APOCRINE SWEAT GLAND CARCINOMA OF THE VULVA* JOHN R. McDONALD, M.D. Section on Surgical Pathology, The Mayo Clinic, Rochester, Minnesota

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1 APOCRINE SWEAT GLAND CARCINOMA OF THE VULVA* JOHN R. McDONALD, M.D. Section on Surgical Pathology, The Mayo Clinic, Rochester, Minnesota The wide variety of neoplasms, both benign and malignant, originates in the vulva largely because of the fact that normally the vulva has many différent types of tissue incorporated in it. In addition to skin and its usual derivatives one encounters involuntary muscle, apocrine sweat glands and the mucous glands which form the Bartholin gland. Accessory breast tissue and remnants of the Wolffian duct hâve been described. The most common epithelial neoplasms are those which originate from squamous epithelium, namely, squamous cell carcinoma and malignant melanoma. Less common in the vulva are the glandular types of neoplasms. In 1904, Ludwig Pick described two cases in which adenomas had been excised from the vulva. Because of the demonstrable connection with sweat glands and the two layers of cells lining the acini, he expressed the opinion that thèse tumors took their origin from rudimentary sweat glands. He was unable to demonstrate any malignant changes in them. For thèse tumors he coined the term "hidradenoma." Hoeck, Blau and Woringer each described one case of hidradenoma of the vulva. Blau noted that thèse tumors were suggestive of malignancy from a histologie standpoint. Burg, in 1930, reviewed the literature on this subject. He was able to find fifteen cases which had been reported up to that time and he added one case of his own. Ail were considered as benign in nature, except for two, one of which was reported by Ruge, in 1905, and the other by Schiffmann, in Eichenberg, in 1934, described twelve papillary adeno- * Read before the nineteenth annual meeting of the American Society of Clinical Pathologists, New York, June 8,

2 APOCRINE SWEAT GLAND CARCINOMA OF VULVA 891 mas of the vulva. Around thèse adenomas he found dilated and cystic sweat glands, from which he considered they originated. In addition to thèse cases which Eichenberg considered as benign, there was one case of this type in which he believed that there was definite évidence of malignancy. He noted the présence of peculiar glands lined by clear epithelial cells. Weiner described a case of Paget's disease of the vulva with metastasis in which he suggested the origin from apocrine sweat glands. However, he was unable to trace the neoplastic cells from the sweat glands. After a study of the photomicrographs it would appear that this is a case of squamous cell carcinoma. Folsome (1940) believed that thèse tumors were essentially benign in character although 27 per cent of them were distinctly invasive in character. He was of the opinion that local excision was sufficient for a cure in ail cases. A thorough pathologie study was made of ail adenocarcinomas of the vulva removed at the Mayo Clinic from 1906 to 1939 inclusive. Several distinct and différent types of adenocarcinoma were found. The largest group corresponded to what has been described in the German literature as hidradenoma of the vulva. There were thirty separate neoplasms in this group. Thèse h ad been removed from twenty-nine patients. CLINICAL DATA Many of the neoplasms in this group were an incidental finding. Several were excised during the course of a perineal repair or some other surgical procédure. Several were not recognized on physical examination. One of the most common clinical diagnoses that had been made was that of sebaceous cyst because many of thèse neoplasms presented a cystic appearance. In every instance except one the tumor was confined to the labium majus. In this case the lésion apparently originated in the labium, but extended to the vaginal wall and involved the perineal body. The majority of the neoplasms were less than 1 cm. in diameter. Only one could be considered large and it measured 5 by 3 by 1.5 cm. In one case there were two separate neoplasms; the remainder were single.

3 892 JOHN E. MCDONALD None of the patients in this séries was less than thirty-two years of âge. The oldest patient was sixty-seven years of âge. Nineteen of the twenty-nine patients were less than fifty years of âge whereas eleven of thèse were less than forty years. Excision of the tumor was sufficient to effect a cure in ail cases. Usually a portion of the surrounding nonneoplastic tissue was taken with the neoplasm. None of the patients showed any évidence of récurrence or metastasis. In one patient a wide excision with the cautery was done including one labium, a FIG. 1. PAPILLABY ADENOCARCINOMA OF THE VULVA The cystic nature is shown. The neoplasm is attached to the capsule at one point. Hematoxylin and eosin X5. portion of the vagina and perineal body. The neoplasm was a large papillary adenocarcinoma, grade 1. The patient died sixteen years later from an unrelated condition and at no time was there any évidence of récurrence. Radium or roentgen therapy was not used in any of the cases. GROSS APPEARANCE The majority of thèse neoplasms presented a cystic appearance on gross examination. Within this cyst the tumor was apparent (fig. 1). However, in approximately 40 per cent of the cases the tumor was solid.

4 APOCRINE SWEAT GLAND CARCINOMA OF VTJLVA 893 HISTOLOGIC APPEARANCE Ail of the neoplasms in this séries represent papillary adenocarcinomas. Mitotic figures are scarce, but can be demonstrated. The nuclei are large and hyperchromatic such as those found in malignant cells. Well formed acini are présent in ail tumors; FIG. 2. PAPILLARY ADENOCARCINOMA OF THE VULVA This neoplasm is slightly more malignant than that in figure 4. Hematoxylin and eosin X140. thèse are indicative of a slowly growing neoplasm (fig. 2). Two are grade 2 according to Broders' classification, whereas the remaining twenty-eight are grade 1. In those spécimens in which there was cyst formation the tumor had a localized attachment at one or more points to the wall, through which the blood supply to the tumor was carried. In this way there is a strik-

5 894 JOHN K. MCDONALD ing similarity between thèse tumors and intraductal neoplasms of the breast. Invasion of the capsule of the tumor by neoplastic cells could be demonstrated frequently. The most interesting part of this study was the fact that in twelve of the thirty neoplasms it was possible to demonstrate cells in which the cytoplasm was clear and strikingly eosinophilic. The acini that show papillary infoldings and lined by cells with clear cytoplasm represent apocrine glands. The transition of thèse cells to carcinomatous cells having dark cystoplasm and larger nuclei is shown. Hematoxylin and eosin X100. In thèse régions papillary projections into the lumen frequently could be exhibited. In ail twelve cases this peculiar epithelium was in the tumor and could be directly traced changing into adenocarcinoma (figs. 3 and 4). Thèse glands correspond in practically every détail with the description of apocrine sweat glands. In human beings, apocrine sweat glands are found most commonly in the axilla, labium majus and perianal région

6 APOCRINE SWEAT GLAND CARCINOMA OF VTTLVA 895 (fig. 5). Thèse glands are consistently présent in certain lower animais and their function apparently is concerned with the sex instinct. In two additional cases apocrine glands could be demonstrated adjacent to the carcinoma but no transition could be traced between the glands and neoplasm. The neoplasms in which apocrine glands could not be traced into carcinomas presented a similar histologie appearance to the carcinomas in FIG. 4. PAPILLARY ADBNOCARCINOMA OF THE VULVA The transition is shown between the clear cells representing apocrine sweat glands and the carcinomatous cells. Hematoxylin and eosin Xl40. the aforementioned cases except that no apocrine glands could be found. Ail thèse neoplasms were situated immediately subjacent to the epidermis of the labium. In several instances, there was ulcération of the epithelium. Those which were small enough to be localized were situated in the corium. Apocrine sweat glands are found in the deep layer of the corium which further substantiates the hypothesis that thèse neoplasms originate from apocrine glands.

7 896 JOHN E. McDONALD In no instance could any connection be demonstrated between the mucous acini which form the Bartholin glands and the adenocarcinomas in this group. The carcinoma was situated on the labium majus, anterior to the usual site of the Bartholin gland in ail cases in which the site could be définitely determined. FIG. 5. APOCBINE SWEAT GLANDS OF THE LABIUM MAJUS The larger apocrine glands are contrasted with the smaller ecorine, or ordinary, sweat glands. Hematoxylin and eosin X195. In one case there were two separate neoplasms. papillary adenocarcinoma grade 1. Each was a COMMENT As far as I hâve been able to ascertain, this is the first description of a neoplasm arising from apocrine sweat glands. Although several German authors believed that thèse tumors in the vulva took their origin from sweat glands, thèse authors did not specifically dérive them from apocrine in contradistinction to the eccrine or ordinary sweat glands. The problem may be broached as to whether thèse neoplasms

8 APOCRINE SWEAT GLAND CAKCINOMA OF VULVA 897 are adenomas or are adenocarcinomas of low grade. Because of their histologie character and infiltrative nature it would seem better to refer to them as adenocarcinomas of a low grade of malignancy rather than as adenomas. In this group there is no évidence that any metastasis occurred. However, if a sufficient number were studied I feel certain that metastasis could be demonstrated in a small proportion. An analogy can be drawn between thèse lésions and certain adenomatous polyps of the colon which many pathologists consider to be carcinoma in the light of our présent knowledge. It is known that thèse neoplasms will infiltrate and that metastasis will occur if left in the body sufficiently long. Because of the slow growth and slow tendency toward metastasis, complète excision of the vulvar tumor preferably including a portion of surrounding tissue is sufficient to effect a cure. SUMMARY Thirty adenocarcinomas of the vulva of a low grade of malignancy hâve been studied and described. Neoplasms of this type hâve been described in the literature as hidradenoma. A sizeable proportion of thèse carcinomas could be shown to arise from apocrine sweat glands. REFERENCES (1) BLATJ, ALBERT: Hidradenoma Vulva. Zentralbl. f. Gynàk., vulvae. Ztschr. f. Geburtsh. 60: (Oct.) u. Gynâk., 93: , (7) PICK, LUDWIG: Uber Hidrade- (2) BBODEBS, A. C. Practical points noma und Adenoma hidradenon the microscopic grading of oides. Virchows Arch. f. path. carcinoma. New York State J. Anat., 176: (Feb. 3) Med., 32: (June 1) (3) BURG, ETÉ: Ûber einen Fall von (8) RUGB, H: Quoted by Burg, E. Adenoma hydradenoides vulvae T. C. 3 Zentralbl. f. Gynak., 64: 395- (9) SCHIFFMANN, J. : Quoted by Burg, 399 (Feb. 15) 193a,, E. T. C.» (4) EICHENBEBG, H. E.: Hidrade- ( 10 ) WEINER, H. A.: Paget's disease of noma vulvae. Ztschr. f. Ge- ^ skin and its relation to burtsh. u. Gynàk. 109: 358- carcinoma of the apoenne sweat Q^Q ia*a glands. Am. J. Cancer, 31:,K\ r? ' A v v t., (Nov.) (5) FOLSOME, C. E.: Tumors of the (11) WORINGEB, F.: Adénomes sudvulva. J. A. M. A., 114: oripares en petites tumeurs de (Apr 20) l a grande lèvre. Bull. Soc. (6) HOECK, WEBNEB: Uber einen Fall franc, de dermat. et syph., von tubulàrem Hidradenom der 46: (Feb.) 1938.