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

• Glomus Tumor of the Urogenital System

What is Glomus Tumor of the Genitourinary System? (Definition/Background Information)

• Glomus Tumors are a group of uncommon tumors caused by the proliferation of glomus cells, which are found in the glomus body. The glomus body is a small organ found in large numbers all over the body. It controls and helps regulate the body temperature and blood flow
• Glomus Tumor of the Genitourinary System is a rare and mostly benign tumor that is seen in the genitourinary tract; the genitourinary or urogenital system includes the urinary and genital organs
• In the genitourinary system, most glomus tumors are noted in the kidney, but extremely rarely in the urinary bladder and testis

Incidence:

• It may affect individuals of any age, though it is frequently observed in young adults
• In kidney, tumors are seen at any age (average age 50 years)
• Both males and females are equally affected (in renal tumors)
• All races and ethnic groups are at risk

Risk factors:

• Positive family history
• The genetic syndrome neurofibromatosis type 1 (NF1)

Cause:

• Since glomus bodies are absent in the urogenital organs, the origin of Glomus Tumor of the Genitourinary System is presently unknown
• When multiple tumors are noted, mostly in familial cases, the involvement of GLMN gene may be seen
• When seen with neurofibromatosis type 1, the inactivation of NF1 gene is observed
• Over 50% tumors show NOTCH2 gene rearrangements
• Malignant tumors may present mutations involving the BRAF gene (specifically BRAF p.V600E mutations)

Signs and symptoms:

• The tumors can arise in the following anatomical locations
  • Kidney (mostly)
  • Urinary bladder (rarely)
  • Testis (rarely)
• Depending on the location of the tumor the signs and symptoms vary
• Typically, small and well-defined tumors are noted
• Kidney tumors can cause abdominal pain
• Urinary bladder tumors can present blood in urine

Diagnosis: A vast majority of the kidney tumors are detected incidentally.

• Blood and urine tests
• Radiological studies
  • Ultrasound of abdomen
  • CT and MRI scan of abdomen
• Tissue biopsy of the tumor mass can help achieve a definitive diagnosis:

Complications:

• Emotional stress
• Rarely, malignant glomus tumors may arise
• Incomplete removal results in tumor recurrence

Treatment:

• The treatment of glomus tumor depends on whether the tumor is benign or malignant
• Treatment depends on the location and the size of the tumor
• The healthcare provider may recommend a ‘wait and watch’ approach for small, asymptomatic and benign tumors
• Simple excision and complete removal of the tumor is generally effective treatment
• Other modalities may be based on the healthcare provider’s evaluation

Prevention: Presently, it is not possible to prevent the formation of Glomus Tumor of the Genitourinary System.

Prognosis:

• Generally, the prognosis is good since most tumors are benign
• In rare cases of malignant tumors, diagnosis generally occurs in advanced stages of tumor development. In such cases, the prognosis may be guarded