Sex Cord Tumor with Annular Tubules

Definition

A distinctive variant of sex cord stromal-tumor with features of Sertoli and granulosa cell differentiation, divided in two subsets including those associated with Peutz-Jeghers syndrome and those without such association.

Clinical Features

33% associated with Peutz–Jeghers syndrome1–3
Symptoms suggestive of hyperestrinism in ≈50%

Gross Pathology

If associated with Peutz–Jeghers syndrome typically:
- benign 4
If unassociated with Peutz–Jeghers syndrome:
- unilateral
- often large
- ≈22% of cases clinically malignant5,6

Histopathology

Combines:
- pattern of growth reminiscent of Sertoli cells 7
Simple and complex annular tubules containing eosinophilic hyaline bodies, often calcified (Fig. 1
Fig. 1: Sex cord tumor with annular tubules. The patient had Peutz–Jeghers syndrome.
):
- morphologic hallmark
Ultrastructurally:
- demonstrates ambiguous or biphasic nature of tumor cells with:
  - features consistent with granulosa cell or nonspecialized ovarian stroma8,9
  - features indicative of Sertoli cell differentiation, notably Charcot–Bottcher filaments 10

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Sex Cord Tumor with Annular Tubules

Gonadoblastoma:
- similar appearance but contains germ cell component
- different clinical and genetic backgrounds

References

1 Hart WR, Kumar N, Crissman JD. Ovarian neoplasms resembling sex cord tumors with annular tubules. Cancer. 1980;45:2352–2363.

2 Scully RE. The prolonged gestation, birth, and early life of the sex cord tumor with annular tubules and how it joined a syndrome. Int J Surg Pathol. 2001;8:233–238.

3 Young RH, Scully RE. Ovarian tumors of probable wolffian origin. A report of 11 cases. Am J Surg Pathol. 1983;7:125–135.

4 Lele SM, Sawh RN, Zaharopoulos P, Adesokan A, Smith M, Linhart JM, et al. Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome: a case report. Mod Pathol. 2000;13:466–470.

5 Gloor E. Ovarian sex-cord tumor with annular tubules. Clinicopathologic report of two benign and one malignant cases with long follow-ups. Virchows Arch [A]. 1979;384:185–193.

6 Young RH, Welch WR, Dickersin GR, Scully RE. Ovarian sex cord tumor with annular tubules. Review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix. Cancer. 1982;50:1384–1402.

7 Anderson MC, Govan ADT, Langley FA, Woodcock AS, Tyagi SP. Ovarian sex cord tumours with annular tubules. Histopathology. 1980;4:137–145.

8 Crissman JD, Hart WR. Ovarian sex cord tumors with annular tubules. An ultrastructural study of three cases. Am J Clin Pathol. 1981;75:11–17.

9 Hertel BF, Kempson RL. Ovarian sex cord tumors with annular tubules. An ultrastructural study. Am J Surg Pathol. 1977;1:145–153.

10 Ahn GH, Chi JG, Lee SK. Ovarian sex cord tumor with annular tubules. Cancer. 1986;57:1066–1073.

Last updated: 24 Mar 2006