Fibrous dysplasia in combination with aneurysmal bone cyst of the occipital bone and the clivus: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Fibrous dysplasia of the cranium is a relatively uncommon disorder that affects primarily the anterior cranial region; its occurrence in the cranial base in combination with aneurysmal bone cyst (ABC) constitutes an extremely rare condition, only two cases of which have been reported previously in the literature. It is important to recognize and treat these cases properly because of the special location in the cranial base and the possibility of neural structure impingement. CLINICAL PRESENTATION: We report the case of a 19-year-old man with a slowly enlarging mass of the occiput, with computed tomographic and magnetic resonance imaging revealing involvement of petrous and basisphenoid bone and growing ABC. INTERVENTION: Open biopsy confirmed the diagnosis of fibrous dysplasia. Partial excision of the lesion and removal of the ABC were performed in a second stage after embolization. CONCLUSION: ABC associated with fibrous dysplasia of the cranial base may enlarge rapidly after puberty and require excision. This is facilitated by preoperative embolization.     

Intradiploic hematoma after skull fracture: case report and literature review

BACKGROUND: Intradiploic hematoma of the skull was first reported in 1934. The pathogenesis of this lesion is unclear. It is a very rare benign reactive process occurring after minor head trauma, with only seven cases reported in the literature to date. CASE DESCRIPTION: A 3-year-old right hand dominant male presented with a non-tender parietal scalp swelling of a 1-year duration. History included a skull fracture located in the same region 24 months before presentation. Neurological examination was unremarkable. Pathological examination after curettage of the lesion revealed features consistent with organizing hematoma. CONCLUSIONS: The pathology of chronic diploic hematoma mimics aneurysmal bone cyst, giant cell tumor, giant cell reparative granuloma, fibrous dysplasia, eosinophilic granuloma, intradiploic epidermoid and dermoid cyst, cavernous hemangioma, circumscribed osteomyelitis, and tuberculous granuloma. Chronic diploic hematoma is a lesion that must be differentiated from various skull lesions both radiologically and histologically as it is amenable to treatment with a complete cure once excised.     

Aneurysmal bone cyst of the cranial base treated by partial resection and calcitonin injection. A case report

STUDY DESIGN: First published report of a cranial aneurysmal bone cyst (ABC) treated successfully with intralesional injection of calcitonin. OBJECTIVES: To describe a safe and effective treatment method for ABCs of the cranial base. Summary of background data: ABC is a rare form of dystrophic pseudotumor. Less than 100 cases involving the skull have been reported in the literature, most of them localised in the cranial vault. Cranial base locations are rare and difficult to treat. We selected this treatment after a very rapid recurrence of the lesion following a partial resection. Method. - After a partial resection of a 10 cm petro-occipital ABC that encased the vertebral artery and the lower cranial nerves, an Ommaya reservoir was implanted with a catheter tip inside the ABC. Repeated intralesional injections of calcitonin were performed through the reservoir. RESULTS: Shrinkage of the cyst occurred with disappearance of its heterogeneous cystic content and ossification of its walls. There was no complication and the lesion remains quiescent at a 3 year follow-up. CONCLUSION: We reviewed the pertinent literature concerning percutaneous treatment of ABC. The percutaneous intralesionnal injection of calcitonin was reported in the literature only in 3 publications reporting 9 cases that did not involve the skull. This treatment seems safe and effective, worthy in cranial base ABCs that are difficult to resect completely.     

Schwannoma of the fibula

We describe a schwannoma located in the mid-diaphyseal region of the fibula of a 14-year-old boy. Radiologically this was an expansile, lytic, globular and trabeculated lesion. MRI showed a narrow transition zone with a break in the cortex and adjacent tissue oedema. Differential diagnosis included schwannoma, fibrous dysplasia, giant cell tumour and aneurysmal bone cyst. The tumour was excised en bloc, with marginal resection limits, and there has been no recurrence two years after surgery. Histopathological examination confirmed the diagnosis of classic schwannoma. There were typical hypercellular Antoni A zones, less cellular Antoni B zones, and diffuse immunoreactivity to S100 protein. This is the first report of schwannoma involving a long bone in a child.     

Giant-cell reparative granuloma of the hand and foot bones

Giant-cell reparative granuloma (GCRG) is an uncommon benign reactive intraosseous lesion. It occurs in the skull, jaw, hand, foot, and facial bones and rarely in other skeletal sites. It is a solitary, lytic, expanded lesion and infrequently may extend into the surrounding soft tissue. Histologically, it is composed of fibrous stroma with spindle-shaped fibroblasts, multinucleated giant cells, and inflammatory mononuclear cells. Areas of hemorrhage are uniformly present. It may be difficult to distinguish this entity from an aneurysmal bone cyst, giant-cell tumor, or brown tumor of hyperparathyroidism because of roentgenographic and histologic similarities. Accurate diagnosis is essential for appropriate treatment; serum calcium, phosphorus, and parathyroid hormone levels should be measured. Curettage and bone graft are effective treatments for both primary lesions and recurrences. Second recurrences are rare.     

Giant cell lesions of the skull

Summary Giant cell tumor, chondroblastoma, brown tumor of hyperparathyroidism, aneurysmal bone cyst, fibrous dysplasia, and reparative giant cell granuloma have been discussed with reference to their behavior in the skull. They have been compared with special regard to clinical presentation, roentgenographic appearance, gross and microscopic pathologic characteristics, treatment, and prognosis.     

Aneurysmal bone cyst with fibrous dysplasia of the parietal bone. Report of two cases

The diagnostic and surgical aspects of aneurysmal bone cysts contiguous to fibrous dysplasia of the parietal bone are reported in two adolescents. While aneurysmal bone cyst and fibrous dysplasia are known to be associated, the association is rare and has not been reported previously in the calvaria.     

Solid Aneurysmal Bone Cyst of the Metacarpal Bone

Background: A solid aneurysmal bone cyst is a rare tumor-like lesion of bone. As conventional aneurysmal bone cyst it is a benign, although locally destructive lesion. While the aneurysmal bone cyst is characterized by cyst-like walls of predominantly fibrous tissue filled with free-floating blood, the solid variant shows essential findings identical to those seen in the walls of conventional aneurysmal bone cysts, except that the lesion almost completely lacks the blood-filled spaces and the cyst walls. Differential Diagnosis: Aneurysmal bone cysts and solid aneurysmal bone cysts may easily be mistaken for a malignant tumor, both radiologically and histologically, due to their great rate of growth, tremendous destruction of bone, and marked cellular exuberance in the early to mid phase of development. Case Study: A case of solid aneurysmal bone cyst of the fourth metacarpal is presented. In spite of the relatively unusual location it allows to clearly demonstrate both, the task of correct differential diagnosis and the surgical spectrum of therapy including local recurrence.     

Monostotic fibrous dysplasia of the cranial vault: a case report

Fibrous dysplasia, when it occurs in the craniofacial region, mostly involves the skull base and is rarely localized in the cranial vault. Although there have been several reports on magnetic resonance imaging (MRI) findings of fibrous dysplasia involving the skull base, cases occurring in the cranial vault have seldom been reported. We describe here a rare case of monostotic fibrous dysplasia that occurred in the parietal bone and discuss the characteristics of the MRI findings. A 47-year-old female was admitted to our hospital with a complaint of vertigo. A computed tomography (CT) scan did not reveal any intraparenchymal lesions in either the infra- or supratentorium, and her vertigo improved immediately without any treatments. However, a solitary osteolytic lesion was found incidentally in the left parietal bone. MRI showed that the lesion demonstrated hypointensity on T1-weighted images and hyperintensity on T2-weighted images, and was enhanced heterogeneously following injection of Gadolinium-DTPA. Removal of the parietal bone containing the lesion was performed according to the patient's wishes. The histopathological findings of the removed tissues corresponded to fibrous dysplasia. Although it is well known that craniofacial fibrous dysplasia demonstrates iso- or hypointensity on T1- as well as T2-weighted images, in the present case, the lesion showed apparent hyperintensity on T2-weighted images. These findings suggest that fibrous dysplasia can display various MR intensities depending on its origin.     

Case of calvarial fibrous dysplasia presenting with cyst degeneration

A 49-year-old female developed a painful dysesthesia in the left forehead 10 years after having become aware of a subcutaneous mass accompanied with mild tenderness. Her medical history was unremarkable. Neurological examination showed normal findings with intact facial nerve function. Blood examination was normal. Neuroimaging revealed a cystic bony mass and an adjacent patchy diploic lesion in the left temporal bone. She underwent total tumor resection by frontotemporal craniotomy. Intraoperatively, a bony defect was noted in the lower surface of the patchy lesion communicating with the diploic venous system. A part of the dura mater was invaginated in the bony defect. The patchy lesion was found to communicate with the bony cyst through the diploic structure. Histological appearance of the bony cyst was consistent with the fibrous dysplasia accompanying cyst degeneration. Histological findings of the patchy lesion were identical to those of the bony cyst except for cyst degeneration. We assumed that the diploic venous system and the meningeal vessel might have been associated with the etiology of cyst degeneration in the present case of fibrous dysplasia.     

Aneurysmal bone cysts in the cranial vault and base of skull

Three cases of aneurysmal bone cyst of the skull are reported. The localization in the base of the skull (two cases) is extremely rare and simulates a space-occupying intracerebral lesion. The clinical and microscopic findings, and their significance for the differential diagnosis from malignant brain tumors, are described.     

Case of aneurysmal bone cyst of the skull

A case of aneurysmal bone cyst of the right temporal bone was reported. The patient was a 36-year-old male who was admitted to our hospital with complaints of decreased right hearing and transient impairment of the right vision. A large tumor was palpated on the right temporal bone. Neurological examinations revealed right auditory loss, along with right facial weakness of peripheral type, and minimal pyramidal signs on the right side. The results of the laboratory examination proved to be normal. Neuroradiological examinations tended to be quite impressive. Plain x-ray films of the skull showed a blow out appearance of the right temporal bone and a bone decay in the right middle fossa, the right anterior clinoid process, and the posterior half of the right zygomatic arch. Angiograms revealed a right temporal extradural mass lesion without vascularity. CT scans showed a moderately enhanced mass lesion of soap bubble appearance in the right middle fossa. Surgical treatment was done under the diagnosis of extradural bone tumor. The operative findings disclosed many cysts containing bloody fluid and xanthochromic fluid. Numerous multinucleated giant cells, spindle cells, and foam cells were found in the microscopic examination. On the basis of macroscopic and microscopic findings, the patient was diagnosed as having an aneurysmal bone cyst. The aneurysmal bone cyst of the skull is very rare and sixteen cases have been reported in detail. This case had two interesting points, one was the transient impairment of visual acuity and the other was the CT findings.(ABSTRACT TRUNCATED AT 250 WORDS)     

Polyostotic Fibrous Dysplasia with Craniofacial Localization Presenting with Frontal Lobe Compression in a 14-year-old Girl

Summary  We describe a rare case of polyostotic fibrous dysplasia with craniofacial localization associated with involvement of the spine and extremities. A 14-year-old girl presented with progressive headache, left frontal swelling, exophthalmos of the left eye, deformity and palpable mass in the left frontal area. Cranial computerized tomography revealed extensive involvement of all cranial bones except right frontal and right parietal bones. Most of the facial bones were invaded. Especially, there was the involvement of ethmoidal air sinuses and frontal sinus also. Computerized tomography showed left frontal lobe compression. In addition, the spine and bones of the extremities were involved in the patient. Craniofacial approach was planned. Cranial surgery was performed by an extradural frontal approach. Firstly, the frontal cyst was excised. For left frontal lobe decompression, we removed successfully all the abnormal bones causing mass effect and frontal deformity. Secondly, facial surgery was performed with external approach. An attempt to remove all of the involved bone is necessary, as the lesion may recur and grow if a portion of dysplastic bone is left in place. However, the present case suggests that removal of all the involved bones in the polyostotic fibrous dysplasia may be possible in spite of extensive involvement. In this situation, it may be removal of only abnormal bones responsible for compression of affected neural elements is indicated.     

Aggressive aneurysmal bone cyst in association with polyostotic fibrous dysplasia: A case report

IntroductionAneurysmal bone cyst occurring in the setting of previously diagnosed fibrous dysplasia is rare. While both are benign processes, pain, compression of nearby structures and risk of fracture can require treatment.Presentation of caseIn this report, we describe a 56 year old male who developed an aggressive aneurysmal bone cyst secondary to fibrous dysplasia in the proximal tibia over a period of 8 months. He required an above knee amputation for disease and symptom control due to the aggressive nature of disease and medical comorbidities.DiscussionThe diagnosis of a secondary lesion can prove difficult. It is important to exclude a malignant disease process, particularly when imaging demonstrates an aggressive appearance. In this case, repeat imaging, CT guided biopsies and an open biopsy were performed to exclude malignancy prior to definitive surgical management.ConclusionIn order to exclude secondary lesions, we suggest further investigation for new onset pain in the setting of a benign lesion.     

Aneurysmal bone cyst of all humeral diaphysis (a case report)

Aneurysmal bone cyst (ABC) is a benign osteolytic lesion usually occurring in the metaphysis of long bones. Authors report a case of a 21-year-old man with an expanding aneurysmal bone cyst in the entire humeral diaphysis and both metaphyses, discovered incidentally. Authors present the therapeutic difficulties encountered and discuss theepidemiology, diagnosis and management of ABCs.     

Aneurysmal bone cyst in association with fibrous dysplasia. A case report

A patient with polyostotic fibrous dysplasia had several fractures of the right lower limb. An above-knee amputation was eventually performed, followed by arthrodesis of the hip. Five years later the stump became painful and swelled with dramatic rapidity. Biopsy showed that this was not due to malignant change, but that an aneurysmal bone cyst had developed in association with the fibrous dysplasia.     

Intracortical and subperiosteal lesion of unknown etiology

A painful intracortical and subperiosteal lesion of the fibula with a 14 year follow-up is reported to regress to a painfree state. Infection is favored in the differential diagnosis. Biopsy with histological and radiographical correlation are essential for exclusion of: osteoid osteoma, osteoblastoma, periostitis, glomus tumor, eosinophilic granuloma, enostosis, hemangioma of bone, giant cell tumor, simple cyst, aneurysmal bone cyst, non-ossifying fibroma, polyostotic fibrous dysplasia, hyperparathyroidism, Paget's disease, localized area of avascular necrosis, stress fracture and even metastatic disease.     

Aneurysmal bone cyst of the ethmoid

Although nearly 500 cases of aneurysmal bone cyst have been recorded in the English literature, involvement of the facial bone is uncommon. To our knowledge, this is the first case of extragnathic, facial aneurysmal bone cyst to be reported in the English literature. A left ethmoid aneurysmal bone cyst was found in a 20-year-old pregnant woman who had a 5-month history of progressive left periorbital swelling, left cystic nasal mass, progressive nasal obstruction, blurred vision, and occasional diplopia. The diagnostic evaluation included a sinus series, facial lamiograms, and an EMI scan. Needle aspirates taken frequently from the intranasal cyst consisted of a dark bloody fluid. The surgical procedure, using external rhinotomy approaches, pathologic findings, and a literature review are presented.     

Aneurysmal bone cyst arising from a fibrous metaphyseal defect in a toddler

This is a case of aneurysmal bone cyst arising from a fibrous metaphyseal defect of the proximal tibia in a toddler. A 32-month-old girl presented with physiologic bowing with a small metaphyseal defect at the medial aspect of the left proximal tibia. A large aneurysmal bone cyst, occupying almost the entire metaphysis of the proximal tibia, evolved from this lesion during the following 1 year 3 months. The lesion healed uneventfully after curettage and bone grafting. This case emphasizes the importance of regular followups to observe the clinical course of a fibrous metaphyseal defect during childhood.