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Case Reports
. 2023 May 9;9(5):149-152.
doi: 10.1016/j.aace.2023.05.003. eCollection 2023 Sep-Oct.

Hypertensive Crisis in a Patient With a Functioning Mesenteric Paraganglioma: Dramatic Response to Octreotide Treatment

Affiliations
Case Reports

Hypertensive Crisis in a Patient With a Functioning Mesenteric Paraganglioma: Dramatic Response to Octreotide Treatment

Omayma T Elshafie et al. AACE Clin Case Rep. .

Abstract

Background/objective: To report a dramatic and immediate clinical and biochemical response during treatment with octreotide in a patient with a functioning mesenteric paraganglioma (PGL).

Case report: A 44-year-old woman was admitted with a severe hypertensive crisis and a blood pressure reaching 260/150 mm Hg. She was 2 months postpartum and had been previously diagnosed with pre-eclampsia. Secondary hypertension was suspected. This was confirmed by finding a 6 × 5-cm2 retroperitoneal mass located using 68-Gallium DOTA-octreotate positron emission tomography/computed tomography and a grossly elevated plasma catecholamine level of 93 000 pmol/L (normal reference range: 650-2433 pmol/L). Treatment was immediately started with high doses of long- and short-acting octreotide. After 6 weeks and before surgery, the patient was normotensive, with a blood pressure of 120/70 mm Hg and a norepinephrine level of 6000 pmol/L. The tumor resection was uneventful, and histology confirmed the diagnosis. Following the surgery, the patient remained normotensive without any medications.

Discussion: PGLs and pheochromocytomas are neuroendocrine tumors, and most have receptors for octreotide. This case and another patient previously reported responded dramatically to treatment with a high dose of octreotide. Earlier reports of patients failing to respond are likely to have been the result of using a smaller octreotide dose.

Conclusion: We conclude that high doses of short- and long-acting octreotide are valuable in severely hypertensive patients. Our experience suggests that octreotide is of value in other patients with PGLs and pheochromocytomas. The response is rapid, sustained, effective, and with minimal reported side effects. To the best of our knowledge, this is the first report of a hypertensive crisis in a functional mesenteric PGL.

Keywords: 68-Gallium DOTATATE PET/CT; octreotide; paraganglioma; plasma catecholamines.

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Conflict of interest statement

The authors have no multiplicity of interest to disclose.

Figures

Fig. 1
Fig. 1
Computed tomography of the abdomen. A, A left retroperitoneal mass measuring 6 × 5 cm2. B, Increased necrosis in the left retroperitoneal mass that demonstrates a minimal increase in interval size. Yellow arrow is the normal reference range (650-2433) pmol/L. CT = computed tomography.
Fig. 2
Fig. 2
A 68-Gallium DOTA–octreotate positron emission tomography/computed tomography whole-body scan showing a moderate somatostatin receptor–avid retroperitoneal mass, consistent with paraganglioma. DOTATATE PET/CT = DOTA–octreotate positron emission tomography/computed tomography.
Fig. 3
Fig. 3
Serum norepinephrine levels before and during treatment with octreotide.
Fig. 4
Fig. 4
A, Hematoxylin and eosin staining at ×40 magnification showing a “zellballen” pattern. B, Immunohistochemical evaluation positive for chromogranin A.

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