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represent less than 2% of all tumors in girls below 16
years of age. Less than 20% of ovarian malignancies re-
portedly arise from ovarian surface epithelial cells [3].
Epithelial tumors of the ovary are rare prior to puberty
[4].
The differential diagnosis of ovarian masses in ado-
lescents includes cyst formation, torsion with consecu-
tive edema, benign or malignant ovarian neoplasm, and
involvement of the ovary in lymphoma, leukemia, or me-
tastatic disease [5]. Deprest et al. reviewed 1700 reported
cases of ovarian neoplasms in females under 20 years of
age, and reported epithelial tumors in 17%, of which
26% were malignant [6]. Ovarian MCA is an epithelial
tumor, it is usually unilateral, and only 10% of cases, are
malignant [7]. They occur principally in middle adult life
and are exceptional in children and adolescents, such as
the present case [5].
CA-125 is a glycoprotein that is produced by certain
tumors, such as malignant epithelial ovarian tumors. In
premenopausal patients, benign findings such as leiomy-
omas, endometriosis, menstruation, and pregnancy may
elevate CA-125. Paran et al. reported that CA-125 was
within the normal range in 6 perimenarchal girls with
ovarain MCA [1]. Especially, MCA of the ovary with
torsion complicated with the elevation of serum CA-125
is an extremely rare occurrence in pediatrics.
K-ras mutations are common in mucinous ovarian tu-
mors, are probably an early event in mucinous ovarian
tumors, and are probably an early event in mucinous
ovarian carcinogenesis [2]. Interestingly, some microdi-
ssected mucinous tumors had the same K-ras mutation in
histologically benign, borderline, and malignant areas of
the same tumor [8]. Unfortunately, we did not examine
K-ras mutation.
In young patients with apparently benign ovarian cysts
requiring removal, the conservative approach of ovarian
cystectomy is advocated to enable the retention of func-
tioning ovarian tissue for endogenous hormone produc-
tion and future conception [4]. However, when a huge
cyst is encountered such as in our case, preserving the
ovarian tissue might be difficult. In the present case, it
was not possible to determine the origin of the mass be-
fore laparotomy, and there were no findings ruling out
malignancy because of the solid component of the tumor
and the elevation of serum CA-125. Therefore, the treat-
ment of choice was complete resection rather than biopsy.
Conservative surgery (i.e., unilateral salpingo-oophore-
ctomy) is generally sufficient for benign lesions and
early-stage ovarian tumors of borderline malignancy [9].
Torsion of MCA such as that reported here is rarely
seen in children or adolescent girls. Ovarian tumors that
undergo torsion can mimic acute appendicitis and may
lead to emergency surgery [10].
4. Conclusions
In conclusion, we need to consider MCA in adolescent
girls with the elevation of serum CA-125 and the pres-
ence of a very large ovarian mass.
5. References
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