https://doi.org/10.14734/PN.2020.31.4.236 pISSN 2508-4887•eISSN 2508-4895
Jeong Min Song, MD1, InHae Na, MD1,
Seung Yeon Pyeon, MD1, Hyun-Joo Seol, MD, PhD1, Sang Hyun Kim, MD, PhD2
1Department of Obstetrics and Gynecology, Kyung Hee University Hospital at Gangdong, Seoul;
2Department of Surgery, Soonchunhyang University Seoul Hospital, Seoul, Korea
Pheochromocytoma is rare during pregnancy and the common symptoms and signs are headache, hypertension; therefore, it is often misdiagnosed for preeclampsia. Here is a rare case of pheochro
mocytoma in the second trimester of pregnancy presenting with acute abdominal pain. After pain relief, she could continue pregnancy with alphablocker to control her hypertension without surgery until 35 weeks of gestation. A healthy female neonate was delivered by cesarean section with con
current retroperitoneal mass excision due to relapsed abdominal pain. Pathological examination confirmed a pheochromocytoma with myxoid change.
Key Words: Pain, Pheochromocytoma, Pregnancy
Introduction
Pheochromocytoma is a catecholamine-secreting tumor with a variety of symptoms and signs including headache, hypertension, palpitation and sweating. Pheochromocytoma during pregnancy is rare and, because the symptoms of pheochromocytoma are usually similar with pregnancy-associated hypertension, antenatal diagnosis is difficult to be made and diagnosis would often be delayed in postpartum period.1,2 Adverse maternal and perinatal outcome would be high if it were undiagnosed during pregnancy but better outcomes have been reported in antenatally diagnosed cases.3,4 The treatment of pheochromocytoma during pre- gnancy may vary depending on the gestational age at diagnosis, so it is common to perform surgery if it is diagnosed by the early second trimester, and if diagnosed after late second trimester, medical treatment is preferred to relieve the patients’ symptoms and the surgery would be delayed until delivery.2
The most common presentation of pheochromocytoma during pregnancy is usually non- specific cardiovascular change associated with the production of catecholamine from the tumor and acute abdominal pain is rare.3,5 Few cases with pheochromocytoma presenting with acute abdominal pain have been reported in pregnant patients. Here, we report a case of pheochromocytoma in the second trimester of pregnancy with severe acute abdominal pain and successfully continued pregnancy with medical treatment with opioid analgesics and alpha-blocker until 35 weeks of gestation, when eventually cesarean section with excision was performed due to relapsed severe abdominal pain.
Case
A 36-year-old pregnant woman at 25 weeks of gestation was referred to our hospital due Received: 15 September 2020
Revised: 3 November 2020 Accepted: 29 November 2020 Correspondence to
Seung Yeon Pyeon, MD Department of Obstetrics and Gynecology, Kyung Hee University Hospital at Gangdong, 892 Dongnamro, Gangdonggu, Seoul 05278, Korea
Tel: +8224406268 Fax: +8224407022 E-mail: [email protected] Copyright© 2020 by The Korean Society of Perinatology
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mons Attribution NonCommercial License (http://creativecommons.org/
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Pheochromocytoma during Pregnancy with
Acute Abdominal Pain–a Case Report and
Review of Literature in Korea
also complained of mild dyspnea but no headache, epigastric pain and visual disturbance. Her vital sign was 153/100 mmHg blood pressure, 118/min pulse rate, and 36.5℃ body tempera- ture. Obstetric ultrasonography revealed unremarkable findings of fetus, amniotic fluid, and placenta. Her laboratory findings showed proteinuria, anemia and thrombocytosis. The ratio of protein/creatinine in random urine was 694 mg/g and hemoglobin was 9.9 g/dL, platelet was 684,000/uL. Plasma postprandial glucose levels of glucose challenge test were high. She was dia gnosed as superimposed preeclampsia, gestational diabetes and anemia with reactive thrombocytosis. One week later, she visited emergency department due to sudden onset of severe left upper abdominal pain with visual analogue scale score of 8. Her vital signs were 213/105 mmHg blood pressure, 78/min pulse rate, 36.4℃ body temperature. Random urinalysis showed proteinuria (2+) and urine protein/creatinine ratio was 1.082.
Upper abdominal ultrasonography showed a well-demarcated
heterogeneous area between the spleen and the left kidney measuring 10 cm with internal low echogenicity (Fig. 1A). Mag- netic resonance imaging revealed 10.0×7.7×6.2 cm mass with heterogenous low signal intensity in left adrenal gland sugges- tive of the pheochromocytoma (Fig. 1B). The level of 24 hour- urinary catecholamine metabolites demonstrated elevated frac- tionated metanephrine of 657 ug/24 h (reference, 52-341) and normetanephrine of 36,087 ug/24 h (reference, 88-444), and elevated vanillylmandelic acid (VMA) of 36 mg/24 h (reference, 0-8). Plasma normetanephrine level increased to 35.7 nmol/L (reference <0.9). From these results, the final diagnosis of pheo- chromocytoma was made. A multidisciplinary team, including Obstetrician, endocrinology and general surgery department, de cided to continue her pregnancy without a surgery because the enlarged uterus made access to the large mass in the retro- peritoneum by laparoscopy difficult. Her pain was controlled by opioid analgesics and alpha- blocker (doxazosin 4 mg) was
A B
Fig. 1. (A) Abdominal ultrasonography image shows a 10 cm sized well-demarcated heterogeneous area with internal low echogenicity between the spleen and the left kidney. (B) Sagittal section of T2-weighted image of magnetic resolution imaging shows about 10 cm sized mass in left adrenal gland (arrow).
A B
Fig. 2. (A) The cut surface of mass shows myxoid change in the center of tumor with focal hemorr- hagic area. (B) Microscopic examination of the tumor confirmed pheochromocytoma (hematoxylin and eosin, ×200).
very rare and the incidence has been estimated as 1 in 15,000 to 1 in 50,000 pregnancies.2 Generally poor maternal and perinatal outcome was reported as 29% of maternal mortality and 29% of fetal loss if it was not diagnosed antenatally, but reports have been seen with improved prognosis as no maternal death in an- tenatally diagnosed cases.6-8 Therefore, timely diagnosis and appropriate treatment is important to improve both the maternal and fetal outcomes. We reviewed cases of pheochromocytoma during pregnancy in Korea to determine the maternal and fetal outcome. We used the term “pregnancy and pheochromocy toma”
and affiliation “Korea” to search Pubmed for relevant English- language articles. In addition, we used the term “preg nancy and pheochromocytoma” to search KoreaMed for relevant Korean- language articles. Nine cases were included, except for the articles that overlapped with each other and one case diagnosed in 2 years after delivery; 5 cases were diag nosed before deli- very, 4 cases were diagnosed intrapartum and after delivery.
Expired case was only one. The brief review of these cases is given in Table 1.9-17 A systemic review of articles published in started to control her blood pressure. The patient was relieved
from symptoms and pregnancy was maintained until 35 weeks of gestation. Blood pressure was well controlled with alpha-blocker below 140/90 mmHg and heart rate ranged between 70 and 100 beats/min. However, the severe abdominal pain relapsed at 35 weeks of gestation. She underwent cesarean section followed by concurrent resection of tumor. The intraoperative period was uneventful and a female neonate weighing 2,790 g was delivered with Apgar score of 7 and 9 for 1 minute and 5 minutes after the birth, respectively. The pathologic examination confirmed the pheochromocytoma (Fig. 2). Her postoperative state was stable without any complications. Her vital signs were stable without any medications accompanied by normalization of urine catecholamine levels. Her post-partum period was uneventful.
Discussion
The prevalence of pheochromocytoma during pregnancy is
Table 1. Review of Reported Cases of Diagnosed Pheochromocytoma during Pregnancy in Korea Age
(years)
GA at diagnosis
(weeks) Symptoms at diagnosis Location of lesion
GA at delivery
(weeks) Indication of delivery Mode of delivery
Maternal morbidity
& mortality
Perinatal outcomes
Surgical excision of
tumor
Kim9 28 22 Headache, hypertension Right 39 VD 3.2 kg
A/S 9/10
25+6 weeks of gestation
Kim et al.10 35 28 Hypertension Left 33 Fetal asphyxia c/sec A/S 8/10 Postpartum
3 weeks Oh et al.11 26 26 Hypertension, tachycardia,
dyspnea, palpitation, blurred vision
Right 33 Suspected acute fatty liver due to elevated LFT
c/sec 2.7 kg
A/S 3/5
Concomitant excision with c/sec Jung et al.12 32 14 Hypertension, RUQ pain, RLQ
pain
Right 41 Placental abruption and fetal distress
c/sec 2.41 kg 22 weeks of
gestation
Jo et al.13 36 24 Headache, edema, palpitation Left 33 Fetal distress c/sec 1.405 kg
A/S 5/7
Postpartum 2 weeks Kim et al.14 32 Postpartum Hypertension, headache,
posterior neck pain, soaked in sweat
Left 37 Regular uterine con
tractions with fetal distress
c/sec 3.67 kg
A/S 8/9
Postpartum
Oh et al.15 28 Postpartum 1 month later
Hypertension, headache Right 32 Preeclampsia Maternal death Postpartum
4 weeks Kim et al.16 34 Postpartum Headache, palpitation, chest
discomfort, dyspnea, postural dizziness
Right VD Cardiomyopathy Postpartum
2 days
Kim et al.17 36 Postpartum Chest pain, headache, dyspnea
Right 38 Maternal MI c/sec 2.87 kg
A/S 2/5
Postpartum 2 weeks Abbreviations: GA, gestational age; VD, vaginal delivery; A/S, Apgar score; c/sec, Cesarean section; LFT, liver function test; RUQ, right upper quadrant; RLQ, right lower quadrant; MI, myocardial infarction.
pain might be caused by internal hemorrhage within the tumor although it was not accompanied by hemodynamic instability.
The treatment of the pheochromocytoma during pregnancy depends on the gestational age at diagnosis. Based on experts’
opinion and available studies, the tumor resection is recom- mended before 24 weeks’ gestation and after 24 weeks, the medical treatment was preferred and surgical resection delayed at or after delivery.2,3 In our opinion, acute abdominal pain itself in pheochromocytoma during pregnancy in the late second trimester is not an indication for surgery if the patient is hemo- dynamically stable, suggesting that pain can be controlled and monitored. In addition, it should be informed that complication such as acute abdominal pain may accompany the patient with pheochromocytoma while maintaining pregnancy by medical treatment. In conclusion, decisions must to be made on an indi- vidual basis by multidisciplinary team.
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