Bilateral pheochromocytoma during the postpartum period

Wattanachanya, Lalita; Bunworasate, Udomsak; Plengpanich, Wanee; Houngngam, Natnicha; Buranasupkajorn, Patinut; Sunthornyothin, Sarat; Shotelersuk, Vorasuk; Snabboon, Thiti

doi:10.1007/s00404-009-1057-5

Cited by 16 publications

(17 citation statements)

References 16 publications

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“…Of note, the spells of hypertension in the supine position but not in the sitting or standing position are explained by the compressing effect of the gravid uterus on the tumour. Uterine contractions, foetal movements and the process of delivery may also cause a release of catecholamines (50). Myocardial ischemia, cardiomyopathy, encephalopathy and multiple organ failure have all been reported during pregnancy (51).…”

Section: Phaeochromocytoma and Paraganglioma In Pregnancymentioning

confidence: 99%

“…Delayed or unmade diagnosis is how ever responsible for a high maternal and foetal mortality. The clues that should alert the clinician include paroxysmal or labile hypertension associating the characteristic triad (headache, sweating and tachycardia), postural hypotension, worsening of the supine position and a family or a past history of phaeochromocytoma, CMT or hyperparathyroidism (as a part of MEN 2A) (49)(50)(51)5). Of note, the spells of hypertension in the supine position but not in the sitting or standing position are explained by the compressing effect of the gravid uterus on the tumour.…”

Section: Phaeochromocytoma and Paraganglioma In Pregnancymentioning

confidence: 99%

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Phaeochromocytoma: State-of-the-art

Donckier

Michel

2010

Acta Chirurgica Belgica

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Phaeochromocytomas are catecholamine-secreting tumours that arise from chromaffin cells of the adrenal medulla and extra-adrenal sites. Extra-adrenal phaeochromocytomas are called paragangliomas. A diagnosis of phaeochromocytoma is suspected by typical paroxysmal symptoms, unusual or refractory hypertension, discovery of an adrenal incidentaloma or a family history of phaeochromocytoma or paraganglioma, possibly associated with other genetic syndromes (multiple endocrine neoplasia type 2 A or B, neurofibromatosis type 1 and von Hippel-Lindau disease). It can be confirmed by measurements of urinary or plasma fractionated catecholamines and metanephrines. The best diagnostic performances are achieved by metanephrines. Twenty-four hour urine fractionated metanephrines are still recommended as a screening test but some experts prefer plasma measurements in high-risk patients. Increased serum chromogranin-A levels, combined with high catecholamine or metanephrine in a patient with normal renal function is also a tool, virtually diagnostic of phaeochromocytoma. Recent studies have suggested that 25% of patients with phaeochromocytoma have germline mutations of several genes (NF1, VHL, SDHD, SDHB and RET). Thus, genetic testing should be carried out according to an algorithm of risk factors and specific characteristics. Once a biochemical diagnosis of phaeochromocytoma is made, a CT scan or MRI of the abdomen and pelvis should be performed first. If these investigations remain negative, the chest and neck should be explored. After anatomical imaging, functional imaging by 123I-MIBG should be considered. If the MIBG scan is negative, other imaging modalities have recently proven to be useful (PET, Octreoscan). After localization, the treatment of phaeochromocytoma is a surgical resection, which may be laparoscopic. Preoperative preparation with alpha- and beta-adrenergic blockade and/or calcium channel blockers associated with volume expansion is essential. Malignant phaeochromocytoma is rare and its treatment still unsatisfying. Phaeochromocytoma during pregnancy is also rare and its diagnosis easily missed because of its clinical resemblance to pre-eclampsia.

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Section: Phaeochromocytoma and Paraganglioma In Pregnancymentioning

confidence: 99%

Section: Phaeochromocytoma and Paraganglioma In Pregnancymentioning

confidence: 99%

Phaeochromocytoma: State-of-the-art

Donckier

Michel

2010

Acta Chirurgica Belgica

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“…These should raise the suspicion of pheochromocytoma. Other clinical features that should alert physicians include hypertension with postural hypotension worsening in the supine position or with a positive history of hereditary pheochromocytoma syndrome [4,10]. Demonstration of an increased catecholamine and its metabolites on 24 h uine or from a plasma collection is required to confirm the diagnosis of pheochromocytoma.…”

Section: Discussionmentioning

confidence: 97%

“…Pheochromocytoma or paraganglioma can occur as a sporadic case or as autosomal dominant inherited syndromes including von Hippel-Lindau (VHL) caused by germline mutations in the VHL gene; multiple endocrine neoplasia (MEN) type 2 caused by mutations in the RET gene; neurofibromatosis type 1 (NF-1) caused by mutations in the NF1 gene; and the familial paraganglioma syndromes (PGL) caused by mutations in the SDHB, SDHC, or SDHD genes [4,5]. The hereditary forms are usually diagnosed earlier or present as paraganglioma or bilateral/ multiple lesions.…”

Section: Introductionmentioning

confidence: 99%

Concurrent bilateral pheochromocytoma and thoracic paraganglioma during pregnancy

Snabboon

Plengpanich

Houngngam

et al. 2010

Endocr

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Although hypertension occurring during pregnancies is not uncommon and its prognosis is generally excellent, some of its unusual causes can lead to catastrophic consequences, especially in undiagnosed cases. Here, we report a pregnant woman who presented with hypertension in her early pregnancy. It was subsequently found to be caused by bilateral pheochromocytoma. After removal of both tumors, catecholamine levels unexpectedly and unexplainably remained elevated. At 23 weeks of gestation, the fetus was found dead in utero. After the fetal death, additional studies were performed and revealed a thoracic paraganglioma. To our knowledge, this is the first report of a case of three catecholamine-producing tumors occurring concurrently during a pregnancy. Genetic analysis helped identify this unprecedented condition; the patient harbored a heterozygous missense mutation c.482G>A in exon 3 of the VHL gene, indicating von Hippel-Lindau syndrome. Physicians who care for hypertensive pregnant patients should be aware of this condition as its diagnosis would probably lead to a better outcome.

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“…Unrecognized MEN 2A syndrome complicating pregnancy has been associated with severe life-threatening complications, including myocardial infarction, cardiovascular collapse at term, peripartum cardiomyopathy, intracranial haemorrhage and fatal adrenergic crisis. [2345] We report a case of MEN 2A or Sipple's syndrome diagnosed in pregnancy, in which medical therapy allowed fetal maturation and vaginal delivery, followed by adrenalectomy, thyroidectomy, and parathyroidectomy.…”

Section: Introductionmentioning

confidence: 99%

Vaginal delivery in a patient with pheochromocytoma, medullary thyroid cancer, and primary hyperparathyroidism (multiple endocrine neoplasia type 2A, Sipple′s syndrome)

et al. 2014

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Multiple endocrine neoplasia 2A (MEN 2A), or Sipple's syndrome is a rare inherited dominant syndrome, characterised by medullary thyroid carcinoma, adrenal pheochromocytoma and hyperparathyroidism, due to specific RET proto-oncogene mutations. The women with MEN 2A syndrome are at risk of complicated pregnancy because of unrecognised pheochromocytoma and transmission of RET mutation to the progeny. We report a case of a woman with MEN 2A diagnosed in early pregnancy. Alpha-blockade medical therapy was used effectively and time was given for fetal maturation. Uncomplicated vaginal delivery performed under epidural analgesia. Six weeks postpartum adrenalectomy, thyroidectomy and parathyroidectomy were performed uneventfully.

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Bilateral pheochromocytoma during the postpartum period

Abstract: MEN2A affected pregnancy is an unusual condition. This syndrome should be suspected when a pregnant patient has a history of MTC. Early detection and appropriate management can prevent serious maternal and fetal complications. We also reviewed the literature of MEN2A-affected pregnancies.

Cited by 16 publications

References 16 publications

Phaeochromocytoma: State-of-the-art

Phaeochromocytoma: State-of-the-art

Concurrent bilateral pheochromocytoma and thoracic paraganglioma during pregnancy

Vaginal delivery in a patient with pheochromocytoma, medullary thyroid cancer, and primary hyperparathyroidism (multiple endocrine neoplasia type 2A, Sipple′s syndrome)

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