Systemic treatment of a metastatic carotid body tumor: A case report and literature review.

RATIONALE: Carotid body tumors (CBTs) are head and neck paragangliomas (PGLs) with a low incidence of distant metastasis. To date, only a few metastatic cases treated with detailed systemic therapy are reported and effective management is still inconclusive. Herein, we reported a metastatic CBT case with systemic therapy and reviewed the reported systemic treatment. PATIENT CONCERNS: A 56-year-old man noticed multiple painless nodules on the right side of the neck and developed debilitating chest and back pain 7 years after the CBT resection. DIAGNOSES: Widespread bone and lymph nodes CBT metastases. INTERVENTIONS: Biopsies of the enlarged lymph nodes confirmed the diagnosis of metastatic CBT and 18F-FDG PET-CT detected multiple right cervical lymph nodes and bone metastases. 24 cycles of cyclophosphamide, vincristine and dacarbazine (CVD) chemotherapy were given since May 2016 to Jul 2018 and dacarbazine maintenance therapy was given in the next 15 months follow-up period. OUTCOMES: Partial remission was achieved according to the Response Evaluation in Criteria in Solid Tumors 1.1 criteria. A prominent control in the metastatic lesions were also observed in 18F-FDG PET-CT scan. LESSONS: Evidence for systemic management of metastatic CBTs is mainly based on studies of PGLs and pheochromocytoma. According to our review on metastatic CBT cases treated with systemic therapy from 1981 to 2018, chemotherapy, especially the CVD regimen, was a common reported management. In SDHB mutated patients, sunitinib and temozolomide could also be considered.

[Individualized treatment of bilateral carotid body tumor].

Objective: To investigate individualized therapeutic strategy for bilateral carotid body tumors. Methods: Clinical data of 16 patients with bilateral carotid body tumor treated from January 2003 to August 2016 were retrospectively studied. Of the 16 patients, 9 were males and 7 were females; 5 were sporadic and 11 were familial; 8 cases were observed, 1 cases was malignant and treated with chemotherapy, and 7 cases were treated with surgery. The treatment course, perioperative complications and clinical efficacy were assessed. Comprehensive evaluation of bilateral carotid body tumors was performed based on the size of bilateral tumor, clinical manifestations, genetic tests and other indicators. Individual treatment strategies included observation, surgery and observation, bilateral surgery, radiotherapy or chemotherapy. Surgical resection of carotid body tumor was unilateral in 3 cases and bilateral in 3 cases; removal of bilateral carotid body tumors plus unilateral jugular bulb in 1 case; and the internal carotid artery was reconstructed with autologous greater saphenous vein in 1 case. Results: All patients were followed up for 3 months to 12 years. There was no patient death during perioperative period. Superior laryngeal nerve injury occurred in 2 case. Baroreceptor failure syndrome occurred in one patient, but it gradually recoverd with medical treatments. Conlusion: It is important to identify whether bilateral carotid body tumors are hereditary and to make an individualized therapeutic strategy for each patient with bilateral carotid body tumors, focusing on the improvement in the quality of life of patient.

Peptide receptor radionuclide therapy with (177)Lu DOTATATE in a case of recurrent carotid body paraganglioma with spinal metastases.

Paragangliomas are rare benign neuroendocrine tumors, and 80% of all paragangliomas are either carotid body tumors or glomus jugulare tumors. We present a case of recurrent unresectable carotid body paraganglioma with nodal and T7 vertebral metastases in a 30-year-old man 6 years postsurgery detected with Ga DOTANOC PET/CT and was administered with peptide receptor radionuclide therapy using Lu DOTATATE. After 5 cycles of Lu DOTATATE (total cumulative activity of 750 mCi [27 GBq]), significant response at the primary site on Ga DOTANOC PET/CT and complete disappearance of nodal and T7 vertebral metastases were noted.

PET scan assessment of chemotherapy response in metastatic paraganglioma.

Paragangliomas are indolent tumors that arise from the chief cells of the paraganglia in the head and neck, mediastinum, and retroperitoneal regions. Less than 10% of paragangliomas metastasize. Paragangliomas are known to regress slowly and usually partially after radiation therapy, which has been attributed to the development of fibrosis within the abundant vascular elements of the tumor. Positron emission tomography (PET) scanning was used to monitor a 33-year-old woman with recurrent paraganglioma of the carotid body with lung and bone metastases before and after chemotherapy with cyclophosphamide, doxorubicin (Adriamycin), and dacarbazine. The patient derived clinical benefit from chemotherapy, with marked improvement of her systemic and respiratory symptoms, improvement of cancer-related anemia, and normalization of chromogranin A levels. A response was demonstrated on PET scan with decreased [18F] fluoro-2-deoxy-d-glucose uptake after chemotherapy, but no significant changes were detected on serial computed tomography (CT) scans. The patient has remained free of disease progression 24 months after chemotherapy completion. It is suggested that metabolic imaging with PET scans is superior to anatomical imaging with CT scans for the monitoring of patients with paragangliomas.

111In-pentetreotide scintigraphy is superior to 123I-MIBG scintigraphy in the diagnosis and location of chemodectoma.

Chemodectomas, or glomus tumours, are unusual head and neck paragangliomas. A non-invasive imaging technique, 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy, has long been used for the diagnosis of all types of paraganglioma. The aim of this study was to evaluate and compare classic 123I-MIBG scintigraphy with the more recent 111In-pentetreotide scintigraphy in the diagnosis and location of chemodectomas. We performed 123I-MIBG and 111In-pentetreotide scintigraphy in eight patients (7 females, 1 male) with histologically or radiologically confirmed chemodectomas (five carotid body and three jugulotympanic chemodectomas). 123I-MIBG uptake was visualized in four patients on planar views and SPET images (sensitivity 50%); uptake was low in three patients. Using 111In-pentetreotide scintigraphy, all chemodectomas in eight patients were visualized (sensitivity 100%) and 111In-pentetreotide uptake was high in all cases. In conclusion, our results indicate that 111In-pentetreotide scintigraphy is superior to 123I-MIBG scintigraphy in the diagnosis and location of chemodectomas. In-pentetreotide or 123I-MIBG uptake suggests a neuroendocrine origin, providing important functional information in the diagnosis of chemodectomas. Moreover, 111In-pentetreotide scintigraphy permits a good classification of patients with or without somatostatin receptors in the chemodectoma in the application of pharmacological therapy with somatostatin analogues to inoperable tumours. The main therapeutic action of cold somatostatin analogues is to inhibit hormonal hypersecretion in different neuroendocrine tumours. In chemodectomas, however, the most important effect of somatostatin analogues is to reduce tumour volume or inhibit growth progression.

[Detection of somatostatin receptors in tumors in the area of the head and neck and their clinical importance]. / Nachweis von Somatostatinrezeptoren in Tumoren des Kopf-Hals-Bereiches und ihre klinische Bedeutung.

The development of a radiolabelled somatostatin analogue Indium-111-Pentetreotide makes the detection of somatostatin receptor-bearing tumours by scintigraphic techniques possible. The existence of high-affinity binding sites for somatostatin has been described previously for most endocrine active tumours of the gastroenteropancreatic system (GEP), malignant lymphomas, small cell lung carcinomas, a subgroup of breast tumours and several types of neuroendocrine related human tumours. Using this new diagnostic tool we investigated some head and neck tumours of neuroendocrine origin (carcinoid of larynx, Merkel cell carcinoma, paragangliomas) with the newly developed radiolabelled somatostatin analogue Indium-111-Pentetreotide whether in vivo visualisation of somatostatin receptors might be possible. In cases not accessible for surgery but with a positive receptor status we started a specific therapy with the somatostatin analogue octreotide. The preliminary results suggest that this new isotopic scanning technique in a diagnostic tool and a predictive method for an effective therapy of those head and neck tumours which revealed highly specifically a positive receptor status. The therapeutical results using the somatostatin analogue octreotide indicate that this new concept is an ideal therapeutic strategy for those neuroendocrine head and neck tumours which cannot be controlled by surgical procedures.