Jaffé-Campanacci Syndrome

Classification according to ICD-10
Q74.8	Other specified congenital malformations of the extremity
Q78.9	Osteochondrodysplasia, unspecified
ICD-10 online (WHO version 2019)

The Jaffe Campanacci syndrome is a circumscribed dysplasia of the tibia. It was described by Henry Lewis Jaffe and Louis Lichtenstein (1942) and Mario Campanacci (1970).

Jaffé-Campanacci syndrome, boy, 7 years old, bending of the tibia with cortical / subcortical sclerosis and osteolysis

definition

The Jaffé-Campanacci syndrome is a cortical (growing) osteofibrous lesion, predominantly in the tibia, which occurs under 10 years of age. Histologically there is a similarity to the ossifying bone fibroma. Increasing deformation with anterior curvature and increase during growing age, cortical destruction and periosteal reaction,

The cause of this rare, non-malignant disease is unknown. It is considered a fibrovascular defect.

clinic

The disease is noticeable in the first 5 years of life with painless protrusion of the shinbone, boys are more frequently affected. The typical localization is anterior in the middle of the shaft in the tibial cortex. At the end of growth, the disease comes to a standstill.

In addition to the typical localization, the diagnosis results from the X-ray image . Characteristic are ventral compact thickening with swelling, stringy cancellous sclerosis sparing the medullary canal, surrounding osteolytic foci with overall honeycomb, marginal sclerosis and sharply demarcated structural changes. In 20% there is a second manifestation on the fibula.

The adamantinoma and fibrous dysplasia must be differentiated from the differential diagnosis .

literature

Mario Campanacci , M. Laus: Osteofibrous dysplasia of the tibia and fibula . The Journal of Bone & Joint Surgery [Am] 63-A (1981), p. 367.
Matthias Napp, Bernd Stengel, Joachim Buschmann, Rüdiger Döhler : Osteofibrous dysplasia of the Campanacci tibia. A 12 year result . The surgeon 3/2009. doi: 10.1007 / s00104-008-1591-x

Individual evidence

↑ Campanacci's syndrome (whonamedit.com)
↑ HL Jaffe, L. Lichtenstein: Non-osteogenic fibroma of bone. In: The American journal of pathology. Volume 18, Number 2, March 1942, pp. 205-221, PMC 2032933 (free full text), ISSN 0002-9440 .
↑ M. Campanacci, C. Leonessa: Displasia fibrosa dello scheletro. La Chirurgia degli organi di movimento 59 (1970), pp. 195-225. ISSN 0009-4749 . PMID 4931053 .
↑ ^a ^b ^c C. Adler: Bone Diseases. Springer 1983, ISBN 3-540-62836-3 , pp. 316, 336.
↑ ^a ^b J. Freyschmidt , H. Ostertag, G. Jundt: Bone Tumors. Springer, 2003, ISBN 3-540-40364-7 , p. 710.
↑ M. Hatori, M. Watanabe, M. Hosaka, H. Sasano, M. Narita, S. Kokubun: A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature. Tohoku J Exp Med 209 (2006), pp. 53-59, PMID 16636523 .

[1] Campanacci's syndrome (whonamedit.com)

[2] HL Jaffe, L. Lichtenstein: Non-osteogenic fibroma of bone. In: The American journal of pathology. Volume 18, Number 2, March 1942, pp. 205-221, PMC 2032933 (free full text), ISSN 0002-9440 .

[3] M. Campanacci, C. Leonessa: Displasia fibrosa dello scheletro. La Chirurgia degli organi di movimento 59 (1970), pp. 195-225. ISSN 0009-4749 . PMID 4931053 .

[Adler-4] C. Adler: Bone Diseases. Springer 1983, ISBN 3-540-62836-3 , pp. 316, 336.

[Freyschmidt-5] J. Freyschmidt , H. Ostertag, G. Jundt: Bone Tumors. Springer, 2003, ISBN 3-540-40364-7 , p. 710.

[6] M. Hatori, M. Watanabe, M. Hosaka, H. Sasano, M. Narita, S. Kokubun: A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature. Tohoku J Exp Med 209 (2006), pp. 53-59, PMID 16636523 .