Pleomorphic Adenoma, Benign Salivary Gland Tumor: Zion’s Story

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What do a pea, a grape, a walnut and a lemon have in common?

They are the sizes that a benign tumor in one of Zion’s salivary glands went through before he had it surgically removed at Children’s Hospital of Philadelphia (CHOP).

Zion in his football uniform The mass, identified in the post-surgery pathology examination as a pleomorphic adenoma, was rare in a few ways. While these are the most common type of salivary gland tumors, they are most frequently found in adults — not in a 12-year-old. Plus, Zion’s was located in the submandibular gland, which is under the jaw bone on each side of the neck, while 84% of pleomorphic adenomas are in a different salivary gland, the parotid glands, which are back toward the ear.

The good news is that CHOP has launched the Head and Neck Disorders Program within the Division of Otolaryngology, also known as Ear, Nose and Throat (ENT). The program was specifically created to treat all kinds of common and rare conditions that can appear in a child’s head or neck.

No pain, no loss of function

Zion’s mother, Tika, first noticed a small bump under his chin late in the summer of 2019. The family was in the process of moving from Virginia to New Jersey, and after she and Zion settled in, they made an ENT appointment at CHOP’s Specialty Care Center in Princeton at Plainsboro to have it checked out. It was bigger, but it didn’t hurt or bother Zion.

Still, it’s important to remove a pleomorphic adenoma because — as Zion experienced — they continue to grow. While the vast majority are benign, there is a risk they can become malignant.

Soon after Zion received the probable diagnosis of pleomorphic adenoma, the COVID-19 pandemic swept across the Philadelphia region. Initially, outpatient appointments were moved to telehealth. The first time they met their surgeon, otolaryngologist Conor Devine, MD, was during a telehealth visit. Several pre-surgery consultations also happened virtually.

Pandemic precautions on display

When Zion came to a CHOP facility for a CT scan, blood work and other evaluations, Tika says she saw CHOP’s infection precautions clearly on display every step of the way. “We absolutely saw what CHOP was doing to keep everyone safe at all our visits,” she says.

Once the June surgery date was set, Zion needed to be tested for COVID-19 since, as a precaution, all CHOP patients are tested before or at admission. (Those who test positive are cared for in a special isolation unit.)

They went to a drive-through testing site at CHOP’s Bucks County Specialty Care Center in Chalfont, Pa. “That was a well-oiled machine,” Tika says. It was all over in just a couple of minutes. Zion tested negative for the coronavirus.

Experienced surgeon’s skills at work

During the surgery, Tika and Zion’s father, Reginald, had to wait longer than anticipated. Dr. Devine needed more time since the tumor had entwined itself around several of the nerves as it grew.

Zion smiling Dr. Devine, as one of the surgeons in the Head and Neck Disorders Program, has a lot of experience removing masses from delicate locations and carefully navigated the nerves to fully remove the tumor while preserving lip and tongue movement and Zion’s ability to taste. Dr. Devine needed to remove the one submandibular gland involved, but Zion’s other salivary glands will compensate so he won’t experience any trouble swallowing — even dry foods.

“Dr. Devine was a gem,” Tika says. “The whole team was great.”

Zion was discharged the next morning and, after taking it easy for a couple of days, was able to return to his normal routine. He will have follow-up visits with ENT since pleomorphic adenomas can grow back in a small percentage of cases.

“I felt completely confident in the care given to my child,” Tika says. “I was familiar with CHOP, so I had a high level of expectation, and CHOP met — exceeded — my expectations.”