Cerebellopontine angle epidermoid cyst case series

Fifty-four patients who underwent surgery for CPA epidermoid cysts were retrospectively analyzed, regarding their surgical approaches, removal rates, preservation rates of CN function 1 year after surgery, and recurrence. Surgical approaches were selected for patients according to the size and extension of the tumor.

Results: Surgical approaches consisted of LSO (20 cases), anterior transpetrosal approach (ATP; 3 cases), combined transpetrosal approach (27 cases), and ATP + LSO (4 cases). Mean tumor content removal and capsule removal rates were 97.4% and 78.3%, respectively. Preservation rates of facial nerve function and useful hearing were 94.4% and 90.7%, respectively. The mean postoperative follow-up time was 62.8 months, and there were two cases of recurrence requiring reoperation about 10 years after surgery.

For CPA epidermoid cyst surgeries, selection of the appropriate surgical approach for each patient and radical resection with an optimal operative field under direct visualization enable tumor removal without the need for reoperation for a long time, with preservation of CN functions ¹⁾.

Twenty-two cases with epidermoid cysts of CPA micro-neurosurgically treated since 2005 were reviewed. Clinical status of the patients before the surgery and post-operative functional outcome were recorded. Available data from the English literature were summarized for comparison. Mass reduction of cyst contents in most cases was usually associated with prompt and marked improvement of the symptoms suggesting neuroapraxia caused by compression of the tumor content and/or mild ischemia. Among them, two cases showed strangulation of the affected nerves by the tumor capsule whose preoperative dysfunction did not improve after surgery in spite of meticulous microsurgical removal of the lesion. Involved facial and abducent nerves in these two cases showed distortion of nerve axis and nerve atrophy distal to the strangulation site.

Hasegawa et al. report the first direct evidence of etiology of cranial nerve dysfunction caused by cerebellopontine angle epidermoid tumors. Young age and rapidly progressive neurological deficit might be the characteristics for strangulation of the affected nerves by the cyst capsule. Even though the number of cases might be limited, immediate decompression and release of the strangulating band might be urged in such patients to prevent irreversible deficits ²⁾.

17 patients, including 7 with tumor limited to the cerebellopontine angle, 7 with cerebellopontine angle tumor penetrating supratentorially, and 3 with cerebellopontine angle tumor extending along skull base to contralateral cerebellopontine angle. All patients were followed-up for the mean duration of 126 months.

On admission cranial nerve symptoms predominated. Total tumor removal was achieved in 5 patients, and incomplete removal (with small tumor remnants left on vessels, nerves, or brainstem) in 12 patients. Postoperatively, preoperative deficits worsened in 2 and new postoperative deficits occurred in 10 patients. The extent of tumor expansion had no effect on postoperative morbidity and risk of recurrence. During long-term follow-up, improvement or resolution of preoperative deficits was seen in 11 of 17 patients, and new postoperative deficits in 8 of 10 patients. Symptomatic recurrences after an average of more than 9 years were noted in 5 patients, 3 of whom were reoperated. Recurrences occurred in some younger patients and always in area of primary tumor. No effect of extent of tumor removal on risk of recurrence was found.

The extent of tumor removal had no effect on the risk of recurrence, and thus it may be acceptable to leave tumor capsule fragments adhering closely to nerves, vessels, or brainstem. During long-term follow-up, resolution or improvement of present preoperatively and new postoperative neurological deficits may be expected in most patients ³⁾.

In a case series, pathophysiology of cranial nerve dysfunction in CPA epidermoid cysts was evaluated with special attention to a new mechanism of capsule strangulation caused by stratified tumor capsule. Twenty-two cases since 2005 were reviewed. Clinical status of the patients before the surgery and post-operative functional outcome were recorded. Available data from the English literature were summarized for comparison. Mass reduction of cyst contents in most cases was usually associated with prompt and marked improvement of the symptoms suggesting neurapraxia caused by compression of the tumor content and/or mild ischemia. Among them, two cases showed strangulation of the affected nerves by the tumor capsule whose preoperative dysfunction did not improve after surgery in spite of meticulous microsurgical removal of the lesion. Involved facial and abducent nerves in these two cases showed distortion of nerve axis and nerve atrophy distal to the strangulation site. Hasegawa et al. report the first direct evidence of etiology of cranial nerve dysfunction caused by cerebellopontine angle epidermoid tumors. Young age and rapidly progressive neurological deficit might be the characteristics for strangulation of the affected nerves by the cyst capsule. Even though the number of cases might be limited, immediate decompression and release of the strangulating band might be urged in such patients to prevent irreversible deficits ⁴⁾

Hu et al. performed a retrospective analysis of clinical data of 13 male and 17 female patients (mean age: 42.4 ± 11.4 years) who presented with a CPA epidermoid cyst and underwent whole course neuroendoscopy. Complications and tumor recurrence were assessed at follow-up. Results Clinical manifestations included an initial symptom of headache (n = 21), gait instability (n = 6), intracranial hypertension (n = 13), posterior cranial nerve symptoms (n = 6), ataxia (n = 5), and hydrocephalus (n = 1). All patients tolerated tumor resection with subsequent symptomatic improvement, and the results of the postoperative magnetic resonance imaging scan did not show any remnants of tumor. Mean duration of surgery was 2.61 ± 0.47 hours, mean loss of blood was 96.8 ± 35.4 mL, and the mean duration of hospitalization was 7.5 ± 2.25 days. Postoperative complications (8 of 30 [26.7%]) included fever (n = 5), communicating hydrocephalus (n = 1), facial nerve paralysis (n = 1), and abducens nerve palsy (n = 1). Tumor recurrence was observed in two patients (6.7%). No deaths or intracranial hemorrhage was reported.

The characteristics of epidermoid cysts make them amenable to whole course neuroendoscopic resection. Use of physiologic/pathologic interspaces and neuroendoscopic angulations decreases traction on the brain, improves complete resection rates, and decreases postoperative complications ⁵⁾.

Between 1996 and 2004, 10 patients with typical symptoms of trigeminal neuralgia were found to have cerebellopontine angle epidermoids and treated surgically.

Total resection was done in 6 patients (60%). Surgical removal of tumor and microvascular decompression of the trigeminal nerve were performed simultaneously in one case. One patient died due to postoperative aseptic meningitis. The others showed total relief from pain. During follow-up, no patients experienced recurrence of their trigeminal neuralgia (TN).

The clinical features of TN from CPA epidermoids are characterized by symptom onset at a younger age compared to TN from vascular causes. In addition to removal of the tumor, the possibility of vascular compression at the root entry zone of the trigeminal nerve should be kept in mind. If it exists, a microvascular decompression (MVD) should be performed. Recurrence of tumor is rare in both total and subtotal removal cases, but long-term follow-up is required ⁶⁾.

compared the surgical outcomes of recent patients with cerebellopontine angle (CPA) epidermoids treated with advanced surgical tools with those of patients treated in earlier series. From November 2000 to June 2004, we treated 12 patients with epidermoid tumors. One patient had a strict CPA lesion. Tumors extended into the prepontine region in seven cases and supratentorially in two. In two cases the CPA was involved bilaterally. All patients but one underwent a lateral suboccipital approach in a semi-sitting position with microsurgical technique. Endoscopic assistance was used in cases with extensions beyond the CPA. In one case, a subtemporal route was used. The mean follow-up was 27 months (range, 8 to 50 months). There were no deaths. Total removal was achieved in 7 of the 10 patients with unilateral CPA epidermoids. Preoperative status improved in eight (80%) patients, particularly the function of cranial nerves (CNs) V and VII. Only two patients had permanent CN deficits. Complete excision with preservation of CN function should be the goals of management of epidermoids of the CPA. In some cases, these goals can be difficult to achieve, even with contemporary surgical equipment. Bilateral and extensive tumors should be removed in staged procedures. The function of CN V and CN VII may recover after decompression, but the outcome of symptoms related to CN VIII is less certain. The endoscope is a reliable tool for assessing the extension of epidermoids, but it cannot be used for tumor remova ⁷⁾.

Thirty cases of cerebellopontine angle epidermoid cysts treated over a period of 20 years are reviewed with regard to their clinical features, the pathophysiology of their symptoms and their management. The predominating symptoms were related to the 7th and 8th cranial nerves and headaches. The signs and symptoms were present for an average period of 4 months. It was not always possible to determine if the signs and symptoms were due to local involvement by the epidermoid, increased intracranial pressure, or both. Diagnostic procedures evolved from angiography and ventriculography to non-invasive computed tomography and MRI. The posterior cranial fossa approach was used in 27 cases. Total excision of the epidermoid was the aim and was carried out in five (18%) patients but concern regarding the preservation of nearby important neurovascular structures forced partial removal in 22 patients. To minimise reformation, the residual epidermoid was carefully coagulated with the aid of the operating microscope and bipolar cautery without damaging surrounding neurovascular structures ⁸⁾.