Case Report: Patient with Multiple Paragangliomas Treated with Long Acting Somatostatin Analogue

Abstract

Abstract. Paragangliomas of the head and neck are uncommon neoplasms. They are usually benign, but tend to be locally invasive. Although surgical resection remains the definitive treatment, important issues about management arise when such lesions are inoperable. Beneficial effects of octreotide treatment have already been reported in a malign paraganglioma case. Here we report a 24 year old female with familial, bilateral, multiple paraganglioma in the head and neck region, who firstly presented with pulsatile tinnitus and hearing loss in her left ear. After embolization was performed, she underwent operation twice because of the gross tumor mass. No significant change in tumor size was determined after the operations, however there were no distant metastases. Although she experienced hypertension attacks, no hormonal overproduction was found in repeated measurements. As the tumor was unresectable, new alternative therapies were sought. Octreotide scintigraphy was positive in the tumoral tissue, so we began to treat her with somatostatin analogue octreotide. After a 16 month follow up period, an improvement of the performance status, the near normalisation of attacks and stabilization of tumor growth were achieved. However, in the last three visits, she began to experience symptoms more frequently and it had been necessary to increase the octreotide dose. She is now well and being followed up. In conclusion, the beneficial effects of octreotide treatment could be quantified by clinical, tumor and scintigraphic criteria. These data suggest that octreotide can be useful in the treatment of inoperable paragangliomas.

Expression of somatostatin receptors, dopamine D2 receptors, noradrenaline transporters, and vesicular monoamine transporters in 52 pheochromocytomas and paragangliomas

“…However, a lower sst 2 expression was observed in four metastatic PCC/PGL by Kolby et al (2006). The similar mean sst 2 mRNA level in PCC/PGL and GEP-NETs in our study is intriguing, considering the fact that the current SRIF analogs (octreotide, lanreotide), which are directed mainly to sst 2 , have little if any efficacy on catecholamine secretion of PCC (Invitti et al 1993, Kopf et al 1997, Lamarre-Cliche et al 2002, or on tumoral growth of PGL (Tonyukuk et al 2003). sst 2 labeling was clearly observed in 6/7 tumors analyzed by immunohistochemistry, confirming the presence of sst 2 protein in our series.…”

“…The presence of sst in PCC/PGL has been demonstrated by ligand binding studies , Reubi & Laissue 1995, Reubi et al 2001, by immunohistochemistry (Mundschenk et al 2003, de Herder & Hofland 2004, Unger et al 2004, and more recently by reverse transcriptase (RT)-PCR (Ueberberg et al 2005, Kolby et al 2006, Binderup et al 2008. However, the current SRIF analogs, directed mainly to sst 2 , have little if any efficacy on catecholamine secretion of PCC (Invitti et al 1993, Kopf et al 1997, Lamarre-Cliche et al 2002, or on tumoral growth of PGL (Tonyukuk et al 2003). Two new somatostatin analogs with different binding profiles are in advanced clinical trials for various endocrine tumors: pasireotide, an sst 'panagonist' and BIM-23A760 dopastatin, with strong affinity for sst 2 and dopamine receptor subtype 2 (D 2 ).…”

Clinical characteristics and surgical treatment of spinal paraganglioma: A case series of 18 patients

“…Chemotherapy, radiotherapy, use of octreotide and somatostatin are effective complementary treatments for paragangliomas [30,31]. As with chemotherapy and radiotherapy, there is still no evidence to support the role for spinal paragangliomas currently, but these adjuvant modalities are preferred.…”

Non-Functional Paraganglioma of Retroperitoneum Mimicking Pancreatic Mass with Concurrent Urinary Bladder Paraganglioma: An Extremely Rare Entity

“…Therapy with radionucleotides (I-131 MIBG) or combination chemotherapy (cyclophosphamide, vincristine and darcabazine) may induce partial responses to malignant paraganglioma [12]. Octreotide may also be used for treatment of inoperable paragangliomas [13]. Although multiple functional and non functional Paragangliomas had been reported [2,5,14], synchronous non functional paragangliomas of retroperitoneum and urinary bladder reported in this case is extremely rare and is not found to be reported after extensive journal search.…”