Multicentric giant cell tumor of bone

Giant cell tumor of bone accounts for 4% to 5% of primary bone tumors in the United States. Multicentric giant cell tumors occur in < 1% of all patients with giant cell tumors, and only 43 patients with multicentric giant cell tumor have been reported on in the literature. This series presents 3 additional cases of multicentric giant cell tumor, includes updated data for 2 patients previously reported on in the literature, and reviews 24 cases previously reported on in detail in the literature. The mechanism by which giant cell tumor involves multiple locations is not known. Multicentric giant cell tumor, in contrast to unifocal giant cell tumor, has a tendency to involve the hands, feet, and metaphysis/diaphysis of long bones and to occur in a slightly younger population. In 15 of the 29 patients reviewed, a second lesion did not develop for > 2 years after their initial presentation. Eighteen of those 29 patients had > 2 sites of tumor involvement, 1 of whom had 11 lesions. Two of the 5 patients in the authors' series presented with a spectrum of disease activity, with latent, active, and aggressive lesions present throughout the observation period.     

Postradiation sarcoma of bone: review of 78 Mayo Clinic cases

Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site.     

Primary chondrosarcoma of long bones and limb girdles

BACKGROUND: Chondrosarcomas are common solid malignant tumors of bone, second in incidence only to osteosarcomas. The biologic evolution of chondrosarcomas is slow, requiring long follow-up intervals for meaningful survival analysis. METHODS: This study describes the clinicopathologic profiles of 344 patients, 194 male and 150 female (M:F, 1.3:1.0), with primary chondrosarcoma of long bones and limb girdles seen at 1 institution over a period of 80 years. RESULTS: The average age at presentation was 46 years (range, 5-82 years). The pelvis was the most common location (1.7% of all patients). Local pain was the most frequently reported initial symptom (81.4%). Survival analysis was limited to 233 patients whose primary treatment was given at the Mayo Clinic. All 233 patients had potential follow-up of at least 5 years. The overall 5-year survival rate was 77% (the expected rate was 96%). Local recurrence developed in 19.7% of patients and metastatic lesions in 13.7%. The recurrence rate was higher for tumors of the shoulder and pelvis than for tumors of long bones. Radiographically, chondrosarcomas had a characteristic appearance, including a combination of bone expansion and cortical thickening. Entering the tumor at surgery increased the risk of local recurrence. Histologic tumor grade was an important predictor of local recurrence and metastasis. CONCLUSIONS: With adequate initial surgical intervention, chondrosarcoma is primarily a local disease with a low metastatic rate.     

Tumour development, histology and grade of breast cancers: prognosis and progression

A case of giant cell tumor localized in the proximal end of the right tibia, initially treated by simple curettage is described. After 11 years there was local recurrence, confirmed anatomopathologically, observed during wide resection and reconstruction by arthroplasty plus homoplastic grafting. After a few months there were metastatic lesions in the left parietooccipital and right retroorbitary area. Biopsy of the parietooccipital lesion showed giant cells in a sarcomatous stroma. The patient died after several attempts at chemotherapy. Even if only sporadic cases have been described of late recurrences and of extrapulmonary metastases for giant cell tumors, the case presented here is exceptionally rare. The factors that influence recurrence of these tumors and their ability to metastasize is discussed.     

Inverted papillomas that invade the orbit

We describe 10 patients with inverted papillomas (IPs), uncommon endophytic epithelial tumors that arose in the nose, paranasal sinuses, and lacrimal sac, that invaded the orbit; review the world literature on IP; and discuss treatment options. Patients ranged in age from 49 to 72 years (mean age, 62 years; median age, 66 years). Six patients were men and four were women. Presenting symptoms and signs included a medial canthal mass (n = 5), epiphora/lacrimal drainage obstruction (n = 3), inability to wear eyeglasses (n = 2), diplopia (n = 1), and painful dentures (n = 1). Histopathologic examination was performed in each patient and revealed all patients to have areas with malignant transformation: six patients with IPs that contained areas of squamous cell carcinoma and four with areas of transitional cell carcinoma in the tumor within the orbit. Eight of the patients with IPs had a recurrence after initial resection. In two patients, the IPs were completely excised and did not recur; yet, follow-up was short. Eight patients required orbital exenteration for local control of disease. Nevertheless, local spread of tumor through bone to brain occurred in three patients with orbital involvement. Indeed, IPs that invade the orbit have a high incidence of malignancy and are locally aggressive tumors. Treatment is difficult, and the recurrence rate is high. Early, wide excision of this aggressive tumor provides the best chance of cure.     

Desmoplastic fibroma of the temporal bone

BACKGROUND: Desmoplastic fibroma is a benign, locally aggressive, intraosseous neoplasm with a propensity for local recurrence. Desmoplastic fibroma most commonly originates within the mandible (70% of cases), and long bones with rare lesions reported in the maxillary, frontal, and parietal bones. We report two patients with desmoplastic fibroma arising within the temporal bone. STUDY DESIGN: Case report and literature review. PATIENTS: Two patients with desmoplastic fibroma with intracranial and extra cranial growth patterns are presented. Both were young females with aural fullness and decreased hearing. INTERVENTIONS: Diagnostic and therapeutic. RESULTS: Both tumors were surgically extirpated and the patients have remained disease free for 18-48 months. Neither tumor expressed estrogen or progesterone receptors. CONCLUSION: Desmoplastic fibroma is a highly invasive local destructive lesion which is best treated by nondestructive surgical intervention. Immunohistochemical analysis may demonstrate hormonal receptors, in which case Tamoxifen may reduce recurrence.     

Malignant lymphoma involving the patella

The majority of skeletal lesions affecting the patella are benign and include entities such as chondroblastoma, giant cell tumor, osteomyelitis, and gout. Malignant processes involving the patella are distinctly unusual. Isolated occurrences of plasmacytoma, osteosarcoma, hemangiosarcoma, and metastatic disease have been reported. Malignant lymphoma involving the patella is extremely uncommon, although lymphomatous infiltration of the skeletal system is not a rare event, especially with the histiocytic lymphoma. The most frequent radiologic manifestations of skeletal lymphoma include osteolytic lesions with ill-defined margins involving the metaphysis of the long bones of the lower extremities. Involvement of the short tubular and flat bones, as well as the axial skeleton, occurs less commonly. The prognosis for lymphoma involving the skeleton is poor.     

Different left ventricular relaxation parameters in isolated working rat and guinea pig hearts. Influence of preload, afterload, temperature, and isoprenaline

Six primary lung tumors with numerous multinucleated osteoclast-like giant cells (OLGCs) and no osteogenic component were evaluated histologically and immunohistochemically to examine pulmonary lesions inciting an OLGC response. The patients comprised four women and two men ranging in age from 61 to 80 years (average age, 69 years). The tumors consisted of one adenocarcinoma, two sarcomatoid carcinomas, and three giant cell variants of malignant fibrous histiocytoma. One tumor was endobronchial in location, while five were situated peripherally. Tumor diameter spanned from 1 to 6.5 cm (average, 2.7 cm). In addition to the giant cells, common characteristics included the malignant nature of the neoplasms and, in five of six cases, histologically malignant mesenchyme. This array of cases exemplifies the variability of lung lesions which may elicit an OLGC inflammatory response resulting in areas resembling the giant cell variant of malignant fibrous histiocytoma. The results of this study suggest that OLGCs occur preferentially in malignant rather than benign nonosteogenic lung tumors and that sarcomatoid regions of malignant tumors are more likely to be infiltrated by OLGCs than epithelial regions.     

Positron emission tomography (PET) methodology for small animals and its application in radiopharmaceutical preclinical investigation

Among the more uncommon tumors that may sometimes be encountered in the laryngeal region is the recently described giant cell tumor of the larynx. This lesion is a true neoplasm, presumably of the fibrohistiocytic series. Histologically, it closely resembles the more familiar true giant cell tumor of long bone. The laryngeal giant cell tumors appear, to date, to be nonmetastasizing lesions; it is possible that they may recur locally if incompletely excised (although this remains to be demonstrated). In view of the rarity of these tumors, a tentative diagnosis of such a neoplasm should always prompt consideration of other (more frequently encountered) differential diagnostic possibilities, including cytologically malignant giant cell-rich tumors such as malignant fibrous histiocytoma and sarcomatoid carcinoma.     

Bone tumors of the coracoid process of the scapula

Bone tumors of the coracoid process of the scapula are rare, and diagnosis and treatment often are delayed. The records of 18 patients with bone tumors of the coracoid process were reviewed. Histologic types included eight cases of ordinary chondrosarcoma, three cases of dedifferentiated chondrosarcoma, two cases of osteoid osteoma, and one case each of osteosarcoma, plasmacytoma, lymphoma, giant cell tumor, and aneurysmal bone cyst. All 18 patients had shoulder pain, and eight of them had been treated with steroid injections for nonneoplastic conditions. Radiologically, chondrosarcoma did not always show clear cortical destruction, and one giant cell tumor had features mimicking those of chondrosarcoma. Five patients (three with dedifferentiated chondrosarcoma, one with chondrosarcoma, one with plasmacytoma) died of disease. The coracoid process was the site with a markedly high proportion of chondrosarcomas. Bone tumors of the coracoid process may be difficult to detect on plain radiographs. In the patient with persistent shoulder pain unresponsive to the selected treatment, additional imaging studies should be considered to eliminate the possibility of a bone lesion.