Anal rim cancer

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Classification according to ICD-10
C44.5 Other malignant neoplasms of the skin
skin of the trunk; Anus: skin, edge (area); Skin of the mammary gland; Perianal skin
ICD-10 online (WHO version 2019)
The anus of a 66-year-old female patient with condyloma acuminata and a carcinoma in situ above it.
Histological preparation of the carcinoma in situ of the 66-year-old patient in HE staining (4x magnification)
The same histological specimen magnified 40 times

The Analrandkarzinom ( Engl. Carcinoma of the anal margin or carcinoma of the perianal area ) is an epidermoid carcinoma with predominantly flat and ulcerative spread. Together with the anal canal carcinoma, it belongs to the group of anal carcinomas . The anal margin carcinoma usually arises from a perianal intraepithelial neoplasia (PAIN, a new tissue formation within the epithelial cells surrounding the anus (a precancerous condition )). The disease is relatively rare.

Definition and description

Carcinomas of the anal margin are defined as malignant tumors that arise below (distal) the anocutaneous line and 5 cm around it. Malignant tumors that are proximal (above, in the oral direction) the anocutaneous line and below the dentate line are, by definition, anal canal carcinomas.

In most cases it is squamous cell carcinoma , which is also known as spinalioma or squamous cell carcinoma . Adenocarcinomas are much less common . Carcinomas of the anal margin grow relatively slowly and metastasize preferentially in the inguinal lymph nodes . Distant metastases are rare and mainly affect the liver and lungs .

frequency

Approximately every fifth anal carcinoma is an anal margin carcinoma. Men and women are equally affected. The maximum age is between 60 and 70 years of age. Approximately 0.2 to 0.4% of all gastrointestinal carcinomas are carcinomas of the anal margin. The incidence is around 0.2 per 100,000 inhabitants per year.

causes

The exact etiology of the anal margin cancer is unclear. Significant risk factors are previously damaged areas of the perianal skin. These include chronic inflammations , such as Crohn's disease and infections with human papillomavirus (HPV), especially with the carcinogenic high-risk types 16, 18 and 58. Accordingly, genital warts ( Condylomata acuminata ), which are caused by the high-risk HPV Types arise to be the starting point for the degeneration of the squamous epithelium. Lichen planus ( lichen planus ), acne inversa , and lichen sclerosus , as well as infections with the herpes simplex virus type 2 can form the basis for the development of anal margin carcinoma. Another important risk factor is immunosuppression , for example after an organ transplant or in immunocompetent HIV- positive patients

Symptoms and diagnosis

In the preliminary stage of perianal intraepithelial neoplasia (PAIN, the term precancerous condition is out of date) there are eczema-like skin changes. The resulting anal margin carcinoma presents itself as a wart-like (verrucous) skin-colored to reddish nodule, which has a coarse, rarely smooth morphology. The carcinoma spreads peripherally and in depth over months and years. It can infiltrate deeper tissues and organs . With the onset of ulcer formation ( ulceration ), patients experience itching, oozing, bleeding and pain .

The attending physician, usually a dermatologist or proctologist, will usually perform a close inspection and palpation of the anus. With the help of the removal of a small amount of tissue, the suspected diagnosis can be histologically confirmed. To treat the tumor, it is necessary to know its degree of spread (classification) and its thickness. With the help of the tissue sample, the degree of differentiation ( grading ) of the tumor can be determined. If the tumor is more than 2 cm thick, an ultrasound examination of the lymph nodes in the groin area (inguinal lymph node ultrasound ) is recommended. This can be used to determine whether the tumor has metastasized in the lymph nodes. In class 4 tumors, imaging techniques such as magnetic resonance tomography or computed tomography are usually used to detect possible distant metastases.

In the differential diagnosis, a distinction must be made between basaliomas , anorectal melanomas , keratoacanthomas , warts and outward-growing anal canal carcinomas.

classification

The TNM classification of anal margin carcinoma:

classification description
Tis Carcinoma in situ
T1 Tumor 2 cm or less in the largest dimension
T2 Tumor more than 2 cm but not more than 5 cm in greatest extent
T3 Tumor more than 5 cm in greatest extent
T4 Tumor of any size with infiltration of neighboring organs (involvement of the sphincter muscles alone is not classified as T4)
N0 no lymph node metastases
N1-N3 regional lymph node metastases
M0 no evidence of distant metastases
M1 Detection of distant metastases

therapy

When the results of the histological and, if necessary, imaging examinations are available, and the extent and differentiation of the anal margin carcinoma are known, treatment can begin, depending on the diagnosis result . The complete cutting out of the tumor ( excision ) is the method of choice and standard therapy in anal margin cancer. The excision margin must be tumor-free from a histological point of view. When removing the carcinoma, a safety distance of 1 cm is usually maintained. One goal of the surgery is to maintain the patient's stool continence . If this goal is endangered by surgical measures, radiation therapy can be used as an alternative. In many cases, the excision wound can be left open or directly sutured . A flap is therefore usually not necessary.

Photodynamic therapy (PDT) can be used in addition to surgical removal . PDT can also be used to treat perianal intraepithelial neoplasias (precancerous lesions).

If the regional lymph nodes are affected, a radical lymphadenectomy (surgical removal of the affected lymph nodes) is performed in most cases . Under certain circumstances, radiation therapy measures can be carried out.

Class T3 anal rim carcinomas that have infiltrated the sphincter or class T4 (involvement of other organs) are usually treated like anal canal carcinoma.

forecast

Due to the low frequency of the disease, there are no reliable data on lethality and survival in the literature . As with most cancers, the prognosis depends on the factors tumor size, primary or recurrent tumor, stage of spread, histological differentiation, and immune status.

further reading

Individual evidence

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  2. ^ A b c Peter Altmeyer: Entry on anal carcinoma. in: Encyclopedia of Dermatology, Venereology, Allergology, Environmental Medicine. Springer-online, accessed on November 15, 2018.
  3. W. Hartschuh, C. Breitkopf: Anal intraepithelial neoplasia (AIN) and perianal intraepithelial neoplasia (PAIN). (PDF) Guideline of the German Dermatological Society (DDG), German Society for Coloproctology (DGK), development stage 1, status July 2009.
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  6. J. Papillon, JL Chassard: Respective roles of radiotherapy and surgery in the management of epidermoid carcinoma of the anal margin. Series of 57 patients. In: Diseases of the Colon and Rectum . Volume 35, Number 5, May 1992, pp. 422-429, ISSN  0012-3706 . PMID 1568392 .
  7. A. Ky, N. Sohn et al.: Carcinoma arising in anorectal fistulas of Crohn's disease. In: Diseases of the Colon and Rectum . Volume 41, Number 8, August 1998, pp. 992-996, ISSN  0012-3706 . PMID 9715154 . (Review).
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  10. SH Lee, DH McGregor, MN Kuziez: Malignant transformation of perianal condyloma acuminatum: a case report with review of the literature. In: Diseases of the Colon and Rectum . Volume 24, Number 6, September 1981, pp. 462-467, ISSN  0012-3706 . PMID 7273984 .
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  17. ^ ST Williams, RC Busby et al.: Perineal hidradenitis suppurativa: presentation of two unusual complications and a review. In: Annals of plastic surgery. Volume 26, Number 5, May 1991, pp. 456-462, ISSN  0148-7043 . PMID 1952719 . (Review).
  18. EK Derrick, CM Ridley et al: A clinical study of 23 cases of female anogenital carcinoma. In: The British journal of dermatology. Volume 143, Number 6, December 2000, pp. 1217-1223, ISSN  0007-0963 . PMID 11122024 .
  19. ^ PJ Sloan, J. Goepel: Lichen sclerosus et atrophicus and perianal carcinoma: a case report. In: Clinical and experimental dermatology. Volume 6, Number 4, July 1981, pp. 399-402, ISSN  0307-6938 . PMID 7307331 .
  20. ^ RH Thomas, CM Ridley et al.: Anogenital lichen sclerosus in women. In: Journal of the Royal Society of Medicine. Volume 89, Number 12, December 1996, pp. 694-698, ISSN  0141-0768 . PMID 9014881 . PMC 1296033 (free full text).
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  22. JH Kim, B. Sarani et al .: HIV-positive patients with anal carcinoma have poorer treatment tolerance and outcome than HIV-negative patients. In: Diseases of the Colon and Rectum . Volume 44, Number 10, October 2001, pp. 1496-1502, ISSN  0012-3706 . PMID 11598480 .
  23. a b c d e f g h i V. Wienert: Anal rim carcinoma. ( Memento from February 19, 2014 in the Internet Archive ) (PDF; 160 kB) Guidelines of the German Society for Coloproctology, development stage S1, as of December 2002.
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