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

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Case Report: Patient with Multiple Paragangliomas Treated with Long Acting Somatostatin Analogue Endocrine Journal 2003, 50 (5), 507–513 Case Report: Patient with Multiple Paragangliomas Treated with Long Acting Somatostatin Analogue VEDIA TONYUKUK, RIFAT EMRAL, ŞULE TEMIZKAN, AYşE SERTÇELIK*, ILHAN ERDEN** AND DEMET ÇORAPÇIOĞLU Department of Endocrinology and Metabolic Diseases, Ankara University, School of Medicine, 06100 Ankara, Turkey *Department of Pathology, Ankara University, School of Medicine, 06100 Ankara, Turkey **Department of Radiodiagnostic, Ankara University, School of Medicine, 06100 Ankara, Turkey 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. Key Words: Multiple paragangliomas, Long acting somatostatin analogue (Endocrine Journal 50: 507–513, 2003) PARAGANGLIOMAS of the head and neck are pattern 4. It is estimated that the familial incidence uncommon neoplasms. According to WHO classi- of head and neck paragangliomas is approximately fication all tumors of the paraganglion system are 10% 5. As reported, the family history of paragan- called paraganglioma. Paragangliomas occurring in gliomas did not predict the presence of hyperfunction- the head and neck region (from the carotid and vagal ing tumors, but did predict a high probability of body, from the glomus jugulare and glomus tympani- multiple tumors 6. The incidence of multicentricity cum) are called chemodectomas or glomus tumors 1, for this tumor is 33% 7. 2. They are in general rare, solitary and slow growing The most frequent presenting symptoms for the tumors of neuroendocrine origin [3. They are usually patients with head and neck tumors are palpable benign, but tend to be locally invasive. Head and neck neck mass (55%), tinnitus (18%), and cranial nerve paragangliomas are recognized to occur in two forms: palsies (16%) 6. They may also cause aural fullness a sporadic form and a familial autosomal dominant and hearing loss [8. Symptoms of paraganglioma are hypertension, tachycardia, etc., nevertheless a consid- Received: July 1, 2002 erable number of paragangliomas are asymptomatic. Accepted: May 6, 2003 They may be endocrinologically active or may be Correpondence to: Dr. Vedia TONYUKUK, İbn-i Sina Hospital, found with normal levels of dopamine, catecholamines 10 Floor, D Block, Samanpazarı, 06100 Ankara, Turkey and their metabolites, especially metanephrine and 508 TONYUKUK et al. vanillylmandelic acid (VMA) 9, 10. A small pro- went operation and had to be operated twice because portion (4%) of head and neck paragangliomas are of the gross residual tumor mass. In the first opera- hyperfunctional 6. Even in patients identified with tion, total excision of glomus jugulare and glomus catecholamine-secreting paragangliomas, the sensi- vagale was tried but tumors were subtotally removed. tivities of hormonal determinations are low, and the Unfortunately, they returned to their prior size 15 sensitivities achieved by measurements in the 24-h months after the first operation. As a side effect facial urine collection were 74% for total metanephrines, nerve palsy occurred. 84% for norepinephrine, 16% for dopamine, and 14% Histopathologic examination revealed that the for epinephrine 6. Although surgical resection tumor was a paraganglioma (Fig. 1). Also the neuro- remains the definitive treatment for chemodectomas, endocrine features of the tumor were demonstrated important issues about management arise when such immunohistochemically by S-100, chromagranin A, lesions are inoperable. Approximately one third of synaptophysin, and neuron specific enolase positivity patients have persistent or recurrent paragangliomas (Fig. 2). Control MRI showed no significant change so long-term follow up is important 6. When a in tumor size after the operations; however, there were paraganglioma is suspected, noninvasive techniques no distant metastases. She was referred to the Endo- such as computerized tomography, magnetic reso- crinology Department because of the hypertension nance imaging (MRI) or nuclear medicine imaging attacks with flushing and tachycardia lasting for 10–15 are being used for the localization of the tumor. minutes, two or three times a day. The 24-hour urine Radioiodinated metaiodobenzylguanidine (MIBG) scintigraphy and lately 111-indium-labelled octreotide scintigraphy have been used mainly to localize head and neck chemodectomas 11, 12. In addition, 131- iodine-MIBG therapy is a safe and usually well toler- ated treatment 13. Uptake of radiolabelled soma- tostatin analogue has already been reported in patients with malignant paragangliomas 14, 15, as well as beneficial effects of octreotide treatment in a malig- nant paraganglioma case 16. In this presentation, we demonstrated relief of symptoms and stabilization of the disease in a para- ganglioma case with positive octreotide scintigraphy who was treated with long acting depot octreotide (Sandostatin LAR®). Fig. 1. Paraganglioma composed of rests of uniform round cells in a delicate vascular stroma (HE × 40) Case A 24 year old female was first referred to Oto-Rhino- Laryngology Department with a pulsatile tinnitus in her left ear and she expressed some hearing loss in the left ear as well. MRI of the head and neck showed multiple paragangliomas (bilateral carotid body, bilateral glomus vagale and left glomus jugu- lare). Embolization was performed through arteria carotis externa and interna for the multiple bilateral lesions but no change in tumor size was achieved although vascularisation of the tumor decreased mini- mally. In addition no symptomatic improvement Fig. 2. Diffuse immunostaining for synaptophysin in tumor occurred after embolization. Afterward, she under- cells (× 40) SOMATOSTATIN TREATMENT IN PARAGANGLIOMA 509 Table 1. Vital signs and hormonal data of the patient during hypertension attacks Date BP Pulse/min VMA Metanephrine NE Epinephrine Dopamine 5-HIAA NE Epinephrine Dopamine (mmHg) (urine) (urine) (urine) (urine) (urine) (urine) (blood) (blood) (blood) 1.9–9.8 0.01–1 0–90 0–20 65–400 10–31.2 0–600 0–100 0–87 mg/24h mg/24h mg/24h mg/24h ug/24h umol/24h pg/ml pg/ml pg/ml 01.06.00 170/120 120 0.7 0.05 553 25 23.06.00 180/110 125 66.6 4.7 308.9 10.2 23.06.00 170/100 110 412.4 17.9 30.06.00 170/100 118 2.9 0.23 05.06.00 160/100 108 0.4 0.2 06.06.00 220/130 144 0.5 0.05 08.02.01 200/150 125 8.0 0.25 334 22.9 57 15.02.01 180/110 130 6.0 0.47 172 42.8 15.02.01 190/120 128 8.0 0.56 22.02.01 185/115 120 2.6 0.25 01.03.01 170/100 120 0.6 0.034 111 7.3 14.09.01 160/110 116 4.5 0.21 21.09.01 170/100 118 3.2 0.12 N: Normal, BP: Blood pressure, NE: Norepinephrine, 5-HIAA: 5-Hydroxyindole acetic acid, VMA: Vanillylmandelic acid Normal values of the determinations are also given in the Table. dopamine, norepinephrine and VMA determinations, and plasma norepinephrine levels were all negative even during attacks and no hormonal overproduction was found in repeated urinary measurements. Table 1 shows the vital signs and hormonal data of the patient during hypertension attacks. We searched our patient’s pedigree and learned from the hospital records that her father died because of a neck paraganglioma at age 53. Though her blood pressure rose up to 260/140 mmHg during hyper- tension attacks, in general her blood pressure had a tendency to decrease down to 80/40 mmHg. In order to control hypertension attacks, alpha blocker, with the lowest dose, was started but she could not tolerate it Fig. 3. Whole body and spot images were obtained by a gamma camera at 2 and 24 hr after the i.v. administration of 3.5 as it caused symptomatic hypotension. Calcium chan- mCi In111-octreotide. Bilateral accumulation in the nel blockers and angiotensin converting enzyme inhib- medial regions of the parotis gland and a focal uptake in itors did the same, so they were all discontinued. As the right cervical region, under the right submandibular the tumor was unresectable, new alternative therapies gland are seen in these images. were sought. MIBG uptake was negative. At that point octreotide scintigraphy was performed by using days and then 10 mg long acting depot octreotide for radioactive somatostatin analog 111-indium-diethyl- every 28 days was started. Before the treatment with enetriaminepentaacetic acid (111In-DTPA0) octreotide. octreotide, she experienced long lasting hypertension Octreotide scintigraphy was positive in the regions attacks every day. However, at the end of first month of bilateral cervical ganglions, under submandibular of octreotide therapy, her hypertension attacks nearly gland and near the medial side of parotis gland, as disappeared (one or two times a month) and became shown in Fig.
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