Orthopedic Basic Science MCQs (Set 4): Biomechanics, Bone Physiology & Biomaterials | AAOS, ABOS Review

An enchondroma is the most common primary tumor of the long bones of the hand. The lesion is usually asymptomatic and often is detected when there is a pathologic fracture. Shimizu K, Kotoura Y, Nishijima N, Nakamura T: Enchondroma of the distal phalanx of the hand. J Bone Joint Surg Am 1997;79:898-900.

Bisphosphonates are a class of drugs that act to inhibit osteoclast resorption of bone. It has been shown that patients with multiple myeloma who are treated with bisphosphonates have fewer pathologic fractures than patients who are not treated with bisphosphonates. Vitamin D and calcium are considered appropriate for patients who are at risk for the development of osteoporosis, as is estrogen in selected women. Chelating agents and progesterones have no use in the treatment of patients with multiple myeloma or osteoporosis. Berenson JR: Bisphosphonates in multiple myeloma. Cancer 1997;15:1661-1667.

The lesion is a chondroblastoma. The plain radiographs show a well-defined radiolucent lesion in the distal femoral epiphysis of a skeletally immature patient. The margins are well defined, suggesting a benign growth. The epiphysis is an unusual location for bone tumors, except for chondroblastomas. Of all chondroblastomas, 95% are located within the epiphysis. The MRI scans show a punctate appearance that is commonly seen in cartilage lesions. The biopsy specimen shows a chondroid lesion with polygonal chondrocytes. These findings are consistent with a chondroblastoma. The natural history of chondroblastomas is for continued growth and bone destruction if left untreated. Treatment should consist of curettage, with or without the use of physical or chemical adjuvants, and bone grafting.

The most likely diagnosis is chronic multifocal osteomyelitis. This is a culture-negative polyostotic disease that is most commonly found in young people. The treatment of choice is anti-inflammatory drugs. The pathology does not suggest eosinophilic granuloma. Antiviral therapy, broad-spectrum antibiotics, and surgical resection are not indicated for this disease.

Findings in patients with McCune-Albright syndrome include polyostotic fibrous dysplasia, multiple cafe-au-lait spots, and precocious puberty. The bone changes in NF-1 resemble nonossifying fibromas, not fibrous dysplasia. NF-2 has little bony change with typical ocular abnormalities. Paget's disease occurs in older individuals and does not present with cafe-au-lait spots. Ollier's disease (multiple enchondromatosis) may show bone changes but not the other findings. Albright F, Butler AM, Hampton AO, et al: Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precocious puberty in females. N Engl J Med 1937;216:727-746. Danon M, Robboy SJ, Kim S, Scully R, Crawford JD: Cushing syndrome, sexual precocity, and polyostotic fibrous dysplasia (Albright syndrome) in infancy. J Pediatr 1975;87:917-921.

In a young person with solitary osteochondroma, the best surgical indication is symptoms that limit activity. A growth deformity is unlikely to occur at this age. Malignant degeneration is exceptionally rare and noted most commonly in adults. Growth is expected until skeletal maturity. Mirra JM: Bone Tumors: Clinical, Radiologic, and Pathologic Correlations. Philadelphia, PA, Lea and Febiger, 1989, pp 1626-1659.

Conventional osteosarcoma most frequently occurs in the second decade, followed by the third decade. Approximately 70% to 75% of patients with osteosarcoma are between the ages of 10 and 25 years. Secondary osteosarcoma (arising in Paget's disease or radiation-induced) is seen in older adults. Simon MA, Springfield DS, et al: Osteogenic Sarcoma: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 266. Mirra JM: Bone Tumors: Clinical, Radiologic, and Pathologic Correlations. Philadelphia, PA, Lea and Febiger, 1989.

A destructive mixed lytic and blastic metaphyseal lesion with a large soft-tissue mass in an adolescent is most likely an osteosarcoma until proven otherwise. The epicenter of the tumor is on the surface of the bone, most likely involves the periosteum, and is more likely to be chondroblastic in nature. Parosteal osteosarcoma is a low-grade tumor, much more radiodense, usually smaller, and found in the posterior distal femur of middle-aged patients. Chondrosarcomas are distinctly rare in childhood.

Patients with neurofibromatosis are at risk for development of soft-tissue sarcomas (most commonly malignant peripheral nerve sheath tumors). Clinical indications of development of a neurofibrosarcoma include a rapidly enlarging soft-tissue mass; therefore, this patient should be considered to have a neurofibrosarcoma until proven otherwise. MRI is superior to CT in characterizing the anatomic location of soft-tissue masses and the signal characteristics of the lesion. Areas of necrosis within the tumor may be apparent on MRI that cannot be appreciated on CT, suggesting a malignant tumor. Local imaging studies of suspected malignant tumors should be performed prior to needle or open biopsy so that the biopsy site can be excised at the time of definitive resection. Additionally, postbiopsy changes may lead to MRI artifacts that alter the interpretation of the MRI. Demas BE, Heelan RT, Lane J, Marcove R, Hajdu S, Brennan MF: Soft-tissue sarcomas of the extremities: Comparison of MR and CT in determining the extent of disease. Am J Roentgenol 1988;150:615-620.

The lesion is an eccentric lytic bone lesion within the epiphyseal-metaphyseal end of the proximal tibia. There is geographic destruction with a "fading border" extending to the articular cartilage. There is no matrix formation or periosteal reaction. The MRI scan shows cortical destruction with extension into the soft tissue. According to the Enneking staging system, benign lesions are stage 1, 2, or 3; malignant lesions are stage I, II, or III. Benign stage 1 lesions are latent; stage 2 are active; and stage 3 are benign aggressive. The histology shows a benign giant cell tumor. Given the cortical breakthrough shown on the MRI scan, the lesion should be classified as stage 3. Enneking WF: Clinical musculoskeletal pathology, in Enneking WF (ed): Appendix A. Gainesville, FL, Storter Publishing, 1986, pp 451-466.

Increased levels of calcium are known to cause anorexia, nausea, vomiting, dehydration, muscle weakness, polyuria, and polydipsia. Treatment may include hydration, saline diuresis, and bisphosphonates.

The main consequence of tumor invading the bone is activation of both osteoblasts and osteoclasts. However, the osteoclastic effect predominates in the majority of tumors early after the invasion of bone by tumor cells, causing resorption of bone. Cramer SF, Fried L, Carter KJ: The cellular basis of metastatic bone disease in patients with lung cancer. Cancer 1981;48:2649-2660.

Secondary chondrosarcomas are most common in patients with multiple hereditary exostosis. Dedifferentiated chondrosarcoma is less common and refers to bone lesions in which a high-grade spindle cell sarcoma component is located immediately adjacent to a low-grade cartilage neoplasm. Mesenchymal chondrosarcoma, clear cell chondrosarcoma, and periosteal osteosarcoma are no more common in patients with multiple hereditary exostosis than in the general population. Mirra JM: Bone Tumors: Clinical, Radiologic, and Pathologic Correlations. Philadelphia, PA, Lea and Febiger, 1989, pp 1660-1669.

The presence of a painless soft-tissue mass that is greater than 5 cm and deep to the fascia should be considered a soft-tissue sarcoma until proven otherwise. The diagnosis of a hematoma should be made with great caution because the absence of a history of trauma, pain, or presence of ecchymosis makes it unlikely. A diagnosis of pyomyositis is unlikely because of the absence of warmth, erythema, or adenopathy. The MRI scans are not consistent with lipoma or hemangioma. The MRI signal characteristics of a lipoma should be the same as subcutaneous fat on all sequences. Soft-tissue hemangiomas are not well defined and have an infiltrative appearance on MRI scans, as does pyomyositis. Sim FH, Frassica FJ, Frassica DA: Soft-tissue tumors: Diagnosis, evaluation and management. J Am Acad Orthop Surg 1994;2:202-211.

Epithelioid sarcoma is a rare soft-tissue sarcoma that most commonly arises in the hand or upper extremity, and it is frequently misdiagnosed as an infection or granuloma. It tends to have a higher incidence of lymph node metastasis than other soft-tissue sarcomas. The mainstay of treatment is wide surgical excision, even if amputation is necessary. Gupta TD, Chaudhuri P (eds): Tumors of the Soft Tissues, ed 2. Stamford, CT, Appleton and Lange, 1998, p 475.

Cytogenetic abnormalities have been well characterized in a number of tumors. Translocation t (2, 13), (x, 18), (12, 16), and (12, 22) have been characterized in rhabdomyosarcoma, synovial cell sarcoma, myxoid liposarcoma, and clear cell sarcoma, respectively. Translocation t(11:22) can be identified in 95% of patients with Ewing's sarcoma. This was first described by Turc-Carel and associates in 1984. Enzinger FM, Weiss SW: Soft Tissue Tumors, ed 3. St Louis, MO, Mosby Year Book, 1995, pp 105-118. Turc-Carel C, Philip I, Berger MP, Philip T, Lenoir GM: Chromosome study of Ewing's sarcoma (ES) cell lines: Consistency of a reciprocal translocation t(11;22) (q24;q12). Cancer Genet Cytogenet 1984;12:1-19.

The patient has a pathologic fracture of the right distal femur; therefore, given the patient's age, the most likely diagnosis is metastatic carcinoma. Staging studies should be obtained prior to surgical treatment. Immediate intramedullary fixation is contraindicated before a diagnosis is made by biopsy. Surgical stabilization should be performed prior to radiation therapy.

The bright signal on the T2-weighted MRI scan suggests fluid. The multiloculated appearance in proximity to the proximal tibiofibular joint suggests that the most likely diagnosis is a ganglion. They typically increase and decrease in size and can be diagnosed by the classic gelatinous fluid obtained through needle aspiration. Bianchi S, Abdelwahab IF, Kenan S, Zwass A, Ricci G, Palomba G: Intramuscular ganglia arising from the superior tibiofibular joint: CT and MR evaluation. Skeletal Radiol 1995;24:253-256.

The five most common primary carcinomas that metastasize to bone are breast, prostate, lung, renal, and thyroid in decreasing order of incidence. Frassica FJ, Gitelis S, Sim FH: Metastatic bone disease: General principles, pathophysiology, evaluation, and biopsy. Instr Course Lect 1992;41:293-300.

Pain secondary to an osteochondroma is usually from soft-tissue irritation and bursal formation. This is particularly common for proximal medial tibia osteochondromas that irritate the pes anserine tendons. Malignant degeneration into a chondrosarcoma rarely occurs, is usually associated with multiple hereditary exostoses, and usually occurs after skeletal maturity. Borges AM, Huvos AG, Smith J: Bursa formation and synovial chondrometaplasia associated with osteochondromas. Am J Clin Pathol 1981;75:648-653.

The histology shows eosinophils with a background of larger cells (Langerhans' cells). This is consistent with eosinophilic granuloma. Localized sites are best treated with curettage, steroid injection, or observation. Chemotherapy is used only if there is systemic involvement. Mirra JM: Eosinophilic granuloma, in Bone Tumors: Clinical, Radiologic, and Pathologic Correlations. London, England, Lea and Febiger, 1989, pp 1023-1060. Sessa S, Sommelet D, Lascombes P, Prevot J: Treatment of Langerhans-cell histiocytosis in children: Experience at the Children's Hospital of Nancy. J Bone Joint Surg Am 1994;76:1513-1525.

The 5-year overall survival rate for deep, high-grade soft-tissue sarcomas is around 50%. The overall survival and disease-free survival rates chiefly depend on the tumor stage, but for all stages combined, most cancer treatment centers report a 5-year overall survival rate of around 70% and a disease-free survival rate of 65%. Fleming ID, et al: Manual for Staging of Cancer/American Joint Committee on Cancer, ed 5. Philadelphia, PA, Lippincott Raven, 1997, pp 149-156.

The patient has a presumed metastatic bone tumor. The approach to evaluating a patient with a bone tumor of unknown primary origin is to obtain laboratory studies that include a CBC, an erythrocyte sedimentation rate, a serum protein electrophoresis, a calcium level, a urinalysis, and a prostate-specific antigen. In addition, a bone scan, a radiograph of the chest, and CT scans of the chest and abdomen should be obtained. These evaluations can identify the primary site in 85% of patients. GI studies rarely are of diagnostic value. Prophylactic stabilization is contraindicated until a diagnosis is confirmed by histology. Frassica FJ, Frassica DA, McCarthy EF, Riley LH III: Metastatic bone disease: Evaluation, clinicopathologic features, biopsy, fracture risk, nonsurgical treatment, and supportive management. Instr Course Lect 2000;49:453-459.

Osteochondroma typically occurs as a bony projection or a sessile growth on the bone, and there can be flaring of the metaphysis. The radiograph shows continuity from the adjacent cancellous bone into the lesion itself. There is no soft-tissue mass or bone destruction to suggest osteosarcoma or Ewing's sarcoma. Periosteal chondroma has a scalloped out radiographic appearance. Chondroblastoma typically is an epiphyseal-based lesion.

To date, only the percent of necrosis after induction chemotherapy in high-grade osteosarcomas seems to be of prognostic value. The value in soft-tissue sarcoma and rhabdomyosarcoma is being evaluated but has not been substantiated. Chondrosarcomas and parosteal osteosarcomas are not treated with chemotherapy. Rosen G, Marcove RC, Caparros B, Nirenberg A, Kosloff C, Huvos AG: Primary osteogenic sarcoma: The rationale for pre-operative chemotherapy and delayed surgery. Cancer 1979,43:2163-2177. Davis AM, Bell RS, Goodwin PJ: Prognostic factors in osteosarcoma: A critical review. J Clin Oncol 1994;12:423-431.