Adult Soft-Tissue Sarcomas of the Extremities.

➤: Soft-tissue sarcomas (STS) in adults comprise a heterogeneous group of tumors of mesenchymal origin that share similar biological patterns of local tumor growth and metastatic dissemination. ➤: The judicious use of imaging studies, biopsy techniques, and pathological evaluations is essential for accurate diagnosis and for planning treatment strategy. ➤: Wide local resection and radiation therapy form the cornerstone of management of high-grade STS. The role of adjuvant radiation therapy is questionable in the management of small (≤5 cm) superficial lesions that can be resected with negative margins. Chemotherapy given to patients who have nonmetastatic, high-grade STS results in varying benefit in terms of local tumor control and overall survival. ➤: Coordinated care by a multidisciplinary team of trained surgeons, medical oncologists, radiologists, radiation oncologists, and pathologists has been documented to improve local tumor control and the overall survival of patients with STS. ➤: Although considerable progress in tumor diagnostics and targeted therapies has been made over the past 2 decades, recommendations and controversies relating to tumor imaging and margins of resection have not changed.

Giant cell tumor of the pes anserine bursa (extra-articular pigmented villonodular bursitis): a case report and review of the literature.

Pigmented villonodular synovitis (PVNS) is a rare, benign, proliferating disease affecting the synovium of joints, bursae, and tendon sheaths. Involvement of bursa (PVNB, pigmented villonodular bursitis) is the least common, and only few cases of exclusively extra-articular PVNB of the pes anserinus bursa have been reported so far. We report a case of extra-articular pes anserine PVNB along with a review of the literature. The lesion presented as a painful soft tissue mass in the medial part of the proximal leg. A magnetic resonance imaging showed areas of low to intermediate signals in all sequences and the lesion enhanced heterogeneously with contrast. Diagnosis was confirmed by an incisional biopsy, and an intralesional resection was performed. The postoperative course was uneventful, and the patient is free of disease with no functional deficit at 2 years followup. As with other rare lesions, clinical and radiographic findings in addition to histological examination are essential for correct diagnosis.

Tumors of the foot and ankle: experience with 153 cases.

BACKGROUND: Both primary and metastatic tumors in the foot and ankle have been reported as rare. The purpose of this study was to describe 153 cases of foot and ankle tumors from a 20-year experience in a tertiary referral center specializing in orthopaedic oncology. It is the largest reported series of both bone and soft tissue tumors in the foot and ankle. MATERIALS AND METHODS: Between 1986 and 2006, a retrospective chart review was performed of a total of 2,660 tumors surgically treated in all anatomic sites by a single surgeon at a musculoskeletal tumor referral center. RESULTS: One hundred fifty-three patients (5.75%) with bone and/or soft tissue tumors of the foot and ankle were treated. There were 84 women and 69 men. The patients' ages ranged from 1 to 84, with a median age of 30 and mean of 33.2. The tissue types included 80 soft tissue and 73 bone tumors. Overall, 60 (39.2%) were malignant, and 93 (60.8%) were benign. The most common diagnosis was giant cell tumor. In addition, giant cell tumor was the most common bone tumor, while pigmented villonodular synovitis and giant cell tumor of the tendon sheath were the most common soft tissue tumors. CONCLUSION: The incidence of tumors of the foot and ankle in this series of a single surgeon over a 20-year practice was 5.75%. The results of this study reaffirm that awareness, correctly diagnosing, and appropriately treating or referring to an orthopaedic oncologist may help with an improved outcome for patients.

Space sarcomas: extra compartmental soft tissue tumors of the lower extremities a systematic approach to sarcomas of the femoral triangle, sartorial canal, and popliteal space.

BACKGROUND: Extra-compartmental soft tissue sarcomas may grow in virtual spaces in close proximity to major neurovascular structures and thus, require a complex resection. We analyzed the general principles by which these resections are planned. METHODS: We retrospectively analyzed 53 patients with sarcomas located in the femoral triangle (15), sartorial canal (16), and the popliteal fossa (22). These lesions were grouped into three categories based on involvement of spatial structure; neurovascular involvement = 13, musculofascial involvement = 19 and no involvement = 11. RESULTS: Limb sparing surgery was feasible in lesions that had either no structural or musculofascial involvement. Amputation, however, was required in 3 of 13 patients with neurovascular involvement because of gross involvement of the surrounding tissues. Overall, limb sparing was feasible in 94% (50 of 53 patients). The 2- and 5-year local recurrence rates were 10% and 14%, respectively. Five-year survival was 88%. CONCLUSIONS: Limb sparing resection of space sarcomas is feasible in the majority of extra-compartmental sarcomas by utilizing a systematic approach which emphasizes specific planes of resection.

The anterior popliteal approach for popliteal exploration, distal femoral resection, and endoprosthetic reconstruction.

Distal femoral resection and endoprosthetic reconstruction are sometimes associated with flap necrosis and inadequate soft tissue coverage. We evaluated the anterior popliteal surgical approach, which was designed to reduce those complications by using a posteromedial myocutaneous flap based upon the vastus medialis. A retrospective analysis of 46 consecutive patients was performed, and results were compared with historical controls. Compared with 19.4% with wound complications and 22.7% with gastrocnemius flap transfers in previous series by the senior author, 7.8% of patients in the present study had minimal superficial flap necrosis, and no gastrocnemius transfers for soft tissue coverage were required. The median Musculoskeletal Tumor Society score was 26, and the local recurrence rate 2 years or more after resection of osteosarcoma was 4%. The anterior popliteal approach to the distal femur limited wound complications and provided good soft tissue coverage of the endoprostheses.

'Telangiectatic' transformation in soft tissue sarcomas. a clinicopathology analysis of an aggressive feature of high-grade sarcomas.

BACKGROUND: 'Telangiectatic' change, which contains a large fluid hemorrhagic component, occurs in a variety of high-grade soft tissue sarcomas. METHODS: In a retrospective database review, we identified 20 consecutive patients (3%) with 'telangiectatic' change in soft tissue sarcomas. RESULTS: Tumors were located in the thigh (55%), shoulder (15%), calf (15%), upper arm (10%), and buttock in one patient. All 20 tumors were high grade. Histological diagnoses were MFH (40%), leiomyosarcoma (15%), synovial sarcoma (10%), and one each of seven other sarcomas (35%). Tumor size was often large-more than 10 cm (35%), between 5 and 10 cm (60%), and less than 5 cm in one case. A history of contusion to the tumor site followed by swelling was recorded in 30% of patients and 80% presented with a painful mass. On MRI imaging, 60% of tumors appeared to contain more than 50% blood, 50% had a hemosiderin-laden rim, and 55% had well-defined tumor nodules within the wall of the hematoma. Limb-sparing surgery was carried out in 90% of patients, the other 10% underwent primary amputation. The 5-year, event-free survival rate was 30%. Of the patients, 15% presented initially with metastatic disease; in 53%, it developed within 2 years of diagnosis. The overall local recurrence rate was 30%. CONCLUSIONS: Telangiectatic transformation in soft tissue sarcomas is a rare feature of aggressive high-grade soft tissue sarcomas and is unique in its clinical presentation, MRI characteristics, pathological pattern, and a tendency for a worse-off prognosis.

Functional reconstruction of the extensor mechanism following massive tumor resections from the anterior compartment of the thigh.

BACKGROUND: The authors describe the surgical technique and functional results of extensor mechanism reconstruction after major resections of soft-tissue tumors from the anterior compartment of the thigh. METHODS: Between 1983 and 2003, 78 patients with soft-tissue sarcomas of the anterior compartment of the thigh were operated on at the authors' institution. Sixteen patients with extensive resections of the quadriceps muscle and one patient with a complete resection of the femoral nerve underwent functional extensor mechanism reconstruction. Depending on the type of resection, one or more of the following muscles were used for reconstruction: the sartorius, biceps femoris, semitendinosus muscles. After two patients with a follow-up of less than 12 months were excluded, 15 patients were evaluated. The authors performed a retrospective analysis of these patients' medical records, with an emphasis on functional outcome. RESULTS: According to the Musculoskeletal Tumor Society score, functional results were estimated to be good to excellent in 13 patients and fair in two. Most patients had a satisfactory active range of motion, and muscle strength and functional results correlated with the extent of resection. CONCLUSION: The authors recommend functional muscle transfer reconstruction after extensive resection of the quadriceps muscle or resection of the femoral nerve with one or more of the following muscles: the sartorius, the biceps femoris, and the semitendinosus muscles.

Large tumors of the axilla: limb-sparing resection versus amputation in 27 patients.

Tumors of the axilla are rare and pose a surgical challenge because they are usually large at presentation and in close proximity to the major neurovascular bundle of the upper extremity. The use of detailed preoperative evaluation studies and extensile surgical exposure for these tumors enabled us to determine tumor resectability and proceed with a safe resection or perform an amputation when required. We retrospectively reviewed 27 patients who underwent resection of an axillary tumor from 1989 to 2004 and analyzed their presenting symptoms, results of preoperative studies, type of surgery, and functional outcome. Tumors were exposed using a utilitarian shoulder approach that revealed no tumor invasion of the neurovascular bundle in 19 patients and invasion in eight. The former group was treated with tumor resection and the latter with forequarter amputation. Neurologic deficit, limb edema, and angiographic observation of arterial narrowing were associated with amputation. Good function was achieved in 15 of 19, shoulder range of motion was preserved in 14 of 19, and local tumor control was maintained in 16 of 19 patients who underwent a limb-sparing resection.

The risk for fractures after curettage and cryosurgery around the knee.

Curettage and cryosurgery have been used successfully for treatment of benign locally aggressive and some low-grade malignant bone tumors. After treating these lesions, we reconstruct residual bone defects around the knee with cement, intramedullary pins, and autogenous bone graft for subchondral augmentation and closure of cortical windows. We questioned the incidence of fractures and the rates of nonunion and malunion and asked whether patients at risk for fractures can be identified. We conducted chart and radio-graphic reviews of 60 consecutive patients who had curettage and cryosurgery of primary bone lesions in the distal femur or proximal tibia. Ten of the 60 patients (17%) sustained postoperative intraarticular fractures. Patients who sustained fractures had (1) more freeze-thaw cycles; (2) metaphyseal defect ratios greater than 0.6 and 0.8 on the anteroposterior and lateral projections, respectively; and (3) 4 mm or less proximity of the defect to the joint. Only one fracture united in good alignment. Radiographic measurements can assist in identifying patients at risk for fractures after curettage and cryosurgery around the knee. We expect the fracture rate to decline by reducing the number of freeze-thaw cycles and improving our reconstruction method.

Popliteal sarcomas: presentation, prognosis, and limb salvage.

Soft tissue sarcomas of the popliteal fossa are rare diseases. Resection is challenging because of their extracompartmental location and proximity to neurovascular structures. Their prognosis is inferior to intracompartmental extremity soft tissue sarcomas. We ascertained the mode of initial presentation, the rates of local recurrence and distant metastasis, and the morbidity and complications of limb salvage procedures. We retrospectively analyzed the data of 29 consecutive patients operated on between 1989 and 2003. The median followup was 79 months. Sixteen, five, and eight patients were diagnosed with high-, intermediate-, and low-grade tumors, respectively. High-grade tumors were smaller than low- and intermediate-grade tumors. Thirty-one percent of patients presented with localized pain. Of the 26 patients who had primary limb salvage resections, 14 had negative surgical margins, six had close margins, and six had positive margins. Seventeen patients were treated with radiotherapy and 10 patients received chemotherapy. The local recurrence rate was 10.3%, four patients with high-grade tumors had distant metastasis, and the limb-salvage rate was 86.2%. Postoperatively, six patients experienced wound dehiscence and four had mild flexion contractures. Soft tissue sarcomas of the popliteal fossa have an atypical presentation. Limb salvage can be accomplished in most patients with low morbidity and good systemic and local control.

Is scapular endoprosthesis functionally superior to humeral suspension?

Humeral suspension was the most popular reconstructive procedure after total scapulectomy until the early 1990s. Since 1992 the senior author has been performing scapular endoprosthetic reconstruction after total scapulectomy whenever the rhomboids, latissimus dorsi, deltoid, and trapezius were preserved. We hypothesized that scapular endoprosthetic reconstruction resulted in better functional and cosmetic results than humeral suspension. From 1979 to 2003, 32 consecutive patients had total scapulectomies. Reconstructions included humeral suspensions in 16 patients and scapular endoprostheses in 16 patients. Functional and cosmetic results were compared retrospectively between the two groups. Patients with scapular endoprostheses had better functional results and superior cosmesis as compared with patients with humeral suspension. The mean Musculoskeletal Tumor Society scores for patients with scapular endoprostheses and humeral suspensions were 78.5% and 58.5% respectively. Seven patients with scapular endoprostheses had greater than 40 degrees abduction and 11 patients with humeral suspensions could not abduct the shoulder greater than 20 degrees. Twelve patients with humeral suspensions and none with scapular endoprostheses wore shoulder pads or customized orthoses for cosmetic purposes. After total scapulectomy, scapular endoprosthetic reconstruction led to better functional and cosmetic results than humeral suspension and therefore we recommend performing this reconstructive procedure whenever the rhomboids, latissimus dorsi, deltoid, and trapezius are preserved.

Two incision synovectomy and radiation treatment for diffuse pigmented villonodular synovitis of the knee with extra-articular component.

Diffuse pigmented villonodular synovitis (PVNS) of the knee is a rare, locally aggressive disease. Since 1995 the senior author has been treating patients with diffuse intra- and extra-articular PVNS of the knee with a bimodality treatment protocol, consisting of anterior and posterior open synovectomies in conjunction with external-beam radiation. The purpose of this study was to describe the surgical technique, evaluate our protocol's efficacy in limiting local recurrences, and assess its functional implications and associated morbidities. The medical records of nine consecutive patients were analyzed. Functional results were measured using the Knee Society score. The mean follow-up was 67 months (range 37-103 months). Eight patients were disease free and one patient had local recurrence, which was extra-articular, localized and non-progressive. The mean knee rating and functional rating had significantly improved from 65.2 and 70 preoperatively to 93.7 and 96.6 postoperatively respectively. Maximal knee flexion ranged from 90 degrees to 130 degrees, and only 3 patients developed flexion contractures of 5 degrees. In conclusion our bimodality protocol of anterior and posterior open synovectomies, in conjunction with external-beam radiation, limited the recurrence of diffuse intra- and extra-articular PVNS of the knee. All patients showed good-to-excellent functional outcomes and were able to return to their previous level of activity.

Knee stability after resection of the proximal fibula.

Managing tumors of the proximal fibula may require en bloc resection of the fibular head with the attachment site for the lateral collateral ligament. These resections of the proximal fibula cause unavoidable knee instability. We describe a reconstructive technique intended to minimize that instability. We retrospectively reviewed 24 patients who had proximal fibular resections from 1987 to 2004 and analyzes their knee stability and functional outcome. Resections were less (Type I) or more (Type II) radical depending upon the tumor type. Reconstruction included stapling the lateral collateral ligament to the lateral tibial metaphysis, cast immobilization, and protected weightbearing for 3 weeks. MSTS function scores were available for 19 of the 24 patients. At their most recent followup, 20 patients had a stable knee, three had 1 to 5 mm lateral joint space opening, and one had 6 to 10 mm lateral joint space opening. Patients with Type I resection had a better stability and function than those with a Type II resection. Stapling the lateral collateral ligament was a reliable technique for reconstructing the lateral collateral ligament after resecting the proximal fibula.

Vacuum-assisted wound closure after resection of musculoskeletal tumors.

UNLABELLED: Resection of musculoskeletal tumors may result in large soft tissue defects that cannot be closed primarily and require prolonged dressing changes and complex surgical interventions for wound coverage. We retrospectively reviewed 23 patients with such defects treated with a vacuum-assisted wound closure system and compared the outcome of these patients with a control group. The study group included 15 women and eight men who had their wounds located at the back (two), pelvic girdle (11), thigh (eight), and leg (two). Treatment included sealed wound coverage with polyurethane foam and overlying tape connected to a vacuum pump. This system was disconnected and changed every 48 hours for 7 to 19 days, after which all defects were reduced in size by an average of 25% and covered with a viable granulation tissue. This allowed primary closure in seven patients, primary closure with skin grafting in 14 patients, and healing by secondary intention in two patients. Compared with the control group, patients in the study group had shorter hospital stays and number of surgical interventions and greater rates of primary wound closure. The use of vacuum-assisted wound closure facilitates wound healing and primary wound closure in patients who have a large soft tissue defect after resection of a musculoskeletal tumor. LEVEL OF EVIDENCE: Therapeutic study, Level III (retrospective comparative study). See the Guidelines for Authors for a complete description of levels of evidence.

Function after resection of humeral metastases: analysis of 59 consecutive patients.

UNLABELLED: Metastatic bone disease of the humerus may require surgery for treatment of an impending or existing pathologic fracture or for alleviating disabling pain. Prompt restoration of function is a main goal of surgery, although published results do not reveal if that goal is being met. We retrospectively reviewed range of motion and function of 59 patients operated on from 1986-2003 for those indications. After resection, tumors around the humeral head and condyles (n = 20) were reconstructed with a prosthesis, and tumors at the humeral diaphysis (n = 39) were reconstructed with cemented nailing. Each patient's range of motion was recorded, and functional outcome was evaluated according to the American Musculoskeletal Tumor Society system. Patients who had cemented nailing had better shoulder motion, hand positioning, lifting ability, and emotional acceptance than patients who had endoprosthetic reconstruction. Pain alleviation and dexterity were comparable in both groups. All patients had a stable extremity, and the overall function of 56 patients (95%) was greater than 68% of normal upper extremity function. An aggressive surgical approach in patients with humeral metastases who met the criteria for surgical intervention was associated with good function. LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series--no, or historical control group). See the Guidelines for Authors for a complete description of levels of evidence.

Telangiectatic osteosarcoma: radiologic-pathologic comparison.

PURPOSE: To describe the imaging characteristics of a large series of telangiectatic osteosarcomas with pathologic findings for comparison. MATERIALS AND METHODS: The authors retrospectively reviewed 40 pathologically confirmed telangiectatic osteosarcomas. Patient demographics and images from radiography (n = 36), bone scintigraphy (n = 17), angiography (n = 4), computed tomography (CT) (n = 25), and magnetic resonance (MR) imaging (n = 27) were evaluated by three authors in consensus for lesion location, size, and intrinsic characteristics. There were 27 men (68%) and 13 women (32%) in the study, with an age range of 4-83 years (mean age, 24 years). RESULTS: Lesions frequently affected the femur, tibia, and humerus. Radiographs showed geographic bone lysis, a wide zone of transition, and matrix mineralization. CT demonstrated low attenuation, MR demonstrated high signal intensity on T2-weighted images, and both demonstrated hemorrhage, which simulated the appearance of aneurysmal bone cyst. Viable sarcomatous tissue surrounding hemorrhagic and/or necrotic regions was best seen at contrast material-enhanced CT and MR imaging, with thick peripheral, septal, and nodular enhancement in all cases. Subtle matrix mineralization in this viable tissue was best seen at CT. An associated soft-tissue mass was also seen in 19 of 25 cases (76%) at CT and in 24 of 27 cases (89%) at MR imaging. CONCLUSION: CT and MR imaging findings of telangiectatic osteosarcoma often include thick nodular tissue (and matrix mineralization at CT) in a largely hemorrhagic and/or necrotic osseous lesion with an associated soft-tissue mass, which allows distinction from aneurysmal bone cyst.