Epidermoid Cancer of the Perianus and the Anal Canal JOHN L. SAWYERS, M.D., F.A.C.S.*
Epidermoid carcinoma of the perianus and anal canal is an infrequent disease for which no standardized treatment has been employed. Because of the relatively small experience at anyone institution, there is a scarcity of information regarding this lesion. Controversy still exists between surgeons and radiotherapists as to the best management of the problem, and surgeons differ regarding conservative local eXClSIOn or radical resection.
Perianal Epidermoid Carcinomas Small superficial epidermoid or squamous cell carcinomas of the perianal skin are usually similar to skin epitheliomas elsewhere in the body. These tumors are less likely to metastasize to the regional nodes than lesions of the anal canal. If metastases do occur, it is to the lymph nodes in the groin. Generally these perianal skin cancers may be treated by wide local excision if they are small (not over 2 cm. in diameter), and appear superficial. Irradiation and electrocoagulation of these lesions prevent adequate microscopic pathological examination and should not be used. If the lesions are not found to be histologically low grade and noninfiltrating after pathological examination, then abdominoperineal resection with wide excision of }he perianal skin should be done. Careful examination and follow-up for possible inguinal node metastasis should be performed. If metastases develop secondarily, then radical groin dissections should be done.
Epidermoid Carcinomas of the Anal Canal Unlike squamous cell carcinoma of the perianal skin, epidermoid carcinoma of the anal canal may metastasize to perirectal and mesenteric From Department of Surgery, Vanderbilt University School of Medicine and Surgical Service of Nashville Metropolitan General Hospital, Nashville, Tennessee * Associate Professor of Surgery, Vanderbilt University School of Medicine; Chief of Surgical Service, Nashville Metropolitan General Hospital, Nashville, Tennessee
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Table 1. Associated Conditions with Epidermoid Carcinoma oj the Perianus and Anal Canal Hemorrhoids ... . Fistula-in-ano ........... . Condylomata acuminata ..... . Lymphogranuloma inguinale ....................... .
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lymph nodes as well as the inguinal lymph nodes and also spread via blood vessels. Most surgeons now advocate abdominoperineal resection, with special attention to wide perineal dissection, for all these lesions. A difference of opinion exists regarding inguinal node dissection. Most recent writings on this aspect of the problem advise radical groin dissection when metastatic inguinal nodes become present, but avoid prophylactic groin dissection.
Clinical Material A recent study has been done at the Vanderbilt University Medical Center on all patients who had epidermoid carcinoma of the perianus and anal canal. These carcinomas comprised about 2.3 per cent of malignant cancers of the colon, rectum and anus seen in this medical center.8 This is similar to the incidence reported by others.4 Epidermoid carcinoma of the anus usually constitutes less than 5 per cent of all rectal and anal tumors. 9 In our series of 44 patients the ages ranged from 28 to 76 years with most of the patients being in their seventh decade. There were a few more female patients than males (23 to 21) in this group. In general, the occurrence of epidermoid carcinoma of the anus has been more frequent in women.
Predisposing Causes The etiology of epidermoid anal carcinomas is not known, but these tumors may be associated with benign anorectal lesions. Turell has reported that chronic fistulas and scars of fistulectomies, hidradenitis suppurativa, and certain venereal diseases as lymphogranuloma and donovanosis are favorable sites for occurrence of this cancer.ll Twelve of our patients had concomitant hemorrhoids, four had fistulain-ano, three had condylomata acurninata, and one had lymphogranuloma inguinale (Table 1). The three patients with condylomata had pathological evidence of the epidermoid carcinoma arising in the venereal wart. All three had abdominoperineal resections. Two are surviving without evidence of.,.tumor, and one patient died with perineal and inguinal node metastasis
Symptoms Rectal pain and bleeding are the most common and earliest symptoms of epidermoid anal carcinoma. Over 60 per cent of our patients complained of bleeding and almost one-half of the group had rectal pain. No patient was entirely asymptomatic and many had several complaints. Tenesmus,
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fullness in rectum, weight loss, diarrhea, and an inguinal mass were all presenting symptoms in this group of patients. The duration of symptoms ranged upward to more than four years with a majority of the patients having been symptomatic for more than six months. Ten patients had been treated by their physicians or treated themselves for hemorrhoids before the cancer was diagnosed. Adequate anorectal examination with biopsy of any suspicious lesion should lead to earlier diagnosis, but all too often the tumor is overlooked by patient and physician for a significant period of time.
Treatment Unlike the treatment of adenocarcinoma of the rectum, there has been no standardized treatment for the management of epidermoid carcinoma of the anus. In our group of patients irradiation, local excision and abdominoperineal resection have all been utilized (Table 2). Since all patients have been closely followed to the present time or to the date of their death, it is possible to draw some conclusions from these various methods of therapy. Five patients had advanced disease when first seen and received only palliation. This usually consisted of a colostomy. All these patients died of their cancer within one year. Irradiation therapy alone for epidermoid carcinoma of the anus has not been widely utilized in this country. Roux-Berger reported a 35 per cent five-year survival rate with radiation therapy.7 Seven of our patients had radiation alone and all patients died of their tumor within less than three years. Stearns reported a 15 per cent five-year survival among 13 patients seen at the Memorial Hospital in New York.lo There is insufficient data to evaluate the role of preoperative irradiation. Only one of our patients received radiation prior to resection. In view of the recent favorable reports for preoperative radiation in the treatment of lung, esophagus and large bowel cancers,3 it would seem reasonable to consider this in selected patients with very large lesions which are unlikely to be cured by resection. Irradiation alone is not advocated as a curative measure, but may be useful for palliation. Local excision of epidermoid carcinoma has been ineffective in treating
Table 2. Methods oj Therapy for Epidermoid Carcinoma oj the Anal Canal as Related to Survivorship TREATMENT
PATIENTS
Palliation only. . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 5 Radiation only ............................. , 7 Local excision. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 3 Abdominoperineal resection .................. , 29
SURVIVORS OVER 3 YEARS
0(0%) 0(0%)
2 (66%) 15 (52%)
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Abdominoperineal Resection
5-year survivors .............................. 11 (38%) 3- to 5-year survivors. . . . . . . . . . . . . . . . . . . . . . .. 4 (14%) Died of cancer. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 11 (38%) Hospital deaths...... . . . . . . . . . . . . . . . . . . . . . . .. 3 (10%)
those tumors involving the anal canal. Wide local excision may be used in small, low grade perianal carcinomas that are noninfiltrating. These lesions may metastasize to the inguinal nodes. If such metastases occur, radical groin dissection is then indicated. Holm and Jackman advocate local excision for those carcinomas distal to the pectinate line. 5 Kuehn and his associates reviewed the epidermoid carcinomas seen in the State of Connecticut and concluded that wide local excisions should be done on small perianal or low anal-canal lesions if the lesion was low grade and noninfiltrating.6 Dillard, Spratt, Ackerman and Butcher recently reviewed their cases and believe that their data support wide local excision for epidermoid cancers of the anal margin having a size of 2 X 2 cm. or less, a histological grade of 0 or 1, and circumscribed or pushing margins not fixed to deeper tissues. 2 Abdominoperineal resection is becoming the standard method of treatment for most of the epidermoid anal carcinomas except those small, superficial perianal tumors, as already described. In treating this tumor by abdominoperineal resection, the standard Miles resection should be enlarged by ligation of the inferior mesenteric artery at the aorta and wide removal of perineal skin including the ischiorectal fossa contents and division of the levator muscles close to their origin. In females the entire posterior wall of the vagina and posterior half of the labium are excised with the tumor. This extensive perineal excision is necessary since 50 per cent of our failures from abdominoperineal resection were the result of perineal recurrence. Twenty-nine patients in our series had abdominoperineal resection with a 38 per cent five-year survival rate. Four other patients are living and well from three to five years for an overall survival of 52 per cent. Three patients died in the postoperative period. Thirty-eight per cent of the patients have died as a result of their cancer (Table 3). Cancer chemotherapy drugs have been used infrequently in treating this tumor. Their use is limited to palliation. A few isolated cases have been treated with 5-FU or 5-FUDR but no conclusions can be drawn from this small number. Two of our patients had pelvic perfusion with nitrogen mustard for perineal recurrences. There was slight pain relief, but no objective response. Radiation therapy is better for palliation at the present time.
Metastatic Inguinal Lymph Nodes A review of our patients and reports from other institutions reveal the need for a logical planned attack regarding inguinal lymph nodes. The
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prognosis is very poor when the inguinal nodes are involved with metastatic tumor at the patient's initial examination. All of our patients with this finding have died of their disease. However, inguinal node dissection should be done in these cases as it provides good palliation and may prevent sloughing from tumor metastasis. Inguinal node dissection is preferable to irradiation as a palliative procedure in the good-risk patient. If metastatic inguinal lymph nodes develop after treatment of the primary tumor, then radical groin dissection is indicated and should include excision of the subcutaneous tissue of the lower abdomen and upper half of the anterior thigh, including the deep fascia over the muscles and femoral sheath along with the retroperitoneal tissue and nodes over the iliac vessels and obturator fossa. The sartorius muscle is moved over the femoral vessels to protect them. The technique has been well described by others.l Controversy still exists regarding bilateral prophylactic groin dissection. Lymphatic spread to the groin nodes may occur across the perineum and over the upper thigh to the superficial inguinal nodes or along the middle hemorrhoidal lymphatics to the hypogastric and obturator nodes and flOm there retrograde to the external iliac and inguinal nodes. Recent reports2 , 6 oppose bilateral prophylactic groin dissection as unnecessary and advocate a therapeutic groin dissection when the nodes become clinically detectable. Our study revealed that 36 per cent of the patients had inguinal node metastases. In most of these patients the tumors arose in the perineal aspect of the anus rather than the anal canal. These patients must be carefully observed for inguinal lymph node metastases. We are not now doing prophylactic groin dissection for anal carcinomas, but closely follow all patients and do unilateral radical inguinal node dissection if the nodes become involved.
SUMMARY
Epidermoid carcinoma of the perianus and anal canal arises in a location that permits early diagnosis by inspection and palpation. However, this tumor has frequently been overlooked by both patient and physician. It may occur in association with other anorectal conditions. Chronic rectal conditions such as anal fistulas, venereal lymphogranuloma and condylomata acuminata should have definitive early therapy as these conditions are thought to possess a tendency toward malignant degeneration. Thorough anorectal examination with biopsy of any suspicious lesion should lead to earlier diagnosis and improvement in surgical treatment of these tumors. Epidermoid anal carcinoma spreads by direct extension, via lymphatics to the perirectal, mesenteric and inguinal lymph nodes, and only infrequently by the blood stream. Even at autopsy, less than 10 per cent of our patients had liver or lung metastases.
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These tumors should be treated by surgical excision. Irradiation should not be used as a curative procedure but reserved for palliation. Preoperative irradiation may prove useful in selected patients with large localized tumors unlikely to be cured by surgical resection. Wide local excision may be used in patients with small, superficial, low grade perianal squamous cell carcinomas. These patients should be followed closely for metastases to the nodes of the groin. Abdominoperineal resection with very wide perineal excision is the treatment of choice for extensive or infiltrating perianal epidermoid carcinomas and for all cancers arising in the anal canal. Radical groin dissection is performed if the inguinal lymph nodes are or become involved. Prophylactic bilateral groin dissections are not advocated.
REFERENCES 1. Bryon, R. L., Jr., Lamb, E. J., Yonemoto, R. H. and Kase, S.: Radical inguinal node dissection in the treatment of cancer. Surg. Gynec. & Obst. 114: 401,1962. 2. Dillard, B. M., Spratt, J. S., Ackerman, L. V. and Butcher, H. R., Jr.: Epidermoid cancer of anal margin and canal. Arch. Surg. 86: 100, 1963. 3. Fletcher, W. S., Allen, C. V. and Dunphy, J. E.: Preoperative irradiation for carcinoma of the colon and rectum. Am. J. Surg. 109: 76, 1965. 4. Grinnell, R. S.: An analysis of forty-nine cases of squamous cell carcinoma of the anus. Surg. Gynec. & Obst. 98: 29, 1954. 5. Hohm, W. H. and Jackman, R. J.: Anorectal squamous-cell carcinoma. Conservative or radical treatment? J.A.M.A. 188; 241, 1964. 6. Kuehn, P. G., Beckett, R., Eisenberg, H. and Reed, J. F.: Epidermoid carcinoma of the perianal skin and anal canal. New England J. Med. 270: 614, 1964. 7. Roux-Berger, J. L. and Ennuyer, A.: Carcinoma of the anal canal. Am. J. Roentgenol. 60: 807, 1948. 8. Sawyers, J. L., Herrington, J. L., Jr. and Main, B. F.: Surgical considerations in the treatment of epidermoid carcinoma of the anus. Ann. Surg. 157: 817, 1963. 9. Sedgwick, C. E. and Wainstein, E.: Epidermoid carcinoma of the anus and rectum. S. CLIN. NORTH AMERICA 39: 759, 1959. 10. Stearns, M. W., Jr.: Epidermoid carcinoma of the anal region. Surg. Gynec. & Obst. 106: 92, 1958. 11. Turell, R.: Epidermoid squamous cell cancer of the perianus and anal canal. S. CLIN NORTH AMERICA 42: 1235, 1962. 72 Hermitage Avenue Nashville, Tennessee 37210