Kenneth A. Jaffe

Orthopaedic implant-related sarcoma: a study of twelve cases.

Sarcoma developing in association with a metallic orthopaedic prosthesis or hardware is an uncommon, but well recognized complication. We review 12 cases of sarcomas arising in bone or soft tissue at the site of orthopaedic hardware or a prosthetic joint. Nine patients were male, and three were female. Their ages ranged from 18 to 85 (mean 55) years at the time of diagnosis of the malignancy. Five patients had undergone hip arthroplasty for degenerative joint disease, four had been treated with intramedullary nail placement for fracture, two had staples placed for fixation of osteotomy, and one had hardware placed for fracture fixation followed years later by a hip arthroplasty. The time interval between the placement of hardware and diagnosis of sarcoma was known in 11 cases and ranged from 2.5 to 33 (mean 11) years. The patients presented with pain, swelling, or loosening of hardware and were found to have a destructive bone or soft tissue mass on radiography. Two sarcomas were located primarily in the soft tissue and 10 in bone. Seven patients developed osteosarcoma, four malignant fibrous histiocytoma, and one a malignant peripheral nerve sheath tumor. All sarcomas were high grade. Three patients had metastatic disease at the time of diagnosis. Follow-up was available on eight patients: five patients died of disease 2 months to 8 years (mean 26 months) after diagnosis; two patients died without evidence of disease 7 and 30 months after diagnosis; and one patient is alive and free of disease 8 years after diagnosis. Sarcomas that occur adjacent to orthopaedic prostheses or hardware are of varied types, but are usually osteosarcoma or malignant fibrous histiocytoma. They behave aggressively and frequently metastasize. Clinically, they should be distinguished from non-neoplastic reactions associated with implants, such as infection and a reaction to prosthetic wear debris.

Osteosarcoma in a patient with McCune-Albright syndrome and Mazabraud's syndrome.

Abstract Sarcomas infrequently develop in osseous sites of fibrous dysplasia. We report a patient with Mazabraud’s syndrome (polyostotic fibrous dysplasia and soft tissue myxomas) complicated by the development of osteogenic sarcoma in a bone affected by fibrous dysplasia. This is the third case of osteosarcoma within the small population of reported patients with Mazabraud’s syndrome. There may be an increased incidence of malignant transformation in these individuals’ dysplastic bones above that associated with patients suffering from fibrous dysplasia alone.

Hand mass in a 15-year-old boy.

A 15-year-old boy presented with an enlarging mass of his left thenar eminence. His mother first noted the mass approximately 1 month earlier. The patient denied pain or other symptoms related to the mass. He denied any trauma, fever, chills, or weight loss. His medical history was unremarkable. Physical examination revealed a firm, nontender mass in the left thenar eminence measuring approximately 4 x 5 cm (Fig 1). There was some mild erythema of the overlying skin. He had full flexion and extension of the thumb interphalangeal joint and full extension of the thumb metacarpophalangeal joint. Thumb metacarpophalangeal flexion was somewhat limited by the mass. Wrist, elbow, and shoulder ROM were normal. Static two-point sensation in the thumb and index finger were normal at 4 mm. Brisk capillary refill was present in all digits. There was no adenopathy at the elbow or shoulder. Laboratory values included normal serum chemistry, hemoglobin of 14 g/dL, leukocyte count of 7900/ L, and an erythrocyte sedimentation rate (ESR) of 9 mm. Conventional radiographs and MRI scans of the left hand were obtained (Figs 2, 3). Based on the history, physical findings, laboratory studies, and imaging studies, what is the differential diagnosis? Orthopaedic • Radiology • Pathology Conference CME ARTICLE

Oncologic uses of the retrograde femoral nail

Destructive lesions about the distal femur represent difficult treatment situations. Patients who present with impending or pathologic fractures require a method of fixation that provides adequate stabilization to allow for early, pain-free ambulation. Other considerations include restoration of functional range of motion, low perioperative morbidity and mortality risk, and minimal soft tissue dissection. Eleven patients with 12 pathologic or impending fractures of the distal femur who were treated with retrograde intramedullary nailing with and without polymethylmethacrylate augmentation were retrospectively reviewed. Followup averaged 17 months. Postoperative range of motion averaged 94° flexion, 90% of patients reported no or modest pain, 81% were functioning with no restrictions or recreational restrictions only, and all patients were enthusiastic or satisfied with their surgical outcome. There were no perioperative mortalities, but one patient had 2 cm shortening secondary to broken distal locking screws and asymptomatic nail protrusion into the knee. Using the system for functional evaluation of reconstructive treatment of tumors of the musculoskeletal system, the average score for each patient was 25 (range, 18–30) or 83% (60%–100%) of the maximum score. The retrograde nail was an easy and successful method of fixation in this clinical setting.

Extraskeletal Osteosarcoma: An Unusual Presentation as Tarsal Tunnel Syndrome

Tarsal tunnel syndrome is a compressive neuropathy caused by intrinsic or extrinsic pressure on the posterior tibial nerve or one of its terminal branches. A mass in association with tarsal tunnel syndrome is most likely a benign tumor or tumor-like condition, although a more malignant tumor must be in the differential diagnosis. We report an unusual case of an extraskeletal osteosarcoma causing tarsal tunnel syndrome.

Florid reactive periostitis ossificans of the humerus: Case report and differential diagnosis of periosteal lesions of long bones

A case of florid reactive periostitis ossificans (RPO) arising in a long bone is presented. This is a rare bone proliferation with a pronounced periosteal reaction. Less than 100 cases have been described in the literature with far fewer outside the bones of the hand, feet, fingers, and toes. Although the etiology is unknown, a relationship to preceding trauma is suggested. The imaging and histologic features show an overlap with other bone lesions including bizarre parosteal osteochondromatous proliferation, subungual exostosis, and malignant surface tumors of bone and cartilage which include, periosteal and parosteal osteosarcoma. It is important to recognize the clinical presentation and diagnostic features of RPO as a benign entity so that it is not mistaken for a more aggressive neoplasm. We present a case of a right distal humeral lesion that on histopathological review revealed florid RPO. This diagnosis was not suspected on imaging studies, but was made on open biopsy of the mass. The patient remains disease free, years postoperatively. In addition to presenting this unique case report, we review the pertinent literature, and offer a differential diagnosis and treatment strategy for its management.