PMC5889869_01
An anteroposterior radiograph of the right tibia and fibula obtained following curettage and bone grafting demonstrating a predominantly cortical-based expansile lesion involving the distal diaphysis consistent with adamantinoma.
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Due to an acute exacerbation of pain, radiographs of the right tibia and fibula were obtained demonstrating a predominantly cortical-based lobulated lesion of the distal tibial diaphysis with overlying soft tissue irregularity (Figure 1).
An axial computed tomography (CT) scan of the chest demonstrating a subpleural lesion (white arrow) identified at the time the adamantinoma was originally diagnosed. The lesion was biopsied due to concerns of metastasis, and the biopsy revealed benign fibrous lung tissue.
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Additionally, a CT scan of the chest revealed several small nodules in the left upper lobe of the lung which were felt to be consistent with metastatic disease; however, an open biopsy demonstrated only evidence of chronic pleuritis with fibrosis (Figure 2).
(a) Well-differentiated variants of adamantinoma including cords and islands of epithelial cells with a basaloid appearance, with some squamoid differentiation, were observed in this patient. (b) In other areas sampled, double rows of epithelial cells impart a tubular appearance or occur in single-cell cords (black arrows).
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A 19 cm section of the medial tibial shaft was resected along with a 20 x 5 cm section of the overlying skin and subcutaneous tissue down to the level of the anteromedial fascia, as this was assumed to have been contaminated from the original biopsy (Figure 3).
(a) A clinical image demonstrating the planned resection margins of the right lower extremity prior to the limb salvage procedure. The osteocutaneous flap from the contralateral extremity has also been marked out. (b) A postoperative image demonstrates that the medial osteocutaneous flap is well perfused. (c) Osteocutaneous free vascularized fibula flap. (d) The flap went on to incorporate well.
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The free vascularized osteocutaneous graft of the fibula was placed to fill the remaining defect of the right medial tibia adjacent to the previously placed allograft (Figures 4 and 5).
An anteroposterior radiograph of the bilateral lower legs following adamantinoma resection and limb reconstruction. On the right, the allograft is positioned laterally with the vascularized fibular graft placed overlying it medially. The grafts are fixed by plates and screws. On the left, the fibula has been resected and the ankle mortise has been stabilized proximally with a screw.
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The free vascularized osteocutaneous graft of the fibula was placed to fill the remaining defect of the right medial tibia adjacent to the previously placed allograft (Figures 4 and 5).
(a) A coronal T1-weighted magnetic resonance image showing a dumbbell-shaped mass in the anterolateral subcutaneous tissues involving the underlying extensor hallucis longus and extensor digitorum longus muscles. (b) The mass enhances diffusely after gadolinium administration, consistent with a soft tissue mass.
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MRI demonstrated a dumbbell-shaped mass measuring 5.4 x 1.7 x 2.1 cm in size arising in the soft tissues of the anterolateral aspect of the shin involving both the extensor hallucis longus and extensor digitorum longus muscles (Figure 6).
An AP and lateral view of the right ankle following a posterior-to-anterior tibialis tendon transfer, peroneal tenodesis, and subtalar fusion for an acquired foot drop resulting from resection of the soft tissue recurrence in the anterior compartment.
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The patient was left with a residual drop foot following the procedure and 6 months later underwent a subtalar arthroereisis and peroneal tenodesis along with a posterior tibialis tendon transfer to anterior tibialis tendon transfer (Figure 7).
(a) A coronal fat-suppressed T1 image with gadolinium enhancement demonstrating a mass in the right groin with an irregular rim of peripheral enhancement. This mass was excised and found to be a metastasis of the adamantinoma to an inguinal lymph node. (b) The epithelial component of the metastasis regionally imparts a pseudopapillary appearance, with intervening hemorrhage.
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Thus, the patient underwent an excisional biopsy of the lymph node that revealed a metastasis of the adamantinoma (Figure 8).
Computed tomography scans of the chest, abdomen, and pelvis demonstrating multiple lesions including a hypodense lesion of the spleen, bilateral kidney lesions, and a large cystic lesion with mural nodularity in the right lower lobe of the lung. The image on the top right demonstrates the lesion that was found to be chromophobe-type renal cell carcinoma in the upper pole of the left kidney, and the image on the bottom right shows the large metastatic adamantinoma lesion in the right lung.
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The patient subsequently had a CT of the chest, abdomen, and pelvis performed which revealed lesions in her bilateral kidneys and spleen and a large lesion in the right lower lobe of the lung (Figure 9).
A hematoxylin and eosin stain demonstrating representative histomorphology of the incidentally diagnosed chromophobe-type renal cell carcinoma.
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Pathology revealed that the right kidney and splenic lesions were both benign cysts, but the left kidney was stage T1A chromophobe-type renal cell carcinoma (chRCC), a second primary cancer, with microscopic positive margins (Figure 10).