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Surgical treatment of anorectal melanomas
Surgical Treatment of Anorectal Melanomas
Bruno Siegal, MD, FACS, Tel-Hashomer, David Cohen, MD, Tel-Hashomer,
Israel
Israel
Erwin T. Jacob, MD, Tel-Hashomer,
Israel
Anorectal melanoma is a rare disease, comprising less than 1 percent of all melanomas and 1 percent of all malignant tumors of the anus and rectum [l--4]. Failure td recognize the specific features of this disease has led to its inclusion with the rest of malignant tumors of this anatomic area. This had led to inappropriate and sometimes unnecessary treatment in a number of patients. Difficulties in reaching an early diagnosis, few therapeutic options, and a very low 5 year survival rate (less then 10 percent) [5-71, characterize this particular melanoma. All anorectal melanomas that occurred in Israel from 1960 to 1981 (30 cases) form the basis of this series. Ten cases occurred in the first decade of life and 20 in the last decade. Material
and Methods
Seventeen women and 13 men formed the study population for this series. The youngest was 35 years old and the oldest 93 years (mean 64 years) (Figure 1). Eighteen patients were Jews of European descent, 9 were Sephardic Jews, and 3 were Arabs (Figure 2). The most frequently noted symptoms were: rectal bleeding (25 patients), anal pain (24 patients), changes in bowel habits (15 patients), tenesmus (13 patients), and anal mass (8 patients). Half of the patients exhibited signs of disseminated disease. Significant loss of weight occurred in nine patients, lower abdominal pains in four, and right upper quadrant pains in two. Half of the patients were severely anemic. Melanin in urine was looked for in 13 patients and was found in only 2. Thirteen patients were initially misdiagnosed, mostly as having hemorrhoids (8 patients). The size of the tumor at the time of diagnosis was over 5 cm in half of the patients (up to 12 cm), and in only two patients was it 0.5 cm. In 23 patients, most of the tumor mass was located below the dentate line, and in only 7 patients was it noted above it, invading the rectum. In all instances except one, a relative between the tumor and the squamous epithelium of the anal canal could be clearly demonstrated. Nine patients had satellite tumors around the main mass. Only four tumors were melanotic. At the time of diagnosis, 5 patients were in stage 1, a small tumor limited to mucosal involvement; 6 patients were in stage 2, a small tumor with muscular penetration; 7 patients were in stage 3, a large From the DepaOment of Surgery and Transplantation, Sheba Medical Center, Tel-Hashomer. Israel. Requests for reprints should be addressed to Bruno Siegal. MD, Chaim Sheba Medical Center, Department of General Surgery and Transplantation, Sackler School of Medicine, Tel-Hashomer 52621, Israel.
336
tumor and perirectal lymphatic involvement but no evidence of distant metastases, and 12 patients were in stage 4, widespread metastatic disease. The 30 patients were treated as follows: 15 underwent abdominoperineal resection of the anorectal area; 9 had local excision of the tumor, and the remaining 6 only had biopsies taken. Three of the 15 patients in the first group underwent groin lymph node dissection in addition to abdominoperineal resection. Seven patients had chemotherapy, and three had preoperative irradiation and immunotherapy (Figure 3). Results
Only two patients stayed alive more than 5 years postoperatively (64 and 124 months), both after local excision and chemotherapy. Twenty-two patients had a mean survival of 10.5 months, and 2 patients succumbed to the disease less then 1 month after diagnosis. All those patients died from the disease. Four patients were lost to follow-up, all of them with metastatic disease. A strong correlation was found between the stage of disease at the time of diagnosis and survival. The mean survival of the patients in stage 1 was 46.1 months (five patients), stage 2 12.4 months (five patients), stage 3 10.1 months (seven patients), and stage 4 only 5 months (nine patients). No correlation could be found between the type of treatment and survival. Fifteen patients who underwent abdominoperineal resection had a mean survival of 12 months. Comments
The first description of anorectal melanoma is attributed to Moore [8] in 1857. Since then, several outstanding reports on the subject have been published [g-14]. The relatively small number of cases in the previous series-34 cases from the Mayo Clinic from 1920 to 1977 and 51 from the Memorial SloanKettering Cancer Center from 1929 to 1977-are a reflection of the low incidence of the disease [Is--181. The peak incidences occur in the sixth, seventh, and eighth decades, later then the incidences for skin melanoma, which occurs in the fifth and sixth decades [19]. No relation was found between the age of the patients and the course of the disease [2,15]. A higher incidence of melanomas in persons with a poor tol-
The American Journal of Surgery
Surgical Treatment of Anorectal Melanomas
20
0
Males
@gg
30-39
40-49
50-59
60-69
Age in
o-79
60-69
Females
90.
year*
European JeWS
Arabs
Sephardic Jews
Figure 7. Age and sex distribution of 30 patients with anorecfal melanomas.
Figure 2. Ethnic origin of patients wffh anorectal melanomas.
erance to sunlight [20] has been reported. This fact has been highlighted in our series by a clear predominance of patients of European descent versus Sephardic Jews and Arabs. No other risk factors were identified in our series nor were they in other series. All researchers have emphasized the lack of specificity of symptoms, comparable with those of benign diseases of this anatomic area (hemorrhoids, for example). It should be noted that five of our patients had concomitant hemorrhoidal disease [15,20]. The treatment until today has been essentially surgical. It is interesting to note that Miles [21], the “father”
of the abdominoperineal resection of the rectum, did not recommend this procedure for anorectal melanomas. In 1931, he described three patients so treated by him and in all three he witnessed rapid postoperative deterioration. His message, however, has been forgotten, and some surgeons still perform the operation in cases of anorectal melanoma, probably because of its success in the treatment of adenocarcinema of rectum [4,16,22]. Pack and colleagues [20,23] went even further and advocated adding groin lymph node dissection to abdominoperineal resection. Procedures of this magnitude carry a significant
?= Lost
with
to follow metaStaSeS
up
30
22.5
Figws 3. ?ype of surgkal procedure and survival rate.
Volume 146, September 1993
Biopsy
only
Local
excision
Abdominoperineal
resection
337
Siegal et al
morbidity and mortality [20,24], and the results are not encouraging. Prophylactic lymph node dissection has been abandoned by most surgeons [14]. Today, only a few advocate radical surgery, since it does not increase the survivalsignificantlybut cripples the patient and affects the quality of life [25--271.Chemotherapy and radiotherapy have not brought about a significant improvement in survival [7,10,23], the melanoma cell being radio-resistant. It is too early to evaluate immunotherapy in the treatment of anorectal melanomas. The present treatment of anorectal melanoma relies on early diagnosis and accurate evaluation of metastatic spread. At an early stage the treatment consists of complete removal of the tumor. At all other stages, the therapeutic approach should aim at paliation, giving priority to quality of life over a minimal increase in survival that is associated with remarkablesuffering. Our impression is that survival has not been affected by the radical surgical approach. Improvement in the survival rate can be expected only when the diagnosis is made early in the course of the disease. Summary Thirty cases of anorectal melanoma have been recorded in Israel from 1960 to 1981. The frequency of the disease doubled in the last decade (from 10 to 20 cases). A clear predominance has been noted among Jews of European descent (18 patients) as opposed to Sephardic Jews (9 patients) or Arabs (3 patients). From this series, the emerging conclusion is that in most cases radical surgery (abdominoperineal resection) is seldom indicated. It is doubtful whether this form of treatment increasesthe survival while affecting considerably the quality of life. Survival correlated well with the stage of disease at the time of diagnosis. No such correlation could be established with the type of treatment employed. References 1. Weston SD. Marren M. Malignant melanoma of the rectum. J Int Coil Surg 1952;17:403-16. 2. Mason JK, Helwig EB. Ano-rectal malignant melanoma. Cancer 1966;19:39-50. 3. Allen AC, Spitz S. Malignant melanoma. A clinico-pathological analysis of the criteria for diagnosis and prognosis. Cancer 1953;6:1-45. 4. Braastad FW, Dockerty MB, Dixon CF. Melano-epithelioma of
338
5. 6.
7. 8.
9. 10. 11. 12.
13.
14. 15. 16. 17. 18.
19.
20.
21.
22.
23. 24. 25.
26. 27.
the anus and rectum. Report of cases and review of literature. Surgery 1949;25:62-90. Morson BC, Volkstadt H. Malignant melanoma of the anal canal. J Clin Pathol 1963;16:126-32. Boey J, Choi TK, Wong J, Ong GB. The surgical management of anorectal malignant melanoma. Aust NZ J Surg 1961; 15:132-6. Knutson CO, Hori GM, Spratt JS. Malignant melanoma. Curr Probl Surg 1971;6:2-55. Moore WD. Recurrent melanosis of the rectum after previous removal from the verge of the anus in a man aged sixty-five. Lancet 1857;1:290-4. Klausner J, Rosin R, Merav A. The surgical treatment of malignant melanoma. Harefuah 1978;95:49-51. Raven RW. Anorectal malignant melanoma. Proc R Sot Med 1948;41:469-74. Dasgupta TK, Brasfield RD. Paglia MA. Primary melanomas in unusual sites. Surg Gynecol Obstet 1969; 128:841-8. Giler S, Kesler H, Kot I. Malignant melanoma of the rectum: report of a case and review of the literature. Harefuah 1978;95:337-8. Chalier A, Bonnet P. Les tumeurs melaniques primitives du rectum Ref Chir (Paris) 1912;46:914-57. 1913;47:235.372, 563. Chin YS, Unni KK, Beart RW. Malignant melanoma of the anorectum. Dis Colon Rectum 1980;23:122-4. &an SHQ, White JE, Deddish MR. Malignant melanoma of the anorectum. Dis Colon Rectum 1959;2:275-83. Quan SHQ. Anal and para-anal tumors. Surg Clin North Am 1978;58:591-603. Wanebo HJ, Woodruff JM, Quan SHQ. Anorectal melanoma. Cancer 1981;47:1891-7. Delaney LT, Scudamore HH, Waugh JM. Malignant melanoma of the rectum: report of case. Mayo Clin Proc 1954;29: 418-20. Kwan TH, Mihm MC. Common disorders of pigmentation and tumors of melanocytes. In: Robbins SL, Cotran RS, eds. The pathologic basis of diseases. 2nd ed. Philadelphia: WB Saunders, 1979:1432-g. Pack GT, Oropeza R. A comparative study of melanoma and epidermoid carcinoma of the anal canal: a review of 20 melanomas and 29 epidermold carcinomas (1930 to 1965). Dis Colon Rectum 1967;10:161-75. Miles WE. The pathology of the spread of cancer of ths rectum, and its bearing upon the surgery of the cancerous rectum. Surg Gynecol Obstet 1931;52:350-9. Abaas JS, Karakousis CP, Holyoke DE. Anorectal melanoma: clinical features, recurrence and patient survival. Int Surg 1980;65:423-6. Pack GT, Martins FG. Treatment of anorectal malignant melanoma. Dis Colon Rectum 1960;3: 15-24. Goligher JC. Surgery of the anus, rectum and colon. 3rd ed. London: Baillier-Tindall, 1975:645-6. Berkley JI. Melanoma of the anal canal: report of a case of five-year survival after abdominoperineal resection. Dis Colon Rectum 1960;3:159-61. Husa A, Hockerstedt K. Anorectal malignant melanoma: a report of fourteen cases. Acta Chir Stand 1974;140:68-72. Garnick M, Lokich JJ. Primary malignant melanoma of the rectum: rationale for conservative surgical management. J Surg Oncol 1978;10:529-31.
The American Journal of
SUWry
Bruno Siegal, MD, FACS, Tel-Hashomer, David Cohen, MD, Tel-Hashomer,
Israel
Israel
Erwin T. Jacob, MD, Tel-Hashomer,
Israel
Anorectal melanoma is a rare disease, comprising less than 1 percent of all melanomas and 1 percent of all malignant tumors of the anus and rectum [l--4]. Failure td recognize the specific features of this disease has led to its inclusion with the rest of malignant tumors of this anatomic area. This had led to inappropriate and sometimes unnecessary treatment in a number of patients. Difficulties in reaching an early diagnosis, few therapeutic options, and a very low 5 year survival rate (less then 10 percent) [5-71, characterize this particular melanoma. All anorectal melanomas that occurred in Israel from 1960 to 1981 (30 cases) form the basis of this series. Ten cases occurred in the first decade of life and 20 in the last decade. Material
and Methods
Seventeen women and 13 men formed the study population for this series. The youngest was 35 years old and the oldest 93 years (mean 64 years) (Figure 1). Eighteen patients were Jews of European descent, 9 were Sephardic Jews, and 3 were Arabs (Figure 2). The most frequently noted symptoms were: rectal bleeding (25 patients), anal pain (24 patients), changes in bowel habits (15 patients), tenesmus (13 patients), and anal mass (8 patients). Half of the patients exhibited signs of disseminated disease. Significant loss of weight occurred in nine patients, lower abdominal pains in four, and right upper quadrant pains in two. Half of the patients were severely anemic. Melanin in urine was looked for in 13 patients and was found in only 2. Thirteen patients were initially misdiagnosed, mostly as having hemorrhoids (8 patients). The size of the tumor at the time of diagnosis was over 5 cm in half of the patients (up to 12 cm), and in only two patients was it 0.5 cm. In 23 patients, most of the tumor mass was located below the dentate line, and in only 7 patients was it noted above it, invading the rectum. In all instances except one, a relative between the tumor and the squamous epithelium of the anal canal could be clearly demonstrated. Nine patients had satellite tumors around the main mass. Only four tumors were melanotic. At the time of diagnosis, 5 patients were in stage 1, a small tumor limited to mucosal involvement; 6 patients were in stage 2, a small tumor with muscular penetration; 7 patients were in stage 3, a large From the DepaOment of Surgery and Transplantation, Sheba Medical Center, Tel-Hashomer. Israel. Requests for reprints should be addressed to Bruno Siegal. MD, Chaim Sheba Medical Center, Department of General Surgery and Transplantation, Sackler School of Medicine, Tel-Hashomer 52621, Israel.
336
tumor and perirectal lymphatic involvement but no evidence of distant metastases, and 12 patients were in stage 4, widespread metastatic disease. The 30 patients were treated as follows: 15 underwent abdominoperineal resection of the anorectal area; 9 had local excision of the tumor, and the remaining 6 only had biopsies taken. Three of the 15 patients in the first group underwent groin lymph node dissection in addition to abdominoperineal resection. Seven patients had chemotherapy, and three had preoperative irradiation and immunotherapy (Figure 3). Results
Only two patients stayed alive more than 5 years postoperatively (64 and 124 months), both after local excision and chemotherapy. Twenty-two patients had a mean survival of 10.5 months, and 2 patients succumbed to the disease less then 1 month after diagnosis. All those patients died from the disease. Four patients were lost to follow-up, all of them with metastatic disease. A strong correlation was found between the stage of disease at the time of diagnosis and survival. The mean survival of the patients in stage 1 was 46.1 months (five patients), stage 2 12.4 months (five patients), stage 3 10.1 months (seven patients), and stage 4 only 5 months (nine patients). No correlation could be found between the type of treatment and survival. Fifteen patients who underwent abdominoperineal resection had a mean survival of 12 months. Comments
The first description of anorectal melanoma is attributed to Moore [8] in 1857. Since then, several outstanding reports on the subject have been published [g-14]. The relatively small number of cases in the previous series-34 cases from the Mayo Clinic from 1920 to 1977 and 51 from the Memorial SloanKettering Cancer Center from 1929 to 1977-are a reflection of the low incidence of the disease [Is--181. The peak incidences occur in the sixth, seventh, and eighth decades, later then the incidences for skin melanoma, which occurs in the fifth and sixth decades [19]. No relation was found between the age of the patients and the course of the disease [2,15]. A higher incidence of melanomas in persons with a poor tol-
The American Journal of Surgery
Surgical Treatment of Anorectal Melanomas
20
0
Males
@gg
30-39
40-49
50-59
60-69
Age in
o-79
60-69
Females
90.
year*
European JeWS
Arabs
Sephardic Jews
Figure 7. Age and sex distribution of 30 patients with anorecfal melanomas.
Figure 2. Ethnic origin of patients wffh anorectal melanomas.
erance to sunlight [20] has been reported. This fact has been highlighted in our series by a clear predominance of patients of European descent versus Sephardic Jews and Arabs. No other risk factors were identified in our series nor were they in other series. All researchers have emphasized the lack of specificity of symptoms, comparable with those of benign diseases of this anatomic area (hemorrhoids, for example). It should be noted that five of our patients had concomitant hemorrhoidal disease [15,20]. The treatment until today has been essentially surgical. It is interesting to note that Miles [21], the “father”
of the abdominoperineal resection of the rectum, did not recommend this procedure for anorectal melanomas. In 1931, he described three patients so treated by him and in all three he witnessed rapid postoperative deterioration. His message, however, has been forgotten, and some surgeons still perform the operation in cases of anorectal melanoma, probably because of its success in the treatment of adenocarcinema of rectum [4,16,22]. Pack and colleagues [20,23] went even further and advocated adding groin lymph node dissection to abdominoperineal resection. Procedures of this magnitude carry a significant
?= Lost
with
to follow metaStaSeS
up
30
22.5
Figws 3. ?ype of surgkal procedure and survival rate.
Volume 146, September 1993
Biopsy
only
Local
excision
Abdominoperineal
resection
337
Siegal et al
morbidity and mortality [20,24], and the results are not encouraging. Prophylactic lymph node dissection has been abandoned by most surgeons [14]. Today, only a few advocate radical surgery, since it does not increase the survivalsignificantlybut cripples the patient and affects the quality of life [25--271.Chemotherapy and radiotherapy have not brought about a significant improvement in survival [7,10,23], the melanoma cell being radio-resistant. It is too early to evaluate immunotherapy in the treatment of anorectal melanomas. The present treatment of anorectal melanoma relies on early diagnosis and accurate evaluation of metastatic spread. At an early stage the treatment consists of complete removal of the tumor. At all other stages, the therapeutic approach should aim at paliation, giving priority to quality of life over a minimal increase in survival that is associated with remarkablesuffering. Our impression is that survival has not been affected by the radical surgical approach. Improvement in the survival rate can be expected only when the diagnosis is made early in the course of the disease. Summary Thirty cases of anorectal melanoma have been recorded in Israel from 1960 to 1981. The frequency of the disease doubled in the last decade (from 10 to 20 cases). A clear predominance has been noted among Jews of European descent (18 patients) as opposed to Sephardic Jews (9 patients) or Arabs (3 patients). From this series, the emerging conclusion is that in most cases radical surgery (abdominoperineal resection) is seldom indicated. It is doubtful whether this form of treatment increasesthe survival while affecting considerably the quality of life. Survival correlated well with the stage of disease at the time of diagnosis. No such correlation could be established with the type of treatment employed. References 1. Weston SD. Marren M. Malignant melanoma of the rectum. J Int Coil Surg 1952;17:403-16. 2. Mason JK, Helwig EB. Ano-rectal malignant melanoma. Cancer 1966;19:39-50. 3. Allen AC, Spitz S. Malignant melanoma. A clinico-pathological analysis of the criteria for diagnosis and prognosis. Cancer 1953;6:1-45. 4. Braastad FW, Dockerty MB, Dixon CF. Melano-epithelioma of
338
5. 6.
7. 8.
9. 10. 11. 12.
13.
14. 15. 16. 17. 18.
19.
20.
21.
22.
23. 24. 25.
26. 27.
the anus and rectum. Report of cases and review of literature. Surgery 1949;25:62-90. Morson BC, Volkstadt H. Malignant melanoma of the anal canal. J Clin Pathol 1963;16:126-32. Boey J, Choi TK, Wong J, Ong GB. The surgical management of anorectal malignant melanoma. Aust NZ J Surg 1961; 15:132-6. Knutson CO, Hori GM, Spratt JS. Malignant melanoma. Curr Probl Surg 1971;6:2-55. Moore WD. Recurrent melanosis of the rectum after previous removal from the verge of the anus in a man aged sixty-five. Lancet 1857;1:290-4. Klausner J, Rosin R, Merav A. The surgical treatment of malignant melanoma. Harefuah 1978;95:49-51. Raven RW. Anorectal malignant melanoma. Proc R Sot Med 1948;41:469-74. Dasgupta TK, Brasfield RD. Paglia MA. Primary melanomas in unusual sites. Surg Gynecol Obstet 1969; 128:841-8. Giler S, Kesler H, Kot I. Malignant melanoma of the rectum: report of a case and review of the literature. Harefuah 1978;95:337-8. Chalier A, Bonnet P. Les tumeurs melaniques primitives du rectum Ref Chir (Paris) 1912;46:914-57. 1913;47:235.372, 563. Chin YS, Unni KK, Beart RW. Malignant melanoma of the anorectum. Dis Colon Rectum 1980;23:122-4. &an SHQ, White JE, Deddish MR. Malignant melanoma of the anorectum. Dis Colon Rectum 1959;2:275-83. Quan SHQ. Anal and para-anal tumors. Surg Clin North Am 1978;58:591-603. Wanebo HJ, Woodruff JM, Quan SHQ. Anorectal melanoma. Cancer 1981;47:1891-7. Delaney LT, Scudamore HH, Waugh JM. Malignant melanoma of the rectum: report of case. Mayo Clin Proc 1954;29: 418-20. Kwan TH, Mihm MC. Common disorders of pigmentation and tumors of melanocytes. In: Robbins SL, Cotran RS, eds. The pathologic basis of diseases. 2nd ed. Philadelphia: WB Saunders, 1979:1432-g. Pack GT, Oropeza R. A comparative study of melanoma and epidermoid carcinoma of the anal canal: a review of 20 melanomas and 29 epidermold carcinomas (1930 to 1965). Dis Colon Rectum 1967;10:161-75. Miles WE. The pathology of the spread of cancer of ths rectum, and its bearing upon the surgery of the cancerous rectum. Surg Gynecol Obstet 1931;52:350-9. Abaas JS, Karakousis CP, Holyoke DE. Anorectal melanoma: clinical features, recurrence and patient survival. Int Surg 1980;65:423-6. Pack GT, Martins FG. Treatment of anorectal malignant melanoma. Dis Colon Rectum 1960;3: 15-24. Goligher JC. Surgery of the anus, rectum and colon. 3rd ed. London: Baillier-Tindall, 1975:645-6. Berkley JI. Melanoma of the anal canal: report of a case of five-year survival after abdominoperineal resection. Dis Colon Rectum 1960;3:159-61. Husa A, Hockerstedt K. Anorectal malignant melanoma: a report of fourteen cases. Acta Chir Stand 1974;140:68-72. Garnick M, Lokich JJ. Primary malignant melanoma of the rectum: rationale for conservative surgical management. J Surg Oncol 1978;10:529-31.
The American Journal of
SUWry