GYNECOLOGIC

ONCOLOGY

41, 259-262

(1991)

CASE REPORT Primary Clear Cell Carcinoma of the Peritoneum KENNETH *Department

of Pathology

R. LEE, M.D.,*,’

USHA VERMA, JEROME BELINSON, M.D.t.3

and TDepartment of Obstetrics College of Medicine, Burlington,

M.D.,tx*

and Gynecology, Vermont 05401

AND University

of Vermont,

Received December 3, 1990

A caseof primary clear cell carcinomaof the pelvic and abdominal peritoneumwhich occuredin a 67-year-oldwomanand with histologicalcharacteristicsof Mullerian derivation is presented.To our knowledge,this is the first report of sucha case. Although clear cell carcinomashave beenpreviously describedin peritonealor retroperitoneallocations, thesehave beenmasslesions thought to arise from endometriosis.All other casesof diffuse primary peritonealadenocarcinomas have beenof the serous type. Q 1991 Academic FVes.s, Inc. INTRODUCTION

Primary serous papillary adenocarcinoma of the peritoneum is recognized as an extraovarian Mullerian neoplasm distinct from mesothelioma [l-13]. The light microscopical features of this tumor are identical to those serous papillary adenocarcinomas involving the surface of the ovary [14,15]. Clear cell carcinoma, another ovarian neoplasm of Mullerian derivation [16], has not been described as a primary peritoneal tumor with diffuse spread similar to that of primary serous papillary peritoneal carcinoma. We report such a case.

CASE REPORT

A 67-year-old multiparous female was in good health until she noticed abdominal distension, a change in bowel habits, and weight loss. An abdominal examination at the ’ To whom correspondence should be addressed. * Current address: J-1/160, Rajouri Gardens, New Delhi, India 110027. ’ Current address: Department of Gynecology, Cleveland Clinic, 9500 Euclid Ave., A-81, Cleveland, Ohio 44195. 259

Medical Center Hospital of Vermont revealed ascites without hepatosplenomegaly. The pelvic examination was inconclusive. A rectovaginal examination detected the presence of a thickening in the midline occupying most of the cul-de-sac. However, the rectum was freely movable over this area. A chest X ray and a barium enema were normal. A CAT scan showed an area of abnormal tissue in the pelvis approximately 6.0 cm in diameter. The kidneys were normal. Ascitic fluid was obtained and demonstrated malignant cells consistent with adenocarcinoma. The CA-125 level was 2218 p/ml (O-35 p/ml). Liver function tests were normal. An exploratory laparotomy disclosed a fine granularity of all pelvic and most abdominal parietal and visceral peritoneal surfaces. The small bowel and both fallopian tubes were adherent and bound down in the cul-de-sac, creating the mass-like effect seen on CAT scan. Both ovaries appeared normal. The liver was normal and there were no palpable para-aortic lymph nodes. Biopsies were taken from the small bowel serosa and cul-de-sac. A supracervical hysterectomy and bilateral salpingo-oophorectomy was performed. Histopathologic examination identified a clear cell adenocarcinoma diffusely involving the surfaces of the ovaries, fallopian tubes, serosa of the uterus, cul-de-sac, and small bowel serosa. No distinct tumor mass was identified. The endometrium, examined with an intraoperative curettage, revealed a well-differentiated endometrioid adenocarcinoma without a clear cell component. The tumor was also seen on three full-thickness sections of the myometrium and appeared similar histologically to the curetted tumor. It invaded approximately two-thirds of the myometrial thickness, but no vascular invasion or communication with the serosal clear cell tumor was seen. 0090~8258/91$1.50 Copyright 0 1991 by Academic Press, Inc. All rights of reproduction in any form reserved.

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AND BELINSON

FIG. 1. Tubules and glands lined by malignant cells with clear cytoplasm (H&E,

Microscopically, the numerous peritoneal tumor nodules were composed of small glands and tubules more or less uniformly spaced in a desmoplastic stroma. Occasional papillary structures were noted, some with hyaline eosinophilic cores. Focally the cells were arranged singly along the peritoneal surface without a stromal response. The malignant cells were enlarged with an expansile clear or eosinophilic cytoplasm. Nuclei were enlarged and moderately pleomorphic. Many contained prominent nucleoli. A hobnail arrangement of cells within glands or lining papillae was often seen. No psammoma bodies were pres-

x

175).

ent (Figs. 1 and 2). The cytoplasm was strongly PASpositive. Diastase eliminated all PAS positivity except that within a rare cell. A mucin stain revealed mucin within some glandular lumina and attached to cytoplasmic membranes. Rare cells with intracytoplasmic mucin were seen. Immunoperoxidase staining for cytokeratin (AEl/AE-3-Mak-6 combination-signet Laboratories, Dedham, MA, Triton Biosciences, Inc., Alameda, CA, neat; polyclonal, Dako Corp, Santa Barbara, CA, 1:400) was diffusely positive in the cytoplasm of tumor cells. Immunoperoxidase stains for CEA (Dako, 1:900), SlOO

FIG. 2. Malignant clear cells lining peritoneal surface (H&E,

X

1100).

261

CASE REPORT

(Dako, (Dako,

1: 1800), and placental 1: 300) were all negative.

alkaline

phosphatase

DISCUSSION In 1959, Swerdlow [l] first described a fully malignant papillary serous ovarian-like neoplasm arising primarily in the peritoneum. Several authors have since described many more cases [l-6,8-13]. These cancers are multicentric or diffuse and involve the ovaries only on the surface, if at all. The distinction between these tumors and peritoneal mesothelioma has been confirmed by light microscopical [7-91, immunohistochemical [2,4,6], and electron microscopical [3,6,13] studies. Their origin is thought to be from the peritoneum, which has the capacity to differentiate toward Mullerian epithelium [3,9], as they are identical to their ovarian counterparts [4]. Nonserous carcinomas similar to other cancers of ovarian type have been described as presenting as discrete mass lesions in extraovarian, peritoneal, or retroperitoneal locations. These have been of the endometrioid [17,18], mutinous [19,20], and clear cell [17,21,22] varieties. Their pathogenesis has been ascribed to the existence of an extraovarian extended Mullerian system [23] or to peritoneal endometriosis. Clear cell carcinoma of the ovary, in particular, is often associated with endometriosis [16,24] and those few reported primary extraovarian peritoneal clear cell carcinomas [ 17,21,22] have also presumably arisen from this anlage. These clear cell carcinomas were tumors which presented as distinct mass lesions in the peritoneum. We have not seen another report of a clear cell carcinoma of Mullerian type presenting with diffuse peritoneal spread in a manner similar to the many cases reported of serous type peritoneal carcinomas. However, Troung et al. [6], in their study of 22 cases of serous peritoneal carcinoma, reported 1 case with widespread clear cell change. Also, Mills et al. [ll] noted occasional cells with clear cytoplasm in their 10 cases of serous surface papillary carcinoma. The morphologic, histochemical, and immunohistochemical features in the case we report are all compatible with clear cell carcinoma of Mullerian type. The diffuse peritoneal seeding without ovarian parenchymal involvement and no recognizable tumor mass is consistent with a primary peritoneal surface origin. We cannot rule out an origin in a small focus of endometriosis which may have been obliterated by the tumor. The presence in the uterus of a Grade I endometrial adenocarcinoma, a finding sometimes associated with clear cell ovarian carcinoma [25] which often arises in ovarian endometriosis, also suggests this possibility. However, we found no actual endometriosis in the uterus, ovaries, or peritoneum examined. Even if this tumor had arisen in endometriosis, the mode of spread is distinctly unusual.

It is somewhat surprising that more cases of the type we describe have not been reported, given the ever-increasing number of serous tumors originating in the peritoneum and the propensity for clear cell carcinoma to develop in other Mullerian structures from the vagina to the ovaries. Perhaps endometriosis is a required precursor of clear cell carcinoma in peritoneal locations, and when rare cases do occur, they are primarily mass lesions without peritoneal seeding. However, it is likely that the tumor we describe is a clear cell carcinoma which arose de nova from the peritoneum. If so, it illustrates the potential for the female peritoneum to give rise to Mullerian carcinoma of the clear cell type as well as that of the serous type. ACKNOWLEDGMENTS The authors thank Dr. Robert E. Scully of Massachusetts General Hospital for reviewing this case and Lisa Kapoor for typing the manuscript.

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Primary clear cell carcinoma of the peritoneum.

A case of primary clear cell carcinoma of the pelvic and abdominal peritoneum which occurred in a 67-year-old woman and with histological characterist...
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