Surgical treatment of perianal Paget\u27 disease using the gracillis musculocutaneous flap was reported on an 83-year-old patient\u27 with some discussions

Fukuda Toshiro

Kunisaki Tadaomi

Tomita Masao

Zibiki Masaaki

Nagasaki University's Academic Output SITE: NAOSITE

Acta Med. Nagasaki 35:203-205Extramammary Paget's DiseaseToshiro FUKUDA, Masaaki ZIBIKI, Tadaomi KUNISAKI              and Masao TOMITA** Fukuda Private Hospital in Sasebo. the First Department of Surgery,                  Nagasaki University HospitalReceived for publication, June 25, 1990SUMMARY: Surgical treatment of perianal Paget' disease using the gracillis musculocutaneous flap was reported on an 83-year-old patient' with some discussions.       INTRODUCTION  The term, extramammary Paget disease refers to a disease which is neoplastic lesion like eczema around genitalien and perianus regions. The extramammary Paget diseases occurred so frequently in heavily growing sites of the apocrine glands that the genitalien and the perianal regions are well known as a pre-dominant sites.  The best way to cure is complete resection before infiltrative extension via lymphatic channels and bloodbore metastasis of cancer cells. However, a great deal of difficulty in wide resection is how to manage the tissue defect. Recently advancement in plastic surgey have made it possible to reconstruct well, functionally and cosmetically. Pedicled musculocutaneous flap is suitable for reconstruction of tissue defect.  Surgical experience with a case suffering from extramammary disease in the perianal region is herein reported in terms of the use of musculocutaneous flap to cover the tissue defevt after the wide resection.         PATIENTS  A 83-years-old man has complained of erosion in the perianal region since December 1987.  As this lesion was gradually extending in itsextent, he consulted a dermatist and it was diagnosed as extramammary Paget' disease by biopsy in October 1988. Therefore, he was admitted in our hospital for surgical treatment in February 1989.  His general condition was good, and the values of CEA and AFP were within normal limit.  In the perianal region, erosion and redness were severe, and the lesions of depigmentation and pigmentaion coexisted. It was 9 X 6cm in size with slight degree of elevation as compared with the normal as shown in Fig. 1. Digital examination was free of any induration and stenosis. Colonoscopy revealed no abnormal finding in the proximal portion of the ampullaFig. 1. Preoperative macroscopic findings around       the anus, showing erosidon and depigmenta-      tion in part.recti. Surgical finding : The skin, maximun 3cm to minimum 1cm apart from the margin of the lesion, including subcutaneous fatty tissues was widely excised to expose the muscular fascia. In the anus the external sphincter muscles were preserved as much as possible and the anal canal was removed 2 cm proximal to the dented line. The surgically cut edge of the skin and the anal mucosa were histologically examined to exclude Paget cells by using frozen section.  Pedicled musculocutaneous flap was prepared from the gracillis muscles for the tissue defect as shown in Fig. 2. In the middle of the pedicled musculocutaneous flap, the anus plasty was made by suturing the anal mucosa to the hole of the skin flap as shown in Fig. 3.  The surgical specimen showed the skin of 12.0X 8.5cm in size and there were small polypoid lesions with erosion around the anus.  There were numerous large Paget cells withFig. 2. Schema of the operation method. Wide        resection of the skin around the anus and       transferred pedicled gracillis muscul-       ocutaneous flap.Fig. 3. Macroscopic finding at completion of re-       construction for skin defect around anus.foamy vacuolation cytoplasms and vesicular nuclei. The surgical margin was free of a presence of paget's cells.  Postoperative course: although oral intake was inhibited by IVH control, postoperative infection on day 6 in the anastomotic line between the skin and the anal mucosa led to hasten a diverting colostomy.  Thereafter, infection subsided under the control,of drugs, and it was possible to take food orally. However, ensuing stenosis required to make it distended and widended by Hegar dilator. He is alive well, enjoying his daily life until now /year after surgery.         DISCUSSION  Extramammary Paget's disease predomi-nates in the genitalien, axilla and perianal regions in which the apocrine glands were densely growing.  It is common that this lesion discloses slow extension of cancer infiltration within the epidermis. However, when it is extending to the basal membrane, it becomes infiltrative and cancer spreads widely metastasizing via lymphatic channels.  It is required in the treatment that it should be dealt with a wide resection of carcinoma, less than precussor in carcinoma and/or carcinoma in the epidermis as reported by Ohara". The questions regarding surgical operartion are how to treat metastatic lymphnodes2), how to determine the resection line') how to recon-struct the tissue as Soeba emphasized'). Nodal involvement has become manifest in infiltrative carcinoma although carcinoma in the epidermis frequently involved neighboring nodes.  When nodal involvement is suspected, the superficial inguinal nodes should be dissected. If definite nodal involvement exists, the surgical inguinal node as well as the internal iliac nodes must be dissected.  In this case there was no nodal involvement although the lesion was widely extended, as large as 9 X 6cm in size. Therefore, node dis-section was not attempted for the reasons of absence of nodal involvement and the old age of the patient.  As to the resection line, it is recommendedthat the resection line should be 3-5cm apart from the diseased margin','). It is emphasized that the diseased margin is to the margin of depigmentaion' ).  A wide resection is essential to carry out a complete removal and to prevent recurrence.  As it is limited for achivement of wide resection, so intraoperative histology is needed for adequate and minimal resections for this disease.  When cancer extension reaches the rectum, urethra, and vagina, it is not easy to determine the resection line apropriately. In particular, according to the rule of the resection extending to the rectum, amputation of the rectum should be selected as the operative procedure of choice. Soeda insisted that the resection line should be 5cm, at least 3cm, apart from the visible margin of the lesion.  In this case, the resection line in the rectum was kept within 2cm because it was non-infiltrative carcinoma and no residue of carci-noma was found by intraoperatively histologic examination.  As for reconstruction, it is recommeded that pedicled skin graft should be used for preven-tion of scar constriction of suture line. It is generally accepted that pedicled gracillis musculocutaneous flap is most favorablebecause it enables us to design easily primary suture and satisfactory wrap for the tissue defect.  It is also emphasized') that the pedicled musculocutaneous flap has rich blood flow and high resistance to infection.  Extramammary Paget's disease in the perianal region occurs frequently. When the lesion extends to the rectal mucosa, it is difficult to determine an ideal resection line. As far as we can apply frozen section for this purpose, adequate surgery would be promised.         REFERENCES 1) Ohara K et al: Surgical experience with perianal    Paget' disease. Clin. Dermat. 35:261-266, 1981 2) Soeda S et al: Operation for genital Paget'    disease in femal, Reconstruction using skin graft    and gracillis musculocutaneous flap and ure-   thral reconstruction. Jap. J Plastoc Surg. 1 : 150-  158, 1981. 3) Ohara K : Perianal skin lesion. Stomach and   Intestine 22 : 313-318, 1987. 4) Okumura K et al: Extramammary Paget' disease    plastic Surg. 30: 600-606, 1987. 5) Mizutani K et al: Treatment for extramammary    Paget disease. Jap. J. dermat, 97:171, 1987.

Extramammary Paget\u27s Disease

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Extramammary Paget\u27s Disease

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