A rare desmoid tumor arising from the manubrium
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CASE REPORT
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A rare desmoid tumor arising from the manubrium Chenyang Ye, Guofei Zhang and Ying Chai*
Abstract Desmoid tumors are rare soft tissue tumors derived from fascia and connective tissue of the muscular layers. The abdominal region is the most frequent site of involvement, whereas involvement of sternal manubrium is rare. We report the case of a rare desmoid tumor in the sternal manubrium mimicking radiological and metabolic features of malignant tumor, which was successfully treated by sternal resection and reconstruction with autogenous rib grafts. Keywords: Desmoid tumors, Manubrium, Sternal reconstruction, Autogenous rib grafts
Background Desmoid tumors are rare, benign, soft-tissue neoplasms, and are characterized by infiltrative growth and a tendency towards local recurrence. Common sites of desmoid tumors include abdominal wall, extremities, shoulder, neck, and chest wall. The sternum is the least common site. We herein report a rare desmoid tumor of sternal manubrium mimicking malignant tumor such as chondrosarcoma, which was successfully treated by complete resection of the tumor and immediate sternal reconstruction with autogenous rib grafts. Case presentation A 64-year-old man was referred to our hospital with bulging of the sternal manubrium. He was otherwise asymptomatic. There was no antecedent history of Gardner syndrome, trauma or surgery. Physical examination revealed a firm, 3× 3 cm palpable sternal mass. The contrasted computed tomography (CT) scan of the chest showed a 4× 4 cm mass based on the sternal manubrium, with no apparent involvement of the mediastinal structures (Figure 1A and B). 18F-fluorodeoxyglucose–positron emission tomography (FDGPET) revealed metabolically active areas around the manubrium, both sternoclavicular joints, and bilateral first sternocostal joints, which was suggestive of * Correspondence: [email protected] Department of Thoracic Surgery, the Second Affiliated Hospital, College of Medicine, Zhejiang University, No. 88 Jiefang Rd, Hangzhou 310009, China
malignant tumor of the sternal manubrium. The patient refused to undergo preoperative fine-needle aspiration of the lesion. Thus the patient underwent a radical en bloc resection of the tumor and sternal reconstruction with autogenous rib grafts. At surgery, an expansile lesion involving the entire manubrium, both sternoclavicular joints, and bilateral first sternocostal joints was found. En bloc radical manubriectomy was performed including portions of the bilateral clavicles and first ribs. The specimen was removed 4 cm beyond the margin of the lesion. Then through a left anterior lateral incision, about 10 cm of the left fifth rib was harvested from the subperiosteum and carved into two parts with preservation of intact periosteum and pleura. Slots were cut into the ends of the two rib clips, bilateral clavicles, and the edge of the sternum using an electric drill. The two rib grafts were placed to form a T shape between the edges of the residual sternum and bilateral clavicles using rib nails,
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