Signet Ring Cell Carcinoma of the Prostate Gland: A Review and Update

Abstract

Signet-ring cell carcinoma of the prostate gland (SRCCP) an uncommon and aggressive malignant tumour of the prostate gland which is characterized by histopathology examination features of compression of the nucleus into the form of a crescent by a large cytoplasmic vacuole. SRCCPs that have so far been reported have been either (a) primary tumours, metastatic tumours with the primary tumour elsewhere with the gastro-intestinal tract being the site of the primary tumour but the primary tumour could originate elsewhere, and additionally some reported SRCCPs have been classified as carcinoma of unknown primary. SRCCP could be a pure tumour or a tumour that is contemporaneously associated with other types of tumour including various variants of adenocarcinoma. SRCCP can manifest in various ways including: Incidental finding following prostatectomy that has been undertaken for a presumed benign prostatic hyperplasia, lower urinary tract symptoms, visible and non-visible haematuria, raised levels of serum PSA but some SRCCPs have been diagnosed with normal / low levels of serum PSA, there may be a history of dyspepsia in cases of metastatic signet-ring cell carcinoma in association with contemporaneous primary signet-ring cell carcinoma of the stomach or there may be a past history of surgical treatment for signet-ring cell carcinoma of the gastrointestinal tract, or bleeding from the gastrointestinal tract in cases of upper gastrointestinal tract and rectal bleeding as well as change in bowel habit for primary tumours of the anorectal region, retention of urine, and rarely a rectal mass in the case of SRCCP with an anorectal primary tumour. In order to exclude a primary signet ring cell carcinoma elsewhere, a detailed past medical history is required as well as radiology imaging including contrast – enhanced computed tomography (CECT) scan and contrast-enhanced magnetic resonance imaging (CEMRI) scan as well as upper gastrointestinal endoscopy and colonoscopy to exclude a primary lesion within the gastrointestinal tract. Diagnosis of SRCCP requires utilization of the histopathology and immunohistochemistry examination features of prostate biopsy, prostatic chips obtained from trans-urethral resection of prostate specimen or radical prostatectomy specimen. SRCCPs upon immunohistochemistry staining studies tend to show tumour that tend to exhibit positive staining for the following tumour markers as follows: PSA – positive staining for PSA has been variable in some studies, AE1/AE3, CAM 5.2, Ki-67 with a mean of 8%, PAS-diastase, Mucicarmine (50%), Alcian blue (60%), Alpha-methyl-acyl coenzyme A racemase (P504S), and Cytokeratin 5/6. SRCCPs also tend to exhibit negative staining for: Bcl2 (rare positive), and CEA (80%). Traditionally the treatment of Primary Signet-Ring Cell Carcinoma of the Prostate Gland has tended to be similar to the treatment of the traditional adenocarcinoma of the prostate gland which does include: hormonal treatment, radiotherapy, and surgery. Nevertheless, considering that primary SRCCPs and metastatic SRCCPs that have been reported in the literature have generally tended to be associated with an aggressive biological behaviour, even though there is no consensus opinion on the treatment of the disease it would be strongly recommended that these tumours that tend to be associated with rapid progress of the disease and poor survival there is an urgent need to treat all these tumours with aggressive surgery including radical prostatectomy plus adjuvant therapies including: radical radiotherapy, combination chemotherapy, selective prostatic angiography and super-selective embolization of the artery feeding the tumour including intra-arterial infusion of chemotherapy agents directly to the tumour, radiofrequency ablation of the tumour as well as irreversible electroporation of the tumour which should form part of a global multicentre study of various treatment options. With regard to metastatic signet-ring cell carcinomas of the prostate gland with a contemporaneous primary tumour elsewhere the primary tumour should also be treated by radical and complete excision of the primary tumour plus radical surgery and aggressive adjuvant therapy. Considering that SRCCPs have tendered not to respond well to available chemotherapy agents, there is need for urologists, oncologists, and pharmacotherapy research workers to identify new chemotherapy medicaments that would more effectively and safely destroy signet-ring cell tumours in order to improve upon the prognosis.