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Extra-mammary Paget’s disease is an adenocarcinoma of the skin that is clinically and histologically identical to the lesions of Paget’s disease, originating from the apocrine glands, but patients do not have concomitant breast cancer. This ailment is an extremely rare disease.
What is extra-mammary Paget’s disease?
Paget’s extramedullary disease is an adenoma of the skin, a rare type of intra-epidermal cancer. This adenoma is clinically and histologically identical to the lesions in Paget’s disease and originates from the apocrine glands. This ailment is most common in women between the ages of 50 and 80. It was first described in 2000 when it referred to the cases of a man diagnosed with Paget’s cancer who had attacked his right cheek. The lesions in the form of slowly enlarging erythema covered with scabs are usually located in the anal genital area, i.e. where there are a large number of apocrine and eccrine glands. Much less often, Paget’s extramammary disease affects the buttocks or armpits.
Extra-mammary Paget’s disease – causes
The causes of extrasculptal Paget’s disease have to do with its forms.
1. In the first form, the neoplasm begins to develop in the epidermis to spread successively into the ducts of the sweat glands and the epithelium of the hair follicle. On the other hand, another hypothesis is that the disease develops in the epidermis and spreads to the dermis (metastasizing from there).
2. In the second type of disease, the neoplasm originates in the digestive or genitourinary system, from where it spreads into the adjacent epidermis.
Symptoms of extrasuminal Paget’s disease
The microscopic picture of post-tracheal Paget’s disease is identical to that of classical Paget’s disease. The lesions appearing in the genital area are clearly defined eruptions that may peel off (erythema is often covered with crusts). Accompanying symptoms are burning and itching, sometimes there is also oozing. Some patients may develop bleeding erosions.
Diagnosis of extra-mammary Paget’s disease
Diagnostics is mainly based on biopsy and taking material for histopathological examination. It is supposed to reveal the presence of specific Paget cells and assess the degree of invasiveness of the whole process. In the case of small lesions within the vulva and suspicion of their invasiveness, a biopsy taken from the middle of the eruption may confirm the assumptions. In turn, in the course of extensive changes, but apparently superficial – histopathological examination will confirm intra-epidermal cancer growth. Very often, an unspecified clinical picture causes a specialist to misdiagnose, which automatically extends the time of diagnosis and treatment – local and general. Therefore, it is important to exclude other ailments:
- psoriasis,
- atopic dermatitis,
- Bowen’s disease,
- contact dermatitis,
- seborrheic dermatitis,
- mycosis fungoides,
- melanoma,
- lichen,
- tinea,
- histiocytoza,
- candidiasis.
In most patients, the occurrence of Paget’s cells is confined to the epidermis, and then the prognosis is good. The situation is different when the tumor metastasizes through the lymphatic vessels or infiltrates the skin – then the prognosis is much worse.
How to treat extra-mammary Paget’s disease?
The type of treatment to be implemented depends on the presence or absence of coexisting neoplastic tumors. If they are present, the best solution is procedure carried out by an experienced oncologist surgeon. If no neoplastic tumors are found, the lesions should be surgically removed with an appropriate margin. It is also necessary to assess the lymph nodes. It is used in patients whose tumor infiltrates locally in the perianal area abdomino-perineal resection or surgery in combination with chemoradiotherapy. When the tumor infiltrates the vulva area, it is performed vulectomythat is, the removal of part or all of the external genitalia of a woman. With metastasis, treatment is based solely on chemotherapysometimes additional radiotherapy and local palliative treatment are implemented.
Non-surgical treatment in patients who do not qualify for surgery or refuse to consent to it, local or systemic chemotherapy with 5-fluorouracil and bleomecin, as well as radiotherapy and laser therapy and PTD (photodynamic therapy) are implemented. For the treatment to be beneficial, it is necessary to take into account the multifocal nature of skin lesions and the irregular margin of the affected tissues, which very often goes beyond the area of clinical diagnosis. Extra-mammary Paget’s disease is a rare condition that is always progressive, never with spontaneous recovery. The prognosis of the disease is very poor if proper treatment is not implemented early.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House