Skin adnexal carcinomas are rare skin cancer, developing from pilosebaceous, eccrine and apocrine unit. Treatment of localised tumours usually includes surgery and radiotherapy. Indications and modalities of radiotherapy depend on the pathological subtype with a lack of consensus for some histologies. This review summarises the place of radiotherapy in terms of indication, dose and fractionation, volumes to irradiate and discuss ongoing studies.

Sebaceous carcinoma (SC) of the submandibular gland is extremely rare. Owing to the low morbidity and nonspecific clinical manifestations, diagnosis is commonly delayed, which increases metastasis and mortality. To date, there have been five reported cases of SC of the submandibular gland. Here, we present a new case and review the relevant literature.

A 36-year-old woman presented with an enlarged left submandibular gland. Clinical features included a non-tender solitary nodular mass with normal overlying skin. There were no special findings on computed tomography or ultrasound examination except for a swollen mass in the left submandibular gland. The patient underwent surgical resection. Pathological examination confirmed the diagnosis of SC with nerve infiltration. Immunohistochemical examination of this case showed positive staining for P63, P40, CK7, CK8/18, MLH1, MSH2, MSH6, and PMS2. The specimen was negative for androgen receptor, CEA, S-100, CK5/6, SOX-10, SOX-11, SMA, and GCDFP-15. The KI-67 labeling index was determined to be 15%. PAS and anti-epithelial membrane antigen were positive in partial area. The patient is still undergoing follow-up, and no metastasis or recurrence has been observed for 2 months.

This case highlighted the fact that despite its rarity, SC should be considered as a differential diagnosis for masses located in the head and face. Early and accurate diagnosis, followed by wide surgical excision, has a favorable prognosis. Therefore, clinicians should be familiar with the clinical and pathological features of this disease.

Sebaceous carcinoma (SC), a malignancy primarily characterized by aggressive growth, affects cutaneous tissues of the periocular region. Extraocular SC is extremely rare, especially in the extremities, as evidenced by only a handful of reported cases.

A 65-year-old man presented with a rapidly enlarging swelling on the left inner thigh, which was initially misdiagnosed as a subcutaneous abscess. The lesion had appeared two months prior to admission. Clinical examination revealed a cauliflower-like swollen content, with an ulcerated and infected mass located on his left thigh. At the same time, we observed solitary nodular lesions in his lungs and brain, with biopsy pathology of the lung lesions found to be consistent with the mass in the thigh. The patient received chemotherapy comprising cis-platinum with fluorouracil, followed by targeted therapy with anlotinib hydrochloride and chemotherapy with vinorelbine, implantation of iodine-125 seeds in the thigh and pulmonary tumor. The initial stage intervention achieved partial remission. The efficacy of maintenance treatment was evaluated as stable disease after the first 5 cycles; however, the patient developed a new brain lesion after the sixth cycle of treatment, which resulted in progressive disease and he received whole brain gamma knife radiotherapy.

We analyzed the clinical presentation, imaging features, pathology and treatment of a rare case of lung, brain and lymph node metastasis of SC located in the thigh. It is evident that cis-platinum combined with fluorouracil, vinorelbine combined with anlotinib hydrochloride may be an effective therapeutic regimen in advanced SC. However, brain metastatic lesions should receive early radiotherapy.

Extraocular sebaceous carcinoma (EOSC) is an aggressive malignancy of the sebaceous gland. Surgery is considered the cornerstone of treatment, but there is lack of clarity about extent and adjuvant treatment.

We conducted a systematic review and analysis of individual patient data of all published cases of EOSC to look into demography, pattern of care, importance of type of surgery, and other adjuvant treatment and survival outcome. A search of PubMed and Google Scholar was done with the key words sebaceous carcinoma, extraocular sebaceous carcinoma, and Muir-Torre syndrome till December 2017. The data were compiled in an Excel chart and analyzed using SPSS IBM software.

Data of 206 patients were retrieved. Median age at presentation was 65 years (range: 11-96 years). Surgery was performed in all except 13 patients. Of these 13, eight were deemed inoperable for extensive disease, and five had metastatic disease. Median PFS and OS for the entire cohort were 84 months (95% CI: 10-158 months) and 92 months (95% CI: 59-126 months). Univariate analysis revealed significantly poor survival for patients with a metastatic disease, regional nodal metastasis, and those with Mohs micrographic or incomplete surgery.

EOSC is a disease of elderly patients with good prognosis. Complete surgery with regional lymph node dissection is standard treatment. The role of adjuvant radiotherapy is debatable but can be considered in patients with incomplete surgery or high-risk factors.