Evaluation of a Nep-Score Threshold and the Derived Nep-D Score in Predicting Survival of Patients With Typical and Atypical Bronchial Carcinoids

Background: Typical and atypical bronchial carcinoids (TBC and ABC) display a wide range of clinical presentations and may behave very differently. Survival prognostic markers are necessary to better define therapeutic strategies. AIM: verify that the NEP-Score, recently proposed as prognostic score, can be applied in a homogeneous TBC and ABC cohort and identify a derivate prognostic marker taking into account clinical and pathological characteristics at diagnosis. Methods: Age, site of primary tumor, primary tumor surgery, symptoms, Ki67, timing of metastases of 64 patients including TBC and ABC were evaluated to calculate the NEP-Score at the end of follow-up (NEP-T). We then assessed a derivative score considering the NEP-Score at diagnosis (NEP-D): this score does not consider the appearance of new metastases during follow-up. We then considered the patients that were alive or dead at the end of follow-up (EOF). A NEP-Score threshold to predict survival was investigated. Results: live patients at EOF displayed a mean NEP-T and mean NEP-D significantly lower as compared to those that were dead. A NEP-T threshold >138 significantly predicts survival. ABC relapsed more frequently as compared to TBC. Male gender as well as previous malignancy were negative prognostic factors for survival. Conclusions: We found that NEP-Score is applicable to a series of bronchial neuroendocrine neoplasms. In addition, we propose NEP-D as a simple, quick and cheap prognostic score that can help clinicians in decision making. Moreover, the use of a NEP-D threshold can predict NEN aggressiveness and may be used to define the best personalized therapeutic strategy. Furthermore we found additional prognostic factors that together with the NEP-Score could improve prognosis evaluation at diagnosis by using easily accessible information.

NEP-Score Thresholds Predict Survival of Patients With Bronchial Carcinoids

Survival prognostic markers are extremely needed to better define therapeutic strategies in patients with bronchial carcinoids (BC). We aim to verify the applicability of the NEP-Score in a homogeneous BC cohort and identify a derivative prognostic marker, the NEP-Score at diagnosis (NEP-D) that does not consider new metastases during follow-up. Sixty-four patients (38 females, and 26 males, mean age at diagnosis 58.9 ± 1.7 years) with BC were retrospectively evaluated. NEP-Score was calculated at the end of follow-up (NEP-T). A derivative score, the NEP-Score at diagnosis (NEP-D) that does not consider new metastases during follow-up, was then assessed. Patients were subdivided according to their living status at the end of follow-up. A NEP-Score threshold was investigated to predict survival. Mean NEP-T and mean NEP-D were significantly lower in live patients at end of follow-up. A NEP-T cut-off >138 significantly predicts survival. Atypical BC relapsed more frequently than Typical BC. Male gender and previous malignancy were negative prognostic factors for survival. We confirmed NEP-Score applicability in BC and NEP-D utility, being the latter a simple, quick, and cheap prognostic score that can help clinicians in decision making. The identified NEP-D threshold can predict NEN aggressiveness and may be used to define the best personalized therapeutic strategy. In this context, a validation study is needed.

Bronchial Carcinoid Tumor in the Era of Covid-19 Pandemic: A Case Report

Introduction: Bronchial carcinoid tumors are rare, slow growing, malignant neuroendocrine tumors which arise from Enterochromaffin (Kulchitsky) cells. Early diagnosis is extremely important as the main stay of treatment is surgical excision. Presentation of case: We present a rare case of bronchial typical carcinoid tumor in a 27-year-old male who presented with a complaint of intermittent dry cough of 2 weeks’ duration associated with shortness of breath, low grade intermittent fever and loss of appetite. He was initially misdiagnosed as covid-19 pneumonia and was admitted to covid-19 treatment center. Right lung bi-lobectomy with regional lymph node resection was done and he was discharged home in good condition.Discussion: Majority of typical carcinoids are located in the central airways leading to bronchial obstruction with recurrent pneumonia, chest pain, wheezing and hemoptysis. Due to such nonspecific presentation most patients are misdiagnosed or diagnosed late. In the era of covid-19 pandemic, in addition to the wide spread anxiety on the health care workers access to diagnostic work up of patients with rare diseases like bronchial carcinoids is very challenging especially in resource limited areas as invasive diagnostic procedures like bronchoscopy are usually avoided in covid-19 suspected patients during the pandemic. Although hilar and mediastinal lymph nodes are the most common metastatic sites for typical carcinoids most lymphadenopathies are caused by a reactive inflammatory reaction.Conclusion: Bronchial carcinoids are rare, malignant neuroendocrine tumors with complete surgical resection being the only curative management. Although the world is over burdened by Covid-19 pandemic crisis, other lung pathologies are also still present in communities. In such challenging circumstance diagnosing rare pulmonary diseases like bronchial carcinoids need high index of suspicion and meticulous diagnostic work up. The outcome of typical carcinoids with lymph node metastasis is excellent with complete resection but close follow up is mandatory when dealing with larger tumors.

The role of 68Ga-DOTA derivatives PET-CT in patients with ectopic ACTH syndrome

Introduction and aim Ectopic ACTH secretion (EAS) is mostly secondary to thoracic/abdominal neuroendocrine tumours (NETs) or small cell-lung carcinoma (SCLC). We studied the diagnostic accuracy of CT with 68Ga-Dota derivatives (68Ga-SSTR) PET in localizing ACTH-secreting tumor in patients with EAS. Materials and methods 68Ga-SSTR-PET/CT was performed and compared with the nearest enhanced CT in 18 cases (16 primary and 2 recurrent neoplasms). Unspecific, indeterminate and false-positive uptakes were assessed using conventional imaging, follow-up or histology. Results We diagnosed 13 thoracic (9 primary and 2 recurrent bronchial carcinoids, 2 SCLCs) and 1 abdominal (pancreatic NET) tumors. Eight ACTH-secreting tumors were promptly identified at EAS diagnosis (’overt’, four pulmonary carcinoids with two recurrences and two SCLC); six EAS have been discovered during the subsequent follow-up (’covert’, five bronchial carcinoids and one pancreatic NET). At the time of EAS diagnosis, imaging was able to correctly detect the ACTH-secreting tumour in 8/18 cases (6 new diagnosis and 2 recurrences). During the follow-up, six out of initially ten ‘occult’ cases became ‘covert’. At last available follow-up, CT and 68Ga-SSTR-PET/CT were able to diagnose 11/18 and 12/18 ACTH-secreting tumours, respectively (11/14 and 12/14 considering only overt and covert cases, respectively). Four cases have never been localized by conventional or nuclear imaging (’occult EAS’), despite an average follow-up of 5 years. Conclusion The 68Ga-SSTR-PET/CT is useful in localizing EAS, especially to enhance positive prediction of the suggestive CT lesions and to detect occult neoplasms.