Title: Paraganglioma of the Paravesicle Region –A Case Report and Review of Literature

Authors: Vidhya Manohar, Mahesha Vankalakunti, Mohammad Shahid Ali, Deepak Dubey, Srinivas A K, Somanna M, Amrith Raj Rao

 DOI:  https://dx.doi.org/10.18535/jmscr/v6i7.34

Abstract

The generic term paraganglioma is applied to tumours arising from paraganglia regardless of the location. The only exception is the tumours of the adrenal medulla which is known as pheochromocytoma. [1]The tumours can be found practically every site in which paraganglia are located. The most commonly seen locations include: the carotid body, jugular foramen, middle ear, aorticopulmonary region, posterior mediastinum and abdominal para aortic region.

Extra-adrenal paragangliomas are neoplasms arising from cells of neural crest origin anywhere along the distribution of the sympathoadrenal neuroendocrine system. Nearly 85% are intra-abdominal, 12% are intrathoracic, and 3% are cervical.[2]

This report describes a 66-year-old male presenting with difficulty into micturition with increased frequency and decreased flow.  MRI showed a well-defined paravesicular soft tissue mass measuring 33x35mm in the lateral aspect of the left seminal vesicle. The complete biochemical work-up and haematological investigations were within normal limits. The patient underwent surgical exploration with complete excision of the right seminal vesicle. Gross examination revealed a well-defined yellow brown tumour with a rim of seminal vesicle. Microscopy revealed characteristic Zellballen pattern of arrangement of the tumour, with neoplastic cells having abundant granular cytoplasm. The cells uniformly expressed synaptophysin and chromogranin. S100 positivity was noted in the sustentacular cells and tumour cells. Ki67 index was less than 2%.

The patient is on follow-up and is healthy till date.

Keywords: seminal vesicle, paraganglioma, paravesicle.

References

  1. Lack EE. Paraganglioma. In: Sternberg SS, ed. Diagnostic surgical pathology, 2nd New York, NY: Raven Press 1994 : 599-621
  2. Extra adrenal paragangliomas of the sympathoadrenal neuroendocrine system. Atlas of Tumor Pathology, Tumors of the Adrenal Gland and Extra Adrenal Paraganglia. 3rd ed. Bethesda, Md: Armed Forces Institute of Pathology; 1997:269–284.
  3. Ramchandani P, Banner MP, Pollack HM. Imaging of the seminal vesicles.Semin Roentgenol. 1993;28:83. 
  4. Reddy MN, Verma S. Lesions of the Seminal Vesicles and their MRI Characteristics.Journal of Clinical Imaging Science. 2014;4:61. doi:10.4103/2156-7514.143734.
  5. Das S., Lowe P. Malignant pheochromocytoma of the bladder. J Urol. 1980;123:282–284. 
  6. Hanji A.M., Rohan V.S., Patel J.J., Tankshali R.A. Pheochromocytoma of the urinary bladder: a rare cause of severe hypertension. Saudi J Kidney Dis Transpl. 2012;23(4):813–816. 
  7. Alvarenga C.A., Lopes J.M., Vinagre J., Paravidino P.I., Alvarenga M., Prando A. Paraganglioma of seminal vesicle and chromophobe renal cell carcinoma: a case report and literature review. Sao Paulo Med J.2012;130(1):57–60. 
  8. Gupta R., Howell R.S., Amin M.B. Paratesticularparaganglioma: a rare cause of an intrascrotal mass. Arch Pathol Lab Med. 2009;133(5):811–813.
  9. O’Riordain DS, Young WF Jr, Grant CS, Carney JA, van Heerden JA. Clinical spectrum and outcome of functional extra-adrenal paraganglioma.World J Surg 1996; 20:916-921.[ Crossref] [Medline].
  10. Roman S. Pheochromocytoma and functional paraganglioma.CurrOpinoncol 2004; 16:8-12[ Crossref] [Medline].
  11. Sauborn DP, Kruskal JB, Stillman IE, Parangi S. Best cases from the AFIP: Paraganglioma of the organs of Zuckerkandl. Radiographics 2003; 23:1279-1286 [ Crossref] [Medline].
  12. Dunnick NR, Korobkin M. Imaging of adrenal incidentalomas: current status. AJR 2002; 179:559-568 [Abstract]
  13. Timmers HJ, Pacak K, Bertherat J, Lenders JW, Duet M, Eisenhofer G, et al. Mutations associated with succinate dehydrogenase D-related malignant paragangliomas.ClinEndocrinol (Oxf) 2008;68:561–6. 
  14. Wang H., Chen Y., Kao H., Lin S., Lee C., Huang G. Extra-adrenal paraganglioma of prostate. Can UrolAssoc J (North America) 2013;7 
  15. ElderEE, Xu D, Hoog A, Enberg U, Hou M, Pisa P, Gruber A, Larsson C & Backdahl M 2003 KI-67 and hTERT expression can aid in the distinction between malignant and benign pheochromocytoma and paraganglioma Modern Pathology 16; 246–255.
  16. BoltzeC, Mundschenk J, Unger N, Schneider-Stock R, Peters B, Mawrin C, Hoang-Vu C, Roessner A & Lehnert H 2003 Expression profile of the telomeric complex discriminates between benign and malignant pheochromocytoma. Journal of Clinical Endocrinology and Metabolism88; 4280–4286
  17. National Cancer Institute at the National Institute of Health. http://www.cancer.gov/cancertopics/pdq/treatment/pheochromocytoma/HealthProfessional/page2

Corresponding Author

Mahesha Vankalakunti

Consultant, Dept of Histopathology, No.98, Manipal Hospitals,

Old Airport Road, Bengaluru 560017, India

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