Fig. 10.1
38-year-old woman with a large jugular paraganglioma, intact lower cranial nerves, and excess dopamine and norepinephrine secretion resulting in arrhythmia (a, b). She underwent an aggressive subtotal resection with minimal residual disease left at the medial wall of the jugular bulb and internal carotid artery. She had normalized catecholamine levels and intact lower cranial nerves postoperatively (c, d)
Types of Subtotal Resection
In general, three forms of subtotal resection have been described and will be discussed further in this chapter: limited resection of only the middle ear portion of the tumor for audiological symptom improvement, resection of the intracranial portion of the tumor to relieve brain stem compression, and aggressive resection of all tumor except for that which is intimately involved with the carotid artery or functional cranial nerves.
Resection of the Middle Ear Component
Many jugular paragangliomas are indolent and can be observed for several years without risk of rapid growth. With this finding in mind, some authors [12, 13] have reviewed the role of limited surgery with the primary goal of symptom relief (Fig. 10.2). Cosetti et al. [12] treated three patients over the age of 70 with a limited resection that primarily addressed the middle ear component of tumors. All three patients had immediate relief of their pulsatile tinnitus after surgery, improved hearing, and no new cranial nerve deficits. One of these patients had radiologic progression 6 years after surgery, and this was treated with radiation therapy. Willen et al. [13] described similar subtotal resections in five patients over the age of 60 with Fisch class C3 tumors or greater; however, all patients in their series also underwent postoperative radiosurgery to the residual tumor. All patients had relief of their pulsatile tinnitus and stable or improved hearing. They reported no new lower cranial nerve deficits as a result of their treatment, and no tumors had grown after a mean of 19 months of follow-up. It should be noted that in the elderly population, subtotal resection has been described in symptomatic patients with a primary goal of symptom relief while avoiding cranial nerve injury. In asymptomatic patients with advanced age, conservative therapy can be considered.
Fig. 10.2
51-year-old man with right-sided conductive hearing loss and pulsatile tinnitus with a right-sided jugular paraganglioma (a, b). A postauricular transcanal approach was utilized for removal of the middle ear tumor component (c). The patient experienced resolution of his pulsatile tinnitus following surgery. Postoperative otoscopy and CT imaging are shown (d, e)
Subtotal Resection for Brain Stem Decompression
Rarely, patients may present with symptomatic brain stem compression or hydrocephalus from advanced tumors. In these cases, primary radiation treatment is not advisable due to concerns for posttreatment swelling leading to progressive brain stem compression. While gross total resection is still preferred in young, relatively healthy patients, it can be extremely challenging and cause unnecessary morbidity in patients of advanced age or limited life expectancy. In this scenario, performing a subtotal resection with the primary goal of brain stem decompression is a viable strategy. Carlson et al. [14] described four cases of advanced (Fisch grade D2) jugular paragangliomas who presented with significant brain stem compression. Subtotal resections were performed in three of these via a combined transtemporal and transcervical approach. All three patients eventually received postoperative radiation treatment to their residual tumor. Successful decompression, as well as, long-term tumor control was achieved in all three patients with 6–9 years of follow-up.
Aggressive Subtotal Resection
Wanna et al. [15] described subtotal resection in 12 patients with Glasscock-Jackson grade 3 or 4 tumors and intact lower cranial nerves (Fig. 10.3). In eight (66.7%) cases, no subsequent growth was observed after surgery with a mean follow-up of 3.7 years. The remaining four tumors grew at an average of 2 years after surgery. It was noted that the latter cases had significantly higher residual tumor following subtotal resection compared to those that showed no growth (59.2% vs. 11.9%). Moreover, no cases achieving greater than 80% of tumor resection when comparing pre- and postoperative imaging showed postoperative growth. There were no new permanent cranial nerve deficits following surgery in any of the cases, and no patient experienced carotid injury.
Fig. 10.3
Intraoperative photos of an aggressive subtotal resection of a large right-sided jugular paraganglioma. (a) Neck dissection with the white arrowhead pointed to the lower cranial nerves IX, X, and XI entering the skull base. (b) Black arrow pointing out the facial nerve bridge and white arrows denoting tumor. (c) The sigmoid sinus is extraluminally compressed with Surgicel packing. The black arrowhead points to the sigmoid opened with scissors. (d) The sigmoid sinus is completely transected to facilitate a transjugular approach. White arrows showed stumps of the transected sigmoid and the black arrow points to the facial nerve. Tumor lies just between the two sigmoid sinus stumps. Cottonoids are in place over the cerebellum. (e) Closure with an abdominal fat graft