How to diagnose and manage basic pediatric fractures in the primary care clinic
Identifying fractures and injuries that require further workup and referral
Initiating early evaluation for benign and malignant bone tumors
Implementing appropriate referrals
This virtual lecture is part of a series provided by CHOC that aims to bring the latest, most relevant news to community providers. You can register here.
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The rarity of sarcomas and their large number of diverse histologies have made this group of cancers very challenging to manage. At the Hyundai Cancer Institute at CHOC, collaborative research, experimental treatment protocols and surgical advancements are setting the stage to change that.
“The sarcoma and solid tumor field hasn’t advanced much in the last 50 to 60 years, unfortunately,” says Dr. Elyssa Rubin, pediatric oncologist at CHOC. “But we’re making exciting progress that will hopefully improve the care pediatric patients with bone and soft tissue sarcomas receive in the future.”
As a member of the Children’s Oncology Group (COG), CHOC is involved in a number of clinical trials that seek to improve the outcome for children with cancer. The international collaboration allows for the compilation of larger data sets that can be used to improve both research and treatments.
“Currently, we are working with COG to harmonize all the clinical trial research from the last 50 to 60 years and creating common data dictionaries so we’re all speaking the same language and being consistent with our methods,” says Dr. Rubin. “Sharing data and having this larger database gives us a better understanding of what’s working, what isn’t and what to target with our treatments. Our ultimate goal is to have our collective data in one central location so we can work together and hopefully, make more advances. This collaboration is what’s needed if we’re really going to make progress.”
Besides her role as principal investigator in clinical trials, Dr. Rubin is researching an experimental maintenance therapy protocol for sarcoma patients.
“I’ve been fascinated by applying the advancements made in leukemia treatment to my sarcoma patients,” Dr. Rubin says. “Leukemia patients are treated with aggressive therapy upfront and then they go into a maintenance phase where they’re taking their medicine over an extended period of time. Over the last seven or eight years, I’ve used a similar approach with my high-risk sarcoma patients, which isn’t the standard of care. The encouraging trend I’ve noticed is a change in the pattern of their relapse and a longer extension of time until they relapse, which tells me this protocol is working to keep their disease under control.”
While it’s still early and more research is needed, Dr. Rubin says her protocol is picking up interest within the bone and tumor committee, and larger studies will be conducted that will have patients follow this protocol for at least six months.
As Dr. Rubin continues her research and the further investigation of her maintenance therapy protocol, other advances are being utilized at CHOC today, particularly in surgical technologies for the treatment of bone and soft tissue sarcomas.
“Recent advances in surgical options help us achieve our goal of preserving as much function as possible so kids can get back to being kids and doing what they love to do,” says Dr. Amir Misaghi, pediatric orthopaedic oncology surgeon at CHOC. “With advances in growing-type prostheses for limb salvage and restoration, we are able to meet this goal now more than in the past.”
3D printing is also revolutionizing the field of orthopaedic oncology, allowing surgeons to print custom bone models for surgical planning.
“When you do so much preoperative planning, the actual surgical time can be minimized,” says Dr. Misaghi. “We’re also using 3D-printed custom cutting guides to help make the surgery as precise as possible, which helps preserve as much of the patient’s native tissue as possible.”
When it comes to the bone and soft tissue sarcoma program at CHOC, Dr. Rubin and Dr. Misaghi emphasize the robust team and comprehensive capabilities.
“Between oncology, orthopaedic surgery, plastic surgery and radiology, as well as general surgery and pathology, we really have the full package here at CHOC,” Dr. Misaghi says. “We are fully equipped to take care of all benign and malignant bone and soft tissue tumors, and we all focus specifically on pediatrics.”
Our Care and Commitment to Children Has Been Recognized
CHOC Hospital was named one of the nation’s best children’s hospitals by U.S. News & World Report in its 2020-21 Best Children’s Hospitals rankings and ranked in the cancer specialty.
CHOC’s Blueprint for Achieving Successful Outcomes for Bone Marrow Transplant Patients Long-term survivorship and improved quality of life among pediatric cancer patients undergoing bone marrow transplants is routinely threatened by three ...
When 7-year-old Naomi Adrian took a spill on the school playground early this year and a schoolmate fell on top of her left leg, she got up with a slight limp.
After what appeared to be a bruise kept growing, and as she continued to walk awkwardly, her mother, Maria Nino, took her to the doctor.
A subsequent visit to a specialist revealed a tumor — an osteosarcoma, a type of bone cancer — on the Riverside girl’s left distal femur, the area of the leg just above the knee joint.
When a Riverside doctor who was supposed to remove the tumor was unable to see her on the day of the planned surgery, Maria rushed Naomi to CHOC.
Thus began a medical journey that showcases CHOC’s commitment to outside-the-box thinking when it comes to patient care, including investigational therapies, diagnostics, and devices — as well as the benefits of CHOC’s partnership with UCI Health.
In Naomi’s case, her lead physician at CHOC, Dr. Amir Misaghi, an orthopaedic surgeon with specialties in pediatric orthopaedics and musculoskeletal oncology, was able to secure a device that doesn’t have U.S. Food & Drug Administration approval yet for use in pediatric patients to help him successfully remove the tumor and reconstruct Naomi’s leg.
“I’ve used it before,” Dr. Misaghi said of Onkos Surgical cutting guides, “but this was the first time I’ve used it in a pediatric patient.”
Ruling out other surgical options that he deemed less promising and more onerous on young Naomi’s growing body, Dr. Misaghi, who came to CHOC in October 2019 from Children’s Hospital of Philadelphia, used the Onkos device after his one-time compassionate use trial request was approved by the FDA.
In the surgery, in which Dr. Misaghi was assisted by orthopaedic surgeon Dr. Carl Weinert, the Onkos 3D resection guides, which were customized to Naomi’s leg and the growing tumor on it, helped the surgeons make extremely precise cuts as they removed the baseball-sized tumor and surrounding bone while sparing Naomi’s knee joint and the part of her bone that grows through adolescence.
Dr. Raj Vyas, chief of plastic surgery at CHOC and vice chair of plastic surgery at UCI School of Medicine, then began to reconstruct Naomi’s femur. To do this, he dissected free a segment of Naomi’s fibula bone along with the artery and vein that supply that segment of bone.
The fibula bone runs on the outside of the leg from the knee joint to the ankle joint and can be mostly removed without affecting one’s ability to bear weight.
Drs. Misaghi and Vyas then worked together to hollow out a cadaveric arm bone (humerus) and insert Naomi’s fibula bone into its core. The cadaver humerus bone was custom selected to match the size of Naomi’s femur and provide structural support. Dr. Vyas then connected the artery and vein supplying the fibula bone to a nearby artery and vein in the leg using microsurgical techniques. This allowed the vascularized fibula to “bring back to life” the cadaveric humerus bone so that it can continue to grow with Naomi.
“This was pushing the envelope as far as reconstructive surgeries go for this type of thing,” Dr. Misaghi said.
Said Dr. Vyas of the 10 hours he worked on Naomi’s leg: “In kids, it’s especially an advantage to use their own tissue if possible; if we didn’t have the ability to do this miscrosurgery, then we would have just done an inferior operation. Being able to work together and plan to do this at a children’s hospital with Dr. Misaghi’s expertise and our expertise at UCI Health, we were able to provide to Naomi state-of-the-art reconstruction.
“A while ago,” Dr. Vyas added, “maybe at some hospitals they would have just performed an amputation. Because we have Dr. Misaghi, who can do a limb salvage using a cadaver graft, we were able to really perform the best operation possible.”
Alternative treatments that Dr. Misaghi ruled out for Naomi included a rotationplasty, which would have resulted in the loss of her knee joint and a large portion of her leg, thus requiring a prosthesis; and another technique that also would have meant the loss of her knee joint and a prosthesis that would have required several subsequent surgeries as Naomi grew.
A very active child, Naomi loved to participate in ballet and gymnastics before her diagnosis on March 3, 2020.
“Hopefully we can get her back dancing,” Dr. Misaghi said. “That would be the goal.”
Trips to the park
These days, as Naomi continues to undergo chemotherapy, the now-8-year-old enjoys trips to the park where her sister, Itzel, or other relatives push her in her wheelchair.
Naomi, whose family since has relocated to the Fresno area, also loves arts and crafts, her dolls, and playing with her twin sister, Natalie.
“I felt sad (when diagnosed), but I knew that someone was always with me,” Naomi says of her cancer journey. “Thanks to Dr. Misaghi (and others), they saved my leg and I’m feeling happier now.”
So far, Naomi is progressing very well, said Dr. Misaghi, who keeps regular tabs on her. A year out from surgery, Naomi will be back at CHOC for a second surgery to remove screws that were placed near her growth plate.
“It remains to be seen how her growth plate responds,” Dr. Misaghi said. “But the survival part of her prognosis is very good since she had clear margins and she’s gotten back on the chemo, and so the function of her leg prognosis is very good. She has a normal knee joint. And hopefully when everything heals, she’ll have some metal plates remaining but be able to grow normally with her own growth plate.”
In a Zoom call, Naomi, wearing a white bow on her head, was asked how her leg felt.
“Good,” she said.
Can you walk and do normal things?
“Not yet. Maybe in a year.”
Naomi’s oldest sibling, Itzel, 20, said Naomi has been a champ through the entire process.
“She never complained about pain even after the surgery,” Itzel said.
Itzel and her mother are grateful for the excellent care Naomi received at CHOC.
“We knew that it would be very hard for her to accept losing her leg,” Itzel said. “We were happy that there was a way that that could save not only her life, but her leg.”
Said Maria: “We are extremely grateful to God for listening to our prayers and with the amazing doctors, nurses, and staff at CHOC. I want to especially thank Dr. Misaghi and Dr. Vyas as they made sure Naomi got the care she needed. They are a great team. May God continue giving them the intelligence and determination to continue to save other kids’ lives.”
Naomi already knows what she wants to be when she grows up.
“I want to be a surgeon so I can save other people’s hands and legs,” she said.
For more information about the Hyundai Cancer Institute at CHOC, click here.
Earlier this year, orthopaedic surgeon Dr. Amir Misaghi joined the CHOC Orthopaedics Institute. A member of CHOC’s sarcoma team, his special clinical interests range from bone and soft tissue tumors to limb deformities. He shared with us why he decided to become a doctor, new developments in his field, his hobbies outside of work and more.
What is your education and training?
I completed my undergraduate, medical school and orthopaedic surgery residency all at the University of California, San Diego, followed by a pediatric orthopaedic fellowship at Children’s Hospital of Philadelphia and an orthopaedic oncology fellowship at Mayo Clinic in Rochester, Minn.
What are your special clinical interests?
My clinical interests are bone and soft tissue tumors, trauma, hip disorders, limb deformities and other standard pediatric orthopaedic conditions. The vast majority of tumors in kids are benign but can still be complicated. Tumors can be found in difficult-to-operate areas, or be benign but hard to eradicate, so having both a pediatric orthopaedic and an orthopaedic oncology background helps me manage these complexities.
Why did you decide to become a doctor?
I love working with and helping others, and I was drawn to orthopaedics because I’ve always enjoyed using my hands to build and repair things. I also like working in pediatrics because I find kids fun and fascinating to be around. Oftentimes, especially when working with pediatric cancer patients, some very difficult situations and decisions arise, but I feel privileged to partner with families and navigate these challenges alongside them. Kids tend to do remarkably well and have incredible resilience, and I have encountered very powerful moments working in this field. It is an amazing feeling to witness my patients growing up and thriving.
What are some new programs or developments within your specialty?
CHOC is an amazing place with a fantastic pediatric orthopaedic department and cancer center.
Pediatric orthopaedics and pediatric orthopaedic oncology have changed with advances in growing type prosthesis for limb salvage surgery, as well as advances in lengthening limbs and restoring function after major trauma or cancer treatment. We have access to many exciting new pediatric-focused technologies at CHOC.
CHOC also has a great sarcoma team, and it is incredibly rewarding to be part of this multidisciplinary group. We take care of complex patients with the shared goal of providing the best care and getting them back to living happy, healthy lives. I love being able to work closely with Dr. Elyssa Rubin, medical oncologist, and Dr. Raj Vyas, plastic surgeon, to grow our limb salvage and musculoskeletal oncology/sarcoma program. We also have fantastic pediatric interventional radiology with Dr. Tamman Beydoun, and an amazing pathology department with Dr. Aaron Sassoon and Dr. Ali Nael.
What would you most like referring providers to know about you or your division at CHOC?
I want providers to feel confident referring patients to us. We are set up to take care of all benign and malignant bone and soft tissue tumors with a comprehensive team. The oncology program at CHOC is a dedicated group of multidisciplinary providers, including oncology, pediatric surgery, radiation oncology, interventional radiology, radiology and pathology. We meet weekly at our oncology tumor board meeting to discuss complex cases, ensuring we can provide up-to-date, cutting-edge treatment. We are fully equipped as a sarcoma team to manage all malignant bone and soft tissue tumors, and we all focus specifically on pediatrics.
What inspires you most about the care being delivered here at CHOC?
The various providers and patients inspire me. I am constantly impressed by the dedication of the nurses, physicians, child life specialists and countless other staff who work so hard. Taking care of pediatric patients, seeing their strength and witnessing them overcome their challenges is incredible.
If you weren’t a physician, what would you be and why?
I would open a small restaurant – I have always loved cooking, especially outdoors. One of the things I love most about my current job is talking to and getting to know my patients and their families. I think that the restaurant world might offer similar rewarding interactions with people, like chatting with regulars over the years, all while making delicious food.
What are your hobbies and interests outside of work?
I enjoy outdoor activities like camping and fishing, and have recently been trying to interest my kids, ages 3 and 7, in these hobbies as well. We’ve also taken up gardening as a family, and we are always ready for a good barbecue or soccer match.
What have you learned from your patients?
I’ve learned that life is a marathon, not a sprint. It is a joy to watch kids heal after surgery, and to then see them months or years later recovered and back to life. They have also taught me some practical things to keep me plugged in to what is cool and trendy. Recently I was educated about TikTok and some of its popular personalities – one patient suggested I even make a TikTok profile and start showing off some of my dance moves.