JBJS | Occult Sarcoma of the Femoral Head in Patients Undergoing Total Hip Arthroplasty : A Report of Two Cases*

The Journal of Bone & Joint Surgery, Volume 82, Issue 11

Articles | November 01, 2000

Occult Sarcoma of the Femoral Head in Patients Undergoing Total Hip Arthroplasty : A Report of Two Cases*

Steven D. Billings, M.D.†; L. Daniel Wurtz, M.D.†; Eduardo Tejada, M.D.‡; John D. Henjey, M.D.†

Investigation performed at the Departments of Pathology and Laboratory Medicine and Orthopaedic Surgery, Indiana University School of Medicine, and the Department of Pathology and Laboratory Medicine, Richard L. Roudebush Veterans Affairs Medical Center, Indianapolis, Indiana
*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.
†Departments of Pathology and Laboratory Medicine (S. D. B. and J. D. H.) and Orthopaedic Surgery (L. D. W.), Indiana University Hospital, 550 North University Boulevard, Indianapolis, Indiana 46202.
‡Department of Pathology and Laboratory Medicine, Richard L. Roudebush Veterans Affairs Medical Center, 1481 West 10th Street, Indianapolis, Indiana 46202.

The Journal of Bone & Joint Surgery. 2000; 82:1536-1536

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Introduction

Introduction | Case Reports | Discussion | References

Total hip arthroplasty is a common treatment for patients with arthritic conditions of the hip joint. Removal of the diseased femoral head is an essential part of this operative procedure. A subsequent pathological examination of the femoral head generally confirms the clinical diagnosis. Recently, however, primary sarcoma of the femoral head was discovered in the specimens from two patients undergoing total hip arthroplasty for presumed benign disease. Although neither patient had clinical evidence of a malignant tumor, both had risk factors for the development of sarcoma in their medical history.

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Fig. 1-A:: Figs. 1-A and 1-B: Case 1.
Fig. 1-A: Anteroposterior plain radiograph of the pelvis, showing postoperative changes from the previous pelvic operation. Note the subtle radiodensity and the subchondral collapse of the femoral head.

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Fig. 1-B:: Coronal T1-weighted magnetic resonance image of the pelvis, showing abnormal signal in the left femoral head with an appearance consistent with avascular necrosis.

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Fig. 2:: Case 2. Anteroposterior radiograph of the left hip, showing the coarse trabecular pattern of the proximal part of the femur that is typical of Paget disease.

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Fig. 3-A:: Figs. 3-A and 3-B: Photomicrographs of Case 1.
Fig. 3-A: The osteosarcomatous component had markedly pleomorphic tumor cells embedded within an osteoid matrix. The malignant osteoid had focal mineralization (left side of figure) (hematoxylin and eosin, Â¥ 200).

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Fig. 3-B:: Scattered atypical, multivacuolated lipoblasts were present (hematoxylin and eosin, Â¥ 200).

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Fig. 4-A:: Figs. 4-A and 4-B: Photomicrographs of Case 2.
Fig. 4-A: Low-power view demonstrating irregular woven bone with abnormal cement lines characteristic of Paget disease (hematoxylin and eosin, Â¥ 40).

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Fig. 4-B: Malignant fibrous histiocytoma with a somewhat fascicular arrangement of pleomorphic spindle cells (hematoxylin and eosin, Â¥ 100).

Case Reports

Introduction | Case Reports | Discussion | References

Case 1. A forty-six-year-old woman presented with a two-week history of pain in the left hip and an inability to walk. She had a history of cervical cancer; treatment had included pelvic exenteration with adjuvant radiation and chemotherapy seventeen years prior to the onset of the hip symptoms. Plain radiographs and magnetic resonance imaging showed changes consistent with avascular necrosis and secondary degenerative joint disease (Fig. 1-A and Fig. 1-B). No abnormal masses were visible on these studies. The patient underwent a left total hip arthroplasty. Intraoperatively, the femoral head was noted to be soft, with areas of deterioration about the femoral neck. During dislocation of the femoral head, the femoral neck fractured. The remainder of the operation proceeded uneventfully, and postoperative radiographs showed the prosthesis to be in a good position.

Case 2. A seventy-five-year-old man with Paget disease presented with a ten-month history of pain in the left hip and an inability to bear weight on the affected limb. Plain radiographs showed a coarse trabecular pattern consistent with Paget disease with superimposed degenerative joint disease (Fig. 2); no malignancy was suspected. The patient underwent an uneventful left total hip arthroplasty.

Pathological Findings

Both specimens demonstrated gross degenerative changes, with thinning and eburnation of the articular cartilage. In the first case, examination of the cut surface revealed focal softening of the medullary bone. A separate segment of the femoral neck was submitted for pathological evaluation. In the second case, the femoral head was deformed and flattened, with areas of red-brown necrosis evident on cut section. Neither specimen had obvious gross tumor.

Histological examination of the femoral head specimen in Case 1 demonstrated a high-grade osteosarcoma with focal lipoblastic differentiation. The osteosarcomatous component exhibited tumor cells with enlarged, hyperchromatic nuclei embedded in neoplastic osteoid (Fig. 3-A). The lipoblastic component was composed of atypical cells (lipoblasts) with cytoplasmic fat vacuoles and hyperchromatic nuclei (Fig. 3-B). Immunohistochemically, the tumor stained negatively for cytokeratin, epithelial membrane antigen, and S-100 protein. The tumor was confined within the medullary bone. Evaluation of the surgical margin was not possible because of the fragmented nature of the specimen. In Case 2, the femoral head was involved by a high-grade malignant fibrous histiocytoma arising in a background of Paget disease. Histologically, the tumor demonstrated malignant spindle cells arranged in a storiform to vaguely fascicular pattern, pleomorphic nuclei, and tumor giant cells (Fig. 4-A and Fig. 4-B). There were large areas of coagulative tumor necrosis. Immunohistochemically, the tumor cells were negative for cytokeratin and desmin.

Discussion

Introduction | Case Reports | Discussion | References

The unexpected finding of sarcoma of the femoral head is not well described in the medical literature. We encountered two cases of femoral head sarcoma discovered in patients who underwent total hip arthroplasty because of degenerative joint disease with avascular necrosis or degenerative joint disease associated with Paget disease. Notably, both patients had risk factors for the development of sarcoma, including prior radiation therapy (Case 1) and Paget disease (Case 2).

The sarcoma in Case 1 was unusual in that it exhibited both osseous and lipomatous differentiation. Sarcomas exhibiting divergent cellular differentiation have been called malignant mesenchymoma by previous authors^9,18. Although they are quite rare, cases of malignant mesenchymoma arising in bone have been described^{4,5,7,9,10,13-16,19}. Most commonly, they are encountered in young adults. Radiographically, the typical appearance is an osteolytic, expansile mass^{9,10,14-16,19} that frequently extends into adjacent soft tissue^9,10,13-16. In Case 1, the tumor arose within a field of prior irradiation for the treatment of cervical cancer. Radiation-induced malignant mesenchymomas of bone⁷ and soft tissue³, including a case in a patient who had received radiation therapy for cervical cancer³, have been described. The lack of radiographic or gross evidence of tumor in our patient is unusual, though it may have been attributable, in part, to confinement of the tumor to medullary bone. Although prognostic factors have not been well delineated, primary malignant mesenchymoma of bone is biologically aggressive and was fatal in seven of eleven reported cases with follow-up¹⁹. Parenthetically, we are aware of only one previously described case of radiation-induced malignant mesenchymoma of bone⁷.

Sarcomatous degeneration of Paget disease is a well known but relatively uncommon phenomenon occurring in up to 1 percent of patients^6,11,20. Patients usually present with pain, which may be chronic, and soft-tissue swelling around the affected site^6,8,17,20. Our patient with Paget disease (Case 2) reported pain but did not exhibit any swelling about the hip; the latter observation is not surprising given that the tumor had not extended into the adjacent soft tissue. Most sarcomas arising in patients with Paget disease are osteolytic lesions associated with cortical destruction and soft-tissue masses. Radiographic studies are usually helpful in identifying the development of a Paget sarcoma, but the radiographic features can overlap with those of the primary pagetic lesion, metastatic disease, or other disease processes affecting the bones^1,2,6,11. Moreover, magnetic resonance imaging can be useful in detecting soft-tissue extension, but its utility in showing early sarcomatous transformation may be limited¹¹. Therefore, it is important to maintain a high index of suspicion for sarcomatous transformation in the setting of Paget disease.

Osteosarcoma is the most common histological type of sarcoma associated with Paget disease⁶; malignant fibrous histiocytoma is the second most common type. Sarcomatous transformation has a dismal prognosis. The five-year survival rate has been 5 percent or less in most studies. In series of sixty-five, eighty, and eighty-five patients, there were only three, four, and three survivors, respectively, at five years^6,8,17. The response to radiation therapy or chemotherapy has not been promising^6,12,17,20; therefore, early detection followed by complete surgical excision offers the best hope for long-term survival.

Primary sarcomatous bone tumors in older adults often occur as a secondary transformation of either an existing benign bone lesion, such as a bone infarct, fibrous dysplasia, or Paget disease, or subsequent to the effects of previous radiation exposure. Surgeons must obtain a pertinent history from patients prior to a planned total hip arthroplasty, be cognizant of the recognized risk factors, and maintain a high level of suspicion for an occult sarcoma in at-risk patients who present with the common, seemingly benign disease of painful hip arthritis.

The existence of an occult primary bone sarcoma in our two patients with otherwise benign clinical findings underscores the necessity for routine pathological examination of femoral head specimens from patients who are at risk for the development of a secondary malignant tumor. For patients with predisposing risk factors, the procedure should be planned in order to provide an assessable surgical margin in the event that an incidental sarcoma is subsequently discovered. Evaluation of frozen intraoperative sections merits consideration, in selected cases, when an unexpected mass is found or the clinical suspicion of a malignant tumor is high. It is important to note, however, that the frozen-section technique is limited to assessment of soft tissue and/or soft marrow curettings that are free of larger bone fragments. Failure to recognize an occult primary malignant tumor results in a delay of diagnosis and in possible therapeutic mismanagement.

References

Introduction | Case Reports | Discussion | References

Anderson, J. T., and Dehner, L. P.: Osteolytic form of Paget's disease: differential diagnosis and pathogenesis. J. Bone and Joint Surg.,58-A: 994-1000, Oct 1976.58-A994 1976

Bowerman, J. W.; Altman, J.; Hughes, J. L.; and Zadek, R. E.: Pseudo-malignant lesions in Paget's disease of bone. Am. J. Roentgenol.,124: 57-61, 1975.12457 1975

Chung, C. K.; Stryker, J. A.; Cohen, C.; and Mortel, R.: Malignant mesenchymoma developing 6 years after radical hysterectomy and postoperative radiotherapy for cervical carcinoma. Gynecol. Oncol.,12: 367-372, 1981.12367 1981 [PubMed]

Cremer, H.; Koischwitz, D.; and Tismer, R.: Primary osteoliposarcoma of bone. J. Cancer Res. and Clin. Oncol.,101: 203-211, 1981.101203 1981

Downey, E. F., Jr.; Worsham, G. F.; and Brower, A. C.: Liposarcoma of bone with osteosarcomatous foci: case report and review of the literature. Skel. Radiol.,8: 47-50, 1982.847 1982

Hadjipavlou, A.; Lander, P.; Srolovitz, H.; and Enker, I. P.: Malignant transformation in Paget disease of bone. Cancer,70: 2802-2808, 1992.702802 1992 [PubMed]

Hatlinghus, S.; Rode, L.; Christensen, I.; and Vaage, S.:: Sarcoma following irradiation for breast cancer. Report of three unusual cases including one malignant mesenchymoma of bone. Acta Radiol. Oncol.,,25: 239-242, 1986.25239 1986

Huvos, A. G.; Butler, A.; and Bretsky, S. S.: Osteogenic sarcoma associated with Paget's disease of bone. A clinicopathologic study of 65 patients. Cancer,52: 1489-1495, 1983.521489 1983 [PubMed]

Kessler, S.; Mirra, J. M.; Ishii, T.; Thompson, J. C.; and Brien, E. W.: Primary malignant mesenchymoma of bone: case report, literature review, and distinction of this entity from mesenchymal and dedifferentiated chondrosarcoma. Skel. Radiol.,24: 291-295, 1995.24291 1995

Lamovec, J.; Zidar, A.; Bracko, M.; and Golouh, R.: Primary bone sarcoma with rhabdomyosarcomatous component. Pathol. Res. and Pract.,190: 51-60, 1994.19051 1994

Mirra, J. M.; Brien, E. W.; and Tehranzadeh, J.: Paget's disease of bone: review with emphasis on radiologic features, Part II. Skel. Radiol.,24: 173-184, 1995.24173 1995

Price, C. H. G., and Goldie, W.: Paget's sarcoma of bone. A study of eighty cases from the Bristol and the Leeds Bone Tumour Registries. J. Bone and Joint Surg.,51-B(2): 205-224, 1969.51-B(2)205 1969

Reijnierse, M.; Kroon, H. M.; Van Der Heul, R. O.; and Mulder, J. D.: Mesenchymoma of bone: a case report. J. Bone and Joint Surg.,75-A: 112-115, Jan 1993.75-A112 1993

Ross, C. F., and Hadfield, G.: Primary osteo-liposarcoma of bone (malignant mesenchymoma). Report of a case. J. Bone and Joint Surg.,50-B(3): 639-643, 1968.50-B(3)639 1968

Schajowicz, F.; Cuevillas, A. R.; and Silberman, F. S.: Primary malignant mesenchymoma of bone. A new tumor entity. Cancer,,19: 1423-1428, 1966.191423 1966

Scheele, P. M., Jr.; Von Kuster, L. C.; and Krivchenia, G., II: Primary malignant mesenchymoma of bone. Arch. Pathol. and Lab. Med.,114: 614-617, 1990.114614 1990

Smith, J.; Botet, J. F.; and Yeh, S. D.: Bone sarcomas in Paget disease: a study of 85 patients. Radiology,152: 583-590, 1984.152583 1984 [PubMed]

Stout, A. P.: Mesenchymoma, the mixed tumor of mesenchymal derivatives. Ann. Surg.,127: 278-290, 1948.127278 1948

Van Dorpe, J.; Sciot, R.; Samson, I.; De Vos, R.; Brys, P.; and Van Damme, B.: Primary osteorhabdomyosarcoma (malignant mesenchymoma) of bone: a case report and review of the literature. Mod. Pathol.,10: 1047-1053, 1997.101047 1997 [PubMed]

Wick, M. R.; Siegal, G. P.; Unni, K. K.; McLeod, R. A.; and Greditzer, H. G., III: Sarcomas of bone complicating osteitis deformans (Paget's disease): fifty years' experience. Am. J. Surg. Pathol.,5: 47-59, 1981.547 1981 [PubMed]

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