Linhagem
- Contraparte maligna do adenoma sebáceo
- Nas pálpebras → glândulas de Meibomian (sebáceas)
Clinical presentation
- Ocular form
- Ulcerated tumor unde the lower eyelid
- Eyelid or caruncular tumor
- Recurrent chalazion
- Chronic unilateral blepharoconjunctivitis (if the tumor spreads pagetoid along the ciliary margin into the conjunctival epithelium)
- Non-ocular form
- Yellowish tumor nodule
- Nipple-like plaque
- Face, scalp, neck and more rarely the trunk or genitals
- Treatment: wide excision
Associations
Muir-Torre SyndromeNevo/Hamartoma Sebáceo de JadassohnHistologia
- large multilobulated tumors comprising and unorganized mixture of immature basal cells and sebaceous cells at various stages of maturation
- Cells with low sebum content have microvacuolar cytoplasm with a central stellate nucleus
- Mature cells contain only one or more large vacuoles with a displaced or pycnotic nucleus.
- Malignancy is confirmed by foci of necrosis, nucleocytoplasmic atypia, images of pagetoid spread in the epidermis (particularly noticeable in eyelid locations) or vascular invasion
- Immunohistochemisty
- CK17
- Androgen receptors
Histopathology diagnostic pearl
- Immunohistochemistry should be performed on all sebaceous adenomas and carcinomas
- 100% PPV: loss of MLH1 and MSH6; MSH2, MLH1, MSH6