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.

Spindle cell hemangioendothelioma of the sacrum: case report.

Spindle cell hemangioendothelioma is a rare benign vascular tumor that is not known to involve the sacrum. The authors describe the case of a 31-year-old woman presenting with low-back and radicular pain without weakness or bowel or bladder dysfunction. Admission CT and MRI studies revealed a large S1-3 lytic sacral lesion. The patient initially underwent a nondiagnostic percutaneous biopsy. She subsequently underwent an open biopsy, during which the lesion was found to be highly vascular. Histological investigation revealed a vasoformative lesion consistent with spindle cell hemangioendothelioma. Preoperative embolization followed by resection via intralesional currettage resulted in resolution of symptoms up to 9 months postintervention. Despite the authors' recommendation, the patient became pregnant at that time and requested no additional follow-up imaging. The authors present the first reported case of a spindle cell hemangioendothelioma of the sacrum and review the current literature.

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.

Late complications and survival of endoprosthetic reconstruction after resection of bone tumors.

BACKGROUND: While complications following massive endoprosthetic reconstruction have been previously described, the incidence and effects of these complications over extended periods of time have not been well characterized in large series. QUESTIONS/PURPOSES: We therefore determined: (1) incidence and types of complications; (2) relative risk of complications; (3) likelihood of secondary complications; (4) whether modularity altered such complications; (5) implant failure and limb salvage rates and (6) implant survival over extended followup. METHODS: We retrospectively reviewed 232 patients (241 implants: 50 custom,191 modular) who underwent endoprosthetic reconstruction for malignant and aggressive bone tumors between 1980 and 2002. Complications were classified as infection, mechanical, superficial soft tissue, deep soft tissue, or dislocation. Survival was determined by Kaplan-Meier analysis. Minimum followup was 5 years (mean: 10 years; range: 5-27 years). RESULTS: One hundred thirty-seven of 232 patients (59%) underwent a single reconstruction. Ninety-five patients had 242 additional procedures. Forty-four revised patients retained their original prosthesis. Limb salvage rate was 90%; implant failure (removal of the cemented part) was seen in 29% (70/241) with a median survival of 190 months. Twenty-five of 50 custom implants failed (8 then failed again) while 30/180 modular implants failed (7 then failed again). Of 70 instances of implant failure, 38/70 were mechanical, 27/70 infectious. Risk of infection increased 30% after a second procedure; 16 of 24 amputations were performed because of infection. CONCLUSIONS: Mechanical complications were the most common cause of implant failure. Infection was the leading cause of both complication and amputation; risk of infection increased substantially with revision surgery. Modular implants had fewer mechanical complications, thus leading to fewer revisions and subsequent infections.

Osteosarcoma of the proximal tibia: limb-sparing resection and reconstruction with a modular segmental proximal tibia tumor prosthesis.

A limb-sparing resection was performed for osteosarcoma of the proximal tibia in a young patient. A special modular segmental proximal tibial endoprosthesis that includes a hinged total knee component was used for reconstruction. A medial gastrocnemius rotational flap was utilized to reconstruct the extensor mechanism of the knee. The muscle flap aids in covering the prosthesis and helps to protect against infection. Limb-sparing resection for tumors arising from the proximal tibia, and reconstruction with a modular segmental proximal tibia tumor prosthesis and gastrocnemius muscle flap, is a safe and reliable method for treating tumors involving this area.

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.

Implant design and resection length affect cemented endoprosthesis survival in proximal tibial reconstruction.

Endoprosthetic reconstruction of the proximal tibia continues to pose many challenges. A retrospective analysis of 44 consecutive patients who underwent cemented proximal tibial replacement were included to investigate if patient age, surgical stage, type of implant, stem diameter, or resection length could be associated with implant failure. Fifteen patients (34%) suffered prosthetic failure, 7 due to infection. Prosthetic-related complications occurred in 13 patients (30%). Custom design prosthesis and longer length of resection were significantly associated with prosthesis survival in a Cox regression analysis (P = .001, hazard ratio = 8.747 and P = .044, hazard ratio = 1.217, respectively). Cemented proximal tibial replacement offers a functional knee, but reducing risk of complications still remains challenging. Prosthetic design and length of resection affect overall cemented endoprosthesis survival.

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.

Metastatic carcinoma of the long bones.

Breast, prostate, renal, thyroid, and lung carcinomas commonly metastasize to bone. Managing skeletal metastatic disease can be complex. Pain is the most common presenting symptom and requires thorough radiographic and laboratory evaluation. If plain-film radiography is not sufficient for diagnosis, a bone scan may detect occult lesions. Patients with lytic skeletal metastases may be at risk for impending fracture. Destructive lesions in the proximal femur and hip area are particularly worrisome. High-risk patients require immediate referral to an orthopedic surgeon. Patients who are not at risk for impending fracture can be treated with a combination of radiotherapy and adjuvant drug therapy. Bisphosphonates diminish pain and prolong the time to significant skeletal complications.

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.

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.

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.