An adrenal incidentaloma is a mass lesion greater than 1 cm in diameter, incidentally found during radiologic examination for other reasons. 1. Such “adrenal incidentalomas” are increasingly recognised in clinical practice. 2. This is attributed to routine use of sophisticated and sensitive imaging techniques, with a reported prevalence of 4.4%. 3. Incidental findings of such masses pose dilemmas in evaluation and management, as current recommendations based on expert opinion. 4. Are open to debate in terms of cost and clinical benefits. The uncertainties in management multiply with such adrenal incidentalomas in the context of pregnancy. We report a rare case of a large adrenal incidentaloma complicating second trimester of pregnancy. This case outlines the huge decisional dilemmas, both for the patient and healthcare provider.

1. Adrenal Mass in Pregnancy: Diagnostic Approach and
Dilemmas

2. Case Report
Adrenal mass in pregnancy: Diagnostic approach and
dilemmas
K. Swaminathan a,
*, A. SureshKumar b
, K. UshaRani c
, R. Hemaleka d
a
Consultant Endocrinologist, Department of Endocrinology, Apollo Speciality Hospital, Madurai 625 020, India
b
Consultant Laparoscopic Surgeon, Department of Laparoscopic Surgery, Apollo Speciality Hospital, Madurai, India
c
Consultant Pathologist, Department of Pathology, Apollo Speciality Hospital, Madurai, India
d
Consultant Obstetrician & Gynaecologist, Department of Obstetrics & Gynaecology, Apollo Speciality Hospital, Madurai, India
a r t i c l e i n f o
Article history:
Received 13 April 2013
Accepted 15 May 2013
Keywords:
Adrenal incidentalomas
Pregnancy
Ganglioneuromas
a b s t r a c t
Adrenal incidentalomas refer to incidentally discovered adrenal masses during radiological
examination for other reasons. Such findings have only been rarely reported in pregnancy
due to predominant use of ultrasound rather than Computed Tomography (CT) in a
pregnancy setting. The main concerns of such incidentally discovered adrenal masses in
pregnancy are whether they are functioning or malignant. Evaluation of functionality
presents its own set of challenges due to physiological changes that occur during preg-
nancy. Any adrenal mass greater than 4 cm raises the suspicion of malignancy as well. We
report a rare case of a 7 cm left adrenal incidentaloma in pregnancy and the associated
dilemmas in management of such a presentation.
Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved.
1. Introduction
An adrenal incidentaloma is a mass lesion greater than 1 cm
in diameter, incidentally found during radiologic examination
for other reasons.1
Such “adrenal incidentalomas” are
increasingly recognised in clinical practice.2
This is attributed
to routine use of sophisticated and sensitive imaging tech-
niques, with a reported prevalence of 4.4%.3
Incidental find-
ings of such masses pose dilemmas in evaluation and
management, as current recommendations based on expert
opinion4
are open to debate in terms of cost and clinical
benefits. The uncertainties in management multiply with
such adrenal incidentalomas in the context of pregnancy.
We report a rare case of a large adrenal incidentaloma
complicating second trimester of pregnancy. This case
outlines the huge decisional dilemmas, both for the patient
and healthcare provider.
2. Case report
A 26-year-old lady presented to us with an incidental finding
of a 7 cm left adrenal mass during a routine ultrasound ex-
amination for pregnancy. She was 20 weeks gestation in her
first pregnancy, with no significant past medical or surgical
history. She had long standing multiple neurofibromas,
consistent with Type 1 neurofibromatosis. There were no
clinical symptoms or signs suggestive of a phaeochromocy-
toma or Cushing’s syndrome. Blood pressure showed normal
recordings (110e120 mm Hg systolic and 70e80 mm Hg
* Corresponding author. Tel.: þ91 (0) 8526421150; fax: þ91 (0) 452 2580199.
E-mail address: k_swaminathan@hotmail.com (K. Swaminathan).
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/apme
a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 1 5 9 e1 6 1
0976-0016/$ e see front matter Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.apme.2013.05.012

3. diastolic) on multiple occasions. Electrocardiogram and
Echocardiography showed normal left ventricular function
with no evidence of left ventricular hypertrophy. Fundi
showed no evidence of hypertensive retinopathy. Electrolytes,
especially potassium was within normal limits. Multiple uri-
nary 24 h urinary fractionated metanephrines showed levels
to be within normal limits. MRI (Magnetic Resonance Imaging)
of the abdomen showed a 7 cm heterogeneous mass in the left
adrenal gland with calcifications.
In view of the size of the tumour, lack of a tissue diagnosis,
the concern about malignancy and the possibility of a func-
tional tumour with the background of Type 1 neurofibroma-
tosis, a detailed discussion was done with the patient, her
family and the obstetrician. Both the patient and the multi-
disciplinary team, after much deliberations, were keen for
the tumour to be removed during the second trimester,
accepting the pros and cons of surgery during this stage of
pregnancy. The tumour was dissected through a laparoscopic
approach under general anaesthesia at 22 weeks gestation.
Macroscopic appearances showed a large encapsulated
whitish grey mass (Fig. 1). Microscopic examination showed
spindle shaped cells with mature ganglion cells consistent
with a diagnosis of ganglioneuroma (Fig. 2). Post-operative
recovery was uncomplicated and the patient delivered a
healthy male baby at 38 weeks gestation.
3. Discussion
Adrenal disorders complicating pregnancy are relatively rare
but a timely diagnosis and management plan is essential as
many of these disorders can be associated with significant
maternal and foetal morbidity.
Any adrenal incidentalomas raises two important ques-
tions a) whether the mass is functioning b) whether the mass
is malignant. This assumes great significance during preg-
nancy in terms of decision to operate as implications can be
potentially devastating for the mother and the baby. Our main
concern with this patient’s adrenal mass was the possibility of
a phaeochromocytoma complicating pregnancy, especially in
view of her Type 1 neurofibromatosis. Traditionally, the
prevalence of this association was thought to be in the order of
1% but recent studies have shown that the prevalence of
phaeochromocytoma in type 1 neurofibromatosis may be as
high as 15%.5
Phaeochromocytoma complicating pregnancy is a poten-
tially dangerous condition for both the mother and the
foetus. In a systematic review of 77 pregnancies complicated
by phaechromocytomas, foetal and maternal mortality rates
were 17% and 8% respectively. Survival of both the mother
and foetus were much better when the diagnosis was made
in the ante-natal period than during labour of immediate
postpartum.6
Extensive testing failed to reveal features
consistent with a phaeochromocytoma in our patient.
Screening for Cushing’s syndrome was not done due to cost
implications, normal blood pressure and lack of clinical
features.
The other concern with our patient was the possibility of
malignancy, considering the size of the mass. The two major
predictors of malignancy in an adrenal incidentaloma are the
size of the mass and imaging characteristics. In a large Italian
study of 1004 patients with adrenal incidentaloma, a mass
size of 4 cm had the highest sensitivity in differentiating
benign from malignant tumours, although the specificity was
low.7
In a Mayo series, all the adrenal carcinomas were be-
tween 4 and 6 cm in diameter. Therefore, an adrenal mass of
7 cm diameter in our patient raised the genuine possibility of
malignancy. However, adrenal mass size alone should not be
used as a predictor to make treatment decisions. Imaging
characteristics have a strong predictive value to guide man-
agement options. A CT scan could not be carried out in our
patient in view of her pregnancy. The well circumscribed
nature of the mass along with calcifications did raise the
possibility of a benign adrenal ganglioneuroma in our pa-
tient. However, imaging studies may be misleading as both
the benign (ganglioneuromas) and the malignant (ganglio-
neuroblastomas) forms of the tumour are identical
radiologically.
Ganglioneuromas are large slow growing tumours that
arise from primitive sympathetic ganglion cells. They tend to
occur more commonly in females with common locations
being in the adrenal glands and the retroperitoneum. There
have been occasional case reports of pelvic and retroperito-
neal ganglioneuromas complicating pregnancy with features
of obstructed labour.8,9
Adrenal ganglioneuromas compli-
cating pregnancy is extremely rare with a single case report inFig. 1 e Macroscopic specimen of the large adrenal mass.
Fig. 2 e Microscopic appearances showing spindle shaped
cells with mature ganglion cells (black arrow).
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4. 1989.10
The patient in this report had undergone a successful
second trimester right adrenalectomy. To the best of our
knowledge, our case report is the second case of adrenal
ganglioneuroma complicating pregnancy in the literature.
It is always a challenge to both the families and the
healthcare professionals to decide on the timing of surgery in
patients with such large adrenal masses. This largely depends
on the gestational age at presentation, size and position of the
tumour, functionality and the possibility of malignancy. The
dilemmas in our case were multi-fold. The presence of Type 1
neurofibromatosis with a large adrenal mass strongly raised
the suspicion of a phaeochromocytoma. It was imperative to
exclude a phaeochromocytoma as the complications of a
poorly prepared patient on the operating table can be devas-
tating. The next dilemma was to decide whether to intervene
with surgery during the second trimester or to continue with a
conservative line of management till delivery. While the MRI
features were suggestive of a benign ganglioneuroma, the size
of the mass was concerning. As discussed before, radiology
cannot differentiate a benign ganglioneuroma from a malig-
nant ganglioneuroblastoma. In the absence of firm evidence,
lack of tissue diagnosis and the possibility of malignancy, the
patient and the multi-disciplinary team mutually agreed to go
ahead with surgery with high-risk consent. We acknowledge
that a decision for “watchful waiting” may have also been
equally reasonable.
To summarise, we report a rare case of a large adrenal
ganglioneuroma complicating pregnancy, on a background of
generalised neurofibromatosis. This case highlights the chal-
lenges of evaluating such patients and the decisional di-
lemmas in management.
Conflicts of interest
All authors have none to declare.
Acknowledgements
We would like to acknowledge Dr. Ramesh Babu and Dr. Abdul
Khader for their help with this patient’s management.
r e f e r e n c e s
1. Young Jr WF. Clinical practice. The incidentally discovered
adrenal mass. N Engl J Med. 2007;356(6):601.
2. Aron DC. The adrenal incidentaloma: disease of modern
technology and public health problem. Rev Endocr Metab
Disord. 2001 Aug;2(3):335e342.
3. Bovio S, Cataldi A, Reimondo G, et al. Prevalence of adrenal
incidentaloma in a contemporary computerized tomography
series. J Endocrinol Invest. 2006 Apr;29(4):298e302.
4. NIH state-of-the-science statement on management of
the clinically inapparent adrenal mass (“incidentaloma”).
NIH Consens State Sci Statements. 2002 Feb 4e6;19(2):1e25.
5. Zinnamosca L, Petramala L, Cotesta D, et al.
Neurofibromatosis type 1 (NF1) and pheochromocytoma:
prevalence, clinical and cardiovascular aspects. Arch Dermatol
Res. 2011 Jul;303(5):317e325.
6. Biggar MA, Lennard TW. Systematic review of
phaeochromocytoma in pregnancy. Br J Surg. 2013
Jan;100(2):182e190.
7. Mantero F, Terzolo M, Arnaldi G, et al. A survey on adrenal
incidentaloma in Italy. Study Group on Adrenal Tumors of the
Italian Society of Endocrinology. J Clin Endocrinol Metab.
2000;85(2):637.
8. Kamin G, Theissig F, Lo¨ffler F, Lu¨ ckert G. Retroperitoneal
ganglioneuroma as a cause of obstructed labor. Zentralbl
Gynakol. 1986;108(16):1003e1005.
9. Kurzel RB, Durso N. Pelvic ganglioneuroma during pregnancy.
A case report.
10. Marks F, Young BK, Raghavendra BN, et al. Diagnosis of
adrenal ganglioneuroma in pregnancy with magnetic
resonance imaging and ultrasonography. A case report. J
Reprod Med. 1989 Jan;34(1):59e61.
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