1. Case Presentation December 19, 2007

2. 21 y.o. male CC: Right leg pain HPI: 1 year ago had surgery for a “tumor” on right leg. “Replaced my shin bone. But I don’t remember what they called it.”

3. PMH/PSH: as above NKDA Meds: None SHx: denies ETOH, TOB or illicit drug use, recently discharged from the Navy, swims for exercise, BA in nursing but currently on disability FHx: N/C

4. Pertinent Positives: –Well-healed scars on right shin, including skin graft site mid-shaft –Intact neurovascular exam Imaging:

6. Plain XR

7. Allograft Nonunion 8 – 17% reported Lack of host-donor junction @ 1 year Union = gap obliterated or bridging bone @ 3 or greater cortices on AP and Lateral imaging Worse outcomes if: –Adjuvant chemotherapy –Adjuvant radiation –Infection – usually occurs in 1 st year –Fracture – usually occurs in first 3 years –Original disease stage II or III (MTS) –Allograft is part of arthrodesis procedure

8. Allograft Nonunion Retrospective review 945 allograft patients: –163 nonunion (17.3%)  No chemo, no XRT  11.3%  XRT only  18%  Chemo  27% –162 revised:  47% required further procedures for nonunion  49 ultimate failures required metallic prosthesis, replacement of allograft or amputation Menken HJ et al, CORR 2001; 382: 87-98.

9. Allograft Nonunion 718 patients observed greater than 2 years: –75% retained and successful for >20 years –17% nonunion –11% infection –19% fracture –6% unstable joint Of the failed allografts: –>85% of the failures due to infection, fracture, recurrence of primary tumor –Susceptibility to infection primarily in 1 st year –Susceptibility to fracture in first 3 years Menken HJ et al.; CORR, 1996; 324:86-97.

13. Adamantinoma 0.4 - 1% of all primary bone tumors 2 nd & 3 rd decade; range 3-86 yrs Pain, swelling in adolescent or young adult 85 – 90% tibial shaft –10% of these  ipsilateral fibula –Femur, humerus, ulna, radius, hands/feet

14. Adamantinoma Mets in 12-29% –Lung, bone, regional lymph nodes –Mortality 13-18% –Survival with metastatic disease ~ 13 yrs Risk factors: –Intra-lesional treatment –Male –Short duration of symptoms –Young age on presentation (<20 yrs) –Lack of squamous differentiation of tumor

15. Adamantinoma - XR Eccentric, cortical, diaphyseal, long bone Sclerotic edge  slowly growing lesion Slight expansion of cortex with thinning Cystic or multiloculated appearance Lack of periosteal reaction, even in presence of extensive cortical destruction Two most characteristic features: –Location in tibia –Intracortical involvement

16. Adamantinoma MRI: –T1 – isointense, enhances with gadolinium –T2 – hyper-intense

17. Histology -Basiloid cells, pseudoglandular pattern and peripheral palisading, characteristic of an adamantinoma

18. MTS System Stage Grade* Site† Metastasis‡ IA G1T1 M0 B G1 T2 M0 IIA G2 T1 M0 B G2 T2 M0 III G1 or G2 T1 or T2 M1 *G1 = low grade and G2 = high grade. †T1 = intracompartmental and T2 = extracompartmental. ‡M0 = no regional or distant metastasis and M1 = regional or distant metastasis.

19. AJCC Staging System StageA/BSizeNodeMetastasesGrade IABAB </= 8 cm > 8 cm None N0None M0G1-2 (Low) IIABAB </= 8 cm > 8 cm None N0None M0G2-3/G3-4 (High) IIIAnyNone N0None M0G2-3/G3-4 (High) IVABAB AnyNone N0 Present N1 M1a M0 or M1b Any