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Beitragstitel Osteofibrous Dysplasia-like Adamantinoma (OFD-AD) of the Tibia progressing into Adamantinoma (AD) after 36 years
Beitragscode P63
  1. Tobias C. Bühler Kantonsspital Baden AG Vortragender
  2. G. Ulrich Exner Orthopädie Zentrum Zürich
  3. Natasha Forster Swissparc AG
  4. Michael O. Kurrer Pathologikum
  5. Pascal A. Schai LUKS Luzern und Wolhusen
Präsentationsform Poster
  • A07 - Spezialgebiet 3 | Tumore
Abstract Introduction
Osteofibrous dysplasia (OFD) is a rare benign, fibro-osseous lesion predominantly affecting the tibia (Campanacci). Osteofibrous dysplasia-like adamantinoma (OFD-AD) differs from OFD by the inclusion of clusters of epithelial cells spread throughout the lesion, whilst Admantinoma (AD) is a malignant biphasic tumor with nests of sheets of epithelial cells surrounded by spindle cells with an osteofibrous component (WHO).
There is controversy whether OFD can progress to OFD-AD and AD. In a single-institution study [1], no patient with OFD (42 cases) or OFD-AD (10 cases f/u 36 to 316 months, average 97m) progressed to AD. In a large multicenter study [2], OFD-AD showed aggressive behaviour without metastatic potential; only one of the 117 OFD-AD patients developed classic AD over 39 years, remaining disease free at 5 years after treatment. Classic AD (190 cases) in this study showed full malignant potential with local recurrence, metastases (17%) and fatal outcome (13%). One more case [3] with transition from OFD-AD to AD between the age of 12 to 23 years was reported. Our case is presented to draw attention for the need of principally ‘open-end’ follow-up for patients with OFD-AD.

F *1975. At 6 years of age (1981) biopsy diagnosed OFD with AD-elements (OFD-AD). At age 20 years (1995) increasing anterior tibial bowing and overlength was treated by shortening and corrective osteotomy. Pathologic examination showed elements of OFD-AD. Imaging (MRI, X-Ray) remained unchanged until MRI 2020 showed osteolytic lesions within the preexisting changes, FDG-avid in PET-CT. Curetted material contained typical AD.
An extralesional resection of the involved diaphyseal tibial segment was performed with replantation after preparation and pasteurization to support the transposed pedicled ipsilateral fibula.

At present 12 months after resection/reconstruction the patient appears disease free with negative bone scan and improving bone remodeling.

Besides the pathologic ‘diagnostic etiquette’ understanding the biology in the spectrum of OFD to OFD-AD to AD is needed. This case is of special interest because of the long-term follow-up of an OFD-AD over 36 years and late transition into AD. The correct management between careful observation and resection appears mandatory but difficult.

1 Scholfield et al. Bone Joint J 2017; 99-B: 409-16
2 Schutgens et al. J Bone Joint Surg Am 2020; 102: 1703-13
3 Hatori et al. Tohoku J Exp Med 20