Surgical resection of an adrenocorticotropic hormone-producing pulmonary typical carcinoid with mediastinal lymph node metastasis and high programmed death-ligand 1 expression.

Abstract

The programmed death-ligand 1 positivity rate in typical carcinoid is generally low; however, cases with programmed death-ligand 1 expression are more frequently associated with lymph node metastasis and have a poorer prognosis. While immune checkpoint inhibitors were incorporated into adjuvant chemotherapy regimens for programmed death-ligand 1-positive non-small cell lung cancer, their efficacy in pulmonary carcinoids remains unclear. A 47-year-old woman presented to a local clinic with moon face. Laboratory testing revealed elevated levels of adrenocorticotropic hormone and cortisol and adrenocorticotropic hormone-dependent Cushing's syndrome was suspected. For further evaluation, the patient was referred to our hospital. Computed tomography revealed a nodule in the left lower lobe of the lung and enlargement of the left hilar lymph node, suggesting ectopic Cushing's syndrome. Left lung cancer (cT1bN1M0, cStage IIB) was suspected, and the patient underwent robot-assisted thoracoscopic left lower lobectomy with ND2a-2 lymph node dissection for diagnostic and therapeutic purposes. Pathological examination confirmed a diagnosis of typical carcinoid (pT1bN2M0, pStage IIIA) with high programmed death-ligand 1 expression. Considering the presence of mediastinal lymph node involvement and programmed death-ligand 1 expression, the patient received four courses of cisplatin and vinorelbine, followed by treatment with atezolizumab. Postoperatively, her adrenocorticotropic hormone levels normalized, and the patient has been alive 18 months postoperatively without recurrence. Programmed death-ligand 1-positive typical carcinoids are associated with a higher frequency of lymph node metastasis and poorer prognosis. Additional case investigations are required to assess the efficacy of immune checkpoint inhibitors in typical carcinoid.