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A 63-year-old woman with acute thigh pain in the setting of subacute back, thigh pain
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A 63-year-old woman developed persistent, insidious, atraumatic low back and right posterior thigh pain. She was seen by her primary care physician and, in the absence of red flag symptoms, was diagnosed as having sustained a herniated lumbar disc with associated radiculopathy/sciatica. Plain radiographs of the lumbar spine were negative and given her lack of motor or sensory findings, no advanced imaging was ordered. She was treated with NSAIDs and prescribed a course of physical therapy. Despite compliance with treatment, her symptoms failed to resolve. She was subsequently treated with an escalating course of oral steroids, anti-spasmodic medication, and lidocaine patches. After at least 2 months of such treatment, her symptoms were severe enough to warrant a visit to the ED, although she was discharged home after evaluation by the emergency physician.
Her past medical history was significant for benign hypertension and gastroesophageal reflux. Her surgical history was significant for cataract extraction and biopsy of a benign breast mass. Home medications included amlodipine, lisinopril, pantoprazole, naproxen, lidocaine patches, diazepam, cyclobenzaprine and prednisone. She endorsed allergies to amoxicillin and trimethoprim-sulfamethoxazole. She was a non-smoker, abstained from alcohol and denied the use of illicit drugs.
On the day the patient presented to the outside-hospital ED, she described how her right foot slipped as she got out of the car. She did not immediately fall to the ground but twisted and felt a sudden pop and pain in her right leg. She was subsequently unable to weight bear on the right lower extremity and was transported to the ED.
On physical examination in the ED, she was afebrile with normal vital signs. Focused examination of the right lower extremity revealed significant right thigh pain with any movement or manipulation of the limb. The skin was without lesions, wounds, ecchymosis or erythema. Distal motor function was fully intact as was sensory function throughout the limb. Distal pulses were easily palpated.
Figure 2. Nuclear bone scan performed following a 3-hour delay demonstrates asymmetric increased uptake in the right proximal femur without additional sites of disease.
Figure 3. MRI shows a pathologic right subtrochanteric femur fracture with edema/hematoma that contaminates the surrounding muscle compartments down the length of the femur.
Source: A. Scot Brown, MD
Plain radiographs demonstrated a right subtrochanteric femur fracture (Figure 1). The patient was admitted to a medicine service with an orthopedic surgery consultation. However, subsequent review of the films by the consultant orthopedist and radiologist prompted concern for pathologic fracture given the presence of metaphyseal cortical irregularity. The patient was subsequently transferred to our level-1 tertiary care center with an associated National Cancer Institute Designated Center for definitive evaluation and management.
Additional history revealed no prior history of malignancy and appropriate cancer screening within the preceding 12-month period including mammography, pap smear and colonoscopy. Laboratory studies appropriate for a newly diagnosed bone lesion without known primary malignancy were ordered and revealed no abnormalities. CT of the chest, abdomen and pelvis revealed no other obvious sites of osseous or visceral malignancy nor metastasis. A bone scan was significant only for increased uptake in the right proximal femur at the site of the patient’s pathologic fracture (Figure 2). MRI revealed no lesions in the remaining femoral diaphysis, distal metaphysis or epiphysis; however, detailed evaluation of the proximal segment was confounded by the acute pathologic fracture (Figure 3). However, MRI demonstrated fracture hematoma contaminating all compartments of the thigh along the entire length of the femur.
Given no known or identified primary malignancy and no alternative lesions, CT-guided biopsy of the right proximal femur at the site of the fracture was performed by our colleagues in interventional radiology. This sample was reviewed by our experienced musculoskeletal pathologists, but it demonstrated extensive necrosis and was deemed nondiagnostic. The patient subsequently underwent open biopsy with the orthopedic oncologist providing definitive care through a lateral incision to maintain the possibility of subsequent limb salvage. Pathology review of the open biopsy was performed at our institution with a differential of high-grade spindle cell sarcoma vs. sarcomatoid carcinoma. After outside consultation with the NIH, the patient’s consensus preliminary diagnosis was high-grade spindle cell sarcoma.
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Dedifferentiated chondrosarcoma of the proximal femur with pathologic fracture
The patient is an otherwise healthy 63-year-old woman who had months of low-back and right lower extremity pain that was diagnosed as sciatica by her primary care physician. Despite her failure to respond to treatment and evidence of worsening symptoms, no imaging of the lower extremity was obtained, which likely would have demonstrated the cortical changes evident in the metaphysis at the time of her pathologic fracture. At the time this patient presented to our institution, her course was significantly complicated by pathologic fracture and contamination of all compartments of the right thigh along the entire length of the femur due to fracture hematoma. Given the involvement of multiple compartments, as well as the high-grade nature of her sarcoma, it was felt that limb salvage could not be safely performed nor negative margins obtained.
Following completion of her work-up, a lengthy and frank discussion was held with the patient regarding her diagnosis and treatment options; a shared decision was made to proceed with radical resection via hip disarticulation. Hip disarticulation was performed, and surgical margins were negative (Figure 4). Final surgical pathology revealed areas of well-demarcated differentiated chondrosarcoma at the periphery of the patient’s lesion in addition to the high-grade spindle cell sarcoma morphology and extensive tumor necrosis seen on prior CT-guided needle biopsy and open biopsy. The final diagnosis was revised to dedifferentiated chondrosarcoma.
Postoperatively, in consultation with our colleagues in medical oncology, given the patient’s apparent lack of metastatic disease on presentation and aggressive, high-grade pathologic diagnosis, the decision was made to treat her with adjuvant chemotherapy (adriamycin/cisplatin) to maximize disease control in an attempt to reduce the risk of recurrence/metastasis.
Chondrosarcoma is a malignant, primary sarcomatous neoplasm composed of chondrocytes. It typically presents in patients older than 40 years and is most commonly found in the pelvis, proximal femur, distal femur and scapula. In contrast to benign chondroid lesions, patients with chondrosarcoma often report pain. Additional signs and symptoms at presentation may be due to a mass effect that depends on the location of the tumor, such as bowel/bladder obstruction with pelvic chondrosarcoma.
Plain radiographs may demonstrate intralesional calcification/mineralization consistent with chondroid lesions, however chondrosarcomas are also distinguished by reactive cortical changes that include thickening and erosion. The patient in this case demonstrated such metaphyseal cortical changes which, in conjunction with a low-energy mechanism, alerted her initial physicians to the possibility of pathologic fracture and prompted them to transfer her to our facility. MRI is valuable for determining the extent of marrow involvement, as well as the size and location of the soft tissue component. Chondrosarcoma is typically hot on a bone scan, which provides valuable information regarding overall disease burden/sites of metastatic disease.
Dedifferentiated chondrosarcoma represents 10% to 15% of all chondrosarcomas and, in contrast to most chondrosarcomas, is a high-grade malignant tumor. The histologic appearance is typically bimorphic, as it was in this patient. Areas of lower-grade chondrosarcoma are interspersed with areas of high-grade sarcoma, which include osteosarcoma, malignant fibrous histiocytoma and spindle cell sarcoma.
Several small published series report a poor prognosis and difficulty in improving outcome despite intervention. As a result, management recommendations have largely focused on wide or radical resection of the tumor as was done in this patient. Obtaining negative margins with excision has been demonstrated to reduce local recurrence, however, unfortunately, this has not appeared to dramatically alter the overall reported survival. The role of chemotherapy in treatment of dedifferentiated chondrosarcoma remains controversial, with only one study suggesting this therapy improved survival.
This case is a critical reminder to clinicians to maintain a broad differential, particularly when initially benign symptoms worsen and fail to resolve. We must continually reassess presumptive diagnoses and maintain constant vigilance for uncommon, potentially catastrophic diagnoses.
- References:
- Campanacci M. Bone and Soft Tissue Tumors. 2nd Ed. Padova, Italy: Piccin Nuova Libraria; 1999: 319-333.
- Capanna R, et al. J Bone Joint Surg Am. 1988;70:60-69.
- Dickey ID, et al. J Bone Joint Surg Am. 2004;86-A:2412-2418.
- Frassica FJ, et al. J Bone Joint Surg Am. 1986;68:1197-1205.
- Grimer RJ, et al. Eur J Cancer. 2007;doi:10.1016/j.ejca.2007.06.016.
- Marco RA, et al. J Am Acad Orthop Surg. 2000;8:292-304.
- Mercuri M, et al. Skeletal Radiol. 1995;24:409-416.
- Mitchell AD, et al. J Bone Joint Surg Br. 2000;82:55-61.
- For more information:
- Michael C. Ciccotti, MD; and A. Scot Brown, MD, can be reached at Rothman Orthopaedic Institute at Thomas Jefferson University, 925 Chestnut St., 5th Floor, Philadelphia, PA 19107; Ciccotti’s email: michael.ciccotti@jefferson.edu; Brown’s email: scot.brown@rothmaninstitute.com.
- Edited by Michael C. Ciccotti, MD, and Michael C. Fu, MD, MHS. Ciccotti is a chief resident in the department of orthopaedic surgery at Thomas Jefferson University and Rothman Orthopaedic Institute and will be a sports medicine fellow at the Steadman Phillipon Research Institute in Vail, Colorado following residency. Fu is a chief resident at Hospital for Special Surgery and will be a sports medicine and shoulder surgery fellow at Rush University Medical Center following residency. For information on submitting Orthopedics Today Grand Rounds cases, please email: orthopedics@healio.com.
Disclosures: Ciccotti and Brown report no relevant financial disclosures.