Summary
The authors report 12 cases (8 men and 4 women) of sarcomatous degeneration in Paget's bone disease, with an average age of 72.3 years. Sarcomatous degeneration occurred often in polyostotic Paget's disease, and osteitis deformans was seen in 4 cases. Femur and pelvis were the most affected bones. Pain was a constant feature, whereas tumefaction and fracture were less common. Osteolytic lesions were more frequent than condensed or mixed lesions and radiological signs of malignancy were usually found. Seven cases were histologically clasiified as osteogenic sarcoma and 3 cases as fibrosarcoma. Electron microscopy was performed on 2 osteogenic sarcomas and in 1 case revealed microcylindrical inclusion in Pagetic osteoclasts and in multinucleated giant tumor cells, but none in mononucleated tumor cells. The average survival time for the patients in this study was only 4.5 months.
Similar content being viewed by others
References
Barry HC (1961) Sarcoma in Paget's disease of bone in Australia. J Bone Joint Surg 43 A: 1122–1134
Boutouras GD, Goodsitt E (1963) Sarcoma arising in Paget's disease. Report of two cases and review of the literature. J Intern Coll Surg 40:380–392
Choquette D, Haraou B, Altman RD, Pelletier JP (1983) Simultaneous multifocal sarcomatous degeneration in Paget's disease of bone. A hypothesis. Clin Orthop 179:308–311
Fergusson RJ, Yunis PhD, Yunis EJ (1978) The ultrastructure of human osteosarcoma: a study of nine cases. Clin Orthop 131:234–246
Friedlander GE, Mitchell MS (1976) A virally induced osteosarcoma in rats. A model for immunological studies of human osteosarcoma. J Bone Joint Surg 58 A:295–302
Fujinaga S, Poel WE, Dmochowski L (1970) Light and electron microscope studies of osteosarcomas induced in rats and hamsters by Harvey and Moloney sarcoma viruses. Cancer 30:1698–1708
Gherardi G, Lo Cascio V, Bonnuci E (1980) Fine structure of nucleic and cytoplasm of osteoclasts in Paget's disease of bone. Histopathology 4:63–74
Greditzer HG, Mc Leod RA, Unni KK, Peabout JB (1983) Bone sarcomas in Paget's disease. Radiology 146:327–333
Haibach H, Farrell C, Dittrich FJ (1985) Neoplasms arising in Paget's disease of bone: a study of 82 cases. Am J Clin Pathol 83:594–600
Hamdy RC (1981) Paget's disease of bone, Assessment and management. Praeger, Eastbourne, New York
Hauw JJ, Henin D, Chomette G, Escourolle R (1973) Sarcomes osseux développés sur localisations rachidiennes de maladie de Paget. Arch Anat Cytol Pathol 21:241–249
Hyang TL, Cohen NJ, Sahgal S, Tseng CH (1979) Osteosarcoma complicating Paget's disease of the spine with neurologic complications. Clin Orthop 141:260–265
Huvos AG (1979) Bone tumors. Diagnosis, treatment and prognosis. W.B. Saunders, Philadelphia
Huvos AG, Butler A, Bretsky SS (1983) Osteogenic sarcoma associated with Paget's disease of bone. Cancer 52:1489–1495
Mc Kenna RJ, Schwinn ChP, Soong KY, Higinbotham NL (1964) Osteogenic sarcoma arising in Paget's disease. Cancer 17:42–46
Marquant KH (1983) Intranuclear vermicellar bodies in human osteosarcoma and ossifying fibroma cells. J Cancer Res Clin Oncol 106:74–76
Mills BG, Singer FR (1976) Nuclear inclusions in Paget's disease of bone. Science 194:201–202
Mills BG, Singer FR, Weiner LP, Holst PA (1981) Immunohistological demonstration of respiratory syncytial virus antigens in Paget's disease of bone. Proc Natl Acad Sci USA 78:1209–1213
Mirra JM, Bauer FCH, Grant TT (1981) Giant cell tumor with viral-like intranuclear inclusions associated with Paget's disease. Clin Orthop 158:243–251
Morlock G, Sany J, Serre H (1975) Les sarcomes pagétiques. Rev Rhum Mal Osteoartic 42:669–678
Murray AB, Buscher H, Erfle V (1983) Intranuclear undulating membranous structures in cells of a human parosteal osteosarcoma. Ultrastruc Pathol 5:163–170
Paget J (1882) Additional cases of osteitis deformans. Med Clin Trans 65:225–236
Poreta C, Dhalin DC, Jame JM (1957) Sarcoma in Paget's disease of bone. J Bone Joint Surg 39 A:1314–1329
Price GH (1962) The incidence of osteogenic sarcoma in South-West England and its relationship to Paget's disease of bone. J Bone Joint Surg 44 B:366–376
Price GH, Goldie W (1969) Paget's sarcoma of bone; a study of eighty cases from the Bristol and the Leeds bone tumor registries. J Bone Joint Surg 51 B:205–224
Rebel A, Malkani K, Basle MF (1974) Anomalies nucléaires des ostéoclastes de la maladie osseuse de Paget. Nouv Presse Méd 3:1299–1301
Rebel A, Basle MF, Pouplard A, Malkani K, Filmon R, Le Patezour A (1980) Bone tissue in Paget's disease of bone. Ultrastructure and immunocytology. Arthritis Rheum 23:1104–1114
Rebel A, Basle MF, Pouplard A, Malkani K, Filmon R, Le Patezour A (1981) Towards a viral etiology for Paget's disease of bone. Metab Bone Dis Relat Res 4 and 5:235–238
Reddick RL, Michelicht HJ, Levine AM, Triche TJ (1980) Osteogenic sarcoma. A study of the ultrastructure. Cancer 45:64–71
Rubens-Duval A (1978) Aspects histologiques de la dégénérescence sarcomateuse de l'os pagétique. Rev Rhum Mal Osteoartic 42:663–668
Schajowicz F, Araujo ES, Berenstein M (1983) Sarcoma complicating Paget's disease of bone. A clinicopathological study of 62 cases. J Bone Joint Surg 65:299–307
Singer FR, Mills BG (1983) Evidence for a viral etiology of Paget's disease of bone. Clin Orthop 178:245–251
Smith J, Botet JF, Yeh SDJ (1984) Bone sarcomas in Paget's disease: a study of 85 patients. Radiology 152:583–590
Vacher-Lavenu MC, Louvel A, Daudet-Monsac M, Le Charpentier Y, Abelanet R (1981) Inclusions tubulo-filamenteuses intranucléaires dans les cellules multinucléées des tumeurs à cellules géantes des os. Etude ultrastructurale d'une série de 31 tumeurs. CR Acad Sc Paris Série III 293:639–644
Viola MV, Eilon G, Lazarus M (1982) Virus-like inclusions in osteosarcoma cells arising in Paget's disease. Lancet I:848
Welsh RA, Meyer AJ (1970) Nuclear fragmentation and associated fibrils in giant cell tumor of bone. Lab Invest 22:63–72
Wick MR, Siegal GP, Krishnan KV, Mc Leod RA, Greditzer HG (1981) Sarcomas of bone complicating osteitis deformans. Fifty years' experience. Ann J Surg Pathol 5:47–59
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Seret, P., Basle, M.F., Rebel, A. et al. Sarcomatous degeneration in Paget's bone disease. J Cancer Res Clin Oncol 113, 392–399 (1987). https://doi.org/10.1007/BF00397726
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00397726