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Giant-cell tumor of the sphenoid bone. Review of 10 cases.

Giant-cell tumor rarely affects the sphenoid bone. Ten cases of this tumor seen at the Mayo Clinic are reviewed, bringing the number of cases in the world literature to 31. These tumors, which present in the second and third decades of life and are slightly more frequent in women than in men, may present with various symptoms, including headache, visual field defects, blindness, and diplopia. The symptom complex and roentgenographic findings are seldom, if ever, sufficiently characteristic to allow preoperative diagnosis. The microscopic differential diagnosis of giant-cell tumor of the sphenoid region includes giant-cell reparative granuloma, aneurysmal bone cyst, fibrous dysplasia, and "brown tumor" of hyperparathyroidism. Interesting features of this series include the presence of multifocal giant-cell tumor in one case, and the absence of Paget's disease of bone in every case. Complete follow-up review in these cases allowed a discussion of therapy and prognosis; it appears that subtotal excision with postoperative radiation therapy is the treatment of choice. While the histological appearance of giant-cell tumor is characteristic, caution must be exercised in interpreting small samples, and various reparative and metabolic disorders must be considered in the differential diagnosis.

Adolescent↗

Endoscopic surgery for fibrous dysplasia of the sinonasal tract in pediatric patients.

Fibrous dysplasia (FD) is a non-neoplastic, expansile lesion of unknown origin. In about one-fourth of cases this disorder affects the head and neck area, where the mandible and maxilla are the most frequently involved sites. Its localization to the ethmoid is a rare event. Since the disease slowly progresses, its management is delayed until significant clinical symptoms or non-well-tolerated aesthetic deformities are present. When required, surgery is the treatment of choice. Several external procedures have been used to manage the lesion, but recently, more conservative transnasal approaches have been proposed. We report the history of a 6-year-old boy with fibrous dysplasia of the ethmoid labyrinth that underwent successful transnasal endoscopic removal. Furthermore, an analysis of the literature is presented with particular emphasis on clinical picture, diagnosis, and treatment of this rare illness.

Child↗

The origins of osteochondromas and enchondromas. A histopathologic study.

Histopathologic sections of large pieces of tissue obtained from both surgical and postmortem specimens of osteochondromas and enchondromas were analyzed to elucidate the pathogenesis of these two lesions. The osteochondroma is derived from aberrant cartilaginous epiphyseal growth plate tissue, which proliferates autonomously and separates from the normal growth plate near its edge. As growth progresses, the aberrant tissue remains in a subperiosteal location, where it may either disappear through remodeling or proliferate as an early osteochondroma perpendicular to the orientation of the growth plate from which it was derived. The enchondroma also is derived from the actively proliferating cartilaginous tissue of growth plates. For unknown reasons certain groups of chondrocytes do not proceed to undergo hypertrophy and death. As a result, a column of uncalcified cartilage extends from the underside of the growth plate into the region in which all other cartilaginous tissue has been remodeled into primary bone. The bridge to the plate may either remain intact or become interrupted by normal bone. An isolated group of chondrocytes may (1) be walled off from normal tissue by lamellar bone; (2) undergo calcification and secondary osseous remodeling, either in part or in total; or (3) proliferate as an intraosseous chondroma (benign enchondroma). A similar but not identical process of enchondroma formation may occur in fibrous dysplasia of bone, systemic growth plate dysplasias similar to achondroplasia, and osteogenesis imperfecta.

Adolescent↗

Nonossifying fibroma of bone: a case report.

An atypical case of nonossifying fibroma involving the femoral neck of a 7-year-old white male is presented. The radiographic characteristics of the lesion were consistent with this diagnosis, although the location is rare. The differential diagnosis included fibrous dysplasia, unicameral bone cyst, chondroblastoma, and eosinophilic granuloma. A final diagnosis was obtained through tissue biopsy and histopathologic review.

Child↗

Differential diagnosis of pedal osseous neoplasms.

Topics discussed in this article include osteogenic sarcoma, osteoid osteoma, osteoblastoma, chondrosarcoma, chondromyxoid fibroma, chondroblastoma, chondroma, Ollier's disease, Maffucci's syndrome, osteochondroma, hereditary multiple exostoses, unicameral bone cyst, fibrous dysplasia, Albright's syndrome, nonossifying fibroma, giant cell tumor, Ewing's sarcoma, and metastasis. Numerous radiographs, CT scans, MR images, arteriograms, and photomicrographs supplement the text.

Bone Neoplasms↗

A probable monogenic form of polyostotic fibrous dysplasia.

A 37 year-old female patient with polyostotic fibrous dysplasia (PFD) is described. She presented the typical "café au lait" spots and severe bone involvement including a maxillary osteosarcoma. The father, four sibs, two nephews, two paternal aunts and two paternal first cousins were clinically examined, and seven of them also radiologically evaluated. "Café au lait" spots were found in the father, three sibs, one nephew, one aunt and one first cousin. Although no definite PFD bone lesions, mild radiological abnormalities were found in the father, three sibs and one nephew. These findings were interpreted as the variable expression of a pleiotropic gene. The present observation and three previous familial cases of this entity strongly suggest the existence of a form of PFD determined by an autosomal dominant gene.

Adolescent↗

Tamoxifen improved final height prediction in a girl with McCune-Albright syndrome: patient report and literature review.

McCune-Albright syndrome (MAS) is characterized by gonadotropin-independent precocious puberty, café-au-lait spots on the skin and polyostotic fibrous dysplasia of bones. Treatment of precocious puberty (PP) in MAS should be considered in patients with poor predicted adult height (PAH). Treatment of gonadotropin-independent PP in MAS with ketoconazole, cyproterone acetate or testolactone, an aromatase inhibitor, does not appear to be always effective in slowing bon. maturation. We report here a Thai girl with MAS who received tamoxifen, one of the selective estrogen receptor modulators, for the management of advanced puberty and rapid bone maturation. Her pubertal progression, vaginal bleeding, growth rate and PAH improved during treatment with tamoxifen despite persistently elevated serum estradiol levels and an enlarged ovarian cyst.

Body Height↗

Infantile McCune-Albright syndrome.

McCune-Albright syndrome is a rare disorder caused by an activating mutation of the alpha subunit of the Gs protein and is characterized by multiple hormonal abnormalities, fibrous dysplasia of bone, and cutaneous pigmentation. We report a 10-week-old infant with McCune-Albright syndrome. He presented with failure to thrive, cardiorespiratory distress, and pigmented macules on his face, trunk, and limbs. Following the clinical diagnosis of McCune-Albright syndrome, he was subsequently shown to have hypercortisolism and hyperthyroidism.

Cafe-au-Lait Spots↗

Titanium miniplate fixation for osteotomies in facial fibrous dysplasia--a histologic study of the screw/bone interface.

In four patients who had osteotomies of the jaws affected by fibrous dysplasia (FD), screws embedded in bone blocks were removed at a re-entry operation 20 months postoperatively. Morphometric measurement of the bone density and calculation of the bone contact percentage were performed. Both normal and dysplastic bone were found to have some direct bone contact with the titanium screws. Although the bone contact percentage was higher in the normal bone when compared with FD, statistics failed to show any significant difference (P < 0.05). The dysplastic bone healed well around the titanium screws without inflammatory reaction and direct dysplastic bone/screw contact was noted. Longer screws should be used in facial FD in order to compensate for the reduced bone contact percentage.

Adult↗

Fibrous dysplasia.

Three cases of monostotic fibrous dysplasia of the cranial bones are presented. Several theories of the etiology of this uncommon condition are put forward and the relationship between monostotic and polyostotic fibrous dysplasia, Albright's disease, and hyperparathyroidism is examined. The difficulty in diagnosis and the ways they may present to the ear, nose and throat surgeon are discussed. It is suggested that conservative surgery is the best mode of treatment for maxillary or temporal lesions. However, ethmoid lesions, particularly when the optic foramen or base of skull are eroded, may need a neuro-surgical approach. The use of radiotherapy in any case is deprecated.

Child↗

Fibrous tumors of bone.

Benign and malignant fibrous tumors of bone are some of the most common tumors encountered by the orthopedic surgeon. Fibrous dysplasia, fibrous cortical defect, non-ossifying fibroma, benign fibrous histiocytoma and osteofibrous dysplasia are benign tumors best treated conservatively or by intralesional/marginal excision. Desmoplastic fibromas are locally aggressive and require a wide margin for surgical cure. Malignant fibrous histiocytoma and fibrosarcoma of bone are malignant bone tumors and must be treated with wide/radical margins and for malignant fibrous histiocytoma adjunctive chemotherapy.

Bone Neoplasms↗

Posttraumatic fibro-osseous lesion of rib.

Eleven cases are described of an unusual, benign, fibro-osseous lesion of rib previously reported under a variety of designations, including painless fibro-osseous lesion resembling osteoid osteoma, symmetrical fibro-osseous dysplasia, focal Erdheim-Chester disease, and fibro-osseous pseudotumor. All patients were adults, most of whom were asymptomatic, the lesion discovered by bone scans done to rule out metastatic disease. A single rib was involved in eight patients and multiple ribs in three. A roentgenographic abnormality was apparent in only five patients. Histologically, all lesions showed a bland fibrous stroma in which resided an anastomosing network of bone trabeculae, having a zonal pattern of maturation from metaplastic woven to mature lamellar bone, with or without an associated xanthomatous component. Seven patients had a history of previous trauma, three with fractured ribs. Considering the relative infrequency of solitary rib lesions attributable to metastatic disease, it is proposed that in most cases there is no need for a diagnostic rib resection for these incidentally discovered, posttraumatic reparative lesions.

Aged↗

Fibrous dysplasia masquerading as extramedullary relapse after bone marrow transplantation for chronic myeloid leukemia.

A 19-year old girl suffered from relapse of chronic myeloid leukemia (CML) after bone marrow transplantation. The disease was controlled by interferon and imatinib mesylate, but was complicated by autoimmune hyperthyroidism. She presented with unilateral proptosis with no extraocular muscle or visual defect at 26 months follow-up. Systemic investigations showed no recurrence of leukemia or thyrotoxicosis. Magnetic resonance imaging revealed an extensive retro-orbital base of skull lesion. A trans-oral biopsy showed fibrous dysplasia and continuous observation was advised. The unusual sequence of events and the differential diagnoses for unilateral proptosis in post bone marrow transplantation (BMT) cases are discussed.

Adult↗

"Café-au-lait spots" caused by vitiligo in McCune-Albright syndrome.

Café-au-lait spots, fibrous dysplasia of bone, and endocrine gland hyperactivity are the principal features of McCune-Albright syndrome (MAS). Café-au-lait spots appear at, or soon after, birth. We illustrate "café-au-lait spots" acquired during middle age in a patient with MAS that are an illusion caused by vitiligo. This 64-year-old woman is the oldest patient reported with this disorder.

Cafe-au-Lait Spots↗