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Year : 2016  |  Volume : 15  |  Issue : 1  |  Page : 65-67

Unusual presentation of bladder paraganglioma: Comparison of 131I MIBG SPECT/CT and 68Ga DOTANOC PET/CT

1 Department of Nuclear Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, Haryana and Punjab, India
2 Department of Urology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, Haryana and Punjab, India

Date of Web Publication18-Dec-2015

Correspondence Address:
Bhagwant Rai Mittal
Department of Nuclear Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh - 160 012, Haryana and Punjab
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DOI: 10.4103/1450-1147.167591

PMID: 26912984

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Extraadrenal chromaffin cell-related tumors or paragangliomas are rare, especially in the bladder, accounting for less than 1% of cases. We report a 16-year-old boy who presented with hematuria and paroxysmal headache and was found to have a prostatic growth infiltrating the urinary bladder on anatomical imaging. Iodine-131 (131I) metaiodobenzylguanidine (MIBG) whole-body scanning and subsequently gallium-68 (68Ga) DOTANOC positron emission tomography/computed tomography (PET/CT) were performed. The MIBG scan revealed a non-tracer-avid soft-tissue mass, while DOTANOC PET/CT revealed a tracer-avid primary soft-tissue mass involving the urinary bladder and prostate with metastasis to the iliac lymph nodes. He underwent surgical management; histopathology of the surgical specimen revealed a bladder paraganglioma, whereas the prostate was found to be free of tumor.

Keywords: Bladder paraganglioma, iodine-131 (131I) metaiodobenzylguanidine (MIBG), gallium-68 (68Ga) DOTANOC positron emission tomography.computed tomography. (PET/CT)

How to cite this article:
Jain TK, Basher RK, Gupta N, Shukla J, Singh SK, Mittal BR. Unusual presentation of bladder paraganglioma: Comparison of 131I MIBG SPECT/CT and 68Ga DOTANOC PET/CT. World J Nucl Med 2016;15:65-7

How to cite this URL:
Jain TK, Basher RK, Gupta N, Shukla J, Singh SK, Mittal BR. Unusual presentation of bladder paraganglioma: Comparison of 131I MIBG SPECT/CT and 68Ga DOTANOC PET/CT. World J Nucl Med [serial online] 2016 [cited 2021 Nov 27];15:65-7. Available from: http://www.wjnm.org/text.asp?2016/15/1/65/167591

   Introduction Top

Paragangliomas, derived from neural crest cells, are rare tumors and occur in diverse anatomic locations from the skull base to the sacrum.[1] Most paragangliomas are benign and progress slowly, but metastases may occur in about 10% of patients. About 25% of paragangliomas have hereditary forms with a propensity for multifocal disease.[2] Diagnosis is made from clinical, biochemical, and typical imaging features. Combined anatomical and functional imaging is required to exactly delineate the extent of the disease. Nuclear scintigraphy is commonly employed to identify multifocal or metastatic disease for accurately staging the disease before therapy. Iodine-131/123 (131 I/123 I) metaiodobenzylguanidine (MIBG) scintigraphy and indium-111 (111 In) diethylenetriaminepentaacetic acid (DTPA)-pentetreotide scintigraphy are well-established nuclear imaging technologies for the staging and restaging of paragangliomas. Fluorine-18 (18 F) fluorodopa (FDOPA) and gallium-68 (68 Ga) DOTANOC positron emission tomography/computed tomography (PET/CT) also show promising results in the identification of paragangliomas.

   Case Report Top

A 16-year-old boy presented with complaints of multiple episodes of painless gross hematuria associated with recurrent paroxysms of headache, sweating, and palpitations over the past 3 months. His physical examination was unremarkable. On evaluation, ultrasonography (USG) abdomen revealed gross hydronephrosis of the right kidney with thinned-out cortex. Magnetic resonance imaging (MRI) of the pelvis showed prostatic growth with infiltration into the seminal vesicle and the urinary bladder. The patient underwent transrectal ultrasound (TRUS) biopsy of mass, which revealed a paraganglioma, with positive immunohistochemistry for synaptophysin and chromogranin. Plasma free metanephrine and normetanephrine levels were 26 pg/mL (<90) and 1153 pg/mL (<180) respectively. The patient underwent 131 I MIBG whole-body and subsequently 68 Ga DOTANOC PET/CT to look for functional status as well as other possible lesions.131 I MIBG whole-body scan [Figure 1] showed physiological tracer distribution except increased pelvic activity. Subsequent single-photon emission computed tomography/computed tomography (SPECT/CT) pelvis revealed a non-tracer-avid soft-tissue mass involving the bladder and prostate, while visualized activity on planar images was localized to accumulated urine in the bladder.68 Ga DOTANOC PET/CT [Figure 2] revealed a somatostatin receptor (SSTR)-expressing heterogeneous soft-tissue mass (~8.0 × 6.4 cm) with coarse calcification in the prostate, infiltrating the posterior wall of the urinary bladder and extending into the bladder lumen, with metastatic involvement of the right common iliac, internal iliac, and bilateral external iliac lymph nodes. The patient underwent radical cystoprostatectomy, bilateral standard pelvic lymph node dissection, and continent cutaneous diversion. Finally, histopathology revealed a urinary bladder paraganglioma with lymph node involvement; the prostate and seminal vesicles were free of tumor.
Figure 1: 131I MIBG whole-body (a) anterior and (b) posterior images show physiological tracer distribution but for increased pelvic tracer activity. Subsequent SPECT/CT and CT (c and d) sagittal (e and f) transaxial (g and h) coronal images of pelvic region show non-tracer-avid soft-tissue mass involving bladder and prostate, while visualized tracer activity in the pelvis was localized to accumulated urine in bladder

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Figure 2: 68Ga DOTANOC PET/CT maximum intensity projection (MIP) image (a) shows large tracer activity in pelvis with dilated left ureter. Fused PET/CT and CT coronal (b-c and f-g) and transaxial images (d-e and h-i) show SSTR-expressing heterogeneous soft-tissue mass (~8.0 × 6.4 cm) with coarse calcification (arrow) in the prostate, infiltrating posterior wall of the urinary bladder and extending into bladder lumen with metastatic involvement of the right common iliac, internal iliac (arrow head), and bilateral external iliac lymph nodes

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   Discussion Top

Urinary bladder paraganglioma is a very rare tumor, accounting for less than 0.05% of all bladder malignancies and 6% of all paragangliomas.[3],[4] A bladder paraganglioma is difficult to distinguish radiologically from other bladder lesions.[5] If genetic status is not available before surgery, the possibility of multifocal or metastatic disease should be considered, and functional imaging is recommended. Both 131 I MIBG and 111 In DTPA-pentetreotide SPECT/CT have shown promising results in pheochromocytoma and paraganglioma.[6] MIBG is taken up by paraganglioma via norepinephrine transporter (NET). However, paragangliomas may not show MIBG tracer avidity, as observed in our case.[1] But poor resolution, artifacts, attenuation, and inability for quantification make SPECT inferior to PET imaging. Increased uptake of fluorodeoxyglucose (FDG) on 18 F FDG PET/CT is not specific to paragangliomas.[7] Only 5-15% of urinary bladder paragangliomas may be malignant, and histologically, differentiation between benign and malignant lesions may not be possible.[8],[9]68 Ga DOTANOC PET/CT shows tracer avidity in neural crest tumors, such as paragangliomas, due to SSTR expression.[10] DOTANOC imaging facilitates curative treatment by the identification of metastatic spread. On DOTANOC imaging, if a malignant paraganglioma is found to be surgically nonassessable, then it can be considered for lutetium-177 (177 Lu) DOTANOC therapy. In our case, the DOTANOC scan showed tracer avidity in primary bladder paraganglioma and iliac group lymph nodes. The final impression was primary urinary bladder paraganglioma with locoregional nodal metastases. Urinary excretion of the tracer hampered the clear visualization of the primary bladder lesion. Visualization of lesions can significantly improve with the use of diuretics; however, in our case the primary mass was causing bladder outlet obstruction.[11] The patient underwent surgery and the primary as well as metastatic lymph nodes were also removed. To the best of our knowledge, this is the first case of malignant bladder paraganglioma that showed 131 I MIBG negativity and 68 Ga DOTANOC positivity.

   Conclusion Top

The combined functional and anatomical imaging modality helps in early detection and facilitates curative treatment of paragangliomas by potentially ruling out metastatic spread.68 Ga DOTANOC PET/CT is very useful in the imaging of paragangliomas.

   References Top

Taïeb D, Neumann H, Rubello D, Al-Nahhas A, Guillet B, Hindié E. Modern nuclear imaging for paragangliomas: Beyond SPECT. J Nucl Med 2012;53:264-74.  Back to cited text no. 1
Cascón A, Pita G, Burnichon N, Landa I, López-Jiménez E, Montero-Conde C, et al. Genetics of pheochromocytoma and paraganglioma in Spanish patients. J Clin Endocrinol Metab 2009;94:1701-5.  Back to cited text no. 2
Ranaweera M, Chung E. Bladder paraganglioma: A report of case series and critical review of current literature. World J Clin Cases2014;2:591-5.  Back to cited text no. 3
Pastor-Guzmán JM, López-García S, Giménez-Bachs JM, Ruíz-Mondejar R, Cañamares-Pabolaza L, Atiénzar-Tobarra M, et al. Paraganglioma of the bladder: Controversy regarding treatment. Urol Int 2004;73:270-5.  Back to cited text no. 4
Bhalani SM, Casalino DD, Manvar AM. Paraganglioma of the bladder. J Urol2011;186:279-80.  Back to cited text no. 5
Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005;366:665-75.  Back to cited text no. 6
Shulkin BL, Thompson NW, Shapiro B, Francis IR, Sisson JC. Pheochromocytomas: Imaging with 2-[fluorine-18]fluoro-2-deoxy-D-glucose PET. Radiology 1999;212:35-41.  Back to cited text no. 7
Ansari MS, Goel A, Goel S, Durairajan LN, Seth A. Malignant paraganglioma of the urinary bladder. A case report. Int Urol Nephrol 2001;33:343-5.  Back to cited text no. 8
Doran F, Varinli S, Bayazit Y, Bal N, Ozdemir S. Pheochromocytoma of the urinary bladder. APMIS 2002;110:733-6.  Back to cited text no. 9
Naswa N, Karunanithi S, Sharma P, Soundararajan R, Bal C, Kumar R, et al. Pre-operative 68 Ga-DOTANOC somatostatin receptor PET/CT imaging demonstrating multiple synchronous lesions in a patient with head and neck paraganglioma. Rev Esp Med Nucl Imagen Mol 2014;33:374-7.  Back to cited text no. 10
Dhull VS, Karunanithi S, Arora S, Jain TK, Kumar R. Diuretic 68Ga DOTANOC PET/CT in imaging of bladder paraganglioma. Clin Nucl Med 2014;39:915-6.  Back to cited text no. 11


  [Figure 1], [Figure 2]


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