MANAGEMENT OF ADOLESCENT CERVICAL

PARAGANGLIOMA: 13 CASES

FOUAD A.FOUADAssistant professor of surgical oncology

National Cancer Institute (NCI)

Cairo University

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INTRODUCTION

• A paraganglioma is a rare neuroendocrine tumor.

• 75% of paragangliomas are sporadic. • 25% are hereditary .• 90% adrenal (pheochromocytoma).• 10% extra-adrenal .

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INTRODUCTION

• Extra-adrenal– 85% abdomen– 12% thorax– 3% head and neck:

• Carotid body most common(CBT)• Vagal(VP)• Jugulotympanic(JP;TP)• Other

• Cervical paragangliomas [CP] generally present in mid-adult life.

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TREATMENT

• The different therapeutic options include:

Surgical resection. Conventional radiotherapy . Stereotactic radio surgery In selected cases a ‘wait and scan’ Foote RL,

et al.[2002]

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AIM OF THIS STUDY

• The aim to review our experience at NCI in management of cervical paraganglioma in the adolescent age as regard:

Surgical treatment Functional results.

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MATERIAL AND METHODS

• During the period 2002–2011, thirteen patients with cervical paragangliomas have been presented to NCI, Cairo university for surgical treatment.

• All patients were adolescents with mean age 17.2 years (range, 13 to 21 years).

• Complete surgical resection was possible in all cases in this study

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 PREOPERATIVE DATA

Percentage No; of the patients Item

84.6%:15.4% 11/2 Female/male

17.2±2.3 Mean Age(Years)

69.2%23%7.6%69.2%:30.8%

9/133/131/139:4

Main symptom: Lateral neck mass Mixed symtomatology Headache Lt. /Rt.

15.1±9.4 Duration of symptoms (mean in months)

23%53.1%23%

3/137/133/13

Size of the tumor >2 cm 2-4cm >4 cm

15.3% 2/13 Pre op. biopsy

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Accidental pre-operative biopsy

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Pre-operative investigation

• Ultrasonography (US) • Computerized tomography (CT)• Magnetic Resonance Imaging (MRI)

[seven out of 13 (53.8%) ]

• Digital subtraction angiography (DSA) {All cases}

• Balloon test occlusion (BTO) [three cases with large tumors more than 4 cm]

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In all cases

CP-IMAGING

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CP-IMAGING

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CP-IMAGING

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CP-IMAGING

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CP-IMAGING

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CP-IMAGING

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CP-IMAGING

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CP-IMAGING

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Preoperative selective embolization

• Remains a matter of debate as major complications such as cerebrovascular accidents might occur.

• For some authors, allows safer surgical excision of the paraganglioma

• In our series, no patient underwent selective embolization

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OPERATIVE TECHNIQUE

• Incision along the anterior border of the sternocleidomastoid

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OPERATIVE TECHNIQUE

• Proximal exposure of the CCA.• The branches from the ECA were ligated

and divided near the tumor border . • The dissection was performed in the

relatively a vascular subadventitial plane between the artery and the tumor called “white line”.

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OPERATIVE TECHNIQUE

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POST OPERATIVE SPECIMEN

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OPERATIVE TECHNIQUE

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POST OPERATIVE SPECIMEN

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OPERATIVE TECHNIQUE

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OPERATIVE TECHNIQUE

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POST OPERATIVE SPECIMEN

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OPERATIVE DATA

• The mean operative time was 84.7 ±36.4 ml minutes;

• Mean blood loss was 98.07±39 ml.• Mean size of the resected tumors was

3.6±1.5 cm (range 1.8–6.8 cm).

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POST-OPERATVE DIAGNOSIS

Percentage Number Type of the tumor

61.7% 8

2 [25%]5 [62.5]1 [12.5%]

CBTShamblin 1971 class:

• I [Localized] • II [Adherent Partially ]• III [Encasing]

23% 3 VP

15.3% 2 JP

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Vascular and neurological injuries

Cranial nerves injuries Carotid arteries injuries

Item

XII XI X IX ICA injury

ECA injury

2(15%) 1(7.6%) 3(23%) CBPs

3(23%) 1(7.6%) VPs

1(7.6%) 2(15.3%) JPs

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MORBIDITY AND MORTALITY

• Post operative difficulty in deglutition in 3 patients.

• No transient ischemic attack or stroke was noticed in any patient .

• No perioperative mortality was documented 

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CONCLUSION• Cervical paragangliomas in the adolescent

age are a rare entity • More predominant in females• Carotid body tumors are the most common

head and neck CP in adolescent age.• Best treated by proper surgical resection.

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CONCLUSION• Early management of patients and expert

surgeons minimize neurovascular complications

• Cranial nerve deficits were more common in patients with JP and VP relative to those with CBT but all improved rapidly in that young age.

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THANK YOU