Feocromocitomul în sarcină: prezentare de caz şi review privind dificultățile de management

Introduction

Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla, characterized by excessive secretion of catecholamines – primarily epinephrine and norepinephrine⁽¹⁾. These tumors are found in approximately 0.1-0.6% of patients with hypertension, but occur in only 0.002-0.007% of all pregnancies⁽²⁾. The clinical presentation of pheochromocytoma often mimics more common conditions such as preeclampsia, hyperthyroidism or panic disorder, especially during pregnancy, leading to diagnostic delays⁽³⁾. The diagnosis relies on biochemical confirmation through measurement of plasma or urinary fractionated metanephrines, followed by localization using cross-sectional imaging (MRI or CT)⁽⁴⁾. Surgical resection remains the definitive treatment. The optimal timing of surgery depends on gestational age and maternal stability. Preoperative alpha-adrenergic blockade is essential to prevent intraoperative hypertensive surges⁽⁵⁾. With timely diagnosis and appropriate management, maternal and fetal outcomes have improved significantly in recent decades. This case highlights the challenges in diagnosing and managing pheochromocytoma in early pregnancy, and emphasizes the importance of a structured, multidisciplinary approach.

Case presentation

A 36-year-old primigravid woman presented at 7 weeks of gestation with episodic headaches, palpitations, diaphoresis and intermittent nausea. She had no prior medical or surgical history, and she was not taking any medications. Her menstrual history revealed amenorrhea of six weeks’ duration. On physical examination, her blood pressure fluctuated between 130/70 mmHg and 190/120 mmHg, and her heart rate ranged from 110 to 120 bpm during symptomatic episodes. There was no peripheral edema, thyromegaly or abdominal tenderness, and cardiovascular, respiratory and neurological exams were otherwise unremarkable. Although the initial suspicion was preeclampsia due to labile hypertension and tachycardia, the absence of proteinuria or other systemic signs prompted further investigation into secondary causes of hypertension. Laboratory investigations revealed microcytic anemia (hemoglobin: 8.4 g/dL), normal renal and liver function and a random blood glucose level of 82 mg/dL. Plasma free metanephrines were measured, showing elevated levels of free normetanephrine at 1160 ng/L (reference range: 20.10-135.40) and methoxytyramine at 24.7 ng/L (<18.40), with free metanephrine within normal limits, at 56.1 ng/L (7.90-88.70), as presented in Table 1. These findings strongly supported the diagnosis of pheochromocytoma. Imaging modalities included ultrasound, which revealed a hypoechoic lesion measuring 53×42×32 mm in the right suprarenal region and confirmed a single intrauterine pregnancy of 6+5 weeks. MRI further confirmed a right adrenal mass measuring 51×35×52 mm with signal characteristics consistent with pheochromocytoma, as presented in Figure 1. Following multidisciplinary consultation involving obstetrics, endocrinology, urology and anesthesia, it was decided to proceed with surgical resection after adequate preoperative preparation. The patient was started on alpha-blockade with prazosin 10 mg TID, followed by the addition of beta-blockade with labetalol to control tachycardia. She was closely monitored for maternal vital signs and fetal well-being. After two weeks of medical optimization, she underwent laparoscopic right adrenalectomy at 12 weeks and 5 days of gestation, under general anesthesia, with invasive hemodynamic monitoring. The specimen is presented in Figure 2. Histopathological findings were suggestive of pheochromocytoma, as shown in Figure 3. Intraoperative blood pressure fluctuations were managed with intravenous antihypertensives and vasopressors, while uterine perfusion and fetal oxygenation were maintained throughout the procedure. The estimated blood loss was minimal, and the operative time was approximately 120 minutes. She was observed in the intensive care unit (ICU) for 24 hours postoperatively, and she had an uncomplicated recovery, with normalization of blood pressure. Fetal heart activity was confirmed postoperatively by ultrasound. She was discharged on the sixth postoperative day, with continuation of labetalol and a gradual tapering of prazosin. Regular antenatal follow-up showed normal fetal growth and development, and she ultimately delivered a healthy term baby by spontaneous vaginal delivery, without complications.

Discussion

Pheochromocytoma is a rare but potentially life-threatening catecholamine-secreting tumor, most commonly arising from the adrenal medulla. It presents with a classic triad of symptoms – paroxysmal headache, diaphoresis and palpitations, although this triad may not always be present, making diagnosis challenging, especially in pregnancy⁽⁶⁾. In this case, the patient’s episodic headaches, palpitations, labile hypertension and tachycardia initially raised the suspicion for preeclampsia. However, the absence of proteinuria or other systemic signs prompted further investigation into secondary causes of hypertension. During pregnancy, similar symp­toms can also be seen in thyrotoxicosis, anxiety dis­orders and cardiac arrhythmias. Key distinguishing features of pheochromocytoma include paroxysmal rather than sustained hypertension, elevated plasma me­ta­ne­phrines, and the absence of biochemical markers such as hepatic dysfunction or proteinuria. The diagnosis of pheo­chro­mo­cy­toma during pregnancy is particularly cri­ti­cal, due to its association with high maternal and fe­tal morbidity and mortality. Untreated, it carries a ma­ter­nal mortality rate of up to 29% and fetal mortality as high as 55%⁽⁷⁾. Maternal complications include hypertensive crisis, arrhythmias, pulmonary edema and acute coronary syndrome, while fetal risks stem largely from uteroplacental vasoconstriction and include spontaneous abortion, intrauterine growth restriction, preterm labor and fetal hypoxia. Early diagnosis and timely intervention are essential for improving outcomes, reducing maternal mortality to less than 1% and ensuring fetal well-being. In this case, laboratory evaluation revealed significantly elevated levels of plasma free normetanephrine (1160 ng/L) and methoxy­tyramine (24.7 ng/L), with normal renal and liver function tests and microcytic anemia. These findings, along with imaging confirmation by ultrasound and MRI showing a right adrenal mass consistent with pheochromocytoma, led to a definitive diagnosis. The presence of an intrauterine pregnancy at 6+5 weeks necessitated a multidisciplinary approach involving obstetrics, endocrinology, urology and anesthesia, in order to optimize both maternal and fetal safety⁽⁸⁾. The management of pheochromocytoma during pregnancy involves careful preoperative medical preparation, appropriate timing of surgery and meticulous intraoperative hemodynamic control. Alpha-blockade remains the cornerstone of preoperative management, with agents such as prazosin used to control blood pressure and prevent hypertensive crises⁽⁹⁾. Once alpha-blockade is established, beta-blockers such as labetalol can be added to manage tachycardia. Intravenous hydration is also crucial to expand intravascular volume and mitigate the risk of post-resection hypotension. The optimal timing for surgical resection is between 12 and 24 weeks of gestation, as this period minimizes the mechanical challenges posed by the enlarging uterus later in pregnancy while allowing sufficient time for maternal stabilization⁽¹⁰⁾. In our case, laparoscopic right adrenalectomy was successfully performed at 12 weeks and 5 days, under general anesthesia, with invasive hemodynamic monitoring. Intraoperative blood pressure fluctuations were effectively managed with intravenous antihypertensives and vasopressors, while maintaining adequate uteroplacental perfusion throughout the procedure. Laparoscopic adrenalectomy was favored over open surgery, due to its lower morbidity, faster recovery time and better fetal outcomes. Postoperatively, the patient had an uneventful recovery with normalization of blood pressure and confirmed fetal viability. She was discharged on the sixth postoperative day, with continued labetalol and a gradual taper of prazosin. Regular antenatal follow-up demonstrated normal fetal growth and development, culminating in a healthy term delivery via spontaneous vaginal delivery, without complications. This case underscores the importance of considering secondary causes of hypertension in pregnant women presenting with atypical features. A high index of suspicion, timely biochemical testing and prompt multidisciplinary intervention are crucial in managing pheochromocytoma during pregnancy to ensure favorable maternal and fetal outcomes⁽¹¹⁾. The key management steps are shown in Table 2.

Conclusions

Pheochromocytoma in pregnancy remains a rare but potentially life-threatening condition. Due to its nonspecific presentation, it is often misdiagnosed as preeclampsia or other common hypertensive disorders of pregnancy. A high index of suspicion, prompt biochemical evaluation and timely imaging are essential for early diagnosis.

Optimal outcomes depend on a multidisciplinary approach involving obstetricians, endocrinologists, surgeons and anesthesiologists. Medical stabilization, followed by laparoscopic adrenalectomy in the second trimester, is associated with excellent maternal and fetal outcomes.

This case underscores the importance of integrating clinical acumen, biochemical testing and advanced imaging to ensure timely diagnosis and safe management of pheochromocytoma in pregnancy.

Declaration. Written informed consent was obtained from the patient for the publication of this case report and any accompanying images.

Consent. Written informed consent was obtained from the patient for the publication of this case report and any accompanying images.

Data availability. All data supporting the findings are included within the article.

Competing interests. The authors declare no competing interests.

Acknowledgments. We thank the patient for her cooperation and the multidisciplinary team involved in her care for their support and expertise.

Autor corespondent: Smit Bharat Solanki E-mail: drsmitbharat@gmail.com

CONFLICT OF INTEREST: none declared.

FINANCIAL SUPPORT: none declared.

This work is permanently accessible online free of charge and published under the CC-BY.