Neuroendocrine Renal Carcinoma – Therapeutic and Diagnostic Issues

Abstract

introduction. Neuroendocrine renal carcinoma represents less than 1% of all primary neoplasia of the kidney. Most frequently poorly differentiated carcinoma is diagnosed in advanced stages and they have an aggressive evolution and limited survival rate. Neuroendocrine carcinomas that arise from the renal pelvis are frequently associated with squamous cell carcinoma or adenocarcinoma.material and method. We present the case of a female patient, known for 3 years before with an undefined retroperitoneal lymph node metastasis, being diagnosed at present with a left large cell neuroendocrine renal carcinoma, who initially had lymph node metastasis.results. Until now, 118 cases of primary neuroendocrine renal carcinomas have been reported. A limited number of poorly differentiated neuroendocrine carcinomas have been reported.discussion. Due to the clinical and biological findings, the aggressive evolution with early metastasis of lung and bone, the patient is included in the group of poorly differentiated carcinomas. In these cases, multimodal treatment is a gold standard. After surgical treatment and palliative chemotherapy with platinum salts, we obtained a partial remission of the disease and the control of symptoms.conclusions. Regarding large cell neuroendocrine carcinoma, the surgical treatment remains the treatment of choice. Chemotherapy can determine limited results, improve the quality of life and enhance the overall survival rate.

Rarely Described Renal Malignancies Associated With Venous Tumor Thrombus

“…Lastly, neuroendocrine renal carcinoma (commonly referred to as carcinoid tumors), represents less than 1% of all primary renal malignancies, occurring both in the renal parenchyma, as well as in the renal pelvis (27,28). Patients with horseshoe kidneys have been reported to have up to 62to 82-fold higher risk of developing neuroendocrine tumors due to abnormal migration of posterior nephrogenic cells (6,7).…”

“…Adverse prognostic factors include age greater than 40 years, tumor size greater than 4 cm, mitotic rates higher than 1/10 high power fields, purely solid tumors and metastasis at initial diagnosis (28). Renal carcinoid tumors should be managed by radical nephrectomy, and good outcomes have been obtained for organ-confined disease after radical excision (27,31). Up to date, the biggest case series was published by Jain et al (32), in which 90 cases reports were reviewed, results showed that there is no gender predilection and age at diagnosis ranged between 23 to 78 years, however the mean age of diagnosis is lower than for RCC.…”

A Rare Case of a Large Primary Renal Neuroendocrine Tumour: A Case Report and Brief Review of Literature

“…Perhaps owing to their rarity, the pathophysiology of NENs is not completely understood. Cells of the neuroendocrine system are not usually present within the kidney, but one theory is that tumours could arise from the activation of aberrant gene sequences of multipotent stem cells, which are common to those of neuroendocrine differentiated cells [ 12 , 13 ]. Other theories regarding the origin of abnormal neuroendocrine cells within the kidney include metaplasia of the pyelocaliceal urothelium by chronic inflammation, metastasis from undiscovered primary tumours or entrapped neural crest tissue in the kidney during embryogenesis [ 13 ].…”

“…A significant number present asymptomatically, but for those with symptoms, flank or abdominal pain (with or without haematuria) are the most common [ 6 , 13 , 11 ]. Presentation with carcinoid syndrome or features of neuroendocrine crisis is rare, and most renal NENs are not found to be biochemically active [ 5 , 12 ]. On retrospective questioning, our patient did report some mild flushing episodes prior to diagnosis, but no diarrhoeal symptoms, and haematuria was not present despite the obliteration of the normal sinus architecture on microscopy.…”