by Antonella Sparvoli
It affects one child out of 100,000 born, it extends over a large part of the body and requires early diagnosis and monitoring over time. Interview with the experts of the Bambino Ges Pediatric Hospital in Rome
Alongside the classic acquired moles (or moles) that we all develop throughout our lives, there is another type of congenital moles, i.e. moles already present at birth, which represent a real skin malformation and are associated with the risk of malignant transformation into melanoma . Of great impact in particular the so-called giant congenital neo (or nevus). This rare skin lesion, which affects about one child out of 100,000 born, can in fact reach a diameter of over 40 centimeters, with important aesthetic and consequently psychological repercussions, which add to the risk of pediatric melanoma.
Features
The giant congenital mole extends over a large part of the body, the scalp or even the face, and is often associated with other moles of various sizes, from a few millimeters to a few centimetres, called satellitosis, also at risk of malignant degeneration. Not only that, it can also be associated with a localization in the brain, called neuro-melanosis, because there is only one neural crest, which is the embryonic structure from which the skin and the central nervous system originate. Any neuro-melanosis can only be identified within the first months of a child’s life, by performing an MRI scan of the brain and marrow – he points out – explains May El Hachem, head of Dermatology at the Bambino Ges Children’s Hospital in Rome-. After three or four months of age, the completion of the maturation process of the central nervous system prevents the identification of the cerebral nevus which is no longer “visible” on the MRI. Precisely for this reason, an early diagnosis of giant congenital nevus is fundamental.
Surveillance and treatment
Due to the risk of malignant transformation of the giant congenital nevus, patients should be followed up for life with periodic dermatological checks. In our series, which counts about 70 new cases a year, being the Child Jesus a reference center, we have had more cases of melanoma on the satellites than on the giant nevus. Both types of injuries are at risk, which is why they need to be monitored, says Eh Hachem. In the case of giant congenital nevus, the treatment of choice is surgical removal in several stages during the growth of the child. Surgical treatment, there are no alternative therapies – clarifies Mario Zama, head of plastic and maxillofacial surgery at the Bambino Ges in Rome -. The removal serves to prevent an oncological problem: with surgery we reduce the size of the nevus and therefore also the oncological risk. Therapies such as dermabrasion and laser act only on a superficial level, leaving the entire deep component in place, and do not allow the mole to be analysed. This approach may appear to be cosmetically helpful, but it makes adequate monitoring impossible, and the child risks late diagnosis of advanced stage melanoma or metastasis, without knowing the origin of the disease, El Hachem noted.
Surgery
Surgical treatment depends on where the nevus is located. If localized on the face, especially on the nasal pyramid or the eyelids, the treatment of choice is represented by skin grafts with areas of skin taken from other parts of the body – explains Zama -. Differently for other areas of the skin, the preferred approach is skin expansion because the portion of skin covered by the nevus is very extensive and therefore it is not possible to radically remove it in a single operation. The surgical process begins with the subcutaneous insertion, in the area adjacent to the nevus, of a skin expander, a sort of silicone prosthesis with a valve that allows it to be filled periodically, once or twice a week, with physiological solution. The aim is to get as much healthy skin as possible to cover the portion of the nevus that will be removed. In some cases two or three expansion cycles are enough, other times, in the case of large snow, even 10 cycles may be necessary – clarifies the expert -. Bearing in mind that each cycle involves two surgeries under general anesthesia (one to place the expander and the other to remove a portion of the nevus), the young patient can even undergo 20 surgeries as he grows up.
The psychological impact
As it is easy to imagine, the giant congenital nevus and its satellitosis can have a very strong aesthetic impact with enormous psychological repercussions for the parents of the newborn and later for the child as it grows. Children and young people with giant nevus tend to feel “different” and hide away. At the same time, the complex process of care, including surveillance and surgery, can interfere with school activities and fun with peers. Not to mention that the risk of developing melanoma exists for life so there is always a basic concern for one’s health – observes El Hachem -. For all these reasons, it is very important to offer psychological support to patients with giant congenital nevus and their parents, and this support must form an integral part of the multidisciplinary treatment plan for these children. The doctors of Bambino Ges are also making an effort in this sense to better structure the path of psychological support, hoping that it will be accepted by the families.
July 29, 2023 (change July 29, 2023 | 09:24)
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