Hyperguides

Orthopaedics / Hand & wrist

Bone and Cartilage Tumors of the Hand and Wrist

O. Ugwonali, MD Vishal Sarwahi, MD Melvin P. Rosenwasser, MD
Article

Cartilage Tumors

Cartilaginous tumors are the second most common class of primary bone tumor,1 and the most common primary bone tumor of the upper extremity.1,2 Histologically, cartilaginous tumors contain some form of hyaline cartilage. Most lesions are found in otherwise healthy young adults. Presenting features include pain, swelling, or limited range of motion due to pathological fracture through the lesion.

Osteochondroma
Osteochondroma is the most common of all cartilaginous tumors and is the most common benign bone tumor.1-3 Frequently found in the ulna or radius, osteochondroma is formed from distorted cartilaginous areas that form subungal or metaphyseal bony mass by endochondral ossification. The hand is always involved in multiple hereditary exostoses, although it is rarely a site of involvement of solitary lesions.1,4 Osteochondroma is usually asymptomatic but may cause pain, angular deformity, mechanical block of joint motion, and inhibition of longitudinal growth. It appears in the first or second decade of life,1,3,5 but its growth ceases at skeletal maturity when the associated epiphysis closes. By radiograph and histology, osteochondroma has a normal cortex, cartilaginous cap over which a bursa commonly develops,5 and a connection of its cavity with the medullary canal. The lesion may be pedunculated or sessile. Deformity and loss of motion are the two usual indications for surgery because malignant transformation is rare in isolated lesions.

Enchondroma
Although enchondroma is the second most common benign cartilaginous neoplasm after osteochondroma,1,3 it is the most common primary bony tumor of the hand (90% of hand tumors).5,6 Enchondromas usually occur in the second and third decades of life but can occur at any age.1,3,5,7 It is painless and typically presents as a pathologic fracture and appears as a radiolucent mass with specks of calcification in the metaphyseal-diaphyseal area and a thin cortex. Enchondroma involves the phalanges (proximal) and metacarpals but rarely the carpal bones. If surgical treatment is elected, curettage with or without grafting through a dorsal or dorsolateral incision is effective.5,6 For unstable fractures, internal fixation is recommended. Ollier’s disease (multiple enchondromatosis) is associated with deformities of the axial skeleton and larger, unilateral lesions. The combination of multiple enchondromatosis and venous-lymphatic vascular malformations (angiomas) is termed Maffucci’s syndrome. Maffucci’s syndrome is associated with a higher rate of degeneration into chondrosarcoma than in Ollier’s disease. Malignant change of solitary lesions is rare, but a sudden increase in pain or size should raise suspicion of degeneration.

Chondromyxoid fibroma
Chondromyxoid fibroma is so-named because it contains variable amounts of chondroid, myxoid, and fibromatoid elements. This commonly eccentric and metaphyseal lesion usually affects the forearm and hand of adolescents and young adults. Because it is also sharply demarcated from normal bone, chondromyxoid fibroma is only differentiated from giant cell tumor of bone by histology. Treatment is by curettage and bone graft.8

Bone Tumors

Osteoid osteoma
Osteoid osteoma is found in the metaphyseal-diaphyseal area of short tubular bones or carpal bones, especially the scaphoid. Classical presentation is nocturnal bone pain that is responsive to aspirin with local swelling and tenderness. Diagnosis of osteoid osteoma is frequently delayed by an average of 15 months due to multiple differential diagnoses. Radiographically, osteoid osteoma appears as a mass smaller than 1 cm in diameter with an eccentric sclerotic border with a radiolucent nidus. A lytic lesion is often present on radiographs. Because of its small size, a bone scan or computed tomography (CT) scan may aid diagnosis. Curettage of the nidus through a cortical window is usually curative; however, symptoms may persist and local recurrence is possible if removal of the nidus is incomplete.8 The lesion also responds to prolonged nonsteroidal anti-inflammatory drugs with a mean course duration of 33 months.

Osteoblastoma
Osteoblastoma is the larger (greater than 2 cm) and more centrally located (medullary cavity of small tubular bones) relative of osteoid osteoma. Osteoblastoma is radiolucent and expansile with irregular areas of opacification. Because of its island of mature bone in osteoid tissue on histology, it must be differentiated from osteogenic sarcoma. Curettage and bone grafting is curative. Resection with more extensive bone grafting may be required in the rare instance of recurrence or local destruction of bone.8

Unicameral bone cyst
Unicameral bone cysts are found following a pathologic fracture and typically manifest as pain and swelling. They are commonly located eccentrically in the metaphyseal area of the distal radius. Curettage and bone grafting and intraosseous injection of methylprednisolone are the two treatment options.8

Aneurysmal bone cyst
Although benign and rare in the hand, aneurysmal bone cysts are rapidly growing and locally invasive tumors that have the potential to cause peripheral nerve impingement. An aneurysmal bone cyst is a cystic and hemorrhagic lesion that appears expansile and radiolucent on radiographs. If treatment with resection and bone grafting is inadequate, recurrence rates are high.8

Giant cell tumor of bone
Giant cell tumor of bone (GCT) often presents in the distal radius of young adults. Patients present with pain and local swelling, especially if associated with a pathologic fracture. GCT is radiolucent, expansile, and eccentrically located in the epiphyseal and subchondral region of a tubular bone. Many giant cells with nuclei similar to the proliferating mononuclear cells are seen histologically. Treatment options range from curettage with adjuvant therapy of liquid nitrogen, phenol, cementing to en bloc resection, or amputation for more aggressive tumors.8 GCT has a higher risk of local recurrence with intralesional treatment (80%) and a greater propensity to metastasize to lungs than elsewhere.

Distal end radius is the third most common site for GCT in the body with a 50% risk of recurrence with curettage, phenol, polymethylmethacrylate (PMMA), and burring. This risk is reduced to 25% when curettage is combined with cryotherapy. The most predictable method of local control is wide resection of distal radius. Reconstruction can be performed with intracalary bone grafting for arthrodesis, articular fibula autograft, distal radius articular allograft, and by creating a single bone forearm. Lesions with breech of the cortex are adequately treated with curettage and bone grafting, cryosurgery, and PMMA. Patients with pathological fracture are best treated with wide excision of the distal radius using intercalary fibula reconstruction.

Malignant Bone and Cartilage Tumors

Chondrosarcoma
Chondrosarcoma is the most common primary malignant bone tumor of the hand occurring after the sixth decade. Proximal phalanx and metacarpals are most commonly involved. Survival is related to tumor grade and site. Tumor locations mimic those of enchondromas, and the usual presenting symptom is pain and swelling. Radiographs reveal cortical destruction of the margins to a greater extent than with enchondromas. Further differentiation is made histologically. Resection with wide margins, frequently accompanied with ray resection, is the preferred treatment. If treated appropriately, prognosis is good.8 Risk of metastases is approximately 10% and occurs after local recurrence. CT scan of the chest is needed to rule out metastases.

Osteogenic sarcoma
Osteogenic sarcoma of the hand is a rare tumor and occurs in the first and second decades of life. The presentation is a progressively painful, swollen mass at the metacarpal and proximal phalanges. A sclerotic and expansile destructive bone lesion is seen on radiographs. Treatment consists of adjuvant or neoadjuvant chemotherapy, en bloc excision, or ray amputation. There is no significant difference in survival between neoadjuvant and adjuvant chemotherapy. Surgical resection, however, may obtain better margins after neoadjuvant chemotherapy and reduce the risk of local recurrences. Osteogenic sarcoma may be classified as high or low grade depending on the cellular activity at the time of diagnosis.9-12

Ewing’s sarcoma
Ewing’s sarcoma comprises 6% of all malignant bone tumors and approximately 20% of all Ewing’s tumors are found in the upper extremity.13 This rare skeletal malignancy in the hand usually affects the metacarpals and phalanges in the first and second decade of life. Typical presentation is that of an inflammatory process with pain, swelling, erythema, and possibly fever with an elevated sedimentation rate. These findings may be mistaken for an infectious process.9,14-16 Ewing’s sarcoma appears as a destructive lytic lesion with a soft tissue component. Incisional biopsy followed by wide en bloc resection or amputation is treatment of choice. Effective modalities also include radiotherapy and adjuvant chemotherapy. Its prognosis in the hand is better than other locations, possibly due to earlier detection.

Metastases
Osseous metastases far outnumber primary bone tumors and develop in 12% to 70% of all patients with cancer. Twenty percent of metastases occur in the upper extremities. Half of all these tumors occur in the humerus.13 Metastasis below the elbow is rare, and the hand is the least frequent site. Breast, colon, kidney, and lung are the most common metastases to the hand. Pain, swelling, and erythema are common symptoms often mistaken for an infection. Palliative digit or ray amputation or radiotherapy is instituted after diagnostic biopsy.

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