极为罕见的病例，非常感谢王主任的分享！

1）文献已报道卵巢可分泌ＡFP的肿瘤包括：卵黄囊瘤、伴有卵黄囊成分的生殖细胞肿瘤、卵巢表面上皮癌（浆液、粘液、内膜样）伴有肝样分化（肝样腺癌）、性索间质肿瘤伴有肝样分化、苗勒氏管混合性肿瘤。

2）如何鉴别性索间质肿瘤（支持－间质细胞瘤）伴肝样分化和肝样癌伴性索间质肿瘤成分？二者是一回事还是二回事？

3) 此病例如诊断为“伴肝样分化的支持-间质肿瘤”，可能为世界英文文献报道第26例；诊断为“肝样腺癌伴性索间质肿瘤”， 为文献报道的第2例。

4）如何鉴别诊断及其临床意义（肿瘤的恶性程度和治疗建议）？

5)理论上如何解释“性索间质细胞向肝细胞分化”？

谢谢！

D'Antonio A, De Dominicis G, Addesso M, Caleo A, Boscaino A.

Hepatoid carcinoma of the ovary with sex cord stromal tumor: a previously unrecognized association.unrecognized association. Arch Gynecol Obstet. 2010:765-8.

BACKGROUND: Hepatoid carcinoma (HC) of ovary is a rare type of epithelial tumor composed mainly of epithelioid cells with abundant acidophilic cytoplasm,histologically indistinguishable from hepatocellular carcinoma. We report a previously unrecognized case of HC of ovary concurrent with a Sertoli cell tumor. CASE REPORT: A 42-year-old woman patient with a long-term history of hepatitis C  presented with a mass of left ovary without evidence of hepatic tumor. After initial diagnosis of primary ovarian carcinoma (FIGO Stage I), she had experienced a first recurrence in upper abdomen. Histologically, the primary tumor was composed of epithelioid cells with "hepatoid features" in association with a sex cord stromal tumor of Sertoli-type. Immunohistochemistry hepatoid cells stained positively for hepatocyte paraffin-1, alpha-fetoprotein and alpha-1 antitrypsin; moreover, Sertoli-type cells were positive for alpha-inhibin, calretinin and CD99. A final diagnosis of HC concurrent with Sertoli-type tumor was made. CONCLUSION: The occurrence of this unreported association of HC with Sertoli-like tumor, the problems of differential diagnosis and therapeutic management of these tumors are the subject of this presentation. A diagnosis of ovarian metastasis from hepatocellular carcinoma is easy in patients with known primary tumor of liver and should be always excluded in these cases as an hepatoid variant of yolk sac tumor. Immunohistochemistry is not useful in these cases. However, a combination of clinical and pathological features is necessary for a correct diagnosis.

 肝样分化的肿瘤    是最后的诊断？

不是最后诊断。

1. Indian J Cancer. 2009 Jan-Mar;46(1):64-6. Recurrent alpha-fetoprotein secreting Sertoli-Leydig cell tumor of ovary with an unusual presentation.

Poli UR, Swarnalata G, Maturi R, Rao ST.

Department of Surgical Oncology, MNJ Institute of Oncology and Regional Cancer Centre, Hyderabad, India. ushapoli@yahoo.co.in

Alpha-fetoprotein secreting (AFP) Sertoli-Leydig cell tumors of ovary (SLCT) are now identified as a distinct entity among the uncommon group of sex cord tumors of ovary. We report an unusual case of recurrent AFP secreting ovarian tumors and as ileocecal mesenteric cyst in a 25-year-old patient resulting in difficulty in initial diagnosis of AFP producing SLCT. Although six recurrent cases were described out of the 25 reported cases of AFP secreting SLCTs, this patient with an unusual presentation of recurrence is the second case in the literature to the best of our knowledge.

2. Gan No Rinsho. 1989 Jan;35(1):107-13.

[An alpha-fetoprotein producing Sertoli-Leydig cell tumor--a case report] [Article in Japanese]

Taniyama K, Suzuki H, Hara T, Yokoyama S, Tahara E.

Dept. of Pathology, Shizuoka General Hospital.

A case of an ovarian, poorly differentiated, Sertoli-Leydig cell tumor in a 55-year-old woman is reported. The patient showed no virilization, but did show elevated serum alpha-fetoprotein (AFP) levels. The tumor, measuring 70x45x50 mm, consisted mainly of a diffuse proliferation of spindle cells.Among this proliferation, some tubules of Sertoli cells and groups of leydig cells were found. Immunoperoxidase studies, using anti-AFP, localized the AFP in the leydig cells.

3. Arch Gynecol. 1985;236(3):187-96.

AFP-producing Sertoli-Leydig cell tumor of the ovary.

Sekiya S, Inaba N, Iwasawa H, Kobayashi O, Takamizawa H, Matsuzaki O, Nagao K.

A tumor of the right ovary in a 21-year-old single woman is reported. Secondary amenorrhea, hirsutism, acne and deepening of the voice were associated with the tumor. Light and electron microscopic examinations showed that the tumor was composed of cells resembling Sertoli and Leydig cells of the testis in their cytology features and growth patterns. High levels of circulating dehydroepiandrosterone, androstenedione, testosterone and alpha-fetoprotein (AFP) were found preoperatively. Preoperative estrogen and progesterone levels were all slightly above the upper limits of normal for females. These hormone and AFP levels fell to within the normal range after removal of the tumor. Direct hormone and AFP production of this tumor was confirmed by immunohistochemical techniques  and long-term cell cultures in vitro. This is possibly the first report on a Sertoli-Leydig cell tumor in which AFP has been identified in the patient's plasma, in part of the tumor cells and the culture fluid.

4. Int J Gynecol Pathol. 1984;3(2):213-9.

Sertoli-Leydig cell tumor of the ovary producing alpha -fetoprotein.

Chumas JC, Rosenwaks Z, Mann WJ, Finkel G, Pastore J.

A 16-year-old girl with virilization and elevated serum alpha-fetoprotein (AFP) levels was found to have a Sertoli-Leydig cell tumor of the ovary. Immunoperoxidase studies using anti-AFP localized AFP in cells with the histologic appearance of Leydig cells. Serum AFP fell to undetectable levels after excision of the tumor. The patient is alive and well 1 year postexcision.