Hyperandrogenism, Elevated 17-Hydroxyprogesterone and Its Urinary Metabolites in a Young Woman with Ovarian Steroid Cell Tumor, Not Otherwise Specified: Case Report and Review of the Literature

Table 3

A summary of steroid cell tumors, not otherwise specified (SCT–NOS) with 17-OHP concentration reported (, including the current case). Please refer to Supplementary Table S1 for a detailed summary of all cases.

		n	Reference
Age (years)^b	23 (3–68)^a	21	[9, 11, 22–27, 29–35, 44–48]
Extraovarian (%)	14 (3/21)	21
Tumor size (cm)^c	4.9 (1–12)^a	20	[9, 11, 22, 24–27, 29–35, 44–48]
Evidence of malignancy (%)	5 (1/21)	21	[9, 11, 22–27, 29–35, 44–48]
Serum testosterone concentration (nmol/L)^d	12 (1.2–37)^a	21
Elevated testosterone concentration (%)	100 (21/21)	21
Serum DHEA-S concentration (µmol/L)^d	2.9 (0.6–19.7)^a	14	[9, 11, 23, 24, 26, 27, 29–32, 44, 45, 48]
Elevated DHEA-S concentration (%)	28 (5/18)	18	[9, 11, 23, 24, 26, 27, 29–34, 44–48]
Serum androstenedione concentration (nmol/L)^d	35 (6.3–78)^a	15	[9, 23–27, 29, 31–35, 44, 45]
Elevated androstenedione concentration (%)	88 (14/16)	16	[9, 23–27, 29, 31–35, 44, 45, 47]
Serum 17-OHP concentration (nmol/L)^d	48 (1.8–312)^a	16	[9, 11, 23–27, 29–32, 35, 44, 45, 48]
Elevated 17-OHP concentration (%)	81 (17/21)	21	[9, 11, 22–27, 29–35, 44–48]
Positive 17-OHP response after 1–24 ACTH stimulation (%)^e	20 (2/10)	10	[11, 25, 27, 29–31, 33–35]
Hormonal cosecretion (hormones other than androgens) (%)	33 (7/21)	21	[9, 11, 22–27, 29–35, 44–48]
Cortisol	10 (2/21)
Estradiol	14 (3/21)
Estradiol and cortisol	10 (2/21)

^a Results expressed as median (range). ^bAge at diagnosis. ^cLargest dimension. If tumor size was not reported, the size of the affected ovary was used. ^dFor results reported as “larger than X units”, the value of X was used. For cases in which the diagnosis was delayed, the values at the age of diagnosis was used. Cases with no values reported (stated as “normal”), suspected errors in reporting units or unknown conversion factor to SI units were excluded. ^eDefined as positive if commented to be increased from baseline by authors and peak 17-OHP >30 nmol/L. Otherwise, it was arbitrarily defined an increase in 17-OHP, at either 30 min or 60 min after ACTH stimulation, by equal to or more than 100% of the basal value, and a peak value >30 nmol/L.