Uterine tumor resembling ovarian sex-cord tumor – a case report and review of the literature

Abstract

A uterine tumor resembling an ovarian sex-cord tumor (UTROSCT) is a very rare lesion with only 38 cases reported in the literature so far. Here, we show an additional case of a pure UTROSCT with a DNA stemline at 1c in a 49-year-old woman presenting with abnormal vaginal bleeding. Problems in differential diagnosis arise mainly due to the variable histological picture of UTROSCT. Immunohistochemically, these tumors express cytokeratin, epithelial membrane antigen, vimentin, and smooth muscle actin. Moreover, in some cases, CD99 and alpha-inhibin are detectable. Although 36% of UTROSCT have infiltrative margins, almost all of them behave benignly. It is thus questionable whether the same prognostic criteria apply for these tumors as for endometrial stromal sarcomas. However, in the so-called mixed UTROSCT, the endometrial stromal sarcoma component determines the outcome.

Uterine tumors resembling ovarian sex cord tumors are polyphenotypic neoplasms with true sex cord differentiation

“…Our results support that UTROSCT are polyphenotypic neoplasms with true sex cord differentiation. As shown in Table 5, our data are in general agreement with previous studies addressing the issue of immunohistochemical expression of sex cord markers in UTROSCT 6,7,14,[17][18][19][20][21]24,30 and is the first to concurrently examine the four most commonly used markers. This literature review also highlights that ESTSCLE, or Type I tumors, less frequently show immunoreactivity for markers of sex cord differentiation, a finding which correlates with their morphologic appearance and relatively minor component of sex cord elements.…”

“…Results have been variable, with ultrastructural and immunohistochemical evidence of myogenic, epithelial, and sex cord differentiation. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] In the present study, we applied a panel of antibodies, inclusive of the most recently identified markers of sex cord differentiation (calretinin, melan A, CD99, and inhibin) as well as smooth muscle and endometrial stroma, to a series of UTROSCT and ESTSCLE. We found that all UTROSCT were immunoreactive for at least two markers of sex cord differentiation, often with coexpression of cytokeratin, CD10, vimentin, estrogen receptor, and progesterone receptor; desmin immunoreactivity, when present, was restricted to minor foci of smooth muscle.…”

“…[4][5][6][7] Numerous immunohistochemical and ultrastructural studies have attempted to phenotype the sex cord-like cells in UTROSCT and ESTSCLE, with variable evidence supporting myoid, [8][9][10][11][12][13][14] epithelial, 4,15 and true sex cord differentiation. 6,7,[16][17][18][19][20] Specifically, inhibin and CD99, 3,6,7,14,[17][18][19][20][21] as well as Melan-A, 19,21 and most recently, calretinin, 7,20 have been utilized as immunohistochemical markers of sex cord differentiation in UTROSCT and ESTSCLE based on application of these markers to sex cordstromal tumors of the ovary. [22][23][24][25][26][27][28][29] The present study was initiated following the diagnosis of a UTROSCT exhibiting a striking retiform pattern and Leydig-like stromal cells, indistinguishable from a Sertoli-Leydig cell tumor of the ovary.…”

“…30 However, it is noteworthy that 24 of 28 UTROSCT (86%) have been CD99-positive (Table 5). 6,7,17,19,21,24,30 Studies of Melan-A expression in UTROSCT are limited. Two of five UTROSCT in the present study were focally Melan-A positive, similar to Krishnamurthy et al 17 who found four of seven UTROSCT positive for Melan-A in o10 to 10-50% of tumor cells.…”

Müllerian Tumor (Atypical Polypoid Adenomyoma) with Sex-Cord Differentiation Arising from the Oviduct in an Adolescent Cynomolgus Monkey (Macaca fascicularis)

“…Morphological aspects of the lipid-rich foamy cells resembled those of ovarian sex-cord tumors of granu- trial tumors, nests of lipid-rich cells have been reported in sarcomatous elements with sex-cord-like differentiation (3,4,6). They are believed to originate from the endometrial stromal cells, or may arise from the displaced ovarian tissue in the myometrium (10,11).…”