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Somatostatin Receptor 2 Negative Pheochromocytoma Masked by Normal Adrenal Gland on Gallium-68 DOTATATE

Year: 2024

Type: article

Abstract: Background/Objective Gallium-68 DOTATATE ( 68 Ga-DOTATATE) positron emission tomography/computed tomography (CT) is a somatostatin receptor (SSTR)-based imaging with high sensitivity that can be used for detection of pheochromocytomas and paragangliomas. We report a pheochromocytoma with negative SSTR2 expression and low uptake on 68 Ga-DOTATATE, whose detection was masked by the uptake of normal adrenal tissue. Case Report A 50-year-old man presented with a right adrenal incidentaloma. He had mildly elevated plasma normetanephrine levels of 194 pg/mL (ref. 0-145 pg/mL). Adrenal CT scan showed a right 1.9-cm lesion with unenhanced attenuation of 38 Hounsfield units. 68 Ga-DOTATATE showed a 1.9-cm right adrenal lesion and reported diffuse uptake in the adrenal glands, with maximum standardized uptake value (SUVmax) of 17.23 on the right and SUVmax of 22.78 on the left. After a 2-year interval, plasma normetanephrine level increased to 420 pg/mL (ref. 0-136.8 pg/mL). Adrenal CT scan showed the right adrenal lesion increased in size to 2.6 cm. He underwent right adrenalectomy, and pathology reported a 2.3-cm pheochromocytoma. Subsequent review of the initial 68 Ga-DOTATATE identified the pheochromocytoma as a photopenic area in the right adrenal gland with 7.73 SUVmax. Tissue staining was negative for SSTR2 expression. Genetic testing was negative for pheochromocytoma syndromes. Discussion Although 68 Ga-DOTATATE has strong affinity for SSTR2, some pheochromocytomas have low expression of SSTR2. The negative SSTR2 expression, small lesion size, and background uptake of the adrenal gland can affect the detection of pheochromocytoma. Conclusion 68 Ga-DOTATATE may have limitations when evaluating small pheochromocytomas or other neuroendocrine tumors with low SSTR2 expression.

Source: AACE Clinical Case Reports

DOI: 10.1016/j.aace.2024.12.011

Authors: Sanghwa E. Park, Thanh D. Hoang, Derek J. Stocker, Mohamed K.M. Shakir, Andrew J. Spiro

Institutions: Carl R. Darnall Army Medical Center, Walter Reed National Military Medical Center

Language: English

Topic: Adrenal and Paraganglionic Tumors

Subfield: Surgery

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $900