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Childhood Melanoma PowerPoint Presentation

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Description : Check out this medical presentation on Familial Malignant Melanoma, which is titled "Childhood Melanoma", to know about pediatric melanoma.

Tags : Familial Malignant Melanoma, Childhood Melanoma, pediatric melanoma

Published on : May 18, 2021 | Views : 1566 | Downloads : 2

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Childhood Melanoma Bhaskar N. Rao, MD Department of Surgery March 3, 2006 St. Jude Children’s Research Hospital
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☼ Childhood Melanoma Is pediatric melanoma different from adult melanoma?
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☼ Childhood Melanoma Most virulent of skin malignancies Estimated 26,000 new cases in 1996 Represents 1-3% of all malignancies Less than 1% in children (@ 300 / yr) Second to Thyroid cancer (Adult)
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☼ Childhood Melanoma Adult vs. Pediatric Similar sex distribution Similar anatomic location Thicker lesions Prognosis is stage dependent
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☼ Childhood Melanoma Reported Series 588 patients caucasians 80% second decade of life Extremity most common site 75% localized disease Predisposing conditions in 20% Nodular melanoma in 50% Prognosis is stage dependent
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☼ Childhood Melanoma St Jude Experience Over 2/3 of children presented with intermediate to thick melanomas (> 1.5 mm) Incidence of nodal disease vary with depth of invasion
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☼ Childhood Melanoma Risk Factors Giant congenital nevi 2-3% Xeroderma pigmentosa 22-57% skin cancers Immunodefficiency Hodgkins disease 8 fold Other (HIV / Transplant) 4 to 8 fold Radiation therapy 44% of melanoma occur in nevi present at birth Neurocutaneous melanosis
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☼ Childhood Melanoma
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Copyright restrictions may apply. ABCDE Features of Cutaneous Melanoma Abbasi, N. R. et al. JAMA 2004;292:2771-2776. Asymmetry - Focal growth - Ulceration - Tenderness - Dark pigmentation - Pruritis - Bleeding Evolving Color Variegation Diameter > 6 mm Border Irregularity
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☼ Childhood Melanoma
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☼ Childhood Melanoma
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☼ Childhood Melanoma Histologic Parameters of Prognostic Significance Clark’s level of Invasion: Difficult to reproduce Breslow’s thickness: Measured from granular layer of epidermis to greatest depth of invasion Ulceration Mitotic rate Vascular invasion Lymphatic infiltration
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☼ Childhood Melanoma Mole phenotype 1% of newborns have nevi 15% of patients will say nevi were at the site of melanoma MM assoc with cong. nevi occurs before puberty Role of solar exposure Dysplastic nevi in 5-10% are larger than 5 mm (familial / non familial) risk of MM 5-10% Germline mutations
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☼ Childhood Melanoma Congenital Nevi May develop in utero in absence of maternal melanoma Fetus from Giant mel nevus or denovo Transplacental transmission (46%) Latter have disseminated disease
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☼ Childhood Melanoma Giant Pigmented Nevi Size of palm (face) or twice elsewhere Surgery and primary closure not feasible Dark brown / black and Irregular surface / margins Many have coarse terminal hairs Melanoma risks 2-30% (Mean 12%) a.) Staged excision Surgery b.) Tissue expanders c.) Tissue culture d.) dermabrasion
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Incidence ~1 in 500,000 newborns Appearance Pigmented Hairy Cerebriform appearance (corrugated) Nodular ☼ Childhood Melanoma Giant/Large CMN
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Natural history Expands with growth Change in color, topography Surface pigment may fade with time Hair growth Limb discrepency Associations Scoliosis, spina bifida, clubfoot ☼ Childhood Melanoma Giant/Large CMN
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☼ Childhood Melanoma Giant/Large CMN
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Malignant transformation Cutaneous and non-cutaneous Melanoma is most common, but also Rhabdomyosarcoma, neuroblastoma, liposarcoma, MPNST, neurofibroma Presenting symptoms ☼ Childhood Melanoma Giant/Large CMN
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☼ Childhood Melanoma Dysplastic Nevus Variety of compound nevus seen in Familial Dysplastic Nevus Syndrome Large number irregularly bordered nevi Commonly seen on the back Usually greater than 5 mm with irregular pigmentation and margins Lifetime risk approx. 10%
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☼ Childhood Melanoma Dysplastic Nevus
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☼ Childhood Melanoma Management of Dysplastic Nevus Syndrome Thorough exam and photography Patient education Regular follow-up Screen first degree relatives over the age of ten Excisional biopsy of representative suspicious lesions (scalp and difficult areas
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☼ Childhood Melanoma Xeroderma Pigmentosa Autosomal recessive, photosensitivity, > 1000 fold of skin ca Results from defect - XPA - XPG - XPV Malignant skin ca develops in 70% (median 8 yrs) 57% Squamous or Basal cell 22% melanomas Majority head and neck and exposed areas Prevent sunlight exposure, clothing, sunscreens (preventative)
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Incidence Rare Non-familial females = males ~100 cases described Risk factors Satellite nevi (> 10) Cutaneous nevus in midline overlying the trunk or calvarium ☼ Childhood Melanoma Neurocutaneous Melanosis (NCM)
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Prognosis Symptomatic – death within 2-3 years from dx Asymptomatic – lifetime risk of CNS melanoma = cutaneous melanoma Treatment Supportive No effective treatment ☼ Childhood Melanoma Neurocutaneous Melanosis (NCM)
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Surgical resection of primary tumor <1mm thick and no ulceration and no Clark level IV or V Adequate margins (1cm) Follow clinically < 1mm thick and ulceration or Clark’s IV or V or any lesion >1mm Sentinel LN biopsy + - WLE (2 cm margin), LN Dissection and Metastatic w/u WLE (2 cm margin) Consider interferon therapy - Stage III Stage Ib/II Stage Ia Follow clinically or consider clinical trial + Clinical trial IL-2 DTIC or TMZ Biochemotherapy Stage IV ☼ Childhood Melanoma Management
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☼ Childhood Melanoma SurgicalManagement Suspicious lesion Excisional biopsy Insitu Simple excision <1 mm 1 cm margin 1-4 mm 2 cm margin Two thirds (> 1.5 mm) have nodal involvement Do MRI, lymphoscintigraphy and sentinel node biopsy
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☼ Childhood Melanoma SurgicalManagement
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☼ Childhood Melanoma SurgicalManagement
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☼ Childhood Melanoma SurgicalManagement
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☼ Childhood Melanoma SurgicalManagement
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☼ Childhood Melanoma SurgicalManagement
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☼ Childhood Melanoma Role of Pediatrician and Surgeon Prevention Awareness Recognition Research
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