0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Pathological Case of the Month FREE

[+] Author Affiliations

Section Editor: Enid Gilbert-barness, MD

More Author Information
Arch Pediatr Adolesc Med. 2000;154(4):419-420. doi:.
Text Size: A A A
Published online

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.

Tavassoli  FANorris  HJ Sertoli tumors of the ovary: a clinicopathologic study of 28 cases with ultrastructural observations. Cancer. 1980;462281- 2297
Link to Article
Zaloudex  C The ovary. Gompel  CSilverberg  Seds.Pathology in Gynecology and Obstetrics 4th ed. Philadelphia, Pa JB Lippincott Co1994;373- 374
King  D Ovarian cysts and tumors. Welch  KRandolph  JRanitch  MJ  O'NeillRowe  Meds.Pediatric Surgery 4th ed. Chicago, Ill Yearbook Medical Publishers1986;1345- 1347
Young  RHScully  RE Ovarian Sertoli cell tumors: a report of 10 cases. Int J Gynecol Pathol. 1984;4349- 363
Link to Article
Gustafaser  MLee  MScully  R  et al.  Mullerian inhibiting substance as a marker for ovarian sex-cord tumors. N Engl J Med. 1992;326466- 471
Link to Article
Lack  EYoung  RScully  R Pathology of ovarian neoplasms in childhood and adolescence. Pathol Annu. 1992;27281- 356

Tables

References

Tavassoli  FANorris  HJ Sertoli tumors of the ovary: a clinicopathologic study of 28 cases with ultrastructural observations. Cancer. 1980;462281- 2297
Link to Article
Zaloudex  C The ovary. Gompel  CSilverberg  Seds.Pathology in Gynecology and Obstetrics 4th ed. Philadelphia, Pa JB Lippincott Co1994;373- 374
King  D Ovarian cysts and tumors. Welch  KRandolph  JRanitch  MJ  O'NeillRowe  Meds.Pediatric Surgery 4th ed. Chicago, Ill Yearbook Medical Publishers1986;1345- 1347
Young  RHScully  RE Ovarian Sertoli cell tumors: a report of 10 cases. Int J Gynecol Pathol. 1984;4349- 363
Link to Article
Gustafaser  MLee  MScully  R  et al.  Mullerian inhibiting substance as a marker for ovarian sex-cord tumors. N Engl J Med. 1992;326466- 471
Link to Article
Lack  EYoung  RScully  R Pathology of ovarian neoplasms in childhood and adolescence. Pathol Annu. 1992;27281- 356

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 2

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections