Retroperitoneal extralobar pulmonary sequestration mimicking a pheochromocytoma

Lee, C K; Baliski, Christopher; Zetler, Peter

doi:10.1111/j.1365-2559.2008.02944.x

Cited by 4 publications

(1 citation statement)

References 11 publications

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“…The treatment remains controversial, mainly because the prenatal detected RPS has potential to resolve spontaneously [10], as well as to malignant transformation especially when coexists with congenital cystic adenomatoid malformation (CCAM) [11]. Most RPS are asymptomatic, although they link to potential complications, like compression of the normal structures [4] causing dysphagia, GERD, and RDS, pheochromocytoma-like symptoms [12], or high flow heart failure caused by the systemic blood supply [4]. We agree with the surgical indication of symptomatic lesions.…”

Section: Discussionmentioning

confidence: 97%

Prenatal detected retroperitoneal pulmonary sequestration with elevated serum levels of CA 19-9 – Case report and review of the literature

Zhao

Zhang

et al. 2013

Journal of Pediatric Surgery Case Reports

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a b s t r a c tWe present a case of prenatal detected retroperitoneal pulmonary sequestration (RPS) with an interesting finding. Immunohistochemical staining of the specimen indicated that the elevated carbohydrate antigen (CA) 19-9 serum level was very likely caused by the sequestration. A review of the literature was made. RPS is an infrequent congenital malformation, which can be detected by routine obstetric sonographic screening during prenatal life. It can be misdiagnosed as neuroblastoma. The differential diagnosis can be tricky, which we hope our finding can help with. The management remains controversial.Ó 2013 Elsevier Inc. All rights reserved. Case reportA left suprarenal fetal mass was discovered at 5 months' gestation on routine prenatal ultrasound scan of a 31-year-old gravida 1, para 0 woman. The mass was hyperechoic, measured 2.3 Â 1.8 cm at 28 weeks' gestation ( Fig. 1A). An magnetic resonance imaging (MRI) scan at the same time showed the lesion was hyperintense on T2-weighted ( Fig. 1B). To our knowledge, the most possible diagnosis was either neuroblastoma or adrenal hematoma. We applied the strategy of "wait and see." Three repeat ultrasound scans at 37 weeks' gestation, 1 week after born (full-term normal delivery at 38 week's gestation, mother and child didn't show any abnormal symptoms and physical signs), 4 weeks showed the persistence of the lesion. Further evaluation was performed because of the suspicion of malignancy at 6 weeks after birth. The urinary catecholamines assay, AFP, b-HCG were all normal. The carbohydrate antigen (CA) 19-9 was 53 ng/ml, when the normal upper limit was 38 ng/ml. The detection for neuroblastoma minimal residual disease in bone marrow from posterior superior iliac spine was negative. Computerized tomography (CT) showed a non-calcified heterogeneously enhanced solid mass in the left adrenal gland region (Fig. 1C). We made the decision of surgery for that neuroblastoma couldn't be excluded. At laparotomy, a 3 Â 2 Â 2 cm solid mass with capsule, rich in blood supply, was found in the retroperitoneal left suprarenal region. It was superior, posterior, medial to the adrenal gland, clinging to adrenal gland without any adhesion or infiltration. A complete dissection and removal was achieved. The histologic study of the specimen revealed the presence of dysplastic lung tissue (Fig. 1D). The diagnosis of retroperitoneal pulmonary sequestration (RPS) was finally made. Immunohistochemical staining of the specimen with a specific monoclonal antibody against CA 19-9 showed immunoreactivity (Fig. 1E). The postoperative course has been uneventful by 3 months follow-up. DiscussionPulmonary sequestration (PS) is a segment of lung that has no communication with the normal bronchial tree and receives blood supply from systemic arteries. The incidence is about 0.15e1.8% [1]. Extralobar pulmonary sequestrations (ELS), which account for 25% of prenatal detected lung lesions [1], are masses of lung parenchyma that have a distinct pleura. About 10e15% of ELS will occur in retroper...

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