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Türk Plast Cer Derg (1993) Cilt: 1, Sayı:2

GIANT PİL AR TUMOR OF THE SCALP

Seyhan ÇENETO(iLU, Serol İNCEOGLU, Cemalettin ÇELEBİ, Namık K. BARAN, Ömür ATAOGLU, Tülin OYGÜR,

* Frıuıı Deparmeııt of Plastic and Reconstructîve Surgery Gazi t iniverdi y Schooî of M e di dne, Ankara, TlIRKEY.

+ From Department of Pathology,

Gazi University Sclıool of Metli dne, Ankara, TURKEY.

SU M M A R Y

G IA N T PILAR TUM OR OF THE SCALP

We preseni a case o f a giant pilar turno r o f the sc.alp w kıçlı has the largest tumor volüme ever reported in English litera­

türe, The growth. pattern and possihle. role o f heredity in such lesions ar e discussed. The importance o f adequate surgical excision and diligent histöpathological examination is eınpha- siz.ed,

Key Word : Pilar tumor.

Pil ar tumors are beııign neoplasms derived from the ou ter root sheath of the hair follicle (1). They are be- lieved to have an association with pilar cysts whielı are sometimes observed to precede or accompaııy these tu- 1 mors (2). Although they are known to lack the potential 5 for malignant tmnsfoıınation, evidence of regional rneta- 1 stasis has been shown in several pilar tumor eases in the J literatüre (3, 4, 5). Both because of the locally aggres- i şive growth characteristics and the rare occuırence of re-

* gional metastasis, particularly İn neglected cases, iınpor- 1 tance of adequate surgical exeision of these tumors has

been emphasized previously (6).

In this report, we preseni the ease of an elderly

^ woman with a large, infeeted and ulcerated scalp rnass 2 showıı by pathological examination to be a pikır tumor,

§ Personal and family history of the patieııt were iııterest- T ing in revealing the role of both budding and multiple

^ cyst formation iıı tumor growth and pointing out the S possibility of inheritance in etiology. This giaııt pilar tu- li mor (with the largest. volüme ever reported in English li-

O ZET

SA Ç IJ D E R ÎN İN D E V P İIA R TÜMÖR Ü

İngilizce literatürde bugüne kadar bildirilen pilar tümörlerin hepsinden daha büyük hacimli dev bir sc.alp pilar tümörü su­

nulmaktadır. Tümörün büyüme özelliği ve kalıtım faktörünün böyle lejyonlardaki muhtemel rolü üzerinde durulmuştur. Te­

davide yeterli cerrahi eksizyonun ve titiz histopatolojik incele­

menin önemi vurgulanmıştır.

Anahtar Kelime : Pilar tümör.

teralure) was totally excised mıd the scalp defect so created was successfully covered with skin grafts. Dur- İııg the three-year follow- up period, no evidence of ei- ther Iocal recurrence or regional metastasis was detect- ed.

CASE REPORT

A 78-year old woman who has been suffering from a large scalp mass for 4 years was referred to our depar- ment with a diagnosis of squamous aacmoma of the scalp. S he had First noted a small scalp nodule twenty ye ar s ago and later 4 to 5 similar nodules which gradual- ly iııcreased in size and coalesced to from a single mass.

Surgical excision had been attempted in past but failed to cure Üıe lesion. She noted that the surface of the tumor beaune ulcerated and purulent; foul-smelling discharge and oeeasional bleeding occured in the last few years. Three of her children, one son and two dauglıters, both middle-aged, had similar lesions on their scalps. Neitlıer personal nor family history was 1 AO

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GIANT PILAR TUMOR OF THE SCALP

othenvise noteworthy. The scalp mass. located in the pa- rietal and occipital regions, had a surface area of 25x20 cm. Its height from the scalp surface varied between 6 to 10 cm. There were foci of ulceration and purulent dis- charge över its surface (Figüre 1).

The cultures from the discharge revealed growth of Staphylococcus Aereus and Pseudomonas Aeroginosa.

Her physical examination was normal except for pale conjunctivas. Total blood count and biochemistry showed no abnormality other than mild anemia and hy- poalbuminemia. Cranıal computerized tomography showed almost uniform invasion of the periosteum and cortical thickening under tumor base but no sign of in- tracranial spreading (Figures 2 and 3).

Tumor was excised circumferentially with a 1 cm.

margiıı of normal scalp tissue under general anesthesia.

Both the invaded and intact appering porüons of the per­

iosteum underlying the tumor were included with the excised mass (Figüre 4). Burr-holes were formed using a power-drill through the outer table of the skull and ca- pillary bleeding from below w as observed.

Three months postoperatively, the whole defect was fîlled with healthy granulation tissue. Split-thickness skin grafts taken from anterior thigh applied över this tissue provided satisfactory coverage of the defect.

Periodical examinations failed to show any evi- dence of local recurrence or metastasis in the past three years (Figüre 5). The son of the patient was available for excision and pathological examination of his scalp le- sion upon his request.

Figüre 1: Lateraî wiew o f the pilar tumor located in the parie- tal and occipital regions.

PATHOLOGİCAL f i n d i n g s

Multiple hematoxyline and eosine sections prepared from the tumoral mass showed lobules of squamous epi- thelial cells and eosinophilic amorphous keratin in the çenter of these lobules (Figüre 6). Some of the lobules appeared as large cysts filled almost entirely with kera- tin. The change of the epithelium to keratinous material was abrupt and there was focal calcifıcation in the kerat­

inous material. Epithelial cells showed a slight to mod- erate degree of atypia and pleomorphism but the mitotic figures were rare; being 1 per 10 high power field İn the most active-appearing areas. There was no sign of atypi- cal mitotic figures. Periosteum and bone spicules includ­

ed with the specimen did not show any sign of tumoral invasion. The lesion excised from the scalp of the pa- tieııt's son showed typical feature of a pilar cyst.

Figüre 2: CT o f the craniurn showing the hııge tumor with no intracranial spreading.

Figüre 3: Another CT section from the same patient showing the ulcerations on tumor surface, periosteal irregularities, and cortical thickening in the skull.

143

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']'ürk PlasL Cer Derg {1993) Cilt: 1. Sayı:2

Figüre 4: Photo graph ofthe e.scisetl tumor. Nötr the e.stcmive injlammal'um a m! ni­

ce m ti on on its .s m fa r e.

r Figüre 5; Two years postoperatively, no vviüenrv o f tumor rt 2 cıırrence or skin raft hreakdtnvn ımv ohsrn'cd.

§

P h

§ II

1/1/1

(4)

GIANT PILAR TUMOR OF THE SCALP DISCUSSION

Pilar tumor, which is also called giaııt lıak matrix tumor (7), pilomatrixoına, proliferating triehilemmal cyst or triehilemmal pilar tumor (8), is believed to take origin from the outer root sheath of the hak follicle (1).

The lesion is typically seen iıı elderly womeıı and is mostly located in the sealp, usually presenting as a soli- îary cyst or nodule, The size of the cyst inereases with time and either budding of the original cyst or occur- ence of other eysts leads to a mass comprised of milti- ple eysts (9). This course has a variable speed and its duratioıı is from a few to över t.wenty years (8). In this course of events, spontane ou s healing or ımırsupializa- tion and later disappearence of the cyst may be ob- served. Less frecjuently, the cyst undergoes proliferation and a pseudoepiteliomathous mass is produeed (9). This form of the lesion is likely to be conlused witlı squa- mous celi carcinoma and, indeed, pilar tumors showing malignant bahaviour have been reported by s ever al out- hors (3, 4, 5). This fact points out the signifıcance of a diligent pathological examination for suclı lesioııs. Hos- okawa et al. (10) and Pinkus (11) have amphasized the impor timce of histochemical metlıods used to charaç ter­

iz e triehilemmal keıatiıı in lesioııs such as epidermoid eysts and pilar eysts versus lıair matrix. Cotton et id.

Showed the difterence between the properties of pilar and epidermal eysts using two aııti-keratiıı aıuibodies derived from two differeııt keratin ffactions (12). Vari- ous mechtmisıns of inheritaııee have been reported for sebaceous eysts and a Mendeliaıı dominant keait has been suggested for a pilar hımor of the sealp that devel- oped in a sebaceous cyst (13). There is no clear doeu- mentation of inheritaııee of de novo pilar tumors. İt is iııteresting to note that the son of the patieııt in our ease had a nodular lesion on his sealp whiclı was excised and eonfirmed by m i eros cop y to be a pilin cyst.

The pilar tumor pıeseııted in this ease has the larg- est volüme ever reported in English literatüre. It is eon- eluded from the patieııt's histoıy tlıat both budding and separete eystie growth played a role İıı tumor growth, as was suggested by others (2, 14). Malignant behaviour of the tumor could not be deteeted by mieroseopy. Keep- ing İn mind that the lesion recurred twiee despite two reseetions made in the past ten years and reaehed a lıuge mass yet retained its benigıı ehiiracter, we are eouviııeed that proliferation does not ııecessarily lead to midign transformatioıı in pilai' tumors. The long-lastiııg iıı nam­

ın ati on with purulent dischaıge from the lesion, as ob- served in this patient, may be a stimulus tor this proliie- ration (1).

Surgical excision witlı im adequate margin both cir- cumferentially and in deeper layers is the unique keat- meııl for pilin tumors. We beli eve that the two previous excisions made previously in this ease would have been curative had this rule been adhered to. Even in cases where the lesion has invaded the periosteum, the defect created by such extensive reseetion can be satisfactorily covered with skin grafts.

Seyhan ÇENETOĞLU Berdan sokak, 4-3, Kurtuluş, 06600, Ankara, Turkey.

REFERENCES

1. BaptisLa. A.P., Sİlva, L.G.E., and B ron, M C. Proliferating triehilem mal cyst. ,T. Cutaneons Pathology 10:178, 1983.

2. Heııdricks, D.L., Liang, M.D., B orochovitz, D., and M ill­

er, T.A. ease of m ultiple pilar tum ors and pilar eysts in- vulvimi the sealp and buek. Plast. Reconstr. Surg. 87:763,.

1991. "

3. Salda, 3'., Oobara, K., Hori, Y. and Tsuehiya, S. Develop- jııeut of a m alignant proliferating triehilem m al cyst in a patient with m ultiple triehilem m al eysts. D ennatologica

166: 203, 1983.

4. Batm an, P.A. and Evans, H.J.R. M etastasising pilar tu­

rnom- of sealp. J. Clİn. Pathol. 39:757, 1986.

5. M ehregan, A.H. and Lee K.C. M alignant proliferating tri- ehüem m al tuınors-report of three cases. J. Dermatol.

S urg.O ncnl. 13:1339, 1987.

6. M organ, R.F., Dellon, A.L., and H oopes, ,T.E. Pilar tu­

mors. Plast. Reeonstr. Surg. 63:520, 1979.

7. Dalısku. M. Giaııt hair matrix tumor. Cancer 28:701, 1971.

8. Brovvnsteiıı, M.H. and Arluk, D..T. Proliferating triehilem- mal cyst: A sLimulaııt of stjuamous celi carcinoma. Canc­

er 48:1207, 1981.

9. Leppard, B.J., and Sanderson, K.V. The natural history of triehilem mal eysts. Br. .1. Dermatol. 94:379, 1976.

10. Hosokawa, M., Rkugo, S., Aiba, S., Igarashi, M., and Ta- gıfmi, H. B iochem ical characterİstics of Keratins from proliferating triehilem m al cyst. Arch. Dermatol, Res.

276:250, 1984.

11. Pinkus, H. "Sebaceous Cysts" are triehilem mal eysts.

Arch. Derm. 99:544, 1969.

12. Cotton, D.W .K., Kİrkham, N., and Young, B..T..T. Immu- noperoxİdase anti-keratiıı staining o f epidermal and pilar eysts. Br. .1. Dermatol. 11:63, 1984.

13. SLruııc, M.F., Bemıett, M.F. Bennett, M.H., and Melmed, E.P. Pilar tum our of the sealp developing in hereditary se- baceons eysts. Br. .T. Plast. Surg. 24:82, 1971.

14. Reed, R. .1, and Lamar, L.M. Invasive h ak matrix tumors of the sealp, Arch. D em i. 94:310, 1966.

Referanslar

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