Monostotic craniofacial fibrous dysplasia: report of two cases with interesting histology

Fibrous dysplasia (FD) is a relatively rare osseous disease of unknown etiology, wherein the normal bone is replaced by collagen-rich tissue, comprising of fibroblasts and variably abundant immature woven bone. Clinically, it may involve a single bone or multiple bones. It commonly arises in the jaw bone, skull, rib, and proximal femur. Those arising in the skull and the jaw are together termed "craniofacial fibrous dysplasia." The differential diagnosis at this location includes meningioma and metastatic carcinoma. In this report, we highlight two diagnostically challenging cases presenting with orbital swelling and headache as the main complaints. Our first case was misinterpreted as meningioma on intraoperative squash smear, and paraffin sections revealed characteristic features of FD. The second case highlights the morphological feature of non-specific cystic degeneration occurring in FD. Radiographs in such cases show cystic swelling, which is indicative of a secondary aneurysmal bone cyst.

Resección de quiste óseo aneurismático en húmero proximal con aplicación de injerto peroneo no vascularizado: Presentación de un caso / Resection of an aneurysmal bone cyst in the proximal humerus with a non-vascularized fibular graft: Case report

El quiste óseo aneurismático es una lesión benigna que afecta la médula de huesos largos, supone 6% de las lesiones óseas primarias y puede surgir secundariamente con otros tumores óseos benignos o malignos. Presentamos el caso de una mujer de siete años de edad con un quiste óseo aneurismático, diagnosticado por clínica, radiología, tomografía axial computarizada y confirmado por histopatología. Se le realizó resección con técnica eggshell e injerto óseo no vascularizado de peroné izquierdo, su evolución fue satisfactoria hasta los cuatro años de operada, lo que concuerda con lo descrito en la literatura mundial.

An aneurysmal bone cyst is a benign lesion involving the marrow of long bones; it accounts for 6% of primary bone lesions and may occur as a secondary lesion with other benign or malignant bone tumors. We describe herein the case of a seven year-old female with an aneurysmal bone cyst which was diagnosed clinically, radiographically and with a CAT scan, and confirmed with histopathology. Resection was performed using the eggshell technique and a non-vascularized left fibular bone graft. The patient did well up to the fourth postoperative year, which is consistent with what has been reported in world literature.

Quiste óseo aneurismático simulando tumoración de rinofaringe / Aneurismatic bone cyst simulate rhino pharyns tumor

Los quistes óseos aneurismáticos del área de cabeza y cuello representan menos del 5% de todos los tumores óseos. Son lesiones benignas, que comúnmente afectan las metáfisis de los huesos largos y las vértebras como en el caso que se describe a continuación, simulando una lesión de la rinofaringe. Se describe su manejo y tratamiento.

The aneurismal of bone cyst of the head and neck localization, represent less than the 5% of the all bone tumors. They are benign lesions, and commonly affecting the metaphysis of the long bones and vertebrae, like in the case we described below, it simulating the rhino pharynx lesion. We describe the management and treatment.

Benign tumors and tumor-like lesions of the calcaneum: A study of 12 cases.

Benign tumors of the calcaneum are rare. Cystic lesions such as simple bone cysts and aneurysmal bone cysts are commonly seen. Aims and Objectives: To evaluate tumors of the calcaneum, which were seen over a 12-year period. Materials and Methods: We analyzed noninfectious, noninflammatory, benign lesions of the calcaneum seen in the Orthopedic Out Patient Department from 1991 to 2003. Twelve such tumors were encountered. There were 11 males and one female and their ages varied from 18 to 53 years with a median of 31. Data was collected from the histopathology reports, radiographs, and inpatient and outpatient records. One of the coauthors reviewed the histopathologic findings of all the tumors. Results: Twelve benign lesions were seen in 12 patients. In our series, cysts predominated, with three aneurysmal bone cysts and five simple bone cysts. The other benign tumors were: one fibrous dysplasia, one vascular hamartoma, one osteoblastoma, and one chondromyxoid fibroma. The bone cysts were treated by curettage, with or without bone grafting, except for one large aneurysmal bone cyst, which was treated by excision of the calcaneum. The postoperative function in this patient was good, with modified footwear. Conclusion: The calcaneum is an uncommon site for most bone tumors, and in our series, bone cysts were the most common benign lesions. Curettage and bone grafting or the use of bone substitutes can be effectively used in the treatment of symptomatic bone cysts of the calcaneum.

Cisto ósseo aneurismático orbitário - estudo clínico-patológico: relato de caso / Aneurysmal bone cyst of the orbit - a clinicopathological study: case report

O cisto ósseo aneurismático orbitário é doença rara, usualmente acomete pacientes jovens e é causada por alterações hemodinâmicas intra-ósseas. Relatamos um caso de cisto ósseo aneurismático orbitário, encaminhado com suspeita clínica e radiológica de cisto dermóide, comentando seus aspectos clínicos e anátomo-patológicos.

Aneurysmal bone cyst of the orbit is a rare disease that usually affects young patients and is caused by osseous hemodynamic alterations. We report a case of aneurysmal bone cyst of the orbit, referred to us as a dermoid cyst, discussing its clinical and histopathological aspects.

Quiste óseo aneurismático de la columna vertebral: reporte de un caso / Aneurysmal bone cyst of the spine: case report

El quiste óseo aneurismático es un tumor de naturaleza neoplásica indefinida, de comportamiento benigno, crecimiento rápido y ocasionalmente de comportamiento agresivo, cuyo tratamiento de elección es la resección completa, aunque existe el riesgo de sangrado transquirúrgico excesivo. Se presenta el caso de una paciente con deformidad en columna torácica, con parestesias y debilidad muscular progresivas en extremidades inferiores, que evolucionó hasta la parálisis de dichas extremidades e incontinencia de ambos esfínteres. Mediante estudios de gabinete se localizaron lesiones líticas en cuerpos vetebrales T7 a T9 e invasión a conducto raquídeo. Los estudios electrofisiológicos identificaron bloqueo completo de la vía somatosensorial. Previa biopsia incisional, se realizó resección de la lesión y estabilización de la columna toracolumbar. La paciente evolucionó sin mejoría de la función medular. Los hallazgos morfológicos correspondieron a quiste óseo aneurismático en T8. Esta lesión se localiza principalmente en huesos largos y con mucho menor frecuencia en la columna vertebral, donde puede provocar inestabilidad y compresión de la médula espinal. Es posible confundirla con otras neoplasias, por lo que el diagnóstico definitivo mediante biopsia es imprescindible a fin de establecer el plan terapéutico adecuado, que elimine el riesgo de recurrencia o secuelas neurológicas asociadas, y lograr la estabilidad adecuada de los segmentos vertebrales afectados.

The aneurysmal bone cyst (ABC) is a fast-growing tumor of undefined neoplastic nature. It is occasionally an aggressive benign lesion whose treatment of choice is a complete resection, even though the risk of profuse transoperative bleeding exists. We present a female patient with thoracic spine deformity, with progressive paresthesias and muscle weakness of lower extremities that evolved to paralysis of both lower extremities and sphincter incontinence. Based on radiographic films, lytic lesions were identified at T7 to T9 vertebrae as well as medullary space invasion. In electrophysiologic tests, a complete somatosensorial pathway block was reported. Prior to resection of the neoplastic lesion and thoracolumbar stabilization, an incisional biopsy was performed. There was no postoperative medullary functional improvement. Morphological findings corresponded to an aneurysmal bone cyst at T8. This lesion is mainly located in the long bones and less frequently of the spine, where instability and medullary compression may occur. It is possible to confuse this neoplasia with other lesions. Hence, definite diagnosis with biopsy is necessary for determining an adequate therapeutic plan to eradicate recurrence risk or associated neurologic sequelae, as well as to gain proper stability at the involved vertebral segments.

Aneurysmal bone cyst of the coronoid process of the mandible.

A rare case of aneurysmal bone cyst (ABC) located in the coronoid process of the mandible in a 12-year-old girl is presented. Treatment consisted of excision of the lesion through preauricular, submandibular and intraoral approach. An access osteotomy distal to second molar region was required to gain access to medial side of the coronoid process. To our knowledge, this is the third case of an aneurysmal bone cyst of the coronoid process of mandible. While examining a patient with a large expansile intrabony jaw cavity with thin peripheral bone, which is filled with blood without presence of bruit, thrills and pulse pressure, the diagnosis of aneurysmal bone cyst should be on top of the differential diagnosis list. Seventy-four to eighty-five percent of aneurysmal bone cysts of jaws occur in 10-20 years age group. Therefore, a pediatric dentist may be the first person to see such a lesion.

Quiste aneurismático del maxilar superior. Reporte de un caso / Aneurysmal bone cyst of the maxilla. Report of one case

Aneurysmal bone cysts are benign fibro osseous lesions, rarely present in maxillary region. We report the CT and morphological findings of aneurysmal bone cyst of the maxilla in a 15 years old female. The patient was subjected to a maxillectomy, preserving the orbit floor. The postoperative evolution has been uneventful. The tumor mainly involved the maxillary sinus with extension to the orbit wall and osseous palate. Very few cases of aneurysmal bone cysts of the maxilla have been reported in the literature.

Quiste óseo aneurismático de Patela: informe de un caso / Patella aneurismal bone cyst: case presentation

Informamos el caso de un paciente masculino de 15 años que ingreso por Sala de Emergencia de Ortopedia en el Hospital Escuela con historia de dolor, edema y limitación funcional en la rodilla izquierda. Radiológicamente presentaba megatela

Intracranial aneurysmal bone cyst manifesting as a cerebellar mass.

A 17-year-old boy presented with symptoms of raised intracranial pressure for a month. Investigations revealed a large extra-cerebellar mass. The lesion was radically resected. It arose from the petrous bone. Histology revealed that the lesion was an aneurysmal bone cyst [ABC].

Aneurysmal bone cyst of the temporal bone: a case report.

Aneurysmal bone cyst in temporal region is rare. It is a benign condition and may extend intracranially. Total surgical removal is recommended, whenever possible in one stage or in multiple stages. Prognosis is excellent and total removal will effect a cure. An unusual case of aneurysmal bone cyst of temporal region is reported.

Petrous apex region. Surgical anatomy, lesions, and operative techniques

The author describes the anatomy of the region of the petrous apex giving emphasis to the limits of surgical resection. The cochlea, located at the apex of the angle formed by the intersection of a line along the sphenopetrosal groove and a line from the facial hiatus to the internal auditory canal, has to be protected to preserve hearing. Surgical approaches can be divided in the posterior presigmoid approach that provides exposure to the posterior petrous bone and upper two thirds of the clivus, and the anterior subtemporal approach that provides exposure to the trigeminal ganglion, petrous carotid artery, Meckel's cave, and posterior cavernous sinus. Opening of Meckel's cave provides additional space for medial and inferior resection of the petrous apex. Two illustrative cases of primary petrous apex lesions are presented to demonstrate the surgical exposure obtained with each approach

Punção aspirativa por agulha fina de cisto ósseo aneurismático: relato de caso / Fine needle aspiration cytology of aneurysmal bone cyst: case report

O diagnóstico de tumores ósseos deve ser realizado baseado na correlação dos dados clínicos, radiológicos e anatomopatológicos. Antes do tratamento, é freqüentemente necessário um diagnóstico morfológico. A citologia pode ajudar a estabelecer um diagnóstico presuntivo antes da cirurgia. É um método tecnicamente simples e rápido, que fornece valiosas informações suplementares aos dados clínico-radiológicos. Esse trabalho relata um caso de cisto ósseo aneurismático diagnosticado por punção aspirativa por agulha fina. As vantagens e limitações do método são discutidas.

Quiste óseo aneurismático

Hemos estudiado 85 casos de quistes óseos aneurismáticos, en los que hallamos como principal manifestación clínica, el tumor y el dolor. Radiológicamente, imágenes osteolíticas multicavitarias expansivas, preferentemente en los sectores metafisarios de los huesos largos, donde predomina (fémur, 21,2 por ciento). La mayor incidencia por edad correspondió a la 2da. década de la vida (47,1 por ciento), con un ligero predominio, en nuestro material, del sexo masculino (53,0 por ciento). El estudio anatomopatológico hizo evidente, como se conoce a través de las descripciones clásicas, una lesión ósea multicavitaria con contenido hemático y paredes o septos parduscos, con trama ósea, las que histológicamente no tienen cubierta endotelial, sino condensación de elementos celulares fusiformes. El tratamiento de elección es el quirúrgico, decidiéndose el curetaje o la resección, según el tamaño y la localización. Las recidivas en nuestro material fueron muy bajas (5,8 por ciento), pero carecemos de información completa como para considerar ese dato representativo