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Molecular Imaging and Radionuclide Therapy
E-ISSN: 2147-1959
18F-FDG PET/CT and 68Ga-DOTATATE PET/CT Findings of Polycystic Kidney-derived Paraganglioma
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18F-FDG PET/CT and 68Ga-DOTATATE PET/CT Findings of Polycystic Kidney-derived Paraganglioma

Mol Imaging Radionucl Ther 2024;33(3):182-184 DOI: 10.4274/mirt.galenos.2024.03264
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Abstract

Paragangliomas (PGLs) are neuroendocrine tumors originating from the neural crest. They usually arise from the adrenal medulla and sympathetic or parasympathetic ganglions. Approximately 10% of PGLs are located in the extra-adrenal gland. Renal PGL is a rare condition. In this case report, we present the 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and 68Ga-DOTATATE PET/CT findings of polycystic kidney-derived PGL.

Keywords: Renal paraganglioma, 18F-FDG PET/CT, 68Ga-DOTATATE PET/CT

Figure 1
Figure 1. A 46-year-old man with no known medical conditions presented to the urology outpatient clinic with a complaint of left side pain and abdominal swelling that had been ongoing for 3 weeks. Blood and urine tests were normal. Abdominal magnetic resonance imaging and ultrasound revealed a mass in the lower pole of the left kidney measuring approximately 10x15 cm. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) scan was performed with a preliminary diagnosis of renal cell cancer (RCC) (A). Transaxial CT sections showed a mass lesion with irregular borders measuring 8.5x15 cm that extended anteriorly from the inferior part of the left kidney and contained calcifications (B). Intense 18F-FDG uptake was detected on the fusion images [maximum standardized uptake value (SUVmax): 32.7] (C). There were no regional or distant metastases. Tru-cut biopsy of the mass was diagnosed as paraganglioma (PGL) (Figure 2). 68Ga-DOTATATE PET/CT imaging was performed for staging (F). Transaxial CT sections showed the left kidney to have a polycystic appearance, and a mass lesion measuring approximately 10x15 cm was present in the medial part of the left kidney (D). Intense DOTATATE receptor activation was observed on the fusion images (SUVmax: 75.8) (E). No regional or distant metastases were observed.

Extra-adrenal PGLs most commonly occur in the carotid body, vagal body, mediastinum, and retroperitoneum (1). Renal PGL is rare (1,2). In the genitourinary tract, the renal pelvis (4.9%) is the third primary site of PGLs, followed by the bladder (79.2%) and urethra (12.7%) (3). The clinical symptoms depend on the location of the mass in the kidney (2,4). Functional imaging methods play an important role in confirming diagnosis, staging, and restaging (5). PGLs contain high levels of somatostatin receptors (6,7). In a study evaluating 22 PGL patients with 68Ga-DOTATATE PET/CT, the detection rate of lesions was 100% (8). In another study evaluating 23 patients with PGLs using 18F-FDG PET/CT and 68Ga-DOTATATE PET/CT, many regions were 68Ga-DOTATATE and 18F-FDG positive, but 18F-FDG uptake was lower than 68Ga-DOTATATE uptake (median SUVmax values were 12.5-21, respectively) (9). 68Ga-DOTATATE PET/CT has higher diagnostic accuracy than 18F-FDG PET/CT (10). Renal PGL should be considered when evaluating kidney masses because it can mimic RCC, and caution should be exercised.

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Figure 2
Figure 2. Tumor cells are arranged in a Zellballen pattern within a fibrovascular stroma (A, H&E x200). The tumor is strongly positive for chromogranin-A (B, x200). S-100 immunostain highlighting the focally preserved sustentacular cells (C, x200). Strong cytoplasmic positivity for tyrosine hydroxylase confirms the diagnosis of PGL (D, x200).
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Footnotes

Ethics

Informed Consent: The patient consent was obtained.

Authorship Contributions

Surgical and Medical Practices: Z.T., S.B.K., N.E., T.F.Ç., E.A., Concept: Z.T., S.B.K., T.F.Ç., E.A., N.E., Design: Z.T., N.E., S.B.K., T.F.Ç., E.A., Data Collection or Processing: Z.T., S.B.K., N.E., T.F.Ç., E.A., Analysis or Interpretation: Z.T., S.B.K., N.E., T.F.Ç., E.A., Literature Search: Z.T., S.B.K., N.E., T.F.Ç., E.A., Writing: Z.T., T.F.Ç., E.A., S.B.K., N.E.

Conflict of Interest: No conflicts of interest were declared by the authors.

Financial Disclosure: The authors declare that this study has received no financial support.

References

1

Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and Paraganglioma. N Engl J Med. 2019;381:552-565.

CrossRefPubMedGoogle Scholar
2

Bahar B, Pambuccian SE, Gupta GN, Barkan GA. Renal paraganglioma: report of a case managed by robotic assisted laparoscopic partial nephrectomy and review of the literature. Case Rep Urol. 2014;2014:527592.

CrossRefPubMedGoogle Scholar
3

Peng C, Bu S, Xiong S, Wang K, Li H. Non-functioning paraganglioma occurring in the urinary bladder: A case report and review of the literature. Oncol Lett. 2015;10:321-324.

4

Yi C, Han L, Yang R, Yu J. Paraganglioma of the renal pelvis: a case report and review of literature. Tumori. 2017;103(Suppl 1):e47-e49.

CrossRefPubMedGoogle Scholar
5

Taïeb D, Timmers HJ, Hindié E, Guillet BA, Neumann HP, Walz MK, Opocher G, de Herder WW, Boedeker CC, de Krijger RR, Chiti A, Al-Nahhas A, Pacak K, Rubello D; European Association of Nuclear Medicine. EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2012;39:1977-1995.

CrossRefPubMedGoogle Scholar
6

Sobocki BK, Perdyan A, Szot O, Rutkowski J. Management of Pheochromocytomas and Paragangliomas: A Case-Based Review of Clinical Aspects and Perspectives. J Clin Med. 2022;11:2591.

CrossRefPubMedGoogle Scholar
7

Şahin R, Baykal Koca S, Yücetaş U, Çermik TF, Ergül N. 18F-FDG PET/CT and 68Ga-DOTATATE PET/CT Findings in a Patient With Primary Renal Well-Differentiated Neuroendocrine Tumor. Clin Nucl Med. 2022;47:e503-e505.

8

Skoura E, Priftakis D, Novruzov F, Caplin ME, Gnanasegaran G, Navalkissoor S, Bomanji J. The impact of Ga-68 DOTATATE PET/CT imaging on management of patients with paragangliomas. Nucl Med Commun. 2020;41:169-174.

CrossRefPubMedGoogle Scholar
9

Chang CA, Pattison DA, Tothill RW, Kong G, Akhurst TJ, Hicks RJ, Hofman MS. (68)Ga-DOTATATE and (18)F-FDG PET/CT in Paraganglioma and Pheochromocytoma: utility, patterns and heterogeneity. Cancer Imaging. 2016;16:22.

10

Carrasquillo JA, Chen CC, Jha A, Ling A, Lin FI, Pryma DA, Pacak K. Imaging of Pheochromocytoma and Paraganglioma. J Nucl Med. 2021;62:1033-1042.

CrossRefPubMedGoogle Scholar
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