Extraovarian Endometrioid

Transcription

1 Extraovarian Endometrioid Carcinoma Review of the Literature and Report of Two Cases with Unusual Features MICHAEL P. HYMAN, M.D., FCAP Hyman, Michael P.: Extraovarian endometrioid carcinoma. Review of the literature and report of two cases with unusual features. Am J Clin Pathol 68: , Two cases of extraovarian endometrioid carcinoma are reported. The patients had had total hysterectomies and bilateral salpingooophorectomies 17 and 7 years before discovery of the endometrioid tumors. The ovaries in both cases contained endometriosis with adenomatous or atypical changes. Endometriosis should be routinely examined for hyperplastic or atypical features, as these may be significant harbingers of ovarian or extraovarian endometrioid carcinoma. Progestogen therapy for endometrioid neoplasm is theoretically promising, but has yet to be systematically investigated. (Key words: Extraovarian endometrioid carcinoma; Endometriosis; Endometrioid carcinoma.) IN 1921, Sampson wrote a lengthy treatise on endometriosis in the ovary. 19 Four years later, he definitively described endometrioid carcinoma of the ovary. 20 In 1961, the Cancer Committee of the International Federation of Gynecology and Obstetrics broadly defined endometrioid carcinoma on a histologic basis, and subsequent studies have shown that 18-24% of ovarian carcinomas are endometrioid It is now accepted that although endometrioid carcinoma has a special histologic and prognostic relationship to the endometrium, only 20-25% of patients have coexistent endometriosis and only 10-15% have endometrial adenocarcinoma in the uterus at the time of diagnosis. 3,5,6 It is also well-recognized that many endometrioid carcinomas have mucinous, clear cell, 12,21 acanthomatous (as many as 50%), 6,23,25 and serous components, thus further strenghthening the contention that at least some of the endometrioid Received July 26, 1976; received manuscript October 20, 1976; accepted for publication October 20, Address reprint requests to Dr. Hyman: Department of Pathology, Presbyterian Medical Center, 1719 East Ninettenth Ave., Denver, Colorado From the Department of Pathology, New Britain General Hospital, New Britain, Connecticut tumors arise de novo from the germinal epithelium of the ovary. 3,22 Dockerty, Pratt, and Decker, in 1954, began the contemporary discussion of extraovarian endometrioid carcinoma by reporting a tumor that had been resected from the rectovaginal septum. 7 Subsequently, additional sporadic cases were reported. 8_10,14,18 In 1966, Scully and associates 21 emphasized the rarity of this neoplasm, finding one case in the previous 15 years. In a case cited by Thompson, an encapsulated adenocanthoma was removed from the cul de sac five years after the original ovarian adenoacanthoma had been demonstrated. 23 Symptoms referable to a pelvic mass frequently lead to laparotomy and hysterectomy with diagnostic resection of the mass. However, in the case reported by Young and Gamble, a focus of endometriosis in the rectovaginal septum, discovered 11 years after hysterectomy, over the next four years evolved into endometrioid carcinoma and acanthoma. 25 The two cases of extraovarian endometrioid carcinoma reported here illustrate several unusual features. 522 Report of Two Cases Case 1: A 70-year-old white woman had had a total abdominal hysterectomy-bilateral salpingooophorectomy in The ovaries had showed endometriosis with atypia (Figs. 1 and 2). The glands were compact and hyperchromatic, and the epithelium was tall, crowded, and slightly pleomorphic. The endometrium was in the proliferative phase. The next 17 years were uneventful until urinary incontinence developed. A pelvic mass was palpable.

2 < ^ -» FIG. 1 (upper) and 2 (lower). Case I. Although banal endometriosis was found in this ovary, several areas revealed the changes shown. Glands are enlarged, arranged close to one another, and show epithelial crowding with nuclear enlargement and slight hyperchromatism. Hematoxylin and eosin. x 100.

3 ... v. v* : T / / "- '>*»»* ->wr..v -" S, '' \ %, \ ** "I * ":"' ' ', V.- FIG. 3 [upper). Case 1. The larger, friable, polypoid excrescences represented a low-grade papillary carcinoma of the endometrioid type. Anastomosing, blunt-tipped papillations line scant fibrovascular stalks and show moderate cellular pleomorphism. Hemotoxylin and eosin. xloo. FIG. 4 (lower). Case 1. Cystic, mucin-filled spaces lined by simple, low columnar epithelium. Histologically benign, this type of change corresponds to the mucinous and cystadenomatous component of endometrioid tumors previously described. Hematoxylin and eosin. x25.

4 FIG. 5 (upper). Case I. The stromal and glandular patterns are identifiable as endometriosis. Nevertheless, transition from this benign lesion to the endometrial carcinoma was not found. Instead, the endometriosis was adjacent to extensive hemorrhagic necrosis of the cyst lining. Hematoxylin and eosin. xloo. FIG. 6 (lower). Case 2. The endometriosis in this ovary varies from the usual type to that seen here. Glandular cells are hyperchromatic, enlarged, and contain angular, moderately pleomorphic nuclei. These changes are interpreted as atypical or "borderline." Hematoxylin and eosin. x 100.

5 A.J.C.P. October 1977 HYMAN 526 #5>>( i&y$;*\ ffi! *? ;* &s. * «.' ' aw FIG. 7. Case 2. Less well-differentiated than the tumor in Case 1, the large, basophilic, abortive glandular masses form confluent sheets with a few papillary formations. Hematoxylin and eosin. xloo. The intravenous pyelogram showed left hydronephrosis and virtually no renal function on the right. At laparotomy, a cystic tumor, 10 cm in diameter, was found compressing the right ureter and colon posteriorly and the bladder anteriorly. The mass was on the rectovaginal septum and seemed to peel easily off the surrounding structures. The cyst was multilocular. Some cavities were filled with yellow, clear fluid, while others contained tenacious, yellow, mucoid material. The wall, which was partly hemorrhagic, supported friable brown excrescences; these ranged in diameter from a few mm to 1.7 cm. Microscopic examination showed several types of epithelium lining the cyst wall. The larger fragments contained papillary columns of anastomosing, hyperchromatic, well-differentiated glands, propped by thin fibrovascular stalks (Fig. 3). Foci of squamous metaplasia were identified. The tumor was a lowgrade neoplasm of the endometrioid type. Adjacent to carcinoma were areas of hemorrhage and cystic nodules lined by bland, simple, mucinous epithelium (Fig. 4). Other sections showed endometriotic tissue with a compact, congested, spindled stroma support- ing simple columnar cells (Fig. 5). A thorough workup has revealed no evidence of another primary tumor. The patient received external radiotherapy and is asymptomatic. Case 2: A 50-year-old white woman had had a total abdominal hysterectomy-bilateral salpingo-oophorectomy in The left ovary had contained an endometriotic cyst. Subsequent review of those slides in 1974 revealed areas of atypia or borderline malignancy within the endometriotic epithelium (Fig. 6). In 1974 a hemorrhagic nodule of the vaginal vault was biopsied and showed endometrioid carcinoma (Fig. 7). Widespread metastases eventually developed and the patient died. Discussion In both of these cases extraovarian endometrioid carcinoma developed many years after extirpation of ovaries, which contained atypical, adenomatous, or borderline foci of endometriosis. In the first case, the extraordinarily long interval (17 years) between hysterectomy and removal of the pelvic mass emphasizes the indolent nature of endometriosis. Al- #1

6 Vol. 68 No. 4 CASE REPORTS 527 though the origin of the carcinoma in this case is uncertain, several pathogenetic sequences are possible: (1) Pelvic endometriosis may have been left behind 17 years previously, only to transform eventually into carcinoma. (2) A bit of ovary with atypical endometriosis may have been left in the patient, with subsequent malignant progression. (3) Rests of pelvic pluripotential epithelium developed de novo endometriosis and endometrioid carcinoma. The cystic and mucinous regions in Case 1 resemble a cystadenoma. Scully has hypothesized that if endometrioid carcinoma has a germinal origin in common with mucinous and serous cystadenocarcinomas of the ovary, then the former might very well have a benign counterpart to the serous and mucinous cystadenomas 21 He reported a case showing a benign papillary mucinous cystadenoma arising within an endometrioid cyst. As in many of the reported cases of extraovarian endometrioid carcinoma, it is very difficult to demonstrate histologically, not just coexistence, but transition from endometriosis to carcinoma. Much of the tumor cyst wall was hemorrhagic and scarred. In Case 2 the vaginal vault mass contained only endometrioid carcinoma; no endometriosis was identified. Endometriosis has manifested in bizarre extrauterine sites 1 ; it is therefore no surprise that endometrioid carcinoma should be found in such places as the umbilicus and within endometriosis in obturator lymph nodes However, its occurrence is rare, and the neoplasm is almost always discovered while the uterus and ovaries are still in the patient, frequently along with ovarian endometriosis. The importance of recognizing a carcinoma as being endometrioid is primarily prognostic. The similarities of endometrioid and endometrial carcinomas as seen by electron microscopy are reflected in their biologic behaviors, less aggressive than those of their ovarian serous and mucinous counterparts. 4 In the ovary, endometrioid carcinoma is associated with 40-50% five-year and 33-37% ten-year survival rates, higher than those for other ovarian carcinomas. 12 However, in 1974, Malkasian and colleagues, and more recently, Tobias and Griffin, concluded that tumor grade and stage rather than cell type were most influential in survival. 17,24 The prognosis of extraovarian endometrioid carcinoma has not been studied because the neoplasm is so uncommon. However, case reports have stressed extended periods of survival or apparent cure even when cyst contents have been spilled or solid tumor has been left behind at operation. 7,8 Surgical resection is the primary mode of therapy, as in the ovary. Anderson reported success with progestogens, 2 and these have been used along with external radiotherapy. 16 Close attention should be paid to the histologic characteristics of endometriotic glands; an adenomatous appearance in a clinically banal case of endometriosis might be the harbinger of eventual extraovarian endometrioid carcinoma. References 1. Acosta AA, Buttram VC, Besch PK, et al: A proposed classification of pelvic endometriosis. Obstet Gynecol 42: 19-25, Anderson DG: Management of advanced endometrial adenocarcinoma with medroxyprogesterone acetate. Am J Obstet Gynecol 92:87, Anderson MC: Endometrioid tumor of the ovary with mucinous and serous components. Am J Obstet Gynecol 113: , Cummins PA, Fox H, Langley FA: An electron microscopic study of the endometrioid adenocarcinoma of the ovary and a comparison of its fine structure with that of normal endometrium and of adenocarcinoma of the endometrium. J Pathol 113: , Curling OM, Hudson CN: Endometrioid tumours of the ovary. Br J Obstet Gynaecol 82: , Czernobilsky B, Silverman BB, Mikuta JJ: Endometrioid carcinoma of the ovary A clinicopathologic study of 75 cases. Cancer 26: , Dockerty MB, Pratt JH, Decker DG: Primary adenocarcinoma of the rectovaginal septum probably arising from endometriosis. Cancer 7: , Dockerty MB: Malignant complications of endometriosis. Am J Obstet Gynecol 83: , Ferreira HP, Clayton SG: Three cases of malignant change in endometriosis including two cases arising in the rectovaginal septum. J Obstet Gynaecol Br Emp 65:41-44, Hejda V: Malignant transformation in ovarian endometriosis. Neoplasma 21:69-74, Koss LG: Miniature adenocanthoma arising in endometrial cyst in obturator lymph node. Cancer 16: , Kurman RJ, Craig MM: Endometrioid and clear cell carcinoma of the ovary. Cancer 29: , Lamm DL, Gittes RF, Benirschke K: Ectopic endometrial glands in lymph nodes masquerading as metastatic adenocarcinoma. J Urol 111: , Lash SR, Rubenstone AI: Adenocarcinoma of the rectovaginal septum probably arising from endometriosis. Am J Obstet Gynecol 78: , Lauslahti K: Malignant external endometriosis A case of adenocarcinoma of umbilical endometriosis. Acta Pathol Microbiol Scand 233:98-102, Mabry EB: Adenocarcinoma of endometriosis of the ovary A case report. Am J Obstet Gynecol 110: , Malkasian GD: Jorgensen EO, Webb MJ: Factors influencing ovarian cancer survival after chemotherapy. Obstet Gynecol 44: , Ridley JH: Primary adenocarcinoma in implant of endometriosis. Obstet Gynecol 27: , Sampson JA: Perforating hemorrhagic (chocolate) cysts of the ovary. Arch Surg 3: , Sampson JA: Endometrial carcinoma of the ovary arising in endometrial tissue in that organ. Arch Surg 10:1-80, Scully RE, Richardson GS, Barlow JF: The development of malignancy in endometriosis. Clin Obstet Gynecol 9: , Stevenson CS: Malignant transformation of ovarian endometriosis Nature, treatment and report of two cases. Obstet Gynecol 36: , Thompson JD: Primary ovarian adenocanthoma Its relationship to endometriosis. Obstet Gynecol 9: , Tobias J, Griffiths CT: Management of ovarian carcinoma. N Engl J Med 294: , Young EE, Gamble CN: Primary adenocarcinoma of the rectovaginal septum arising from endometriosis Report of a case. Cancer 24: , 1969