On Oct. 1, 2017, Aarti and Ashish Sarin took their daughter to urgent care because of a low grade fever. The nurse there noticed some swelling along her 3-year-old daughter Shivaya’s right jaw. Perhaps a swollen lymph node, she thought, from a low-grade fever? But when she touched her child’s jaw, she felt a hard mass. Differential diagnoses from ear and staph infections followed until Aarti met with a radiologist for the results of a scan of her daughter’s right mandible, which had grown dramatically in just a few days.
“The radiologist said, ‘What the hell is this? I’ve never seen anything like this—go get her admitted,’” Aarti says, describing a watershed moment. “The world stopped.”
Aarti and Ashish were told it was a bone cancer, Ewing’s Sarcoma. What it turned out to be, after workups at hospitals in northern Virginia and Washington, D.C., was a central giant cell granuloma, not a malignant cancer but an aggressive tumor that was eating away her right jawbone and now beginning to inflict substantial pain. Searching for a treatment for this rare tumor, Aarti and her husband consulted with and visited children’s hospitals up and down the East Coast. In November 2017, they found pediatric plastic and reconstructive surgeon Jordan Steinberg at Johns Hopkins Children’s Center, who recommended surgery but also supported a less aggressive treatment with corticosteroid injections to shrink the tumor.
The family chose the simpler steroid therapy at a hospital closer to home. Unfortunately, the treatment showed limited effects and, in February 2018, the parents revisited Steinberg. Considering Shivaya’s deteriorating condition, he recommended an extensive resection and reconstruction of her jawbone. Also, he would repurpose one of the girl’s ribs as a graft to replace the jawbone, a technique Steinberg has used in surgery for congenital deformities such as hemifacial macrosomia, in which the lower half of one side of the face is underdeveloped.
“What I proposed seemed a little radical as the first path, but I figured, given the extent of this tumor, nothing short of that was really going to work,” says Steinberg. “There was this huge mass that had eroded the jaw and was growing out of control. We knew that part of her jaw was no longer functional.”
As clearly and straightforwardly as Steinberg had explained the surgery, it was far from uncomplicated. The major challenge would be separating the tumor from the normal structures, including the facial nerve branches, which was no simple matter in that the lesion had grown in a very amorphous fashion. To strengthen the odds of a good outcome, Steinberg chose as a collaborator pediatric otolaryngologist Jonathan Walsh, a specialist in operating on facial nerves as well as head and neck tumors. The two surgeons also relied on a 3D model of the tumor in planning the surgery.
In the 13-hour operation, using facial nerve monitoring, Steinberg and Walsh meticulously peeled the tumor off the normal structures of the jaw, leaving the nerve branches unharmed. Steinberg and Walsh also had to take a tissue flap from the neck to plug an internal portion of the mouth damaged by the tumor. The outcome?
Noting that Shivaya’s parents report she is eating and behaving like a normal child and “back to herself again” following physical therapy, Steinberg says, “Things went very well with her, she’s doing great. We chose the rib graft, which unlike an artificial graft will grow with the growing child, but she will likely still have some asymmetry issues and may require additional surgery in the future.”
Steinberg adds that giant cell granulomas have a high recurrence rate, a factor that will require close monitoring.