Managing Sarcomas in Pediatric and Adult Patients

May 2020 Vol 11, No 5



Although not the most common of cancers, pediatric and adult sarcomas affect a significant proportion of patients, and managing these malignancies first requires understanding them, according to Paula Sanborn, BSN, RN.

At the AONN+ 10th Annual Navigation & Survivorship Conference, Ms Sanborn, a sarcoma nurse navigator at Nationwide Children’s Hospital in Columbus, OH, educated attendees on understanding and navigating patients with soft-tissue and bone sarcomas in both the adult and pediatric populations.

Adult Occurrence of Soft-Tissue Sarcomas

Sarcoma is a type of cancer that forms in the bone or soft tissues of the body, including cartilage, fat, muscle, blood vessels, fibrous tissue, or other connective or supportive tissue. Different types of sarcoma are based on where the cancer forms—osteosarcoma in bone, liposarcoma in fat, and rhabdomyosarcoma in muscle.

In the United States, 1.8 to 5 cases of soft-tissue sarcoma occur per 100,000 adults each year, with slightly more than 5000 deaths annually. Clinical presentation for both adult and pediatric patients depends on the disease site: orbital (swelling, ptosis, drainage, but rarely pain), facial/oral (swelling, pain), extremity (lumps and bumps, often without pain), genitourinary (urinary symptoms, vaginal protrusion, testicular swelling), trunk (associated with organ involvement and constipation), or paraspinal (constipation, enuresis, numbness, weakness, and tingling of extremities).

Treatment and prognosis depend on the type and grade of the sarcoma, but treatment approaches are highly similar in the adult and pediatric populations.

“Surgical resection with wide-margin function-sparing excision is the cornerstone of effective treatment of all tumors, but especially extremity tumors,” she said, pointing out that surgical tumor resection without good margins can sometimes contaminate the field and do more harm than good. In patients with low-grade disease whose tumors are unresectable, treatment should consist of high-dose chemotherapy with or without radiation.

Local control of high-grade sarcomas is achieved with a combination of surgery and radiation, in addition to a variety of adjuvant and neoadjuvant chemotherapy approaches. When lung metastasis is present, “you’ve got to remove it and treat it,” she said. “This can be a very long process for people because they just want to get through therapy. It extends the process, and that can be very anxiety provoking.”

Pediatric Occurrence of Soft-Tissue Sarcomas

Annually, approximately 4.5 cases of soft-tissue sarcomas occur per 1 million children in the United States, with approximately 350 new cases per year. “It feels like we’re having a run on rhabdomyosarcoma under the age of 2 in my hospital,” Ms Sanborn noted. Peak incidence occurs between the ages of 2 and 6 years, and again between ages 15 and 19 years, accounting for nearly 6% of all malignant solid tumors in children.

More than 50 types of pediatric soft-tissue sarcomas exist, but the most common types are rhabdomyosarcoma (the most common) and non-rhabdomyosarcoma (NRSTS), consisting of several subtypes (synovial sarcoma, fibrosarcoma, liposarcoma, etc). Approximately 35% of these malignancies occur in the head and neck, 24% in the extremities, 18% are genitourinary, 11% are in the trunk, and about 12% occur in other places in the body.

Favorable sites include the orbit, head and neck (excluding the parameningeal area [nasopharynx, sinuses, middle ear]), as well as the genitourinary system (excluding the bladder and prostate). She noted that in these patients, taking care to schedule treatment around school and other important activities (backpacking fluids when necessary) is absolutely feasible.

In contrast to the adult population, in which disease stage is an important prognostic factor, Ms Sanborn noted that pediatric patients with rhabdomyosarcoma are more commonly classified as either metastatic or localized. “Are you metastatic or not metastatic up front? Do you progress on therapy or do you relapse quickly as soon as you stop therapy?” she asked. “Sometimes it’s hard for people, because they want to know a stage. But we really don’t live in the ‘stage world’ in pediatric sarcomas.”

Other important prognostic factors in rhabdomyosarcoma include nodal status, histology (botryoid/spindle cell > embryonal rhabdomyosarcoma > alveolar rhabdomyosarcoma > undifferentiated), tumor size, location, and age (patients younger than 10 years generally have a better prognosis). In NRSTS patients, prognostic factors are focused around the extent of the disease, the extent of tumor resection, tumor diameter, and tumor grade.

Adult and Pediatric Bone Tumors

Osteosarcoma and malignant fibrous histiocytoma are the most common primary bone tumors in pediatric cancer (approximately 700 cases/year in those younger than 20 years), and account for approximately 200 cases per year in adults older than 20 years of age.

Incidences of Ewing sarcoma, extraosseous Ewing sarcoma, Askin’s tumor, primitive neuroectodermal tumor (PNET) of bone, and PNET of soft tissue (treated with most of the same methods), have remained unchanged over the past 30 years. These are the second most commonly diagnosed bone tumors in the United States, but they still only account for 1 case per 1 million individuals.

“I cofacilitate an adolescent and young adult (AYA) group at Ohio State and Nationwide, and we meet at a coffee talk about once a month,” she noted. “On average, about 10 to 15 people come, but probably about 7 of them have Ewing sarcoma. We say it’s such a rare tumor, but that just tells you the incidence—at least in our area—and it also tells you they’re living longer because we’ve done such a good job at managing recurrent disease.”

Patients with osteosarcoma or Ewing sarcoma almost always present with pain, typically with limited range of motion with longstanding disease, and about half have an associated soft tissue mass and local swelling. Again, the prognosis for these patients relies heavily on metastatic versus localized tumors, and treatment consists of a combination of chemotherapy (backbone and continued systemic therapy), surgery, and/or radiation.

“Many of our patients have been in physical therapy for bone, joint, or knee pain for months before someone does an x-ray and they get to us. Many are also treated as sports injuries before they get to us,” she said. “If you’ve got a patient with osteosarcoma or Ewing sarcoma who has been out there for months or years with this lowlevel pain—especially with Ewing’s—they typically will have metastatic disease up front.”

Many patients also present with bilateral lung disease. “When you look at osteosarcoma or Ewing’s, whenever there’s a tumor, the only thing that we know for sure is that you have to remove it,” she emphasized. “This means multiple surgeries and multiple therapies to treat these patients.”

In patients with tumors of the extremities, she noted that the choice between amputation and limb-sparing surgery comes down to personal preference and quality of life, but that choice will significantly affect the life of the patient, particularly for younger individuals. For example, after limb-sparing surgery, patients will not be able to run or play high-intensity sports, so many athletes opt for amputation.

Rotationplasty, a surgical procedure used to treat bone tumors that occur near the knee, is another option for these patients. In this surgery, the bottom of the femur, the knee, and the upper tibia are surgically removed. The lower leg is then rotated 180 degrees and attached to the femur, so essentially the ankle joint becomes the knee joint, and a prosthesis is attached below, allowing for a wide range of movement and physical activity.

The choice is most often the patient’s, but it is important to note that the psychosocial impact of amputation and rotationplasty in particular can be profound, especially on younger patients, so psychosocial services should be offered to those considering these operations.

Ms Sanborn leads a ski trip in Colorado every year for her AYA sarcoma patients, and she emphasized the positive psychosocial impact of bonding experiences like these. “You walk in there and everybody is taking their legs off and talking about what cream works best for sweating, or talking about their backwards knee from their rotationplasty,” she said. “They’re people from all walks of life, but when they get together on that trip they share and they immediately bond. And those are lasting relationships for them.”

Novel therapies on the horizon for sarcomas include a number of antimitotic agents, targeted inhibitors, immunotherapies, and virotherapies. However, Ms Sanborn stressed again that complete surgical resection of all sites of disease remains the only proven treatment for possible long-term survival in these patients.

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Last modified: August 10, 2023

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