Full Text - Acta Endocrinologica

doi: 10.4183/aeb.2015.381
Case Report
Secreting ovarian Leydig cell tumor with complex CLINICAL features:
virilizing syndrome, polyglobulia, hypertension, dyslipidemia and uterine
fibromatosis
C.M.V. Ghervan1,*, C. Nemes5, D. Muresan2, D. Crisan3, L. Ghervan4
University of Medicine and Pharmacy, 1Dept. Endocrinology, 2Dept. Obstetrics and Gynecology I, 3Dept.
Pathology, 4Institute of Clinical Urology and Renal Transplantation, Dept. Urology, Cluj-Napoca, Cluj,
5
Emergency County Hospital, Dept. Endocrinology, Satu-Mare, Romania
Abstract
Background. Virilizing syndrome in a postmenopausal woman is a concerning matter, raising the suspicion
of androgen-secreting tumour.
Case report. A 65 years old woman presented with
severe virilization features evolving rapidly over 4 years,
accompanied by: severe polyglobulia, severe hypertension,
dyslipidemia and uterine fibromatosis compressing both
ureters, producing first degree hydroureteronephrosis.
The hormonal dosages showed very high levels of both
testosterone (15.5 ng/mL) and estradiol (299 pg/mL),
meanwhile DHEAS level was normal, indicating an ovarian
pathological secretion. The endovaginal ultrasound and
computed tomography scan revealed an enlarged right
ovary of 5.5/2.8 cm. A total hysterectomy and bilateral
oophorectomy was performed. Pathological examination
confirmed the diagnosis of right ovarian hilum Leydig
cell tumour. After surgery, the testosterone and estradiol
levels normalized (concordant to the age and menopausal
status), the virilizing syndrome progressively improved
and polyglobulia, hypertension and dyslipidemia remitted
showing their secondary etiology.
Conclusion. We present a very rare case of secreting
ovarian Leydig cell tumour in a postmenopausal woman,
showing besides the virilizing syndrome, four unusual
features: severe polyglobulia, due to androgen excess, severe
hypertension and dyslipidemia, all remitted after tumour
removal, and severe compressive uterine fibromatosis that
was the consequence of the estrogen excess.
Key words: ovarian Leydig cell tumour,
polyglobulia, virilizing syndrome, secondary hypertension.
Introduction
The virilizing syndrome is defined as the
association of severe hirsutism, clitoromegaly,
deepening of the voice and alopecia occurring in
a woman. It is caused by the excess of androgen
hormones secreted by the adrenal glands, the ovaries
or of exogenous origin. This syndrome, especially
when it occurs in post-menopausal women, raises the
suspicion of androgen hypersecretion by an adrenal or
an ovarian tumour. Other possible causes at this age
are: hyperthecosis, Cushing’s syndrome, congenital
adrenal hyperplasia (extremely rare to be diagnosed at
menopause age) and iatrogenesis.
Adrenal androgen-secreting tumours are rare
and highly suggestive of malignancy. Ovarian androgen
secreting tumours are also rare but mainly benign;
seldom, these tumours may be driven by the high levels
of the gonadotropins seen in the post-menopausal state
(1, 2).
The majority of functioning ovarian tumours
are sex cord-stromal tumours and comprise only
approximately 8% of ovarian neoplasms. A sex cordstromal tumour is composed of: granulosa cells, theca
cells, Sertoli cells, Leydig cells and fibroblasts of
stromal origin, single or in various combinations (3).
Leydig cell tumour is a rare member of this
group that accounts for less than 0.1% of all ovarian
tumours. They are usually benign, unilateral and
more frequently appear in post-menopausal women.
Histologically, they are characterized by the presence
of Reinke crystalloids in the steroid cells, present in
more than 50% of these tumours (4, 5).
These tumours may secrete large amounts
of testosterone that may produce rapidly progressing
androgenic effects manifested by the virilizing
syndrome. Testosterone also stimulates renal
production of erythropoietin and has a direct effect on
erythropoietic stem cells, erythrocytosis being a well
known adverse effect of overtreatment with androgen
hormones, but erythrocytosis due to testosterone over
production from an ovarian Leydig cell tumour was
*Correspondence to: Cristina Mariana Valeria Ghervan MD, University of Medicine and Pharmacy, Endocrinology, 3 Pasteur street, ClujNapoca, Cluj, 400349, Romania, E-mail: cghervan@yahoo.com
Acta Endocrinologica (Buc), vol. XI, no. 3, p. 381-388, 2015
381
C.M.V. Ghervan et al.
very rarely reported (5). Occasionally, these tumours
may exhibit a hyperestrogenic state, determining
vaginal bleeding and/or uterine leiomyoma. Leydig cell
tumours are usually benign, with an excellent prognosis
and reversion of symptoms after surgical treatment (6).
Case report
A 65-year-old post-menopausal woman was
admitted to our service presenting progressive and
rapidly evolving virilizing syndrome: she had a 5-year
history of severe facial and body hirsutism, male-pattern
baldness, voice deepening, seborrhea and sweating.
The last menstrual bleeding was at age of 57 preceded
by one year of irregular menses and succeeded by four
years of hot flushes. Her reproductive history was
uneventful: menarche occurred at 16 years old, with
rhythmic normal menstruation; she had 3 pregnancies,
one spontaneous abortion and 2 normal births.
One year before, she was diagnosed with
erythrocytosis interpreted by the hematologist as
myeloproliferative syndrome (Polycythemia Vera)
requiring repeated phlebotomy and chemotherapy with
Hydroxyurea 1000 mg/day. Five years before she has
been diagnosed with severe hypertension and treated
with an association of four antihypertensive drugs
(beta1 receptor blocker, calcium channel blocker,
diuretic, angiotensin converting enzyme inhibitor) with
poor control of blood pressure values. She was a nonsmoker and consumed no alcohol.
The physical examination showed: severe
hirsutism of the face, chest, abdomen, shoulders and
legs (score 33, Ferriman-Gallwey scale); frontotemporal baldness, plethora, hyperpigmentation of
areole, extended pubic hair, clitoromegaly, male
habitus, well defined hypertrophic muscle masses and
centripetal obesity (Fig. 1). Her blood pressure was
150/100 mmHg with medication.
The hormonal evaluation showed markedly
elevated levels of total testosterone and estradiol,
with suppressed levels of gonadotropins: luteinising
hormone (LH) and follicle stimulating hormone
(FSH), in discordance with the post-menopausal status,
suggesting a secreting neoplasm (Table 1).
Dehydroepiandrosterone sulphate (DHEAS)
level was normal and computed tomography (CT)
of the abdomen showed normal adrenals, excluding
adrenal hypersecretion. Prolactin (PRL) level was
slightly elevated, cortisol level and thyroid function
were in normal ranges (Table 1). As the patient also
Table 1. Hormone levels at diagnosis and two months after surgery:
FSH = Follicle stimulating hormone, LH= Luteinizing hormone,
DHEAS = Dehidroepiandrosterone sulphate, PRL = Prolactine,
TSH = Thyrotropin hormone, FT4 = Free thyroxine, IGF1 = Insuline
growth factor one, GH = Growth hormone
Hormone levels
Figure 1. The clinical appearance of the patient at the moment of
diagnosis.
382
FSH (n.r. 30-150 U/L)
LH (n.r. 8.2-41 U/L)
Testosterone (n.r. 0.2-1 ng/mL)
DHEAS (0.9-3.6 μg/mL)
Estradiol (n.r. 15-60 pg/mL)
PRL (n.r. 1.3-20 ng/mL)
Cortisol (n.r. 5-25 μg/dL)
TSH (n.r. 0,4-4 μUI/mL)
FT4 (n.r. 0,89-1,76 ng/dL)
IGF1 (n.r. 29-204ng/mL)
GH nadir (n.r.< 1 ng/mL)
At diagnosis
0.09
0.16
15.5
2.3
299
27.6
13.2
1.49
1
198.6
0.08
2 months
after surgery
31.1
23.8
0.15
0.6
24.5
Ovarian Leydig cell tumor
Figure 2. CT scan showing enlarged uterus (grey arrow) and
enlarged and inhomogeneous right ovary (white arrow).
Figure 4. Macroscopy: right enlarged ovary (5/3/1.5 cm) with
nodular aspect on section, brown with whitish areas and purple
streaks.
Figure 5. Microscopy (HEX20) of the ovarian tumor: medium-sized
cells with diffuse disposal, finely granular eosinophilic cytoplasm
and lipofuscin deposits, with rounded or slightly oval nucleus,
along with the presence of prominent nucleoli. Some vessels show
parietal fibrinoid deposits.
showed some clinical signs of growth hormone excess
(enlargement of the nose and lips, prognathism, teeth
spacing, hyperhidrosis along with hypertrichosis
Figure 3. CT scan showing bilateral hydronephrosis (white arrows).
and increased sebum production) IGF1 level and
GH suppression during glucose tolerance test were
measured, with normal results, excluding acromegaly
(Table 1). The MRI of the brain focusing on the sella
turcica after gadolinium administration showed a
3.2 mm pituitary microadenoma, which, in the light of
the mentioned hormonal assays, was interpreted as an
incidental non-secreting pituitary adenoma.
The hemoglobin concentration (14.9 g/dL),
hematocrit (42.8%) and red blood cell count of
(3.45x106/mm3) were normalized under hydroxyurea
treatment.
Transvaginal ultrasound revealed a uterus of
20x15 cm with myomatosis pattern and an endometrial
thickness of 11 mm; the right ovary was enlarged (5.5 x
2.8 cm) with inhomogeneous structure. These findings
were confirmed by computer tomography (CT) scan
(Fig. 2). CT scan also revealed a compressive effect of
the uterus on the urinary bladder and on both ureters
with first degree ureterohydronephrosis (Fig. 3).
Urologic exam, including cystoscopy, excluded other
causes of bilateral ureterohydronephrosis.
The final diagnosis being: androgen and
estrogen secreting tumour of the right ovary, the
surgical cure was performed (abdominal hysterectomy
and bilateral salpingo-oophorectomy).
The pathological exam showed an enlarged
uterus with diffuse myometrial hypertrophy (7 cm
maximum thickness) and the presence of an intramural
leiomyoma of 1 cm diameter. The right ovary was
enlarged (5/3/1.5 cm) with nodular aspect on section,
brown with whitish areas and purple streaks (Fig. 4).
The microscopy showed a well-delimited tumoral
proliferation, composed of medium-sized cells with
383
C.M.V. Ghervan et al.
A
B
C
Figure 6. Immunohistochemistry of the ovarian tumor: positive for inhibin (A), vimentin (B) and calretinin (C).
Table 2. The anthropometric and biologic parameters before and
two months after surgery. BMI = Body mass index
Parameter
Before
surgery
Weight (kg)
87
BMI (n.r. 17.5-25 kg/m2)
34.19
Waist (< 80 cm)
102
108
Hips (cm)
Red blood cells count (n.r 4.2-5.4x106) 6.21x106
Hematocrit (n.r. 38-48%)
62.5 %
Hemoglobin (n.r. 12-16 gr/dL
21.2
138*
Total cholesterol (n.r. 170-200 mg/dL)
101*
Triglycerides (n.r. < 150 mg/dL)
89
Glycemia (n.r. 70-99 mg/dL)
2 months
after
surgery
83
32.62
97
110
3.4x106
36.5
12.7
206˟
125 ˟
101
*with 20 mg Atorvastatin
˟ without treatment
diffuse disposal, finely granular eosinophilic cytoplasm
and lipofuscin deposits, with rounded or slightly oval
nucleus, along with the presence of prominent nucleoli
(Fig. 5). Focal nuclear agglomerations were present,
separated by eosinophilic areas and also small areas of
focal hemorrhage and cystic degeneration were found.
Some vessels showed parietal fibrinoid deposits.
Microscopic examination did not reveal Reinke
crystalloids. Immunohistochemistry showed that the
tumour cells were positive for: inhibine, vimentin and
calretinin (Fig. 6); negative for pancytokeratine, AE1/
AE3 and focal positive for alpha–fetoprotein. The
histological and immunohistochemical aspect was
highly suggestive for a Leydig hilus cell tumour, even
in the absence of Reinke crystalloids. The left ovary
(2.5/1.5/0.5 cm) was normal.
Four days after surgery, serum testosterone
and estradiol levels normalized (testosterone = 0.15 ng/
ml, estradiol = 31.5 pg/mL).
Two months after the surgery, the patient
was asymptomatic, normotensive without medication
(130/80 mmHg), showing regression of the virilizing
syndrome (growth of capillary hair and improvement
of hirsutism (score 28 on Ferriman-Gallwey scale).
384
The serum testosterone and DHEA-S were low, LH,
FSH and estradiol have returned to normal levels for a
post-menopausal state (Table 1). AFP level was normal
(1.91 ng/ml, n.r.<7). Total blood count showed mild
megaloblastic anemia, the treatment with hydroxyurea
being interrupted one month before (Table 2).
Before surgery the patient presented also
dyslipidemia, controlled with statins which remitted
after surgery. A slightly increase in glycemia was
noted. Upon follow-up, there was decreased weight
and abdominal circumference (Table 2).
The patient signed an informed consent to the
publication of her case, with data and unidentifying
images.
Discussion
The occurrence of hirsutism accompanied
by signs of virilization such as alopecia, deepening
of the voice, or clitoromegaly, must always raise the
suspicion of an underlying androgen-secreting tumour.
Postmenopausal virilization may result from adrenal
tumours, including androgen-secreting carcinomas and
adenomas or from ovarian tumours, including Sertoli
and/or Leydig cell tumours, granulosa-theca cell
tumours, and hilus-cell tumours; there are also benign
ovarian conditions such as ovarian stromal hyperplasia
and hyperthecosis (7). After menopause ovarian causes
of virilization are more common than adrenal ones (6).
The clinical history is very important – the
absence of the symptoms before menopause excluding
the functional causes of hyperandrogenism such as:
polycystic ovary syndrome, hyperthecosis, or nonclassic
congenital adrenal hyperplasia which appear around
puberty, progress slowly and worsen after menopause.
More severe causes of hyperandrogenism, including
tumours, are rare around puberty, progress rapidly and
associate signs of virilization or defeminization (2, 4,
7-9). In our case the patient was asymptomatic before
menopause, without hirsutism, infertility or menstrual
Ovarian Leydig cell tumor
disturbances, the signs of virilization appearing 5 years
previous, so tumoral etiology was more probable.
There are not established cut-off values
for androgens that direct toward the source of
hyperandrogenism, but a total testosterone above 150–
200 ng/dl and/or DHEAS level above 600 µg/dL may
suggest an androgen-secreting neoplasm (10). The lowdose dexamethasone suppression test has been proposed
to distinguish tumour secretion from non-neoplastic
causes of hyperandrogenism, because both adrenal and
ovarian tumours would fail to suppress androgen levels
after glucocorticoid administration (11, 12). Similarly,
suppression of hyperandrogenism in response to longacting GnRH agonists in postmenopausal women
would be suggestive of gonadotropin dependent
ovarian secretion (13). Concerning the tumour adrenal
source of the androgen excess, rarely, adrenal tumours
are adenomas that secrete mostly testosterone; more
frequently adrenal tumours are carcinomas that often
over secrete not only androgens (mostly DHEA and
DHEA-S), but also cortisol (2, 6, 7, 9, 14). In our
case, the normal DHEAS and cortisol levels and the
normal appearance of adrenals in CT excluded the
adrenal origin of hypersecretion. The high testosterone
and estradiol levels, in the presence of suppressed
gonadotropins, oriented toward an autonomous
secreting ovarian tumour. The high level of estradiol
may be due to estrogen secretion by the tumour, to
peripheral conversion of testosterone, to associated
stromal hyperthecosis or to a combination of these
factors (4-6). Huge uterus hyperplasia compressing
the ureters, leiomyoma, endometrial hyperplasia
and hyperprolactinemia are highly unusual in a postmenopausal woman and were the consequences of
estradiol excess.
CT and magnetic resonance imaging (MRI)
scanning of the adrenal glands are sensitive tests for
confirming the presence of an adrenal adenoma or
adenocarcinoma which is usually greater than 1 cm
in diameter, and in the latter, even greater than 6 cm
in diameter. Androgen-secreting ovarian tumours are
frequently small (< 4 cm) solid masses, with nonspecific ultrasonographic appearances and unilateral
in 95% of cases. These are very difficult to diagnose
by conventional radiological imaging methods like
ultrasound and CT scanning, because of their small
size and in part because they are isoechoic to the
uterus on ultrasound, and isodense on CT. Ultrasound
examination, using a combination of transabdominal
ultrasonography and transvaginal ultrasonography, is
the procedure of choice when imaging ovaries in the
evaluation of hyperandrogenism (2, 5, 6, 8). In our
patient, previous ultrasound examination missed the
enlarged right ovary and even at the time of diagnosis
when the search of an ovarian tumour was obvious, CT
exam first missed the ovarian tumour and focused on
the enlarged and inhomogeneous uterus compressing
the urinary bladder and both ureters, with secondary
ureterohydronephrosis. As the clinical and hormonal
arguments pointed to an ovarian tumour, a second
opinion on the CT exam was demanded, which
confirmed a slightly enlarged right ovary, without
precise tumour image, result that was reproduced by
the transvaginal sonographic examination.
Ovarian and/or adrenal selective venous
sampling was used in some cases to localize the androgen
source, when conventional radiological imaging
methods failed (1, 2, 7), but this exam was not available.
In doubtful cases, in post-menopausal women with
virilization syndrome and elevated testosterone levels,
most of the authors recommend that an oophorectomy
should be considered after the exclusion of adrenal
causes (5, 6). In our case, the decision for surgery was
facilitated by the aspect of the uterus, compressing the
bladder and the ureters, even if, in the absence of the
venous sampling we were not completely convinced of
the ovarian tumour. The intra-operatory tumoral aspect
of the right ovary confirmed our clinical judgment and
justified the bilateral oophorectomy accompanying the
hysterectomy. A conservative surgery with unilateral
salpingo-oophorectomy is justified reasonable only
for premenopausal patients who want to preserve their
fertility (4).
In the rare case of gonadotropin dependent
ovarian androgen-producing tumour, the use of a
gonadotropin releasing hormone analogue is a good
option when surgery is not straightforward because of
high surgical risk (1, 15).
Ovarian androgen secreting tumours are very
rare entities, affecting only 1:300-1000 of patients with
hirsutism. Leydig cell tumours belong to the group of
sex-cord stromal tumours of the ovary, and account for
less than 0.1% of all ovarian tumours (5). They are seen
most often in post-menopausal women and are almost
always benign, with excellent prognosis. They cause
hirsutism and virilization in 75% of cases due to the
clinically significant amounts of androgens produced
(5, 9). This tumour is distinguished from the SertoliLeydig cell tumour by the absence of the follicular
granulosa cells which are homologue of Sertoli cells
in the testis (4). Some ovarian Leydig cell tumours
arise in the hilus from hilar Leydig cells, which can
385
C.M.V. Ghervan et al.
be identified in over 80% of normal adult ovaries.
Other Leydig cells tumours that are located within
the ovarian stroma are referred to as non-hilar Leydig
cell tumours, and the term Leydig cell not otherwise
specified is used when it is impossible to be certain
about the origin (4). These tumours are solid, fleshy
and well circumscribed and appear yellow, orange or
more commonly brownish in color. Histologically they
consist of cells with abundant cytoplasm, eosinophilic
or clear and vacuolated. Reinke crystalloids (RC) are
pathognomonic for the diagnosis of Leydig cell tumour.
They are elongated, hexagonal eosinophilic crystals
present in the cytoplasm or rarely in the nucleus. They
are found in just more than 50% of these tumours but
are irregularly distributed in the tumour and thus may
require extensive searching to locate them (5, 9, 16).
A paper published by Paraschevas M and Al in 1989,
comparing hilus Leydig cell tumours with and without
RC showed that tumours RC negative are secreting
both androgen and estrogen hormones in 44% of cases,
meanwhile, the tumours RC positive are secreting only
androgens (27). Our patient’s tumour was RC negative,
so a cosecretion of both hormones is probable.
Many immunohistochemical markers have
been identified for the differential diagnosis of sex
cord stromal tumours, and inhibin was found to be
particularly useful in differentiating sex cord stromal
from non sex cord stromal tumours. Although some
of these tumours are cytokeratin AE1/AE3 positive,
epithelial membrane antigen (EMA) negativity may
be useful for the differential diagnosis with epithelial
ovarian tumours. Inhibin, a hormonal polypeptide, is
present in ovarian granulosa and lutein cells, as well as
testicular (Sertoli’s) and Leydig cells, and suppresses
the production of pituitary gonadotropins, particularly
FSH. The tumour Leydig cells have negative reactivity
for keratins and show positive immunoreactivity
for vimentin (75% of cases) and for inhibin (91%
of cases) (3, 17-20). In our case the histological and
immunohistochemical aspect was highly suggestive
for a Leydig hilar cell tumour even in the absence
of Reinke crystalloids. There were also tumour cells
focally positive for alfa–fetoprotein, which are rarely
associated with Leydig cell tumours (21). Postoperative follow-up revealed serum AFP level within
normal limits.
Androgen excess stimulates bone marrow
and increases the production of red blood cells and
also stimulates the renal production of erythropoietin.
Secondary erythrocytosis due to androgen excess is
most commonly seen in the context of testosterone
386
replacement therapy in men, but the association of
androgen-secreting tumour and erythrocytosis has been
reported only in a few cases: in a man with gonadotropinsecreting seminoma of the testis (22) and in another
patient with an endometriod tumour (23). In 2011 Yetkin
et al. presented the first case of a Leydig cell ovarian
tumour with an erythropoietic effect of testosterone
excess (5). Saraceno et al., in 2013, reported the case
of a middle-age woman affected by a breast cancer and
by an occult androgen secreting Leydig cell tumour of
the ovary causing erythrocytosis (24). Recently, Pelusi
et al. described two similar cases of polycythaemia in
their group of post-menopausal women with severe
hyperandrogenism caused by Leydig cell tumours (25).
Kozan et al. 2014 presented a post-menopausal woman
with extreme hyperandrogenism, erythrocytosis
and recurrent pulmonary embolism also due to a
Leydig cell tumour of the ovary (26). Our patient was
diagnosed with severe erythrocytosis, interpreted
as mieloproliferative disorder (Polycitemia vera)
one year before the diagnosis of the ovarian tumour,
requiring repeated phlebotomies and chemotherapy
with hydroxyurea. After surgery, the blood parameters
normalized, confirming that the erythrocytosis was
secondary to androgen excess and the diagnosis
of mieloproliferative disorder was erronate, which
significantly improves the survival prognosis and also
the quality of life prognosis for this patient.
Our patient also developed severe hypertension,
poorly controlled with four antihypertensive drugs,
interpreted by the cardiologist as primary hypertension,
which miraculously remitted after the tumour ablation.
Stephen et al. in 1998 reported a case of a steroid cell
ovarian tumour with androgen and renin cosecretion,
presenting with virilization, erythrocytosis and
hypertension (28). In our case, although plasmatic renin
was not measured, a cosecretion is very probable since
blood pressure normalized after the tumour removal
and the antihypertensive medication had to be stopped.
Metabolic abnormalities including insulin
resistance, dyslipidemia and abdominal fat
distribution are common features of women with
hyperandrogenism, particularly those with polycystic
ovary syndrome (PCOS), who are additionally at high
risk of early development of type2 diabetes and possibly
cardiovascular disease. In a recent study of 5 women
with ovarian androgen-secreting tumours, Pelusi et al.
observed that four of the five patients with baseline
normal glucose values had a modest but significant
increase, always in the normal range, in glucose
levels but no significant change in insulin or HOMA-
Ovarian Leydig cell tumor
IR values after surgery. In our patient, similarly to
these patients, a slight increase in basal glycemia was
noted after surgery. In the diabetic patient, values of
hemoglobin A1c decreased by 0.7% and her body mass
index fell from 30.4 kg/m2 to 28.9 kg/m2 indicating
that the metabolic impact of androgen normalization is
different in diabetic patients compared to non-diabetic
patients. Fasting levels of total serum cholesterol,
HDL cholesterol, LDL cholesterol and triglycerides
were within normal limits at baseline and did not show
any significant variation with androgen normalization
after surgery in any subjects. The hypothesis for the
lack of impact of severe hyperandrogenism in women
with ovarian androgen-secreting tumours was that very
high testosterone concentrations may be ineffective
on tissue metabolism, because of profound downregulation of its receptors, which cause a significant
decrease in cellular responsiveness and also by the
timing and duration of tissue exposure to high androgen
levels, that might play an important role in the future
development of metabolic abnormalities (25). In
another case report, Rivera-Arkoncel et al. presented
a patient with a pure Leydig cell ovarian tumour and
metabolic syndrome who presented a decreased weight
and abdominal circumference and improvement in
metabolic parameters after tumour removal (9). Our
patient presented abdominal obesity and dyslipidemia
(controlled with statins). After surgery the lipid profile
improved and the statin treatment was interrupted.
There was also a decrease in weight and in abdominal
circumference (Table 2).
The last particular aspect of this case is the
presence of the dysmorphic acromegalic syndrome and
a pituitary microadenoma image on IRM, concomitant
with normal hormonal results excluding a growth
hormone excess. In this context we can hypothesize
a spontaneous necrosis of a previously functioning
GH secreting adenoma, as reported in few other cases
(29-31), or an incidentaloma. A further surveillance of
IGF1 levels was proposed to the patient.
In conclusion, we present here the very rare
case of a hilus Leydig cell ovarian tumour in a postmenopausal woman, presenting with severe and
rapidly evolving virilizing syndrome and also with
rarely (and for the first time simultaneously) described
complications of the hormonal excess: severe
erythrocytosis, giant compressive uterine fibromatosis,
severe hypertension and dyslipidemia, all remitted after
tumour removal and hormonal normalization.
Conflict of interest
The authors declare that they have no conflict of
interest concerning this article.
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