Anal and Perianal Neoplasms


Anal Canal Cancers

  1. Anatomic Considerations
    • AJCC has defined 3 anatomic regions in which anal or perianal squamous cell cancer may be manifested
    • anal canal lesions: cannot be visualized or are incompletely visualized by gentle traction of the buttocks
    • perianal lesions: completely visible and arise within 5 cm of the anal opening when the buttocks are gently spread
    • skin lesions: arise outside of the 5-cm radius and are treated like skin lesions in any other site of the body
    • there can be overlap between anal canal lesions and perianal lesions

  2. Lymphatic Drainage
    • spreads through 3 different sets of regional nodes
    • superiorly, above the dentate line, lymph vessels follow the superior hemorrhoidal artery and terminate in the preaortic and paraaortic nodes
    • laterally, the drainage follows the middle hemorrhoidal vessels to the internal iliac nodes
    • inferiorly, below the dentate line, the drainage follows the inferior hemorrhoidal vessels and terminates in the inguinal nodes

  3. Anal Canal Neoplasms
    1. Squamous Cell Cancer
      • arises from the anal transition zone
      • strongly associated with HPV infection
      • occurs more commonly in women, and women should be screened for cervical cancer
      • other risk factors include receptive anal intercourse, HIV, immunosuppression, smoking

      1. Diagnosis
        • patients may have only minor symptoms and are often misdiagnosed as having benign anorectal disorders (hemorrhoids, anal fissure)
        • perianal pain, bleeding, and an indurated mass on exam should suggest the diagnosis of anal cancer
        • rectal examination reveals the size, location, and degree of fixation of the tumor
        • anoscopy verifies the exact location of the tumor relative to the dentate line
        • the mass should be biopsied for histologic diagnosis
        • examination of both inguinal regions determines if there is any suspicious nodal enlargement
        • staging includes CT of the chest, abdomen, and pelvis, and pelvic MRI

        Anal Squamous Cell Cancer
      2. Therapy
        1. Chemoradiation
          • now the standard treatment
          • 5-year survival rates are as high as 92% in node-negative patients
          • combines external beam radiation (at least 45Gy) with a continuous infusion of 5-FU and a bolus injection of Mitomycin-C

        2. Abdominoperineal Resection
          • standard treatment prior to 1974
          • 5-year survival ranged from 50% to 70%
          • local recurrence rates in the pelvis ranged from 25% to 50%
          • additional morbidities include nonhealing of the perineal wound, sexual dysfunction, and a permanent colostomy
          • now reserved as salvage therapy after failed chemoradiation or recurrent disease

        3. Management of the Inguinal nodes
          • sentinel node biopsy can improve lymph node staging, but its value is unclear since the inguinal lymph nodes are routinely included in the radiation field
          • superficial and deep inguinal node dissection is reserved for patients with bulky nodal disease or recurrent nodal disease after irradiation

    2. Melanoma
      • 3rd most common site for melanoma (skin, eye)
      • frequently amelanotic
      • often mistaken for a thrombosed hemorrhoid
      • has a poor prognosis, with 5-year survival rates < 10%
      • does not respond to chemoradiation
      • if an R0 resection can be performed with a wide local excision, this is preferred over an APR, since extent of resection does not improve survival

      Multiple Anal Melanomas
    3. Adenocarcinoma
      • usually is a distal extension of a low rectal cancer
      • rarely may arise from the columnar epithelium of the anal glands or from a chronic anal fistula
      • most patients are treated with multimodality therapy – chemoradiation and APR

Anal Margin Tumors

  1. Condyloma Acuminatum
    • caused by sexually transmitted HPV virus
    • considered a premalignant lesion
    • more common in immunocompromised patients

    1. Clinical Presentation
      • pruritus, bleeding, pain, discharge, palpable mass
      • exam reveals pinkish warts that may coalesce to form a mass
      • anoscopy may reveal disease within the anal canal
      • high-resolution anoscopy with or without 5% acetic acid may improve detection of disease
      • perineum and genitals must also be carefully inspected for disease
      • HIV testing should be done
      • sexual partners should be contacted and evaluated

      Anal Condyloma
    2. Treatment
      • no consensus on a gold-standard treatment
      • recurrence rates are between 20% - 50% irrespective of treatment approach

      1. Topical Agents
        • podophyllotoxin, imiquimod cream, trichloroacetic acid, 5-FU
        • recurrence rate is much higher than with surgical treatment
        • may be used to prevent recurrence after surgical treatment

      2. Invasive Treatment
        • cryotherapy, CO2 laser, electrocautery fulguration, surgical excision
        • a few samples should be sent for pathology to rule out malignancy
        • must leave intact skin bridges to prevent anal stenosis
        • adjuvant interferon can reduce recurrence after surgical treatment

  2. Verrucous Carcinoma
    • giant condyloma acuminatum or Buschke-Lowenstein tumor
    • slow-growing neoplasm that tends to recur and to form abscesses and fistulas
    • may progress to invasive squamous cell carcinoma
    • treatment is wide local excision, often requiring flap closure
    • invasive disease will require an APR
    • role of chemoradiation is unclear

    Verrucous Carcinoma
  3. Anal Intraepithelial Neoplasia (AIN)
    • often a precursor to invasive squamous cell cancer
    • may involve the anal canal as well as the perianal skin
    • strongly associated with HPV
    • graded according to the degree of dysplasia present
    • LSIL is a low-grade dysplastic lesion
    • HSIL is a high-grade dysplastic lesion
    • Bowen’s disease (carcinoma in situ) is now classified as an HSIL

    Anal Intraepithelial Lesions
    1. Diagnosis
      • often presents with minor symptoms such as burning or pruritis
      • one-third of patients present with a mass or bleeding
      • exam may reveal brown-red pigmented, noninfiltrating, scaly plaques, with a moist surface and nodules
      • differential diagnosis includes eczema, psoriasis, and leukoplakia

      Anal Bowen's Disease (Carcinoma in Situ)
      Carcinoma in Situ (Bowen's Disease)

    2. Treatment
      • for unifocal lesions, the standard treatment is wide surgical excision, but recurrence rates are 30% and flap closure may be necessary
      • perianal disease may also be treated with the topical application of imiquimod

      1. High-Resolution Anoscopy (HRA)
        • for anal canal lesions, anal mapping with HRA with acetic acid aids in identifying dysplastic lesions
        • suspicious areas should be biopsied
        • definitive treatment may include excision or ablation

        High Resolution Anoscopy
  4. Squamous Cell Cancer
    1. Diagnosis
      • may present with a palpable mass, bleeding, itching, pain, or tenesmus
      • associated anal condylomata or chronic anal fistula may be present

      Anal Margin SCCA
    2. Treatment
      • small lesions (T1) within 5 cm of the anal verge are treated with a wide local excision with a 2-cm margin
      • large lesions that impinge on the anal margin are treated with chemoradiation, just like squamous cell cancers of the anal canal

  5. Basal Cell Carcinoma
    • behaves like basal cell carcinoma elsewhere on the skin
    • has raised edges and central ulceration
    • treated by wide local excision with primary closure or skin grafting
    • 30% local recurrence rate, which is treated by reexcision
    • rarely metastasizes

    Anal Margin BCCA
  6. Paget’s Disease
    • arises from the intraepidermal portion of the apocrine glands
    • frequently associated with an underlying noncontiguous carcinoma (colorectal, bladder, vagina, cervix, endometrium)
    • if left untreated, adenocarcinoma of the apocrine glands may develop
    • severe, intractable pruritus is common
    • examination reveals an erythematous, eczematous rash
    • treatment is wide local excision with negative margins
    • if there is an invasive component, an APR should be considered
    • XRT can be used as adjuvant treatment or primary therapy
    • Moh’s micrographic surgery is associated with lower recurrence rates

    Anal Margin Pagets






References

  1. Sabiston, 20th ed., pgs 1412 - 1415
  2. Schwartz, 10th ed., pgs 1217 - 1218
  3. Cameron, 11th ed., pgs 224 – 231