METHODS AND RESULTS
Our patient was a 20-year-old lady who had initially presented to the
medicine outpatient services at an outside hospital in September 2019
for recurrent episodes of palpitations, headaches and abdominal pain. On
imaging she had been detected to have bilateral adrenal masses. Due to
the high prevalence of tubercular adrenalitis in India, she had been
given a trial of empirical anti-tubercular therapy, which had been
unsuccessful and she was subsequently referred to the Medicine
department of our institute for further evaluation. Review of her
clinical history and imaging led us to suspect the possibility of a
pheochromocytoma which was confirmed by the elevated catecholamine
levels noted in her urine along radiological evidence with 68Ga-DOTANOC
PET/CT
[68Ga-Labeled(1,4,7,10-tetraazacyclododecane-N,N’,N”,N”’-tetraaceticacid)-1-NaI3-octreotide-
positron emission tomography/computed tomography] which were
consistent with bilateral pheochromocytoma (Table S1 and Figure 1, S2).
Considering the occurrence of bilateral pheochromocytoma at a young age
(seen in only 10% cases), the possibility of MEN syndrome was kept, and
further evaluation was performed. Ultrasound (USG) neck revealed
bilateral thyroid nodules and parathyroid adenoma. Fine needle
aspiration cytology (FNAC) of the thyroid nodules revealed MTC thus
completing the classical triad of MEN 2A syndrome. In light of these
findings, the patient’s family history was reviewed but was
noncontributory.
Our patient was a 20-year-old lady who had initially presented to the
medicine outpatient services at an outside hospital in September 2019
for recurrent episodes of palpitations, headaches and abdominal pain. On
imaging she had been detected to have bilateral adrenal masses. Due to
the high prevalence of tubercular adrenalitis in India, she had been
given a trial of empirical anti-tubercular therapy, which had been
unsuccessful and she was subsequently referred to the Medicine
department of our institute for further evaluation. Review of her
clinical history and imaging led us to suspect the possibility of a
pheochromocytoma which was confirmed by the elevated catecholamine
levels noted in her urine along radiological evidence with 68Ga-DOTANOC
PET/CT
[68Ga-Labeled(1,4,7,10-tetraazacyclododecane-N,N’,N”,N”’-tetraaceticacid)-1-NaI3-octreotide-
positron emission tomography/computed tomography] which were
consistent with bilateral pheochromocytoma (Table 1 and Figure 1-2).
Considering the occurrence of bilateral pheochromocytoma at a young age
(seen in only 10% cases), the possibility of MEN syndrome was kept, and
further evaluation was performed. Ultrasound (USG) neck revealed
bilateral thyroid nodules and parathyroid adenoma. Fine needle
aspiration cytology (FNAC) of the thyroid nodules revealed MTC thus
completing the classical triad of MEN 2A syndrome. In light of these
findings, the patient’s family history was reviewed but was non
contributory.
The patient was reviewed by the surgery and endocrinology services at
our centre and was planned for bilateral adrenalectomy followed by total
thyroidectomy for definitive management of her condition in two
sittings. The patient underwent a laparoscopic transperitoneal
adrenalectomy with a plan to perform a thyroidectomy for MTC in the next
sitting. Histopathological examination of the adrenal glands confirmed
the diagnosis of pheochromocytoma. However, India entered a nationwide
lockdown from 24th March 2020 in view of the COVID-19
pandemic and massive reorganization occurred in the government
healthcare system. Outpatient services were restricted and largely
replaced by teleconsultation services were offered to outpatients. All
elective surgeries were deferred and only emergency procedures were
being performed with a large number of physicians being diverted to
centres dedicated to the management of the burgeoning load of patients
afflicted with COVID-19 pneumonia.[7] Considering the exceptional
situation at hand the patient was discharged and asked to follow up
through teleconsultation for her second surgery. She was kept on
glucocorticoids (Tablet Prednisolone 5 mg once a day) and
mineralocorticoids (Tablet Fludrocortisone 100 mcg once a day). However,
she conceived in her subsequent menstrual cycle in April 2020. This
posed a unique challenge of delivering appropriate antenatal care and
following up with the patient closely to optimize her for surgery, which
was now deferred to the second trimester of her pregnancy. These
services were provided by a multidisciplinary team comprising her
treating obstetricians, endocrinologists and surgeons. Her first
trimester was uneventful and she successfully underwent a near total
thyroidectomy with level IV lymph node dissection and right inferior
parathyroid adenectomy in October 2020 at 24 weeks period of gestation
(POG). The histopathology revealed bilateral medullary thyroid carcinoma
each measuring 0.5 x 0.5 x 0.3 cm with lympho-vascular invasion and no
lymph node involvement along with right parathyroid adenoma (1 x 0.5 x
0.5 cm). Post-operative period was uneventful and she was discharged on
thyroid replacement therapy (Tab Thyroxine sodium 75 mcg/day).
Coincidentally there was a gradual downregulation of travel restrictions
at this time which made it easier for the patient to access healthcare
services provided at our centre. Unfortunately, in the postoperative
period she developed a mild COVID-19 infection at 27 weeks and was
admitted at our COVID facility, where close maternal and fetal
surveillance was done by a dedicated obstetric team. Her recovery was
uneventful and she continued to follow up with the treating team post
COVID through teleconsultation. She sought confinement at 36 weeks of
gestation with pre-eclampsia without severe features, intrahepatic
cholestasis of pregnancy (ICP) and late onset fetal growth restriction
and a decision was taken to terminate her pregnancy by cesarean section
at 37 weeks of gestation due to fetal bradycardia. She delivered a
healthy female child weighing 2380 grams ( appropriate for gestational
age) with an APGAR score of 9, 9. She received an intravenous stress
dose of hydrocortisone during peripartum period.