Contemporary nuclear medicine imaging of neuroendocrine tumours.

Neuroendocrine tumours (NETs) are rare, heterogeneous, and often hormonally active neoplasms. Nuclear medicine (NM) imaging using single photon- and positron-emitting radiopharmaceuticals allows sensitive and highly specific molecular imaging of NETs, complementary to anatomy-based techniques, such as computed tomography (CT) and magnetic resonance imaging (MRI). Somatostatin-receptor scintigraphy is a whole-body imaging technique widely used for diagnosis, staging and restaging of NETs. The increasing availability of hybrid single-photon emission CT (SPECT)/CT cameras now offers superior accuracy for localization and functional characterization of NETs compared to traditional planar and SPECT imaging. The potential role of positron-emission tomography (PET) tracers in the functional imaging of NETs is also being increasingly recognized. In addition to 2-[(18)F]-fluoro-2-deoxy-d-glucose (FDG), newer positron-emitting radiopharmaceuticals such as (18)F-dihydroxyphenylalanine (DOPA) and (68)Ga-1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid (DOTA) peptides, show promise for the future. This article will summarize the role of current and emerging radiopharmaceuticals in NM imaging of this rare but important group of tumours.

Spectrum of catecholamine-secreting tumors of the organ of Zuckerkandl.

In a series of 24 pheochromocytomas, four were tumors in the organ of Zuckerkandl. Their presenting symptoms of headache, palpitation, diaphoresis, and anxiety were similar to symptoms of adrenal pheochromocytomas. Two patients had paroxysmal and two patients sustained hypertension. All four had elevated urinary vanillylmandelic acid, metanephrine or catecholamine levels. Two of the tumors were localized with angiography before the availability of computerized tomography. Two patients underwent initial computerized tomography, which was nondiagnostic. Later scans looking specifically for organ of Zuckerkandl tumors were positive. Organ of Zuckerkandl tumors, like other ectopic pheochromocytomas, have a higher rate of malignancy. Two of the four were malignant tumors. One patient died 4 years after operation, but the other patient remains stable despite metastatic disease 8 years after operation. The two patients with benign tumors remain well 2 and 3 years after resection and treatment with alpha-blockade.

[Pheochromocytoma of Zuckerkandl's organ. Value of angiographic study. Apropos of a case]. / Phéochromocytome de l'organe de Zuckerkandl. Intérêt de l'exploration angiographique. A propos d'un cas.

The authors report a case of pheochromocytoma of Zuckerkandl's organ revealed by permanent hypertension. Angiography, combined with the clinical and biological data suggesting a catecholamine secreting tumor, made the diagnosis of pheochromocytoma of Zuckerkandl's organ prior therapy.

[Paraganglioma of the organs of Zuckerkandl. A case report (author's transl)]. / Paragangliome de l'organe de Zuckerkandl. A propos d'un cas.

Endocrine manifestations were absent in a patient presenting with pain in the left peri-umbilical region from a paraganglioma of the organs of Zuckerkandl. The authors describe the embryology of paragangliomas and review the published literature concerning the noradrenaline-secreting forms of these tumors. Only 86 references to those of Zuckerkandl, situated on the side of the aorta in relation to the inferior mesenteric artery, were found. Diagnosis of non-secreting forms is confirmed by arteriography which in this present case demonstrated three pedicles arising from the inferior mesenteric, celiac, and one of the lumbar arteries respectively. The only evidence of malignancy is the presence of metastases. Their diagnosis is therefore difficult but essential as one-third of the localisations of malignant pheochromocytomas (10 p. cent of pheochromocytomas) outside of the adrenals affect the organs of Zuckerkandl.

[Secreting paragangliomas of the organ of Zuckerkandl. A report on two cases (author's transl)]. / Paragangliomes sécrétants de l'organe de Zuckerkandl. Apropose de deux cas

The authors describe two cases of secreting paragangliomas of the organ of Zuckerkandl, beinging the number of known cases to 78. They underline the interest of arteriographic and phlebographic explorations which define more accurately the origin and spread of these tumours.