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A patient underwent a modified temporal bone resection and total auriculectomy to remove a sebaceous carcinoma arising from the tragus of the right auricle that extended into the external auditory meatus. Because the tumor extended into the temporal bone, the osseous sites originally selected for immediate placement of the craniofacial implants were resected and no longer available. Subsequently, after an appropriate period of healing, implants were placed into the residual temporal bone, considerably more superior and posterior to the external auditory meatus such that they emerged through hair-bearing skin. The purpose of this report was to describe the fabrication of an implant-retained auricular prosthesis with implants in suboptimal positions. Challenges included optimization of the stability and retention of the prosthesis, effectively managing the prosthetic space without compromising the esthetic outcome, providing adequate hygiene access for the implant connecting bar, and maintaining the health of the peri-implant tissues when the implant emerged through hair-bearing skin.

AimsTo determine the distribution of three different intraepithelial growth patterns (pagetoid, bowenoid and papillary) in eyelid sebaceous carcinoma (SC) and correlate them with the clinical characteristics and prognosis.MethodsA retrospective cohort study. The medical charts and pathological sections were retrospectively reviewed. All eligible patients were followed up for recurrence, metastasis and tumour-related mortality. The clinical significance of each intraepithelial growth pattern was determined by Cox regression.ResultsOf the 214 patients, 67 (31%) presented with intraepithelial invasion, among them, 34 (16%) were pagetoid, 27 (13%) were bowenoid and 6 (2.8%) were papillary. Patients of pagetoid intraepithelial spread showed significantly longer diagnostic delay (p=0.001) and more initial misdiagnoses of blepharitis (p=0.035). After a median follow-up period of 34.0 months, 67 (46%) patients in the non-intraepithelial group, 17 (50%) in the pagetoid group, 8 (30%) in the bowenoid group and 2 (33%) in the papillary group recurred. And 30 (20%) patients in the non-intraepithelial group, 9 (27%) in the pagetoid group and 4 (15%) in the bowenoid group developed metastasis. Moreover, 15 (10%) patients in the non-intraepithelial group, 6 (18%) in the pagetoid group and 1 (3.7%) in the bowenoid group died of SC. Cox regression indicated that pagetoid intraepithelial growth pattern was remarkably associated with increased chances of tumour-related mortality (HR=2.95, 95% CI 1.14 to 7.64, p=0.026).ConclusionsIntraepithelial tumour invasion was presented in nearly one third of patients with eyelid SC. Pagetoid intraepithelial neoplasia, the predominant growth pattern, significantly increased the risk of tumour-related mortality. Meticulous histopathological intraepithelial examination is recommended for every patient of eyelid SC. Special attention should be paid to those with pagetoid invasion, who may require more intensive managements.

Sebaceous carcinoma (SC) is a rare, aggressive malignancy. Most lesions occur in the periocular region, with few cases presenting with extraocular lesions. Here, we report a case of an 89-year-old woman with a 3-month history of a rapidly growing giant extraocular SC. The diagnosis was based on skin biopsy findings. We advised the patient to undergo surgical excision of the carcinoma. However, the patient and her family rejected our proposed surgical treatment, opting for topical traditional Chinese medicine as alternative treatment. The patient subsequently died of unknown causes at home, four months after the first visit. This case of treatment failure emphasizes the importance of early detection and treatment for these aggressive tumors.

Radiation therapy may be performed for a variety of cutaneous malignancies, depending on patient health status, tumor clinical and histologic features, patient preference, and resource availability. Dermatologists should be able to recognize the clinical scenarios in which radiation therapy is appropriate, as this may reduce morbidity, decrease risk of disease recurrence, and improve quality of life. The second article in this 2-part continuing medical education series focuses on the most common indications for radiation therapy in the treatment of basal cell carcinoma, cutaneous squamous cell carcinoma, dermatofibrosarcoma protuberans, Merkel cell carcinoma, Kaposi sarcoma, angiosarcoma, cutaneous lymphoma, melanoma, undifferentiated pleomorphic sarcoma, and sebaceous carcinoma.

Hordeolum and chalazion are the most frequent inflammatory eyelid tumors. They can occur in association with underlying diseases causing Meibomian gland dysfunction and/or chronic blepharitis. Due to the typical morphological features and clinical course, the diagnosis can mostly be clinically established. The majority of these lesions resolve spontaneously over time. In some instances, surgical intervention is unavoidable. In persistent, recurrent or clinically atypical cases malignant tumors must be excluded as a differential diagnosis by excisional biopsy and histopathological assessment.

Sebaceous carcinoma is a malignant neoplasm that usually arises in the sebaceous glands of the eyelids. Its pathogenesis is unknown; however, irradiation history, immunosuppression, and use of diuretics are known risk factors. The mainstay of treatment for sebaceous carcinoma of the eyelid is wide surgical resection with a safety margin of 5 to 6 mm, which often results in full-thickness defects. The reconstruction of a full-thickness defect of the eyelid should be approached using a three-lamella method: a mucosal component replacing the conjunctiva, a cartilage component for the tarsal plate, and a flap or skin graft for the skin of the eyelid. In this case, a fullthickness defect of the upper eyelid was reconstructed after tumor removal using a combination of a nasal septum chondromucosal composite graft and a forehead transposition flap, also known as a “Fricke flap.” The flap was designed to include a line of the eyebrow on the lower margin of the flap to replace the eyelash removed during tumor excision. The wound healed completely, without any early or late complications, and the outcome was satisfactory.

Sebaceous carcinoma is a rare, malignant tumor of the sebaceous glands. This is a case report of a 75-year-old man who presented with a right lower axillary mass which initially was considered to be a benign sebaceous cyst from sonography. The lesion rapidly changed in clinical and sonographic appearances and on histology was a sebaceous carcinoma. This unusual tumor accounts for less than 1% of all cutaneous malignant tumors, and the chest wall is a rare extraocular site with only nine cases described in the literature. Immunohistochemistry was negative for MSH2 and MSH6, and positive for MLH1 and PMS2, raising the possibility of Lynch or Muir-Torre Syndrome which is a known association with this rare tumor.

Nevus sebaceous (NS) is a benign tumor with the potential to develop secondary benign and malignant neoplasms. It is a rare phenomenon to develop 2 or more skin tumors in a single NS lesion. We report a case of multiple secondary tumors, such as sebaceoma, sebaceous carcinoma, syringocystadenoma papilliferum, and trichoblastoma, in a single NS lesion.

Background Numerous reports have described methods for repairing full-thickness upper eyelid defects using lower eyelid tissue. To avoid sacrificing the lower eyelid and thereby prevent lower eyelid sagging or dysfunction, we used innervated orbicularis oculi myocutaneous flaps harvested from the upper eyelid for upper eyelid reconstruction. Methods We modified a method reported by Moschella and Cordova for repairing full-thickness upper eyelid defects using innervated bipedicled orbicularis oculi myocutaneous flaps mobilized from the upper eyelid and labial mucosal grafts. First, we avoided the use of a Fricke flap, which is used in the original method to repair the myocutaneous flap donor site, since it can cause asymmetry of the eyebrows on both sides. Second, reconstruction of the double-fold eyelid was performed with buried sutures in the myocutaneous flap in secondary surgery, since the double eyelid fold of the original method is not necessary or is too wide for Asian people. Functional and cosmetic outcomes were assessed by six evaluators using postoperative photographs. Results Six patients with malignant upper eyelid tumours underwent repair of upper eyelid defects by our modified method. Donor sites of the myocutaneous flaps were repaired with a medial forehead flap in one patient with Merkel cell carcinoma, a lateral orbital flap in one patient with sebaceous carcinoma, and a two-stage skin graft in two patients with sebaceous carcinoma and basal cell carcinoma, respectively. The two remaining patients (one with sebaceous carcinoma and the other with basal cell carcinoma) required no donor site repair. Functional and cosmetic outcomes were good or excellent in most cases. Conclusion Our modified method for repairing full-thickness upper eyelid defects using innervated orbicularis oculi myocutaneous flaps resulted in optimal eyelid function and support, with morphologically satisfactory results including bilateral symmetry. This method provides a useful alternative to conventional methods of upper eyelid reconstruction.

Background: Collision tumors are rare clinical entities that two heterogeneous neoplasms are concurrently adjacent to each other at the same location. The association of a squamous cell carcinoma and a malignant adnexal tumor is even infrequent. Case presentation: The case of a 79-year-old woman having a slow-growing and painless tumor on the left lower eyelid was presented. The lesion, about 15 mm in diameter, was nodular, irregular, and yellow-discolored. Histopathologic and immunohistochemical findings disclosed concurrence of sebaceous carcinoma as well as squamous cell carcinoma in one specimen. Wide excision of the tumor with frozen section control and eyelid reconstruction were performed. Oncologic survey revealed no other lesion. At 6-month follow-up, no evidence of recurrence or metastasis was presented. Conclusion: Collision tumor composed of sebaceous carcinoma and squamous cell carcinoma in eyelid may be the first case described in the literature. For increasing accuracy of diagnosis and management, a thorough clinical examination and detailed histopathologic analysis, along with multidisciplinary discussion, are prerequisites.

Considering the low incidence rates of primary sebaceous carcinoma (SC) of extraorbital sites, let alone those occur in intraoral sites, clinicopathological characteristics and histogenesis are not fully understood. In the present case, a maxillary mass was presented and a low-grade malignant tumor was suspected according to the CT scans, preoperative FNA, and clinical conditions. Other carcinomas, including acinar cell carcinoma (ACC), basaloid cell carcinoma (BCC), SCC, mucoepidermoid carcinoma, and epithelial-myoepithelial carcinoma (EMC), were also considered before surgery. Due to the rare occurrence of SC and no preoperative suspects, a fresh sample was not kept. Sadly, thus cause those special stains (e.g., Oil Red O and Sudan IV) which form the primary basis for a diagnosis of SC in academic circles were missing. A comprehensive literature review identified only 10 cases of intraoral SC, of which the primary sites reported in the English literature were the buccal mucosa, mouth floor, upper labial mucosa, and tongue. Due to an absence of specific biomarkers and simulated characters, histochemistry and immunohistochemistry such as PAS, CK, EMA, p63, p53, S-100, calponin, CD117, Ki-67, a-SMA, and AR form the diagnostic standard of SC. Postoperative immunohistochemistry of our case showed S100(−), Ki-67(−), calponin(−), CD117(−), CK20(−), PAS(−), AR(+), CK(+), CK5/6(+), P53(+), P63(+), a-SMA (+). Thus the diagnosis of SC was finally made. Through discussing the findings of our case and reviewing literature, we present the histological features and discuss possible outstanding biomarkers of this neoplasm.

Purpose To investigate the usefulness of the reverse Hughes flap procedure combined with a sandwich graft of an acellular dermal matrix for reconstruction of large full-thickness defects of upper eyelids after cancer excision. Methods Clinical data were obtained from patients who underwent upper eyelid reconstruction using a reverse Hughes flap combined with a sandwich graft of an acellular dermal matrix (AlloDerm) as a tarsal substitute. The tarsoconjunctival flap of the donor lower eyelid was mobilized to reconstruct the posterior lamella, and acellular dermal matrix was grafted onto the tarsoconjunctival flap. A skin-orbicularis muscle flap superior to the defect was advanced to cover the acellular dermal matrix graft, followed by application of lid crease formation sutures to prevent postoperative entropion. The tarsoconjunctival pedicle was divided 3-8 weeks after the surgery. Results Six patients with sebaceous carcinoma were included, and all had ≥70% full-thickness upper eyelid defects after tumor excision. After a median follow-up of 40 months (range 6-62 months), all 6 showed satisfactory functional and cosmetic outcomes. Tumor recurrence, exposure keratopathy, upper eyelid entropion, persistent use of bandage contact lenses, lower eyelid deformity, and dermal matrix-related complications were not observed by the last follow-up. Conclusions Reverse Hughes flap combined with a sandwich graft of an acellular dermal matrix as a tarsal substitute was successful in reconstructing large upper eyelid defects. Acellular dermal matrix graft and lid crease formation sutures enhance marginal stability and prevent postoperative entropion of the reconstructed upper eyelid.

Sebaceous neoplasms are rare adnexal tumors that can present a challenge to clinicians. Only four cases of sebaceous carcinoma with sebaceoma have been reported in the literature. Herein, we describe the case of a sebaceous carcinoma originating from a sebaceoma in a solitary nodule of the posterior neck. Immunohistochemically, the tumor cells were strongly positive for epithelial membrane antigen and p53. It is possible that adnexal carcinomas may arise from malignant transformation of their benign counterparts as well as de novo. Malignant transformation was likely in this case because the lesion was composed of distinct benign and malignant components, and the benign component showed the typical histopathological features of sebaceoma. This case underscores the fact that partial and superficial biopsies sometimes may not provide the correct diagnosis. If a surgeon suspects malignancy based on a clinical examination, then it is mandatory to perform a deep biopsy.

Sebaceous carcinoma (SC) is a rare tumor, accounting for approximately 0.7% of skin cancers. SC can be classified as ocular SC (OSC) or extraocular SC (EOSC) depending on its location. Because EOSC accompanied by metastases is rare, there is a paucity of data about EOSC accompanied by metastasis. This study presents a case of an aggressive EOSC of the scalp with lymph node metastases. The patient underwent wide local excision of the primary tumor with a 1 cm safety margin and bilateral radical lymph node dissection. However, recurrence was observed 1 month after surgery. Radiation therapy and resection were performed for the recurrent tumor. However, distant metastases to both lungs eventually occurred. Here, we describe a rare recurrent case of EOSC of the scalp with distant organ metastasis with a review of the literature.

Sebaceous carcinoma is a rare malignant cutaneous neoplasm that is most commonly arises in the ocular region. Although it can occur in extraocular sites, sebaceous carcinoma is rarely encountered in the vulva. The use of immunohistochemical staining is very crucial to exclude other differential diagnoses including primary cutaneous and metastatic neoplasms. Unlike ocular sebaceous carcinoma, little is known about the clinical behavior and the prognostic factors in vulvar sebaceous carcinoma. Herein, we present a case of vulvar sebaceous carcinoma in a 27-year-old female, who presented with a labial tumor with lung metastases. To the best of our knowledge, only 11 similar cases were previously reported in the literature.

Sebaceous carcinoma (SC) is an uncommon and potentially aggressive adnexal neoplasm. SC presents most often as a periocular tumor arising from the ocular adnexa with unclear pathogenesis. Aggressive SC of the scalp is extremely rare. Here, we describe a rare case of aggressive SC of the scalp in a 61-year-old female, who presented with a scalp neoplasm lasting for two months. Cranial magnetic resonance imaging (MRI) revealed a partly necrotic and cystic tumor with mixed signal shadow. The parietal multiple destructive lesions invaded the skull, involved the dura mater, and compressed the associated superior sagittal sinus. A wide local lesion excision with I stage repair of dura mater and I stage bone graft of skull was performed in our department. The patient was treated with external radiation to prevent recurrence and was followed for 3 years with favorable results. The relevant literature regarding aggressive SC was reviewed, and the clinical manifestations, radiological characteristics, surgical strategies, histopathological findings, and prognosis are discussed. This cutaneous malignancy invaded skull, dura mater or brain, or metastasized to the lymph nodes and viscera, with high recurrence and fatality rates. It is necessary to be aware of these rare examples which showed unexpectedly clinical behaviors. Early precise diagnosis and improved radical treatment remain essential steps against aggressive cutaneous malignancy. Patients with aggressive SC of the scalp should be closely followed to assess recurrence and distant metastasis.

Cosmetics with unknown chemical components may cause unexpected cancers. Here, we report a rare case of hair dye-induced intracranial communicating scalp sebaceous carcinoma in a young female who dyed her hair 10 years ago. The histological origin of the intracranial communicating neoplasm was of priority for subsequent therapeutic decisions, therefore, requiring comprehensive diagnostic strategy. In conclusion, this case report emphasizes careful evaluation of the long-term risks for unlabeled cosmetics.

Background: Skin adnexal tumours are heterogenous group of uncommon tumours having distinct histological features. They have varied clinical presentation and morphological differentiation towards one or more types of adnexal structures found in normal skin. The aim of this study was to study the different histomorphological patterns of appendageal tumours and correlate them with clinical presentations. Materials and Methods: This was a retrospective study of three year duration of all cases clinically diagnosed to be appendageal tumours and confirmed by histopathology in our department. The tumours were classified according to their differentiation after a detailed histopathological examination in haematoxylin and eosin sections. Results: The total number of cases in the study were 43,in which 28 were males and 15 females. Most of the tumours (97.7 %)were benign and there was only one case sebaceous carcinoma. The majority of the tumours had an eccrine differentiation (30.9%), nodular hidradenoma among them being the most common lesion (20.9%). Trichilemmal cyst (30.2%) constituted the maximum cases of pilar origin. Conclusion: Skin adnexal tumours are relatively rare with benign ones being more common than the malignant tumours. Proper clinical correlation and histopathological study helps in reaching a correct diagnosis. Keywords: Appendageal, Skin tumour, Eccrine, Nodular hidradenoma, Sebaceous carcinoma.

OBJECTIVE: To analyze the prognosis of cutaneous adnexal malignancies, survival relative to surgical management, and utility of lymph-node biopsy. DESIGN: Population-based study of the SEER-18 database from 1975 to 2016. PARTICIPANTS: 7591 patients with sweat gland carcinoma, hidradenocarcinoma, spiradenocarcinoma, sclerosing sweat duct tumor/microcystic adnexal tumor (SSDT/MAC), porocarcinoma, eccrine adenocarcinoma, and sebaceous carcinoma RESULTS: Five-year OS ranged from 68.0 to 82.6%, while 5-year DSS ranged from 94.6 to 99.0%. The majority of patients were treated with narrow (42.4%) or wide local excision (16.9%). DSS at 5 years showed that patients with stage IV had significantly poorer survival (50.3%) than I, II, or III (99.3%, 97.8%, and 89.0% respectively). 5-year OS was significantly higher for narrow excision (excision with < 1 cm margin, 78.5%) than observation (65.0%), excisional biopsy (66.8%), or wide local excision (WLE, 73.2%). Lymph-node biopsy was performed in a minority of cases (8.1%) and patients showed no significant difference in survival based on nodal status. The sensitivity and specificity of lymph-node biopsy for all malignancies were 46% and 80%, respectively. The PPV and NPV for that group were 0.46 and 0.80, respectively. Invasion of deep extradermal structures was a poor predictor of nodal positivity. CONCLUSIONS: These malignancies have excellent DSS. Narrow excisions demonstrate better 5-year DSS and OS compared with WLE. Lymph-node biopsy is a poor predictor of survival in advanced stage disease and utility is limited.

Aim To evaluate the demographic features, location, and histopathologic results in primary eyelid tumors. Methods We retrospectively reviewed the records of patients with primary eyelid tumor diagnosed and treated between November 1997 and June 2016 on our service. Results Nine hundred and eleven lesions from 874 patients were included in this study. Four hundred and forty-six (51.0%) of the patients were females and 428 (49.0%) were males. The mean age was 51.0y (range: 3mo-94y). The lesions were located in the upper eyelid (418 cases, 45.9%), lower eyelid (378 cases, 41.5%), medial canthus (89 cases, 9.8%), and lateral canthus (26 cases, 2.8%). Four hundred and seventy (51.6%) lesions were located on the right side and 441 (48.4%) on the left. Of the eyelid lesions, 666 (73.1%) were benign, 230 (25.2%) malignant, and 15 (1.6%) premalignant. When the eyelid tumors were classified according to their tissue or cell of origin, 527 (57.8%) of the lesions were found to be epidermal, 171 (18.8%) adnexal, 131 (14.4%) inflammatory and infectious, and 65 (7.1%) stromal. The most common benign lesions were squamous cell papilloma (139 lesions, 15.2%), intradermal nevus (97 lesions, 10.6%), epidermoid inclusion cysts (78 lesions, 8.6%), seborrheic keratosis (60 lesions, 6.6%), and inflammatory masses (59 lesions, 6.5%). Basal cell carcinoma was the most common malignant tumor (191 lesions, 21.0%) followed by squamous cell carcinoma (16 lesions, 1.8%), sebaceous carcinoma (14 lesions, 1.5%), and malignant melanoma (5 lesions, 0.5%). Conclusion In this study, 73.1% of eyelid lesions are benign and the remaining 26.9% are premalignant and malignant. Basal cell carcinoma is the most common tumor among all histopathological diagnosis followed by squamous papilloma.

Purpose To evaluate the clinical features, diagnostic challenges, management, and prognosis of sebaceous carcinoma (SC) of the eyelids and periocular region in a Nordic country. Methods Patients were identified from the Finnish Cancer Registry and the Helsinki University Hospital databases during the 21-year period 1998-2018. Age, sex, location, clinical and histopathologic diagnosis, treatment and outcome were registered. Results Sebaceous carcinoma (SC) was diagnosed in 32 patients. The incidence was 0.6 per million. Median age at the time of histopathologic diagnosis was 74 years, and 72% of patients were women. Diagnostic delay was often long, median 12 months. The most common cause for delay was misdiagnosis (72%): a chalazion in 34% and a benign tumour in 22%. The most common location was the upper eyelid (53%) and tumour type a solitary nodule (94%). The SC was not correctly diagnosed in 12 (40%) of 30 preoperative biopsies. The treatment for 31 (97%) patients was complete surgical removal with reconstruction. Conjunctival intraepithelial growth was found in 50%. The leading postoperative problem was ocular irritation (30%). During a median follow-up of 58 months, two patients (6%) experienced a local recurrence and one patient died from metastatic SC. Conclusions The estimated incidence of SC in Finland was somewhat higher than in other Western countries. The diagnosis was often markedly delayed. Especially differentiation from chalazion continues to be essential. To improve outcomes, it is essential to inform the pathologist about the possibility of SC in eyelid biopsies and specimens and ideally submit them to an ophthalmic pathology service.

Sebaceous carcinoma of the male breast is an exceedingly rare occurrence. Literature is inadequate about the prognosis and treatment of such malignancies. We report histo-pathological and immuno-histochemical (IHC) findings in a case of sebaceous carcinoma of breast of a 60-year-old male. An initial biopsy suggested sebaceous carcinoma. Total mastectomy was performed, followed by local radiotherapy and adjuvant chemotherapy. The receptor status was triple negative. Pan cytokeratin and epithelial membrane antigen were positive, whereas S-100 was focally positive and HMB-45 and Melan A were negative. IHC is central to diagnosis and differentiation of these rare kind of tumours from the common invasive ductal carcinomas. The exact line of treatment following excision of the tumour is unclear. Local radiotherapy is generally indicated, whereas the role of adjuvant chemotherapy is uncertain. Wider reporting of such cases will help in widening the horizon of prognosis and management of these tumours.

Ocular adnexal sebaceous carcinoma (OASeC) is an aggressive eyelid carcinoma. Analysis of molecular-genetic drivers of this disease could reveal new prognostic markers and actionable targets for treatment. To identify somatically acquired genomic mutations in OASeC and explore their associations with metastasis, whole-exome sequencing on DNA extracted from retrospectively collected tumor samples was performed. Thirty-one patients in two orbital oncology centers with OASeC were included. Sequencing results were analyzed to detect mutations and explore their possible association with metastasis. The median patient age was 64 years. A total of 1780 candidate somatic mutations were identified with median mutation rate of 1.0/Mb (range, 0.2-13.6). The five most commonly mutated genes (as determined by MutSig; q value < 0.25) were TP53 (mutated in 22 cases), ZNF750 (13 cases), RB1 (12 cases), NOTCH1 (8 cases), and PCDH15 (5 cases). Mutations in ZNF750 or NOTCH1 pathway genes were present in 24 (77%) of the 31 cases; there was a trend toward mutual exclusivity of ZNF750 and NOTCH1 mutations. All eight tumors with NOTCH1 mutations also had TP53 and/or RB1 mutations. Four of the five PCDH15 mutations and all four PCDH15 missense mutations were identified in patients with metastatic disease, including one patient with distant metastasis and three with nodal metastasis. PCDH15 was significantly associated with metastasis (P = 0.01). We identified the most commonly mutated genes in a series of OASeCs and found a previously unreported mutation in OASeC, PCDH15 mutation, that was significantly associated with metastasis. NOTCH1 mutation is an actionable mutation; clinical trials targeting this mutation are available throughout the US and could be considered for patients with metastatic NOTCH1-mutant OASeC. TP53, ZNF750, RB1, and PCDH15 mutations are most likely loss-of-function mutations and may have diagnostic and prognostic importance.

Extraocular sebaceous carcinoma (ESC) is a rare appendiceal skin tumor. In contrast to ocular sebaceous carcinoma, information about the exact cellular architecture of these lesions is scarce and the histogenesis of ESC is unknown. Here, we extend our previous study and investigate 28 extraocular carcinomas in comparison to 54 benign sebaceous tumors and 8 cases of normal sebaceous glands using a broad spectrum of antibodies against p63, several keratins, adipophilin, EMA, Ki67, androgen receptor, and mismatch repair proteins. This observational study demonstrates that p63- and K5/14-positive basaloid cells are key cells in normal sebaceous gland and in all sebaceous tumors and that these basaloid cells give rise to EMA+, adipophilin+ sebocytes, and K5/14+, K7±, K10± ductal structures. Finally, about half of ESC is associated with superficial in situ neoplasia, which provides evidence that at least part of these carcinomas arises from flat superficial in situ carcinoma. In contrast to the normal sebaceous gland, about half of all sebaceous tumors lack keratin K7. MMR protein IHC-profiles role will be discussed.

Sebaceous carcinoma is a rare malignant skin neoplasm arising from sebaceous glands. Its clinical diagnosis is difficult and reports of dermoscopic findings have been limited. This study aims to analyze its dermoscopic features and differential diagnosis in dermoscopic examination. The study included patients diagnosed with histologically proven sebaceous carcinomas as well as diagnosed cases of sebaceous hyperplasia, sebaceoma, squamous cell carcinoma and basal cell carcinoma for comparison of dermoscopic findings. The dermoscopic criterion of presence of sebaceous carcinoma was scored only if the two evaluators reached a consensus. Fifteen cases of histologically diagnosed sebaceous carcinoma were included in our study. All cases were extraocular sebaceous carcinoma. A total of 60 (15 basal cell carcinomas, 15 squamous cell carcinomas, 15 sebaceous hyperplasias and 15 sebaceomas) cases were collected for comparing dermoscopic features with sebaceous carcinoma. In dermoscopic analysis of sebaceous carcinoma, the majority of tumors (66.67%) presented polymorphic vessel pattern. Other features included whitish-pink areas (80%), yellowish structures (73.33%) and yellowish structureless areas (60%). Yellowish structures in sebaceous carcinomas are the main dermoscopic findings to differentiate squamous cell and basal cell carcinomas (P < 0.001), whereas purplish globules, shiny white blotches and strands and whitish-pink area distinguish sebaceous carcinomas from other sebaceous tumors (P < 0.05).

Over several months, a painless, multinodular, non-erythematous swelling of the deep tissues of his left upper eyelid developed in a 63-year-old man. An excisional biopsy with histopathologic evaluation disclosed a unique sebaceous papilloma within a cyst lined by non-keratinizing squamous epithelium that focally displayed a variably thick, superficial, eosinophilic cuticular layer. Immunohistochemical staining demonstrated that the tumor and its epithelial cystic lining had a profile consistent with Meibomian gland duct epithelium. Adipophilin highlighted cytoplasmic vacuolar lipid positivity. The encapsulation of the lesion, absence of nuclear atypia, and Ki-67 nuclear positivity restricted to the basilar cells established its intrinsically benign nature. The patient's clinical history was remarkable for pulmonary and colonic carcinomas resected, respectively, 20 years and 8 years earlier. DNA mismatch repair protein expression studies disclosed loss of nuclear immunostaining of MSH6 protein, pointing to the possibility of an underlying rare MSH6 variant of the Muir-Torre syndrome, not yet described in the ophthalmic literature. p16 nuclear positivity was also found in the tumor cells, indicating the possible role of high-risk human papillomavirus as an additional factor in the genesis of the tumor. Genetic evaluation of normal and tumoral tissues in future similar cases will detect if there is an underlying germline mutation versus a somatic mutation limited to the tumor. This will be required to fully establish a predictable linkage with this new subtype of the Muir-Torre syndrome.

Background Sebaceous carcinoma (SC) is frequently classified as periocular or extraocular. Extraocular SC is rare and mainly occurs in the head and neck, the major salivary glands, or oral mucosa. SC of the breast, lung, and ovary is particularly rare, and the few cases of SC of the breast predominantly exhibit intraductal growth. Case presentation A 47-year-old Japanese woman was referred to our hospital with accumulated polymorphic calcification in the left breast which was detected using mammography. Ultrasonography revealed an irregular 13-mm mass in the left breast, and analysis of a core needle biopsy revealed noninvasive ductal carcinoma. Total mastectomy and sentinel lymph node biopsy were performed. Histopathology demonstrated that carcinoma in situ (CIS) represented a significant lesion, and the cytoplasm of tumor cells was clear with numerous minute vacuoles. Immunohistochemical analysis demonstrated that most tumor cells expressed adipophilin. Together, these findings led to a diagnosis of SC, mainly comprising CIS. Conclusions We encountered a rare case of SC of the breast with predominant CIS.

Purpose To report our 14-year experience with orbital exenteration and assess risk factors for poor prognosis by focusing on conjunctival melanoma. Patients and method A retrospective study was conducted in our tertiary care centre (Jules Gonin Eye Hospital, Lausanne, Switzerland) between 2003 and 2017. Inclusion criteria were patients aged ≥18 years with a follow-up >12 months, without metastatic spread at the time of surgery. Data recorded were age, gender, tumour histology, surgical technique, postoperative complications, surgical margin status, local recurrence, postoperative radiation beam therapy and metastatic status. Results Twenty-five patients with a mean age of 63.2 years (38–92) were included. Conjunctival melanoma was the most frequently identified tumour (n = 14, 56%) followed by conjunctival squamous cell carcinoma (n = 4, 16%), sebaceous carcinoma (n = 3, 12%), choroidal melanoma (n = 2, 8%) and basal cell carcinoma (n = 2, 8%). Eighteen tumours (72%) originated from the conjunctival tissue. Clear surgical margins were achieved in 21 (84%) patients. Fourteen (56%) patients experienced distant metastases and died from metastatic spread after a mean follow-up of 52.3 months (6–120). The 1-, 3- and 5-year overall survival (OS) was 96%, 72% and 60%, respectively. In the univariate analysis, positive surgical margins, local recurrence and metachronous metastases were associated with a decreased OS (p = 0.002, p = 0.005 and p = 0.007, respectively). In the multivariate analysis, positive surgical margins and metachronous metastases were also associated with a decreased OS (p = 0.02 and p = 0.042, respectively). Conjunctival melanoma was not associated with a poorer prognosis (p = 0.280). Conclusion Free surgical margins are needed to increase OS. To achieve clearer surgical margins, neoadjuvant targeted therapies/immunotherapies may be considered.

Purpose Sebaceous carcinoma can be highly malignant and difficult to treat. Surgical excision followed by periocular reconstruction is the primary method of treatment. In aggressive cases, radiation, topical chemotherapy, and systemic chemotherapy have been explored as adjuvant therapy. Immunotherapy, through immune checkpoint inhibitors, has proven to have significant antitumor effect in many cancer types, including melanoma, non-small cell lung cancer, renal cell carcinoma, and cutaneous squamous cell carcinoma. Little is known about endogenous immune response directed against sebaceous carcinoma. In this study, we aim to characterize the expression pattern of PD-1 and its ligands PD-L1 and PD-L2 in both sebaceous carcinoma and in infiltrating immune cells to explore the potential use of checkpoint blockade as therapy. Methods We performed a retrospective chart and histology review of patients with sebaceous carcinoma between 1990 and 2017 at the University of Wisconsin. Tissue microarrays were made from paraffin blocks. Immunohistochemistry was performed for evaluation of tumor and immune cell infiltration for expression of PD-1, PD-L1, and PD-L2. Tumor or infiltrating immune cells were considered positive if ≥5% of cells had membranous (cell surface) expression. Results Twenty-eight patients were included. PD-L1 and PD-1 were not significantly expressed on tumor cells; however, PD-L1 and PD-1 were expressed on infiltrating immune cells in 46% and 25% of patients, respectively. In contrast, PD-L2 demonstrated positive expression on tumor cells in 46% of the cases along with positive expression on infiltrating immune cells in 38% of the cases. Conclusions Sebaceous carcinoma currently has few effective adjuvant treatment options. The expression of PD-1, PD-L1, and PD-L2 on infiltrating immune cells and PD-L2 on tumor cells restrains T-cells from full activation and proliferation, therefore limiting the antitumor effect of T-cells, tipping the balance toward unopposed tumor progression. Consequently, PD-1 or PD-L1 inhibitors may have a role in sebaceous carcinoma treatment. Given the prevalence of PD-L2 expression in sebaceous carcinoma and the lack of PD-L2 blockade therapy available, PD-1 blockade may provide benefit over PD-L1 inhibitors. PD-1 blockade in combination with current methods may be a viable therapeutic option for patients with sebaceous carcinoma and deserves further study.Sebaceous carcinoma of the ocular and periocular regions showed expression of immune checkpoint ligands PD-1, PD-L1, and PD-L2, which suggests PD blockade may provide benefit as an adjuvant therapy for patients with sebaceous carcinoma.

Background: Muir-Torre syndrome (MTS) is defined clinically as the association of cutaneous sebaceous neoplasm and visceral malignancy. Ancillary tests are considered crucial for diagnosis. Although screening guidelines for MTS, including the Mayo MTS scoring system, have been proposed, there are no ophthalmic site-specific guidelines. Summary: A literature review conducted by PubMed search for articles describing patients with periocular sebaceous neoplasm and MTS disclosed 31 publications describing 60 patients, 36 (60%) of whom fulfilled clinical criteria for MTS, 6 (10%) whose diagnosis was based on screening ancillary studies, 14 (23%) who fulfilled clinical criteria and had supporting screening ancillary studies, and 4 (7%) who fulfilled clinical criteria and had supporting diagnostic genetic testing. Most patients were male (34 vs. 15 females), with a median age of 59 years (range 37–79 years). The most common diagnosis was sebaceous carcinoma (40/60, 67%), followed by sebaceous adenoma (16/60, 27%), followed by other tumors with sebaceous differentiation (4/60, 6%). The periocular lesions were identified prior to visceral malignancy in 10 out of 45 (22%) cases, after visceral malignancy in 34 out of 45 (76%) cases, and concurrently with visceral malignancy in 1 out of 45 (2%) cases. Immunohistochemistry for mismatch repair proteins was performed in 41 out of 60 (68%) and 14 out of 38 (37%) of the tumors had lost MSH2. Based on Mayo-MTS scores of 2 or greater, and after removing visceral malignancies not included in their scoring algorithm, 26 out of 30 of patients (87%) with complete data were considered to be appropriate candidates for further work-up. A survey of current practice was conducted by questionnaires, distributed to ophthalmic pathologists, ocular oncologists, and oculoplastic surgeons from national and international professional societies. Of the 103 physicians who participated in the survey, 91 (88%) felt that MTS evaluation guidelines were not sufficiently clear. Key Messages: Our findings suggest that Mayo MTS screening guidelines may be applicable to periocular sebaceous neoplasms. The uncertainty of ophthalmic specialists about optimal screening guidelines for MTS reflects the heterogeneity of defining criteria for MTS and limited molecular genetic data. Larger studies with detailed clinical, histopathologic, and molecular genetic data are required to formally assess screening guidelines for MTS in patients with periocular sebaceous neoplasms.

Highlights • Sebaceous carcinoma is a rare adnexal tumour. • It occurs sporadically or in association with Muir-Torre Syndrome (MTS). • MTS is a rare genetic disorder that predisposes patients to visceral and skin malignancies. • Chest wall is rare site of sebaceous carcinoma. • Surgical excision with wide margins is the standard treatment., Background Sebaceous carcinoma (SC) is a rare aggressive cutaneous malignant tumour. It accounts for less than 1 % of all cutaneous malignant tumours. Sebaceous carcinomas are divided into ocular and extraocular constituting 75 % and 25 % respectively. The most common extraocular site is parotid gland. Chest wall is a rare site of this tumour. Case presentation In line with SCARE criteria (Agha et al., 2018 [1]), this report is a case of a 45-year-old African male patient who presented with a two-year history of a large right anterior chest wall tumour. He reported that the tumour started as a small lump, which grew gradually over a two-year period. The patient reported no history of visceral malignancy and radiation exposure. However, his risk factors were age and immunosuppression in the form of Human Immunodeficiency Virus (HIV). The tumour was initially diagnosed as sebaceous adenocarcinoma by incisional biopsy before the patient had been referred to the treating team a year before. Excision of the tumour with wide margins was undertaken, and histology results confirmed sebaceous carcinoma. Discussion Sebaceous carcinoma is a rare and aggressive adnexal tumour. It is categorized as ocular and extraocular. Of these two, the more common is extraocular. The chest wall is a rare site for this tumour. The definitive diagnosis of this kind of tumour is done by tissue biopsy. In dealing with this tumour, surgical excision with wide margins is the standard treatment. Conclusion Sebaceous carcinoma is a rare aggressive malignant tumour originating from sebaceous gland. Early diagnosis and wide excision with negative margins improves survival.

Muir-Torre syndrome (MTS) a genetic disorder characterized by predisposition to cutaneous neoplasms. Lipedematous scalp is characterized by the presentation of a thick, sponge-like scalp due to the altered and increased deposition of adipose tissue. We present a case of Muir-Torre-associated sebaceous carcinoma of the scalp consistent with a lipedematous scalp.

Introductions: Preoperative diagnosis of orbital and ocular lesions is necessary for optimum treatment. The study aims to analyze the histomorphological spectrum of orbito-ocular lesions and to evaluate the need of ancillary techniques for confirmation of diagnosis. Methods: A cross sectional hospital based study of orbito-ocular surgical biopsy samples obtained in the Department of Pathology, at Birat Medical College Teaching Hospital, Nepal during one-year period was analysed for clinical and histopathological findings. Demographic data, site and tissue type, benign or malignant, recommendations for special stains and immunohistochemistry panel study were analysed. Results: Out of 185 total samples, male to female ratio of 1.1:1, age ranged from ten month to 82 years, 11-20 year age group had 39 (21.1%) orbito-ocular lesions and cornea-conjunctiva was involved in 104 (56.2%). Clinical diagnosis correlated well with histopathological diagnosis, p

Introduction Sebaceous carcinoma is a rare malignant skin tumour of the sebaceous glands which is most frequently located in the ocular region (75% of cases). Prompt recognition and treatment is vital, as it bears a high mortality rate ranging from 9 – 50%. We present a rare case of a giant sebaceous carcinoma of the upper back, managed as a day-surgery case because of resource and safety restrictions due to the COVID-19 pandemic. Case report We present a case of a 58-year-old male patient with a giant lesion on his upper back measuring 13 x 13cm in size. Due to the COVID-19 pandemic, this was resected under local anaesthetic. The histology and immunohistochemistry confirmed the diagnosis of a giant sebaceous carcinoma. Resection margins were clear and subsequent investigations confirmed there were no metastases. The defect was reconstructed with Matriderm © and a split-thickness skin graft, achieving a satisfactory cosmetic outcome at 3 months follow-up. Conclusion This is the first report which demonstrates the wide local excision under local anaesthetic for a rare presentation of a giant sebaceous carcinoma. This approach may be more widely utilised in the extraocular manifestation of this tumour, reducing the morbidity and length of hospital stay for such patients.

Collision tumors are neoplasms composed of two or more distinct cellular lineages coexisting at the same anatomic site. Incomplete biopsy, partial pathological slide examination or failure to include this diagnosis into the clinical differential may complicate and delay appropriate therapy. Although collision tumors are well documented, basal cell carcinoma (BCC) occurring with sebaceous carcinoma (SC) has only been reported in a single case report. The aim of the authors is to present a case of collision BCC and SC to highlight a rare clinicopathological case. We also present this case to advise caution to detect mimickers of BCC that warrant greater clinical workup and use this case to emphasize the importance of Mohs micrographic surgery for the treatment of SC.

Background Sebaceous carcinoma (SC) is a very rare, aggressive, malignant tumor arising in the adnexal epithelium of the sebaceous gland. SC in the oral cavity is extremely rare, in literature there are only 14 cases. We reported the 4th case of sebaceous carcinoma involving the lip Case presentation A 71-year-old male smoker presented an ulcerated lesion in the lateral region of the lower lip. The patient stated that the lesion had been present for 1 year. The past medical history was unremarkable. Extra-oral examination revealed a markedly ulcerated, exophytic, irregularly shaped, indurated mass of the lower right labial region, measuring 1.8 cm in size. The nodular lesion, located at the point of transition between mucosa and skin, showed a central ulceration. No other intraoral lesions were identified. The clinical differential diagnosis included squamous cell carcinoma, basal cell carcinoma with sebaceous differentiation and salivary gland neoplasms. The operation was performed under local anaesthesia. On histopathological examination, the tumor was composed by nodules or sheet of cells separated by a fibrovascolar stroma. The neoplastic tissue was deeply infiltrating, involving the submucosa and even the underlying muscle. Neoplastic cells showed a range of sebaceous differentiation with finely vacuolated rather than clear cytoplasm. Neoplastic cells were positive for S-100 protein, EMA, but negative for CEA. Therefore, based on these findings, a diagnosis of sebaceous carcinoma of the lower lip was rendered. Conclusion The histogenesis, differential diagnosis, and clinicopathologic conditions of this disease in the literature were reviewed. SC should be distinguished from other tumors full of vacuolated clear cells. Some useful biomarkers can be Ki-67, P53, CK, PAS, S-100, EMA, and AR.

PURPOSE To investigate the risk of second primary neoplasms (SPNs) after primary sebaceous carcinoma of the eyelid (SCE). METHODS Data on patients diagnosed with primary SCE as their first malignancy were extracted from the Surveillance, Epidemiology, and End Results database from 2000 to 2016. Observed-to-expected ratios of SPNs were calculated to estimate standardized incidence ratios (SIRs). Patients were compared with a reference population (RP) matched for age, gender, and race. RESULTS Five hundred fifty-nine patients with primary SCE were identified, 16% of whom developed SPNs. SCE patients displayed a 61% increased risk of developing SPNs compared with the RP (p < 0.001). Overall, the risk of SPNs of the lungs (SIR = 1.82; p < 0.05), pancreas (SIR = 2.94; p < 0.05), salivary glands (SIR = 41.65; p < 0.001), and skin (SIR = 8.33; p < 0.05) was elevated. Only non-Hispanic Whites were at an increased risk (SIR = 1.51; p < 0.05). Patients 40-54 years old at the time of diagnosis were at the highest risk of developing SPNs compared with the RP (SIR = 3.15; p < 0.05). Women with SCE experienced an increased risk of breast cancer (SIR = 3.6; p < 0.05) and chronic lymphocytic leukemia (SIR = 8.8; p < 0.01). CONCLUSION SCE patients are more likely to develop SPNs of the lungs, pancreas, salivary gland, and skin than the RP. Forty to fifty-four years old Caucasian patients are at the highest risk. Women are at an increased risk of developing breast malignancies and chronic lymphocytic leukemia. Clinicians should be cognizant of these risks when managing SCE patients.

Sebaceous carcinoma is characterized by its aggressive local tumor behavior and ability to metastasize. Small periocular sebaceous carcinoma are typically treated by excision with cryotherapy. Larger tumors often require adjuvant external beam radiotherapy (EBRT) and/or exenteration surgery. When used alone, EBRT techniques typically exceed the tolerance of critical normal ocular structures. The interstitial orbital brachytherapy-boost technique permits dose escalation to the tumor bed, while minimizing radiation dose to critical normal ocular structures. Here, we present a case of orbital sebaceous carcinoma treated with excision, cryotherapy, and super-thick amniotic membrane fornix reconstruction. Then, after 3 weeks of healing, adjuvant-combined electron interstitial high-dose rate brachytherapy-boost was added to electron-beam radiotherapy to optimize the orbital radiation dose distribution, increase dose to inferonasal orbit, and allow relative sparing of orbital tissues. At 1-year follow-up, there was no evidence of orbital tumor, no significant eye lash loss, normal ocular motility, no radiation retinopathy, optic neuropathy and a visual acuity of 20/20., Here, the authors describe a technique using adjuvant interstitial high-dose rate brachytherapy followed by electron-based external beam radiation therapy to increase the dose to a sebaceous carcinoma tumor bed while reducing dose to normal orbital structures.

Muir–Torre syndrome (MTS), a rare subtype of Lynch syndrome, is mostly autosomal dominant, which is caused by germline mutations in DNA mismatch repair (MMR) genes, the resulting microsatellite instability (MSI) of which increases the risk of developing sebaceous and other visceral tumors. Several reports have showed an association between immunosuppressive agents and the progression of latent MTS. In this report, we described a 41-year-old man with a history of kidney transplantation, having a rapid growth of the nodule on the anterior chest under immunosuppressive therapy, which was histologically proved to be sebaceous carcinoma. Systemic evaluation for visceral malignancies revealed sigmoid adenocarcinoma. These findings were consistent with the clinical diagnosis of MTS. Histological findings showed an absence of MMR proteins, including MSH2 and MSH6 both in the sebaceous carcinoma and sigmoid adenocarcinoma on immunohistochemical (IHC) analysis. A frame-shift mutation of c.229_230delAG (p. Ser77fs) in the MSH2 exon 2 in the lesion was detected by next-generation sequencing (NGS) analysis. This case report not only reveals a new site of MSH2 mutation in this family of East Asian descent but also highlights the importance of adequate diagnosis for Muir–Torre syndrome, as well as further prevention of the development of latent visceral tumors in kidney transplant recipients.