Pheochromocytoma In Pregnancy Research Articles

PP-04 Pheochromocytoma in pregnancy

PP-04 Pheochromocytoma in pregnancy

PP-04 Pheochromocytoma in pregnancy

PP-04 Pheochromocytoma in pregnancy

Pheochromocytoma in pregnancy: a case report.

Pheochromocytoma (PHEO) in pregnancy is a rare disease, and the management of this situation is not well established. The misdiagnosis of the disease often leads to adverse outcomes for both mothers and infants. Here, we describe a case of a pregnant woman at 25 weeks' gestation presenting with headache, chest tightness, and shortness of breath, which was found to have a left adrenal mass and hypertensive urgency and diagnosed pregnancy with PHEO in our hospital. The timely diagnosis and proper treatment came with an optimal maternal and fetal outcome. The case of pheochromocytoma in pregnancy we report demonstrated that early diagnosis and a multidisciplinary approach ensured a favorable prognosis for both maternal and fetal, and we also addressed the importance of individual basis evaluation during the whole journey.

Phaeochromocytoma in pregnancy: not all hypertension is essential

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Pheochromocytoma in pregnancy - a rare but dangerous diagnosis.

Pheochromocytoma is a rare cause of hypertension in pregnancy. Unrecognized, it carries a great risk for both mother and the foetus. The main reason for missing the diagnosis is the misconception that any hypertension occurring in pregnancy is gestational hypertension or pre (eclampsia). As many as 90% of patients report one or more pheochromocytoma-related symptoms antenatally, but the diagnosis is made in 75% of patients, meaning that 3 out of 10 patients are diagnosed after childbirth or post-mortem. The symptoms are similar to other more common causes of hypertension in pregnancy, which presents a major diagnostic challenge. The diagnosis is based on determination of metanephrines in plasma or 24-hour urine. Magnetic resonance imaging (MRI) and ultrasound (US) are used to localize the tumour. If the diagnosis is made before the 24th week of pregnancy, laparoscopic removal of the tumour in the second trimester is recommended. If diagnosed later, the tumour could be removed during or after delivery. Preoperative preparation with alpha blockers is required to stabilize blood pressure. The decision on the mode of delivery depends on several factors, so an experienced multidisciplinary team is needed to minimize maternal and foetal mortality.

Pheochromocytoma in pregnancy: A case report and updated management review

Pheochromocytoma is a catecholamine-producing neuroendocrine tumor leading to hypertensive crises in untreated patients. Presentation in pregnancy can mimic gestational hypertension or pre-eclampsia. Physicians need to keep a high index of suspicion to have timely diagnosis and management of pheochromocytoma during pregnancy. We report a 27-year-old female who was diagnosed with pheochromocytoma at 18 weeks of gestation and she underwent laparoscopic adrenalectomy at 22 weeks of gestation with a good pregnancy outcome and with no recurrence over 8 years of follow-up.

Pheochromocytoma as a rare hypertensive complication rarely associated with pregnancy: Diagnostic difficulties (Review).

This review provides a brief clinically relevant review of pheochromocytoma in pregnancy, to raise awareness among doctors in obstetrics and the aim is to serve as the first point of reference when confronted by their presence. Pheochromocytomas are neuroendocrine, catecholamine-secreting tumours. Despite having the highest incidence rate among other hormone-secreting adrenal tumours, they remain rare especially when associated with pregnancy. The non-specific presentation of pheochromocytomas, the difficulties in their diagnosis during pregnancy as well as the high maternal and fetal mortality rates associated with them, present a challenge. Clinical suspicion and meticulous patient history-taking remain the primary lines of defense, while biochemical proof of catecholamine excess (or their metabolites) and imaging-based localisation of the tumour are required for diagnosis. Antenatal diagnosis and complete localisation of the tumour increase the likelihood of successful outcomes for both mother and newborn. Magnetic resonance imaging (MRI) remains the method of choice during pregnancy without excluding the use of ultrasound. Treatment goals should include the avoidance of hypertensive crises while maintaining adequate uteroplacental circulation. The target blood pressure is not strictly defined but is in line with the general guideline addressing chronic hypertension during pregnancy. Antihypertensive medications remain the cornerstone in managing pheochromocytoma. As a first-line, the α-adrenergic, nonselective antagonist phenoxybenzamine is the most frequently used agent, while α1-selective adrenergic antagonists with or without the addition of β- or β1-blockers are also prescribed in certain cases, rendering calcium channel blockers as 'second-choice'. Blood-pressure control of the mother and the well-being of the fetus are determining factors in deciding the time of delivery, which is preferably conducted by Caesarean section. Excision of the tumour(s) remains the final treatment goal. Lifelong biochemical testing is required with or without medical treatment, to address mineralocorticoid or glucocorticoid deficits. Despite ever-improving positive outcome rates, pheochromocytoma associated with pregnancy remains a pathology with high mortality and morbidity rates.

Pheochromocytoma in pregnancy: the need for a multidisciplinary approach

Pheochromocytoma in pregnancy: the need for a multidisciplinary approach

Phaeochromocytoma in pregnancy

The Sri Lankan Journal of Anaesthesiology is the official journal of the College of Anaesthesiologists of Sri Lanka. It publishes clinical investigations, research articles, audits, case reports, review articles and CME articles relating to anaesthesiology, critical care and pain. It is published bi annually in January and June. Sri Lankan Journal of Anaesthesiology is included on DOAJ and Scopus.

SAT-212 Management of VHL-Associated Pheochromocytoma in Pregnancy

Background: The appropriate treatment of pheochromocytoma during pregnancy is crucial because, while its incidence is rare (0.007%), it is associated with increased maternal and fetal mortality1. Clinical Case: A 32 year old Caucasian primigravida female presented with intrauterine pregnancy at 25 weeks for evaluation and management of a 9 mm left adrenal mass. The patient was previously diagnosed at age 10 with Von-Hippel Lindau (VHL) syndrome and genetic testing was positive for a VHL gene mutation (Type 2C VHL). At age 14, she underwent laparoscopic adrenalectomy to excise a right sided pheochromocytoma. At age 20, imaging revealed an enhancing left adrenal mass with imaging characteristics consistent with pheochromocytoma and with catecholaminergic labs that were normal or only mildy increased to 1.5 times upper normal range. Pre-partum the patient had been experiencing periodic episodes of headaches and palpitations. She reported experiencing baseline levels of palpitations and sweating with new onset of pre-syncope and hypotension during her pregnancy. Labs revealed normal serum levels of fractionated free metanephrines. Alpha adrenergic blockade, beta adrenergic blockade, and surgery were considered but deemed unnecessary as the patient was asymptomatic, imaging indicated the left adrenal mass was stable in size, and the patient wished to avoid lifelong adrenal replacement therapy. The patient’s blood pressure and heart rate were monitored closely throughout pregnancy and the use of alpha blockade or beta blockade was not needed. The patient had an uncomplicated induced delivery at 39 weeks. MRI with contrast of abdomen performed 10 months postpartum confirmed the presence of an enhancing 8 mm lesion on the inferior pole of the left adrenal gland with imaging characteristics again consistent with pheochromocytoma. Conclusion: This case demonstrates that a conservative approach of monitoring blood pressure and heart rate can be sufficient and appropriate for management of some patients with pheochromocytoma and VHL during pregnancy.Citation:1. ENDOCRINOLOGY IN PREGNANCY: Pheochromocytoma in pregnancy: case series and review of literature in: European Journal of Endocrinology Volume 177 Issue 2 (2017).

SAT-211 Is Doxazosin the Right Choice for Preoperative Management of Pheochromocytoma in Pregnancy?

Pheochromocytoma (PCC) in pregnancy is a very rare condition, with a reported incidence of less than 0.2 per 10.000 pregnancies (1), having fetal and maternal mortality of 50% if untreated (2). Choosing between selective vs. nonselective alpha blockers as preoperative management in pregnancy is controversial.We report a case of a 39-year-old female having episodes of nervousness, hand tremors, palpitations, diaphoresis, and headaches since 2012; she also had a history of multiple miscarriages and uncontrolled hypertension(HTN) since 2018. In 2019, she was found to have plasma metanephrines 690pg/mL (0-62) and plasma normetanephrine of 3803pg/mL(0-145). Repeat labs showed: plasma metanephrines 2.071pg/mL(0-62), normetanephrine 6.289pg/mL(0-145), norepinephrine 4.268pg/mL(0-874), epinephrine 555pg/mL(0-62). CT abdomen showed a 6.2x5.1x6.4cm left adrenal mass, with 44 Hounsfield units and less than 50% of washout. She was started on Doxazosin 2mg/d, which eventually was increased to 6mg/d with optimal blood pressure (BP) control. After her preoperative workup, she was found to be 7 weeks pregnant. OB-GYN recommended left adrenalectomy before 14 weeks gestation. She had left open adrenalectomy, with normal range postoperative BP, off of antihypertensive medications.The diagnosis of PCC in pregnancy should be considered in the setting of paroxysmal HTN, with no proteinuria, episodic palpitations, diaphoresis, facial flushing, and orthostatic hypotension. Anterior adrenalectomy early in pregnancy is recommended. The increased intraabdominal pressure, fetal movements, uterine contractions, delivery process, and abdominal surgical intervention can trigger the catecholamine release by the PCC, which could lead to placental abruption and miscarriage; and the rebound hypotension may lead to severe hypoxia, causing fetal demise (2). Definitive preoperative treatment between selective vs non-selective alpha-blockers remains controversial. Phenoxybenzamine, appeared to produce better attenuation of intraoperative HTN, however, it is associated with more maternal intraoperative/postoperative hypotension, and reflexive tachycardia. It crosses the placenta and accumulates in the fetus, increasing the risk of neonatal hypotension and respiratory depression (3,4,5). On the other hand, Doxazosin, can be displaced by high levels of catecholamines, but it is less associated with intraoperative/postoperative hypotension (6,7), with no reports of neonatal hypotension, and respiratory depression. Due to the lack of presynaptic a2-adrenoceptor blockade there is less reflex tachycardia, reducing the use of Beta-blockers (8). Doxazosin seems to be a safe, affordable alternative for preoperative management of PCC in pregnant patients.

Late diagnosis of pheochromocytoma in pregnancy with poor fetal outcome

Pheochromocytomas are rare tumors producing catecholamines that could be a cause of secondary hypertension. On that basis, pheochromocytoma can occur as an extremely rare cause of hypertension in pregnancy and if diagnosed late can lead to adverse maternal-fetal outcome. In this case report we describe a case of pheochromocytoma affected pregnancy with poor fetal outcome. A 27-year-old pregnant woman was admitted for severe pre-eclampsia due to pre-gestational hypertension that was diagnosed during the first trimester. Due to high and uncontrolled maternal blood pressure and the worsened maternal-fetal condition after the admission induction of fetal lung maturity and emergency cesarean section were performed, but with poor fetal outcome. Later, an adrenal gland mass was identified using abdominal ultrasound and confirmed by computed tomography. After surgical adrenalectomy blood pressure of the patient was normalized. Pheochromocytoma should be considered as a possible cause of hypertensive disorder during pregnancy, particularly in cases of severe and unresponsive hypertension in order to provide for timely and appropriate treatment.

Successful Pregnancy Outcomes After Laparoscopic Management of Pheochromocytoma.

Background: Pheochromocytoma is a rare cause of hypertension in pregnancy, which is often overlooked; especially in late pregnancy because of more prevalent pre-eclampsia. It has been associated with significant morbidity and mortality rates in both mother and fetus, if not diagnosed and treated in time. Minimally invasive surgery has been infrequently used for surgical management of pheochromocytoma in pregnancy, with <20 reported cases in English literature. Case Presentation: A 26-year-old pregnant woman presented at 9 weeks of gestation with complaints of palpitations, sweating, and headache; with past history of first trimester spontaneous abortion caused by accelerated hypertension. She was found to have hypertension and diabetes, but no pedal edema, weight gain, or proteinuria. Ultrasonogram and MRI of abdomen revealed a left adrenal mass and 24 hours urinary catecholamines levels were increased, suggesting a pheochromocytoma. After preoperative optimization in consultations with obstetricians, endocrinologists, and anesthetists, she underwent laparoscopic left adrenalectomy during 15th week of gestation. Perioperative hospital course was uneventful for both mother and the fetus. After adrenalectomy, her diabetes was cured and hypertension was controlled with single antihypertensive. She was readmitted at 31 weeks of gestation with accelerated hypertension and underwent emergency caesarean for impending eclampsia at 32 weeks, and delivered a healthy female baby. 131I-meta-iodobenzylguanidine (MIBG) scan and 68Ga-[1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI(3)-octreotide positron emission tomography-CT (68Ga-DOTANOC PET-CT) scan was obtained in postpartum period to rule out any extra-adrenal pheochromocytoma, both of which did not reveal any abnormality. At 1 year follow-up, she is normoglycemic and hypertension controlled on single antihypertensive. Conclusion: Pheochromocytoma in pregnancy is a rare but potentially lethal condition, and high index of suspicion is required for early diagnosis. Multidisciplinary coordination is required for effective management of this rare condition. Laparoscopic adrenalectomy is safe in second trimester of pregnancy for both mother and fetus.

The diagnostic and therapeutic difficulties in management with pheochromocytoma in pregnancy – a review

The diagnostic and therapeutic difficulties in management with pheochromocytoma in pregnancy – a review

Phaeochromocytoma in pregnancy

Phaeochromocytoma in pregnancy

SAT-313 Catecholamine-Induced Cardiomyopathy in Pheochromocytoma during Pregnancy

Introduction: This case illustrates an uncommon initial presentation of cardiomyopathy in a pregnant woman who was later found to have Pheochromocytoma. Case: A 33-year-old female who is 19 weeks pregnant with no significant past medical or family history presented to the hospital with pleuritic chest pain associated with palpitations and dyspnea. Echocardiogram showed mildly dilated left ventricle with mild global left ventricular (LV) systolic dysfunction and an ejection fraction (EF) of 40-50%. Electrocardiogram (EKG) showed Q waves, with peak troponin of 1.8. Her symptoms were attributed to a post-viral perimyocarditis, was treated symptomatically and discharged home. She presented again at 22 weeks of pregnancy due to the same symptoms. Repeat echocardiogram showed improvement of cardiac function with normal EF and LV function; hence, she was once again treated symptomatically and discharged to home. The rest of her pregnancy was unremarkable. She carried to term and had an uncomplicated vaginal delivery. One week postpartum she presented to the hospital this time with complaints of intermittent headaches, episodic palpitations and new onset hypertension with systolic blood pressure (BP) in the 170s (<130mmHg). Pre pregnancy and BP during pregnancy were within normal limits. Diagnostic testing for secondary causes of hypertension were done which were all negative except for plasma catecholamines: metanephrine 24 nmol/L (<.50), normetanephrine 8.3nmol/L (<.9), epinephrine >1354 pg/ml (<642), and norepinephrine 2669 pg/ml (<642). 24 hour urine results showed: metanephrine 20 600mcg/24hr (<400), normetanephrine 3540 mcg/24hr (<900), total metanephrines 24 140 mcg/24hr (<1300), epinephrine 583 mcg/24hr(<21), norepinephrine 645 mcg/24hr (15-80). CT of the abdomen showed a 7.9x7x8.2 cm right adrenal mass. She underwent a laparoscopic right adrenalectomy and histopathology confirms the diagnosis of Pheochromocytoma. Her symptoms resolved after surgery with BP returning to normal. Repeat plasma catecholamines post surgery also returned to normal limits. Discussion: Pheochromocytoma during pregnancy is an extremely rare event with a reported incidence of .007% with an antepartum diagnosis that is only made 75% of the time (Van der Weerd et al., 2017). It is most often found during the third trimester of pregnancy and patients are more symptomatic during this time due to the compression of the enlarging gravid uterus on the tumor causing catecholamine release. Diagnosis is difficult especially in this case because the patient did not initially present with the classic symptoms until postpartum. Reference: Weerd, K. V., Noord, C. V., Loeve, M., Knapen, M. F., Visser, W., Herder, W. W., Feelders, R. A. (2017). ENDOCRINOLOGY IN PREGNANCY: Pheochromocytoma in pregnancy: Case series and review of literature. European Journal of Endocrinology,177(2). doi:10.1530/eje-16-0920

MON-390 Giant Cystic Adrenal Incidentaloma in Pregnancy: A Case Report

Introduction Adrenal incidentalomas in pregnancy are extremely rare but can be very dangerous. Management of such incidentalomas in pregnancy are not clearly defined, and published literature mostly consists of case reports. Clinical Case A 29-year-old female presented with severe right-sided flank pain. She was 15 weeks pregnant and had completed her first pregnancy 18 months ago without complications. In 2012 she presented with similar symptoms and had a CT KUB which showed a right renal stone in the proximal ureter and underwent stenting. A right-sided adrenal incidentaloma in the size of 4cm x 4.5cm x 3.9cm with 19HU (Hounsfield Units) was also detected and was not further evaluated. She did not have a family history of malignancy and was not on regular medication. Her routine observations were normal with a blood pressure of 110/70mmHg. The examination was unremarkable apart from right-sided flank tenderness. Routine biochemistry was normal. An ultrasound of the abdomen showed a sizeable adrenal cyst which has significantly increased in size (now 900cc, previously 312cc). An abdominal MRI revealed a 13 x 12 x 12 cm right-sided adrenal mass which is heterogeneous with a multi-loculated haemorrhagic component. The mass appeared well capsulated with compression of the inferior vena cava and displacing the right kidney. There was no apparent invasion into adjacent structures. A morning cortisol was 401nmol/l (no reference range) with ACTH 1.7pmol/L (no reference range) and plasma metanephrines were 98pmol/L (<447pmol/L). However, normetanephrine was elevated at 961pmol/L (<590pmol/L) with repeat levels at 1196pmol/L, raising concerns of a possible cystic phaeochromocytoma. Appropriateness for surgery discussed in an MDT meeting comprise of endocrinologists, obstetrician and endocrinology surgeon. The patient herself was not keen for surgery, and we decided on close observation with monthly repeat catecholamine levels and ultrasound scans. The patient remained asymptomatic with normal blood pressure. Repeat plasma Metanephrines (25/05/2018- 85pmol/L , 22/08/2018- 75pmol/L), Normetanephrine (25/05/2018- 410pmol/L, 22/08/2018- 410pmol/L) and plasma 3-Methoxytyramine (25/05/2018- <50pmol/L, 22/08/2018- <50pmol/L) were all normal. A repeat ultrasound confirmed reduced mass size measuring 9.4 cm x 7.5 cm x 7.8 cm. The patient underwent elective caesarian on 08/10/2018 without complications, giving birth to a healthy baby. She is awaiting an endocrine surgeon review for planned adrenalectomy. Conclusion There is only a limited number of reported cases on large adrenal incidentalomas in pregnancy. In fact, we were unable to find any reported evidence of large cystic pheochromocytoma in pregnancy. There is no consensus and guidelines for treating pheochromocytoma during pregnancy, and a multidisciplinary approach should determine management.

Abstract #116 Incidental Finding of Pheochromocytoma in Pregnancy

Abstract #116 Incidental Finding of Pheochromocytoma in Pregnancy

Pheochromocytoma diagnosed during pregnancy: lessons learned from a series of ten patients.

Pheochromocytoma (PHEO) in pregnancy is a life-threatening condition. Its management is challenging with regards to the timing and type of surgery. A retrospective review of the management of ten patients diagnosed with pheochromocytoma during pregnancy was performed. Data were collected on the initial diagnostic workup, symptoms, treatment, and follow-up. PHEO was diagnosed in ten patients who were between the 10th and the 29th weeks of pregnancy. Six patients had none to mild symptoms, while four had complications of paroxysmal hypertension. Imaging investigations consisted of MRI, CT scan and ultrasounds. All had urinary metanephrines, measured as part of their workup. Three patients had MEN 2A, one VHL syndrome, one suspected SDH mutation. All patients were treated either with α/β blockers or calcium channel blockers to stabilize their clinical conditions. Seven patients underwent a laparoscopic adrenalectomy before delivery. Three out of these seven patients had a bilateral PHEO and underwent a unilateral adrenalectomy of the larger tumor during pregnancy, followed by a planned cesarean section and a subsequent contralateral adrenalectomy within a few months after delivery. Three patients had emergency surgery for maternal or fetal complications, with C-section followed by concomitant or delayed adrenalectomy. All newborns from the group of planned surgery were healthy, while two out three newborns within the emergency surgery group died shortly after delivery secondary to cardiac and pulmonary complications. PHEO in pregnancy is a rare condition. Maternal and fetal prognosis improved over the last decades, but still lethal consequences may be present if misdiagnosed or mistreated. A thorough multidisciplinary team approach should be tailored on an individual basis to better manage the pathology. Unilateral adrenalectomy in a pregnant patient with bilateral PHEO may be an option to avoid the risk of adrenal insufficiency after bilateral adrenalectomy.

A rare presentation of pheochromocytoma in pregnancy: a case report

BackgroundEarly diagnosis of pheochromocytoma and its proper management can lessen its mortality and morbidity. This case report describes a 24-year-old pregnant woman with an unusual presentation of pheochromocytoma.Case presentationAn Iranian 24-year-old primigravid woman from Kordistan province was referred to our center with left flank pain at 37 weeks of gestation. She had a history of gestational diabetes mellitus since the 12th week of gestation which was managed by insulin administration. She also had a history of pulsatile bi-temporal headache for 2 years prior to her referral to us. She underwent complete abdominal and pelvic ultrasound imaging for her flank pain. This examination revealed a heterogeneous mass of 119 × 87 × 79 mm above her left kidney, highly suspicious of being an adrenal-originating tumor. Subsequently, we consulted an endocrinologist. She underwent abdominopelvic magnetic resonance imaging and her 24-hour urine metanephrine, normetanephrine, and vanillylmandelic acid were assessed. Finally, the diagnosis of pheochromocytoma was confirmed. She underwent a cesarean section and adrenal mass excision at the 40th week of gestation. This timely diagnosis resulted in her proper management and good maternal and neonatal treatment outcomes.ConclusionsOur patient had pheochromocytoma during pregnancy. She had no complaints about hypertension before or during pregnancy until giving birth to her child; her only symptoms were a vague left flank pain, gestational diabetes, and headaches for the past 2 years. The unusual symptom of flank pain led to timely diagnosis and a good treatment outcome.