THE CLINICAL PICTURE Andrea Tan, MD Daniel Gutierrez, MD Nooshin K. Brinster, MD Jennifer A. Stein, MD, PhD The Ronald O. Perelman Department of The Ronald O. Perelman Department of The Ronald O. Perelman The Ronald O. Perelman Department of , New York University Grossman Dermatology, New York University Department of Dermatology, Dermatology, New York University Grossman School of , New York, NY; Grossman School of Medicine, New York University Grossman School of School of Medicine, New York, NY University of Texas Southwestern Medical New York, NY Medicine, New York, NY Center, Dallas, TX

Polypoid mistaken for verruca vulgaris

throughout the dermis (Figure 2A) • Focal epidermal contiguity with atypical melanocytes arranged in single cells and nests in the epidermis (Figure 2B) • A mitotic rate of greater than 30 mitoses/ mm2 (Figure 2C) • Lymphovascular invasion (Figure 2D) • Melanocytes highlighted by S-100 protein on immunohistochemical staining. These fi ndings were diagnostic of mela- noma, specifi cally the polypoid variant, with a tumor thickness of 5 mm. The patient was referred for wide local ex- cision with sentinel lymph node biopsy, which demonstrated inguinal node involvement and BRAF mutation on immunostaining. Positron- Figure 1. A solitary ulcerated, purple-red polypoid nodule emission tomography–computed tomography, with overlying serous crust on the lower mid-back. magnetic resonance imaging, and computed tomography of the head, chest, abdomen, and 30-year-old man presented with a pelvis were unrevealing. The formal diagnosis A 6-month history of an exophytic mass was stage IIIC disease (T4bN1M0). The pa- growing on the lower mid-back (Figure 1). It tient began immunotherapy with nivolumab. was initially suspected to be verruca vulgaris, He was treated with this drug for 1 year and and he had been referred to a specialty clinic now is on active surveillance. for sexually transmitted infections to confi rm it. The lesion was occasionally tender to pal- ■ IMPORTANCE OF ACCURATE pation and bled spontaneously. DIAGNOSIS Physical examination revealed a 1.5-cm ul- Polypoid melanoma is a rare clinical variant cerated, violaceous-to-erythematous polypoid of nodular melanoma characterized by an exo- nodule with overlying serous crust on the low- phytic mass with frequent ulceration, young er back. No pigmentation was noted within age at onset (20 to 39 years), and poor prog- the mass or adjacent to the base of the lesion. nosis.1,2 It has been reported to account for 2% Histopathologic examination of a shave bi- to 43% of all , with the wide vari- opsy specimen revealed: ability attributed to discrepancies in clinico- • An exophytic polypoid lesion with broad pathologic criteria used in different reports.1 ulceration, composed of markedly atypi- Lesions can affect the mucosa of the up- cal melanocytes in a sheet-like pattern per respiratory tract, esophagus, and anorectal junction, although cutaneous lesions are most doi:10.3949/ccjm.87a.19079 frequently on the back.2–5 Polypoid melano-

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A B

C D

Figure 2. Histopathologic images show (A) an exophytic polyp with ulceration and atypical melanocytes ar- ranged in a sheet-like pattern throughout the dermis (hematoxylin and eosin [H&E] stain, original magni- fi cation × 2) and (B) focal epidermal contiguity with atypical melanocytes arranged in single cells and nests in the epidermis (H&E stain, original magnifi cation × 20). High-power images (H&E stain, original mag- nifi cation × 40) show (C) confl uent, atypical melanocytes with conspicuous mitoses and focal cytoplasmic melanin, and (D) lymphovascular invasion at the periphery of the lesion. mas have a propensity for ulceration, rapid nodular melanoma and 77% for superfi cial progression over several weeks to months, and spreading melanoma.2,5 early metastasis to the lymph nodes, followed by possible metastasis to distant sites such as Potential for misdiagnosis the skin, brain, liver, and subcutaneous soft Polypoid melanoma is often misdiagnosed, as tissue.2,4 it may be confused with benign skin conditions Of the melanoma variants, polypoid mela- such as verruca vulgaris, leading to inappropri- noma has the poorest prognosis, given the ate treatment with cryotherapy or electrodes- risk of regional lymphatic and distal micro- sication and curettage. Other conditions to metastatic involvement, often attributed to consider in the differential diagnosis include increased tumor thickness and ulceration at pyogenic granuloma, keratoacanthoma, and presentation.2–4 The 5-year survival rate for infarcted intradermal nevi or acrochordons, as the polypoid nodular variant ranges from 32% these lesion types may similarly present with to 42%, compared with 57% for nonpolypoid small protruding or dome-shaped papules.

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Although these lesions often have distin- ■ MANAGEMENT guishing features, such as central hyperkerato- Because polypoid melanoma may closely mim- sis in keratoacanthomas or the characteristic ic benign lesions, patients with suspect lesions collarette of acanthotic epidermis at the base should be referred to dermatology for evalua- of pyogenic granulomas, polypoid melanomas tion and biopsy as soon as possible. If timely may still be diffi cult to diagnose clinically, giv- dermatologic care is unavailable, priority en that both pedunculated and sessile forms should be given to biopsy techniques yielding exist, as well as both pigmented and amelanot- adequate material for histopathologic analysis, ic variants. Additionally, ulceration may have such as a shave biopsy. Destructive treatments many causes and can be seen in both benign such as cryotherapy, used to manage benign and malignant growths. It may also obscure lesions with similar appearances, should be clinical presentation of cutaneous neoplasms, avoided, as inappropriate treatment may delay and tissue biopsy with histopathologic review accurate diagnosis and management. should be considered for diagnostic guidance Management of polypoid melanoma begins in such cases. with prompt surgical excision. If it is not di- Given the highly aggressive clinical behav- agnosed early, sentinel lymph node biopsy and ior and poor prognosis of polypoid melanoma, imaging studies may be needed to assess for dis- clinicians should maintain a low threshold for ease progression. Additional depends removal of rapidly growing pedunculated le- on extent of the disease and can include immu- sions with histopathologic evaluation, espe- notherapy with immune checkpoint inhibitors cially if the patient is relatively young and the or targeted therapy, such as BRAF and MEK lesion has progressed quickly or has ulcerated. inhibitors.  ■ REFERENCES 4. Plotnick H, Rachmaninoff N, VandenBerg HJ Jr. Polypoid melanoma: a virulent variant of nodular melanoma. 1. Cutler K, Chu P, Levin M, Wallack M, Don PC, Weinberg Report of three cases and literature review. J Am Acad JM. Pedunculated malignant melanoma. Dermatol Surg Dermatol 1990; 23(5 pt 1):880–884. 2000; 26(2):127–129. doi:10.1016/0190-9622(90)70309-6 doi:10.1046/j.1524-4725.2000.99092.x 5. Manci EA, Balch CM, Murad TM, Soong SJ. Polypoid 2. Dini M, Quercioli F, Caldarella V, Gaetano M, Franchi A, melanoma, a virulent variant of the nodular growth pat- Agostini T. Head and neck polypoid melanoma. J Cranio- tern. Am J Clin Pathol 1981; 75(6):810–815. fac Surg 2012; 23(1):e23–e25. doi:10.1093/ajcp/75.6.810 doi:10.1097/SCS.0b013e3182420801 3. McGovern VJ, Shaw HM, Milton GW. Prognostic Address: Daniel Gutierrez, MD, The Ronald O. Perelman signifi cance of a polypoid confi guration in malig- Department of Dermatology, New York University School of nant melanoma. Histopathology 1983; 7(5):663–672. Medicine, 240 East 38th Street, New York, NY 10016; doi:10.1111/j.1365-2559.1983.tb02278.x [email protected]

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