Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review

Sebaceous carcinoma of the vulva is a rare malignancy of the sebaceous glands, with potentially aggressive behaviour, that is usually found in the peri-ocular area. Nonetheless, there are sebaceous glands in the vulva and this diagnosis is especially rare, with only ten cases described in the literature. We report a case of 78-year-old female patient who presented with vulvar pruritus, previously treated with topical steroid and antifungal treatments, without improvement. The vulvar examination showed a visible yellow papule, 12 x 10 mm on the right major labia, which was biopsied and the microscopic examination revealed an invasive sebaceous carcinoma of the vulva, with an in situ component. We performed an uneventful excisional biopsy, followed by a subsequent margin widening. Three months after the diagnosis, she presented with the first recurrence. Two and half years after the diagnosis, she recurred with a larger lesion (13 mm) in the upper half of small right lip, more than 10 mm away from the midline. In a multidisciplinary meeting it was decided that the patient should undergo partial right vulvectomy with homolateral inguino-femoral sentinel node biopsy (one negative node). There was no evidence of recurrence one-year post-surgery.

Clinicopathological Features of 19 Eyelid Pilomatrixomas

Introduction: Pilomatrixoma is a relatively rare, benign tumor arising from the hair root matrix. It is found frequently on the head and neck, with most involving the eyebrow in the periocular region. In contrast, eyelid pilomatrixoma is less common, and often clinically misdiagnosed. Here, we present clinical and histological data from 19 pilomatrixomas arising in the eyelid. Methods: The study represents a retrospective study of eyelid pilomatrixoma diagnosed at our institution since 1981. All slides were reviewed, and demographic as well as clinical data obtained. Results: Patient ages ranged from 2 to 63 years (mean 24 years), including 12 (63%) females and 7(37%) males. Eight (42%) and 4 (21%) cases arose in the first and second decades of life, respectively. Upper eyelid involvement was found in 14 (74 %) of cases. Microscopically, the tumors were characterize by basaloid and shadow cells accompanied by calcification and foreign body giant cells. Conclusions: Eyelid pilomatrixoma is rarely suspected clinically, and can be mistaken for cyst, chalazion, sebaceous carcinoma and other tumors. Physicians should consider the possibility of pilomatrixoma in the eyelid area, especially in children or young female patients. Complete excision is curative, and diagnosis can generally be established by histopathological examination.

Intraepithelial growth pattern for eyelid sebaceous carcinoma: a cohort of 214 patients from a single institution

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.

EP.WE.741Management of a giant extraocular sebaceous carcinoma during the COVID-19 Pandemic: a case report

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.