Abstract
Sarcomas are rare tumours, accounting for 1% of all malignancies. They are histologically diverse, presenting with more than 50 different subtypes. Sarcoma can be broadly divided into two categories—soft tissue sarcoma and bone sarcoma. Extraskeletal osteosarcoma is a unique subtype which is histologically similar to bone osteosarcoma but resides within soft tissue and has no attachments to bone. We present a rare case of a patient who initially presented with an extraskeletal osteosarcoma that was complicated by a chronic chest wall sinus and lymphedema of the left arm. He later developed enlarging left chest wall nodules at the same region that were proven to be dedifferentiated liposarcoma on biopsy 20 years later. We examine the occurrence of both extremely rare diseases of differing pathologies over the same site but separated by an extraordinarily long interval, and possible new associations to account for this phenomenon.
Highlights
Sarcomas are rare and uncommon tumours, representing 1% of adult malignancies [1]
We present a rare case of a patient who initially presented with an extraskeletal osteosarcoma that was complicated by a chronic chest wall sinus and lymphedema of the left arm
Extraskeletal osteosarcomas are different from its primary counterpart of osseous origin—it occurs rarely in the younger population, and are poorly responsive to chemotherapy, with local and distant failures in more than 80% of those affected
Summary
Sarcomas are rare and uncommon tumours, representing 1% of adult malignancies [1]. They are a heterogeneous group of tumours of mesenchymal origin that can occur anywhere in the body. Extraskeletal osteosarcoma is a unique subtype of sarcoma, presenting with similar histology to that of osteosarcoma but lies within soft tissue and is not attached to any bones [2]. It represents approximately 1% - 2% of all soft tissue sarcomas, and behaves aggressively, carrying mortality rates up to 80% [3]. We report an interesting case of a patient who underwent resection for extraskeletal osteosarcoma 20 years ago and recently presented with a dedifferentiated liposarcoma at the same site
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