Update: Superior semicircular canal dehiscence

Superior semicircular canal dehiscence

Superior semicircular canal dehiscence (SSCD) is a disorder of the skull base that is gaining increasing recognition among neurosurgeons.

Superior semicircular canal dehiscence (SSCD) is an osseous defect of the arcuate eminence of the petrous temporal bone.

With this dehiscence, the fluid in the membranous superior canal (which is located within the tubular cavity of the bony canal) can be displaced by sound and pressure stimuli, creating certain vestibular and/or auditory signs and symptoms.

Since this was first reported in 1998 by Minor and colleagues, there has been much advancement made in terms of diagnosis and treatment 1).

Diagnosis

The condition is confirmed on high-resolution computed tomography (CT) imaging.

High-resolution computed tomographic temporal bone images were imported into a freely available segmentation software. Dehiscence lengths and volumes were ascertained by independent authors. Inter-rater observer reliability was assessed using Cronbach’s alpha. Correlation and regression analyses were performed to evaluate for relationships between dehiscence size and symptoms (pre- and post-operative).

Thirty-seven dehiscences were segmented using the novel volumetric assessment. Cronbach’s alpha for dehiscence lengths and volumes were 0.97 and 0.95, respectively. Dehiscence lengths were more variable as compared to dehiscence volumes (σ 2 8.92 vs σ 2 0.55, F = 1.74). The mean dehiscence volume was 2.22 mm 3 (0.74, 0.64-0.53 mm 3 ). Dehiscence volume and headache at presentation were positively correlated ( R pb = 0.67, P = .03). Dehiscence volume and vertigo improvement after surgery were positively correlated, although this did not reach statistical significance ( R pb = 0.46, P = .21).

SSCD volumetry is a novel method of measuring dehiscence size that has excellent inter-rater reliability and is less variable compared to dehiscence length, but its potential as a predictor of symptom outcomes is not substantiated. However, the study is limited by low power 2).

MRI FIESTA scans have recently been used to image SSCD. Additionally, audiometry and vestibular evoked myogenic potential (VEMP) testing are useful screening tools.

Treatment

Currently, the middle fossa approach is the most common and standard surgical approach to repair SSCD. The transmastoid, endoscopic and transcanal or endaural approaches have also been recently utilized. Presently, there is no consensus as to the best approach, material or technique for repair of SSCD. As we learn more, newer and less invasive approaches and techniques are being used to treat SSCD 3).

Symptoms are often improved by surgical repair. Although a classic middle fossa craniotomy has been used with good results, recent advances in technique have allowed for modification of the traditional approach into a smaller skin incision and a minimally invasive middle fossa keyhole craniectomy roughly 1.7 cm in diameter.

To delineate this novel approach and describe the technique for accurate localization of the dehiscence using preoperative measurements and intraoperative image guidance, thereby minimizing the need for a larger skin incision and craniotomy.

Patients were independently diagnosed with SSCD by the senior authors. Once relevant imaging was acquired, the novel keyhole technique was performed. Patients’ vestibular and auditory symptoms before and after the procedure were assessed. Outcomes from a series of patients treated with this keyhole approach were tabulated and reported.

Twelve cases from 11 patients were included in this series. Auditory symptoms had high rates of resolution with pulsatile tinnitus, internal amplification of sounds, and autophony being resolved in a majority of cases. Only 2 cases reported hearing decline. Sound/pressure induced vertigo and disequilibrium also demonstrated high rates of resolution. No complications were reported.

The minimally invasive middle fossa keyhole craniectomy is a novel approach for the repair of SSCD. This approach may contribute to resolved auditory and vestibular symptoms with low morbidity and quick recovery 4).

Reviews

A analysis included 24 studies that described 230 patients that underwent either an middle cranial fossa (MCF) (n = 148, 64%) approach or a transmastoid approach (TM) (n = 82, 36%) for primary surgical repair of SSCD. A greater percentage of patients in the MCF group experienced resolution of auditory symptoms (72% vs 59%, p = 0.012), aural fullness (83% vs 55%, p = 0.049), hearing loss (57% vs 31%, p = 0.026), and disequilibrium (75% vs 44%, p = 0.001) when compared to the TM group. The MCF approach was also associated with higher odds of symptom resolution for auditory symptoms (odds ratio [OR] 1.79, 95% confidence interval [CI] 1.14-2.82), aural fullness (OR 4.02, 95% CI 1.04-15.53), hearing loss (OR 2.91, 95% CI 1.14-7.42), and disequilibrium (OR 3.94, 95% CI 1.78-8.73). The mean follow-up was 9 months.

The literature suggests that the MCF approach for the repair of SSCD is associated with greater symptom resolution when compared to the TM approach. This information could help facilitate patient discussions 5).

Case series

A total of 72 cases of SSCD in 60 patients were repaired via a middle fossa approach at a single institution. Main Outcome Measures  The distance from the proposed reference point to the dehiscence was statistically analyzed using Shapiro-Wilk’s goodness-of-fit test and Student’s t -test. Results  Average distance for all patients was 28.84 ± 2.22 mm (range: 22.96-33.43). Average distance for females was 29.08 mm (range: 24.56-33.43) versus 28.26 mm (range: 22.96-32.36) for males. There was no difference in distance by sex ( p  = 0.174). The distance measurements followed a normal distribution with 95% of the patients between 24.49 and 33.10 mm.

This study analyzed a potential reference point during a middle fossa approach for SSCD surgery. The distance from this reference point to the SSCD was found to be consistent and may serve as a readily identifiable landmark in localizing the dehiscence 6).

Case reports

A 35-year-old man with superior semicircular canal dehiscence treated by a joint neurosurgical and otolaryngological team 7).

1) , 3)

Mau C, Kamal N, Badeti S, Reddy R, Ying YM, Jyung RW, Liu JK. Superior semicircular canal dehiscence: Diagnosis and management. J Clin Neurosci. 2018 Feb;48:58-65. doi: 10.1016/j.jocn.2017.11.019. Epub 2017 Dec 7. Review. PubMed PMID: 29224712.
2)

Lagman C, Beckett JS, Chung LK, Chen CHJ, Voth BL, Gaonkar B, Gopen Q, Yang I. Novel Method of Measuring Canal Dehiscence and Evaluation of its Potential as a Predictor of Symptom Outcomes After Middle Fossa Craniotomy. Neurosurgery. 2017 Aug 9. doi: 10.1093/neuros/nyx430. [Epub ahead of print] PubMed PMID: 28945893.
4)

Vanessa T, Pelargos PE, Spasic M, Chung LK, Voth B, Ung N, Gopen Q, Yang I. Minimally Invasive Middle Fossa Keyhole Craniectomy for Repair of Superior Semicircular Canal Dehiscence. Oper Neurosurg (Hagerstown). 2017 Jun 1;13(3):317-323. doi: 10.1093/ons/opw046. PubMed PMID: 28521355.
5)

Nguyen T, Lagman C, Sheppard JP, Romiyo P, Duong C, Prashant GN, Gopen Q, Yang I. Middle cranial fossa approach for the repair of superior semicircular canal dehiscence is associated with greater symptom resolution compared to transmastoid approach. Acta Neurochir (Wien). 2017 Oct 11. doi: 10.1007/s00701-017-3346-2. [Epub ahead of print] Review. PubMed PMID: 29022108.
6)

Beckett JS, Chung LK, Lagman C, Voth BL, Jacky Chen CH, Gaonkar B, Gopen Q, Yang I. A Method of Locating the Dehiscence during Middle Fossa Approach for Superior Semicircular Canal Dehiscence Surgery. J Neurol Surg B Skull Base. 2017 Aug;78(4):353-358. doi: 10.1055/s-0037-1601886. Epub 2017 Apr 18. PubMed PMID: 28725523; PubMed Central PMCID: PMC5515662.
7)

Martin JE, Neal CJ, Monacci WT, Eisenman DJ. Superior semicircular canal dehiscence: a new indication for middle fossa craniotomy. Case report. J Neurosurg. 2004 Jan;100(1):125-7. PubMed PMID: 14743924.

Update: Geniculate neuralgia treatment

Geniculate neuralgia treatment

The treatment for geniculate neuralgia has not been established, although it seems reasonable that the therapeutic approaches used in other more common craniofacial neuralgias, such as trigeminal neuralgia, should be effective.

Conservative medical treatment is always the first-line therapy.

Mild cases may respond to carbamazepine sometimes in combination with phenytoin.

May responde to valproic acid.

Topical antibiotics for secondary infections of herpetic lesions.

Local anesthetic to external auditory canal.

Surgery

Surgical treatment should be offered if medical treatment fails. The two commonest surgical options are transection of the nervus intermedius, and microvascular decompression of the nerve at the nerve root entry zone of the brainstem. However, extracranial intratemporal division of the cutaneous branches of the facial nerve may offer a safer and similarly effective treatment.

The response to medical treatment for this condition varies between individuals. The long-term outcomes of surgery remain unknown because of limited data 1).

Rupa et al., postulate that geniculate ganglionectomy may be ineffective as the sole treatment for certain cases of geniculate neuralgia, and that nervus intermedius section may also be required to achieve a more complete deafferentation 2).

Excision of the nervus intermedius and/or of the geniculate ganglion by the middle cranial fossa approach without the production of facial paralysis, sometimes in combination with selective section of the Vth cranial nerve, has been successful in relieving the pain of geniculate neuralgia.

Microvascular decompression

Microvascular decompression may be effective as a treatment. Along its cisternal course, the nerve may be difficult to distinguish from the facial nerve. Based on case reports and small series, long-term pain control can be seen after nerve sectioning or microvascular decompression, but no prospective studies exist. Such studies are now necessary to shed light on the efficacy of surgical treatment of nervus intermedius neuralgia 3).

Complications

High-frequency hearing loss occurred after MVD for TGN, GPN, or GN, and the greatest incidence occurred on the ipsilateral side. This hearing loss may be a result of drill-induced noise and/or transient loss of cerebrospinal fluid during the course of the procedure. Changes in intraoperative BAEP waveforms were not useful in predicting HFHL after MVD. Repeated postoperative audiological examinations may be useful in assessing the prognosis of HFHL 4).

Case series

2002

Surgically excision of the nervus intermedius and geniculate ganglion via the middle cranial fossa approach, Review the long-term outcomes in 64 patients who were treated in this manner. Findings indicate that excision of the nervus intermedius and geniculate ganglion can be routinely performed without causing facial paralysis and that it is an effective definitive treatment for intractable geniculate neuralgia 5).

1991

A total of 31 surgical procedures were performed. Seventeen patients had sequential rhizotomies and one patient had microvascular decompression alone. Based on the clinical diagnosis, the nerves sectioned were singly or in combination: the nervus intermedius (14 patients), geniculate ganglion (10 patients), ninth nerve (14 patients), 10th nerve (11 patients), tympanic nerve (four patients), and chorda tympani nerve (one patient). Microvascular decompression of the involved nerves was undertaken in nine patients, in whom vascular loops were discovered. Adhesions (six patients), thickened arachnoid (three patients), and benign osteoma (one patient) were other intraoperative abnormalities noted. The overall success of these procedures in providing pain relief was 72.2%, and the mean follow-up period was 3.3 years (range 1 month to 14.5 years). There was no surgical mortality. Expected side effects were: decreased lacrimation, salivation, and taste related to nervus intermedius nerve section, and transient hoarseness and diminished gag related to ninth and 10th nerve section. Four patients developed sequelae consisting of sensorineural hearing loss, vertigo, and transient facial nerve paresis. One patient had a cerebrospinal fluid leak and another developed aseptic meningitis as postoperative complications. Except when primary glossopharyngeal neuralgia is the working diagnosis, a combined posterior cranial fossa-middle cranial fossa approach is recommended for adequate exploration and/or section of the fifth, ninth, and 10th cranial nerves as well as the geniculate ganglion and nervus intermedius 6).

1976

Excision of the nervus intermedius and/or of the geniculate ganglion by the middle cranial fossa approach without the production of facial paralysis, in any of 15 cases with geniculate neuralgia is reported. Use of these new techniques, sometimes in combination with selective section of the Vth cranial nerve, has been successful in relieving the pain of geniculate neuralgia 7).

Case reports

A 39-year-old man presented with a history of left “deep” ear pain within his ear canal. He noted occasional pain on the left side of his face around the ear. He had been treated with neuropathic pain medications without relief. His wife described suicidal ideations discussed by her husband because of the intense pain.

The patient’s neurologic examination was normal, and otolaryngologic consultation revealed no underlying structural disorder. Anatomic imaging revealed a tortuous vertebral artery-posterior inferior cerebellar artery complex with the posterior inferior cerebellar artery loop impinging on the root entry zone of the nervus intermedius-vestibulocochlear nerve complex and just inferior to the root entry zone of the facial nerve and a small anterior inferior cerebellar artery loop interposed between the cranial nerve VII-VIII complex and the hypoglossal and glossopharyngeal nerves. A left-sided retromastoid craniotomy was performed, and the nervus intermedius was transected. An arterial loop in contact with the lower cranial nerves at the level of the brainstem was mobilized with a polytetrafluoroethylene implant.

The patient indicated complete relief of his preoperative pain after surgery. He has remained pain-free with intact hearing and balance 8).

1)

Tang IP, Freeman SR, Kontorinis G, Tang MY, Rutherford SA, King AT, Lloyd SK. Geniculate neuralgia: a systematic review. J Laryngol Otol. 2014 May;128(5):394-9. doi: 10.1017/S0022215114000802. Review. PubMed PMID: 24819337.
2)

Rupa V, Weider DJ, Glasner S, Saunders RL. Geniculate ganglion: anatomic study with surgical implications. Am J Otol. 1992 Sep;13(5):470-3. PubMed PMID: 1443083.
3)

Tubbs RS, Steck DT, Mortazavi MM, Cohen-Gadol AA. The nervus intermedius: a review of its anatomy, function, pathology, and role in neurosurgery. World Neurosurg. 2013 May-Jun;79(5-6):763-7. doi: 10.1016/j.wneu.2012.03.023. Epub 2012 Apr 3. Review. PubMed PMID: 22484073.
4)

Thirumala P, Meigh K, Dasyam N, Shankar P, Sarma KR, Sarma DR, Habeych M, Crammond D, Balzer J. The incidence of high-frequency hearing loss after microvascular decompression for trigeminal neuralgia, glossopharyngeal neuralgia, or geniculate neuralgia. J Neurosurg. 2015 Dec;123(6):1500-6. doi: 10.3171/2014.10.JNS141101. Epub 2015 May 1. PubMed PMID: 25932612.
5)

Pulec JL. Geniculate neuralgia: long-term results of surgical treatment. Ear Nose Throat J. 2002 Jan;81(1):30-3. Review. PubMed PMID: 11816385.
6)

Rupa V, Saunders RL, Weider DJ. Geniculate neuralgia: the surgical management of primary otalgia. J Neurosurg. 1991 Oct;75(4):505-11. PubMed PMID: 1885967.
7)

Pulec JL. Geniculate neuralgia: diagnosis and surgical management. Laryngoscope. 1976 Jul;86(7):955-64. PubMed PMID: 933690.
8)

Tubbs RS, Mosier KM, Cohen-Gadol AA. Geniculate neuralgia: clinical, radiologic, and intraoperative correlates. World Neurosurg. 2013 Dec;80(6):e353-7. doi: 10.1016/j.wneu.2012.11.053. Epub 2012 Nov 23. PubMed PMID: 23178920.

Niedermeyer’s Electroencephalography: Basic Principles, Clinical Applications, and Related Fields

Niedermeyer’s Electroencephalography: Basic Principles, Clinical Applications, and Related Fields

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Neurosurgery Bestseller Books for 2018

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Events Update

2018


January

British Neurosurgical Trainee Course

January 8, 2018 — January 10, 2018

Cambridge, UK

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EANS Lyon Hands-On Course

January 8, 2018 — January 12, 2018

Lyon, France

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The impact of White Matter Anatomy for Brain tumor and Epilepsy surgery

January 17, 2018 — January 19, 2018

Zurich, Switzerland

More Information

17
Jan
» Microscopic and Endoscopic Approaches to the Skull Base
17 – 19 January 2018
 https://www.ircad.fr/training-center/course-calendar/?type=advanced&spec=neuro
 Brochure 


Leeds Neuroanatomy Course – Part II

January 18, 2018 — January 19, 2018

Leeds, UK

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AOSpine Principles Level Specimen Course—Principles and practice of spinal surgery: A circumferential approach

January 26, 2018 — January 27, 2018

Belfast, UK


Neurology and Neurosurgery – On the wards and on call

January 29, 2018

London, UK

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February


Ultrasound In Neurosurgery Training Course

February 1, 2018 — February 2, 2018

Birmingham, UK

More Information


Microsurgery of Aneurysms: Recent Advances

February 26, 2018 — March 1, 2018

St Louise, Missouri, USA

More Information


March


AOSpine Masters Symposium—Controversies in the management of degenerative spine disorders

March 2, 2018 — March 3, 2018

Porto, Portugal

More Information


Microsurgery Course on Vascular Anastomoses

March 5, 2018 — March 8, 2018

Pavia, Italy

More Information


 

SBNS (Society of British Neurological Surgeons) Spring Meeting – TORQUAY
11 – 13 April 2018
 http://www.sbns.org.uk/index.php/conferences/plymouth-2018/
 Call for Abstract 
 Brochure 

62nd Scientific Annual Meeting of the German Society for Clinical Neurophysiology and Functional Imaging (DGKN)

March 15, 2018 — March 17, 2018

Berlin, Germany

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International Conference on Neurology and Brain Disorders

March 19, 2018 — March 21, 2018

Valencia, Spain

More Information


 

The Third WFNS Foundation Rabat Live Surgery Seminar for Young African Neurosurgeons
23 – 25 March 2018
 Flyer 

April


SBNS Spring Meeting – Torquay

April 11, 2018 — April 13, 2018

Torquay, UK

More Information


11th Annual Cervical Spine Research Society Hands-On Cadaver Course

April 12, 2018 — April 14, 2018

St Louis, Missouri, USA

More Information


 

12th Annual Meeting of the Saudi Association of Neurological Surgery & 8th Neurosurgery Update Conference (SANS 2018)
15 – 16 April 2018
 http://www.sans.org.sa/index.php/event/sans-2018/


WFNS education course in conjunction with 4th ISMINS-Congress
19 – 21 April 2018
 http://www.congress-ph.ru/

Eurospine Spring Meeting 2018

April 26, 2018 — April 28, 2018

Vienna, Austria

Please see the website for further details.


86th AANS Annual Scientific Meeting

April 28, 2018 — May 2, 2018

New Orleans, LA, USA

More Information


May

26th Biennial Congress of the European Society for Pediatric Neurosurgery

May 1, 2018

Bonn, Germany

More Information


Global Spine Congress 2018

May 2, 2018 — May 5, 2018

Singapore

Website: http://www.gsc2018.org


26th Biennial Congress of the European Society for Pediatric Neurosurgery

May 6, 2018 — May 9, 2018

Bonn, Germany

More Information


34th Annual Meeting Cervical Spine Research Society – Europe

May 9, 2018 — May 11, 2018

Lisbon, Portugal

More Information


45th ISSLS Annual Meeting

May 14, 2018 — May 18, 2018

Banff, Canada

Annual meeting of The International Society for the Study of the Lumbar Spine.

Website: http://www.issls.org


Israeli National Neurosurgical Society Annual Meeting

May 16, 2018 — May 18, 2018

Galilee, Israel

More Information


SENEC 2018

May 16, 2018 — May 18, 2018

Toledo, Spain

22nd Congress of the SENEC.

Contact: secretaria@senec.es


ESOC 2018 – 4th European Stroke Organisation Conference

May 16, 2018 — May 18, 2018

Gothenburg, Sweden

More Information


White Matter Dissection, Lectures + Hands-On Cadaver Course

May 23, 2018 — May 24, 2018

Graz, Austria

More Information


aStroke Meeting Puglia 2018

May 24, 2018 — May 25, 2018

San Giovanni Rotondo, Italy

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Surgery Follows Function

May 25, 2018 — May 25, 2018

Graz, Austria

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June


69th Annual Meeting of the German Society of Neurosurgery (DGNC)
3 – 6 June 2018
 http://www.dgnc-kongress.de

15th Interdisciplinary Cerebrovascular Symposium

June 6, 2018 — June 8, 2018

Magdeburg, Germany

More Information


Microscopic and Endoscopic Approaches to the Skull Base

6 – 7 June 2018

 https://www.ircad.fr/training-center/course-calendar/?type=advanced&spec=neuro

 Brochure 


9th European Japanese Cerebrovascular Congress (EJCVC)

June 7, 2018

Milan, Italy

More Information


Endoscopy in Neurosurgery: the advanced three-day course

June 20, 2018 — June 22, 2018

London, UK

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13th European Low Grade Glioma Network

June 22, 2018 — June 23, 2018

Lisbon, Portugal

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EANS Lyon Hands-On Course

June 25, 2018 — June 29, 2018

Lyon, France

More Information


July


Seventh Annual World Course in Advanced Brain Tumour Surgery

July 12, 2018 — July 15, 2018

London, UK

More Information


CAANS 2018 Congress

24 – 27 July 2018

 http://www.caanscongress.info/

 Announcement 


 

August

INTS 2018

August 11, 2018 — August 16, 2018

Toronto, Canada

More Information

WFNS Symposium 2018

15TH – 19TH
AUGUST 2018

Hilton Kuala Lumpur,
Malaysia

http://wfns-symposia2018.com/


Prague Neurosurgical Week

August 29, 2018 — September 3, 2018

More Information


September

EUROSPINE 2018

September 19, 2018 — September 21, 2018

Barcelona, Spain

For more information please visit http://www.eurospinemeeting.org/f130000847.html

October


Spine in XXI Century

October 4, 2018 — October 8, 2018

Nis, Serbia

Association of Neurosurgeons of Russia
Russian Association of Spinal Surgeons
Serbian Neurosurgical Society

4th Meeting of the Serbian Neurosurgical Society
Joint Meeting with the Souteast Europe Neurosurgical Society

First announcement.


CNS Annual Meeting

October 6, 2018 — October 10, 2018

Chicago, IL, USA


13th EANO Meeting

October 9, 2018 — October 14, 2018

Stockholm, Sweden

Annual Meeting of the European Association of Neuro-oncology

Website.


Surgical anatomy of the arm in relation to nerve injuries

October 11, 2018 — October 12, 2018

Leiden, The Netherlands

More Information


EANS 2018

October 21, 2018 — October 25, 2018

Brussels, Belgium

More Information


4th European Congress of NeuroRehabilitation (ECNR) 2017

October 25, 2018 — October 28, 2018

Lausanne, Switzerland

More Information


ANTC 2017 – AIIMS NEUROTRAUMA CONFERENCE

October 27 — October 29

New Delhi, India

Taking place at the All India Institute of Medical Sciences.

Flyer.

November


Joint Global Neurofibromatosis Conference

November 2, 2018 — November 6, 2018

Paris, France

More Information


SNO 2018

November 15, 2018 — November 18, 2018

New Orleans, LA, USA

Society for Neuro-Oncology (SNO) Annual Meeting 2018

Website.

2019


January


2nd International Conference on Complications in Neurosurgery

January 25, 2019 — January 27, 2019

Mumbai, India

More Information


April


87th AANS Annual Scientific Meeting

April 13, 2019 — April 17, 2019

San Diego, CA, USA


September


EANS2019

September 24, 2019 — September 28, 2019

Dublin, Ireland

More Information


WFNS 2019 Interim Meeting

(9/9/2019 – 12/9/2019) / Beijing, China

http://www.wfns2019.org/index/


October


EUROSPINE 2019

October 16, 2019 — October 18, 2019

Helsinki, Finland

Please click HERE for more information.


CNS Annual Meeting

October 19, 2019 — October 23, 2019

San Francisco, CA, USA


SNO 2019

November 20, 2018 — November 24, 2018

Phoenix, AZ, USA

Society for Neuro-Oncology (SNO) Annual Meeting 2019

Website.

Update: Giant GH secreting pituitary adenoma

Giant GH secreting pituitary adenoma

Patients with acromegaly usually harbor pituitary macroadenomas measuring between 10 and 30 mm in maximal diameter. Giant (adenoma size ≥40 mm) GH secreting pituitary adenoma are rarely encountered.

They are invasive, uncontrolled by surgery, and respond poorly to medical treatment. Aggressive multimodal therapy is critical for their management, enhancing control rate and biochemical remission 1).

Case series

2017

Giordano et al., present the clinical, radiological and hormonal status of three patients affected by invasive GH-secreting pituitary adenomas without clinical signs and symptoms of acromegaly with elevation of serum IGF-1 from a series of 142 pituitary adenomas operated in the Department of Neurosurgery, International Neuroscience Institute-Hannover, Germany with the aid of intraoperative magnetic resonance imaging(MRI). Total tumor removal was possible in two of the three cases; the patients show normal hormonal status and no recurrence at long-term follow-up. In the third case, due to the different features of the tumor, complete resection was not possible and a multimodal treatment was performed that allowed regularization of the hormonal status and control of the residual tumor. GH-secreting adenomas without clinical manifestation of acromegaly are uncommon lesions. Total microsurgical excision can be curative. However, in case of partial removal, a tailored adjuvant treatment should be considered to preserve the quality of life of the patient and avoid regrowth of the lesion. In not resectable tumors, preoperative medical treatment with somatostatin analogues is always an option 2).

2015

Shimon et al. identified 34 patients (15 men and 19 females) with giant adenomas among 762 subjects (4.5%) with acromegaly, and characterized their clinical characteristics and response to treatment.

Mean age at diagnosis was 34.9±12.5 years (range, 16-67 years). Mean adenoma size was 49.4±9.4 mm (range, 40-80 mm); 30 adenomas showed cavernous sinus invasion and 32 had suprasellar extension. Twenty-nine (85%) patients had visual field defects. Mean baseline IGF1 was 3.4±1.8×ULN. All patients except one underwent pituitary surgery (one to three procedures), but none achieved hormonal remission following first surgery. Among the 28 subjects with visual disturbances, 14 recovered post-operatively and 13 improved. Treatment with somatostatin analogs was given to all patients after surgical failure. Six achieved remission, nine others were partially controlled (IGF1<1.5×ULN; 3/9 when combined with cabergoline), and 17 did not respond (two were lost). Nine patients were treated with pegvisomant, alone (n=4) or in combination with somatostatin analogs (n=5); five are in remission and two are partially controlled. Pasireotide-LAR achieved hormonal remission in one of the six patients. Currently, after a mean follow-up period of 8.9 years, 17 patients are in biochemical remission, eight are partially controlled, and seven are uncontrolled (two were lost to follow-up).

Giant GH-secreting adenomas are invasive, uncontrolled by surgery, and respond poorly to medical treatment. Aggressive multimodal therapy is critical for their management, enhancing control rate and biochemical remission 3).

Case reports

A 23-year-old male patient presented with continuous increase in height during the past 6 years due to a GH-secreting giant pituitary adenoma. Because of major intracranial extension and failure of octreotide treatment to shrink the tumour, the tumour was partially resected by a trans-frontal surgical approach. At immunohistochemistry, the tumour showed a marked expression of GH and a sparsely focal expression of prolactin. Somatostatin receptors (sst) 1-5 were not detected. Tumour tissue weakly expressed dopamine receptor type 2. The Gs alpha subunit was intact. Conversion from somatostatin analogue to pegvisomant normalized insulin-like-growth-factor-I (IGF-I) levels and markedly improved glucose tolerance.

Pegvisomant is a potent treatment option in patients with pituitary gigantism. In patients who do not respond to somatostatin analogues, knowledge of the SST receptor status may shorten the time to initiation of pegvisomant treatment 4).

1)

Shimon I, Jallad RS, Fleseriu M, Yedinak CG, Greenman Y, Bronstein MD. Giant GH-secreting pituitary adenomas: management of rare and aggressive pituitary tumors. Eur J Endocrinol. 2015 Jun;172(6):707-13. doi: 10.1530/EJE-14-1117. Epub 2015 Mar 19. PubMed PMID: 25792375.
2)

Giordano M, Samii A, Fahlbusch R. Aggressive somatotrophinomas lacking clinical symptoms: neurosurgical management. Neurosurg Rev. 2017 Dec 30. doi: 10.1007/s10143-017-0940-y. [Epub ahead of print] PubMed PMID: 29290044.
3)

Shimon I, Jallad RS, Fleseriu M, Yedinak CG, Greenman Y, Bronstein MD. Giant GH-secreting pituitary adenomas: management of rare and aggressive pituitary tumors. Eur J Endocrinol. 2015 Jun;172(6):707-13. doi: 10.1530/EJE-14-1117. Epub 2015 Mar 19. PubMed PMID: 25792375.
4)

Müssig K, Gallwitz B, Honegger J, Strasburger CJ, Bidlingmaier M, Machicao F, Bornemann A, Ranke MB, Häring HU, Petersenn S. Pegvisomant treatment in gigantism caused by a growth hormone-secreting giant pituitary adenoma. Exp Clin Endocrinol Diabetes. 2007 Mar;115(3):198-202. PubMed PMID: 17427111.