Adrenal Mass in Pregnancy: Diagnostic Approach andDilemmas

Case Report

Adrenal mass in pregnancy: Diagnostic approach anddilemmas

K. Swaminathan a,*, A. SureshKumar b, K. UshaRani c, R. Hemaleka d

aConsultant Endocrinologist, Department of Endocrinology, Apollo Speciality Hospital, Madurai 625 020, IndiabConsultant Laparoscopic Surgeon, Department of Laparoscopic Surgery, Apollo Speciality Hospital, Madurai, IndiacConsultant Pathologist, Department of Pathology, Apollo Speciality Hospital, Madurai, IndiadConsultant 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).

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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 cmheterogeneousmass 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 themother and the baby. Ourmain

concernwith this patient’s adrenalmasswas the possibility of

a phaeochromocytoma complicating pregnancy, especially in

view of her Type 1 neurofibromatosis. Traditionally, the

prevalence of this associationwas 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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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 continuewith 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 themulti-disciplinary teammutually 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

Wewould 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

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5. Zinnamosca L, Petramala L, Cotesta D, et al.Neurofibromatosis type 1 (NF1) and pheochromocytoma:prevalence, clinical and cardiovascular aspects. Arch DermatolRes. 2011 Jul;303(5):317e325.

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7. Mantero F, Terzolo M, Arnaldi G, et al. A survey on adrenalincidentaloma in Italy. Study Group on Adrenal Tumors of theItalian Society of Endocrinology. J Clin Endocrinol Metab.2000;85(2):637.

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