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.