Giant cell tumor.
Subject: Tumors
Author: Kulenovic, Elvedin
Pub Date: 12/01/2008
Publication: Name: Applied Radiology Publisher: Anderson Publishing Ltd. Audience: Academic Format: Magazine/Journal Subject: Health Copyright: COPYRIGHT 2008 Anderson Publishing Ltd. ISSN: 0160-9963
Issue: Date: Dec, 2008 Source Volume: 37 Source Issue: 12
Topic: Canadian Subject Form: Tumours
Accession Number: 209239267
Full Text: CASE SUMMARY

A 28-year-old white woman fell from a step and landed on an outstretched hand. She presented with persistent pain and swelling over her left ring finger metacarpophalangeal joint.

IMAGING FINDINGS

Radiography of the left hand revealed a lytic expansile epiphyseal lesion in the head of the ring finger metacarpal bone. The lesion caused cortical destruction and tiny septations with poorly visualized matrix. There was associated widening of the third and forth meta carpal spaces, which suggested a soft tissue component (Figure 1).

Magnetic resonance imaging (MRI) showed a subarticular expansile lesion in the head of the ring finger metacarpal bone. On T1-weighted imaging, the lesion exhibited a inhomogeneous low signal intensity lesion. On short tau inversion recovery imaging, it had inhomogeneous high signal intensity. There was cortical destruction with soft tissue invasion that almost touched the middle finger metacarpal bone (Figure 2, A and B). The middle finger meta carpal bone showed the normal contour and signal. The extensor tendons were effaced and exhibited the normal signal, surrounded with the abnormal bright signal that involved subcutaneous fat. The overlying skin was normal. On the palmar side there was a tiny normal soft tissue plane between the flexor tendons and the expansile process. The flexor tendons were normal. The subcutaneous fat on this image appeared normal. The mass on the ulnar side touched the little finger metacarpal bone, which had normal contour and signal (Figure 2C). A whole-body bone scan and spot films of the hands showed a single lesion in the skeleton located in the left hand (Figure 3).

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HISTOPATHOLOGY

Microscopic examination showed a tumor composed of numerous multi nucleated giant cells admixed with the mononucleated stromal cells. The mono nucleated stromal cells were oval-shaped and lacked atypia, and their nuclei displayed features similar to those present within the giant cells (Figure 4).

DIAGNOSIS

Giant cell tumor of the left ring finger metacarpal bone

DISCUSSION

Giant cell tumors comprise 4% to 5% of primary bone tumors and 18.2% of benign bone tumors. They usually occur in the 20- to 40-year-old age group (60% to 70% of tumors), although they may appear in older patients and in younger patients who have not yet ceased growing. The frequency of giant cell tumors is greater in women than in men. Giant cell tumors predominate in the long tubular bones (75% to 90% of cases). The distal portions of the femur, radius, and the proximal portion of the tibia are the most characteristic sites of involvement (approximately 50% of cases). Approximately 5% of giant cell tumors localize in the small bones of the hands or feet; more commonly, they are found in the hands. Although usually benign, 5% to 10% of these tumors are malignant. As in this case, 10% of patients have a pathological fracture. The predominant clinical features are nonspecific and include local swelling, warmth, and radiating pain. (1) The symptoms may go on for months before becoming severe enough to demand clinical attention. Acute onset of pain is frequently associated with fractures, bringing the tumor to clinical attention. (2)

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The pathology involves a predominance of giant cells, although this is not specific for the diagnosis. They are among mononuclear stromal cells, and fusion of the mononuclear cells leads to formation of the giant cells. They both have similar-appearing nuclei, a feature that aids in excluding other diagnoses. (2) There is eosinophilic, granular cytoplasm surrounding the regularly outlined nuclei. Rarely are mitotic figures found within the giant cells. There may be bands of fibrous tissue that have no giant cells present or osteoid foci within a fracture callus. Up to 40% of giant cell tumors show evidence of vascular invasion, a feature that is present in our case. (3)

The first step in evaluation involves plain radiographs, on which there is a characteristic appearance. An eccentric, osteolytic lesion that is subarticular in location is often seen. Giant cell tumors commonly appear radiolucent and do not show periosteal reaction. (1) If the tumor is complicated by a fracture, there may be reactive bone formation. Some tumors may show areas of infarct or intravascular tumor emboli. Septa sometimes are seen and are caused by uneven tumor growth. Variation in appearance warrants more in-depth examination. A computed tomography (CT) scan shows findings similar to those seen on plain films and often does not reveal additional information. MRI has a high diagnostic accuracy, especially if correlated with findings from X-ray and/or CT scanning. (4) MR images can display the intraosseous, intra-articular, and soft tissue extension of the tumor. Typically, there is a large encapsulated soft tissue mass with low-to-intermediate signals on T1-weighted images. A T2-weighted image exhibits a homogeneous enhancement of medium-to-high intensity signal. Findings will depend on the amount of cystic, necrotic, or hemorrhagic material present. There may be inhomogeneous signal intensity or a poorly outlined tumor when this content is found. (5)

Definitive diagnosis is made with a biopsy. A patient who has a giant cell tumor should undergo a whole-body bone scan because 40% of patients will have giant cell tumors in other areas of the body. The differential diagnosis includes an eurysmal bone cysts, chondroblastoma, chondromyxoid fibroma, giant cell reparative granuloma, nonossifying fibroma, Langerhans' cell histiocytosis, synovial sarcoma, and high-grade central osteosarcoma. These lesions have similar radiographic appearances. (3,5)

The treatment of choice for most giant cell tumors is intralesional "extended" curettage. Curettage alone is associated with recurrence in almost 50% of cases. To avoid recurrence, physicians have added adjuvant treatments such as liquid nitrogen, phenol, and high-speed burring. (6) One study showed that the use of bisphosphonates may help reduce recurrence. (7) After tumor removal, the space can be filled with polymethylmethacrylate cement or bone graft. Using cement with curettage allows weight-bearing quickly, but a recurrence can easily develop around the cement. Bone grafts may provide better movement and use of the joint area.6 In small bones, giant cell tumors tend to show a permeative growth pattern. This feature makes a complete evacuation of the tumor difficult using only curettage. An en bloc resection with a bone graft has been recommended. When giant cell tumors recur and are benign, it usually is not fatal. With recurrence, it is imperative to do a detailed curettage with adjuvant therapy or joint-sparing surgery, if possible. With any giant cell tumor, follow-up on a regular basis is necessary. (1)

CONCLUSION

Giant cell tumors are relatively common bone tumors that are usually seen at the end of long bones in patients [less than or equal to]40 years of age. They usually have grown to a large size upon discovery. They often show no host immune response and no matrix. Our case presents giant cell tumor in a rare location with internal trabeculation and aggressive features in a patient with a history of trauma. A healing pathologic fracture through an enchondroma or aneurysmal bone cyst can have a similar radiographic appearance and create diagnostic uncertainty. An additional consideration in this case would include the malignant alteration of enchondroma to chondrosarcoma, but this has not been reported in this location to our knowledge. Particular attention should be made to patients with fractures because this is a common presentation for giant cell tumors. It is important to evaluate both the clinical and radiologic findings, and proceed with a biopsy. As with this case, treatment is appropriate removal of the tumor. Recurrent tumor is always of concern and can be reduced by certain techniques. (7)

REFERENCES

(1.) Szendroi M. Giant-cell tumor of bone. J Bone Joint Surg Br. 2004; 86:5-12.

(2.) Murphey MD, Nomikos GC, Flemming DJ, et al. From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: Radiologic-pathologic correlation. RadioGraphics. 2001;21:1283-1309.

(3.) Manaster BJ, Doyle AJ. Giant cell tumors of bone. Radiol Clin North Am. 1993;31:299-323.

(4.) Biscaglia R, Bacchini P, Bertoni F. Giant cell tumor of the bones of the hand and foot. Cancer. 2000; 88: 2022-2032.

(5.) Resnick D, Kyriakos M, Greenway G. Tumors and tumor-like lesions of bone: Imaging and pathology of specific lesions In: Resnick D, ed. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia, PA: Saunders; 2002:3939-3962.

(6.) DeGroot H. Giant cell tumor of bone. Available on line: www.bonetumor.org/tumors/pages/page106.html. Posted April 6, 2003. Accessed August 2005.

(7.) Chang SS, Suratwala SJ, Jung KM, et al. Bisphosphonates may reduce recurrence in giant cell tumor by inducing apoptosis. Clin Orthop Relat Res. 2004;426:103-109.

Elvedin Kulenovic, MD, PhD

Prepared by Elvedon Kulenovic, MD, PhD, University of Louisville Hospital, Department of Radiology, Louisville, KY.
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