrió entre los nervios craneales tercero y sexto. En la segunda de 22 años a quien se resecó un osteocondroma gigante de la fosa media derecha, desarrolló una parálisis total del tercer nerviocraneal con sinquinesis trigémino-oculomotora entre el músculo pterigoideo derecho y el elevador del párpado superior; así como también entre el tercero (recto inferior) y sexto nervios (recto externo) ipsolateral. Se revisa la literatura al respecto.

Synkinesis are simultaneous or coordinated sequential movements of muscles that are supplied by different nerves or have independent nerve branches. They occur after the recovery a peripheral axonal injury. They are due to axons taking a wrong direction ("misdirection") towards muscles that do not constitute their objetives or targets. Thus, when the patient attempts to move a muscle, other muscles show anatomically unexpected involuntary contractions. It is a sort of confusing rewiring. Our report is based on the exceptional oculomotor alterations that occurred in two patients. In the first patient, a 60 years-old female carrying a giant aneurysm of the left cavernous sinus, the aberrant nerve synkinesis ocurred between the third and sixth cranial nerves. In the second patient, a 22 year-old female to whom was resected a giant osteochondroma of the right middle fossa, developed a total paralysis of the third cranial nerve with trigeminal oculomotor synkinesis between the right pterygoid muscle and the elevator of the upper eyelid; as well as between the inferior rectus and ipsilateral external rectus. We review the literature on the subject.

Osteosarcoma arising from osteochondroma of the tibia: case report and cytogenetic findings.

Osteochondroma is a cartilage capped benign tumor developing mainly at the juxta-epiphyseal region of long bones. The rate of malignant transformation, mainly into chondrosarcoma, is estimated to be less than 1-3%. Transformation into osteosarcoma is very rare and has been reported only thirteen times. There is little information on treatment and outcome. We report the case of a secondary osteosarcoma arising in the left tibia of a 23-year-old male, 10 years after the initial diagnosis of osteochondroma and after two partial resections. Malignant transformation occurred at the stalk and not at the cartilage cap, as would normally be expected. Chromosome banding analysis revealed the karyotype: 46,XY, t(3;13)(q21;q34) [2]/46,XY [18]. Records from additional cases will help determine the parameters that define these rare secondary bone lesions.

Osteochondroma of the temporomandibular joint: a case report.

Osteochondroma of the mandibular condyle has been found in the oral and maxillofacial region rarely. This paper describes a case of osteochondroma of the mandibular condyle in a 20-year-old woman, who was referred to our service with facial asymmetry, prognathic deviation of chin, cross-bite to the contralateral side, changes in condylar morphology, limited mouth opening, and malocclusion. Computed tomography (CT) was performed for better evaluation to the pathological conditions on the temporomandibular joint. Based on the clinical examination, patient history, and complementary exams, the hypothesis of osteochondroma was established. Condylectomy was performed using a preauricular approach with total removal of the lesion. After 3 years of postoperative follow up and orthodontic therapy, the patient is symptom-free, and has normal mouth opening with no deviation in the opening pattern.

Osteochondroma of the temporomandibular joint: a case report

Osteochondroma of the mandibular condyle has been found in the oral and maxillofacial region rarely. This paper describes a case of osteochondroma of the mandibular condyle in a 20-year-old woman, who was referred to our service with facial asymmetry, prognathic deviation of chin, cross-bite to the contralateral side, changes in condylar morphology, limited mouth opening, and malocclusion. Computed tomography (CT) was performed for better evaluation to the pathological conditions on the temporomandibular joint. Based on the clinical examination, patient history, and complementary exams, the hypothesis of osteochondroma was established. Condylectomy was performed using a preauricular approach with total removal of the lesion. After 3 years of postoperative follow up and orthodontic therapy, the patient is symptom-free, and has normal mouth opening with no deviation in the opening pattern.

Osteocondroma de côndilo mandibular é raro na região craniofacial. Este artigo descreve um caso de osteocondroma de côndilo mandibular em uma mulher de 20 anos que foi encaminhada ao nosso serviço apresentando assimetria facial, desvio de mento, mordida cruzada para o lado contralateral, alterações na morfologia condilar, limitação de abertura bucal e maloclusão. Tomografia computadorizada foi realizada para melhor avaliação da condição patológica da ATM. Devido à base no exame clínico, histórico do paciente e exames complementares, foi estabelecida uma hipótese de osteocondroma. Um procedimento de condilectomia utilizando abordagem preauricular com uma total remoção da lesão foi executado. Após três anos de acompanhamento pós-operatório e ortodôntico, o paciente está livre dos sintomas e tem uma abertura normal sem desvio de padrão durante a abertura.

Epidemiology of bone tumors in Mexico City: retrospective clinicopathologic study of 566 patients at a referral institution.

A retrospective analysis of all bone tumors accessioned at a large referral center (Instituto Nacional de Rehabilitacion) in Mexico City between 2000 and 2005 is presented. A total of 6216 biopsies and surgical resection specimens were reviewed during this period, of which 566 corresponded to bone tumors. Benign bone tumors accounted for 71.6% of cases and malignant bone tumors for 28.4%. The tumors affected men in 53.7% of cases and women in 46.3% of cases, with an average age at presentation of 25 years. The femur was the most common location of the tumors (39.9%), followed by the tibia (17.7%) and humerus (11.8%). The commonest malignant bone tumors were osteosarcoma (46.6%) and chondrosarcoma (8.7%). Of malignant bone tumors, 18.6% corresponded to metastases of carcinomas from internal organs and 8.1% were multiple myeloma. The most common benign bone tumor was osteochondroma (43.7%) followed by giant cell tumor of bone (14.6%) and enchondroma (10.1%). The age distribution showed a peak in children and adolescents comprised predominantly of benign lesions and a second peak in young adults that corresponded to malignant bone tumors (principally osteosarcoma). Malignant bone tumors most often involved the femur, vertebra, and tibia. Our results parallel the findings previously reported in the world literature and show a similar distribution and epidemiology as in other developed and underdeveloped countries. Geographic location does not appear to represent a risk factor for any particular type of bone tumor and does not affect the age distribution, location, or histopathologic type of the lesions.

Endoscopic resection of symptomatic osteochondroma of the distal femur.

The surgical risk and complication rates for an open excision of a benign osteochondroma have been reported as high as 13%. The advent of minimally invasive techniques allows a surgical resection with a smaller incision and a potential for less postoperative morbidity. We presumed endoscopic resection of symptomatic osteochondroma of the distal femur would decrease postoperative morbidity of open surgery and improve functional outcome. We performed endoscopic resection of a symptomatic osteochondroma in seven patients among 24 who had surgical excision. Preoperative studies include radiographs and computed tomography scans. At a mean followup of 33 months, patients were functionally evaluated according to the Lysholm score and the International Knee Documentation Committee scale. Four exostoses were located at the anterolateral femoral cortex, one at the trochlea, and two at the anteromedial cortex. Histologic diagnosis of an osteochondroma was confirmed in all cases. The average Lysholm score improved 22 points from a mean of 74 preoperatively to 96 postoperatively, and according to the International Knee Documentation Committee scale, all patients showed a normal knee. In 7 of 24 patients with a symptomatic osteochondroma of the distal femur close to the knee, we performed endoscopic resection with low morbidity and prompt functional recovery.

Tumores del esqueleto: 20 años de experiencia (1984-2003) / Tumors of the skeleton: 20 years of experience (1984-2003)

Se presenta la experiencia del Servicio de Partes Blandas y Tumores Oseos del Hospital Oncológico Padre Machado, de Caracas, en el manejo de los tumores del esqueleto, por un período de 20 años (1984-2003). la evaluación de la experiencia fue retrospectiva, con un análisis objetivo estadístico lineal, presentándose el espectro de la patología atendida, y la diversidad de alternativas terapéuticas quirúrgicas utilizadas. Se reporta una incidencia preferencial de esta patología en los miembros inferiores (58,58 por ciento), particularmente alrededor de la rodilla (fémur distal y tibia proximal). El grupo de tumores más frecuentemente observado fue el de formadores de tejido óseo (20,95 por ciento, seguido de los formadores de tejido cartilaginoso (14,79 por ciento), tumor de células gigantes (9,93 por ciento), y las lesiones pseudotumorales (5,85 por ciento). Entre los tumores formadores de tejido óseo llama la atención la alta incidencia de las variedades malignas (90,52 por ciento); miemtras que en los formadores de tejido cartilaginoso, las variedades benignas fueron las más frecuentes (69,80 por ciento). Entre los tipos de cirugía realizadas, una proporción importante tuvo finalidad diagnóstica (58,41 por ciento), aunque en muchas de ellas la intención fue simultánea o adicionalmente terapéutica (escisión, drenaje, relleno, etc.). Entre las cirugías con finalidad terapéutica, hay una amplia diversidad de opciones, destacándose, la posibilidad de la práctica de una cirugía preservadora, siempre que se respeten los principios oncológicos quirúrgicos. se resalta la importancia del manejo de esta patología en centros espeializados.

The experience of the Service of Solft Tissue and Bone Tumors, of the Oncology Hospital Padre Machado, Caracas, in the management of the tumors affecting the skeleton, for a period of 20 years (1984-2003), is presented. The evaluation of this experience was a retrospective revision, with an objective statistics lineal analysis, showing the spectrum of the pathology, and the therapeutic surgical alternatives utilized. The report shows a higher incidence of this pathology affecting the lower limbs (58,58 per cent), particular..around the knee joint (distal femur and proximal tibia). The most frequent type of tumor was the bone forming tumors group (20.95 per cent), followed by the cartilaginous tissue forming tumors group (14.79 per cent, giant cell tumors (9.93 per cent), and pseudotumoral lesions (5.85 per cent). Among the bone forming tumor group, called our attention the prevalence of the malignant variety (90.52 per cent); whereas in the cartilaginous tissue forming type, the bening options were seen more frequently (69.80 per cent). Looking at the performed surgeries, an important proportion had a diagnostic goal (58.41 per cent), although many of them allowed a therapeutic solution at the same time (resection, drainage, folling defects, etc). Among the surgical options with a therapeutic goal, we performed a very large variety of them, proposing limb presenvation when the surgical oncology principles could be respected. The importance of treating this pathology in specialized centers is underlined.

Cervical spinal cord compression due to an osteochondroma in hereditary multiple exostosis: case report and review of the literature.

BACKGROUND: Hereditary multiple exostosis is a benign disorder characterized by multiple osteochondromas affecting long and flat bones, although occasionally vertebral column involvement can be seen. Cervical spinal cord compression in HME is a rare condition. The objective of this manuscript is to describe a rare case of cervical myelopathy due to an exostosis arising from C7 in a patient with HME and a comprehensive review of the current literature. CASE DESCRIPTION: We describe a case of HME in an 18-year-old girl with myelopathy characterized by quadriparesis due to an osteochondroma arising from the lamina of C7. The patient underwent surgery, and a laminectomy was performed with a complete removal of the exostosis and spinal cord decompression. One month after surgery, patient presented an excellent recovery without neurologic deficits. CONCLUSIONS: Cervical spinal cord compression resulting from osteochondroma is an extremely serious complication of HME. Neurosurgical approach should be recommended in order to achieve a spinal cord decompression, which usually results in excellent functional recovery.

[Osteochondroma of the atlantooccipital joint. Extreme lateral transcondylar approach. A case description]. / Osteocondroma de la articulación atlantoccipital. Abordaje extremo lateral transcondilar. A propósito de un caso.

INTRODUCTION: We describe a patient diagnosed as suffering from a skull base osteochondroma which affected the atlantooccipital joint and originated in the occipital condyle. It also displayed a growth toward the foramen magnum, which was resected using an extreme lateral transcondylar approach. CASE REPORT: Patient aged 35, with a one year history of vertiginous seizures and unsteady gait, associated with cervical pain that irradiated to the right upper extremity, dysphagia, changes in the tone of the voice and distal numbness of the four extremities. The patient was made to lie in the three quarter prone position and an incision was made in the skin from the C3 spinous apophysis to a point 2 cm below the end of the mastoid process, in relation with the transversal apophysis of C1. The intervention continued with early identification and rotation of the vertebral artery; total resection of the tumour (osteochondroma) with its base in the right occipital condyle and growth toward the foramen magnum, in which the greater resection of the posteromedial third of the condyle is included. CONCLUSIONS: The location of osteochondromas can vary widely, and the condyle is one of the least frequent places inside the occipital bone. The approach employed provides excellent access to the region, in particular to the atlantooccipital joint. The width and angle of exposition are increased as compared with the traditional suboccipital approach, which facilitates the radical resection of the lesion with no neural retraction and without any surgical complications.

Osteocondroma da foice: relato de caso e revisäo da literatura / Falx osteochondroma: case report and review of the literature

Descrevemos o caso de um paciente com osteocondroma intracraniano originado da foice cerebral. O tumor foi parcialmente ressecado. Discutimos os condromas da foice, em particular quanto a seus aspectos cirúrgicos e etiopatogênicos, comparados com a literatura e ressaltando o valor diagnóstico da tomografia computadorizada e da ressonância magnética.

[Falx osteochondroma: case report and review of the literature]. / Osteocondroma da foice. Relato de caso e revisão da literatura.

One case of an intracranial osteochondroma originating from the falx is described. The tumor was partially removed. Surgical aspects and etiopathogenesis of falx chondromas are discussed. The values of computerized tomography and magnetic resonance are emphasized.

Osteocondroma Subungueal / Subungueal Osteochondroma

Presentamos un caso inusual de osteocondroma subungueal a nivel del primer dedo del pie derecho.En la literatura revizada no es frecuente la localización referida de esta neoplasia benigna. El estudio radiológico muestra la excresencia ósea sobre la falange distal, adoptando la forma de espolón óseo. En la siguiente revisión se exponen las características clínicas, radiológicas e histopatológicas