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Malignant Paraganglioma in Pregnancy

      A 38-year-old, G2, P1 woman at 25 weeks gestation was found to have a 20-cm multiloculated retroperitoneal mass on MRI (Figure 1). The incidentally found mass was removed 2 months after term delivery. Histopathology and hormonal findings led to the diagnosis of a non-functioning malignant paraganglioma. Additionally, SPECT/CT-MIBG showed a metastatic lesion on the patient's liver (Figure 2). After a course of combined radiotherapy and chemotherapy, the patient had not experienced recurrent lesions at 2 years of follow-up.
      Paraganglioma is classified according to its sympathetic hypersecretion and location. One-third of abdominal paragangliomas are non-functioning, and 50%–70% of them are malignant.
      • Liu S
      • Song A
      • Zhou X
      • Kong X
      • Li WA
      • Wang Y
      • Liu Y
      Malignant pheochromocytoma with multiple vertebral metastases causing acute incomplete paralysis during pregnancy: Literature review with one case report.
      Paraganglioma in pregnancy is rare, with less than 30 cases reported, and malignant paraganglioma in pregnancy is exceptionally.
      • Lenders JWM
      • Langton K
      • Langenhuijsen JF
      • Eisenhofer G
      Pheochromocytoma and Pregnancy.
      Bright lesions on T2-weighted MRI with avid enhancement after gadolinium administration is a classical finding; however, the appearance of paraganglioma can be quite variable in the presence of necrosis or hemorrhage.
      Consent: Consent to publish these images was obtained from the patient.

      References

        • Liu S
        • Song A
        • Zhou X
        • Kong X
        • Li WA
        • Wang Y
        • Liu Y
        Malignant pheochromocytoma with multiple vertebral metastases causing acute incomplete paralysis during pregnancy: Literature review with one case report.
        Medicine (Baltimore). 2017; 96: e8535
        • Lenders JWM
        • Langton K
        • Langenhuijsen JF
        • Eisenhofer G
        Pheochromocytoma and Pregnancy.
        Endocrinol Metab Clin North Am. 2019; 48: 605-617

      Linked Article

      • Paragangliome malin pendant la grossesse
        Journal of Obstetrics and Gynaecology Canada Vol. 44Issue 2
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          Une masse rétropéritonéale multiloculaire de 20 cm a été détectée par IRM chez une femme de 38 ans, G2P1, 25 SA (figure 1). La masse révélée de façon fortuite a été enlevée 2 mois après l'accouchement à terme. L'histopathologie et les analyses hormonales ont mené au diagnostic de paragangliome malin non fonctionnel. De plus, des examens d'imagerie par SPECT-CT et MIBG ont révélé une lésion métastatique sur le foie de la patiente (figure 2). Après une radiothérapie et une chimiothérapie combinées, la patiente ne présentait aucune lésion récidivante à 2 ans de suivi.
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