Hyperprogression After Immunotherapy for Primary Small Cell Neuroendocrine Carcinoma of the Ureter: A Case Report

Abstract

BackgroundPrimary small cell neuroendocrine carcinoma (SCNEC) in the ureter is extremely rare and has been sporadically reported in case reports. Its incidence, diagnosis, treatment, and outcomes have not yet been thoroughly understood. Here we present a patient with advanced SCNEC in the ureter who was treated by multimodal strategies. To the best of our knowledge, this is the first literature report about the clinical outcomes of the combination of programmed death ligand 1 (PD-L1) immune checkpoint inhibitors (ICIs) and radiotherapy in patient with primary ureteral SCNEC.Case PresentationA 71-year old male presented with right flank pain and gross hematuria. A laparoscopic right nephroureterectomy was performed. He was diagnosed with primary ureteral SCNEC, pT3N0M0. Following the surgery, 4 cycles of adjuvant chemotherapy with carboplatin and etoposide (CE) were administered, with disease-free survival (DFS) of 10.1 months. He was then offered 4 cycles of palliative first-line chemotherapy with nedaplatin and irinotecan. The disease was continuously progressed, with progression-free survival (PFS) of 3.7 months. The patient subsequently received second-line treatment with PD-L1 ICI combined with radiotherapy. Unfortunately, hyperprogressive disease was found at the end of treatment. MRI and CT scan showed bilateral pubic bones, right acetabulum, and liver metastases. Without further intervention, the patient died from extensive metastatic disease 2 months after diagnosis, with overall survival (OS) of 18.2 months.ConclusionPhysicians must be aware of this rare and aggressive carcinoma at its initial presentation. Special attention should be paid to the potential likelihood of hyperprogression during the treatment.

Cardiac infarction caused by PD-1 inhibitor during small cell neuroendocrine carcinoma of the ureter treatment: A case report

“…Primary ureteral SCNEC is commonly seen in elderly men and is presented with gross hematuria and lumbar pain ( 4 ). However, with no recommendation for extensive or advanced disease, PD-1/PD-L1 inhibitor remains the treatment for ureteral SCNEC ( 6 ). Immune-related adverse events are a range of complications associated with the use of immune-checkpoint inhibitors what cantinas PD-1/PD-L1 inhibitors.…”

Imaging manifestations of small cell neuroendocrine carcinoma of the ureter

“…Small cell neuroendocrine carcinoma of the ureter is a highly malignant tumor originating from the metaplasia of urothelial cells with or without neuroendocrine function 1 . Due to its rare nature, ureteral SCNEC is always misdiagnosed or escapes diagnosis in the early stage and has a poor prognosis in the later stage on imaging 2 .…”

“…Small cell neuroendocrine carcinoma of the ureter is a highly malignant tumor originating from the metaplasia of urothelial cells with or without neuroendocrine function. 1 Due to its rare nature, ureteral SCNEC is always misdiagnosed or escapes diagnosis in the early stage and has a poor prognosis in the later stage on imaging. 2 However, SCNEC is usually detected by CT but rarely by ultrasonography and MRU, which could provide more features of ureteral SCNEC, including its composition, pattern, and blood flow.…”

Neuroendocrine Neoplasms of the Female Genitourinary Tract: A Comprehensive Overview

“…Primary ureteral NENs are rare, and no more than 50 cases of small-cell ureteral NEC have been reported in the literature [2,102]. Small-cell NEC of the ureter is usually observed in elderly patients, and around 15 cases have been observed in females so far [2,102,103]. As per our knowledge, less than 10 cases of large-cell NEC of the ureter [73,74] have been reported in the literature.…”

“…Endoscopy provides the biopsy specimen, which aids in diagnosing the mass by revealing malignant neuroendocrine cells [74,110]. A few may also present with paraneoplastic syndromes, which indicate advanced or extensive disease [102]. Ouzzane et al reported that 72% of patients presented with pT3 or pT4 stages, and 54% developed metastases within 13 months [108].…”