Ovarian Oxyphilic Sertoli Cell Tumor: Case Report And Review Of The Literature

摘要

Background: the ovarian oxyphilic Sertoli cell tumor is a rare neoplasm (only three cases were reported in literature). Sometimes the rarity itself of a lesion may rise some problem in the diagnosis, especially if it is send to several consultants. Case: an unusual case of ovarian neoplasm came to our attention, and we sent some slides of the case to two renowned referees of different Departments of Pathology. Pathologist 1 made a diagnosis of endometrioid adenocarcinoma, while Pathologist 2 made the diagnosis of oxyphilic Sertoli cell tumor. He sends the same slides to Pathologist 1, who confirmed his diagnosis. Conclusion: the two different diagnosis set different managements of the lesion for the clinician, but overall they set the pathologist who requested the consultation in a difficult position. In fact, when a pathologist sends to two experienced consultants a case, he really thinks to solve definitely the case itself. Unfortunately, sometimes it not happens. Introduction The oxyphilic Sertoli cell tumore is an extremely rare neoplastic pathology of the ovary. It was described for the first time from Ferry et al (1). In this paper they reported three young women (aged respectively 19, 21 and 30 years), two with the Peutz-Jeghers syndrome, which had unilateral ovarian tumors composed of Sertoli cells with abundant with eosinophilic cytoplasm (“oxyphilic”). The authors emphasized the need to include the Sertoli cell tumor in the differential diagnosis of oxyphilic ovarian neoplasms, particularly if there is a tubular pattern resembling the Sertoli structures. In the following years there wasn't any other report published in the literature about this tumor. In 1996 we observed a case of ovarian neoplasia simulating the oxyphilic Sertoli cell tumor. Since the lesion is extremely unusual, we decided to send in consultation to two expertise pathologists. When a “non specialist” pathologist has the diagnostic suspicion of an extremely rare lesion, it is a good way of thinking to send the histological slides and paraffin blocks to the pathologist which first described the lesion itself. Moreover, to obtain a conclusive confirmation it could be wise to send the case to another consultant with as expert in that field of pathology. The sending to two different pathologists should obviously be limited to very selected cases. In almost all cases the answers of the consultants give us a precise, definite, conclusive diagnosis of the lesion, but sometimes as in the present case the answer could create a “hard” condition for the clinician and the pathologist who requested the consultation, condition that may take to an enigmatic, difficult management of the lesion itself. Patient and Methods A woman, aged 50 years, came to the attention of the gynecologist in 1997 for the incidental finding of a right ovarian mass during an abdomen ultrasonographic exam. After a laparotomy the surgeon performed an isterectomy with bilateral annessiectomy. The right ovary weighted 210 gr, its main diameter was 16 cm with outer smooth, pinky surface. On the cut the mass was firm, yellowish, cystic centrally: the major thickness of the wall of the lesion was of 4 cm, and the inner surface appeared rough with yellowish, papillomatous proliferations, the major of the diameter of 0.8 cm. The left ovary appeared normal, and the uterus on the cut surface showed some leiomyomas of the anterior wall of the corpus and an atrophic endometrium. The microscopic examination of the ovarian mass showed a component of elongated tubules that grew in lobules separated by a typical fibrous stroma, sometimes hyalinized. The tubules were lined by cuboidal cells, closely packed, with small nuclei and scanty cytoplasm, accompanied by a great component constituted by large cell with abundant, oxyphilic cytoplasm (figure 1-4). Our preliminary diagnosis was of “ovarian Sertoli cell tumor, oxyphilic cell variant”: we excluded the diagnosis of endometriod adenocarcinoma (in our opi