Sites of the Primary Tumor

Primary tumors may originate in all sites of sympathetic ganglia or paraganglia; in particular along the sympathetic paravertebral chain, in the adrenal medulla, in the organ of Zuckerkandl, and in the ganglion stellatum at the seventh cervical vertebral transverse process. As shown in Table 7.3, adrenal origin predominates and cervical sites are exceptional in stage 4S and stage 4. With improved imaging techniques the number of undetected primaries decreased from 5 % in the 1980s to 2 % in 2000 - 2003. In localized disease, the incidence of thoracic primaries is usually higher than that of adrenal origin.

It is still unclear whether the site of the primary tumor is associated with the biological properties of the disease. Conspicuously, cervical neuroblastomas are associated only rarely with distant metastases. In contrast, in nearly 90% of patients with metastatic disease the primary site is located in the abdomen.

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