Access Keys:
Skip to content (Access Key - 0)

High-Grade Pleomorphic Undifferentiated Sarcoma of Bone

DEFINITION AND PATHOGENESIS

  • High grade malignancy with histiocytic mesenchymal cell of origin
  • Previously known as Malignant Fibrous Histiocytoma

IMPORTANCE

  • May be primary (70%) or secondary (30%)
  • Diagnosis should be made after excluding other sarcomas with specific matrix production (eg, bone)

CLINICAL FEATURES

  • Most commonly in middle aged patients (45-55)
  • M:F = 3:2
  • Can be associated with preexisting Paget's disease, XRT, bone infarcts (most common malignancy associated with bone infarcts (idiopathic, sickle-cell, caisson's disease, use of steroids, Gaucher's disease as underlying etiologies) (less common secondary tumors are osteosarcoma, fibrosarcoma, angiosarcoma, poorly differentiated sarcoma), FD, NOF, ABC, hereditary bone dysplasia (up to 50% reported)
  • Pain ± swelling
  • Has been reported in brothers, one with multiple lesions
  • 10% with pathologic fx
  • ↑ alkφ with Paget's disease or osteosarcoma

RADIOLOGIC FEATURES

  • There is benign variant histologically similar in the cervical spinous processes
  • Aggressive osteolysis, premeative, destructive tumor growth centrally located in the metaphysis of long bones
  • Sequestrum may be seen
  • Most common site is distal femoral and proximal tibial metaphysis
  • Diaphyseal involvement in < 10%
  • Periosteal reaction is reported rare to frequent, cortical expansion infrequent
  • May appear as a cold defect on bone scan
  • MRI: Inhomogeneous, nodular pattern with peripheral Gd-DTPA enhancement on T1- and T2-weighted images
  • ± extraosseous extension (80-100% reported)
  • ± joint invasion (up to 50%)

GROSS PATHOLOGY

  • Extensive permeation of host bone
  • ± soft tissue mass

HISTOLOGIC FEATURES

  • Subtypes
    • Storiform-pleomorphic (50-60%)
    • Myxoid (25%)
  • Spindle cells in a storiform pattern
  • Little collagen present with the fibroblast having abundant cytoplasm
  • Nuclear pleomorphism
  • Anaplastic giant cells can distinguish this lesion from fibrosarcoma
  • Malignant foamy cells
  • 50% with necrosis and hemosiderin
  • Most with chronic inflammatory cells
  • 1/3 with giant cells

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Osteosarcoma
  • Fibrosarcoma
  • Secondary MFH (30% reported cases) (arising in bone infarcts, Paget disease, irradiated tissue, fibrous dysplasia, enchondroma, GCT, chronic osteomyelitis)

DISEASE COURSE AND TREATMENT

  • Wide resection
  • Adriamycin the only chemotherapeutic agent found effective (50% 5 year survival vs 20-30% with surgery alone) and adjuvant chemotx may be better than neoadjuvant to ? drug resistance but neoadjuvant better to ? safety for limb salvage
  • Higher rate of disease free survival (94 vs 61%) if a good histologic response to chemotx
  • XRT — different opinions re: pre- or post-resection doses; used for unresectable lesions, or those presenting with wide spread metastases
  • Lymph node metastases may occur rarely
  • LR reported in up to 51%

REFERENCES

Abrahams TG, Hull M: MFH arising in an infarct of bone. Skeletal Radiol 15:578-83,1986.
Bacci G, Picci P, Mercuri M, Bertoni F, Ferrari S: Neoadjuvant chemotherapy for high grade malignant fibrous histiocytoma of bone. Clin Orthop 346:178-89,1998.
Bacci G, Springfield D, Campanna R, Picci P, Bertoni F, Campanacci M: Adjuvant chemotherapy for malignant fibrous histiocytoma in the femur and tibia. J Bone Joint Surg 67:620-5,1985.
Bidwell JK, Young JW, Saylor L: MFH of spine: CT appearance and review of the literature. J Comput Tomogr 11:355-8,1987.
Boland P, Huvos A: Malignant fibrous histiocytoma of bone. Clin Orthop 204:130-4,1986.
Bullough PG, Kambolis CP, Marcove RC, Jaffe HL: Bone infarctions not associated with caisson disease. J Bone Joint Surg 47A:477-91,1965.
Capanna R, Bertoni F, Bacchini P, Gacci G, Guerra A, Campanacci M: Malignant fibrous histiocytoma of bone: the experience of the Rizzoli Institute. Report of 90 cases. Cancer 54:177-87,1984.
Chen KT: Multiple fibroxanthosarcoma of bone. Cancer 42:770-3,1978.
Dahlin DC, Unni KK, Matsuno T: Malignant (fibrous) histiocytoma of bone--fact or fancy? Cancer 39:1508-16,1977.
den Hecten GJ, Schraffordt Koops H, Kamps WA, Walter Oosterhus J, Sleijfer DTh, Oldhoff J: Treatment of malignant fibrous histiocytoma of bone: a plea for primary chrmotherapy. Cancer 56:37-40,1985.
Dunham WK, Wilborn WH: Malignant fibrous histiocytoma of bone. Report of two cases and review of the literature. J Bone Joint Surg 61:939-42,1979.
Enzinger FM: Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer 44:2147-57,1979.
Ghandur-Mnaymneh L, Zych G, Mnaymneh W: Primary malignant fibrous histiocytoma of bone: report of six cases with ultrastructural study and analysis of the literature. Cancer 49:698-707,1982.
Feldman F, Lattes R: Primary malignant fibrous histiocytoma (fibrous xanthoma) of bone. Skeletal Radiol 1:145-60,1977.
Finci R, Gunhan O, Ucmakli E, Sarlaki O: Multiple and familial malignant fibrous histiocytoma of bone. A report of two cases. J Bone Joint Surg 72A:295-8,1990.
Hantamaa PV, Gaither DW, Thompson RC, Jr: Malignant fibrous histiocytoma arising in the region of a femoral fracture. A case report. J Bone Joint Surg 74A:777-80,1992.
Hudson TM, Hawkins IF, Jr, Spanier SS, Enneking WF: Angiography of malignant fibrous histeiocytoma of bone. Radiology 131:9-15,1979.
Huvos A, Heilweil M, Bretsky S: The pathology of malignant fibrous histiocytoma of bone: a study of 130 patients. Am Surg Pathol 9:853-71,1985.
Huvos AG, Woodard HQ, Heilweil M: Postradiation malignant fibrous histiocytoma of bone. A clinicopathologic study of 20 patients. Am J Surg Pathol 10:9-18,1986.
Johns WW, Coburn TP, Pratt CB, Smith JW, Kumar AP, Dahlin DC: Ultrastructure of malignant histiocytoma arising in the acromion. Hum Pathol 9:199-209,1978.
Kahan LB, Webber B, Mills E, Anstey L, Heselson NG: Malignant fibrous histiocytoma (malignant fibrous xanthoma: xanthosarcoma) of bone. Cancer 42:640-51,1978.
Kenan S, Abdelwahab IF, Hemann G, Klein MJ: Malignant fibrous histiocytoma associated with a bone infarct in a patient with hereditary bone dysplasia. Skeletal Radiol 27:463-7,1998.
Kennedy C, Stoker D: Case report: malignant fibrous histiocytoma complicating chronic osteomyelitis. Clin Radiol 41:435-6,1990.
Kyriakos M, Kempson RL: Inflammatory fibrous histiocytoma. An aggressive and lethal lesion. Cancer 37:1584-1606,1976.
Link TM, Haeussler MD, Poppek S, Woertler K, Blasius S, Lindner N, Rummeny EJ: Malignant fibrous histiocytoma of bone: conventional x-ray and MR imaging features. Skeletal Radiol 27:552-8,1998.
Lopez-Barea F, Rodriguez-Peralto JL, Burgos-Sizalde E, Gonzalez-Lopez J, Sanchez-Herrera S: Case report 639: malignant fibrous histiocytoma (MFH) of the patella. Skeletal Radiol 20:125-8,1991.
Marks, KE, "Fibrous Tumors of Bone", Orthop Clin NorthAm 20:3:377-394,1989.
McCarthy EF, Matsuno T, Dorfman HD: Malignant fibours histiocytoma of bone: a study of 35 cases. Hum Pathol 10:57-70,1979.
Meis-Kindblom JM, Enzinger FM: Color Atlas of Soft Tissue Tumors. Mosby-Wolfe, Portland, 1996, pp 3-71, 190-9, 242-3, 276-7, 289-94, 296-7.
Merkow RL, Lane JM: Current concepts of Paget's disease of bone. Orthop Clin North Am 15:747-63,1984.
Michael RH, Dorfman HD: Malignant fibrous histiocytoma associated with bone infarcts: report of a case. Clin Orthop 118:180-3,1976.
Mirra, JM: Fibrohistiocytic tumors of intramedullary origin. In Bone Tumors. Clinical, radiologic, and pathologic correlations. JM Mirra (Ed), Lea & Febiger, Philadelphia, 1989, pp 691-799.
Mirra JM, Bullough PG, Marcove RC, Jacobs B, Huvos AG: Malignant fibrous histiocytoma and osteosarcoma in association with bone infarcts; report of four cases. tow in caisson workers. J Bone Joint Surg 56A:932-40,1974.
Mirra JM, Gold RH, Marafiote R: Malignant (fibrous) histiocytoma arising in association with a bone infarct in sickle-cell disease: coincidence or cause-and -effect? Cancer 39:186-94,1977.
Naka T, Fukuda T, Shinohara N, Iwamoto Y, Sugioka Y, Tsuneyoshi M: Osteosarcoma versus malignant fibrous histiocytoma of bone in patients older than 40 years. Cancer 76:972-84,1995.
Nakashima Y, Morishita S, Kotoura Y, Yamamuro T, Tamura K, Onomura T, Sudo Y, Awaya G, Hamashima Y: Malignant fibrous histiocytoma of bone. A review of 13 cases and an ultrasturctural study. Cancer 55:2804-11,1985.
Newland RC, Harrison MA, Wright RG: Fibroxanthosarcoma of bone. Pathology 7:203-8,1975.
Nishida J, Sim FH, Wenger DE, Unni KK: Malignant fibrous histiocytoma of bone: a clinicopathologic study of 81 patients. Cancer 79:482-93,1997.
Paling MR, Hyams DM: Computed tomography in malignant fibrous histiocytoma. J Comput Assist Tomogr 6:785-8,1982.
Panicek D, Casper E, Brennan M, Hajdu S, Heelan R: Hemorrhage simulating tumor growth in malignant fibrous histiocytoma at MR imaging. Radiology 181:398-400,1991.
Papagelopoulos PJ, Galanis EC, Sim FH, Unni KK: Clinicopathologic features, diagnosis, and treatment of malignant fibrous histiocytoma of bone. Orthopedics 23:59-65,2000.
Picci P, Bacci G, Ferrari S, Mercuri M: Neoadjuvant chemotherapy in malignant fibrous histiocytoma of bone and in osteosarcoma located in the extremities: analogies and differences between the two tumors. Ann Oncol 8:1107-15,1997.
Roger DJ, Uhl RL, Carl A: Malignant fibrous histiocytoma of the patella. Orthopedics 17:189-92,1994.
Ros PR, Viamonte M, Jr, Rywlin AM: Malignant fibrous histiocytoma: mesenchymal tumor of ubiquitous origin. AJR 142:753-9,1984.
Saito R, Caines MJ: Atypical fibrous histiocytoma of the humerus: a light and electron microscoic study Am J Clin Pathol 68:409-15,1977.
Sakkers R, van der Heul R, Kroon H, Taminiau A, Hoyendoorn P: Late transformation of a benign giant-cell tumor of bone: a case report. J Bone Joint Surg 79:259-62,1997.
Sanders TG, Wilf LH, Northup HM: MFH: etiology for a cold defect on Tc-99m-MDP bone scan. J Nucl Med 31:1104-5,1990.
Shuman LS, Chuang VP, Wallace S, Benjamin RS, Murray J: Intra-arterial chemotherapy of malignant fibrous histiocytoma of the pelvis. Radiology 142:343-6,1982.
Spanier SS: Malignant fibrous histiocytoma of bone. Orthop Clin North Am 8:947-61,1977.
Spanier SS, Enneking WF, Enriquez P: Primary malignant fibrous histiocytoma of bone. Cancer 36:2084-98,1975.
Taconis WK, Mulder JD: Fibrosarcoma and malignant fibrous histiocytoma of long bones: Radiographic features and grading. Skeletal Radiol 11:237-45,1984.
Taconis WK, van Rijssel TG: Fibrosarcoma of long bones. A study of the significance of areas of malignant fibrous histiocytoma. J Bone Joint Surg 67B:111-6,1985.
Taxy JB, Battifor H: Malignant fibrous histiocytoma. An electron microscopic study. Cancer 40:254-67,1977.
Ward JJ, Thornbury DD, Lemons JE, Dunham WK: Metal-induced sarcoma. A case report and literature review. Clin Orthop 252:299-306,1990.
Weiner M, Sedlis M, Johnston AD, Dick HM, Wolff JA: Adjuvant chemotherapy of malignant fibrous histiocytoma of bone. Cancer 51:25-9,1983.
Yuen WW, Saw D: Malignant fibrous histiocytoma of bone. J Bone Joint Surg 67A:482-6,1985.

Content

Download: PDF | EPUB

Famous Quote
"It is extremely difficult to improve the status of the asymptomatic patient." Henry J. Mankin
Figures
Related Content

Resources for High-Grade Pleomorphic Undifferentiated Sarcoma of Bone and related topics on OrthopaedicsOne.