Dilemma: A Case of Ovarian Sertoliform Endometrioid Carcinoma

Arasse Dino G. Martinez, Harriza M.Haron-Gangco, Humphrey C. Bitun, John A. Coloma

Department of Pathology and Laboratories, Jose R. Reyes Memorial Medical Center, Manila

Date of Submission: November 20, 2018

ABSTRACT

Sertoliform Endometrioid Carcinoma is a rare variant of Endometrioid Carcinoma which

commonly occurs in women with an average age of 25 years and has histologic resemblance to
sex cord–stromal tumors such as Sertoli-Leydig cell tumors (SLTs). This report aims to present a
case of Ovarian Sertoliform Endometrioid Carcinoma, its clinical presentation, histomorphologic
and immunohistochemical features. A 52 year old menopausal woman G2P2(2002) came in for
consult with complaint of abnormal uterine bleeding. The patient underwent Total
Hysterectomy with Bilateral Salpingooophorectomy, and Bilateral Lymph Node Dissection
Infracolic Omentectomy and Random Peritoneal Sampling. On morphological evaluation, the
case was signed out as a case of Sertoli Leydig Cell Tumor, Intermediate Type with Retiform
Pattern, cannot totally rule out Carcinosarcoma, Ovary, Left. With the varied morphologic
features seen, the role of Immunohistochemistry is deemed essential.

Key words: Sertoliform Endometrioid Carcinoma, SEC, Ovary, Immunohistochemistry, Epithelial

Membrane Antigen, Calretinin, Estrogen, Progesterone, CD99, Inhibin, Carcinoembryonic
Antigen, S100
presentation, histomorphologic features
Introduction and immunohistochemical features.

Sertoliform Endometrioid Carcinoma was Case Summary:

first described separately by Young et. al.
and Roth et.al in 1982. Roth et.al. decribed
four cases of an unusual variant of well
differentiated endometrioid carcinoma that
was predominantly composed of tubules,
solid or hollow, as well as cord-like areas
histologically mimicking Sertoli and Sertoli-
Leydig cell tumors.
This case report aims to present a case of
Ovarian Sertoliform Endometrioid
Carcinoma and to describe its clinical
A 52 year old menopausal woman
G2P2(2002) came to consult with complaint
of abnormal uterine bleeding. No endocrine
symptoms such as hirsutism or deepening
of voice was noted. Consult was done.
Ultrasound revealed unilateral complex
ovarian mass. The patient underwent Total
Hysterectomy with Bilateral
Salpingooophorectomy, and Bilateral Lymph
Node Dissection Infracolic Omentectomy
and Random Peritoneal Sampling.
 The specimen submitted for frozen and and eosinophilic with irregular borders
permanent section labeled as “left ovary” (Figure 2).
consists of a previously opened, tan-red,
irregularly-shaped, rubbery tissue
measuring 26.0 x 4.0 x 3.0 cm comprising of
a solid area measuring 8.0 x 4.0 x 3.0 cm
and two (2) cystic areas measuring 10.0 x
5.0 x 0.7 cm and 13.0 x 10.5 x 3.0 cm. Cut
section of the solid mass shows tan-white
to tan-red rough surface. Cut section of the
larger cystic area shows hollow cavity filled
with serous fluid lined by tan-red walls. Cut
section of the smaller cystic area shows
hollow cavity with tan-red walls lined by
tan-brown necrotic material. The attached
left fallopian tube consists of a tan-red
tubular, rubbery tissue measuring 5.0 x 0.8
x 0.8 cm.

Microscopic sections of specimen

labeled “left ovary” show confluent,
crowded vague glands that merge with loss
of the intervening stroma and back to back
cribriform glands (Figure 1).

Some areas show hypercellularity of

the stroma with spindle shaped cells having
moderate
nuclear pleomorphism and few
mitoses(Figure 3).

The cells show mild to moderate

atypia, hyperchromatic nuclei and
prominent nucleoli. The cytoplasm is scanty
 There are also solid tubules seen that
are round to elongated containing
S columnar epithelial cells with round to
oblong nuclei without prominent nucleoli
1. (Figure 5).

2.

Other areas show nodular arrangement The cells lining the hollow and solid
of tumor cells with fibrous bands separating tubules have moderate to abundant pale
lobules composed of hollow tubules that cytoplasm (Figure 6).
are round to oval, some are small and some
are cystically dilated with eosinophilic
secretions (Figure 4).

N N

N
N

Some of the stromal fibrous band
separating the lobules are slightly
edematous containing cells with large
eosinophilic cytoplasm with large round
nucleus that may be eccentric in location
(Figure 7).
Lymphovascular space invasion was not
demonstrated.
Considering morphological features, the
case was signed out as a case of Seroli
Leydig Cell Tumor, Intermediate Type with
Retiform Pattern, cannot totally rule out
Carcinosarcoma, Ovary, Left. To further
strengthen the histopathologic diagnosis,
immunohistochemistry panel was deemed
essential.
The following Immunohistochemical
stains are requested: Carcinoembryonic
Antigen with Inhibin, Epithelial Membrane
Antigen (EMA), Carcinoembryonic Antigen
(CEA), S100, Calretinin, Estrogen (ER),
Progesterone (PR), and CD99. The EMA is
diffusely positive. The Calretinin, ER, PR,
and CD 99 yield are likewise positive.
Inhibin, CEA and S100 yield negative (Figure
8).
Discussion:
Sertoliform Endometrioid Carcinoma (SEC)
is a rare variant of Endometrioid Carcinoma
which commonly occurs in women with an
average age of 25 years. The mean age
group for this tumor has been reported as
60 years. It affects postmenopausal females
in the age group of 50–70 years in contrast
to SLTs which are common in younger
females. In a study of 13 cases of SEC ovary,
the age of 41 years was found to be the
youngest age affected while others
reported this entity in a 55-year-old
female.1To date, there are nearly 80 cases
in the ovary and 20 cases in endometrium
that have been reported in literature.2
As stated in Blaustein’s Pathology of the
Female Genital Tract, patients with
Endometrioid Carcinoma commonly present
with abdominal distention and pelvic or
abdominal pain. Abnormal vaginal bleeding
is also a frequently associated symptom.
This was said to be partly related to the
association of endometrioid ovarian
carcinoma with endometrial hyperplasia
and carcinoma. Most patients have an
adnexal mass on pelvic examination.3In a
study by Ordi et. Al., they noted abdominal
enlargement secondary to a unilateral
ovarian mass as the most frequent clinical
presentation. Additionally, they noted that
up to 50% of SLT patients may exhibit
endocrine manifestations.Patients with an
SEC tumor may, however, present with
virilizing signs that may lead to an incorrect
clinical diagnosis.4In a study by Pai et al,
they noted tenderness associated with
1
Pallavi BD, Maithili KM, Sinai Khandeparkar SG,
Jadhav AB. Sertoliform endometrioid carcinoma of the
ovary in a young female. Indian J PatholMicrobiol
2018;61:459-60
2
Kankaya D, Kahraman K, Ortaç F, Ensari A. Ovarian
sertoliform endometrioid adenocarcinoma: A rare
variant which causes diagnostic pitfalls. Ankara
ÜnivTıpFakMecmuası 2015;68:133-6.
3
Seidman JD, Russel P, Kurman RJ. Surface
epithelial tumors of the ovary. In: Kurman R, editor.
Blaustein′s Pathology of the Female Genital Tract.
6 th ed. New York: Springer-Verlag Inc.; 2011. p. 751-
758.  of the ovary: A potential diagnostic pitfall. Arch Pathol
4
Ordi J, Schammel DP, Rasekh L, Tavassoli FA.
Sertoliform endometrioid carcinomas of the ovary: A
clinicopathologic and immunohistochemical study of
13 cases. Mod Pathol 1999;12:933-40.  clinicopathological analysis of 13 cases. Am J
torsion secondary to a gradually enlarging
ovarian cyst as a presenting symptom.5
According to studies done by Young et. Al.,
Ordi et. Al and Remadi et. Al, Sertoliform
Endometrioid Carcinoma tumors vary
considerably in size, with reported cases
ranging from 4 to 43 cm and weighing from
49 to 1650 grams.Preference for laterality
was not noted, though bilateral tumors
were said to occur approximately 10% of
the time. Most are predominantly solid,
with foci of hemorrhage and necrosis
present in some cases.6
As mentioned in Blaustein, Sertoliform
endometrioid carcinoma is characterized to
have a predominant pattern resembling a
sex cord–stromal tumor (Sertoli–Leydig Cell
Tumor), characterized by small tubular
glands lined by cuboidal or low columnar
epithelium with a paired cell arrangement
resembling well- differentiated Sertoli cell
tumor. Anastomosing solid tubules are
often said to be present. Cellular fibrous
stroma resembling the spindle cell
component of stromal tumors may also be
present.1In addition, Young et. al noted that
microscopically, typical glands of
endometrioid carcinoma, squamous
differentiation, or an adenofibroma
component are each present in 75% of
SECs, facilitating their recognition as an
endometrioid carcinoma.7Furthermore,
5
Pai P, Jadhav MN, Patil RK, Kittur SK. Sertoliform
endometrioid tumor of ovary presenting as torsion. J
Lab Physicians 2016;8:112-5
6
Misir A, Sur M. Sertoliform endometrioid carcinoma
Lab Med 2007;131:979-81.
7
Young RH, Prat J, Scully RE. Ovarian endometrioid
carcinomas resembling sex cord-stromal tumors: A
SurgPathol 1982;6:513-22. 
Ordi et al., reported that SEC typically
demonstrates (1) the presence of areas with
usual pattern of endometrioid carcinoma
and(2) presence of mucin at the apical
borders of the tumor cells which are not
seen in Sertoli Leydig Cell Tumor.2
According to Dabbs, Sertoliform
endometrioid carcinomas stain not only for
CK but also for
EMA, and they are usually negative for
Inhibin and Calretinin. 8
Epithelial Membrane Antigen (EMA), along
with cytokeratins stain positive in the
cytoplasm and membrane of cells in
endometrioid carcinoma. Inhibin, a
cytoplasmic stain, is a sensitive and
relatively specific marker of sex cord–
stromal tumors of the ovary, and its main
use in gynecologic pathology is in the
differential diagnosis of such tumors.
Calretinin, a cytoplasmic and nuclear stain,
stains a broader range of sex cord–stromal
tumors than inhibin, and it is a more
sensitive but less specific marker of such
tumors.It is typically used in an IHC panel
that also includes inhibin, steroidogenic
factor 1 (SF-1), and FOXL2.
Sex cord–stromal tumors—including
granulosa cell tumors,Sertoli cell tumors,
and tumors in the fibroma-thecoma group
—occasionally show positive staining for S-
100, a cytoplasmic stain. ER and PR
expression is seen in a wide range of
nonuterine tissues and in both benign and
malignant. ER and PR expression is
moderate to strong in the nuclei of cells in
8
David J. Dabbs, Diagnostic Immunohistochemistry
Theranostic and Genomic Applications, 4th edition,
Saunders 2014. pp 653-709
endometrioid carcinomas.
Carcinoembryonic Antigen (CEA), a
cytoplasmic stain, stains positive for ovarian
mucinous tumors.The
immunohistochemical stain CD99, which
stains the membrane, stains positive for
both Ovarian surface epithelial tumors and
ovarian sex-cord stromal tumors.
Conclusion:
Seroliform Endometrioid Carcinoma is a
rare variant of Endometrioid Carcinoma
which presents a diagnostic dilemma on
morphological evaluation due to its
resemblance to sex cord stromal tumors
such as Sertoli-Leydig Cell Tumor. The
diagnosis of such involves
immunohistochemistry with an antibody
panel composed of epithelial cell markers
such as Epithelial Membrane Antigen
(EMA), sex cord markers (Inhibin and
Calretinin), hormone receptors (Estrogen
and Progesterone). CD99, Carcino-embronic
Antigen (CEA), and S100. In addition to
which, the demographics and the clinical
presentation must be taken into
consideration and carefully correlated with
histopathological and immunohistochemical
findings to arrive at a specific diagnosis.
References:
David J. Dabbs, Diagnostic
Immunohistochemistry Theranostic and
Genomic Applications, 4th edition, Saunders
2014. pp 653-709
Kankaya D, Kahraman K, Ortaç F, Ensari A.
Ovarian sertoliform endometrioid
adenocarcinoma: A rare variant which
causes diagnostic pitfalls. Ankara
ÜnivTıpFakMecmuası 2015;68:133-6.
Misir A, Sur M. Sertoliform endometrioid Pai P, Jadhav MN, Patil RK, Kittur SK.
carcinoma of the ovary: A potential Sertoliform endometrioid tumor of ovary
diagnostic pitfall. Arch Pathol Lab Med presenting as torsion. J Lab Physicians
2007;131:979-81. 2016;8:112-5

Ordi J, Schammel DP, Rasekh L, Tavassoli

FA. Sertoliform endometrioid carcinomas of
the ovary: A clinicopathologic and
immunohistochemical study of 13 cases.
Mod Pathol 1999;12:933-40. 

Pallavi BD, Maithili KM, Sinai Khandeparkar

SG, Jadhav AB. Sertoliform endometrioid
carcinoma of the ovary in a young female.
Indian J PatholMicrobiol 2018;61:459-60

Seidman JD, Russel P, Kurman RJ. Surface

epithelial tumors of the ovary. In: Kurman R,
editor. Blaustein′s Pathology of the Female
Genital Tract. 6 th ed. New York: Springer-
Verlag Inc.; 2011. p. 751-758. 

Young RH, Prat J, Scully RE. Ovarian

endometrioid carcinomas resembling sex
cord-stromal tumors: A clinicopathological
analysis of 13 cases. Am J SurgPathol
1982;6:513-22.