The biopsy is the most important aspect of the diagnostic workup, allowing for histologic classification and in turn both prognostication and treatment planning. Unlike many carcinomas in which a fine-needle aspiration can be used to identify cytologic aberrations, sarcomas often require a much larger sample for proper diagnosis. The larger sample is needed to appreciate tissue architecture, staining patterns, and infiltration into surrounding tissue, as well as to appreciate subtle differences between different areas within the tumor. While an open biopsy has slightly higher diagnostic yield, a core needle biopsy performed by an experienced clinician participating in a multidisciplinary sarcoma service can often obtain adequate tissue, and this technique is widely accepted. Alternatively, there is an increasing need for tissue samples in order to pursue basic science and translational research endeavors. Toward this end, an open biopsy permits for collection of larger samples and should be considered if tumor banking is feasible.

Regardless of whether an open or a core needle technique is utilized, the biopsy should be performed in accordance with accepted tenets of sarcoma management. Specifically, esmarch use is contraindicated, though elevation of the limb and use of a tourniquet is permissible. The biopsy tract should be maintained within a single compartment to facilitate complete excision a later time. Neurovascular structures need to be avoided. The biopsy should be performed either by the same individual who will ultimately perform the definitive surgery or by a sarcoma team member after discussion with the surgeon regarding the panned surgical approach. The biopsy should be placed in line with the planned definitive resection incision in order to allow for resection of the biopsy tract. The incision should be extensile, which in the extremity is almost invariably longitudinal. Incisions with transverse, oblique, curvilinear, and other complicated patterns should be avoided with rare exception. Careful attention to hemostasis is essential to avoid hematoma formation, local contamination, and subsequent tumor spread. Drain placement, when required, should be close to and in line with the incision, and similarly, sutures should be close to the skin edges to facilitate complete resection of all involved tissue at the time of definitive resection. It is important to obtain tissue for permanent pathology, cytogenetics, and, in many instances, cultures. Additionally, the biopsied lesion should be reviewed under frozen section at the time of biopsy in order to confirm that representative tissue has been obtained. While some diagnostic information can be gleaned from the frozen section evaluation, its accuracy is recognized as inferior to that of permanent paraffin-embedded histologic review (Athanasian 2002). For this reason, it is often preferred to defer treatment until after permanent slides have been assessed and a final diagnosis has been officially rendered.

An incisional biopsy indicates that the tumor is sampled by removing only a small portion of the lesion’s volume. Alternatively, an excisional biopsy is defined as removing the entire lesion, often with a surrounding cuff of normal tissue. Excisional biopsies are indicated in cases where the lesion is relatively small in size and where the surgical difference between an incisional and excisional procedure is minimal. Although there is no clear quantitative cutoff value, an incisional biopsy is often undertaken for lesions measuring less than 3 cm in diameter (Athanasian 2004). If the additional surgical resection results in meaningful morbidity, an excisional biopsy is probably not appropriate.

Staging is the single-most relevant determinant of overall survival. It is frequently performed following histologic confirmation of a sarcoma. This avoids unnecessary diagnostic procedures, which is relevant in terms of cost, radiation exposure, and resource utilization. Because most metastatic sarcomas will disseminate hematogenously, the lungs are the primary sites of distant disease. A chest CT offers the greatest resolution with which to identify thoracic disease. It may be obtained in conjunction with a PET scan, which has the added benefit of surveying other potential soft-tissue sites. Although the exact role of PET continues to evolve, it is increasingly being utilized in the context of soft-tissue sarcoma evaluation. If utilized, it is important to obtain a whole-body study, which in some institutions is referred to as a “melanoma protocol.” This ensures the entire body will be imaged, rather than limiting the study to the torso. Finally, a sentinel node biopsy (SNB) may be relevant, particularly in the context of epithelioid sarcoma, rhabdomyosarcoma, clear-cell sarcoma, and synovial sarcoma. SNB is a technique utilized to identify lymphatic spread of tumor, whereby technetium-99 m-labeled sulfur colloid is injected in close proximity to the lesion of concern. Once scintigraphic imaging localizes radioactive uptake in the axilla, an intraoperative gamma probe can identify the involved lymph node, and its subsequent surgical removal can ensue. Additionally, a blue dye, such as methylene blue, can be injected prior to surgery, visually aiding radioactive localization. For the few histologic subtypes that have a predilection for lymphatic spread, SNB is required for proper prognostication and treatment strategies. For the majority of soft-tissue sarcomas, staging is typically reported in accordance with the guidelines set forth by the American Joint Committee on Cancer, which is based upon histologic grade, tumor size, tumor location, nodal dissemination, and/or distant dissemination. A tumor’s histologic type is based upon inherent features defined by microscopic morphologic appearance, immunohistochemical staining, and identification of known cytogenetic aberrations such as translocations, deletions, and trisomies. The grade, which is an important component of tumor stage, is determined based upon a variety of features including tumor differentiation, mitotic activity, and necrosis. Depending upon the grading system utilized, other features such as cellularity, pleomorphism, histologic subtype, and location may be considered. Tumor grade determines the likelihood a tumor will ultimately metastasize, making it an important prognostic indictor and relevant for therapeutic management. Although not as important as surgical margins, it has been, in some reports, predictive of local recurrence.

In general, there is no therapeutic role for nonoperative management of soft-tissue sarcomas. A nonoperative course may rarely be elected in the case of advanced and widely disseminated disease, in which local control is neither needed for palliative nor curative purposes. Conversely, local control may offer better quality of life for patients with large, fungating, and ulcerated lesions, and surgery may be considered despite the presence of metastatic disease. Such scenarios are fortunately very rare within the pediatric population.

Surgery remains the mainstay of treatment for localized soft-tissue sarcoma. In such instances, surgery is undertaken with a curative intent. Historically, this has been realized via ablative procedures such as amputations and disarticulations. This radical approach has evolved over time, with the realization that wide excisions offered equivalent survival outcomes to those of amputations, albeit with slightly higher local recurrence rates (Williard et al. 1992). Given the tremendous implications of losing an upper extremity, most surgeons and patients prefer limb-salvage surgery whenever oncologically feasible. However, functional outcome should never take priority over oncologic cure, and properly prioritizing these goals in cooperation with the patient is essential. Overall, limb-salvage surgery is appropriate in approximately 90 % of patients with extremity soft-tissue sarcomas (Lewis et al. 1997), and this figure is probably somewhat higher within the pediatric population. Fingers, and to some extent the hand, differ in that limb salvage is frequently not possible owing to their small size, limited muscle mass, and lack of compartmentalization. Additionally, functional outcomes following finger or ray resections can be quite excellent, making this anatomic region an exception to the rule.

In general, the most important aspect of the surgical resection is the margin. Sarcoma surgery necessitates complete extirpation of the tumor, which by definition means removal of the entire lesion en bloc together with a cuff of surrounding normal tissue. The cuff should serve to completely envelope the tumor, thereby avoiding exposure and tumor spillage as well as removing any microscopic satellite tumor cells. Although important, qualitative margin assessment is subject to interpretation and is arguable, and for these reasons, it is less useful in assessing outcomes. Quantitative margin measurement is a standard component of the final pathology report; however, controversy exists as to what exactly constitutes an adequate negative margin. On the other hand, a positive margin has been widely reported to increase local recurrence. Local recurrence, in turn, portends a worse prognosis both in terms of metastatic dissemination and subsequent overall survival (Gronchi et al. 2010). This finding persists despite the use of adjuvant radiation (Al Yami et al. 2010), which primarily aids in local control.

Surgical management should be undertaken by a sarcoma specialist within the larger infrastructure of a sarcoma center. This allows for coordinated multidisciplinary care, which brings together the required diagnostic, oncologic, surgical, reconstructive, and rehabilitation needs necessary for management of these immensely complex conditions. Additionally, having the support of experienced nurse practitioners, social workers, child-life caregivers, dieticians, and other team members allows for comprehensive attention not only for the patient but their family members as well.

Proper surgical management begins well before surgery. Sarcoma centers will typically utilize a multi-disciplinary tumor board conference, at which time the diagnosis, the imaging studies, and the proposed surgical and medical management can be discussed among all involved team members. This promotes a clear understanding of the clinical entity and related relevant issues and hopefully cumulates in an optimal, uniform, and patient-specific management approach. A subsequent discussion with the patient and their family regarding the tumor board discussion is essential. Because a sarcoma diagnosis and its subsequent treatment often have tremendous implications, it is important that the family and, whenever possible, the patient have a clear understanding and realistic expectation regarding the goals and anticipated outcomes. Unrealistic discrepant expectations can lead to physician distrust and poor patient-physician rapport and can undermine future treatment efforts.

Coordination of operative care is also very important. Often, multiple services will collaborate to effect a successful resection and reconstruction. It is not uncommon for an orthopedic oncologist to collaborate with other surgeons such as a hand surgeon, a vascular surgeon, and a microvascular plastic surgeon. For proximal arm or shoulder tumors, in which access to the subclavian vessels is required or for which a chest wall resection is anticipated, a pediatric cardiothoracic surgeon should also be included. Additionally, coordination with the anesthetic team regarding patient position, access needs, expected blood loss, and postoperative pain expectations can be extremely helpful. Reviewing the various perioperative and operative milestones maximizes cooperation, efficiency, and outcomes.

Surgery should be performed in an operating theater that is appropriately sized and staffed for the various surgical teams and their respective equipment. Ensuring that all required equipment is available requires advanced notice to and communication with the operating theater and should not be taken for granted.

Following administration of anesthesia and placement of all required intravenous lines, a Foley catheter should be placed if needed. Many pediatric intensive care units are latex-free environments, and as such latex-free catheters should be used. Careful attention should be paid to the patient’s positioning and padding. Complex surgeries may last many hours. Vigilance is needed to avoid iatrogenic injuries relating to neck or limb positioning or the development of decubitus ulcers secondary to poor padding. Prior to the start of surgery, a “time-out” should be called, confirming proper patient, laterality, procedure, and consent and to clarify any surgical or anesthetic concerns. It is reasonable to identify for the supporting staff critical surgical period during which shift or staff change may not be appropriate. Preoperative and intraoperative antibiotic requirements should be reviewed, and, lastly, a designated team member should confirm that blood products are available if their use is anticipated.

Draping should be wide, allowing for clear visualization of the involved limb. Additionally, it should allow for proximal access to vascular structures in the event of excessive bleeding. The incision should be marked out in keeping with expected preoperative planning. In particular, the tumor’s dimensions should be well known from preoperative MR imaging. The size of the incision and its location need to be carefully considered in order to allow for adequate exposure and to avoid unroofing of the tumor. The incision is marked out after considering the palpable or visible tumor mass, the known radiographic size of the lesion, and the size and location of the biopsy incision.

The surgical incision should ellipse any previous biopsy tract, maintaining this tract together with the en bloc tumor specimen (Fig. 3a, b). Incisions, as a general rule, should be extensile, which typically means longitudinal. Careful attention to hemostasis is required, particularly in cases involving proximally based tumors, where use of a tourniquet is precluded. The surgical dissection should progress in an orderly fashion though dermis, subcutaneous tissue, and fascia. Assuming the tumor resides within the arm musculature, careful dissection around the lesion can now begin. Any neurovascular structures at risk but deemed salvageable based upon preoperative imaging should be traced and protected throughout the procedure. Because large tumors tend to distort normal anatomy by displacing adjacent structures, surgical aids such as a nerve stimulator and a sterile Doppler can help identify and confirm neurovascular structures, thereby minimizing inadvertent iatrogenic injury.

As surgery progresses, the tumor becomes increasingly palpable. It should not be exposed at any time during the procedure, and a normal cuff of tissue should remain encasing it at all times. This requires frequent tactile and visual assessment of both the tumor and the proximate critical structures. Care must be taken to handle the tumor gently to avoid unroofing and exposing the tumor, which in turn may theoretically seed the surgical bed with microscopic disease.

Once medial and lateral (or anterior and posterior) surgical planes are created (Fig. 3c), it becomes helpful to identify, develop, and deliver one pole of the lesion (Fig. 3d). This identifies the deep plane and allows for gentle upward traction of the specimen. Longitudinal dissection through the deeper plane can now ensue. Ultimately, the entire specimen should be freed from surrounding tissue and thereafter oriented with at least two sutures to assist the surgical pathology team in understanding how the tumor resided within the surgical bed (Fig. 3e). The surgeon should carefully inspect the specimen to assess where margins may be close or positive. Ideally, the surgeon should deliver the specimen to the surgical pathologist to guarantee accurate understanding of the specimen’s orientation and to address any surgical or margin-related concerns that may be relevant to the specimen’s evaluation.

Frequently, intraoperative frozen sections can be obtained from either areas of concern or the peripheral extent of the surgical bed. This can offer added intraoperative reassurance that no overt residual disease remains, but it is admittedly subject to and limited by sample error. If postoperative radiation is indicated, 4–6 metallic clips should be placed at the peripheral extent of the surgical bed to facilitate simulation and guide radiation therapy planning. Hemostasis should be obtained, and the wound should be generously irrigated. If soft-tissue reconstruction is not required and a large defect exists, judicious use of drains is important to avoid a postoperative collection. Careful attention to closure is important to avoid excessive tension on the soft-tissues. Given the frequent use of adjuvant radiation for large high-grade soft-tissue sarcomas, additional insult to the soft tissues increases the risk of wound dehiscence and postoperative wound complications. Postoperative splinting should be considered to allow for soft-tissue rest; however, careful attention should be paid to ensure it is well padded and not excessively tight.

The role for conventional chemotherapy in low-grade sarcomas is extremely limited and would largely be utilized in the setting of metastatic disease. Small (<5 cm) localized intermediate and high-grade lesions can usually be successfully managed with wide surgical resection alone, and the addition of adjuvant conventional chemotherapy has marginal benefits at best. Larger high-grade tumors (>10 cm in size) are very likely to disseminate, and in such instances, use of adjuvant chemotherapy should be strongly considered. Cases in which metastatic disease is identified warrant its use, recognizing that it is largely intended for palliation at this point. To some extent, the use of conventional chemotherapy is a histology-specific consideration, with some tumors being more chemoresponsive than others.

The use of adjuvant radiation, either preoperatively or postoperatively, has been shown to improve local control for large high-grade sarcomas. Typical indications for adjuvant radiation include a high-grade sarcoma exceeding 5 cm in size or in the case of a smaller tumor that was removed without adequate surgical margins. There may be an extended role for use of radiation in cases of small hand tumors completely excised where local recurrence could result in a subsequent amputation; however, this would require careful patient-specific consideration.

Usually administered at equivalent doses ranging between 50 and 66 Gy, radiation therapy combined with surgery offers improved local control rates compared with those following surgery alone (Pisters et al. 1996; Yang et al. 1998) and is routinely used to treat residual microscopic disease, improving local control rates beyond that of tumor bed resection alone (Rosenberg et al. 1982). Despite one report demonstrating substantial improvement, it is less frequently employed in the setting of low-grade tumors and in these cases surgery remains the mainstay of treatment .