LAC + USC.

Transcription

1 Jeff McDavit,, M.D. LAC + USC mcdavit@usc.edu

2 Clinical History 55 year old male with large, deep, non- tender left thigh mass. Seen at LAC+USC Med Ctr FNA clinic No h/o trauma or radiation

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6 Vimentin + S-100, PanK, CD31 Dx: Malignant cells present, favor high-grade sarcoma, recommend biopsy for definitive diagnosis

7 Work-up and Clinical Course MRI 8x5cm thigh mass AIM chemotherapy x1 (pre-operative) Adriamycin (Doxorubicin) + Ifex (Ifosfamide)) + Mesna Post chemotherapy MRI 2 weeks later Increased size to 9.4cm = Failed chemotherapy

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11 Surgery Wide excision limb-salvage surgery Specimen Left thigh resection specimen Grossly well-circumscribed (10cm) Soft, variegated with hemorrhage and necrosis. Surrounded by skeletal muscle

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17 DIAGNOSIS??

18 DIAGNOSIS Extraskeletal Osteosarcoma,, high-grade grade All surgical margins negative T2bNXMO, Stage III

19 Extraskeletal Osteosarcoma A malignant neoplasm Cells have osteoblast phenotype Synthesis of osteoid/bone Located in the soft tissues No attachment to the skeleton including periosteum.

20 Extraskeletal Osteosarcoma 1-2% of all soft tissue sarcomas First described in 1941 by Smith Patients generally >40 years old Site: Thigh, pelvic and shoulder girdles Presents as an enlarging soft tissue mass Painful in about 1/3 rd of cases Usually present 6-8MO 6 from onset of symptoms Size at presentation: cm M:F = 1.9:1 Lee JS, et al. A review of 40 patients with Extraskeletal Osteosarcoma. Cancer 1995; 76:

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22 Extraskeletal Osteosarcoma Microscopic Findings Neoplastic osteoid and/or bone Wide variation in amount Foci of neoplastic cartilage common Different patterns Fibroblastic (most common) Resembles undifferentiated pleomorphic sarcoma (UPS) or MFH-like except with the presence of osteoid Chondroblastic may have better prognosis

23 Extraskeletal Osteosarcoma Subtypes Osteoblastic Very cellular, highly pleomorphic tumor cells, numerous mitoses, and lots of osteoid production. Chondroblastic Elsevier, Inc Weiss and Goldblum. Enzinger and Weiss s Soft Tissue Tumors, 5 th edition. Giant cell rich (osteoclast( osteoclast-like like giant cells) Telangiectatic (markedly dilated blood filled spaces lined by malignant tumor cells) Small cell pattern.

24 Extraskeletal Osteosarcoma Immunohistochemistry Vimentin + SMA, EMA, Desmin,, S-100 S main value is to exclude other malignant tumors with metaplastic osteoid/bone Synovial Sarcoma, Epithelioid Sarcoma, Melanoma, MPNST

25 Extraskeletal Osteosarcoma Differential Diagnosis Myositis Ossificans (Early stage) A benign reactive ossifying process in muscle Distinct Zonation Pattern - most important feature to distinguish it from EOS Central immature zone is highly cellular (often confused with EOS)

26 Myosistis Ossificans: Innermost Zone

27 Myositis Ossificans: Intermediate zone

28 Myositis Ossificans: Peripheral Zone

29 Extraskeletal Osteosarcoma Differential Diagnosis Myositis Ossificans Malignant tumors with metaplastic bone Parosteal Osteosarcoma Periosteal Osteosarcoma High-grade grade surface osteosarcoma

30 Extraskeletal Osteosarcoma Prognosis Poor Most patients die from metastatic disease Treatment Surgery Preoperative or postoperative multiagent chemotherapy Radiation Prognostic variables <5cm, chondroblastic subtype = better prognosis)

31 Continued Clinical History MRI 1/08 Interval development of irregular enhancing mass 3/08 re-resection resection showed recurrent disease Patient treated with radiation therapy

32 Parakrama Chandrasoma,, M.D. Nancy Klipfel,, M.D. Yanling Ma, M.D. Wesley Naritoku,, M.D., Ph.D. Sarka Cernocek,, M.D.

33 References Lee JS, et al. A review of 40 patients with Extraskeletal Osteosarcoma.. Cancer 1995; 76: Weiss SW, Goldblum JR. Enzinger and Weiss s Soft Tissue Tumors, 5 th edition. Mosby Elsevier, 2008: Varma DG, et al. MRI of Extraskeletal Osteosarcoma.. Journal of Computer Assisted Tomography 1993; 17(3) Okada K, et al. A low-grade extraskeletal osteosarcoma.. Skeletal Radiology 2003; 32: