Paul Long Term Quality-of-Life Outcomes in Pediatric Bone Cancer : A Systematic Review

Purpose: To evaluate the current evidence on quality-of-life (QOL) outcomes for survivors of pediatric bone cancer in the lower extremities and to ascertain whether limb-sparing surgery does indeed bestow an advantage to these survivors. Methods: A thorough search of the literature was conducted. Inclusion criteria were as follows: (a) published in the last 10 years in English, (b) focused upon patients diagnosed when they were < 25 years of age, and (c) focused on QOL and functional outcomes in pediatric bone cancer patients who underwent either limbsparing surgery or amputation in their course of treatment. Results: Sixteen articles were found that fulfilled all inclusion criteria. No significant differences in QOL outcomes were found. However some differences were found in functional outcomes when pediatric bone cancer survivors were compared by tumor site. Survivors with more proximal tumors had better functional outcomes with limb-sparing sparing surgery than their counterparts with amputation. Conclusions: Future nursing research should focus on interventions to improve short and long term QOL in these patients, as no studies were found that addressed potential interventions. This journal article is available in Journal of Student Nursing Research: http://repository.upenn. edu/josnr/vol1/iss2/2 Vol 1, Iss 2, 2008 Journal of Nursing Student Research 6 Susan J. Paul Long Term Quality-of-Life Outcomes in Pediatric Bone Cancer: A Systematic Review Abstract Purpose: To evaluate the current evidence on quality-of-life (QOL) outcomes for survivors of pediatric bone cancer in the lower extremities and to ascertain whether limb-sparing surgery does indeed bestow an advantage to these survivors. Methods: A thorough search of the literature was conducted. Inclusion criteria were as follows: (a) published in the last 10 years in English, (b) focused upon patients diagnosed when they were < 25 years of age, and (c) focused on QOL andPurpose: To evaluate the current evidence on quality-of-life (QOL) outcomes for survivors of pediatric bone cancer in the lower extremities and to ascertain whether limb-sparing surgery does indeed bestow an advantage to these survivors. Methods: A thorough search of the literature was conducted. Inclusion criteria were as follows: (a) published in the last 10 years in English, (b) focused upon patients diagnosed when they were < 25 years of age, and (c) focused on QOL and functional outcomes in pediatric bone cancer patients who underwent either limb-sparing surgery or amputation in their course of treatment. Results: Sixteen articles were found that fulfilled all inclusion criteria. No significant differences in QOL outcomes were found. However some differences were found in functional outcomes when pediatric bone cancer survivors were compared by tumor site. Survivors with more proximal tumors had better functional outcomes with limb-sparing sparing surgery than their counterparts with amputation. Conclusions: Future nursing research should focus on interventions to improve short and long term QOL in these patients, as no studies were found that addressed potential interventions. E year in the United States, approximately 650-700 children and adolescents under 20 years of age will be diagnosed with bone cancer (Gurney, Swensen & Bulterys, 1999). This number accounts for 6% of all pediatric cancers diagnosed annually (Gurney et al.). Congruent with many pediatric cancers, recent advances in treatment methods have resulted in increased five-year survival rates for bone cancers. The 1975-84 survival rate of 49% increased to 63% during the years 1985-94 (Gurney et al.). While improved treatment means more survivors, it also indicates that practitioners must now give more consideration to potential long term sequale for these survivors than ever before. For the majority of the 20th Century amputation was the treatment for bone cancer (Enneking, 2000). However, in the late 1980s and early 1990s, limb-sparing surgery gained wide recognition in the pediatric bone cancer community as being the best possible guarantor of positive long term QOL outcomes (Nagarajan, Neglia, Clohisy & Robison, 2002). There is agreement that limb-sparing surgery bestows an advantage when the tumor location is in an upper extremity. However this clarity is lost when the tumor location is in a lower extremity (Nagarajan et al.). Practitioners are just beginning to study this QOL assertion and evaluate if limb-sparing surgery provides better QOL outcomes over amputation. This review seeks to evaluate the current evidence on QOL outcomes for survivors of pediatric bone cancer in the lower extremities and to ascertain whether limb-sparing surgery affords an advantage to these survivors, as has been postulated in the past. The two most common types of malignant bone tumors are osteosarcoma and Ewing’s sarcoma, accounting for 56% and 34% of bone tumors in children, respectively (Gurney et al., 1999). Of these, 57% of tumors are found in the long bones of the lower extremities. There is a significant difference in location incidence between osteosarcoma and Ewing’s sarcoma. Ewing’s sarcoma is more prevalent in the central body axis (45%), while only affecting the lower extremities 29% of the time. However, 78% of osteosarcomas are found in the lower extremities (Gurney et al.). The current model of care for children with bone cancer combines (a) chemotherapy with surgery (Nagarajan et al., 2002), with the chemotherapeutic agents and surgical interventions depending on tumor type, location and aggressiveness; (b) the child’s age at diagnosis; and (c) their skeletal maturity (National Comprehensive Cancer Network, 2007). Some children with bone cancer still undergo amputation and most occur in those patients with a tumor in the 1 Paul: Long Term Quality-of-Life Outcomes in Pediatric Bone Cancer: A Sy Published by ScholarlyCommon , 0 8 Vol 1, Iss 2, 2008 Journal of Nursing Student Research 7 Paul, S. distal femur (Nagarajan et al.). Depending upon the tumor site, however, transtibial (below-the-knee), knee disarticulation, and hip disarticulation amputations can be performed in addition to transfemoral amputations (Nagarajan et al.). Rotationplasty, a procedure that is considered halfamputation and half-limb-sparing surgery (Grimer, 2005), is another option for patients with tumors below, directly above, or around the knee (Grimer; Nagarajan et al., 2002). It is particularly useful for young patients with immature skeletons, since the reconstructed limb will continue to grow (Grimer). In this procedure, the diseased portion of the leg is removed while all of the muscular, nervous and vascular components of the lower leg, ankle and foot are retained. This structure is then rotated 180o and reattached to the remaining femur, placing the ankle joint at the level of the contralateral knee, thus giving the patient a functional “knee” joint (Grimer; Nagarajan et al.). The overarching theme of limb-sparing surgery is to replace the portion of the resected limb with either a graft or an implant, thus retaining the form and function of the limb as much as possible. Allografts are used almost exclusively in surgery for lower extremity tumors due to the need for weight bearing. Nevertheless, some researchers are currently looking into the possibility of using autografts (Grimer, 2005). Allografts are normally implanted in the middle of the tibia or femur, when there is no joint involvement (Nagarajan et al., 2002). The two major types of implants are endoprosthetic implants and expanding endoprosthetic implants (Nagarajan et al., 2002). Endoprosthetic implants are normally used when a patient’s joint has to be removed, to replace both bone and joint. Expanding implants are essentially the same as endoprosthetic implants, except they have the ability to lengthen over time (Nagarajan et al.). Patients with immature skeletons often use expanding implants so that the affected limb has the potential to grow in proportion to the patients’ other limb. However, such growth requires surgical intervention (Nagarajan et al.).