Giant bathing trunk nevus:

To cut or not to cut?

presented by

Henry Foong FRCP Edin

Ipoh, Malaysia

on March 21, 2006

Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, Malaysia

 
Abstract A 3-year-old girl presented with a large congenital melanonytic nevus on her trunk and thighs. There is a significant risk for developing melanoma and neurocutaneous melanocytosis (NCM)in this patient.
Patient
S.H., 3-year-old girl
Duration
Since birth
Distribution
Trunk and lower limbs
History

The patient is a 3 year old girl who presented with a large hairy black patch on her trunk and thighs. This was present since birth. She is the younger of 2 siblings and has no family history of similar pigmented lesions or melanoma. There was no family history of consanguineous marriage.

Physical Examination

Examination showed a giant hairy pigmented nevus measuring 25cm diameter affecting the entire lower half of her trunk extending to both her thighs and perineum. The patch over the buttock appeared more hairy and nodular and was surrounded with satellite lesions. There were similar smaller satellite lesions on her face. No neurological deficit was noted. No erosions or ulcerations were note on the pigmented lesions.

Images

Laboratory Data

nil

Histopathology

nil

Diagnosis Large (Giant) congenital melanocytic nevus (Giant bathing trunk nevus)
Reasons Presented
This is a rare and unique disabling condition and the medical literature does not have a clear consistency on the management of this condition.
Questions

Congenital melanocytic nevi are defined as benign nevomelanocytic proliferations present at birth. In rare cases they appear in chidren between 1 month and 2 years of age. This subset is termed nevus tardive. Large or Giant congenital melanocytic nevi (LCMN) are those greater or equal to 20cm in their greatest diameter.

The association between LCMN and melanoma has been established beyond any doubt. The exact magnitude of the risk is, however, unknown. The life time risk appeared to be between 5% to 40%. It seems that individuals at greatest risk are those with very large LCMN (50 cm), LCMN in axial locations, and those with multiple satellite nevi. This risk is greatest in the first decade of life. Intervention, if recommended, must start early in life.

Options available included staged excision with grafting, dermabrasion, curettage, Q switch Ruby laser or just simple close observation

Does this patient require any intervention? An MRI of the brain has been arranged.

References
  1. Tannous ZS, Mihm MC Jr, Sober AJ, Duncan LM. Congenital melanocytic nevi: clinical and histopathologic features, risk of melanoma, and clinical management. J Am Acad Dermatol. 2005 Feb;52(2):197-203.
  2. De Raeve LE, Roseeuw DI. Curettage of giant congenital melanocytic nevi in neonates: a decade later. Arch Dermatol. 2002 Jul;138(7):943-7. (see abstract)
  3. Duke D, Byers HR, Sober AJ, Anderson RR, Grevelink JM. Treatment of benign and atypical nevi with the normal-mode ruby laser and the Q-switched ruby laser: clinical improvement but failure to completely eliminate nevomelanocytes.Arch Dermatol. 1999 Mar;135(3):290-6. (see abstract)
  4. Bittencourt FV, Marghoob AA, Kopf AW, Koenig KL, Bart RS.Large congenital melanocytic nevi and the risk for development of malignant melanoma and neurocutaneous melanocytosis. Pediatrics. 2000 Oct;106(4):736-41.
  5. Hale EK, Stein J, Ben-Porat L, Panageas KS, Eichenbaum MS, Marghoob AA, Osman I, Kopf AW, Polsky D. Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi--results from the NYU-LCMN registry. Br J Dermatol. 2005 Mar;152(3):512-7.
Keywords large congenital melanocytic nevus, melanoma, neurocutaneous melanocytosis
Comments from Faculty and Members

Abbas Alshammari MD, Army Clinic, Qatar on March 21, 2006

There is no doubt to feel some sort of distress in facing such child with this presentation. In addition to the bad outcome because of the risk of melanoma, I think the psychological impact of disfigurement especially at that site of involvement should be considered against "wait and see". I see the best solution is a staged excision and grafting and I don't expect any role for dermabrasion or curretage as an option.

H. Randolph Byers, MD, PhD. Professor of Dermatology, Department of Dermatology, Boston University School of Medicine, Boston, MA, USA on March 21, 2006

As the Dermatopathologist evaluating the depth of nevomelanocytic destruction by the two ruby lasers in Duke et al., I was amazed how well the melanocytes survived in the mid and deep dermis. As giant congenital nevi extend into the mid and deep dermis, laser treatment would leave many if not most of the nevomelanocytes and the risk would only be partially reduced as melanoma appears to arise in the dermis in such lesions. Similarly, dermabrasion would likely be insufficient.

I recall one teenager with a giant bathing trunk congenital nevus that was followed clinically and had developed a number of epidermal inclusion cysts. Unfortunately a deep nodule of melanoma was followed as a probable epidermal inclusion cyst until it was excised at a thickness of greater than 10 mm.

In my opinion, early surgical intervention with multiple staged excisions with tissue expansion and or grafting is the best approach as well as close clinical follow-up for the remaining areas that are too difficult to be removed or in areas that would produce too much scaring. I refer to our surgical colleagues for the best surgical approach and dermatologists for follow-up.

Jeffrey Dover MD, Adjunct Professor of Medicine (Dermatology) at Dartmouth Medical School, and Director, SkinCare Physicians of Chestnut Hill, Boston, MA, USA on May 21, 2006

Very difficult case indeed. I am still not sure how best to manage these kids. Some believe that a destructive treatment such as dermabrasion or laser treatment decreases the number of melanocytes and this decreases the risk of melanoma. I am not sure this is true and the cosmetic results are dreadful.

It may be better to wait and watch.

David Elpern MD. Williamstown, MA, USA on March 21, 2006

Removing this nevus is well-nigh impossible. I'd be curious to hear how this was going to be done. I had a patient around 20 years ago with an almost identical process. She too, had many smaller nevi scattered over the integument.

You have to remember the mot: "to cure sometimes, to relieve often, to comfort always." You have to adopt this patient. She needs one dermatologist to make a relationship with her and her parents.

Gather as much information as you can. As others have mentioned, this type of lesion can be psychologically devastating. My patient grew up in Hawaii and never went to the beach. She hid her body. She did not get the education her siblings did and had a baby out of wedlock at a young age. She was a sad person, always worried about what others thought of her.

Take your time. Digest all the advice and festine lente: hurry slowly. Good luck.

Amy S. Paller, M.D. Walter J. Hamlin Professor of Dermatology and Pediatrics, Feinberg School of Medicine Northwestern University, Chicago, IL, USA on March 22, 2006

Large bathing trunk nevi here in Chicago are referred to our head of plastic surgery who does expanders and serially removes. Not sure this is the best thing, and she has already gotten to 3 years of age without melanoma, but that is our standard of care.

Khalifa Shaquie MD, PhD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on March 22, 2006

Multiple drugs regime therapy should be strongly encouraged in dermatology, like what we do in acne vulgaris, rosacea, TB, Behcet's Disease, pemphigus vulgaris, melasma, SLE, DLE etc. The aim of this multiple drugs regime in one disease is to maximise response, accelerate healing, minimising dose, decreasing resistance in case of organism and reducing the side effects. This idea could be applied in this problem case (bathing nevus) like doing dermabrasion followed by ruby laser after a certain time to reach and damage the deep nevus cells. Staging excision although appears logical but is a difficult and prolonged task.

Michael Albom MD, Clinical Professor of Dermatology, Ronald O. Perelman Department of Dermatology, New York University Medical Center, New York, NY, USA on March 23, 2006

My heart goes out to this little girl because we know that she will endure much physical and emotional turmoil in her life irrespective of how her case is ultimately managed.

I have a few further thoughts about this child. Hopefully, a network of support can be developed for her. She will need not only excellent medical and surgical care, but also kind and understanding parents, appropriate psychological support for those times when things get difficult with peers in school and as a teenager when a whole array of conundrums will become manifest. Unfortunately, because we live in such a tumultuous world, this child will be exposed to those who will be glad to add to her own personal torment as she develops. One can only hope and pray that she will receive much love by those who come to play the most pivotal roles in her life. It would be divine if she could be guided especially by those who will teach her, from this moment on, that her true value in this world cannot be defined by the covering in which she is wrapped. It may be wishful thinking on my part, but I have hopes that she may be able to flourish if she can eventually find it within herself to believe that she was put on this planet for some purposeful aspect of its betterment as well as her own.

You and David continue to do a great service for the betterment of mankind. I admire both of you because it is clear that you are not only excellent physicians, but you also perceive the bigger picture: the physician's role in the value of humanity. Medicine that is practiced as a sterile academic discipline has severe limitations in providing wellness for our patients. It seems as if statistical p-values for outcomes in published papers are more vital to present than knowing how the actual patients progressed during their illnesses. We seem to avoid addressing the suffering endured by the patients and, how as physicians, we can optimally help to allay that suffering. There is little discussion as to how delving into that suffering affects us as physicians, the toll it takes on us, and what we may do to maintain our own wellness as we attempt to help those in need. Perhaps healing is best facilitated by conscious awareness and practice of empathy and compassion. Patients tend to report that their progress was better than not when they felt cared for by their doctors irrespective of the actual outcomes. Our perception of wellness is our conscious reality of wellness. I believe that you both know this and try to educate us to be better healers and not just "doctors." Keep up the good work.

Nedhal Khalifa MD, Assistant Professor, Department of Dermatology, Medical School, Arabian Gulf University, Bahrain on March 24, 2006

A very challenging case indeed. However, the size of such lesion limits the management options. Surgical excision is likely to result in significant scarring which may not be appreciated by the patient. I would prefer to be more conservative. Regular follow ups, educate the family to look for signs of malignancy and biopsy before deciding to excise any part of it. Good Luck.

Ilene Rothman MD, New York, NY, USA on April 22, 2006

I would consider an MRI for 2 reasons; 1) rule out neurocutaneous melanosis; if present, it may decrease the need to try to remove the bulk of the skin lesions as the neural portion would still remain unaccessable; and 2) to rule out the small but real chance of underlying spinal dysraphism. We are seeing a patient currently with a giant congenital nevus on the lower back (not bathing trunk distribution) who proved to have a tethered cord diagnosed on MRI.

Please Click Here To Comment and Evaluate

Back to February 12 2006 Case