Pilonidal sinus carcinoma

Pilonidal sinus carcinoma

Pilonidal Sinus Carcinoma JOHN L. TERRY, M.D., Columbus, O&o, JOHN C. GAISFORD, M.D. AND DWIGHT Pittsburgh, Pennylvania From tbe Department of Pla...

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Pilonidal

Sinus Carcinoma

JOHN L. TERRY, M.D., Columbus, O&o, JOHN C. GAISFORD, M.D. AND DWIGHT Pittsburgh, Pennylvania

From tbe Department

of Plastic and Reconstructive Surgery, University of Pittsburgh, Pittsburgh, Pennsylvania.

C. HANNA,

M.D.,

medical profession to the occurrence of carcinomatous transformation in degenerating and unstabIe scars. SeemingIy, with the passage of time, economy of medical expression has incIuded any carcinomatous degeneration of Iongstanding scars, cutaneous ulcers and various sinus tracts under the classification of “hlarjolin’s ulcers.”

T case

HE purpose of this paper is to present a of squamous ceI1 carcinoma arising in a pilonidal sinus of twenty-six years’ duration. A review of the Iiterature has produced only eight cases of malignant degeneration occurring in an area of piIonidal disease, and these wiIJ be summarized brieff y. The more common compIications retated to pilonidal disease are IocaI ceIIuIitis, abscess formation and recurrent sinuses [7]. SeveraI less frequently encountered compIications incIude osteomyeIitis of the sacrum, meningitis [I r,~?] and malignant degeneration of the cyst wal1 or sinus tract. MaIignant degeneration has occurred as a Iate compIication of many varieties of persistent wounds and chronicaIIy irritated tissues such as in empyema and osteomyeIitic sinuses [ro,r2J, varicose ulcers, ana fistuIas, bed sores, calIuses, and unstabIe scars of therma1, chemical and radiation burns [ry]. FortunateIy, the incidence of such maIignant change is reIatively uncommon, estimated at about 0.5 per cent in chronic osteomyelitis [r], and no cases of squamous ceI1 carcinoma of piIonida1 origin were reported [2/ in 86,333 admissions among members of the armed services during WorId War II. GiIIis and Lee [4] stress the fact that chronicalIy irritated tissues have Iong been recognized as fertiIe soil for the deveIopment of squamous ceI1 carcinoma, and also that this carcinoma has the potentia1 of becoming virulent, with the abiIity to infiItrate extensively and to become wideIy disseminated. A carcinoma arising under these conditions should be treated as urgently and radicaIIy as any other form of malignant degeneration. In 1828 Jean NicoIas Marjolin [8,9] pubIished a paper caIIing the attention of the

REVIEW OF LITERATURE The first case of maIignant degeneration of a piIonida1 sinus was reported by Wolff [20] in 1900. This occurred in a twenty-one year old white woman in whom a sinus developed foIIowing the excision of a dermoid cyst from the sacrococcygea1 region. Periodically, the sinus drainage wouId spontaneously cease, foIlowed shortIy by the formation of an abscess requiring incision and drainage. Two persistent drainage sites subsequentIy developed, for which an eIective excision was performed. Examination of the specimen showed a squamous cell epitheIioma present. In 1937 Singleton [ry] reported the second case as part of a discussion of a paper presented by Gage 131. Although the complete case is not in the Iiterature, reference is made to a fifty-one year old man who died of a carcinoma seconclary to a pilonidal sinus. The third case was reported by Shubert [r4] in 1939, in which an ulcerated, papiIIomatous Iesion of the sacrococcygeal region occurred in a forty-six year old white man. Excision showed the Iesion to be a dermoid cyst with a Iarge squamous ceI1 epithelioma arising in the wall of the cyst and eroding through to the skin surface. GoIdman and KaIow [TJ reported the fourth case in 1940. This occurred in a fifty-six year oId white woman who had had piIonidal disease characterized by intermittent episodes of sweIling and inflammation for one year. Microscopic examination of the routine 465

American Journal of Surgery,

Volume IOL, September

1961

Terry,

Gaisford

and Hanna which necessitated forma1 incision and drainand age. Four years Iater, a mass appeared was grossly neoplastic, and a biopsy was reported as squamous ceI1 carcinoma. The mass measured 2 by 2.3 inches and was surrounded by a 1.5 inch erythematous margin. Wide IocaI excision to remove this mass and severa adjacent draining sinuses was performed, exposing the sacra1 and gIutea1 fascia, and the wound was packed open. Five weeks postoperatively the wound was covered with split-skin grafts. HistoIogicaIIy the specimen was observed to be a squamous ceI1 carcinoma with IocaI invasion of the adipose tissue and infiltration of the Iymphatics of the surrounding skin.

specimen discIosed a basal ceII epitheIioma arising in the cyst waI1 and adjacent sinus tract. In 1941 TendIer [r6] reported on a fifty-five year oId white man as the fifth case of maIignant degeneration in a piIonida1 disease area. This patient had had a draining Iesion of the sacrococcygea1 region for twenty years, which had enlarged and spread to adjacent tissues one year prior to consuItation. At the time of the patient’s admission to the hospita1, the Iesion was stiI1 confined to the sacrococcygea1 region and was an irreguIar, cauIifIower-Iike, grayish pink, uIcerated mass with roIIed edges, measuring 24 by 30 cm. A biopsy was reported as showing a grade I adenocarcinoma of sweat gIand origin. The patient was treated with radiation, and the lesion progressed, even during the period of treatment. Vara-Lopez [IS] reported a sixth case in 1947, in a sixty-six year old woman with a thirty-nine year history of intermittent episodes of acute inflammation, abscess formation and subsequent drainage from the sacrococcygeal region. Five years prior to seeking surgical aid, she noted the appearance of a mass that bIed easiIy when traumatized. Microscopic section of the Iesion showed an epithelioma arising in the preexisting piIonida1 sinus tract. Ha11 and Lee [a reported on a forty-two year oId white man who had had recurrent sweIIing and pain in the sacrococcygea1 region for twenty years, treated by four previous incisions and drainages, but with no attempt at forma1 excision. A brown, indurated area was noted, containing muItipIe sinus tracts, productive of a purulent discharge, as we11 as a 2 cm. necrotic area with raised, roIIed edges. Only on the fourth biopsy of the ulcerated area was a diagnosis of we11 differentiated squamous ceI1 carcinoma made. The Iesion was removed by wide IocaI resection, incIuding the sacra1 fascia and portions of the gIutea1 muscIes, folIowing which the wound was covered with postoperativeIy spIit-skin grafts. Six months the graft was excised and primary skin closure performed. Five years postoperativeIy, the patient was stiI1 free of disease. The most recentIy reported case [ 191 occurred in a forty-three year oId white man who had had a draining mass in the sacrococcygea1 region for twenty-one years. There had been two episodes of abscess formation with spontaneous drainage, folIowed by a third abscess

CASE

REPORT

This fifty-three year oId white male barber was seen with a twenty-six year history of sacrococcygea1 disease. InitiaIIy repeated episodes of abscess formation and subsequent spontaneous drainage were reported. After a two year period a wide excision of the area was performed, and the surgica1 defect was packed open postoperatively. The operative site heaIed by secondary intention and remained cIosed for ten years, when a small, painIess, draining sinus reappeared. This sinus tract persisted with slight drainage until the area was re-excised three years later and the wound again aIIowed to heal by secondary intention. The area again remained intact for ten years, when another persistent sinus tract appeared which produced puruIent materia1. Six months Iater an uIcerated, roIIed-edged, friabIe Iesion deveIoped at the mouth of the sinus tract (Fig. I). A biopsy of the uIcer base was reported as squamous ceI1 carcinoma (Fig. 2), and the patient was admitted to the hospital. PhysicaI examination at the time of admission showed the patient to be an alert, cooperative, short, we11 nourished white man, with a puIse of 76, bIood pressure I&go mm. Hg, and respirations 15 per minute. Over the sacrococcygeal region, an offensive, deepIy uIcerated, friabIe, sIightIy tender lesion with a necrotic base was present on the skin surface, and measured 9.5 cm. in its greatest diameter. Eight cm. Iateral to the piIonida1 uIceration was an erythematous, sIightIy tender, intracutaneous, parenchymatous, ill defined mass that was cIinicaIIy beIieved to be a metastasis. No axillary or inguina1 Iymphadenopathy was noted at this time. Recta1 examination showed a smooth, bilobed prostate and a norma anterior sacra1 contour. Because of the extremeIy poor hygiene associated with this Iesion, the patient was pIaced on a routine of twice daiIy sitz baths in detergent soIution.

466

PiIonidaI

Sinus Carcinoma

Flc. I. Squamous cell carcinoma arising as a recurrent pilonidal sinus tract. A cutaneous metastasis is present in the skin of the right glutcal region.

During this interval the following preoperative data were obtained: white blood cells, 14,600 per cu. mm., with a slight shift to the Ieft; hemoglobin, 12.8 gm. per cent; total protein, 7.4 gm. per cent, with a normal albumin/globulin ratio; non-protein nitrogen, 30 mg. per cent; fasting blood sugar, 111 mg. per cent; alkaline phosphatase, 5 Bodansky units per IOO cc.; serum cholesterol 208 mg. per cent with 123 per cent esters. A chest roentgenogram was interpreted as showing no abnormalities. IHowever, an electrocardiogram show-cd evidence of old myocardial damage on an arteriosclerotic basis. During the period of preoperative evaluation, a right inguinal mass developed. Excision biopsy of the right inguinal mass and very wide local excision of the sacrococcygea1 Iesion were performed simultaneously. The piIonida1 surgical defect was covered immediately by thin split-skin grafts. The right inguinal lymph node biopsy contained squamous cell carcinoma and the patient subsequently underwent a biIatera1 radica1 groin dissection with metastatic squamous ceil carcinoma present in both the right and left sides. (Figs. 3 and 4.) The patient remained cIinicalIy free of disease for only eight months, after which he first complained of back pain and progressive sweIIing of the right lower extremity. Para-aortic metastases were subsequently demonstrated by intravenous pyelograms and the patient died sixteen months foIlowing the original operative procedure.

FIG. 2. Biopsy of the sacrococcygea1 uIccration shc~wctf a squamous ceII carcinoma Invading the adjacent tissues.

of the patient to determine the extent of the disease, with particuIar attention to examination of the adjacent tissue for local extension and of the inguinal regions for IF-mph node metastases. Roentgenograms of the pelvis, IumbosacraI spine and chest are indicated and a barium enema and intravenous pyelogram should be considered in the presence of inguinal metastases. A rectal examination and the usual preoperative studies for the evaIuation ol’ any patient undergoing a major surgical procedure are indicated. Because of the poor local hygiene accompanying chronic draining wounds, a bath in detergent soap solution twice daily for several days preoperativeIy is an effective means of securing local cIeanliness. This procedure is especiaIIy important if the operative defect is to be covered with split-skin grafts. The bo\vel is prepared by cleansing enemas the evening and morning preceding operation and a Foley catheter may be inserted to alIow the patient

COMMENTS

There have been too few we11 documented cases of squamous cell carcinoma arising in an area of pilonidal disease to propose any forma1 plan of management. A reasonable program must incIude a thorough preoperative study 467

Terry,

Gaisford

and Hanna

FIG. 3. Sections of the inguinal Iymph nodes contained metastatic squamous celI carcinoma characterized by sheets of pIeomorphic, bizarre ceIIs, with numerous mitotic figures. FIG. 4. High power (magnification X 450) view of the metastatic ing mitotic frequency, pleomorphism and Iack of differentiation.

to Iay in a prone position for a minimum of five days postoperativeIy. SurgicaI excision of the Iesion shouId incIude at Ieast a 3 cm. margin of adjacent grossIy normaI skin as we11 as the fascia overIying the gluteus maximus muscuIature and especiaIIy the dense Iigamentous postsacra1 fascia. If the tumor grossly penetrates this deep fascia1 Iayer, wide IocaI excision of the entire coccyx and the outer tabIe of the sacrum shouId be accompIished. Thin (0.008 to 0.010 inch) spIit-skin grafts serve we11 to cover the operative defect,

tumor show-

and they may either be applied primariIy or the wound may be packed for severa days to aIIow granuIation tissue to form. Then the skin grafts may be applied to effect wound cIosure. If it is necessary to perform groin dissection in the presence of palpabIe Iymphadenopathy, it seems reasonable to deIay such a dissection for ten to fifteen days to aIIow the recentIy appIied postsacra1 spIit-skin grafts to become we11 situated. PostoperativeIy the patient should be kept in a prone position for five days and given 468

PiIonidaI

Sinus Carcinoma

a low residue high-protein high-caloric diet supplemented with vitamin C. Use of paregoric during this period wiI1 help maintain a cIean dressing. The initial dressing is changed on the fifth postoperative day and the patient may be ambulated after this time on a progressive basis. Although carcinomatous degeneration in an area of piIonida1 disease is a very unusual compIication, it shouId certainIy be a consideration when deaIing with a patient having a Iong history of intermittent abscess formation and drainage in the sacrococcygea1 region. SUMMARY I. The previously reported seven cases of malignant change in an area of piIonida1 disease are reviewed and an additiona case presented. 2. Wide excision of the primary Iesion with primary operative defect coverage with a thin split-skin graft and groin dissection for paIpabIe lymphadenopathy are presented as the current management of squamous ceI1 carcinoma arising in an area of piIonida1 disease. REFERENCES

BENEDICT, E. B. Carcinoma in osteomyelitis. Surg. Cynec. @ Oh.%, 53: I, 1931. 2. DENNING, J. S., FREDERICK, J. F., GOLD, D. and POTH, E. J. PiIonidal disease; review of Iiterature and a method of closure. Am. Surgeon, 20: 1250, I.

1954. 3. GAGE, M. PiIonidaI sinus: an explanation of its embryologic development. Arch. Swg., 31: 175, 1935. 4. GILLIS, L. and LEE, S. Cancer as a sequel to war wounds. J. Bone 6~ Joint Surg., 33-B: 167, 1951. 5. GOLIMAN, H. and KALOW, I. PilonidaI cyst com-

plicated by basa1 ceI1 epithefioma. Bull. Hoq). Joint Dis., I: 89, 1940. 6. HALL, A. and LEE, G. J. Squamous ~11 carcinoma complicating a pilonidal sinus. Cancer, 9: 760, 1956. 7. I~OPPING, R. A. PiIonidaI sinus: review of the literature with comments on the etiology, differential diagnosis and treatment of the disease. Am. J. Surg., 88: 780, 1954. 8. MARJOLIN, J. N. Dictionnaire de Mkdicin, vol. 21, P. 31. g. l\fAR.IOLIN, J. N. UI&re. In: Dictionnaire de Mbdicin, 2nd ed., vo1. 30, p. IO. Scars, p. 22. IO. MCANLLY, A. K. and DOCKERTY, hl. B. Carcinoma deveIoping in chronic draining cutaneous sinuses and fistulae. Surfi. Gynec. C+ Ohst., 88: 87. 1949. I I. MOISE.T. S. Staphylococcus meningitis secondary to a congenital sacra1 sinus. Surfi. Gynec. t” Obsf., 42: 394, 1926. 12. NIEBAUER, J. J. DeveIopment of squamous cc[I carcinoma in the sinus tracts of chronic osteomyelitis. J. Bone F Joint Surg., 28: 280, 1946. 13. RIPLEY, W. and THOMPSON, D. C. PiIonidaI sinus as route of infection in case of staphylococcus meningitis. Am. J. Dis. Cbild., 36: 785, 1928. 14. SHUBEKT, H. Karzinomatose entartung von Stcissdermoiden. Zentralbl. Cbir., 66: 2098, 1939. 15. SIWLETON, A, Q., SK. Tr. Soutb. SUT,~.A., 5 I : 71, ‘937.

16. TENDLER, M. J. PiIonidaI sinus: a review of its literature and a report of 87 cases. Soutb. M. J., 34: I 156, disc. 1166, 1941. 17. TREVES, N. and PAW, G. T. The dcvclopment of cancer in burn scars: an analysis and report of 34 cases. Surg. Gynec. ?Y Obst.,51: 749. 1930. 18. VARA-LOPEZ, R. DOS cases de cpithcliomas de la region sacrocoxigea consectivos a un quiste pilonidal y a un teratoma presncro. Ret,. clin. espaFi., 24: 367, 1947. 19. WEIUSTEIN, M., ROBERTS, M. and REYNOLDS, B. Carcinoma complicating piIonida1 sinus. New York J. Med., 57: 2089, 1957. 20. WOLFI., H. Carcinom auf dem Boden des Dermoids. Arch. klin. Cbir., 62: 731, Igoo.

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