Extraskeletal osteosarcoma with synchronous regional lymph node and soft tissue metastasis: a rare presentation of an un
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U P - T O D A T E REV I E W A N D C A S E REP O R T
Extraskeletal osteosarcoma with synchronous regional lymph node and soft tissue metastasis: a rare presentation of an uncommon tumour Ashish Gulia · Ajay Puri · Sumeet Jain · Bharat Rekhi · Sashikant Juvekar
Received: 17 April 2012 / Accepted: 3 October 2012 © Springer-Verlag France 2012
Abstract Extraskeletal osteosarcoma is a rare soft tissue neoplasm arising from mesenchymal elements with characteristic production of osteoid. These tumours are known to have high rate of distant metastasis and dismal prognosis. We present a rare case of extraskeletal osteosarcoma arising in medial aspect of knee joint with synchronous inguinal node and soft tissue metastasis. The patient was initially diagnosed as myositis ossiWcans at an outside institute and excised without prior imaging or biopsy. The patient presented to our hospital with recurrent lesion along with palpable inguinal lymph nodes and soft tissue metastasis in thigh. The patient underwent neoadjuvant chemotherapy and wide excision of the primary lesion, soft tissue metastasis and complete groin node dissection. We also discuss the importance of PET scan and intra-operative ultrasonography in the management of these lesions.
Introduction Extraskeletal osteosarcoma is a rare soft tissue neoplasm arising from mesenchymal elements and characterized by producA. Gulia (&) · A. Puri Tata Memorial Hospital, Room no: 43, Bone and Soft Tissue Services, E. Borges Marg, Parel, Mumbai, Maharashtra 400012, India e-mail: [email protected] S. Jain Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, India B. Rekhi Department of Pathology, Tata Memorial Hospital, Mumbai, India S. Juvekar Department of Radiology, Tata Memorial Hospital, Mumbai, India
tion of osteoid, with or without cartilaginous element. In a series documented from Memorial Sloan Kettering Cancer Centre, it constituted less than 1 % of all soft tissue neoplasm and about 4 % of all osteosarcomas [1]. Wilson [2] in 1941 reviewed 10 cases of various extraskeletal ossifying tumours and reported four cases of extraskeletal osteosarcoma. He concluded that mesenchymal elements in soft tissue are totipotent and have ability to diVerentiate into osteoid-producing cells in soft tissue. Hence, an extraskeletal osteosarcoma should be considered as one of the diVerential diagnoses of the osteoid-producing tumour of soft tissues [2]. Subsequently, there have been several publications about this rare neoplasm describing its demographics, clinical features, patterns of spread and prognosis [3–5]. Extraskeletal osteosarcoma is considered as an aggressive disease with high incidence of local and distant metastasis. The common sites of metastasis are lung, bones and lymph nodes [4, 5]. We present a rare case report of an extraskeletal osteosarcoma with synchronous regional lymph node and soft tissue metastasis. We also discuss the role of positron emission tomography-computed tomography scan (PET-CT) and intra-operative ultrasonography (USG) in the manageme
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