J Korean Med Sci.  2011 Jun;26(6):836-838. 10.3346/jkms.2011.26.6.836.

Refractory Hypertension and Isosexual Pseudoprecocious Puberty Associated with Renin-Secreting Ovarian Steroid Cell Tumor in a Girl

• Lee SH
• Kang MS
• Lee GS
• Chung WY

• KMID: 1785974
• DOI: http://doi.org/10.3346/jkms.2011.26.6.836

Abstract

Steroid cell tumor, not otherwise specified (NOS), are rare ovarian tumor, in addition, it is more rare in children. The majority of these tumors produce several steroid hormones, particularly testosterone. Estrogen also secreted by steroid cell tumor, NOS, but it is uncommon. Furthermore, hypertension is an infrequent sign in steroid cell tumor, NOS. An 8.5-yr-old girl with hypertension and frequent vaginal spotting visited at our clinic. On laboratory evaluation, secondary hypertension due to an elevated plasma renin level and isosexual pseudoprecocious puberty was diagnosed. Right solid ovarian mass was detected in radiologic tests. She underwent a right ooporectomy and it revealed renin and progesterone receptor positive steroid cell tumor, NOS. After operation, her blood pressure returned to normal level and vaginal bleeding disappeared. Even though this case is very rare, when hypertension coincides with virilization or feminization, a renin-secreting ovarian steroid cell tumor, NOS, should be considered.

Keyword

Pseudo-Precocious Puberty; Hypertension; Ovarian Neoplasms; Renin-Secreting Tumor

MeSH Terms

Child
Female
Humans
Hypertension/*etiology
Ovarian Neoplasms/complications/*diagnosis/pathology
Puberty, Precocious/enzymology/*etiology
Receptors, Cell Surface/metabolism
Receptors, Progesterone/metabolism
Renin/blood
Sex Cord-Gonadal Stromal Tumors/complications/*diagnosis/pathology
Steroids/biosynthesis
Tomography, X-Ray Computed
Vacuolar Proton-Translocating ATPases/metabolism

Figure

• 

  Fig. 1 Pelvic computed tomography. A 5.1 × 4 cm-sized, well-defined solid mass with multiple calcifications and fat density is noted in right adnexa (arrow).

• 

  Fig. 2 Histological appearance. (A) The individual tumor cells are polygonal. The cytoplasm is clear or eosinophilic, granular and focally show thick eosinophilic crystalloid materials (H&E staining, 400 × original magnifications). (B) Immunohistochemical stains. The tumor cells exhibit a diffuse positive reaction for rennin (brown). (C) Immunohistochemical stains. Ovarian stromal cells are positive for progesterone receptor (brown to black).

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