During pregnancy, you are the reason your baby lives and it’s your job to protect your child to allow it to mature, however in the case of this patient that became near impossible. Mesenchymal hamartoma (MHCW) is a type of tumour in infants that starts at the ribs and on progresses to the chest wall.

It is very rare, only reported in just over 100 cases. Unfortunately, since the tumour is so rare, there are no published guidelines for directing doctors in treatment, and the course of treatment is highly dependent on the symptoms the patient exhibits.

For this mother and her child, it was an extreme case and doctors treated it with a very unusual course of events. However, it worked! This case had a lot of noteworthy symptoms that caused massive distress and harm to the child, as well as her mother but their approach can be very useful for future cases of MHCW. Here’s what they did.

The child was diagnosed with MHCW prenatally and began growing masses on her chest wall. The way they diagnosed the cancer was by doing an ultrasound at 33 weeks, where they noted the mass and after an MRI they found a small lesion on the right and a large lesion on the left. The left lesion was causing significant deformity in the child’s chest, with multiple ribs effected and bulging of the wall as a result. The main concern with the left lesion though was that it initiated a lot of fluid buildup and created susceptibility to hemorrhage or calcification. This means that the tumour could cause major, life threatening bleeding or could harden creating massive obstruction in the chest wall.

X-ray of chest

The X-ray retrieved at birth showing the affected areas. A shows the displaces trachea, B shows scoliosis, C shows the mass on the left side, D shows where the chest tube had to be placed and E slightly shows the displaced ribs. Image taken from paper cited.

Given these findings and the progression of mass growth and fetal distress, the mother had a C-section at 35 weeks. When the child was born, it was intubated for breathing support and taken to the intensive care unit immediately. Upon an x-ray that was taken, the mass on the left side showed deformity of multiple ribs, chest wall shifts and scoliosis in the upper spine. Unfortunately, after birth the mass rapidly grew and this caused issues with respiratory support for the child which majorly impacted oxygen access. Therefore, the doctors decided there was no more waiting to be done, and surgery the risk they would have to take if this child had any chance of living.

showing chest reconstruction

The left side (A) shows the chest wall before reconstruction, and the right (B) shows after. Image taken from paper cited.

 

When the infant was 15 days old, the doctors performed surgery to remove parts of the left chest wall and lung mass. After careful navigation, they successfully removed the tumour, but this showed the intense deformity of ribs 3 through 8. For the chest cavity to make room for the tumour, many heart and lung vessels were displaced, therefore the surgery took longer because of the difficult and precise guidance that needed to take place. As mentioned, the ribs were severely distorted and with the complexity of the tumour, portions of ribs 3 through 6 had to be removed and the chest wall had to be reconstructed.

After hours of surgery, the operation was successful and I can imagine how relieved the doctors and parents must have been. Given the complexity and rarity of this case, it was a massive first win.

Five days after the operation the newborn was weaned off the ventilator and began to breathe on their own. Shortly after the infant was discharged and given a protective shell that was custom made by orthotics and could be used as needed until the chest wall reconstruction became strong enough.

image showing success

The first picture, A, shows the chest and left wall mass 48 hours after birth. Part B shows the successful resection of the tumour and was taken 2.5 months after surgery. Image taken from paper cited.

After 3 months, a checkup revealed that the left mass resection was a success! The right mass eventually showed significant growth and was removed once the child was a year old. Unfortunately, the mass on the left side of the chest cavity came back but was successfully removed once again.

 

The complications with this case and the complexity of the tumour made it very difficult for removal. MHCW is already a very rare cancer, and it did not help that the symptoms were as drastic as they were. However, the doctors that treated this patient acknowledge that their approach was risky and abnormal, but they saw it was the best chance the child had for survival and a good quality of life post-surgery.

And they were right!

This case shows how complex this cancer can be, and shows the importance of an approach that is both risky and assured. As well this case shows how important follow up appointments are considering the reoccurrence rate. An emphasis is needed on how the MHCW was removed, although it should be noted that this success does help but does not guarantee anything because of the intricacy of this type of tumour.

 

 

Source:

Groth, S., Eyerly-Webb, S., LaForest, L., Dion, E., Fisher, J., & Lillegard, J. (2020). Bilateral chest wall mesenchymal hamartoma: Prenatal diagnosis and staged surgical resection. Journal Of Pediatric Surgery Case Reports, 101474. doi: 10.1016/j.epsc.2020.101474