Abstract
Paragangliomas (PGLs) are neuroendocrine tumors that arise embryologically from the
neural crest. Sympathetic PGLs can be located in the thoracic-abdominal region while
parasympathetic PGLs are mainly situated in the head and neck region. Most PGLs are
sporadic, but in 30% of cases they are hereditary (associated with mutations of SDHB,
SDHC, SDHD, SDHAF2, SDHA, TMEM, MAX, and VHL); they can be classified into 4 different
paraganglioma syndromes: PGL1, PGL2, PGL3, and PGL4. Surgery is the treatment of choice
for both sympathetic and parasympathetic PGLs. Other types of treatment include medical
agents (such as gemcitabine, cisplatin, or sunitinib) and radiotherapy (external-beam
radiotherapy or stereotactic surgery). Surgery and radiotherapy, however, can cause
important side effects such as vascular complications and peripheral nerve damage
(hypoglossal, recurrent laryngeal, glossopharyngeal, and vagus). Another possible
treatment option is the use of peptide receptor radionuclide therapy (PRRT), including
PRRT with 177Lu-DOTATATE. We studied 4 patients with hereditary nonmetastatic paraganglioma syndrome
type 1 (PGL1), with progressive disease, in whom surgical excision was not possible.
They were treated with 177Lu-DOTATATE (3–5 cycles) and all had a partial response (PR) or a stable disease (SD)
to the treatment. In conclusion, a good alternative treatment when surgical or radiation
therapy are contraindicated could be radiometabolic therapy with 177Lu-DOTATATE.
Key words
177Lu-DOTATATE - paraganglioma - hereditary
