Figure 1. Abdominal computed tomographic scan showing a heterogeneous mass.
Figure 2. The cut surface of the tumor was predominantly solid with an intact thick capsule and multifocal areas of hemorrhage, necrosis, and thrombosis.
Figure 3. The tumor was composed of well-differentiated tubules, which are cut longitudinally in this section (hematoxylin-eosin, original magnification ×100).
Figure 4. The tubules are lined by columnar neoplastic cells and are surrounded by scant fibrous stroma (hematoxylin-eosin, original magnification ×400).
Sertoli cell tumors are classified as sex-cord stromal tumors and comprise fewer than 0.1% of ovarian tumors. They are included in the category of Sertoli-Leydig cell tumors even though they lack Leydig cells and account for only 4% of tumors in this generic category. These tumors have been reported in patients aged 7 to 79 years, with only a small fraction presenting in prepuberty.1 The tumor has been referred to as arrhenoblastoma, tubular adenoma of Pick, and tubular androblastoma. Androblastoma and arrhenoblastoma denote the masculinizing potential of these tumors. Two thirds are hormonally active, with 70% secreting estrogen and 16% secreting androgens.2
Children with tumors producing functional hormones develop evidence of virilization, such as hirsutism, hoarsening of the voice, clitoral hypertrophy, and an increase in somatic growth. Postmenarchal girls can present with complaints of amenorrhea, hirsutism, acne, masculinization of the body habitus, and breast atrophy.1 The differential diagnosis includes other causes of virilization: heterologous androgen administration, adrenal tumor or adrenal hyperplasia, intersex abnormalities, polycystic ovaries, or other ovarian neoplasms.3
To our knowledge, all pure Sertoli cell tumors reported in the English-language literature have been stage IA at the time of presentation.4 These tumors tend to be quite large, with 60% of the patients presenting with a palpable abdominal mass. The tumors are usually unilateral and confined to the ovary.4 On gross examination, the size ranges from 2½ to 28 cm in diameter.5 The tumor is firm, solid, and the cut surface demonstrates a yellow, fleshy, and tan appearance. Microscopically, the cells may be cuboidal, columnar, or round with either clear granular or vacuolated, cytoplasm.1 Three patterns are described: (1) tubular, (2) complex tubular, and (3) folliculoma lipidic of Lecene (lipid-rich).6 Mitotic figures, if present, are usually observed in younger patients. Charcot-Böttcher crystals, parallel arrays of microfilaments forming a crystal-like lattice, are a feature of human Sertoli cells and have been identified in these tumors.6 The survival rate for patients with Sertoli cell tumors is higher than 90%. The treatment of choice for stage IA tumors is surgical excision with no chemotherapy. Alpha1-fetoprotein and Müllerian inhibiting substances have been used as biochemical markers.5
Accepted for publication April 30, 1999.
Corresponding author: Ronald Scorpio, MD, Department of Medical Education, Spartanburg Regional Healthcare System, 101 E Wood St, Spartanburg, SC 29303.
Thank you for submitting a comment on this article. It will be reviewed by JAMA Pediatrics editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 2
Customize your page view by dragging & repositioning the boxes below.
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.