A n a l Fibrosarcoma: Report of a Case and Review of Literature* MANUEL H . ]~SPINOSA, M . D . , t
STUART H . Q. QUAN, M . D . ~
From the Memorial Sloan-Kettering Cancer Center, New York, New Yorh
FIBROSARCOMA
occurring
at
the
and was unable to sit comfortably. Severe burning pain was experienced after each bowel movement. Wheu he was first seen at the Cancer Center, steady pain had been persistent for the preceding four months, although he had been able to obtain temporary relief with Percodan (Endo). T h e r e was no fecal incontinence. Past medical history included esophageal hiatal hernia repair, exploratory laparotomy for intestinal obstruction due to adhesions, appendectomy, cholecystectomy, and common bile duct exploration. T h e patient had been treated for pulmonary tuberculosis in the past. He had been a heavy smoker and had stopped seven years previously. He had pulmonary emphysema and had had myocardial infarction in May 1973.
anal
region has not been reported in the modern literature. This malignant tumor,
which
connective-tissue
is c o m m o n l y
located in the
extremities and trunk, rarely occurs in the a l i m e n t a r y t r a c t . T h i s first r e p o r t e d case o f a n a l f i b r o s a r c o m a is a n a d d i t i o n lection
of
rare
anorectal
to t h e col-
tumors.
This
m a k e s a t o t a l of 14 cases of f i b r o s a r c o m a located at the anorectum reported in the past half century; rectum
except
the
all w e r e l o c a t e d i n t h e one
described
in
this
report. Report
of a Case
A 66-year-old white male retired laborer was referred to Memorial Sloan-Kettering Cancer Center on January 13, 1974, for further evaluation and management of nonhealing perianal ulceration. On September 26, 1972, fistulectomy with posterior sphincterotomy had been performed for anal fistula. The results of the pathologic examination were consistent with benign fistula. Pain and drainage from the operative site persisted, and the area was re-excised on October 13, 1972. T h e pathologic studies revealed reactive fibrosis. A month later a 2-cm ulcer with a shiny rolled edge was described. This was suspected to be squamous-cell carcinoma. Re-excision was performed on February 2, 1973. T h e pathologic studies showed aggressive fibromatosis, which was confirmed at the Armed Forces Institute of Pathology. Some consultants at that institute, however, called it fibrosarcoma. T h e ulcer recurred and was re-excised two more times on June 22, 1973, and on August 16, 1973. T h e wound did not heal and the ulcer became larger. All this time the patient had been suffering perianal pain * Read at the Spring Residents' Meeting of the New York Society of Colon and Rectal Surgeons, New York, New York, March 14, t974. t Fellow, Department of Surgery. ++Attending Surgeon, Colon and Rectal Service, Department of Surgery. Address reprint requests to Dr. Quan: Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, New York 10021.
FIG. 1. T h e ulcerated lesion in the left posterolateral perianal area, measuring 5 • 2.5 cm and extending 1.5 cm into the anal c a n a l 522
Dis. Col. & Rect. September 1975
Volume 18 Number 6
Volume 18 Number 6
ANAL FIBROSARCOMA
523
FIG. 2. The surgical specimen. Tip of scalpel blade points at anal sphincter, grossly involved by tumor. On admission to Memorial Sloan-Kettering Cancer Center on January 18, 1974, the patient was moderately short of breath and was complaining of perianal pain. He had the habitus and physical findings of emphysema. There were scars in the abdomen from previous operations. There was no significant palpable inguinal node. In the left posterolateral perianal area was an ulcer 9.5 • 5 cm in diameter, with raised indurated irregular edges and a necrotic base (Fig. 1). The ulcer extended posteriorly to the anal canal 1.5 cm from the anal margin. Proctosigmoidoscopic examination disclosed no mucosal abnormality in the rectum and sigmold colon. The EKG was compatible with an old myocardial infarction. Chest x-ray revealed a density in the apex of the right upper lobe which had been present for several years, consistent with the healed pulmonary tuberculosis. The results of intravenous pyelogram and barium-enema studies were normal. Complete blood count, blood chemistries and urine were normal. Pulmonary function study revealed chronic obstructive pulmonary disease. Slides from the outside source and the wedge biopsy of the perianal ulcer done at the Memorial SloanKettering Cancer Center revealed low-grade fibro-
sarcoma. Anticipating the cardiopulmonary condition of the pateint, intensive preoperative pulmonary physiotherapy was instituted and the patient was prepared for surgery. On January 25, 1974, with the patient under general anesthesia, an abdominoperineal resection was performed. Adhesions in the upper abdomen were present from the previous operations, but there was no intra- or retroperitoneal mass. The proximal sigmoid colon was utilized as an endsigmoid colostomy. A wide perineal en-bloc resection including the coccyx was done. In spite of the patient's cardioputmonary status, the postoperative course was essentially uncomplicated. He was discharged on February 16, 1974, on a regular diet, and was able to take care of his colostomy and perianal wound. He has been followed regularly in the colon and rectal clinic and found to be free of disease 11 months after definitive surgery. Gross Pathology: (Fig. 2) The perianal skin elipse, measuring 15.5 • 6 cm, was contiguous with the anal canal, rectum, and distal sigmoid colon. The perianal ulcer, measuring 5 X 2.5 • 1.5 cm, eccentrically located, had a granular base and
524
ESPINOSA AND QUAN
Dis. Col. & Rect. September 1975
FIG. 3. Photomicrograph of fibrosarcoma, grade 2, infiltrating subcutaneous tissue and anal sphincter (X 80). irregular, raised and indurated edges. Cut section revealed a firm whitish tumor mass infiltrating the subcutaneous tissue and the anal sphincter. The lymph nodes of the rectum and sigmoid colon appeared normal. Microscopic Pathology: Microscopic examination showed fully malignant fibrosarcoma, grade 2 (Fig. 3). The skin, subcutaneous tissue and anal sphincter muscles were infiltrated by the fibrous connective-tissue tumor. The margin of resection, rectum, sigmoid colon, and lymph nodes were histologically full of tumor.
Discussion Fibrosarcoma is a m a l i g n a n t t u m o r arising f r o m f b r o u s connective tissue. I t is most c o m m o n in the extremities a n d trunk, o c c u r r i n g less frequently in the h e a d a n d neck, a n d very rarely in the alimentary tract. I t has been r e p o r t e d to have developed in scars from various causes, inc l u d i n g p u n c t u r e wounds, surgical incisions,
burns, hematomas, arsenic injection sites, i n d o l e n t skin ulcers, b l u n t trauma, osteomyelitis, and irradiation portals. Desmoid t u m o r , or the so-called "nonmetastasizing" low-grade fibrosarcoma, h a d often been correlated with stretching of the a b d o m i n a l wall d u r i n g pregnancy. Stout 2a r e p o r t e d that 36 in his series of 218 cases (206 if desmoid t u m o r is excluded) of fibrosarcoma developed f r o m a scar or site of previous tissue injury. However, valid statistical significance c a n n o t be correlated with this as a causative factor. T h e o v e r w h e l m i n g m a j o r i t y of scars and areas of traumatized tissue do n o t develop fibrosarcoma. Evidence indicates that there are m o r e fibrosarcomas o c c u r r i n g at the p r i m a r y site w i t h o u t scars or history of significant tissue injury.
Volume 18 Number 6
ANAL FIBROSARCOMA
Illustrated in this case report is a fibrosarcoma diagnosed after repeated excision of anal fistula. T h e question whether surgical trauma was an etiologic factor, or whether previous excisions were not deep enough to include the tumor that was already present during the initial operation, remains to be answered. Interesting, but not unusual, is the fact that superficial biopsy revealed low-grade fibrosarcoma, while the depth of the resected t u m o r revealed a fully malignant process or a grade 2 fibrosarcoma. Rare malignant anorectal tumors have been reported sporadically. I n order of diminishing frequency these tumors are: 1) squamous-cell carcinoma 21 (variants: cloacogenic, basaloid) 2 and transitionalcell carcinoma, etc.); 2) carcinoid; Is 3) malignant melanoma; 17 4) lymphoma; 5) leiomyosarcoma; 16 6) rhabdomyosarcoma; 7) basal-cell carcinoma; 8) pseudoinflammatory colloid carcinoma2; 9) fibrosarcoma (Table 1). T h e r e are very few cases of fibrosarcoma of the rectum reported in the world literatureA, s, 9, 11, 13, 14, 18, 22 Many of the so-called "fibrosarcomas" of the anorectum reported were not classified as such, but were spindle-cell sarcomas, a broad morphologic term. T h e confusion and difficulty in diagnosis arises from the intermingling of different spindle-cell sarcomas with those arising from fibrous connective tissue. T h e review by Russell and Hughes 18 is a representative illustration of different spindle-cell sarcomas. T h e r e were seven fibrosarcomas of the rectum in that review. In 1923, Pennington 14 reviewed 60 cases of sarcoma of the anorectum and found only two cases of fibrosarcoma. In 1927, Weeks 25 reviewed 100 cases of sarcoma of the rectum but did not describe a single case of fibrosarcoma. In 1933, Kallet and Saltztein 10 quoted the two cases previously mentioned by Pennington but did not find any new cases. At the Ochsner Clinic 9 there
525
Rare Anorectal Tumors in Order o[ Diminishing Frequency
TABLE I.
1. Squamous-cell carcinoma (cloacogenic, basaloid and transitional) 2. Carcinoid 3. Melanoma 4. Lymphoma 5. Leiomyosarcoma 6. Rhabdomyosarcoma 7. Basal-cell carcinoma 8. Pseudoinflammatory colloid carcinoma 9. Fibrosarcoma
were only two cases of fibrosarcoma of the rectum a m o n g 30,000 patients admitted for proctologic evaluation in a period of eight years. B a c o n ) in his series of 2,160 cases of colonic and rectal cancer, reported only three rectal fibrosarcomas. As of 1964, 18 years after the establishment of the cancer registry in Victoria, not a case of anorectal fibrosarcoma had been recorded, is At the Memorial Sloan-Kettering Cancer Center, prior to this report, there was not a single case of anorectal fibrosarcoma recorded in the history of this institution, or at least as tong as the records have been kept. However, there was a case of desmoid t u m o r involving the levator ani muscleJ At the time of this writing there is no reported case of anal fibrosarcoma, but there are 13 cases of rectal fibrosarcoma in the world literature. Data are available for ten patients only (Table 2). Eight were male and two, female. T h e age range was 31 to 73 years; most of the patients were in the sixth and seventh decades of life. Although every patient has either a rectal or an anal mass, the patient usually seeks medical attention because of a particular complaint, predominantly pain. Rectal examinations of three of the patients revealed that the masses had extended to the anal canal. T h e patient reported in this paper was seen because of a problem of non-healing perianal ulcer, but this was accompanied by persistent pain.
526
ESPINOSA
.s
~
0
o
AND
QUAN
Dis. CoL & Rect. September 1975
~ ~
0
z
~a
~
STUART H . Q. QUAN, M . D . ~
From the Memorial Sloan-Kettering Cancer Center, New York, New Yorh
FIBROSARCOMA
occurring
at
the
and was unable to sit comfortably. Severe burning pain was experienced after each bowel movement. Wheu he was first seen at the Cancer Center, steady pain had been persistent for the preceding four months, although he had been able to obtain temporary relief with Percodan (Endo). T h e r e was no fecal incontinence. Past medical history included esophageal hiatal hernia repair, exploratory laparotomy for intestinal obstruction due to adhesions, appendectomy, cholecystectomy, and common bile duct exploration. T h e patient had been treated for pulmonary tuberculosis in the past. He had been a heavy smoker and had stopped seven years previously. He had pulmonary emphysema and had had myocardial infarction in May 1973.
anal
region has not been reported in the modern literature. This malignant tumor,
which
connective-tissue
is c o m m o n l y
located in the
extremities and trunk, rarely occurs in the a l i m e n t a r y t r a c t . T h i s first r e p o r t e d case o f a n a l f i b r o s a r c o m a is a n a d d i t i o n lection
of
rare
anorectal
to t h e col-
tumors.
This
m a k e s a t o t a l of 14 cases of f i b r o s a r c o m a located at the anorectum reported in the past half century; rectum
except
the
all w e r e l o c a t e d i n t h e one
described
in
this
report. Report
of a Case
A 66-year-old white male retired laborer was referred to Memorial Sloan-Kettering Cancer Center on January 13, 1974, for further evaluation and management of nonhealing perianal ulceration. On September 26, 1972, fistulectomy with posterior sphincterotomy had been performed for anal fistula. The results of the pathologic examination were consistent with benign fistula. Pain and drainage from the operative site persisted, and the area was re-excised on October 13, 1972. T h e pathologic studies revealed reactive fibrosis. A month later a 2-cm ulcer with a shiny rolled edge was described. This was suspected to be squamous-cell carcinoma. Re-excision was performed on February 2, 1973. T h e pathologic studies showed aggressive fibromatosis, which was confirmed at the Armed Forces Institute of Pathology. Some consultants at that institute, however, called it fibrosarcoma. T h e ulcer recurred and was re-excised two more times on June 22, 1973, and on August 16, 1973. T h e wound did not heal and the ulcer became larger. All this time the patient had been suffering perianal pain * Read at the Spring Residents' Meeting of the New York Society of Colon and Rectal Surgeons, New York, New York, March 14, t974. t Fellow, Department of Surgery. ++Attending Surgeon, Colon and Rectal Service, Department of Surgery. Address reprint requests to Dr. Quan: Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, New York 10021.
FIG. 1. T h e ulcerated lesion in the left posterolateral perianal area, measuring 5 • 2.5 cm and extending 1.5 cm into the anal c a n a l 522
Dis. Col. & Rect. September 1975
Volume 18 Number 6
Volume 18 Number 6
ANAL FIBROSARCOMA
523
FIG. 2. The surgical specimen. Tip of scalpel blade points at anal sphincter, grossly involved by tumor. On admission to Memorial Sloan-Kettering Cancer Center on January 18, 1974, the patient was moderately short of breath and was complaining of perianal pain. He had the habitus and physical findings of emphysema. There were scars in the abdomen from previous operations. There was no significant palpable inguinal node. In the left posterolateral perianal area was an ulcer 9.5 • 5 cm in diameter, with raised indurated irregular edges and a necrotic base (Fig. 1). The ulcer extended posteriorly to the anal canal 1.5 cm from the anal margin. Proctosigmoidoscopic examination disclosed no mucosal abnormality in the rectum and sigmold colon. The EKG was compatible with an old myocardial infarction. Chest x-ray revealed a density in the apex of the right upper lobe which had been present for several years, consistent with the healed pulmonary tuberculosis. The results of intravenous pyelogram and barium-enema studies were normal. Complete blood count, blood chemistries and urine were normal. Pulmonary function study revealed chronic obstructive pulmonary disease. Slides from the outside source and the wedge biopsy of the perianal ulcer done at the Memorial SloanKettering Cancer Center revealed low-grade fibro-
sarcoma. Anticipating the cardiopulmonary condition of the pateint, intensive preoperative pulmonary physiotherapy was instituted and the patient was prepared for surgery. On January 25, 1974, with the patient under general anesthesia, an abdominoperineal resection was performed. Adhesions in the upper abdomen were present from the previous operations, but there was no intra- or retroperitoneal mass. The proximal sigmoid colon was utilized as an endsigmoid colostomy. A wide perineal en-bloc resection including the coccyx was done. In spite of the patient's cardioputmonary status, the postoperative course was essentially uncomplicated. He was discharged on February 16, 1974, on a regular diet, and was able to take care of his colostomy and perianal wound. He has been followed regularly in the colon and rectal clinic and found to be free of disease 11 months after definitive surgery. Gross Pathology: (Fig. 2) The perianal skin elipse, measuring 15.5 • 6 cm, was contiguous with the anal canal, rectum, and distal sigmoid colon. The perianal ulcer, measuring 5 X 2.5 • 1.5 cm, eccentrically located, had a granular base and
524
ESPINOSA AND QUAN
Dis. Col. & Rect. September 1975
FIG. 3. Photomicrograph of fibrosarcoma, grade 2, infiltrating subcutaneous tissue and anal sphincter (X 80). irregular, raised and indurated edges. Cut section revealed a firm whitish tumor mass infiltrating the subcutaneous tissue and the anal sphincter. The lymph nodes of the rectum and sigmoid colon appeared normal. Microscopic Pathology: Microscopic examination showed fully malignant fibrosarcoma, grade 2 (Fig. 3). The skin, subcutaneous tissue and anal sphincter muscles were infiltrated by the fibrous connective-tissue tumor. The margin of resection, rectum, sigmoid colon, and lymph nodes were histologically full of tumor.
Discussion Fibrosarcoma is a m a l i g n a n t t u m o r arising f r o m f b r o u s connective tissue. I t is most c o m m o n in the extremities a n d trunk, o c c u r r i n g less frequently in the h e a d a n d neck, a n d very rarely in the alimentary tract. I t has been r e p o r t e d to have developed in scars from various causes, inc l u d i n g p u n c t u r e wounds, surgical incisions,
burns, hematomas, arsenic injection sites, i n d o l e n t skin ulcers, b l u n t trauma, osteomyelitis, and irradiation portals. Desmoid t u m o r , or the so-called "nonmetastasizing" low-grade fibrosarcoma, h a d often been correlated with stretching of the a b d o m i n a l wall d u r i n g pregnancy. Stout 2a r e p o r t e d that 36 in his series of 218 cases (206 if desmoid t u m o r is excluded) of fibrosarcoma developed f r o m a scar or site of previous tissue injury. However, valid statistical significance c a n n o t be correlated with this as a causative factor. T h e o v e r w h e l m i n g m a j o r i t y of scars and areas of traumatized tissue do n o t develop fibrosarcoma. Evidence indicates that there are m o r e fibrosarcomas o c c u r r i n g at the p r i m a r y site w i t h o u t scars or history of significant tissue injury.
Volume 18 Number 6
ANAL FIBROSARCOMA
Illustrated in this case report is a fibrosarcoma diagnosed after repeated excision of anal fistula. T h e question whether surgical trauma was an etiologic factor, or whether previous excisions were not deep enough to include the tumor that was already present during the initial operation, remains to be answered. Interesting, but not unusual, is the fact that superficial biopsy revealed low-grade fibrosarcoma, while the depth of the resected t u m o r revealed a fully malignant process or a grade 2 fibrosarcoma. Rare malignant anorectal tumors have been reported sporadically. I n order of diminishing frequency these tumors are: 1) squamous-cell carcinoma 21 (variants: cloacogenic, basaloid) 2 and transitionalcell carcinoma, etc.); 2) carcinoid; Is 3) malignant melanoma; 17 4) lymphoma; 5) leiomyosarcoma; 16 6) rhabdomyosarcoma; 7) basal-cell carcinoma; 8) pseudoinflammatory colloid carcinoma2; 9) fibrosarcoma (Table 1). T h e r e are very few cases of fibrosarcoma of the rectum reported in the world literatureA, s, 9, 11, 13, 14, 18, 22 Many of the so-called "fibrosarcomas" of the anorectum reported were not classified as such, but were spindle-cell sarcomas, a broad morphologic term. T h e confusion and difficulty in diagnosis arises from the intermingling of different spindle-cell sarcomas with those arising from fibrous connective tissue. T h e review by Russell and Hughes 18 is a representative illustration of different spindle-cell sarcomas. T h e r e were seven fibrosarcomas of the rectum in that review. In 1923, Pennington 14 reviewed 60 cases of sarcoma of the anorectum and found only two cases of fibrosarcoma. In 1927, Weeks 25 reviewed 100 cases of sarcoma of the rectum but did not describe a single case of fibrosarcoma. In 1933, Kallet and Saltztein 10 quoted the two cases previously mentioned by Pennington but did not find any new cases. At the Ochsner Clinic 9 there
525
Rare Anorectal Tumors in Order o[ Diminishing Frequency
TABLE I.
1. Squamous-cell carcinoma (cloacogenic, basaloid and transitional) 2. Carcinoid 3. Melanoma 4. Lymphoma 5. Leiomyosarcoma 6. Rhabdomyosarcoma 7. Basal-cell carcinoma 8. Pseudoinflammatory colloid carcinoma 9. Fibrosarcoma
were only two cases of fibrosarcoma of the rectum a m o n g 30,000 patients admitted for proctologic evaluation in a period of eight years. B a c o n ) in his series of 2,160 cases of colonic and rectal cancer, reported only three rectal fibrosarcomas. As of 1964, 18 years after the establishment of the cancer registry in Victoria, not a case of anorectal fibrosarcoma had been recorded, is At the Memorial Sloan-Kettering Cancer Center, prior to this report, there was not a single case of anorectal fibrosarcoma recorded in the history of this institution, or at least as tong as the records have been kept. However, there was a case of desmoid t u m o r involving the levator ani muscleJ At the time of this writing there is no reported case of anal fibrosarcoma, but there are 13 cases of rectal fibrosarcoma in the world literature. Data are available for ten patients only (Table 2). Eight were male and two, female. T h e age range was 31 to 73 years; most of the patients were in the sixth and seventh decades of life. Although every patient has either a rectal or an anal mass, the patient usually seeks medical attention because of a particular complaint, predominantly pain. Rectal examinations of three of the patients revealed that the masses had extended to the anal canal. T h e patient reported in this paper was seen because of a problem of non-healing perianal ulcer, but this was accompanied by persistent pain.
526
ESPINOSA
.s
~
0
o
AND
QUAN
Dis. CoL & Rect. September 1975
~ ~
0
z
~a
~
