Unusual presentation of metastatic osteosarcoma as a giant duodenal polyp. A case report

INTRODUCTION: Osteosarcoma is a malignant bone neoplasm with an usually high metastatic potential. Besides the common metastatic sites such as lungs, bone, and pleura, metastases to unusual sites such as liver, brain and regional lymph nodes have also been reported with increasing frequency; among them, gastrointestinal metastases represent an extraordinarily rare event in the natural history of this neoplasia. MATERIALS AND METHODS: We describe a case of a 27 year old man, who was diagnosed with a grade IV osteoblastic osteosarcoma of the left tibia and submitted to 5 courses of pre-surgical chemotherapy; later he underwent tibial resection with implantation of a prosthesis, followed by 2 further courses of adjuvant chemotherapy. Five years after the patient presented with melena and acute anemia; during endoscopic examination, a large bleeding duodenal polyp was found, so a surgical resection of the gastric antrum, duodenum, head of the pancreas, main bile ducts and gallbladder was performed. The surgical specimen was formalin fixed and paraffin embedded, and sections obtained by the blocks were stained with haematoxylin-eosin; immunohistochemical and ultrastructural analysis were performed. RESULTS: Microscopically, the tumor mass showed a mostly fasciculated architecture, composed of spindle and epithelioid cells in a scarce fibromyxoid stroma, featuring large areas of coagulative necrosis and small foci of sclerohyalinosis. Tumor cells featured large vesciculous nuclei, with a few prominent nucleoli; no foci of osteoid matrix were detectable. The ultrastructural analysis revealed small calcified electron-dense depots both in the perinuclear cytoplasm and in the extracellular collagen matrix compatible with an "early osteoid formation". Due to alteration of the natural history of the tumor induced by multiagent chemotherapy, the rate of metastases of osteosarcoma to unusual sites has been increasing. We report the 9th case of a gastrointestinal metastasis of osteosarcoma reported thus far, and only the second one arising in the duodenum. Both the histological features and the immunohistochemical findings were not suggestive for osteosarcoma metastases because the tumor appeared dedifferentiated; in our case the combination of electron microscopy and clinical history played a pivotal role to establish the final diagnosis.