Soft Tissue Nodules

Written by Anna

While we were dealing with Linus’s airway procedures, we were also spending a considerable amount of time with our genetics team and some dermatologists to investigate some new concerns that were developing. The day after Linus’s cordotomy, I noticed a small, firm lump on the side of his ribs. I pointed it out to one of the residents that evening, who was unsure what it could be. During our brief evening out of the NICU, I noticed a couple more similar lumps on his arms and showed them to the attending neonatologist when we returned to the ICU. She also didn’t know what to make of them but promised to look into it. 

More and more small nodules appeared all over Linus’s body, and nobody really seemed to know what to do about it at first. Since Beckwith-Wiedemann Syndrome predisposes individuals to various types of tumors, I requested that our genetics team get involved to see if they had heard of anything similar happening to other BWS patients. They had not, but together with dermatology, they decided that these nodules appeared to be myofibromas, a type of small benign tumors in muscle fibers. These are common with a genetic condition called myofibromatosis, so we performed some additional genetic testing to see if Linus could have that condition. After some persistent asking, the doctors agreed that performing a biopsy at this would be prudent, and after much debate over what type of biopsy and which spots to take tissue from he eventually got one. Soon after the biopsy was performed, we got the results from the genetics testing stating that Linus does not have myofibromatosis. These results were a relief, but it left us wondering what these mysterious lumps could be.