L Morelli, T Pusiol, F Piscioli
L Morelli, T Pusiol, F Piscioli. Ovarian Oxyphilic Sertoli Cell Tumor: Case Report And Review Of The Literature. The Internet Journal of Pathology. 2005 Volume 5 Number 1.
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 opinion the histological picture didn't fit with that diagnosis), and decided to send the histological slides to some consultants because of the rarity of the lesion. We choose a pathologist (Pathologist 1) of the Department of Pathology, Massachusetts General Hospital in Boston, and another pathologist (Pathologist 2) of the Department of Pathology and Laboratory Medicine (Anatomical Pathology), Vancouver Hospital, University of British Columbia, because of their high experience in the gynecopathologic field.
At the present time after 8 years the patient is still alive, she never had any recurrence and is free of disease.
The answer of Pathologist 1 was the following: “
A pathologist working in an institute to which comes any kind of lesions always has a limited experience in the field of extremely rare tumors. To avoid the misinterpretation of rare pathologies calling them “usual” it is dutiful for the pathologist to send the histological slide to the first who described the lesion in literature and to another expertise in the same field of interest. With this procedure it is always to “solve” the enigmatic case, performing a right diagnosis for the clinician and the patient himself. But sometimes there shall be disagreement between the two consultants that diagnosed the lesion: in this time it arouse the problem, which one of the diagnoses shall the pathologist who requested the consultation choose.
In the present case our lesion was sent to two pathologists with great reputation in the field of gynecopathology, which often contribute each other between themselves. The diagnosis of ovarian oxyphilic Sertoli cell tumor was confirmed both Pathologist 1 than Pathologist 2, who sent the same slides to Pathologist 1. Still Pathologist 1 made a complete different diagnosis (ovarian endometriod adenocarcinoma, grade 2-3) on the same material that we sent him: the histological slides sent to Pathologist 1 and 2 were identical; they were deep sections of the same histological paraffin block. The major problem that arises at this time is: because it is the requesting pathologist who has to make the final diagnosis, and just because it's him who has to talk to the clinician and be responsible of the diagnosis, which one of the two diagnoses shall believe? It is a very important problem for the clinician too, because depending on the diagnosis we give him he has to modify the therapeutic approach to the disease and the information to give to the patient himself, not only for the therapy but also for the prognosis.
The follow up of our case demonstrates that after 9 years the patient is still alive and free of disease, so it seems that the diagnosis of Pathologist 2, diagnosis that we choose, would be “correct” (although Ferry and coll. reported in their paper a recurrence in one of the three patients after 15 years from the surgical intervention). So the present case is the fourth case reported in literature of ovarian oxyphilic Sertoli cell tumor. It is also true that if a pathologist would make the “wrong choose” between two different diagnoses there will be a lot of consequences. Consequences would be not only by medical side (different therapy, different prognosis), but also by legal side (the patient could denounce the pathologist himself, the requesting pathologist, for inaccurate and wrong diagnosis). Our present case demonstrates so how for the expert pathologists too histopathology could sometimes be a real, surprising enigma.
Luca Morelli U.O. di Anatomia e Istologia Patologica e Citodiagnostica Ospedale “S. Maria Del Carmine” Piazzale S. Maria 6 38068 Rovereto (TN) ITALY Tel: 0464 453490 Fax: 0464 453029 Email: email@example.com