What are the other Names for this Condition? (Also known as/Synonyms)

• Large Cell Neuroendocrine Carcinoma of the Urogenital System
• LCNEC of the Genitourinary System
• LCNEC of the Urogenital System

What is Large Cell Neuroendocrine Carcinoma of the Genitourinary System? (Definition/Background Information)

• Large Cell Neuroendocrine Carcinoma (LCNEC) of the Genitourinary System is a rare high-grade neuroendocrine tumor that is seen in the genitourinary tract; the genitourinary or urogenital system includes the urinary and genital organs
• Neuroendocrine tumors (NETs) are tumors showing neuroendocrine differentiation
• LCNECs are rare tumors that occur in a variety of anatomical locations in the male genitourinary tract including the kidney, urinary bladder, prostate gland, ureter, and penis (in very rare cases)

Incidence:

• Large Cell Neuroendocrine Carcinoma of the Genitourinary System is an exceedingly rare tumor; it is less commonly observed than a small cell neuroendocrine carcinoma (SCNEC) 
• A vast majority of cases are noted in adults
• Both males and females are affected
• All races and ethnic groups are at risk

Risk factors: The risk factors for Large Cell Neuroendocrine Carcinoma of the Genitourinary System are presently unknown.

Cause: The cause of tumor formation is presently unknown.

Signs and symptoms:

• The tumors can arise in a variety of anatomical locations including the kidney, urinary bladder, prostate, ureter, urethra, and penis
• The signs and symptoms of the tumor depends on the location of the tumor
  • Large cell neuroendocrine tumors of kidney present with flank (back) pain and blood in urine (hematuria)
  • LCNECs of the urinary bladder present blood in urine as the presenting symptom
  • Tumors arising in the ureter may cause hydronephrosis, due to obstruction of the urinary tract
  • The tumors that arise in other locations, such as the prostate, result in signs and symptoms related to urinary bladder obstruction
• The size of these tumors varies depending on the location
  • Kidney LCNEC can be large, and in many cases over 10 cm along the greatest dimension
  • Tumors at other locations, such as the bladder, prostate, and urethra, are usually smaller and varies between 1-9 cm

Diagnosis:

• Blood and urine tests
• Radiological studies
  • Ultrasound of abdomen
  • CT and MRI scan of abdomen
• Tissue biopsy of the tumor is important to arrive at a definitive diagnosis

Complications:

• Emotional stress
• Tumor metastasis to different organs and regions
• Incomplete removal resulting in tumor recurrence

Treatment:

• The staging is based on American Joint Committee on Cancer (AJCC) classification
• Treatment depends on the location and the size of the tumor
• It may require a combination of surgery, chemotherapy, and radiation therapy

Prevention: Presently, it is not possible to prevent the formation of Large Cell Neuroendocrine Carcinoma of the Genitourinary System.

Prognosis:

• The prognosis depends on the site of the tumor and also the stage of the tumor
• These tumors are typically high grade and have a poor prognosis