Anal Canal Carcinoma with Pagetoid Spread: Report of a Case

A 70-year-old man with a history of colon polyps was found to have a semipedunculated polyp in the anal canal. The patient was asymptomatic. The lesion was 14 mm in diameter and located 5 mm from the dentate line. Histological examination of biopsy specimens revealed well-differentiated adenocarcinoma of the anal canal. During transanal local excision of the tumor, an abnormality of the perianal skin was recognized. Although intraoperative frozen section of the perianal skin did not show malignancy, permanent sections of the perineal skin revealed Paget's cells in the epidermis. Pathological examination of the anal canal carcinoma revealed submucosally invasive well-differentiated adenocarcinoma with a positive distal surgical margin. Thus, we performed additional wide local excision of the perianal skin including the distal margin of the previous local excision. Pathological examination revealed continuance within the epidermis between the anal canal adenocarcinoma and Paget's cells in the perianal skin lesion. Scattered Paget's cells also formed some glandular structures. Thus, we concluded that the perianal skin lesion was Pagetoid spread of anal canal adenocarcinoma. This report shows that the perianal skin should be examined carefully in patients with anal canal carcinoma.     

EXTRAMAMMARY PAGET's DISEASE OF THE PENIS AND SCROTUM: EXCISION, RECONSTRUCTION AND EVALUATION OF OCCULT MALIGNANCY

PURPOSE: We describe treatment and reconstruction in patients after surgery for extramammary Paget's disease of the penis and scrotum. We also investigated whether this disease causes an increased risk of undiagnosed visceral malignancy. MATERIALS AND METHODS: We reviewed the databases at our institution from 1996 to 2000 and identified 6 men 67 to 87 years old (mean age 76). In addition, we reviewed the literature on the clinical and pathological features of this disease. RESULTS: In our 6 patients scrotal involvement was present in 83% and penile extramammary Paget's disease was present in 33%. Each man underwent wide local excision and large skin defects were immediately reconstructed with split-thickness skin grafts. In 1 case extramammary Paget's disease had spread to the superficial inguinal nodes. At a mean followup of 29 months there has been no local recurrence and internal malignancy has not been diagnosed. Our literature review revealed 13 patients with penoscrotal extramammary Paget's disease and visceral malignancy, including 12 (92%) with malignancy of the genitourinary system. CONCLUSIONS: Extramammary Paget's disease of the penis and scrotum is a rare disease that can be managed by excision and immediate reconstruction with skin grafting or a local skin flap. Disease may spread to the regional lymph nodes. Although genitourinary cancer may accompany penoscrotal extramammary Paget's disease, an extensive search for cancer of the thorax or abdomen may be unnecessary because only 1 reported case of colon cancer has been associated with penile or scrotal extramammary Paget's disease.     

Preservation of anal function after total excision of the anal mucosa for Bowen''s disease

Six women with Bowen's disease of the anogenital area were treated by total excision of the anal mucosa, perianal skin and, in some cases, partial vulvectomy. Two patients had foci of microinvasive squamous carcinoma. Adequate tumor margins were determined by frozen sections. The resulting mucosal and cutaneous defects were grafted with medium split-thickness skin grafts applied to the anal canal and sutured circumferentially to the rectal mucosa. Grafts were held in place by a finger cot inserted in the anal canal and stuffed with cotton balls. Patients were constipated five or six days with codeine. The skin grafts healed per primam. One additional patient was similarly treated for a chronic herpetic ulceration of the anus and healed. Contrary to dire predictions, all patients were able to distinguish between gaseous and solid rectal contents and sphincter function was preserved. In one patient, Bowen's disease has recurred in the grafted perianal skin.     

Paget's disease of the anus: a clinicopathological study

Paget's disease of the anus is a rare disorder of controversial origin and is frequently associated with malignancy. We studied eight patients and carried out immunohistochemical studies to determine whether particular functional profiles might be indicators of a malignant association. One patient presented with synchronous carcinoma and two developed carcinomas 3 and 10 years after excision of Paget's disease. Five patients underwent wide local excision and have not developed cancer (median follow-up 6 years, range 5-13 years). However, four patients developed recurrent Paget's disease. Immunohistochemical studies showed that in general Paget cells stained positively with CAM 5.2 (a cytokeratin marker), gross cystic disease fluid protein (a marker for apocrine cells), human milk fat globule glycoprotein (HMFG 1 and 2) and carcinoembryonic antigen but negatively for PR3A5 (a marker for colonic goblet cells). Three cases had a staining profile which was quite different from that usually observed and these were associated with malignancy. One showed an antigenic profile more typical of a large bowel carcinoma. Paget's disease of the anus appears to run one of two clinical courses: to develop malignancy; or to recur locally, often on repeated occasions. Wide local excision is the treatment of choice but long-term follow-up is necessary because of the cancer risk. An immunohistochemical staining pattern which is different from usual may indicate a higher malignant risk and/or identify some cases of Paget's disease as representing a downward 'pagetoid' extension from a anorectal adenocarcinoma rather than a true epidermotropic apocrine neoplasm of the perianal skin.     

Successful Treatment of Recalcitrant Dissecting Cellulitis of the Scalp With Complete Scalp Excision and Split-Thickness Skin Graft

BACKGROUND: Dissecting cellulitis of the scalp (DCS) is a therapeutically challenging, chronic, progressive, suppurative disease of the scalp that is of unknown etiology. In addition to causing considerable discomfort and cosmetic disfigurement, long-standing lesions may result in the development of squamous cell carcinoma. Several treatment modalities for DCS have been employed with variable results. OBJECTIVE: To report the successful treatment of an aggressive, refractory case of DCS with complete scalp excision and split-thickness skin graft. METHODS: A 25-year-old black male with DCS was treated with complete scalp excision and split-thickness graft from the anterior thighs. RESULTS: The patient has remained free of disease activity and is satisfied with the cosmetic result. CONCLUSION: Complete scalp excision with split-thickness skin graft may be curative in patients with DCS and should be considered in recalcitrant cases that fail to respond to medical therapy.     

Bowen's disease of the anal and perianal area. A report and analysis of twelve cases.

Retrospective evaluation of twelve patients treated at the Cleveland Clinic for perianal Bowen's disease showed that these patients can be cured by wide local excision with skin grafting when necessary. No recurrence or metastasis was found during the follow-up period when the systematic technic was used. Only involved anal mucosa was removed, and normal mucosa was preserved; this aids in controlling anal continence. The margins of the resected skin must be subjected to frozen section study to be sure that total excision has been achieved. In this study a diagnosis of perianal Bowen's disease was made incidentally in six of the twelve patients during histologic examination of anorectal tissue removed for other reasons. Seven of the twelve either had had a systemic or cutaneous cancer previously or another systemic or cutaneous cancer subsequently developed, indicating the high association between Bowen's disease and other cancers. Therefore, it is important that all excised skin from anal or perianal operations be submitted for histologic examination, and if the diagnosis is Bowen's disease, the lesion must be completely removed using wide local excision. These patients should be evaluated and followed up because other malignancies may be present or evolve at a later time.     

Double anal canal cancers associated with a long history of perianal Paget’s disease: report of a case

Extramammary Paget's disease (EPD) is rare. We report a case of double anal canal cancers in a patient with a long history of perianal Paget's disease. The patient, a 68-year-old Japanese woman, refused surgery initially and was treated with electron beam therapy, which achieved remission. However, 6 years later, Paget's disease was found to be progressing again and double anal canal tumors were also detected in the proctos and external skin area. We performed abdominoperineal resection (Miles' operation) and lymph node dissection for the Paget's disease with double anal canal tumors. Immunohistochemical staining revealed cytokeratin (CK)-20 expression in the adenocarcinomas and Paget's disease lesion, but not CK-7 or gross cystic disease fluid protein-15 expression. The lesion was joined to the carcinoma by a stalk. The immunohistochemistry results suggested secondary EPD, although it was originally considered to be Paget's carcinoma (primary EPD) based on the clinical history.     

V-Y island flaps for repair of large perianal defects.

BACKGROUND: Some perianal diseases such as Paget disease and Bowen disease are extensive and require a wide circumferential excision including the entire anoderm of the anal canal. METHODS: We describe a technique of V-Y island flaps to cover the large perianal defects and the denuded anal canal. It is important to excise the base of the flaps in order to fit them into the anal canal. RESULTS: There were 10 women and 5 men with an average age of 54 years (range 32 to 77). The mean follow-up was 45 months (range 6 to 92). The underlying pathology included various kinds of neoplastic and nonneoplastic diseases. There were no major complications such as flap loss or infection. Most complications were minor, including superficial wound separation, flap hematoma, and anal stricture. Although initially all patients had some degree of incontinence for gas and liquid stool or discharge, none of them had significant fecal incontinence at the time of last follow-up. A diverting ileostomy or colostomy was created in 5 patients. Its role was not clear but it did help in the management of the wounds and minimized the pain.     

Long-term results of syndactyly correction: full-thickness versus split-thickness skin grafts

In order to compare the long-term results of full-thickness and split-thickness skin grafts after the correction of congenital syndactyly, 27 patients have been investigated after an average follow-up of 21 years. Post-operative functional and cosmetic results have been assessed by patient records, questionnaires and physical examination. The webs that had received split-thickness grafts showed more flexion and extension lags and the overall spreading of the operated fingers was significantly decreased compared to the control fingers. On the other hand, more re-operations because of web creep had to be performed after full-thickness grafts. Hyperpigmentation and hair growth in the grafts was found in most of the full-thickness grafts, while breakdown of the graft was found in some of the split-thickness grafts. Therefore, based on the results of this study, either full- or split-thickness skin grafts can be used when treating of congenital syndactyly.     

Outcome after surgical resection for high-grade anal intraepithelial neoplasia (Bowen's disease).

BACKGROUND: High-grade anal intraepithelial neoplasia (Bowen's disease) may predispose to anal carcinoma. Treatment options include surgical resection but effectiveness remains uncertain. This paper reports long-term follow-up of patients with high-grade anal intraepithelial neoplasia treated by surgical resection. METHODS: Between 1989 and 1996, 46 patients were identified with high-grade anal intraepithelial neoplasia. Thirty-four underwent local excision of all macroscopically abnormal disease and the resulting defect was left open, closed primarily or skin grafted. Regular follow-up subsequently included anoscopy and biopsy of any suspicious lesions. RESULTS: Median follow-up was 41 (range 12-104) months. Total excision was difficult; 19 patients had histological evidence of incomplete excision at the time of initial resection. Some 12 of 19 had histo-logically proven recurrent high-grade intraepithelial neoplasia within 1 year. Even with microscopically complete excision two of 15 patients subsequently developed recurrent high-grade intraepithelial neoplasia at 6 and 32 months after operation. No patient developed carcinoma but five had complica-tions of anal stenosis or faecal incontinence. CONCLUSION: Although no definite recommendations can be made for the treatment of high-grade anal intraepithelial neoplasia, these results illustrate some potential drawbacks of surgical excision with a high potential for incomplete excision and persistent disease, even after complete excision in some patients, and a high morbidity rate.     

Reconstruction of perianal skin defect using a V-Y advancement of bilateral gluteus maximus musculocutaneous flaps: reconstruction considering anal cleft and anal function.

In order to preserve the anal function after ano-perianal skin excision for malignancy, we have reconstructed a deep, symmetrical natal cleft using a V-Y advancement of bilateral gluteus maximus musculocutaneous flaps thinned medially and sutured to the ooccyx, anococcygeal ligament and the central tendon of the perineum. This technique was applied in three cases of Bowen's disease and two cases of Paget's disease. In all five cases, postoperative anal functions such as comfortable defecation and sensation, were well preserved, the perianal skin and underwear stayed clean, and there was no disturbance of walking or exercise.     

Argon laser therapy for perianal Bowen's disease: a case report

Bowen's disease (intraepithelial squamous cell carcinoma) may occasionally occur in anal canal or perianal region. Surgical excision in this area may result in significant complications and a recurrence rate of 9-19%. A case of successful treatment of anal and perianal Bowen's disease using argon laser therapy is presented. No acute or chronic complications resulted from laser therapy. Twenty-eight months after the initial diagnosis the patient is disease free. Laser therapy also provides the potential for repeated treatment of recurrent lesions without significant patient discomfort or inconvenience. Argon laser therapy should be considered as a therapeutic option in the treatment of Bowen's disease.     

复合皮移植修复烧伤功能部位创面疗效评价

目的探讨脱细胞同种异体真皮与自体刃厚皮复合移植修复深度烧伤功能部位创面的疗效。方法 2002年6月-2008年12月,收治30例烧伤及瘢痕整形患者共42个创面。男25例,女5例;年龄3～52岁,中位年龄31岁。烧伤24例35个创面,其中深Ⅱ度23个创面,Ⅲ度12个创面;病程3～45 d,平均24 d。瘢痕整形6例7个创面;病程9～21 d,平均16 d。42个创面分别位于颈部2个,手部4个,前臂及肘部8个,肩部3个,腘窝6个,膝部4个,小腿及足踝部15个。彻底清创、削痂及切除瘢痕后,创面范围为10 cm×10 cm～30 cm×20 cm。采用一步法将脱细胞同种异体真皮与自体刃厚皮复合移植修复创面。结果术后27例39个(92.9%)创面复合皮移植完全成活;3例3个(7.1%)创面复合皮部分坏死,分别经换药和自体皮片移植术后愈合。患者均获随访,随访时间30～34个月,平均32个月。复合皮有轻度色素沉着,外观平整,质地柔软,弹性好,皮肤耐磨;复合皮无挛缩及瘢痕增生,功能部位活动正常。刃厚皮供皮区未见瘢痕增生。结论脱细胞同种异体真皮与自体刃厚皮复合移植修复功能部位深度烧伤及瘢痕整形创面,可获得良好外形及功能。     

阴囊Paget病的诊治分析(附23例报告)

目的:探讨阴囊Paget病的诊断、治疗、复发因素及预后,提高诊治水平。方法:回顾性分析本院1996～2008年诊治的阴囊Paget病患者的临床资料。结果:23例患者中,局限于一侧阴囊者15例,累及双侧阴囊及阴茎者8例;病变同侧腹股沟淋巴结肿大者3例(2例术后病理证实为转移癌,1例为炎症),双侧腹股沟淋巴结肿大者2例(1例术后病理证实为转移癌,1例为慢性炎症改变)。全部病例均经活检明确诊断,并均行手术治疗。术后定期随访2～68个月,其中术后局部复发5例,因远处转移死亡2例。结论:活检是早期诊断阴囊Paget病重要方法,病变处阴囊的早期广泛切除是治疗阴囊Paget病的首选治疗手段。     

The surgical challenge of giant circumferential congenital naevi of the extremities: a 13-year follow-up of two cases

The surgical management of giant congenital melanocytic naevi involving extremities in a circumferential pattern may represent a surgical challenge with respect to their resection and subsequent reconstructive options. We present our experience in the management of circumferential giant congenital melanocytic naevi involving the upper limb in one patient and lower limb in another one. They were managed and followed up for 13 years. The lesion regenerated following dermabrasion in the first patient and regenerated following tangential excision and split-thickness skin graft in both patients. Therefore, another approach was used, full-thickness resection of the lesions and reconstruction with dermal regeneration template (Integra™), followed by ultrathin split-thickness skin graft. The last approach resulted in no recurrence during a 3-year follow-up. The complexity of these lesions and the limitation of different surgical approaches have been highlighted. Full-thickness excision of the affected integument and the use of dermal regeneration template is a useful technique which can be considered in the management of circumferential giant melanocytic naevi, particularly when other established methods have failed to provide satisfactory results.     

Paget's disease of the anal margin

A follow-up study of 22 patients with Paget's disease of the anal margin was conducted to determine the prevalence of invasive disease and coexisting visceral carcinomas, cure and survival rates. Seventy-three per cent of the patients suffered from persistent pruritus ani. Seven (32 per cent) patients had malignancy (invasive Paget's disease (n = 5) and coexisting visceral carcinoma (n = 2] and six (27 per cent) developed malignancy (invasive Paget's disease (n = 4) and coexisting visceral carcinoma (n = 2] during follow-up, giving an overall occurrence of malignancies of 59 per cent (95 per cent confidence limits: 36-79). The median follow-up period was 9 (range 0.5-25) years. No difference in length of history could be found between patients with or without malignancy. The estimated cure rate by actuarial analysis among radically treated patients was 64 per cent (95 per cent confidence limits: 43-91) at 1 year and 45 per cent (95 per cent confidence limits: 18-72) at 8 years after primary treatment. The 5- and 10-year crude survival rates of 54 per cent and 45 per cent, respectively, were significantly lower than the corresponding values of 84 per cent and 60 per cent for the normal population (P less than 0.01). Patients with persistent pruritus ani and a perianal skin lesion should be biopsied frequently. If Paget's disease is diagnosed, physical examination of the patient and anorectal region must be done carefully and repeatedly.     

Perianal Paget's disease-Report of a rare case

INTRODUCTIONPerianal extra-mammary Paget's disease is a rare skin disorder of unknown aetiology, which is frequently associated with malignancy. This case report draws attention to this rare condition and comments upon its diagnosis and treatment.PRESENTATION OF CASEA 64-year-old otherwise fit man, presented to us in 2006 with one-year-long history of perianal irritation. On examination there was an erythematous discoid skin lesion in the right perianal area. The lesion was excised with wide margins and the defect closed with a local transposition flap. Histology confirmed extra-mammary Paget's disease (EMPD) with a focus of invasion showing a well-differentiated mucinous adenocarcinoma. Adjuvant therapy was not advised. On follow-up in 2011, a small irregular skin lesion, well away from the previous excision site was noted on the left perianal area. Biopsies from this lesion confirmed EMPD with no focus of invasion. Once again wide local excision with closure using local transposition flap was undertaken. Long term follow up has been advised.DISCUSSIONThe optimal treatment for Perianal Paget's disease (PPD) remains controversial. Surgery is the commonest modality used with wide local excision being the treatment of choice for resectable disease. We report herein a short review of various therapies reported so far in the management of this rare disorder.CONCLUSIONA thorough initial evaluation and long-term follow-up is essential to identify recurrence and the development of other related malignancies.     

Perianal extramammary paget's disease associated with primary linitis plastica of the rectum: Report of a case

We report herein a case of perianal extramammary Paget's disease associated with primary linitis plastica of the rectum. An 82-year-old woman was admitted to our hospital for investigation and treatment of a perianal eczematous lesion. A skin biopsy of the lesion revealed perianal extramammary Paget's disease and a barium enema demonstrated diffuse narrowing with an irregular contour at the ampulla recti. Under a suspected diagnosis of linitis plastica of the rectum, an abdominoperineal resection was performed to resect both the rectal and perianal lesions with regional lymphadenectomy. Grossly, marked narrowing and wall thickness were observed at the lower rectum, and a histological diagnosis of signet ring cell carcinoma was confirmed. The perianal eczematous lesion revealed many atypical cells with clear cytoplasm, being Paget cells, throughout the entire epidermis. Sparse distributions of signet ring cells were also observed in the subcutaneous tissue beneath the perianal eczematous lesion. The pathogenesis of perianal extramammary Paget's disease in this patient was therefore considered to be an intraepidermal extension of primary linitis plastica of the rectum.     

Perianal Paget's disease: a case report

A case of perianal Paget's disease is presented. The salient features of this disease include perianal irritation, a perianal rash, and usually, as in this patient, an associated underlying carcinoma. The disease is rare and has to be differentiated from malignant melanoma, Bowen's disease and transitional cell carcinoma. This patient was also remarkable since the associated malignancy was an equally rare carcinoma of anal gland origin.     

Scrotal lymphangiomatosis: a case report

Lymphangiomas are benign tumours of the lymphatic system, and there are several reported cases of scrotal lymphangioma in the literature to date. We report a rare case of multilocular cutaneous lymphangiomatosis treated with surgical excision (total scrotectomy and reconstruction using split-thickness skin grafts with vacuum-assisted closure dressing).