Local management of unresectable lung atypical carcinoid tumor: a case report and review of literature

Abstract

Neuroendocrine tumors comprise a rare but increasing heterogeneous group of malignancies arising from neuroendocrine cells, most commonly from the lung and gastrointestinal tract. Due to the vast histopathological differentiation of each subtype and the scarce clinical data published, choosing the most effective therapy can be challenging. Radiotherapy can play a significant role in the treatment of locally advanced metastatic tumors, however there is a lack of randomized clinical trials in this setting. This article reviews the current knowledge on the classification and treatment of unresectable lung atypical carcinoids. We present a clinical case of a ULAC treated with systemic therapy and RT in different settings of the disease. The subject is a 48 years old male, diagnosed with a well differentiated pulmonary NET, classified as cT4N3M1b (supraclavicular and mediastinal adenopathies and an adrenal metastatic lesion) with disease progression after systemic treatment, and with superior vena cava compression. The primary tumor and involved nodal areas were treated to 54Gy/30 fractions using VMAT. SBRT was given to the metastatic left adrenal gland. Five months after RT, CT showed a volumetric reduction of <25% of the thoracic disease and adrenal gland’s lesion stability. The disease remained stable for the next year and a half, when local and distant progression occurred, starting systemic treatment. A year and a half later, the patient presented with brain metastasis and underwent radiosurgery. At last follow-up, 5 years after diagnosis, the patient maintains treatment with capecitabine and temozolomide and is clinically stable. Definitive RT should be considered in the management of ULAC to improve local control.