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Tumor Discovery                                                  Aggressive sebaceous carcinoma of the scalp



            pathogenic  germline  variants  of  DNA mismatch repair   Clear cell squamous cell carcinoma, on the other
            genes MSH2, MSH6, and MLH1 have been identified in 8 –   hand, shows clear cells; however, their cytoplasm is
            29% of individuals with SC . In these patients, the tumors   not as multivacuolated as sebocytes. Without lobular
                                 [7]
            are characterized by microsatellite instability.   arrangement, it is difficult to determine sebaceous
                                                                                                 [11]
              On microscopy, sebaceous neoplasm shows a wide   differentiation in squamous cell carcinoma .
            range of differentiation: Sebaceous adenoma, basal cell   On immunohistochemistry, SC tumor cells are reactive
            carcinoma with sebaceous differentiation, and sebaceous   for epithelial membrane antigen (EMA), cytokeratin,
            carcinoma . In SC, the tumor cells are arranged in cords   Ber-EP4, and adipophilin (adipose differentiation-related
                    [8]
            and lobules, the neoplastic cells show varying degrees of   protein, ADP), which has a membranous vesicular pattern,
            sebaceous differentiation, and there is tumor infiltration to   and androgen receptor . Androgen receptors and ADPs
                                                                                 [12]
            adjacent soft tissue, nerve, or the lymphatic system. Less   are not observed in squamous cell carcinoma, but EMA,
            commonly, it exhibits a broad superficial intraepidermal   cancer antigen (CA)15-3, Ber-EP4, and ADP are observed
            pattern.                                           in basal cell carcinoma, thus differentiating them from SC.
              The  architecture  usually  consists  of  sheets  or  lobules   Immunohistochemistry can also be used to evaluate MSH2,
            separated by fibrovascular stroma. The various histologic   MSH6, MLH1, and PMS2 for microsatellite instability in
            patterns of SC include lobular, cystic with the central   SC, as SC may metastasize in  2.4% of cases. According
            comedo-type  necrosis,  papillary, and mixed type. The   to the literature, local recurrence is more common in
                                                                           [12]
            atypical sebocytes may be well, moderately, or poorly   extraocular SC .
            differentiated. They are arranged as rounded nodular   One of the features that is indicative of poor prognosis
            aggregates or angulated infiltrative aggregates. A  well-  is tumor  size >1  cm  (associated with a 5-year  mortality
            differentiated SC shows increased proportion of mature   rate of 50%). The other features include moderate-to-
            appearing multivacuolated sebocytes with nuclear   poor sebaceous differentiation, tumor necrosis, increased
            indentation, mild nuclear pleomorphism, and minimal   mitotic activity, infiltrative growth, and lymphovascular
            mitoses and necrosis. In contrast, anaplastic cells, with   invasion.
            prominent nuclear pleomorphism and frequent mitoses   For local disease, wide local excision is the preferred
            and necrosis, are observed in moderate to poorly
            differentiated SC. In terms of tumor dissemination, these   treatment. Other modalities of treatment include
                                                               Mohs  micrographic  surgery,  radiation,  and  systemic
            tumors may occasionally spread in a pagetoid manner .   chemotherapy, which may be considered for recurrent or
                                                        [9]
            Tumor multicentricity, differentiation, pagetoid spread,   metastatic disease .
                                                                             [13]
            and perineural, vascular,  and  lymphatic  invasion  should
            be emphasized in pathological reporting since this   Bailet et al. have reported a local recurrence rate of 29%,
            information can aid clinicians in treating patients with SC.  regional nodal metastasis in 15%, and a disease-related
                                                               mortality of 20% . It has also been reported that the 5-year
                                                                            [9]
              Sebaceous adenoma, basal cell carcinoma with
            sebaceous differentiation, clear cell melanoma, clear cell   survival rate of this SC is 92.7%. SC is primarily treated
                                                               with wide local excision, and in cases of localized tumor,
            squamous cell carcinoma, clear cell hidradenocarcinoma,
            metastatic renal cell carcinoma, and prostate carcinoma   the prognosis is good following surgical removal. However,
            are among the benign and malignant conditions that are   in scalp SC, adjuvant treatment with radiation and
            considered differential diagnoses for SC .         chemotherapy is required. A study conducted by Angela
                                            [10]
                                                               Orcurto et al. noted the recurrence of aggressive SC of the
              Sebaceous adenoma is a benign epithelial neoplasm   scalp even after multiple excisions and local radiotherapy .
                                                                                                           [14]
            with hyperplasia of sebaceous lobules associated with   In another study, Bhavaraju noted aggressive SC over the
            expansive aggregates of basaloid germinative cells. It is a   scalp and suggested the need for close follow-up of these
            well circumscribed neoplasm that principally demonstrates   patients to  detect  recurrence  and distant  metastasis .
                                                                                                           [15]
            organoid and lobular configuration and contains a significant   In our case, the patient was asymptomatic without any
            percentage of mature and lipid-rich sebaceous cells.  evidence of locoregional recurrence on follow-up.
              Another differential is basal cell carcinoma with
            sebaceous differentiation. Its tumor cells are small basaloid   4. Conclusion
            with peripheral palisading, surrounded by fibromyxoid   Extraocular SC is an aggressive malignant neoplasm of the
            stroma, with focal differentiation toward mature, benign-  skin. The management of SC is challenging and patient
            appearing, and multivacuolated sebocytes. In contrast,   assessment may be necessary depending on the prognostic
            there is no peripheral palisading in SC.           features. We present a case of SC of the scalp along with


            Volume 1 Issue 2 (2022)                         3                       https://doi.org/10.36922/td.v1i2.203
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