# Primary Mesenteric Sertoli-Leydig Cell Tumor:  A Case Report and Review of the Literature

Authors: Amel Trabelsi; Soumaya Ben Abdelkarim; Mohamed Hadfi; Ridha Fatnaci; Wided Stita; Badreddine Sriha; Sadok Korbi
Journal: Journal of Oncology
 (2008)

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## Abstract

The occurrence of primary sex cord-stromal tumors at extraovarian sites is exceedingly rare. We report a new case of Sertoli-Leydig cell tumor in the mesentery of a 78-year-old woman who presented with occlusive syndrome and reviewed the previously reported cases of extraovarian sex cord-stromal tumors in the English literature.


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## Body

## 1. Introduction

The
occurrence  of primary sex cord-stromal tumors at extraovarian sites is
extremely rare [1]. These tumors are predominantly granulosa cell tumors [1].
To our knowledge, this is the first case of primary mesenteric Sertoli-Leydig
cell tumor.


## 2. Case Report

A new case  of a
78-year-old  woman who
presented with bowel
obstruction is reported.
Three years previously, she underwent bilateral salpingo-oophorectomy and total
abdominal hysterectomy for bilateral mucinous cystadenoma and vaginal prolapse.
Ultrasonography showed a solid mesenteric tumor; exploratory laparotomy showed
a nodular solid tumor at the mesenteric border of the distal ileum, measuring11×8cm. Twenty centimeters of ileum containing the isolated lesion was resected.
Macroscopically, the tumor was nodular and well circumscribed measuring 11×8×4cm. The cut surface was composed of pale yellow-grey soft tissue with foci of haemorrhage.
The tumor was limited to mesentery without extension to the ileal wall.
Microscopic examination revealed cellular lobules with nests and poorly
developed tubules of Sertoli cells (Figure 1) that showed moderate atypia and
mitotic figures that average 5–10 per high-power field (Figure 2). Leydig cells are found at the periphery of the cellular
lobules (Figure 3). By immunohistochemistry, the neoplastic cells showed
positive staining for antibodies against inhibin (Figure 4) and vimentin. Tumor
cells were negative for EMA (epithelial membrane antigen), calretinin,
synaptophysin, chromogranin A, and CD117 (c-kit). The diagnosis of
Sertoli-Leydig cell tumor with intermediate differentiation of the mesentery was
established. The patient has not received any additional therapy, yet she
remains free of disease after five years.Figure 1
Cellular
lobules and nests with poorly developed tubules (HEx200).Figure 2
Sertoli cells with moderate atypia and mitotic figures (HEx200).Figure 3
Leydig cells surrounded tumor lobules (HEx400).Figure 4
Tumor
cells immunoreactive to anti-inhibin (IHCx400).


## 3. Discussion

The occurrence
of primary sex cord-stromal tumor at extraovarian sites is extremely rare [1],
such that in the English literature only 13 cases have been reported [1–13].
Eight were classified as granulosa cell tumors [1–6, 11, 12], two as thecomas
[7, 8], two as sex cord-stromal tumors, and one as an unclassified form of
stromal sex cord tumor [13]. The sites of origin were usually within the pelvis
[1]: six arose in the broad ligament [1], two in the retroperitoneum [2, 12, 13],
one in the fallopian tube [10], one in an umbilical herniae sac [9], one in the
adrenal gland [6], and one in the pelvic sidewall [11]. These previous reported
cases are summarized in Table 1.Table 1
Reported cases of extraovarian
sex cord-stromal tumors.
ReferenceAge (years)Histological diagnosisSite of originRecurrence[2]51Granulosa cell tumorRetroperitoneumNo[3]37Granulosa cell tumorBroad ligamentNo[4]30Granulosa cell tumorBroad ligamentNo[5]40Granulosa cell tumorBroad ligamentNo[6]52Granulosa cell tumorAdrenal glandNo[7]76FibrothecomaBroad ligamentNo[8]70ThecomaBroad ligamentNo[9]66Sex cord tumor with annular tubesUmbilical herniae sacNo[10]32Sex cord tumor with annular tubesFallopian tubeNo[1]45Granulosa cell tumorBroad ligamentYes[11]67Granulosa cell tumorPelvisNo[12]54Granulosa cell tumorRetroperitoneumNo[13]32Unclassified stromal sex cord tumorsRetroperitoneumNoOur case78Sertoli-Leydig cell tumorMesenteryNoTo our
knowledge, this is the first case of a primary mesenteric Sertoli-Leydig cell
tumor. Clinically, these tumors sometimes produce estrogen [1, 6]. However, in
our case, no biologic assay for estrogen content of the patient's urine or
blood was performed, because the nature of the tumor was not suspected until it
was removed.The histogenesis
of extraovarian sex cord-stromal tumors has been reviewed in the literature [12].In recent
years, several investigators have claimed that the sex cords may originate from
the mesonephros [14]. A dualistic theory of both coelomic epithelium and mesonephros
in the origin of the pregranulosa cells has also been proposed [12].
Accordingly, the mesonephros itself or its functional influence seems to be
necessary for creating the sex cords. This is consistent with gonad formation
being limited to the gonadal ridge and may explain why the sites of origin of
extraovarian sex cord-stromal tumors are limited to the broad ligament,
retroperitoneum, mesentery, and adrenal gland, all of which differentiate close
to the mesonephros and mesonephric duct [1]. Sex cord-stromal tumors can be
difficult to distinguish from several other neoplasms including
undifferentiated carcinoma, gastrointestinal stromal tumors (GIST) or
metastatic melanoma. The immunohistochemical findings are invaluable in
confirming the diagnosis.The prognosis
for extraovarian sex cord-stromal tumors seems to be favorable; however, reported
cases and clinical experiences are limited.



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