Diagnosis and treatment of pheochromocytoma during pregnancy

Abstract

Objective: To investigate the diagnosis and treatment of pheochromocytoma during pregnancy.

Materials and methods: The data of four cases of pheochromocytoma was analyzed retrospectively. Their ages were 41, 28, 32 and 30 years old, and the four patients were at 32nd week, 12th week, 14th week and 13th week of gestation. All patients had hypertension during pregnancy, accompanied with headache, dizziness, palpitation and sweating. The 24-h urinary catecholamines (24?h UCA) increased significantly. Ultrasound and MRI confirmed the diagnosis of pheochromocytoma.

Results: One case had Cesarean section at 32 weeks of gestation, and a healthy baby girl was delivered smoothly. Laparoscopic resection of the right adrenal pheochromocytoma was performed at the same time, and an adrenal tumor of 7.0?cm was resected successfully. Two cases chose abortion and laparoscopic resection of pheochromocytoma was performed. One case chose abortion and refused further treatment. Histopathology confirmed the diagnosis of pheochromocytoma.

Conclusions: For hypertension in pregnant women during pregnancy, typical paroxysmal hypertension accompanied by triad of headache, palpitation and sweating, pheochromocytoma should be considered. Early diagnosis can reduce the maternal and fetal mortality significantly. Second trimester of pregnancy is the ideal time for surgical treatment. Laparoscopic resection of pheochromocytoma during pregnancy is safe and effective.     

Recurrence of phaeochromocytoma in pregnancy in a patient with multiple endocrine neoplasia 2A: a case report and review of literature

Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant inherited condition with a prevalence of one in 40?000 individuals. It causes the development of tumours in endocrine glands, such as medullary thyroid cancer, pheochromocytomas, as well as primary hyperparathyroidism. MEN 2A in pregnancy is very rare with only 29 cases reported in the literature. The presence of pheochromocytoma is a rare cause of hypertension during pregnancy with an incidence of 0.007% of all pregnancies. This has severe implications on both mother and the foetus. This case report describes a 22-year-old nulliparous Caucasian woman with known MEN2A syndrome, who underwent thyroidectomy for medullary thyroid carcinoma in childhood and excision of left sided pheochromocytoma at the age of 19. She was found to have a recurrence of pheochromocytoma in the right adrenal gland during pregnancy at 16 weeks of gestation and was oddly normotensive. Catecholamine effects were blocked with phenoxybenzamine and she delivered by an uneventful elective caesarean section at 36 weeks gestation. She underwent a laparoscopic right adrenalectomy six weeks postpartum, followed by lifelong corticosteroid replacement.     

Hypertensive crisis in pregnancy due to a metamorphosing pheochromocytoma with postdelivery Cushing's syndrome

Pheochromocytomas in pregnancy are rare but potentially lethal. Even rarer is the combination of pheochromocytoma in pregnancy with subsequent development of ectopic Cushing’s syndrome. We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes. A cesarean section was performed at 32 weeks following a hypertensive crisis after routine administration of betamethasone. Postnatal persistence of signs and symptoms of catecholamine excess led to the diagnosis of a left adrenal pheochromocytoma. Between diagnosis and planned tumor removal, the patient developed signs and symptoms of Cushing’s syndrome (facial edema and hirsutism, myopathy and fatigue). Biochemical testing confirmed hypercortisolism with extremely elevated levels of plasma adrenocorticotropin, urinary cortisol and multiple steroids of a plasma panel that were all normal at previous testing. The previously noradrenergic tumor also started producing epinephrine. Histopathological examination confirmed the pheochromocytoma, which was also immunohistochemically positive for adrenocorticotropin. Full post-surgical recovery was sustained with normal blood pressure and biochemical findings after one year. This report not only underlines the chameleon behavior of pheochromocytoma but also illustrates its potential for a metamorphosing presentation. Corticosteroid administration in pregnancy requires a cautious approach in patients with hypertension.     

Pheochromocytoma in neurofibromatosis type 1 during pregnancy

Pregnant women with neurofibromatosis type 1 (NF-1) have increased complications during gestation, including hypertensive disorders that are sometimes caused by pheochromocytoma. Pheochromocytoma is an extremely rare condition during pregnancy, and the main clinical manifestation is hypertension. If not properly treated, pheochromocytoma has high maternal and fetal mortality rates. Early recognition and adequate clinical management before delivery have led to better outcomes in the last few decades. Despite the association of NF-1 and pheochromocytoma, there are few clinical reports of these two conditions in pregnant patients. We present a rare case of pheochromocytoma diagnosed during pregnancy in a patient with NF-1, and we describe the treatment and the obstetric and fetal outcomes. We also review other medical conditions related to NF-1 that complicated this patient’s pregnancy.     

Pheochromocytoma in a pregnant woman with multiple endocrine neoplasia type 2a

Pheochromocytoma is a rare cause of hypertension. Its coexistence with pregnancy is exceptional and laparoscopic removal has rarely been reported. We describe the case of a 34-year-old woman with multiple endocrine neoplasia type 2a (MEN 2a) with adrenal pheochromocytoma diagnosed in the 6th week of pregnancy. After pretreatment with phenoxybenzamine ,a successful transperitoneal laparoscopic adrenalectomy was performed in the twentieth week of gestation. The management of pheochromocytoma in pregnancy and the indications for laparoscopic surgery in pregnant patients are discussed.     

Pheochromocytoma diagnosed during pregnancy: a case report

Abstract

Pheochromocytoma is a rare catecholamine producing tumor. During pregnancy, this tumor can mimic gestational hypertension, preeclampsia or eclampsia. We present a case of a 38-year-old, multiparous woman admitted at 36?weeks of gestation due to hypertension. After cesarean section, she developed an unexplainable worsening of her hypertension that was resistant to the traditional therapy. Additional investigation lead to the diagnosis of pheochromocytoma. This case highlights the importance of an early diagnosis and the need of a multidisciplinary approach for the improvement of pregnancy outcomes in patients with pheochromocytoma.     

Pheochromocytoma in a pregnant woman with multiple endocrine neoplasia type 2a.

Pheochromocytoma is a rare cause of hypertension. Its coexistence with pregnancy is exceptional and laparoscopic removal has rarely been reported. We describe the case of a 34-year-old woman with multiple endocrine neoplasia type 2a (MEN 2a) with adrenal pheochromocytoma diagnosed in the 6th week of pregnancy. After pretreatment with phenoxybenzamine, a successful transperitoneal laparoscopic adrenalectomy was performed in the twentieth week of gestation. The management of pheochromocytoma in pregnancy and the indications for laparoscopic surgery in pregnant patients are discussed.     

Serum Uric Acid as a Marker of Pregnancy-Induced Hypertension

Summary: A prospective longitudinal study was conducted, looking at the changes in serum uric acid during pregnancy in women who were normotensive at initial presentation. In our sample of 78 women having a total of 88 singleton pregnancies, 13 developed pregnancy-induced hypertension during labour only, whilst a further 6 developed hypertension during pregnancy. Women who developed hypertension had significantly higher uric acid levels than women who remained normotensive throughout. However, there was an appreciable overlap between the groups. Women with essential hypertension showed similar changes. We conclude that the serum uric acid level is an unreliable indicator of developing hypertension in the individual woman. However, a rapidly rising uric acid level should be viewed with caution.     

Pheochromocytoma associated with pregnancy: case report and review of the literature.

We report on a young woman with pheochromocytoma associated with pregnancy and review 41 other cases reported in the literature from 1988 to 1997. This review reveals that the overall maternal mortality was 4 percent and the fetal loss 11 percent; antenatal diagnosis of pheochromocytoma reduced maternal mortality to 2 percent; however, fetal loss was 14 percent. Diagnosis of pheochromocytoma was made antepartum in 83 percent of the cases. Although pheochromocytoma associated with pregnancy is rare, a high index of clinical suspicion must be kept and all those at risk must be investigated to achieve an early diagnosis and improved outcome. Once the diagnosis is confirmed, alpha-adrenergic blockade is essential and beta-blockade may be required. Magnetic resonance imaging and computerized tomography scan may be used to localize the tumor during the antenatal period. In early pregnancy, i.e., before 24 weeks, both tumor resection and medical treatment are associated with good fetal outcome; in later pregnancy, elective cesarean delivery followed by tumor resection results in favorable maternal and fetal outcome. TARGET AUDIENCE: Obstetricians & Gynecologists, Family Physicians. LEARNING OBJECTIVES: After completion of this article, the reader will be able to understand the clinical manifestations of a pheochromocytoma during pregnancy, how to make the diagnosis of a pheochromocytoma during pregnancy, and to know the medical and surgical management of a pheochromocytoma during pregnancy.     

Bilateral pheochromocytoma during pregnancy

Background Pheochromocytoma is a rare disease of the chromaffin cells that secrete catecholamines. It may occur during pregnancy. Bilateral pheochromocytoma in pregnancy is even rarer.Case A 26-year-old woman, gravida 2, para 0-0-1-0, 18 weeks pregnancy, was initially seen with elevated blood pressure (170/100 mmHg) and mild headache. The cause of hypertension was conventionally investigated and bilateral pheochromocytoma was finally searched for and found. Bilateral adrenalectomy was undertaken at 23 weeks gestation and Cesarean section was performed at 31 weeks gestation due to intrauterine growth retardation (IUGR) and compromised fetal well-being. The maternal outcome was uneventful and the baby was physiologically complicated only by neonatal jaundice.Conclusion Pheochromocytoma should be searched for in the conventionally differential diagnosis in hypertension during pregnancy, especially in the young. Early diagnosis and proper management with medical treatment followed by surgical removal of the tumor usually result in good maternal and fetal outcomes.     

Systolic hypertension in pregnancy and cardiovascular mortality: a 44-year follow-up study.

OBJECTIVE: To assess role of systolic hypertension during pregnancy in long-term mortality. METHOD: A total of 4,090 Finnish women who delivered in the period from 1954 to 1963 were followed up for an average of 44 years. Mortality data were obtained from the Finnish cause-of-death registry. Cox-proportional hazard models were used for analysis. RESULTS: Cardiovascular mortality was significantly higher among women with systolic hypertension in early or late pregnancy. Women with systolic hypertension in early pregnancy without proteinuria had a significantly increased risk for cardiovascular death. Primiparas with systolic hypertension and proteinuria had a higher risk for cardiovascular death. CONCLUSION: By considering women with systolic hypertension in pregnancy as being at higher risk for cardiovascular diseases, there may be an opportunity to prevent untimely mortality.     

Pheochromocytoma and pregnancy: report of three cases.

Three cases of pheochromocytoma associated with pregnancy are reported. Two patients had adrenal pheochromocytoma coexistent with normal pregnancy and one patient had a bladder pheochromocytoma coexistent with molar pregnancy (gestational trophoblastic disease). The diagnosis was made antenatally in the two normal pregnancy patients, both underwent planned tumor resection, one at mid-trimester, complicated by postoperative miscarriage, while the other had tumor resection during cesarean section at term. Bladder pheochromocytoma, preoperatively mistaken for invasive trophoblastic tumor, was resected at the time of planned abdominal hysterectomy for molar pregnancy, pheochromocytoma was recognized only after microscopic study of the resected bladder tumor. The three patients survived with no evidence of disease.     

The significance of uterine notching in Doppler sonography in early pregnancy as a predictor for pathologic outcome of the pregnancy

Introduction Doppler sonography of maternal vessels as a predictor for preeclampsia is an established screening method in the second trimester. Considering the first trimester its benefit has not been conclusively proven so far. Up to now the flow pattern of maternal vessels in the first trimester has only been insufficiently characterized and documented. Thus, there are only few pieces of information concerning the predictive value of the different kinds of flow patterns during the early pregnancy as regards the pathological and physiological outcome of the pregnancy. This survey aimed at evaluating the significance and the clinical importance of Doppler sonographical examinations of the Aa. uterinae during early pregnancy. It had to be analyzed whether various perfusions can be applied as a predictive value for the specific development during the pregnancy and whether therefore a risk group for pregnancy-associated hypertension can already be detected during the early pregnancy. Materials and methods A non-selected collective of 104 pregnant patients was examined with Doppler sonography and retrospectively divided into a standard collective and a high-risk collective. The standard collective contained 89 patients, who in the course of their pregnancy remained without any pathological findings. Fifteen patients, who were assigned to the high-risk collective, either developed a pregnancy-induced hypertension during their pregnancy or an intrauterine fetal growth retardation. The Doppler-sonographic examination of the Aa. uterinae was applied for the first time between the 6 and 8 weeks gestation (WG), then in the 9 WG, 10 WG, 11 WG, between the 12 and 14 WG, as well as between the 20 and 24 WG. Results Contrary to published data, there can be traced a completely transformed flow pattern in the Aa. uterinae already during the first trimester to an increasing extent. This correlates with a positive outcome of the pregnancy. There is a definite association between pathological Doppler sonography in the first third of the pregnancy and the development of a uteroplacental insufficiency as well as a pregnancy-associated hypertension (SIH) in the further course of the pregnancy. Conclusion Doppler sonography is an approved method in order to become early attentive of a reduced uterine blood flow. Applying Doppler sonographic treatment of the maternal vessels, uteroplacental insufficiency can be traced at an early stage. Pregnancies, which might develop a pregnancy-associated hypertension (SIH) or a placental insufficiency, can be detected during the early pregnancy to decrease the morbidity rate and the mortality of premature newborns. Further studies in larger collectives are necessary to obtain adequate solid datasets and to further evaluate the clinical meaning and the significance concerning pathological maternal Doppler parameters.     

Plasma noradrenaline and adrenaline levels in normal pregnancy and in pregnancy-induced hypertension

Summary. Plasma concentrations of noradrenaline and adrenaline were measured serially during normal pregnancy in 52 women who remained normotensive and in nine who developed pregnancy-induced hypertension but were otherwise normal. There was a significant fall in both noradrenaline (P<0.005) and adrenaline (P<0.05) levels during normal pregnancy. Throughout pregnancy the mean levels of both catecholamines were lower in the patients who developed pregnancy-induced hypertension than in the normotensive patients. There was no evidence of increased sympathetic nervous system activity in the women who developed pregnancy-induced hypertension.     

Sodium excretion in normal and hypertensive pregnancy: a prospective study

One hundred fifty-eight intravenous saline solution infusions (3 mmol Na per kilogram body weight) were performed in (1) normal primigravid women during the second and third trimesters and post partum, after 1 week of either a high, low, or ad libitum salt intake; (2) normotensive primigravid women during midpregnancy who later developed pregnancy-induced hypertension, and (3) seven proteinuric and seven nonproteinuric primigravid women with ad libitum salt intake who had established pregnancy-induced hypertension. Sodium excretion did not differ significantly between pregnancy and after pregnancy despite marked differences in plasma renin activity, aldosterone concentration, volume, and glomerular filtration rate. Sodium excretion after saline solution loading varied according to prestudy sodium intake and was reduced between the second and third trimesters, independent of dietary salt intake. Those destined to develop pregnancy-induced hypertension had sodium excretion similar to that of continuously normotensive subjects during the second trimester, but those with established proteinuric pregnancy-induced hypertension had the lowest plasma volume, plasma aldosterone concentration, and plasma renin activity and retained sodium to the same degree as salt-deplete women with normotension. These results demonstrate that the balance of sodium regulatory factors is similar between pregnancy and post partum, that prestudy salt intake and stage of gestation can alter the natriuretic response to saline solution loading, and that normal pregnant women retain more administered sodium in late pregnancy than in midpregnancy despite further increases in plasma volume and no alterations to blood pressure or glomerular filtration rate. Those with established proteinuric pregnancy-induced hypertension retain sodium avidly without stimulation of plasma renin activity or plasma aldosterone concentration, findings not apparent during midpregnancy in those who later developed this disorder.     

Pheochromocytoma and pregnancy. Case report

The management of a pheochromocytoma during pregnancy is uncommon and is at high risk for both mother and foetus. We report a case of a patient whose first pregnancy was complicated by foetal demise in a context suggestive of preeclampsia. She was diagnosed with pheochromocytoma as she was beginning a second pregnancy. A laparoscopic adrenalectomy was performed in the first trimester of pregnancy, and maternal and neonatal outcome were favourable. This case illustrates the difficulty of diagnosing pheochromocytoma in pregnancy, and the benefits of laparoscopic treatment in the first trimester.     

Twin pregnancy in a woman on long-term epoprostenol therapy for primary pulmonary hypertension. A case report

BACKGROUND: Pregnancy associated with primary pulmonary hypertension is an uncommon observation, with maternal mortality > 50%. Experience treating this condition is limited. Past reports have emphasized the need for pregnancy termination. In the last few years there has been considerable interest in long-term intravenous use of epoprostenol (prostacyclin) in patients with primary pulmonary hypertension. CASE: A woman with severe primary pulmonary hypertension who was on long-term epoprostenol therapy became pregnant with twins and was treated with high doses of epoprostenol and nitric oxide during delivery and the postpartum period. She was well six months later on continuous epoprostenol therapy. The one viable infant was alive and still hospitalized at this writing. CONCLUSION: Epoprostenol therapy may be continued during pregnancy in patients with severe primary pulmonary hypertension for long-term pulmonary vasodilatation.     

A prospective controlled five-year follow-up study of primiparas with gestational hypertension

Primiparas with hypertension first recognized in pregnancy and an age- and parity-matched control group, normotensive throughout pregnancy, were monitored during 5-6 years with blood pressure recordings performed in a standardized way. At the end of the study period, 21 out of 49 women with hypertension in pregnancy had developed hypertension requiring therapy or borderline hypertension, compared with 2 women in the control group who had developed borderline hypertension. Therapy was required in 7 of the women. Factors associated with increased risk of developing hypertension were gestational week at diagnosis of gestational hypertension, 1st diastolic blood pressure during follow-up, family history, smoking, and age.     

Pheochromocytoma: rare lethal challenging diagnosis that may be encountered by gynecologists and obstetricians

Pheochromocytomas are rare but potentially lethal tumors responsible for malignant hypertension. They may be encountered by gynecologists and obstetricians. The diagnosis is difficult because it can be mistaken for diseases more frequent like preeclampsia or other pelvic tumors. We report two cases highlighting clinical clues such as labile hypertension, headache, sweating, palpitations and failure to respond to conventional treatment should prompt physicians to screen patients for pheochromocytoma by measuring the 24-hour urinary catecholamines. The surgery must be performed after using an appropriate preoperative treatment, in order not to trigger lethal outcome. During pregnancy, C-section is recommended.