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POLYOSTOTIC FIBROUS DYSPLASIA

POLYOSTOTIC FIBROUS DYSPLASIA Abstract My attention was recently drawn by Dr. Henry L. Jaffe, director of the laboratories at the Hospital for Joint Diseases, to a number of cases (4 from this and 4 more from other hospitals from which pathologic material was studied) presenting multiple osseous lesions and having sufficient features in common to differentiate them as a distinct clinical entity. The condition is designated as polyostotic fibrous dysplasia, for reasons to be indicated presently. DEFINITION AND NOMENCLATURE The name "polyostotic fibrous dysplasia" is being used in the Hospital for Joint Diseases to designate a skeletal developmental anomaly affecting several or many bones with predominantly unilateral involvement. The involved bones show filling of their medullary cavities by gritty, grayish white fibrous tissue containing trabeculae of newly formed primitive bone. The condition apparently results from perverted activity of the specific bone-forming mesenchyme. It manifests itself in childhood or early adult life and evolves slowly, References 1. A great deal of confusion exists with regard to the nomenclature of skeletal diseases, inherited largely from early investigators. The term "osteitis fibrosa" was employed in the German literature until comparatively recently to include the osseous lesions of hyperparathyroidism, Paget's disease, giant cell tumors, cysts of the bone and localized osteitis fibrosa. The term "fibrous osteodystrophy" has been applied to the same miscellaneous, pathologically unrelated group of osseous diseases, and in fact is still used in the Index Medicus synonymously with osteitis fibrosa. The terms nongeneralized fibrous osteodystrophy and unilateral fibrous osteodystrophy, introduced by some authors to obviate the confusion of the condition under discussion with generalized osteitis fibrosa cystica, are open to the same objection. It seems to me that the situation would be much clarified if giant cell tumors, simple cysts of the bone of undetermined origin and localized osteitis fibrosa (i. e., fibrosis of the marrow) due to whatever cause were regarded as different entities until more is known of their pathogenesis and the name polyostotic fibrous dysplasia introduced for the clinical entity discussed in this paper. 2. Goldhamer, K.: Osteodystrophia fibrosa unilateralis (kombiniert mit Pubertas praecox und mit gleichseitigen osteosklerotischen Veränderungen der Schädelbasis) , Wien. klin. Wchnschr. 47:218, 1934 3. Fortschr. a. d. Geb. d. Röntgenstrahlen 49:456, 1934. 4. Borak, J., and Doll, B.: Halbseitige Recklinghausensche Knochenkrankheit mit Pubertas praecox , Wien. klin. Wchnschr. 47:540, 1934. 5. Albright, F.; Butler, A. M.; Hampton, A. O., and Smith. P.: Svndrome Characterized by Osteitis Fibrosa Disseminata, Areas of Pigmentation and Endocrine Dysfunction, with Precocious Puberty in Females , New England J. Med. 216:727, 1937.Crossref 6. Freund, E., and Meffert, C. B.: On the Different Forms of Non-Generalized Fibrous Osteodystrophy , Surg., Gynec. & Obst. 62:541, 1936. 7. Bodansky, A., and Jaffe, H. L.: Phosphatase Studies: III. Serum Phosphatase in Diseases of the Bone; Interpretation and Significance , Arch. Int. Med. 54:88 ( (July) ) 1934. 8. Telford, E. D.: A Case of Osteitis Fibrosa, with Formation of Hyaline Cartilage , Brit. J. Surg. 18:409, 1931. 9. Hunter, D., and Turnbull, H. M.: Hyperparathyroidism: Generalized Osteitis Fibrosa , Brit. J. Surg. 19:203, 1931. 10. Jaffe, H. L.: Hyperparathyroidism (Recklinghausen's Disease of Bone) , Arch. Path. 16:63 ( (July) ) 11. 236 (Aug.) 1933. 12. Phemister, D. B., and Grimson, K. S.: Fibrous Osteoma of the Jaws , Ann. Surg. 105:564, 1937. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

POLYOSTOTIC FIBROUS DYSPLASIA

Archives of Surgery , Volume 36 (5) – May 1, 1938

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Publisher
American Medical Association
Copyright
Copyright © 1938 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1938.01190230153012
Publisher site
See Article on Publisher Site

Abstract

Abstract My attention was recently drawn by Dr. Henry L. Jaffe, director of the laboratories at the Hospital for Joint Diseases, to a number of cases (4 from this and 4 more from other hospitals from which pathologic material was studied) presenting multiple osseous lesions and having sufficient features in common to differentiate them as a distinct clinical entity. The condition is designated as polyostotic fibrous dysplasia, for reasons to be indicated presently. DEFINITION AND NOMENCLATURE The name "polyostotic fibrous dysplasia" is being used in the Hospital for Joint Diseases to designate a skeletal developmental anomaly affecting several or many bones with predominantly unilateral involvement. The involved bones show filling of their medullary cavities by gritty, grayish white fibrous tissue containing trabeculae of newly formed primitive bone. The condition apparently results from perverted activity of the specific bone-forming mesenchyme. It manifests itself in childhood or early adult life and evolves slowly, References 1. A great deal of confusion exists with regard to the nomenclature of skeletal diseases, inherited largely from early investigators. The term "osteitis fibrosa" was employed in the German literature until comparatively recently to include the osseous lesions of hyperparathyroidism, Paget's disease, giant cell tumors, cysts of the bone and localized osteitis fibrosa. The term "fibrous osteodystrophy" has been applied to the same miscellaneous, pathologically unrelated group of osseous diseases, and in fact is still used in the Index Medicus synonymously with osteitis fibrosa. The terms nongeneralized fibrous osteodystrophy and unilateral fibrous osteodystrophy, introduced by some authors to obviate the confusion of the condition under discussion with generalized osteitis fibrosa cystica, are open to the same objection. It seems to me that the situation would be much clarified if giant cell tumors, simple cysts of the bone of undetermined origin and localized osteitis fibrosa (i. e., fibrosis of the marrow) due to whatever cause were regarded as different entities until more is known of their pathogenesis and the name polyostotic fibrous dysplasia introduced for the clinical entity discussed in this paper. 2. Goldhamer, K.: Osteodystrophia fibrosa unilateralis (kombiniert mit Pubertas praecox und mit gleichseitigen osteosklerotischen Veränderungen der Schädelbasis) , Wien. klin. Wchnschr. 47:218, 1934 3. Fortschr. a. d. Geb. d. Röntgenstrahlen 49:456, 1934. 4. Borak, J., and Doll, B.: Halbseitige Recklinghausensche Knochenkrankheit mit Pubertas praecox , Wien. klin. Wchnschr. 47:540, 1934. 5. Albright, F.; Butler, A. M.; Hampton, A. O., and Smith. P.: Svndrome Characterized by Osteitis Fibrosa Disseminata, Areas of Pigmentation and Endocrine Dysfunction, with Precocious Puberty in Females , New England J. Med. 216:727, 1937.Crossref 6. Freund, E., and Meffert, C. B.: On the Different Forms of Non-Generalized Fibrous Osteodystrophy , Surg., Gynec. & Obst. 62:541, 1936. 7. Bodansky, A., and Jaffe, H. L.: Phosphatase Studies: III. Serum Phosphatase in Diseases of the Bone; Interpretation and Significance , Arch. Int. Med. 54:88 ( (July) ) 1934. 8. Telford, E. D.: A Case of Osteitis Fibrosa, with Formation of Hyaline Cartilage , Brit. J. Surg. 18:409, 1931. 9. Hunter, D., and Turnbull, H. M.: Hyperparathyroidism: Generalized Osteitis Fibrosa , Brit. J. Surg. 19:203, 1931. 10. Jaffe, H. L.: Hyperparathyroidism (Recklinghausen's Disease of Bone) , Arch. Path. 16:63 ( (July) ) 11. 236 (Aug.) 1933. 12. Phemister, D. B., and Grimson, K. S.: Fibrous Osteoma of the Jaws , Ann. Surg. 105:564, 1937.

Journal

Archives of SurgeryAmerican Medical Association

Published: May 1, 1938

References