Chronic Malignant Acanthosis Nigricans Lauren Becker, MD John Fenyk, MD Daniel Miller, MD
Chronic Malignant Acanthosis Nigricans
Lauren Becker, MDJohn Fenyk, MDDaniel Miller, MD
We have no financial disclosures
Clinical presentation
• 40‐year‐old Somali male• Presented to dermatology clinic one month after immigrating to Minneapolis from an Ethiopian refugee camp
• Described 8 year history of progressive bumps on head, neck, extremities and mucosae
• Stiff hands and tongue, decreased appetite• Feels otherwise well, socially embarrassed
Aug Sept Oct Nov Dec Jan Feb March April2015 2016
Clinical presentation
• Family History– 2 children in Ethiopia, healthy– No history of renal or GI cancer
• Social History– Estranged from wife and children due to social stigma– Non‐smoker
• Medications– None at time of initial presentation
Clinical impression• Cutaneous/mucosal papillomas• Acanthosis nigricans‐like intertriginousplaques
• Velvety palms
Clinical impression: differential diagnosis
• Cutaneous/mucosal papillomas– Verruca vulgaris– Epidermodysplasia verruciformis– Malignancy associated
• Acanthosis nigricans‐like intertriginousplaques
• Velvety palms
Clinical impression: differential diagnosis• Cutaneous/mucosal papillomas
– Verruca vulgaris– Epidermodysplasia verruciformis– Malignancy associated
• Acanthosis nigricans‐like intertriginous plaques– Acanthosis nigricans (AN)
• Metabolic disease• Syndromic AN• Autoimmune AN• Familial• Malignancy associated
– Confluent and reticulated papillomatosis– Intertriginous granular parakeratosis
• Velvety palms
Clinical impression: differential diagnosis• Cutaneous/mucosal papillomas
– Verruca vulgaris– Epidermodysplasia verruciformis– Malignancy associated
• Acanthosis nigricans‐like intertriginous plaques– Acanthosis nigricans (AN)
• Metabolic disease• Syndromic AN• Autoimmune AN• Familial• Malignancy associated
– Confluent and reticulated papillomatosis– Intertriginous granular parakeratosis
• Velvety palms– Malignancy associated– Severe form of acrokeratosis verruciformis of Hopf
Impression & Plan
• Strongly suspecting malignancy associated process
• Biopsy to rule out HPV• Initiate laboratory and systemic malignancy work up
Aug Sept Oct Nov Dec Jan Feb March April2015 2016
Plan
• Ruled out HPV
• Initiate search for underlying malignancy
Aug Sept Oct Nov Dec Jan Feb March April2015 2016
Plan
• Ruled out HPV
• Initiate search for underlying malignancy– CT of chest, abdomen, pelvis unrevealing– CBC with absolute eosinophils 1.09
Plan• Ruled out HPV• Initiate search for underlying malignancy
– CT of chest, abdomen, pelvis unrevealing– CBC with absolute eosinophils 1.09
• Positive Schistosoma IgG 0.38• Tx: Praziquantel 12/2015
• Referred to oncology and gastroenterology
Further workup and management• Ruled out HPV• Initiate search for underlying malignancy
– CT of chest, abdomen, pelvis unrevealing– Upper endoscopy and colonoscopy with normal biopsies– CBC with absolute eosinophils 1.09
• Positive Schistosoma IgG 0.38• Tx: Praziquantel 12/2015
• Symptomatic referrals:– Ophthalmology, otolaryngology
• Multidisciplinary approach to care:– Oncology following q 6 months– GI with negative scopes– Urology consult secondary to positive Schistosomiasis
• Ongoing malignancy surveillance for 9 months was negative
AcanthosisNigricans
Florid cutaneous(mucosal)
papillomatosis
Tripe palms
Initial presentation 4 month followupAcitretin 50 mg/day
Initial presentation 4 month followupAcitretin 50 mg/day
Urologic evaluation• 5/20/16 cystoscopy
– Occult papillary tumor at the left trigone– Not identified on prior CT C/A/P– Bx = papillary urothelial carcinoma, low grade– Mitomycin D intraoperative treatment
• 11/30/16: persistent tumor OR fulguration • 12/22/16: BCG instillation q6weeks• 3/2017: Followup cystoscopy planned
Aug Sept Oct Nov Dec Jan Feb March April May2015 2016
6 weeks status‐post intraoperative mitomycin D injection of low‐grade papillary urothelial carcinoma
Initial presentation 6 weeks status‐postmitomycin C
Discussion• Florid cutaneous papillomatosis
– Also known as Schwartz‐Burgess Syndrome– Most commonly associated with gastric adenocarcinoma
• Malignant acanthosis nigricans– Most commonly associated with gastric adenocarcinoma
• Tripe palms– GI (30%), lung (20%)
Discussion• Bladder urothelial carcinoma is also known as transitional cell carcinoma (TCC)
• Most common bladder cancer in Europe and USA
• Risk factors: cigarette smoking, chemical and environmental exposures
• Association between S. haematobium and bladder cancer– Most commonly high‐grade SCC > noninvasive TCC
Discussion• Review of the literature:
– Malignant AN + tripe palms + papillomatosis = rare– Reported in renal urothelial carcinomahowever never in bladder carcinoma
• The longest duration to diagnosis previously reported was 2 years
• The stimulating factor is hypothesized to be a substance secreted by the tumor– Transforming growth factor (TGF)‐alpha is structurally similar to epidermal growth factor
• FGFR3 has been shown to be mutated in more than 30% of bladder TCC which is also mutated in AN
Teaching points• Florid mucocutaneous papillomatosis, acanthosis nigricans, and tripe palms occurring in association with bladder urothelial carcinoma
• Rare to not have rapid onset• Highlights possibility of oral retinoids and palliative surgery for treatment of papillomatosis
Many Thanks
• Pathology, Oncology, Ophthalmology, Otolaryngology, Gastroenterology and Urology departments at Hennepin County Medical Center
• Minnesota Dermatological Society• Dr. Joseph Scherman• Dr. Ingrid Polcari
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