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Diagnostics Histology, Training and education, Microscopy and imaging

Case of the Month

Pancreatic tumors of this type occur most often in which syndrome?

a. Multiple endocrine neoplasia type I
b. Multiple endocrine neoplasia type II
c. von Hippel-Lindau syndrome
d. Neurofibromatosis type I

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Question 1:
A. Calretinin

Question 2:
B. Estrogenic changes

This case corresponds to ovarian thecoma. Thecomas are one-third as common as granulosa cell tumors and typically occur in postmenopausal women. They are associated with estrogenic changes, such as uterine bleeding in 60 percent of cases, and as many as 20 percent of cases can be associated with endometrial carcinoma (usually endometrioid) (1). They tend to occur at a slightly older age than granulosa cell tumors (2).

Thecomas typically range from 5–10 cm, and only 3 percent are bilateral. Macroscopically, they are solid and present yellow cut surfaces. Cysts, hemorrhage, necrosis, and focal calcification may occur (1). Microscopically, these tumors are composed of sheets and nodules of round cells with ill-defined borders, including moderate to abundant eosinophilic to pale cytoplasm. Nuclei are round to slightly spindled with little or no atypia. A fibromatous component often forms septa that separate nodules, and hyaline plaques with calcification may be seen (2).

The neoplastic cells are positive for calretinin, inhibin, CD56, and WT1. They are often also positive for estrogen and progesterone receptor (1).

The differential diagnosis comprises several tumors of the sex cord-stromal tumors group, including fibroma, granulosa cell tumor, steroid cell tumor, and others (3).

References

  1. PB Clement, RH Young, Atlas of gynecologic surgical pathology, 3rd edition. Elsevier: 2014.
  2. RH Young, “Ovarian sex cord-stromal tumors: reflections on a 40-year experience with a fascinating group of tumors, including comments on the seminal observations of Robert E. Scully, MD”, Arch Pathol Lab Med, 142, 1459–1484 (2018). PMID: 30500284.
  3. RH Young, “Ovarian sex cord-stromal tumours and their mimics”, Pathology, 50, 5–15 (2018). PMID: 29132723.

Submitted by Luis Humberto Cruz Contreras, Hospital Materno-Infantil, Irapuato, Mexico.

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