Synonyms: Steroidogenic Factor 1

by Jan Klos

Background

Steroidogenic factor 1 (encoded by the NR5A1 gene on chromosome 9p33.3) is a transcription factor involved in organ development and differentiation of the endocrine system. SF1 is essential for fetal development of the hypothalamus, pituitary gland, gonads and the cortex of adrenal gland. SF1 has been shown to promote transcriptional activity of many steroidogenic enzymes from the cytochrome P450 family.

Staining in normal cells

All steroid-producing cells and gonadotropin producing cells in anterior pituitary.

Staining in tumors

All steroid hormones producing tumors and gonadotrophin producing tumors of anterior pituitary gland.

Staining patternControl tissue

Adrenal gland/gonads.

Application

SF-1 is highly sensitive and specific marker of tumors of steroidogenic type. It shows superior sensitivity to inhibin, WT-1, Calretinin, CD56 and CD99. It is recommended for identification of tissues/tumors producing steroid hormones.

• Sex-cord tumors of the ovary and testis: SF-1 shows close to 100% sensitivity in staining of ovarian sex-cord tumors (granulosa cell tumors, Sertoli-Leydig tumors including their variants, steroid cell tumors and fibroma/thecomas etc) and sex cord stromal elements in mixed tumors.
• Adrenocortical carcinoma is SF1+ in majority of nuclei in 90% cases and EMA-, while metastatic carcinoma in the adrenal gland is SF1- but EMA+.
• Sensitive marker of gonadotrophin producing tumors of anterior pituitary gland
• Few cases (10-15%) of clear cell carcinoma of kidney may show few positive nuclei (under 10%)
• Uterine tumors resembling ovarian sex cord-stromal tumor (UTROSCT) are negative

Possible pitfall

Ovarian stromal cells surrounding endometriosis are strongly SF1+ while endometriosis tissue is negative.

Selected references

1. Enriquez ML, Lal P, Ziober A, et al. The use of immunohistochemical expression of SF-1 and EMA in distinguishing adrenocortical tumors from renal neoplasms. Appl Immunohistochem Mol Morphol.2012 Mar;20(2):141-5.
2. McDonald W. SF1. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/stainssf1.html. Accessed June 3rd, 2020.
3. Mete O. ^1 2, Cintosun A ^1 2, Pressman I³, et al.Epidemiology and Biomarker Profile of Pituitary Adenohypophysial Tumors. Mod Pathol 2018 Jun;31(6):900-909.doi: 10.1038/s41379-018-0016-8. Epub 2018 Feb 12.PMID: 29434339
4. Noël JC, Borghese B, Vaiman D, et al. Steroidogenic factor-1 expression in ovarian endometriosis. Appl Immunohistochem Mol Morphol. 2010 May;18(3):258-61. doi: 10.1097/PAI.0b013e3181c06948. PMID: 19875956
5. Zhao C, Vinh TN, McManus K, et al. Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors. Am J Surg Pathol 2009 Mar;33(3):354-66. doi: 10.1097/PAS.0b013e318188373d.