Int J Colorect Dis (1992) 7:167-169
Colorectal Disease 9 Springer-Verlag 1992
Perianal Paget's disease: report of five cases S. Goldman 1, T. Ihre 1 U. Lagerstedt 2, and C. Svensson 3 1 Department of Surgery, S6dersjukhuset, Stockholm, Sweden, Sweden z Department of Pathology, S6dersjukhuset, Stockholm, Sweden, Sweden 3 Department of Oncology, S6dersjukhuset, Stockholm, Sweden, Sweden Accepted: 11 December 1992
Abstract. P a g e t ' s disease o f the a n u s is a r a r e p e r i a n a l d i s o r d e r . T h e c o n d i t i o n is o f t e n a s s o c i a t e d with u n d e r l y ing invasive c a r c i n o m a . T h e p r o g n o s i s is p o o r w h e n rectal a d e n o c a r c i n o m a is present. F i v e o w n cases o f peria n a l P a g e t ' s disease are p r e s e n t e d . I n two o f o u r cases a n u n d e r l y i n g a d e n o c a r c i n o m a was f o u n d in the a n o r e c t u m . A d e n o c a r c i n o m a is sweat g l a n d d u c t s was f o u n d in one case. O n e p a t i e n t d e v e l o p e d a n a d e n o c a r c i n o m a in the a n o r e c t a l j u n c t i o n f o u r y e a r s after the P a g e t d i a g n o s e . In o n l y one o f o u r cases n o u n d e r l y i n g a d e n o c a r c i n o m a was found. R6sum6. L a m a l a d i e de P a g e t de l ' a n u s est une 16sion p 6 r i - a n a l e rare. Cet ~tat est s o u v e n t associ6 avec u n cancer i n v a s i f sous-jacent. Le p r o n o s t i c est m a u v a i s lorsq u ' u n c a n c e r d u r e c t u m est pr6sent. 5 cas p e r s o n n e l s de m a l a d i e de P a g e t p 6 r i - a n a l e s o n t pr6sent6s. D a n s deux de ces cas un c a n c e r s o u s - j a c e n t a 6t6 trouv6 d a n s l ' a n o - r e c turn. U n c a n c e r des c a n a u x g l a n d u l a i r e s s u d o r i p a r e s existait chez un m a l a d e . U n p a t i e n t a d6velopp6 u n a d 6 n o c a r c i n o m e de la j o n c t i o n a n o - r e c t a l e 4 ans apr+s le d i a g n o s t i c de m a l a d i e de Paget. C h e z u n seul de nos cas s e u l e m e n t il n ' e x i s t a i t p a s d ' a d 6 n o c a r c i n o m e sous-jacent.
Introduction P a g e t ' s disease was first d e s c r i b e d b y Sir J a m e s P a g e t in 1874 in 15 p a t i e n t s with u n d e r l y i n g d u c t a l b r e a s t c a n c e r [1]. T h e disease h a s since t h a t time been d e s c r i b e d in o t h e r a p o c r i n e g l a n d b e a r i n g a r e a s such as a r o u n d the anus, vulva, s c r o t u m , g r o i n a n d axilla [2]. A review o f the litera t u r e in 1988 r e v e a l e d o n l y 96 cases l o c a t e d in the a n a l a r e a [3]. Case r e p o r t s i n d i c a t e t h a t the presence o f p e r i a n a l P a g e t cells carries a n a p p r o x i m a t e l y 7 0 % risk o f a n u n d e r l y i n g m a l i g n a n c y [4, 5]. T h e r e is u s u a l l y a very l o n g d e l a y in d i a g n o s i s [6]. A s the p r o g n o s i s is p o o r w h e n rectal a d e n o c a r c i n o m a is present, we w a n t to d r a w a t t e n tion to this c o n d i t i o n b y r e p o r t i n g five cases a c c r u e d a t S r d e r s j u k h u s e t , S t o c k h o l m , d u r i n g the time p e r i o d 1984-1989.
Report of cases Patient 1. A 63-year-old man with psoriasis presented with a 2-year history of anal pruritus. He had been treated with various topical antifungal agents and topical corticosteroids without any improvement. On physical examination there was an erythematous plaque with a maximum diameter of 3 cm, 1.5 cm from the anal verge on the left side. A punch biopsy from the perianal lesion showed Paget's disease confined to the epidermis. Rectal palpation and proctosigmoidoscopy was normal and there were no palpable inguinal lymph nodes. The patient received radiotherapy (anterior/ posterior portals, mid plane fractionation dose 2.0 Gy 5 days/week) up to 40 Gy. Clinical examination and biopsy 3 weeks after completion of irradiation revealed no Paget cells and no further treatment was given. Four years later the patient developed a tumour at the six o'clock position in the upper anal canal with a maximum diameter of 1 cm. Local excision disclosed a well-differentiated adenocarcinoma without Paget pattern. The tumour infiltrated the muscularis mucosae but no deeper. However, the tumour was not radically excised and an abdominoperineal resection was therefore performed. At the time of the first clinical check-up 3 months after surgery there was no sign of recurrence. Patient 2. A 75-year-old man with a 1-year history of pruritus ani consulted a private practitioner for a perianal lesion on the right side close to the anal verge. Clinical examination revealed an erythematous lesion of a maximum diameter of 1.5 cm which was locally excised. Microscopy revealed Paget cells both in the epidermis and in adnexa without underlying invasive carcinoma. He was referred to the surgical department 2 months later for further treatment. The subsequent clinical examination disclosed a perianal lesion on the left side within an area of 3 x 3.5 cm close to the anal orifice. Moreover, a 6 mm anal polyp fixed on an anal prolapse was seen. Proctosigmoidoscopy verified the polyp but no other tumours were seen. A colonic barium enema was normal. A wide local excision of the perianal skin without skin graft was performed. The anal polyp was excised at the same time. Microscopy showed the polypoid lesion to be a villous adenoma with severe dysplasia, but with no sign of invasive carcinoma. Adjacent squamous epithelium from the anus contained Paget cells. The perianal tissue disclosed a typical picture of Paget's disease confined to the epithelium, but apparently not radically excised. A new perianal local excision was performed. The specimen contained adenocarcinoma cells in Paget pattern and in tubular structures, probably sweat gland ducts. Three months later after healing of the defect the patient received radiotherapy (anterior/posterior portals, mid-plane
168
Fig. 1. Case 3. Perianal Paget's disease appearing as an erythematous plaque Fig. 2. Case 5. Moderately differentiated adenocarcinoma - the primary neoplasm was located in the upper anal canal. (H&E) Fig. 3. Case 5. Paget cells infiltrating the perianal epidermis (Hematoxylin and eosin)
fractionation dose 2.0 Gy 5 days/week) up to 66 Gy. Sixteen months after completion of radiotherapy the patient remains recurrence free. Patient 3. An 80-year-old man presented with a 3-month history of alteration of his bowel habits. Rectal palpation disclosed a hyperplastic anal polyp but there was also a perianal erythematous plaque of a maximum diameter of 4 cm close to the anal verge (Fig. 1). Multiple punch biopsies from the perianal plaque showed Paget cellsconfined to the epidermis without underlying invasive carcinoma. Proctosigmoidoscopy was normal and a barium enema showed only diverticulosis in the sigmoid colon. The patient underwent a wide local excision of the perianal disease without skin graft. The operation was microscopically radical. Paget ceils were found only in the epidermis. The wound healed after 8 weeks without complications and the patient is still recurrence free 12 months after surgery. Patient 4. A 66-year-old woman, who at the age of 50 was operated upon for a benign stomach ulcer, presented with a 1-year history of pruritus ani and rectal pain. At clinical examination a perianal erythematous plaque with a maximum diameter of 5 cm, circumfer-
entially located was found. Rectal palpation disclosed a 1.5 cm hard tumour 3 cm above the anal verge. Enlarged inguinal lymph nodes were palpable. Biopsies from the perianal plaque showed Paget cells confined to the epidermis. Biopsies from the tumour in the anal canal showed a poorly differentiated adenocarcinoma. Unfortunately, the patient died of sepsis 1 week after diagnosis. Autopsy revealed a poorly differentiated adenocarcinoma of the rectum. Multiple lymph node metastases were found in the perirectal fat, retroperitoneally around the aorta, and in the liver. Patient 5. A 53-year-old woman was referred to hospital with a 6-month history of pruritus ani. Rectal examination revealed a hard mass in the upper anal canal, but also an erythematous lesion extending perianally 3 cm from the anal verge, circumferentially located. Biopsies of the perianal lesion showed Paget cells in the epidermis, mainly in the basal portion. A biopsy of the turnout in the anal canal revealed an invasive adenocarcinoma. No other abnormality was seen on procto-sigmoidoscopy or barium enema. There were no palpable inguinal lymph nodes. A radical abdominoperineal resection was performed with a wide perianal excision. Histopathology of the resected specimen showed a moderately differentiated adenoearcinoma measuring 4 cm in its greatest di-
169 mension in the ano-rectal region (Fig. 2). One lymph node metastasis was found. The squamous epithelium of the anus and perianal skin contained carcinoma cells in Paget pattern (Fig. 3). Seventeen months after operation the patient died from liver metastasis.
Discussion Paget's disease of the perianal region is rare, so experience of this disease is limited at most institutions and the literature consists mainly o f case reports. The major differential diagnoses include Bowen's disease, pagetoid melanoma, squamous cell carcinoma, cloacogenic carcinoma, mycosis fungoides, and dermatitis [2]. Four of our five patients complained of perianal itching from an erythematous plaque, which is in accordance with other reported cases in the literature [7, 8]. Microscopically, the Paget cell is a rounded large cell with pale vacuolated cytoplasm and a large nucleus often displaced to the periphery of the cell, a so-called signet ring cell [2]. Paget ceils generally stain positive for mucin (in the cytoplasm) with Alcian blue and PAS (periodic acid schiff). All our cases had morphologically typical Paget cells which contained mucin that could be visualized with PAS-stain. In two of our cases an underlying adenocarcinoma located in the anorectum was found (cases 4 and 5). Adenocarcinoma in sweat gland ducts was found in one case (case 2). One patient developed an adenocarcinoma in the anorectal junction 4 years after the diagnosis of Paget's disease diagnose (case 1). In only one of our cases was no underlying adenocarcinoma found (case 3). Our five cases illustrate two of the major theories of the histogenesis of Paget's disease [2, 9, 10]: 1) A primary adenocarcinoma develops in the gastrointestinal tract, mainly rectum or anus, spreads into the contiguous epidermis of the skin (cases 4 and 5); 2) The primary disease develops in the epidermis and spreads to the dermal adnexa, mainly hair follicles and sweat glands, by continuity (cases 1-3). A wide local excision with or without skin graft is recommended for patients with perianal Paget's disease without co-existing anorectal carcinoma, as the clinical limit of Paget's disease does not always correspond with the histological extent. According to Jones et al., Paget cells often extend within epidermis horizontally beyond what is clinically apparent as perianal Paget's disease [2]. It is important that the edge of the surgical specimen is checked by frozen section before ending local surgery. If underlying carcinoma is present abdominoperineal resection seems to give the best chance for cure [7, 8]. However, the prognosis of the patients with an underlying
carcinoma of the anorectum is reported to be much poorer than for patients with Paget's disease restricted to the skin and adnexa [7, 8, 11]. This is in agreement with our experience. Therefore it is of great importance to examine patients with perianal Paget's disease carefully both clinically by rectal palpation and procto-sigmoidoscopy and by x-ray to exclude an underlying adenocarcinoma of the bowel. The experience of primary radiotherapy in cases with underlying adenocarcinoma is very limited. Beck and Fazio reported on two patients who underwent such therapy, However, both died of metastatic disease within a year after treatment [7]. In the present series one o f our two patients who was irradiated is still disease free, but follow-up (16 months) is short. The other patient had a local recurrence after 4 years. However, this patient received a dose of only 40 Gy. Finally, as the risk of developing recurrent cancer is substantial amongst patients with Paget's disease prolonged and careful follow-up is indicated.
References 1. Paget J 0874) On disease of the mammary areola preceding cancer of the mammary gland. St Barth Hosp Rep 10:87-89 2. Jones RE, Craig A, Ackerman AB (1979) Extramammary Paget's disease. A critical reexamination. Am J Dermatopathology 1:101-132 3. Berardi B, Lee S, Chen HP (1988). Perianal extramammary Paget's disease. Surg Gynecol Obstet 167:359-366 4. Weiber H, Ekelund G, Lindstr6m C (1987) Perianal Paget's disease. Acta Chir Scand 153:687-690 5. Tjandra J (1988) Perianal Paget's disease. Report of three cases. Dis Colon Rectum 31:462-466 6. Kavlie H, Stevenson JK, Gould VE (1971) Perianal Paget's disease. Am Surg 37:485-489 7. BeckDE, Fazio VW (1987). Perianal Paget's disease. Dis Colon Rectum 30:263-266 8. Shutze WP, Gleysteen JJ (1990): Perianal Paget's disease. Classification and review of management: Report of two cases. Dis Colon Rectum 33:502-507 9. Wood WS, Culling CG (1975) Perianal Paget's disease: histochemical differentiation utilizing the borohydride-KOH-PAS reaction. Arch Pathol 99:442-445 10. Brender LD, Schwartz RA (1983) Extramammary Paget's disease. Am Fam Physician 28:159-161 11. Williams SL, Rodgers LW, Quan SHQ (1976) Perianal Paget's disease. Report of seven cases. Dis Colon Rectum 19:30-40 Dr. Sven Goldman Department of Surgery S6dersjukhuset S-11883 Stockholm Sweden
Colorectal Disease 9 Springer-Verlag 1992
Perianal Paget's disease: report of five cases S. Goldman 1, T. Ihre 1 U. Lagerstedt 2, and C. Svensson 3 1 Department of Surgery, S6dersjukhuset, Stockholm, Sweden, Sweden z Department of Pathology, S6dersjukhuset, Stockholm, Sweden, Sweden 3 Department of Oncology, S6dersjukhuset, Stockholm, Sweden, Sweden Accepted: 11 December 1992
Abstract. P a g e t ' s disease o f the a n u s is a r a r e p e r i a n a l d i s o r d e r . T h e c o n d i t i o n is o f t e n a s s o c i a t e d with u n d e r l y ing invasive c a r c i n o m a . T h e p r o g n o s i s is p o o r w h e n rectal a d e n o c a r c i n o m a is present. F i v e o w n cases o f peria n a l P a g e t ' s disease are p r e s e n t e d . I n two o f o u r cases a n u n d e r l y i n g a d e n o c a r c i n o m a was f o u n d in the a n o r e c t u m . A d e n o c a r c i n o m a is sweat g l a n d d u c t s was f o u n d in one case. O n e p a t i e n t d e v e l o p e d a n a d e n o c a r c i n o m a in the a n o r e c t a l j u n c t i o n f o u r y e a r s after the P a g e t d i a g n o s e . In o n l y one o f o u r cases n o u n d e r l y i n g a d e n o c a r c i n o m a was found. R6sum6. L a m a l a d i e de P a g e t de l ' a n u s est une 16sion p 6 r i - a n a l e rare. Cet ~tat est s o u v e n t associ6 avec u n cancer i n v a s i f sous-jacent. Le p r o n o s t i c est m a u v a i s lorsq u ' u n c a n c e r d u r e c t u m est pr6sent. 5 cas p e r s o n n e l s de m a l a d i e de P a g e t p 6 r i - a n a l e s o n t pr6sent6s. D a n s deux de ces cas un c a n c e r s o u s - j a c e n t a 6t6 trouv6 d a n s l ' a n o - r e c turn. U n c a n c e r des c a n a u x g l a n d u l a i r e s s u d o r i p a r e s existait chez un m a l a d e . U n p a t i e n t a d6velopp6 u n a d 6 n o c a r c i n o m e de la j o n c t i o n a n o - r e c t a l e 4 ans apr+s le d i a g n o s t i c de m a l a d i e de Paget. C h e z u n seul de nos cas s e u l e m e n t il n ' e x i s t a i t p a s d ' a d 6 n o c a r c i n o m e sous-jacent.
Introduction P a g e t ' s disease was first d e s c r i b e d b y Sir J a m e s P a g e t in 1874 in 15 p a t i e n t s with u n d e r l y i n g d u c t a l b r e a s t c a n c e r [1]. T h e disease h a s since t h a t time been d e s c r i b e d in o t h e r a p o c r i n e g l a n d b e a r i n g a r e a s such as a r o u n d the anus, vulva, s c r o t u m , g r o i n a n d axilla [2]. A review o f the litera t u r e in 1988 r e v e a l e d o n l y 96 cases l o c a t e d in the a n a l a r e a [3]. Case r e p o r t s i n d i c a t e t h a t the presence o f p e r i a n a l P a g e t cells carries a n a p p r o x i m a t e l y 7 0 % risk o f a n u n d e r l y i n g m a l i g n a n c y [4, 5]. T h e r e is u s u a l l y a very l o n g d e l a y in d i a g n o s i s [6]. A s the p r o g n o s i s is p o o r w h e n rectal a d e n o c a r c i n o m a is present, we w a n t to d r a w a t t e n tion to this c o n d i t i o n b y r e p o r t i n g five cases a c c r u e d a t S r d e r s j u k h u s e t , S t o c k h o l m , d u r i n g the time p e r i o d 1984-1989.
Report of cases Patient 1. A 63-year-old man with psoriasis presented with a 2-year history of anal pruritus. He had been treated with various topical antifungal agents and topical corticosteroids without any improvement. On physical examination there was an erythematous plaque with a maximum diameter of 3 cm, 1.5 cm from the anal verge on the left side. A punch biopsy from the perianal lesion showed Paget's disease confined to the epidermis. Rectal palpation and proctosigmoidoscopy was normal and there were no palpable inguinal lymph nodes. The patient received radiotherapy (anterior/ posterior portals, mid plane fractionation dose 2.0 Gy 5 days/week) up to 40 Gy. Clinical examination and biopsy 3 weeks after completion of irradiation revealed no Paget cells and no further treatment was given. Four years later the patient developed a tumour at the six o'clock position in the upper anal canal with a maximum diameter of 1 cm. Local excision disclosed a well-differentiated adenocarcinoma without Paget pattern. The tumour infiltrated the muscularis mucosae but no deeper. However, the tumour was not radically excised and an abdominoperineal resection was therefore performed. At the time of the first clinical check-up 3 months after surgery there was no sign of recurrence. Patient 2. A 75-year-old man with a 1-year history of pruritus ani consulted a private practitioner for a perianal lesion on the right side close to the anal verge. Clinical examination revealed an erythematous lesion of a maximum diameter of 1.5 cm which was locally excised. Microscopy revealed Paget cells both in the epidermis and in adnexa without underlying invasive carcinoma. He was referred to the surgical department 2 months later for further treatment. The subsequent clinical examination disclosed a perianal lesion on the left side within an area of 3 x 3.5 cm close to the anal orifice. Moreover, a 6 mm anal polyp fixed on an anal prolapse was seen. Proctosigmoidoscopy verified the polyp but no other tumours were seen. A colonic barium enema was normal. A wide local excision of the perianal skin without skin graft was performed. The anal polyp was excised at the same time. Microscopy showed the polypoid lesion to be a villous adenoma with severe dysplasia, but with no sign of invasive carcinoma. Adjacent squamous epithelium from the anus contained Paget cells. The perianal tissue disclosed a typical picture of Paget's disease confined to the epithelium, but apparently not radically excised. A new perianal local excision was performed. The specimen contained adenocarcinoma cells in Paget pattern and in tubular structures, probably sweat gland ducts. Three months later after healing of the defect the patient received radiotherapy (anterior/posterior portals, mid-plane
168
Fig. 1. Case 3. Perianal Paget's disease appearing as an erythematous plaque Fig. 2. Case 5. Moderately differentiated adenocarcinoma - the primary neoplasm was located in the upper anal canal. (H&E) Fig. 3. Case 5. Paget cells infiltrating the perianal epidermis (Hematoxylin and eosin)
fractionation dose 2.0 Gy 5 days/week) up to 66 Gy. Sixteen months after completion of radiotherapy the patient remains recurrence free. Patient 3. An 80-year-old man presented with a 3-month history of alteration of his bowel habits. Rectal palpation disclosed a hyperplastic anal polyp but there was also a perianal erythematous plaque of a maximum diameter of 4 cm close to the anal verge (Fig. 1). Multiple punch biopsies from the perianal plaque showed Paget cellsconfined to the epidermis without underlying invasive carcinoma. Proctosigmoidoscopy was normal and a barium enema showed only diverticulosis in the sigmoid colon. The patient underwent a wide local excision of the perianal disease without skin graft. The operation was microscopically radical. Paget ceils were found only in the epidermis. The wound healed after 8 weeks without complications and the patient is still recurrence free 12 months after surgery. Patient 4. A 66-year-old woman, who at the age of 50 was operated upon for a benign stomach ulcer, presented with a 1-year history of pruritus ani and rectal pain. At clinical examination a perianal erythematous plaque with a maximum diameter of 5 cm, circumfer-
entially located was found. Rectal palpation disclosed a 1.5 cm hard tumour 3 cm above the anal verge. Enlarged inguinal lymph nodes were palpable. Biopsies from the perianal plaque showed Paget cells confined to the epidermis. Biopsies from the tumour in the anal canal showed a poorly differentiated adenocarcinoma. Unfortunately, the patient died of sepsis 1 week after diagnosis. Autopsy revealed a poorly differentiated adenocarcinoma of the rectum. Multiple lymph node metastases were found in the perirectal fat, retroperitoneally around the aorta, and in the liver. Patient 5. A 53-year-old woman was referred to hospital with a 6-month history of pruritus ani. Rectal examination revealed a hard mass in the upper anal canal, but also an erythematous lesion extending perianally 3 cm from the anal verge, circumferentially located. Biopsies of the perianal lesion showed Paget cells in the epidermis, mainly in the basal portion. A biopsy of the turnout in the anal canal revealed an invasive adenocarcinoma. No other abnormality was seen on procto-sigmoidoscopy or barium enema. There were no palpable inguinal lymph nodes. A radical abdominoperineal resection was performed with a wide perianal excision. Histopathology of the resected specimen showed a moderately differentiated adenoearcinoma measuring 4 cm in its greatest di-
169 mension in the ano-rectal region (Fig. 2). One lymph node metastasis was found. The squamous epithelium of the anus and perianal skin contained carcinoma cells in Paget pattern (Fig. 3). Seventeen months after operation the patient died from liver metastasis.
Discussion Paget's disease of the perianal region is rare, so experience of this disease is limited at most institutions and the literature consists mainly o f case reports. The major differential diagnoses include Bowen's disease, pagetoid melanoma, squamous cell carcinoma, cloacogenic carcinoma, mycosis fungoides, and dermatitis [2]. Four of our five patients complained of perianal itching from an erythematous plaque, which is in accordance with other reported cases in the literature [7, 8]. Microscopically, the Paget cell is a rounded large cell with pale vacuolated cytoplasm and a large nucleus often displaced to the periphery of the cell, a so-called signet ring cell [2]. Paget ceils generally stain positive for mucin (in the cytoplasm) with Alcian blue and PAS (periodic acid schiff). All our cases had morphologically typical Paget cells which contained mucin that could be visualized with PAS-stain. In two of our cases an underlying adenocarcinoma located in the anorectum was found (cases 4 and 5). Adenocarcinoma in sweat gland ducts was found in one case (case 2). One patient developed an adenocarcinoma in the anorectal junction 4 years after the diagnosis of Paget's disease diagnose (case 1). In only one of our cases was no underlying adenocarcinoma found (case 3). Our five cases illustrate two of the major theories of the histogenesis of Paget's disease [2, 9, 10]: 1) A primary adenocarcinoma develops in the gastrointestinal tract, mainly rectum or anus, spreads into the contiguous epidermis of the skin (cases 4 and 5); 2) The primary disease develops in the epidermis and spreads to the dermal adnexa, mainly hair follicles and sweat glands, by continuity (cases 1-3). A wide local excision with or without skin graft is recommended for patients with perianal Paget's disease without co-existing anorectal carcinoma, as the clinical limit of Paget's disease does not always correspond with the histological extent. According to Jones et al., Paget cells often extend within epidermis horizontally beyond what is clinically apparent as perianal Paget's disease [2]. It is important that the edge of the surgical specimen is checked by frozen section before ending local surgery. If underlying carcinoma is present abdominoperineal resection seems to give the best chance for cure [7, 8]. However, the prognosis of the patients with an underlying
carcinoma of the anorectum is reported to be much poorer than for patients with Paget's disease restricted to the skin and adnexa [7, 8, 11]. This is in agreement with our experience. Therefore it is of great importance to examine patients with perianal Paget's disease carefully both clinically by rectal palpation and procto-sigmoidoscopy and by x-ray to exclude an underlying adenocarcinoma of the bowel. The experience of primary radiotherapy in cases with underlying adenocarcinoma is very limited. Beck and Fazio reported on two patients who underwent such therapy, However, both died of metastatic disease within a year after treatment [7]. In the present series one o f our two patients who was irradiated is still disease free, but follow-up (16 months) is short. The other patient had a local recurrence after 4 years. However, this patient received a dose of only 40 Gy. Finally, as the risk of developing recurrent cancer is substantial amongst patients with Paget's disease prolonged and careful follow-up is indicated.
References 1. Paget J 0874) On disease of the mammary areola preceding cancer of the mammary gland. St Barth Hosp Rep 10:87-89 2. Jones RE, Craig A, Ackerman AB (1979) Extramammary Paget's disease. A critical reexamination. Am J Dermatopathology 1:101-132 3. Berardi B, Lee S, Chen HP (1988). Perianal extramammary Paget's disease. Surg Gynecol Obstet 167:359-366 4. Weiber H, Ekelund G, Lindstr6m C (1987) Perianal Paget's disease. Acta Chir Scand 153:687-690 5. Tjandra J (1988) Perianal Paget's disease. Report of three cases. Dis Colon Rectum 31:462-466 6. Kavlie H, Stevenson JK, Gould VE (1971) Perianal Paget's disease. Am Surg 37:485-489 7. BeckDE, Fazio VW (1987). Perianal Paget's disease. Dis Colon Rectum 30:263-266 8. Shutze WP, Gleysteen JJ (1990): Perianal Paget's disease. Classification and review of management: Report of two cases. Dis Colon Rectum 33:502-507 9. Wood WS, Culling CG (1975) Perianal Paget's disease: histochemical differentiation utilizing the borohydride-KOH-PAS reaction. Arch Pathol 99:442-445 10. Brender LD, Schwartz RA (1983) Extramammary Paget's disease. Am Fam Physician 28:159-161 11. Williams SL, Rodgers LW, Quan SHQ (1976) Perianal Paget's disease. Report of seven cases. Dis Colon Rectum 19:30-40 Dr. Sven Goldman Department of Surgery S6dersjukhuset S-11883 Stockholm Sweden
