CASE OF THE WEEK
2019-20 / JULY 1
(CONTRIBUTOR: JOHN CHEVILLE)
35-year-old female presents with hematuria. She has a history of hypertension. Cystoscopy revealed a 3 cm bladder mass and transurethral resection was performed. The tumor is positive for GATA3 and synaptophysin.
Quiz
1. What ancillary testing should be done?
a. Immunostain for fumarate hydratase
b. Immunostains for DNA repair enzymes
c. Immunostain for SDH B
d. Immunostain for carbonic anhydrase
2. What feature is most predictive of malignancy?
a. The presence or development of metastases
b. Extension into muscularis propria
c. Clinical symptoms
d. Patient age
1. c
2. a
1. Immunostain for SDH B
2. The presence or development of metastases
Patients with paraganglioma of the urinary bladder present with hematuria as well as signs and symptoms related to catecholamine release manifested by hypertensive crises that are associated with headache, sweating, palpitations, and flushing (that may be brought on by micturition). These tumors account for 6 to 10% of extra-adrenal paragangliomas, and although rare, it is critical to recognize them as they can be misdiagnosed as high grade urothelial carcinoma. These tumors may extend into the muscularis propria of the bladder. Approximately 15% of tumors recur and 10% metastasize but it is difficult to accurately predict their biologic behavior based on morphology alone. While the only reliable feature of malignancy is metastases, tumors associated with SDH B mutation have been shown to have a more aggressive clinical course. In addition, germline mutations in SDH A and B have been reported and therefore it is important to test for SDH B immunostain expression. Lastly, it is also important to note that GATA3 can be expressed by these tumors.
Identification of succinate dehydrogenase-deficient bladder paragangliomas. Mason EF, Sadow PM, Wager AJ, et al.
Am J Surg Pathol 2013;37:1612.
John Cheville
Mayo Clinic, Rochester
cheville.john@mayo.edu
Bladder
SDH B, paraganglioma, urothelial carcinoma