ACCKT estimates that there are approximately only 5.5 new pediatric onset adenoid cystic carcinoma (ACC) diagnoses per year in the US, accounting for 0.000003% of the total cancer diagnoses/year ; 1 in 347,070 total cancer diagnoses.

This number is based on:

  • Total number of new cancer diagnoses estimated for 2018 per the National Cancer Institute : 1,735,350

  • Estimated 1224 new ACC diagnosis / year total

  • MD Anderson had only 7 Pediatric ACC cases as of 2011 in 40 years (with approximately 44,000 new patients / year-from 2017 annual report)

  • MSKCC had only 1 pediatric ACC patient in last 7 years and perhaps only 6 in the whole history of the hospital per personal conversations with treating physicians, (with approximately 23,000 new patients/year - from 2016 annual report)

 
 

According to the National Cancer Institute, and The Children's Oncology Group, pediatric onset of Adenoid Cystic Carcinoma (ACC) falls under the umbrella of a rare pediatric cancer, as sited in the International Classification of Childhood Cancer subgroup XI. The National Cancer Institute features some information about Pediatric Salivary Cancers here and here. 

 

Limited Data + Statistics

Currently there is no documented data on how many pediatric patients (initially diagnosed at age 0-20) are diagnosed with Adenoid Cystic Carcinoma (ACC) each year. In total (including adults), ACC is only diagnosed in approximately 1,224 patients each year (0.2% of the total cancer diagnosis' annually). We know that the population of pediatric onset patients is a minute fraction of this already statistically minuscule number.

For those of us unfortunate enough to have received the news of our child being diagnosed with this orphan cancer, the blow is further angst-inducing by the limited information and data available. The information that is available is weighted significantly by the adult population of ACC patients because of it its more typical onset in adults in 5th-7th decades of life. Pediatric ACC patients fall victim to the problem that impacts all pediatric cancers - research is underfunded or nonexistent due to how rare it is compared to cancer in the adult populations. The pediatric ACC population is especially disadvantaged because of its rarity.

We have complied a list of published medical papers here that include and/or reference pediatric ACC patients. While the list spans several decades and provides little information about pediatric ACC patients as a population, it does provide evidence that there are patients out there and we have the opportunity to learn from them.

What We Are Learning

ACCKT has been able to identify nearly 50 patients to-date worldwide who have been diagnosed with ACC in the pediatric age range (ages 0-20). These children and teens were diagnosed with ACC of the following: parotid gland, lacrimal gland, submandibular gland, hard palate, minor salivary glands and vocal chords/larynx. Most of these patients were treated with surgery and/or proton radiation therapy.

We have also funded, initiated and continue to take part in the first research studying pediatric ACC separate from the more typical adult onset. Learn more about that research here.

Like adult-onset ACC, each patient was diagnosed and treated at a different stage of their ACC growth, by varying levels of care facilities and are currently at different places with their health as a result. Some are dealing with metastasis to the lungs and other areas. But many are living without reoccurance, continuing to get scans as prescribed and going on with their lives as normal, attending high school, college, post graduate studies, working, having families, and living otherwise healthy productive lives.

However, they are and will continue to have scans (Xray, MRI, CT and/or PET) to monitor their ACC throughout their lives. This is due to the high propensity for local reoccurrence and distant metastasis to the lungs, liver, brain, spine and/or bones. While ACC is typically a very slow growing cancer, it can be tenacious in its tendency to travel along nerve paths microscopically. Therefore lifelong monitoring is critical.

If you are the parent of, or an adult patient originally diagnosed with ACC between the ages of 0-20, please consider taking part in our ACCKT Patient Registry.
For more information, click here.