SKIN May 2021 Volume 5 Issue 3 Copyright 2021 The National Society for Cutaneous Medicine 316 SHORT COMMUNICATION Multifaceted Facial Tumor Posing a Therapeutic Challenge Brittany Urso, MD1, Vivian Laquer, MD1, Joseph Hillman, MD2, Christopher Zachary, MBBS FRCP1, Janellen Smith, MD1 1Department of Dermatology, University of California Irvine, Irvine, CA 2St. Joseph Mission Hospital, Mission Viejo, CA Nevus sebaceous (NS) are benign congenital hamartomas which primarily affect the head and neck. Most lesions occur sporadically without any familial predisposition; however, NS can be associated with Schimmelpenning syndrome or phakomatosis pigmentokeratotica. At birth, they present as a thin alopecic plaque, but post-pubertally they thicken and become verrucous due to hormonal influences. In late adulthood, secondary appendageal benign and malignant neoplasms, such as trichoblastoma (TBL), syringocystadenoma papilliferum (SPAP), and basal cell carcinoma (BCC), may arise.1 While NS are associated with multiple neoplasms, especially when located on the scalp, they rarely present simultaneously within the same lesion on the face.1,2 Herein, we report an unusual presentation of a NS with numerous concomitant neoplasms. A 58-year-old Vietnamese male presented to clinic for evaluation of a birthmark on his left cheek which has been enlarging over a two-month period. He also reported intermittent bleeding pink and black bumps within the birthmark. Physical exam revealed a 5x8 cm verrucous plaque studded with pink to brown nodules on the left pre- auricular cheek, extending into the lateral hair line (Figure 1A). No lymphadenopathy was noted. Scouting biopsies revealed nodulo- infiltrative BCC and MRI showed no evidence of deep invasion of the tumor or lymphadenopathy. Due to cultural attitudes, he declined surgery under any type of sedation or general anesthesia, but eventually opted for Mohs micrographic surgery. Complete excision revealed the additional presence of TBL, SPAP, BCC, and a trichoblastic carcinoma (Figure 2). The surgical defect could not be closed primarily so it was partially closed leaving the rest to heal by secondary intention. The scar was later revised with intralesional steroids and laser therapy (Figure 1B). Our case is interesting due to the patient’s ethnicity, the presence of multiple and unusual tumors, and the large size of the lesion. Though skin cancers may develop within a NS, among certain populations, it is rare. A recent review of the Taiwanese population showed that malignant conversion in NS is uncommon at about 0.9%, and usually involves BCC.3 In the general population, BCC is the most common malignancy in NS, with an incidence of 1%4 Only once has a trichoblastic carcinoma been reported arising in a NS.5 Surgical intervention was curative for this patient but involved a large surgical defect that required a thoughtful SKIN May 2021 Volume 5 Issue 3 Copyright 2021 The National Society for Cutaneous Medicine 317 Figure 1. (A) Clinical image showing a large verrucous plaque studded with pink and pigmented nodules involving the majority of the left face. (B) Clinical image of well-healed surgical scar. Figure 2. Nevus sebaceous with trichoblastic carcinoma. Microscopic image showing a large tumor composed of atypical pleomorphic basaloid epithelial cells with follicular differentiation and peripheral palisading. approach for ethnic, psychological, and practical reasons. Coming from a rural society in Vietnam where the need for general anesthesia is tantamount to a death sentence, this patient was reluctant. Fortunately, dermatologic surgeons routinely remove large tumors under local anesthesia and large defects can be repaired using many techniques including a combined closure as in this case. We hope this case emphasizes the importance of cultural sensitivity in patient care, highlights the various tumors associated with NS, and encourages providers to biopsy NS readily for early skin cancer detection. Conflict of Interest Disclosures: None Funding: None Corresponding Author: Brittany Urso, MD Department of Dermatology University of California Irvine 118 Med Surg I, Irvine, CA 92697 Phone: 949-824-5515 Fax: 949-824-7454 E-mail: burso@hs.uci.edu SKIN May 2021 Volume 5 Issue 3 Copyright 2021 The National Society for Cutaneous Medicine 318 References: 1. Namiki T, Miura K, Ueno M, et al. Four Different Tumors Arising in a Nevus Sebaceous. Case Rep Dermatol. 2016;8(1):75-79. (PMID 27194974) 2. Cribier B, Scrivener Y, Grosshans E. Tumors arising in nevus sebaceous: A study of 596 cases. J Am Acad Dermatol. 2000 Feb; 42 (2 Pt 1):263-8. (PMID 10642683) 3. Hsu MC, Liau JY, Hong JL, et al. Secondary neoplasms arising from nevus sebaceus: A retrospective study of 450 cases in Taiwan. J Dermatol. 2016;43(2):175-180. (PMID 26361884) 4. Watson IT, DeCrescenzo A, Paek SY. Basal cell carcinoma within nevus sebaceous of the trunk. Baylor University Medical Center Proceedings. 2019; 32(3): 392-393. (PMID 31384196) 5. Kim J, Lee S, Cho P, et al. Trichoblastic Carcinoma Arising from a Nevus Sebaceous. Arch Plast Surg. 2016;43(3):297- 299. (PMID 27218034)