1/23. Orbital osteoclastoma of apparent extraskeletal origin in a pagetic patient: a case report.A large mass in the right orbit, causing proptosis, ptosis of the right upper eyelid, and limitation to eye movements, was surgically removed from a 51-year-old woman suffering from Paget's bone disease (PBD). Histologically, a giant cell tumor of the bone (osteoclastoma) was diagnosed. No bony involvement was apparent either operatively, microscopically, or on preoperative computed tomographic scans. The neoplasm has not recurred in a 3-year follow-up. In addition to the fact that osteoclastoma complicating PBD is rare, the extraskeletal origin of the tumor is a matter of interest and can be tentatively explained by an unusually powerful systemic stimulus acting on circulating osteoclast precursors. HUM PATHOL 31:1527-1531.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/23. Malignant giant-cell tumor of the parietal bone: case report and review of the literature.OBJECTIVE AND IMPORTANCE: Giant-cell tumors (GCTs) are primary bone tumors that involve long bones in 75 to 90% of patients. They seldom develop in the cranium and are very rare in patients older than 60 years of age. A GCT rarely occurs with Paget's disease; when it does, however, it is most commonly associated with the polyostotic form and tends to involve the craniofacial bones. Pagetic GCTs are less aggressive than GCTs that are not associated with Paget's disease. CLINICAL PRESENTATION: We report the case of an 81-year-old woman with a painless left parietal mass and asymptomatic monostotic parietal Paget's disease. INTERVENTION: Surgical resection was performed, and histological examination of the lesion demonstrated Paget's disease with a malignant GCT. An incidental, low-grade, small-cell lymphocytic lymphoma also was noted. The patient experienced local recurrence of the malignant GCT and eventually died after developing pulmonary metastases of the malignant GCT. CONCLUSION: This case is the first reported example of a patient with a malignant GCT of the cranium associated with monostotic Paget's disease. It provides evidence that not all pagetic GCTs in the cranium are benign, as has been reported.- - - - - - - - - - ranking = 4keywords = giant (Clic here for more details about this article) |
3/23. Ultrastructural features of giant cell tumors in Paget's disease.Giant cell tumor is a rare complication of Paget's disease. This association is especially notable in patients originating from Avellino, italy. Many types of evidence point to a viral etiology for Paget's disease and giant cell tumors arising in it. Three patients who had giant cell tumors and Paget's disease were studied. Two of the patients have a connection to Avellino (one was born in Avellino, and one descended from natives of Avellino). Distinctive light microscopic and ultrastructural features common in these three patients were identified. In all three patients, the giant cell tumors had peculiar irregular aggregates of microfilaments of uncertain genesis. The possibility that these reflect viral infection is discussed.- - - - - - - - - - ranking = 7keywords = giant (Clic here for more details about this article) |
4/23. Malignant giant cell tumor of the sphenoid.Malignant giant cell tumors (MGCTs) of the sphenoid sinus are extremely rare neoplasms. They are challenging to diagnose and difficult to treat because of their skull base location. To the best of our knowledge, we report the first case of a primary MGCT of the sphenoid arising in a patient with Paget's disease. A 77-year-old man presented with epistaxis and a history of Paget's disease. There was normal cranial nerve function although radiographic images disclosed a large mass centered in the sphenoid sinus and extending into the ethmoid and maxillary sinuses. Excisional biopsy revealed a MGCT composed of a cellular stroma with increased mitotic activity and necrosis with giant cells present throughout. Additional therapy was declined and the patient died with disease 7 months later. Because of their rarity, no treatment guidelines exist for the management of MGCTs of the sphenoid. We discuss both the diagnostic and therapeutic considerations based on a review of the pertinent literature.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
5/23. Bone scintiscanning in osteolytic lesions.Osteolytic lesions seen on plain radiographs can be caused by various disorders of the bones such as simple bone cyst, aneurysmal bone cyst, plasmacytoma, giant cell tumor, eosinophilic granuloma and tuberculosis. We studied prospectively Tc-99m-methylene diphosphonate bone scan findings in osteolytic lesions seen radiologically and followed them to histopathology. Interestingly, the scans in these patients helped to show if the lesions were monoostotic or polyostotic and, in some cases, ruled out malignant or infective etiology.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
6/23. Giant cell tumours in mandible and spine: a rare complication of Paget's disease of bone.The case of a man who was diagnosed as having polyostotic Paget's disease at the age of 52 years is described. He developed a rare neoplastic complication of Paget's disease--a giant cell tumour in his mandible, which was excised. Nine years after the diagnosis of this tumour he developed a new giant cell tumour arising from the L3 vertebral body. He was born in Avellino in italy, from where five other cases of giant cell tumours arising from Pagetic bone disease have been reported. No family relationship between our patient and the other cases was established. His Paget's disease was particularly aggressive and resistant to treatment with two single high dose infusions of pamidronate almost two years apart.- - - - - - - - - - ranking = 3keywords = giant (Clic here for more details about this article) |
7/23. Giant cell tumor and Paget's disease of bone in one family: geographic clustering.Giant cell tumor is a rare complication of Paget's disease of bone. Typically, this tumor occurs in the case of polyostotic disease and only in pagetic bones. This tumor rarely has been seen in multiple family members who have Paget's disease, although Paget's bone disease clearly has a hereditary component. Our report documents four cases of polyostotic Paget's bone disease complicated by benign giant cell tumor. In two patients, the giant cell tumor also was multifocal. All patients were from one family. They were born in Avellino and reside in Campania, a Southern Italian region. The ancestors of the patients with familial giant cell tumor in Paget's bone disease were born in the same geographic area. These data suggest that a combination of environmental and genetic factors could be responsible for linkage of the patients born in Avellino with this neoplasm that is highly unusual in patients with Paget's disease of bone.- - - - - - - - - - ranking = 3keywords = giant (Clic here for more details about this article) |
8/23. Multiple giant cell tumors and Paget disease of bone: radiographic and clinical correlations.The clinical and radiographic findings of four patients with multicentric giant cell tumor (GCT) of bone and Paget disease were retrospectively reviewed. Three patients underwent magnetic resonance (MR) imaging evaluation; all patients underwent computed tomography (CT). The MR characteristics of the bone component in pagetic GCT appeared to reflect the pagetic phase; a sclerotic pattern was largely represented by hypointense marrow signal intensity on images obtained with both long and short repetition times (TRs) and echo times (TEs). Conversely, a tumor appearing in a mixed pagetic phase demonstrated more heterogeneous signal intensity with all pulse sequences. Extensive soft-tissue components, noted in all cases, showed largely intermediate signal intensity on short TR/TE images and foci of increased signal intensity on longer TR/TE images. In most cases, dramatic reduction in tumor bulk was noted with the use of steroids alone. An awareness of this entity is important because the appearance of lytic lesions with soft-tissue extension in patients with Paget disease does not necessarily imply a grave prognosis. Serial CT or MR imaging is helpful in monitoring the remissions and exacerbations that reflect response to therapy in Paget disease and GCT.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
9/23. An epiphyseal giant cell tumor associated with early Paget's disease. A case report.An epiphyseal giant cell tumor occurred in a 50-year-old woman in the absence of roentgenographic signs of Paget's disease adjacent to the tumor area. Only one case with similar features has been previously reported. Treatment increased bone remodeling changes in the whole femur. Two years later the high bone turnover of the femur was arrested. This case suggests that a giant cell tumor of the epiphysis developed in an early phase of Paget's disease.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
10/23. Giant cell tumor in Paget's disease of bone: familial and geographic clustering.Four patients with benign giant cell tumor and one patient with probable benign giant cell tumor associated with Paget's disease of bone are reported. The familial and geographic clustering of these cases is unique in that three patients were related and all patients traced ancestral roots to the same area of southern italy. Tumors arose from the cranial or facial bones in three patients and from vertebral bodies in two patients. All caused symptoms by local compression, and treatment by curettage or radiotherapy was successful in all patients. Three separate tumors in one patient shrank dramatically in response to treatment with high doses of dexamethasone, and one patient whose tumor caused spinal cord compression showed marked improvement in neurologic function on therapy with dexamathasone.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
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