Author information
Kumar M*, Mali KA**, Warke HS***
(* Junior Resident, ** Assistant Professor, *** Associate Professor, Department of Gynecology and Obstetrics, Seth GS Medical College and KEM Hospital, Mumbai, India.)
Abstract
Pheochromocytoma is a tumor of the chromaffin cells of the adrenal gland. Due to associated high maternal morbidity, it warrants early detection and proper treatment. The successful pregnancy outcome in a patient who had been operated for pheochromocytoma in the past is described here.
Introduction
Pheochromocytoma is rare during pregnancy. It occurs with a frequency of 2-7 per one lakh pregnant women. Clinical manifestations include hypertension, sometimes resembling preeclampsia. Biochemical measurements of catecholamines and their metabolites is necessary to establish the diagnosis during pregnancy.
Case Report
A 32 year old primigravida was referred at 35 weeks of gestation to the emergency department at our tertiary care centre with severe oligohydramnios and a loop of cord around the neck. There were no complaints of pain in abdomen/ leaking or bleeding per vaginum. She was perceiving good fetal movements.
Eight years before, she had undergone an open abdominal procedure for pheochromocytoma. The histopathology report was suggestive of a 6.5 x 5 x 3 cm pheochromocytoma with central infarction. However, she had not been following up with a physician or endocrinologist for this condition in the recent past. She was admitted for evaluation.
She had been registered at a private hospital where all antenatal investigations were found to be normal except deranged blood sugars. For an oral glucose tolerance test with values 132 gm %, 165 gm %, 170 gm % and 165 gm %, she had been started on diabetic diet at around 26 weeks of gestation.
Upon admission, her blood pressure was 120/ 80 mm Hg, and there was no albuminuria or any features suggestive of preeclampsia. Uterus was corresponding to weeks of gestation and fetus was live in vertex presentation.
Haemoglobin was 10.9 gm %, platelets 1,87,000/mm3 and white blood cell count was 11,000/mm3. All other investigations were normal. Non stress test was reactive. Ultrasonography showed 35 weeks of gestation with amniotic fluid index of 1 cm with normal doppler flow studies. Fetal survey showed mild left pelvicalyceal system fullness.
Endocrinologist opinion was taken in view of deranged sugars in a previously operated case of pheochromocytoma. She was started on tablet amlodipine 5 mg once daily due to the possibility of occurrence of hypertension. Blood glucose level monitoring was advised and due to raised sugar levels, tablet metformin 500 mg per day was started initially which was further increased to 500 mg twice a day. Due to persistent raised blood sugar values, injection insulin (regular) 2-0-0-2 units and insulin (normal) 4-0-0-4 units was started which was further increased to regular 4-0-0-4 units and 8-0-0-8 units.
Ultrasound abdomen and pelvis showed no remnants of pheochromocytoma. Plasma free normetanephrine levels were done which was within normal limits and any possibility of remnants of pheochromocytoma was ruled out. NST done biweekly was reactive and weekly doppler revealed increasing amniotic fluid with normal doppler flows.
Endocrinologists advised cesarean section to be the mode of delivery and to avoid a vaginal trial of labor so as to prevent hypertensive crisis, as a possibility of a small residual tumor had not been ruled out since imaging could not be done due to gravid state. She underwent elective lower segment cesarean section at 38 weeks of gestation under epidural anaesthesia. She delivered a male baby of 2.93 kg with Apgar score of 9/ 10. The procedure was uneventful. Intraoperatively, blood pressure remained normal. In the postoperative period her blood sugar monitoring was done and gradually insulin and metformin were stopped. She had an uneventful postoperative course and was discharged on day 10. Amlodipine was continued and she was asked to follow up with endocrinologist.
Discussion
Pheochromocytoma is a rare tumor of the chromaffin cells that cause increased levels of catecholamines and is known to increase morbidity and mortality. Maternal and fetal survival depends a lot on an early diagnosis, appropriate medical therapy and correct timing of surgery and delivery. Despite being an uncommon condition recognising it timely in a pregnant hypertensive woman is extremely important. For the biochemical diagnosis, plasma or urine metanephrines are test of first choice.[1] For reliable diagnosis magnetic resonance imaging is the investigation of choice with sensitivity of 90 %.[2]
If a tumor is diagnosed in the first 24 weeks of gestation it should be treated by laparoscopic adrenalectomy after 10-14 days of medical therapy similar to that in the nonpregnant state. Preoperatively alpha and beta blockers need to be administered to control blood pressure and to prevent intraoperative crises.
If the tumor is diagnosed in the third trimester the patient should be managed until the fetus is viable using the same drug regimen. Cesarean section is preferred over vaginal birth which is possibly associated with higher mortality. Our patient underwent a cesarean section, as advised by the endocrinologists, though she was an operated case of pheochromocytoma in the past.
Some patients with pheochromocytoma have unknown genetic predisposition and are asymptomatic. Approximately 20% cases remain undiagnosed until labor.[3] The high levels of maternal mortality are related to complications of a hypertensive crises like stroke, cardiac arrest, pulmonary oedema. Fetal morbidity may be due to placental abruption, fetal hypoxia and intrauterine growth restriction.
The classical triad of symptoms for pheochromocytoma consist of headache, palpitation and sweating.[4] Other symptoms such as flushing and tremors may be present. Sudden occurrence of symptoms, significant variations in blood pressure, and orthostatic hypotension are features. Our patient was asymptomatic during her pregnancy, and her blood pressure remained normal.
Plasma and urinary catecholamines are not increased in normal pregnancies and can be used as a screening test.[5] In our case it was used as a test to look for recurrence, which was normal. Even in pre-eclampsia, plasma catecholamines are only slightly elevated. Maternal catecholamines do not cross the placental barrier.[6]
Being a rare condition, there are no prospective studies regarding mode of delivery in pheochromocytoma. Vaginal deliveries appear to have a higher maternal mortality (31%) than cesarean section (19%) mainly due to hypertensive crisis. Therefore, cesarean section has been recommended as the preferred mode of delivery.[7] Similar advise was given by our endocrinologists and our patient underwent cesarean section, under epidural anesthesia.
She was advised to follow up with endocrinologists regularly. Patients with pheochromocytoma are at risk of developing tumor recurrences or new tumors, even after successful resection of primary tumor.[8] Regular monitoring of plasma metanephrines is the main method of follow-up, which our patient was not undergoing regularly, but underwent upon admission during this pregnancy. Since the disease is mostly sporadic, no specific follow up of the neonate is required.
Conclusion
To conclude, pregnancy with a pheochromocytoma is a rare but dangerous combination. The prognosis of pregnant patients with a pheochromocytoma has improved and appropriate management at a tertiary care centre with a multidisciplinary approach involving expert obstetricians, endocrinologists and anesthetists is preferred.
References
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- Zuspan FP. Urinary excretion of epinephrine and norepinephrine during pregnancy. Journal of clinical endocrinology and Metabolism; 1970; 30(3): 357-360
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- Sarathi V, Lila AR, Bandgar TR, Menon PS, Shah NS. Pheochromocytoma and pregnancy: a rare but dangerous combination. Endocrine practice. 2010; 16(2): 300-9
- Amar L, Fassnacht M, Gimenez-Roqueplo AP, Januszewicz A, Prejbisz A, Timmers H. Long-term postoperative follow-up in patients with apparently benign pheochromocytoma and paraganglioma. Horm Metab Res.2012 May;4(5):385-9.
Citation
Kumar M, Mali KA, Warke HS. Pregnancy In An Operated Case Of Pheochromocytoma. JPGO 2018. Volume 5 No.4. Available from:http://www.jpgo.org/2018/04/pregnancy-in-operated-case-of.html