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Case Reports
. 2022 Jan 24;15(1):27-33.
doi: 10.1159/000520384. eCollection 2022 Jan-Apr.

A Rare Case of Simultaneous Pheochromocytoma Accompanied with an Ileocecal Neuroendocrine Tumor

Karim M Eltawil  1   2 , Carly Whalen  1 , Julie N Leal  1 , John Kelly MacDonald  2   3   4 , Owen Prowse  2   5
Affiliations
Case Reports

A Rare Case of Simultaneous Pheochromocytoma Accompanied with an Ileocecal Neuroendocrine Tumor

Karim M Eltawil et al. Case Rep Oncol. .

Abstract

The detection rates of neuroendocrine tumors (NETs) are rising, which has led to a better understanding of their histopathologic characteristics. We present a case of adrenal pheochromocytoma with an incidental finding of a concurrent ileocecal NET detected on early post-adrenalectomy imaging. A 59-year-old male was noted to have microscopic hematuria on a routine workup. Further imaging identified a right adrenal lesion suggestive of pheochromocytoma. He was reporting intermittent headaches, and his urinary normetanephrine and norepinephrine levels were elevated. He underwent a right retroperitoneal adrenalectomy, and pathology confirmed a pheochromocytoma with no malignancy. Postoperatively, he developed a hematoma at the surgical bed that was identified on CT scan. An incidental lesion at the level of the ileocecal valve was noted on the CT scan as well. This was further investigated by colonoscopy and biopsy that confirmed a carcinoid tumor. He had no gastrointestinal symptoms, chromogranin A was normal, and 24-h urinary 5-hydroxyindolacetic acid levels were slightly elevated. He subsequently underwent a laparoscopic right hemicolectomy 3 months from his initial adrenalectomy. Final pathology confirmed a grade 2 NET with metastatic involvement of 4 regional lymph nodes. We report an extremely rare case of simultaneous pheochromocytoma accompanied with an ileocecal NET. Genetic testing for multiple endocrine neoplasia was undertaken and was found negative for 9 tested genes.

Keywords: Computed tomography; Neuroendocrine tumor; Pheochromocytoma.

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Conflict of interest statement

The authors declare that they have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
CT showing avidly enhancing right adrenal nodule measuring up to 3.5 cm.
Fig. 2
Fig. 2
CT showing soft tissue density at the ileocecal valve measuring approximately 3 cm.
Fig. 3
Fig. 3
Open surgical specimen showing tumor involving the ileocecal valve area.
See this image and copyright information in PMC

References

    1. Cives M, Strosberg J. An update on gastroenteropancreatic neuroendocrine tumors. Oncology. 2014;28:749–58. - PubMed
    1. Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares J, et al. One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063–72. - PubMed
    1. Dasari A, Shen S, Halperin D, Zhao B, Zhou S, Xu Y, et al. Trends in the incidence, prevalence and survival outcomes in patients with neuroendocrine tumors in the Unites States. JAMA Oncol. 2017;3:1335–42. - PMC - PubMed
    1. Frilling A, Åkerström G, Falconi M, Pavel M, Ramos J, Kidd M, et al. Neuroendocrine tumor disease: an evolving landscape. Endocr Relat Cancer. 2012;19((5)):R163–85. - PubMed
    1. Mittendorf EA, Evan DB, Lee JE, Perrier ND. Pheochromocytoma: advances in genetics, diagnosis, localization and treatment. Hematol Oncol Clin North Am. 2007;21:509–25. - PubMed

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