TY - JOUR
T1 - Treatment with somatostatin analogues and prrt in metastatic middle ear adenoma with neuroendocrine features
AU - Ferreira, Joana Lima
AU - Marques, Bernardo
AU - Menke-van der Houven van Oordt, C. Willemien
AU - de Herder, Wouter W.
AU - Brabander, Tessa
AU - Hofland, Johannes
N1 - Funding Information: JLF and BM thank the Portuguese Society of Endocrinology, Diabetes and Metabolism for the support of the international fellowship programme in Erasmus Medical Center, Rotterdam. Publisher Copyright: © 2021 The authors. Copyright: Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2021/5/1
Y1 - 2021/5/1
N2 - Middle ear adenomas with neuroendocrine features (ANEF) are rare, with an estimated 150 reported cases. They usually pursue an indolent clinical course. Four reported cases of middle ear ANEF with distant metastases were treated with surgery, external beam radiation therapy (EBRT) and chemotherapy. To date, no successful systemic treatment for malignant behaviour of this rare tumour has been reported. Long-acting somatostatin analogues (SSAs) and peptide receptor radionuclide therapy (PRRT) have been used in well-differentiated metastatic neuroendocrine tumours (NETs), but their use has never been described in cases of metastatic middle ear ANEF. We report two patients with grade 1 middle ear ANEF treated with surgery and EBRT. They had stable disease for several years, until clinical symptoms appeared and extensive metastases were detected on68Ga-DOTA0-Tyr3-octreotate (DOTATATE) PET/CT. Treatment with long-acting SSA was started, with stable disease for 1 year. Afterwards, despite undergoing local treatments, both patients presented progressive disease. Due to high-uptake metastases at68Ga-DOTATATE PET/CT, both cases underwent four cycles of PRRT with177Lu-DOTATATE, which secured disease control and improvement of quality of life in both. Similar to other well-differentiated NETs, SSA and PRRT could constitute efficacious therapeutic options in metastatic middle ear ANEF. Its neuroendocrine differentiation, potential to metastasize and somatostatin receptor type 2 expression prompt consideration and management of this disease as a neuroendocrine neoplasm.
AB - Middle ear adenomas with neuroendocrine features (ANEF) are rare, with an estimated 150 reported cases. They usually pursue an indolent clinical course. Four reported cases of middle ear ANEF with distant metastases were treated with surgery, external beam radiation therapy (EBRT) and chemotherapy. To date, no successful systemic treatment for malignant behaviour of this rare tumour has been reported. Long-acting somatostatin analogues (SSAs) and peptide receptor radionuclide therapy (PRRT) have been used in well-differentiated metastatic neuroendocrine tumours (NETs), but their use has never been described in cases of metastatic middle ear ANEF. We report two patients with grade 1 middle ear ANEF treated with surgery and EBRT. They had stable disease for several years, until clinical symptoms appeared and extensive metastases were detected on68Ga-DOTA0-Tyr3-octreotate (DOTATATE) PET/CT. Treatment with long-acting SSA was started, with stable disease for 1 year. Afterwards, despite undergoing local treatments, both patients presented progressive disease. Due to high-uptake metastases at68Ga-DOTATATE PET/CT, both cases underwent four cycles of PRRT with177Lu-DOTATATE, which secured disease control and improvement of quality of life in both. Similar to other well-differentiated NETs, SSA and PRRT could constitute efficacious therapeutic options in metastatic middle ear ANEF. Its neuroendocrine differentiation, potential to metastasize and somatostatin receptor type 2 expression prompt consideration and management of this disease as a neuroendocrine neoplasm.
UR - http://www.scopus.com/inward/record.url?scp=85108650188&partnerID=8YFLogxK
U2 - https://doi.org/10.1530/EDM-20-0172
DO - https://doi.org/10.1530/EDM-20-0172
M3 - Article
C2 - 33982661
SN - 2052-0573
VL - 2021
JO - Endocrinology, Diabetes and Metabolism Case Reports
JF - Endocrinology, Diabetes and Metabolism Case Reports
IS - 1
M1 - 20-0172
ER -