Case sharing: Master Han, 66 years old and in good spirits, suffered from a rare malignant tumor called perianal Paget’s disease three years ago and continued to have recurrent attacks after removal of the mass. This follow-up examination showed that the tumor had recurred again, and after surgical removal of the rectal lesion, Grandpa Han received further radiotherapy and chemotherapy treatment.
Perianal Paget’s disease is not uncommon in clinical practice and manifests itself as recurrent itching that does not heal, similar to perianal eczema, and can easily be misdiagnosed. This was the case with Grandpa Han. The company’s main business is to provide a wide range of products and services to its customers.
When he was first seen, our doctor suspected that this was not a simple and common perianal eczema. As a result, a biopsy of the perianal tissue showed that it was perianal Paijer’s disease, a malignant tumor. The initial examination showed that the tumor had fortunately not yet metastasized and spread, and Master Han was instructed to review it every six months.
What exactly is perianal Paget’s disease?
PerianalPaget’sdisease, also known as eczematoidcancer, is a rare intraepithelial adenocarcinoma that belongs to extramammary Paget’s disease. The damage is characterized by well-defined eczema-like spots with intractable pruritus; the histological features are scattered or clusters of Paget cells in the epidermis.
Etiology of perianal Paget’s disease
The histologic origin is controversial, but there are three hypotheses: (1) Paget cells in the perianal epidermis, metastasized from deep-seated carcinomas. (ii) Paget cells originate from the perianal epidermis. (iii) Paget cells may arise from an unknown oncogenic factor acting on epithelial sweat glands or rectal intestinal glands.
Pathology of perianal Paget’s disease
There are three types: one is adnexal carcinoma that occurs in the perianal region without deeper carcinoma. The second type is associated with adenocarcinoma of the greater or lesser sweat. The third type is perianal cancer that may be associated with deeper rectal, urethral, cervical or breast cancer.
Clinical manifestations and diagnosis of perianal Paget’s disease
The disease is most common in the elderly, with an average age of about 60 years, and there is little difference in gender. Clinical manifestations: ① Slow onset and long history, with an average of about 4 years from the appearance of symptoms to diagnosis. The first common symptom is persistent perianal itching, which cannot be relieved by local application of corticosteroids. ③The lesion is initially a perianal papule or scaly erythema, which gradually expands into an infiltrative plaque, with perianal flushing, similar to eczema, and later forms an ulcer with elevated edges and clear boundaries, with mucous membrane greasy yellow exudate on the surface, followed by yellow crust, and the ulcer does not heal for a long time, with burning pain. If the mucosa of the anal canal is involved, rectal cancer will occur, and if the urethra or cervix is involved, urethral cancer or cervical cancer will easily occur.
The disease should be highly suspected if perianal eczema with intractable itching is not relieved by local corticosteroid application. The following manifestations should draw attention. (1) perianal ulcers that do not heal for a long time, and exclude other diseases; (2) perianal damage with rectal, urethral or cervical cancer manifestations. Pathological examination is the only way to confirm the diagnosis.
Differential diagnosis of perianal Paget’s disease
Perianal eczema: the appearance is very similar to this disease, but local application of corticosteroids can relieve the pruritic symptoms, and biopsy can differentiate it.
Superficial fungal infection: tinea cruris spreads to the perianal area, and its lesions are similar to this disease; steroids cannot relieve the symptoms, but antifungal treatment is effective, and scraping microscopy reveals mycelium or spores.
Three perianal Bowen’s disease: it is a perianal epidermal human squamous cell carcinoma, which can be identified by biopsy.
Treatment of perianal Paget’s disease
Surgical excision is the main treatment method. Surgical methods and indications are: (1) the lesion simply involves the perianal epidermis, and only the local lesion and the surrounding normal skin larger than 1 cm are removed. If the lesion invades the deeper adnexa, the deep fascia at the base of the tumor and the normal tissues around the tumor larger than 1 cm should be included in the resection. ③The lesion involves deeper rectum, urethra or cervix, etc. The corresponding radical surgery for rectal cancer, urethra cancer or cervical cancer should be performed. Currently, it is believed that early lesions should also be extensively and deeply excised (with or without skin grafting) to reduce postoperative recurrence, because Paget cells often enter the subcutaneous tissue along the hair follicles, and simple excision of the skin is often ineffective.
Chemotherapy cannot eliminate lesions, but topical application of 1% 5-FU can improve pruritus. Radiation therapy can make the lesion development temporary.
In this special reminder to the general public friends, if the recurrence of perianal itching does not heal, must be timely to the regular hospital examination and treatment, so as not to delay the disease.