The Place of Somatostatin Receptor Scintigraphy and Other Functional Imaging Modalities in the Setting of Pheochromocytoma and Paraganglioma

Hubalewska‐Dydejczyk, Alicja; Timmers, Henri J L M; Trofimiuk–Müldner, Małgorzata

doi:10.1002/9781119031659.ch10

Cited by 1 publication

(2 citation statements)

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“…PCPG are neuroendocrine tumors arising from chromaffin cells, which can produce catecholamines. e most common location is the adrenal glands (Pheo), while extra-adrenal tumors are called PGL [5]. e classical clinical presentation of functioning PCPG includes paroxistic hypertension, headache, and diaphoresis; a positive family history may be found in 30-40% of cases.…”

Section: Discussionmentioning

confidence: 99%

“…Functional imaging modalities may also be useful adjuncts for both diagnosis and workup for metastatic disease. Somatostatin receptor scintigraphy has been recently proposed as a useful method for localizing PGL, particularly in the head and neck [5]. We hereby present a case of head and neck catecholamine producing PGL, emphasizing on the importance of an individualized approach by a multidisciplinary team to guide diagnosis, treatment, and surveillance protocols based on current management recommendations.…”

Section: Introductionmentioning

confidence: 98%

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Current Approach of Functioning Head and Neck Paragangliomas: Case Report of a Young Patient with Multiple Asynchronous Tumors

Hernández-Hernández¹,

Vera²,

Guinto-Nishimura³

et al. 2020

Case Reports in Endocrinology

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Introduction. Pheochromocytomas (Pheo) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells of the adrenal medulla and from the extra-adrenal autonomic paraganglia, respectively. Only 1–3% of head and neck PGL (HNPGL) show elevated catecholamines, and at least 30% of Pheo and PGL (PCPG) are associated with genetic syndromes caused by germline mutations in tumor suppressor genes and proto-oncogenes. Clinical Case. A 33-year-old man with a past medical history of resection of an abdominal PGL at the age of eleven underwent a CT scan after a mild traumatic brain injury revealing an incidental brain tumor. The diagnosis of a functioning PGL was made, and further testing was undertaken with a PET-CT with 68Ga-DOTATATE, SPECT-CT 131-MIBG, and genetic testing. Discussion and Conclusion. The usual clinical presentation of functioning PCPG includes paroxistic hypertension, headache, and diaphoresis, sometimes with a suggestive family history in 30–40% of cases. Only 20% of PGL are located in head and neck, of which only 1–3% will show elevated catecholamines. Metastatic disease is present in up to 50% of cases, usually associated with a hereditary germline mutation. However, different phenotypes can be observed depending on such germline mutations. Genetic testing is important in patients with PCPG since 31% will present a germline mutation. In this particular patient, an SDHB gene mutation was revealed, which can drastically influence the follow-up plan and the genetic counsel offered. A multidisciplinary approach is mandatory for every patient presenting with PCPG.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 98%