One of the morning sessions on the second day of the 18th annual ESPD meeting was devoted to “Naevi and other mosaics” with moderators Veronica Kinsler (UK) and Peter Steijlen (Netherlands).
The event’s exhibition was furnished with a counter for the charity organization in support of children suffering from Congenital Melanocytic Naevi (CMN) “Caring Matters Now” with 3 main aims of the charity: to support those affected by CMN, to raise awareness about CMN, to raise funds for the CMN research. Prof. Yordanova met the founder of this organization, a father whose child with giant melanocytic nevus has been successfully operated by Dr. Veronica Kinsler’s team in Greath Ormond Street Hospital for Children London, for UK.
Congenital melanocytic naevi are a known risk factor for melanoma, with the greatest risk currently thought to be in childhood. There has been controversy over the years about the incidence of melanoma, and therefore over the clinical management of CMN, due partly to the difficulties of histological diagnosis and partly to publishing bias towards cases of malignancy.
Dr. Veronica Kinsler presented “New (and useful) classification of CMN based on embryological insights” Dr. Kinsler studied Medicine at Cambridge University. She developed an interest in Paediatric Dermatology early in her career, through working at Great Ormond Street Hospital for Children (GOSH) in London. She has PhD in Mollecular Genetics also. Dr.Veronica Kinsler is the academic lead clinician for the Paediatric Dermatology department at GOSH and undertakes her research within the Clinical and Molecular Genetics Unit at the neighbouring UCL Institute of Child Health.
This research has focused on the clinical and genetic characterisation of Congenital Melanocytic Naevi (CMN) and their associated complications. More recently she has established the GOSH rare dermatology diseases resource, a tissue bank for facilitation of research into all rare diseases seen in the GOSH department. During her presentation Dr. Kinsler emphasized that the CMN classification needs to be changed based on the evidence. In the last classification of CMN Dr. Kinsler gave currency to her hypothesis that distribution of the birthmarks can reflect embryonic patterns. Dr. Kinsler reminded that the first classification for CMN that was developed in the year of 1979 was based on the size; the next classification from 1986 was based on different rates of growth of different body parts. The newest classification that Dr. Kinsler proposed CMNs are classified according to the size they will be in adulthood (known as “projected adult size”).
This is grounded upon the Pattern of distribution of giant CMN – Rule 6. Great significance is assigned to the following characteristics: Proportion of CMN to total body proportion, abnormal MRI of the brain/spine, neurodevelopemental problems, requirement of neurosurgery. The risk of melanoma in patients with a single CMN is very low, likely to be comparable to the risk in an acquired melanocytic naevus. They do not therefore need to be resected prophylactically or routinely monitored in childhood at least. A single CMN of any size is not associated with neurodevelopemental problems and never requires neurosurgery as a difference of the multiple CMN. New 2018 CMN classification includes five Grades. According to this the patients with Grade 0 – single CMN at any size and site, Grade 1 – Single CMN<20 cm on the trunk, limbs and <10 cm on the head, never have MRI abnormalities, no neurodevelopemental problems requiring neurosurgery and never developed Melanoma malignum. In patients with multiple CMN – Grade 2-5, the risk of melanoma is most strongly predicted by the results of the screening MRI of the CNS. If MRI is normal - the risk of all-site melanoma in childhood is of the order of 1-2%, independent of the size or number of the multiple CMN. If MRI is abnormal - the risk is approximately 12%, although this is mainly for those with complex congenital neurological disease on MRI.
