Case Report

1. Radiological Category: Muscoloskeletal Principal Modality (1): Principal Modality (2): General Radiography MRI Case Report Submitted by: Farhan Amanullah Faculty reviewer: Sandra Oldham, MD Date accepted: 28 September 2011

2. Case History This is a 44-year-old AA female with no relevant PMH who arrived to the ER at MHH complaining of near-syncope secondary to “intense pain” in her left thigh. Patient relates her problems to prior surgeries of childhood. The patient reports she had some falls when she was 7 years old while playing kick ball and fractured her left hip. At that time, they put plates and screws in her left hip. Later at about 12 years old, she slipped and fell in the shower and they put a rod into her left femur. At about 14 years old, she had a knock left knee and this was corrected surgically on her left proximal tibia surgery. Patient reports her left femur has given her problems for at least 3 months, where she could not stand or walk for prolonged periods of time and was unable to retain her job. She has been unemployed for three months.

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11. Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis of the leg lesion? • Osteomyelitis • Ewing’s Sarcoma • Chondrosarcoma • Osteosarcoma

12. Radiographs of the L femur and knee demonstrate severely eroded bone with apprx. 10 cm long gap of bare rod which is surrounded by a large ovoid soft tissue masslike prominence that measures 20 cm in craniocaudad dimension by 10 cm diameter.MR demonstrates 26 x 10 x 11 cm heterogeneous mass associated with the left mid and distal femur resulting in diffuse destruction of the surrounding bone. Multiple small areas of hemorrhage, cystic change, and fluid signal are present. Surrounding edema. No inguinal or pelvic lymphadenopathy. PET CT shows multiple “hot” areas of increased FDG uptake. Findings and Differentials Findings: Differentials: • Osteomyelitis • Ewing’s Sarcoma • Chondrosarcoma • Osteosarcoma

13. Osteomyelitis Ewing’s Sarcoma Chondrosarcoma Osteosarcoma Discussion Bone expansion, marked sclerosis, replacement of marrow fat with water secondary to edema, exudate, hyperemia, and bone ischemia. Usually develops during puberty, rare in adults. Radiographs show a long, permeativelytic lesion in the metadiaphysis and diaphysis of the bone, with a prominent soft-tissue mass extending from the bone. Older patients (MC >60 yo). Large osteolytic lesion with extension into soft tissues. Endosteal Scalloping. This is the diagnosis.

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19. Mixed lytic & sclerotic lesion Periosteal rxns common Codman’s triangle Calcification as well as soft tissue extension common Males affected more than females Common age group is 10-20 years old Discussion: Osteosarcoma

20. Location: Usually metaphysis of long bones 60% occur in distal femur/proximal tibia region 42% femur 19% Tibia 10% Humerus 15% pelvis 8% facial bones/cranium Discussion: Osteosarcoma

21. Presentation Swelling, pain around area of joint Natural History Persistent growth and early metastasis to lungs Prognosis 5-year survival rate = 15 – 20% when treated by resection of primary tumor (amputation) 5-year survival rate = 60% with adjunctive chemotherapy following resection Discussion: Osteosarcoma

22. An extremely lytic and destructive variant of classic osteosarcoma Same age group and location Accounts for 0.4-12% of all osteosarcomas The pathologic specimen is hemorrhagic, with microscopic evaluation demonstrating the presence of malignant-appearing stromal cells with giant cells Before aggressive multidrug chemotherapy, prognosis extremely poor Now same as prognosis for patients who have classic osteosarcoma and is treated with similar protocols Discussion: TelangiectaticOsteosarcoma

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28. Chronic bowing deformities and multiple bony lesions throughout skeleton.Several groundglass lesions with sclerotic peripheral marginsThe majority of these lesions demonstrate no increased uptake on a PET CT examination. Findings and Differentials Findings: Differentials: • Multiple Myeloma • Multiple Enchondromas • Polyostotic Fibrous Dyplasia • Metastasis

29. Most common primary malignant neoplasm of the skeletal system. The following 3 criteria makes the diagnosis unequivocal: A minimum 10-15% of a bone marrow aspirate demonstrates plasma cells Monoclonal immunoglobulins present in the urine or blood Radiographic survey demonstrates lytic lesions Discussion: Multiple Myeloma

30. Ollier disease - nonhereditary disorder characterized by multiple enchondromas with a predilection for unilateral distribution. Maffuci syndrome is nonhereditary and is less common than Ollier disease. This syndrome results also in multiple hemangiomas. The lesions replace normal bone with mineralized or unmineralized hyaline cartilage, thereby generating a lytic pattern on radiographs. More commonly, there are lytic areas containing rings and arcs of chondroid calcifications. Discussion: Multiple Enchondromas

31. Definition - a sporadic disorder characterized by the presence of one (monoostotic) or more (polyostotic) expanding fibrous skeletal lesions composed of bone-forming mesenchyme Discussion: Fibrous Dysplasia

32. Equal frequency in both sexes The monoostotic form is the most common and is usually diagnosed in patients between 20 and 30 years of age without associated skin lesions The polyostotic form typically manifests in children <10 years of age and may progress with age the lesions most commonly involve maxilla, other craniofacial bones, ribs, and metaphyseal or diaphyseal portions of the proximal femur or tibia. Expanding bone lesions may cause pain, deformity, fractures, and nerve entrapment Discussion: Fibrous Dysplasia

33. The lesions are typically well-defined, radiolucent areas with thin cortices and a ground-glass appearance Lesions may be lobulated with trabeculated areas of radiolucency Involvement of facial bones usually presents as radiodense lesions Discussion: Fibrous Dysplasia

34. Frequency of malignant change is increased in polyostotic forms, especially in patients with concomitant McCune–Albright syndrome. Rate of transformation ~ 4% with MAS and 0.5% with monostotic forms Common tumors: Osteosarcoma Fibrosarcoma Chondrosarcoma Patient’s usually in third or fourth decades of life Anatomic Sites: Craniofacial bones Femur Tibia Discussion: Fibrous Dysplasia

35. Polyostotic Fibrous Dysplasia with secondary malignant degeneration of left femoral lesion into Telangiectatic Osteosarcoma Diagnosis

36. Goldman, Lee. "Bone Tumors: Primary and Metastatic Bone Lesions." Cecil Textbook of Medicine. Philadelphia: Saunders, 2004. Print. Gerard, M. Doherty. Current Surgical Diagnosis and Treatment. 12th ed. [S.l.]: Mcgraw Hill Higher Educat, 2005. Print. Kantarjian, Hagop, Robert A. Wolff, and Charles A. Koller. The MD Anderson Manual of Medical Oncology. New York: McGraw-Hill, 2011. Print. Schwartz, Seymour I., and F. Charles. Brunicardi. Schwartz's Principles of Surgery. 9th ed. New York: McGraw-Hill, Medical Pub. Division, 2010. Print. Kattapuram TM, Treat ME, Kattapuram SV. Magnetic resonance imaging of bone and soft tissue infections. Curr Clin Top Infect Dis 2001;21:190-226Ekram T, Elsayes KM, Cohan RH, Francis IR. Computed tomography and magnetic resonance features of renal Ewing sarcoma. Acta Radiol, Nov 2008;49(9):1085-90 Durie BG, Salmon SE. A clinical staging system for multiple myeloma.Correlation of measured myeloma cell mass with presenting clinical features, response to treatment, and survival. Cancer, Sep 1975;36(3):842-54 References