Bilateral anterior cingulotomy

Bilateral anterior cingulotomy

Bilateral anterior cingulotomy is a form of psychosurgery, introduced in 1948 as an alternative to lobotomy.

Lesioning of the target area is typically performed using bilateral stereotactic electrode placement and target ablation, which involves transparenchymal access through both hemispheres.

Lauri Laitinen was a pioneer of stereotactic psychosurgery in the 1950s to 1970s, especially by introducing the subgenual cingulotomy.

Bilateral anterior cingulotomy has been used to treat chronic painobsessive-compulsive disorder.

In the early years of the twenty-first century, it was used in Russia to treat addiction.

The objective of this surgical procedure is the severing of the supracallosal fibers of the cingulum bundle, which pass through the anterior cingulate gyrus.

Early localizationists linked anterior cingulate cortex (ACC: Brodmann’s area 24 and adjacent regions) with emotional behavior, paving the way for bilateral cingulotomy psychosurgery in severe, treatment resistant, cases of obsessive-compulsive disorder, chronic pain, depression, and substance abuse.

Limbic system surgery based on initial cingulotomy offers a durable and effective treatment option for appropriately selected patients with severe obsessive compulsive disorder who have not responded to conventional pharmacotherapy or psychotherapy 1).


There are features of anterior cingulate cortex structure and connectivity that predict clinical response to dorsal anterior cingulotomy for refractory obsessive compulsive disorder. These results suggest that the variability seen in individual responses to a highly consistent, stereotyped procedure may be due to neuroanatomical variation in the patients. Furthermore, these variations may allow us to predict which patients are most likely to respond to cingulotomy, thereby refining our ability to individualize this treatment for refractory psychiatric disorders 2)


The presence of neuropathic pain can severely impinge on emotional regulation and activities of daily living including social activities, resulting in diminished life satisfaction. Unfortunately, the majority of patients with neuropathic pain do not experience an amelioration of symptoms from conventional therapies, even when multimodal therapies are used. Chronic refractory neuropathic pain is usually accompanied by severe depression that is prone to incur suicidal events; thus clinical management of chronic neuropathic pain and depression presents a serious challenge for clinicians and patients

Two patients presented with neuropathic pain and severe depression. The patients had different pain symptoms emerging a few months after central or peripheral nervous system impairment. These symptoms were associated with the development of severe depression, social isolation, and a gradual inability to perform daily activities. Both patients were referred for bilateral anterior cingulotomy. After surgery, both patients showed significant progressive improvements in perceived pain, mental health status, and daily functioning.

Bilateral anterior cingulotomy may serve as an alternative treatment for medically refractory neuropathic pain, especially for patients who also experience depression 3).


Stereotactic anterior cingulotomy has been used in the treatment of patients suffering from refractory oncological pain due to its effects on pain perception. However, the optimal targets as well as suitable candidates and outcome measures have not been well defined. We report our initial experience in the ablation of 2 cingulotomy targets on each side and the use of the Brief Pain Inventory (BPI) as a perioperative assessment tool.

A retrospective review of all patients who underwent stereotactic anterior cingulotomy in our Department between November 2015 and February 2017 was performed. All patients had advanced metastatic cancer with a limited prognosis and suffered from intractable oncological pain.

Thirteen patients (10 women and 3 men) underwent 14 cingulotomy procedures. Their mean age was 54 ± 14 years. All patients reported substantial pain relief immediately after the operation. Out of the 6 preoperatively bedridden patients, 3 started ambulating shortly after. At the 1-month follow-up, the mean preoperative Visual Analogue Scale score decreased from 9 ± 0.9 to 4 ± 2.7 (p = 0.003). Mean BPI pain severity and interference scores decreased from levels of 29 ± 4 and 55 ± 12 to 16 ± 12 (p = 0.028) and 37 ± 15 (p = 0.043), respectively. During the 1- and 3-month follow-up visits, 9/11 patients (82%) and 5/7 patients (71%) available for follow-up reported substantial pain relief. No patient reported worsening of pain during the study period. Neuropsychological analyses of 6 patients showed stable cognitive functions with a mild nonsignificant decline in focused attention and executive functions. Adverse events included transient confusion or mild apathy in 5 patients (38%) lasting 1-4 weeks.

The initial experience indicates that double stereotactic cingulotomy is safe and effective in alleviating refractory oncological pain 4).

Four MRgLITT bilateral cingulotomy procedures were performed in 3 patients. Two patients had a single MRgLITT procedure while the third had repeat ablation after pain recurrence. First time ablation coordinates were (medians): x = 7.9 mm (range, 6.9-8.6); y = 20.5 mm (range, 20-22); z = 6.9 mm (range, 2.9-7.0) above the lateral ventricle roof. Median trajectory length was 85.5 mm (range, 80-90). Median ablation volume was 1.5 cm3 (range, 0.6-1.2). Median ablation time was 257 seconds (range, 136-338) per cingulum and power was 10.0 Watts (range, 10-11). Median preoperative pain severity (PSS) and interference scores (PIS) were 7.7 (range, 7.5-9.3) and 9.9 (range, 9.7-10.0), respectively. Median postoperative PSS and PIS scores were 1.6 (range, 1.0-2.8) and 2.0 (range, 0.3-2.6), respectively.

MRgLITT cingulotomy is well tolerated for treatment of cancer pain and can be easily performed framelessly for appropriate candidates 5).


Seven patients suffering from refractory OCD underwent stereotactic surgery and were followed for 12 months. The Yale-Brown Obsessive Compulsive Scale (Y-BOCS) was used to assess the efficacy. The test was taken before and 6 and 12 months after surgery.

The mean Y-BOCS scores decreased significantly from 32.9 ± 4.7 at baseline to 20.6 ± 5.3 after 12 months. Five out of the 7 patients showed a decrease of more than 35%. During the 12-month follow-up, the effective rate had increased from 28.6 to 71.4%. There were no significant adverse effects observed after surgery.

The BACI and BACA were effective for the treatment of refractory OCD, and no significant adverse effects on long-term follow-up were found 6).


Bilateral radiofrequency cingulotomy was performed in 10 patients. The technique involved stereotaxis using magnetic resonance guidance and local anesthesia, with the placement of a radiofrequency lesion (75 degrees, 60s). Of the 10 patients, 8 had metastatic lesions with musculoskeletal (6) or neurogenic (2) pain. Pain relief was judged excellent (4 patients), fair (1), poor (2) and excellent for 6 months poor in the last patient. The two benign lesions were neurofibromatosis with neurogenic pain and thalamic pain from an old stroke. Pain relief (with 1 year follow-up) in this group was judged excellent in one and poor in the other (thalamic pain) 7).


Forty-two patients out of 300 who had undergone bilateral stereotactic cingulotomies were studied by means of computerized tomography (CT). The appearance showed bilateral encephalomalacia, measuring on the average 5 X 7 mm2, located in the cingulate gyrus. These induced lesions had attenuation values similar to cerebrospinal fluid and did not enhance with contrast. CT is a useful technique for initial evaluation, management, and follow up of these patients 8).

In end-stage cancer, oncologic pain refractory to medical management significantly reduces patient’s quality of life. In recent years, ablative surgery has seen a resurgence in treating diffuse and focal cancer pain in terminal patients. The anterior cingulate gyrus has been a key focus as it plays a role in the cognitive and emotional processing of pain. While radiofrequency ablation of the dorsal anterior cingulate is well-described for treating cancer pain, MRI-guided laser-induced thermal therapy (LITT) is novel. Allam et al. describes a patient treated with an MRI-guided LITT therapy of the anterior cingulate gyrus for intractable debilitating pain secondary to terminal metastatic cancer 9).


Huotarinen et al., found 1 patient alive who underwent subgenual cingulotomy in 1971 for obsessive thoughts, anxiety, and compulsions, diagnosed at that time as “schizophrenia psychoneurotica.” MRI showed bilateral subgenual cingulotomy lesions (254 and 160 mm3, respectively). The coordinates of the center of the lesions in relation to the midcommissural point for the right and left, respectively, were: 7.1 and 7.9 mm lateral; 0.2 mm inferior and 1.4 mm superior, and 33.0 and 33.9 anterior, confirming correct subgenual targeting. The patient reported retrospective satisfactory results.

The lesion in this patient was found to be in the expected location, which gives some verification of the correct placement of Laitinen’s subgenus cingulotomy target 10).


A case of debilitating thoracic wall pain due to malignant mesothelioma relieved by bilateral anterior cingulotomy is described and changes in dyspnoea investigated.

Improvements in pain, dyspnoea and the extent to which either symptom bothered the patient was seen for 2 months after surgery before disease progression led to death 5 months after surgery. Quality of life improvements were also seen for 2 months after surgery and pain relief was sustained from surgery to death. Arterial blood gas and lung function tests were unchanged by surgery, suggesting a reduction in pain and dyspnoea awareness by cingulotomy.

Bilateral anterior cingulotomy effectively relieved both pain and dyspnoea. The role of the anterior cingulate cortex in pain and autonomic control of respiration is discussed alongside the evidence for this palliative procedure for cancer pain 11).

by Ernest. Feigenbaum (Author)


1)

Sheth SA, Neal J, Tangherlini F, Mian MK, Gentil A, Cosgrove GR, Eskandar EN, Dougherty DD. Limbic system surgery for treatment-refractory obsessive-compulsive disorder: a prospective long-term follow-up of 64 patients. J Neurosurg. 2013 Mar;118(3):491-7. doi: 10.3171/2012.11.JNS12389. Epub 2012 Dec 14. PubMed PMID: 23240700.
2)

Banks GP, Mikell CB, Youngerman BE, Henriques B, Kelly KM, Chan AK, Herrera D, Dougherty DD, Eskandar EN, Sheth SA. Neuroanatomical Characteristics Associated With Response to Dorsal Anterior Cingulotomy for Obsessive-Compulsive Disorder. JAMA Psychiatry. 2014 Dec 23. doi: 10.1001/jamapsychiatry.2014.2216. [Epub ahead of print] PubMed PMID: 25536384.
3)

Deng Z, Pan Y, Li D, Zhang C, Jin H, Wang T, Zhan S, Sun B. Effect of Bilateral Anterior Cingulotomy on Chronic Neuropathic Pain with Severe Depression. World Neurosurg. 2019 Jan;121:196-200. doi: 10.1016/j.wneu.2018.10.008. Epub 2018 Oct 10. PubMed PMID: 30315971.
4)

Strauss I, Berger A, Ben Moshe S, Arad M, Hochberg U, Gonen T, Tellem R. Double Anterior Stereotactic Cingulotomy for Intractable Oncological Pain. Stereotact Funct Neurosurg. 2018 Jan 10;95(6):400-408. doi: 10.1159/000484613. [Epub ahead of print] PubMed PMID: 29316566.
5)

Patel NV, Agarwal N, Mammis A, Danish SF. Frameless stereotactic magnetic resonance imaging-guided laser interstitial thermal therapy to perform bilateral anterior cingulotomy for intractable pain: feasibility, technical aspects, and initial experience in 3 patients. Neurosurgery. 2015 Mar;11 Suppl 2:17-25; discussion 25. doi: 10.1227/NEU.0000000000000581. PubMed PMID: 25584953.
6)

Zhang QJ, Wang WH, Wei XP. Long-term efficacy of stereotactic bilateral anterior cingulotomy and bilateral anterior capsulotomy as a treatment for refractory obsessive-compulsive disorder. Stereotact Funct Neurosurg. 2013;91(4):258-61. doi: 10.1159/000348275. Epub 2013 May 7. PubMed PMID: 23652367.
7)

Pillay PK, Hassenbusch SJ. Bilateral MRI-guided stereotactic cingulotomy for intractable pain. Stereotact Funct Neurosurg. 1992;59(1-4):33-8. PubMed PMID: 1295044.
8)

Bernad PG, Ballantine HT. Computed tomographic analysis of bilateral cingulotomy for intractable mood disturbance and chronic pain. Comput Radiol. 1987 May-Jun;11(3):117-23. PubMed PMID: 3301189.
9)

Allam AK, Larkin MB, Katlowitz KA, Shofty B, Viswanathan A. Case report: MR-guided laser induced thermal therapy for palliative cingulotomy. Front Pain Res (Lausanne). 2022 Nov 1;3:1028424. doi: 10.3389/fpain.2022.1028424. PMID: 36387414; PMCID: PMC9663803.
10)

Huotarinen A, Kivisaari R, Hariz M. Laitinen’s Subgenual Cingulotomy: Anatomical Location and Case Report. Stereotact Funct Neurosurg. 2018;96(5):342-346. doi: 10.1159/000492058. Epub 2018 Oct 2. PubMed PMID: 30278436.
11)

Pereira EA, Paranathala M, Hyam JA, Green AL, Aziz TZ. Anterior cingulotomy improves malignant mesothelioma pain and dyspnoea. Br J Neurosurg. 2014 Aug;28(4):471-4. doi: 10.3109/02688697.2013.857006. Epub 2013 Nov 7. PubMed PMID: 24199940.

Transradial access

Transradial access

Radial artery approach is based on the desire to diminish the incidence rate of haemorrhagic complications in the zone of the puncture and to avoid the necessity of a long-term bed rest in femoral artery approach. The findings obtained in numerous studies of coronary stenting and in a series of works on stenting of carotid arteries have demonstrated that the transradial approach reduces the risk of haemorrhage and local vascular complications.


It is important to be aware of the Aberrant right subclavian artery (ARSA) before surgical approaches to upper thoracic vertebrae in order to avoid complications and effect proper treatment. In patients with a known ARSA, a right transradial approach for aortography or cerebral angiography should be changed to a left radial artery or transfemoral artery approach 1).


Neurointerventionalists attempting the transradial approach can expect to achieve moderate early success and a low complication rate 2).

They can overcome the right transradial learning curve and achieve high success rates and low crossover rates after performing 30-50 cases 3).


The femoral artery is the most common access route for cerebral angiography and neurointerventional procedures. Complications of the transfemoral approach include groin hemorrhages and hematomas, retroperitoneal hematomas, pseudoaneurysms, arteriovenous fistulas, peripheral artery occlusions, femoral nerve injury, and access-site infections. Incidence rates vary among different randomized and nonrandomized trials, and the literature lacks a comprehensive review of this subject.

Oneissi et al. gather data from 16 randomized clinical trials (RCT) and 17 nonrandomized cohort studies regarding femoral access-site complications for a review paper. They also briefly discussed management strategies for these complications based on the most recent literature.

A PubMed indexed search for all neuroendovascular clinical trials, retrospective studies, and prospective studies that reported femoral artery access-site complications in neurointerventional procedures.

The overall access-site complication rate in RCTs is 5.13%, while in non-RCTs, the rate is 2.78%. The most common complication in both groups is groin hematoma followed by access-site hemorrhage and femoral artery pseudoaneurysm. On the other hand, wound infection was the least common complication.

The transfemoral approach in neuroendovascular procedures holds risk for several complications. This review will allow further studies to compare access-site complications between the transfemoral approach and other alternative access sites, mainly the trans-radial artery approach, which is gaining a lot of interest nowadays 4).

Radial Access for Neurointervention.

Edited by Pascal Jabbour and Eric Peterson

The only book on the market on Transradial Access (TRA) Includes many movies and pictures to give practical help to people transitioning their practice to TRA Chapters are written by 2 pioneers in TRA who started the technique and have published most of the papers on TRA

McPheeters MJ. Book Review: Radial Access for Neurointervention. Neurosurgery. 2022 Mar 1;90(3):e63-e64. doi: 10.1227/NEU.0000000000001824. Epub 2021 Dec 27. PMID: 35849496.

In a high-volume, dual-center, retrospective analysis of each institution’s data base between June 2018 and June 2020 and a collection of all patients treated with flow diversion via transradial access. Patient demographic information and procedural and radiographic data were obtained.

Results: Seventy-four patients were identified (64 female patients) with a mean age of 57.5 years with a total of 86 aneurysms. Most aneurysms were located in the anterior circulation (93%) and within the intracranial ICA (67.4%). The mean aneurysm size was 5.5 mm. Flow diverters placed included the Pipeline Embolization Device (Flex) (PED, n = 65), the Surpass Streamline Flow Diverter (n = 8), and the Flow-Redirection Endoluminal Device (FRED, n = 1). Transradial access was successful in all cases, but femoral crossover was required in 3 cases (4.1%) due to tortuous anatomy and inadequate support of the catheters in 2 cases and an inability to navigate to the target vessel in a patient with an aberrant right subclavian artery. All 71 other interventions were successfully performed via the transradial approach (95.9%). No access site complications were encountered. Asymptomatic radial artery occlusion was encountered in 1 case (3.7%).

Conclusions: Flow diverters can be successfully placed via the transradial approach with high technical success, low access site complications, and a low femoral crossover rate 5).


Intra-arterial chemotherapy (IAC) has become one of the most important pillars in retinoblastoma (Rb) management. It allows for targeted delivery of chemotherapy by superselective catheterization of the ophthalmic artery, thus, reducing systemic toxicity. As in most neurovascular procedures, IAC has traditionally been performed through transfemoral access. However, recent publications have spurred the use of the trans-radial route for neuroendovascular procedures due to its lower complication rates and higher patient satisfaction. They presents the first case series in the literature on the technique, safety, and feasibility of IAC via the trans-radial route in the pediatric population.

Al Saiegh et al. retrospectively analyzed the prospectively maintained database and present the technique and initial experience from 5 consecutive pediatric patients aged between 3 and 15 years who underwent 10 trans-radial IAC treatments.

All IACs were performed successfully. Two patients had repeat IACs through the same wrist. There were no thromboembolic events or access site complications, such as hand ischemia or hematoma. All patients were discharged home the same day of the procedure.

This case series demonstrates the safety and feasibility of transradial IAC in pediatric patients with Rb. As more experience is gained with the transradial route for neurovascular procedures in adults, it may become the preferred route in some pediatric patients as well 6).


Chen et al. reviewed a prospective institutional database for all patients who underwent a transradial neurointerventional procedure between 2015 and 2019. Index procedures were defined as procedures performed via TRA after which there was a second TRA procedure attempted. Reasons for conversion to a transfemoral approach (TFA) for subsequent procedures were identified.

104 patients underwent 237 procedures (230 TRA, 7 TFA). 97 patients underwent ≥2 TRA procedures, 20 patients >3, four patients >4, three patients >5, and two patients >6 TRA procedures. The success rate was 94.7% (126/133) with 52% (66/126) of successive procedures performed via the same radial access site (snuffbox vs antebrachial) while the alternate radial artery segment was used for access in 48% (60/126) of subsequent procedures. There were seven (5.3%) cases requiring crossover to TFA, six cases for radial artery occlusion (RAO) and one for radial artery narrowing.

Successive TRA is both technically feasible and safe for neuroendovascular procedures in up to six procedures. The low failure rate (5.3%) was primarily due to RAO. Thus, even without clinical consequences, strategies to minimize RAO should be optimized for patients to continue to benefit from TRA in future procedures 7).


A study from Shchanitsyn et al., was aimed at comparative analysis of the transradial versus transfemoral approach used in carotid stenting. They retrospectively analysed the results of transradial and transfemoral stenting of carotid artery in a total of 168 patients. The operations had been performed in two centres over the period from 2012 to 2017. They evaluated the clinical and angiographic data, technical aspects of the operations, as well as the outcomes and complications. In particular, they compared such complications as stroke, transient ischemic attack, myocardial infarction and local complications of the approach. They carried out a univariate analysis of the risk for the development of complications depending on the method of the approach. Stenting of carotid arteries had been performed in 75 patients through the radial artery approach and in 93 patients via the femoral one. Comparing the two groups, the main clinical and angiographic data appeared to have no statistically significant differences. Various techniques of catheterization had been used depending upon anatomical peculiarities. The success of the procedure was achieved in 100% of cases, with the frequency of conversion amounting to 4% for the radial approach and to 1% for the femoral one (p=0.087). Amongst complications encountered, disabling stroke was revealed in two (1.2%) patients and minor stroke in four (2.4%). The groups did not differ by the incidence of neurological complications. Within 30 postoperative days neither lethal outcomes nor myocardial infarction were registered. Neither were there haemorrhagic events or other approach-related complications, however in the transradial-approach group, seven (9.3%) patients were found to have developed asymptomatic occlusions of the radial artery. The duration of the operation, the radiation load, and the length of hospital stay had no statistically significant differences depending on the approach used. Hence, the transradial approach is an effective and safe method in stenting of carotid arteries. In patients with high risk of haemorrhagic complications from the side of the vascular approach and with difficult anatomy of the aortic arch and its branches, hampering catheterization of the carotid artery via the femoral approach, the radial artery may be considered as an advantageous site of access 8).


1)

Choi Y, Chung SB, Kim MS. Prevalence and Anatomy of Aberrant Right Subclavian Artery Evaluated by Computed Tomographic Angiography at a Single Institution in Korea. J Korean Neurosurg Soc. 2019 Mar;62(2):175-182. doi: 10.3340/jkns.2018.0048. Epub 2019 Feb 27. PubMed PMID: 30840972; PubMed Central PMCID: PMC6411572.
2)

Zussman BM, Tonetti DA, Stone J, Brown M, Desai SM, Gross BA, Jadhav A, Jovin TG, Jankowitz BT. A prospective study of the transradial approach for diagnostic cerebral arteriography. J Neurointerv Surg. 2019 Mar 6. pii: neurintsurg-2018-014686. doi: 10.1136/neurintsurg-2018-014686. [Epub ahead of print] PubMed PMID: 30842303.
3)

Zussman BM, Tonetti DA, Stone J, Brown M, Desai SM, Gross BA, Jadhav A, Jovin TG, Jankowitz BT. Maturing institutional experience with the transradial approach for diagnostic cerebral arteriography: overcoming the learning curve. J Neurointerv Surg. 2019 Apr 27. pii: neurintsurg-2019-014920. doi: 10.1136/neurintsurg-2019-014920. [Epub ahead of print] PubMed PMID: 31030189.
4)

Oneissi M, Sweid A, Tjoumakaris S, Hasan D, Gooch MR, Rosenwasser RH, Jabbour P. Access-Site Complications in Transfemoral Neuroendovascular Procedures: A Systematic Review of Incidence Rates and Management Strategies. Oper Neurosurg (Hagerstown). 2020 May 4. pii: opaa096. doi: 10.1093/ons/opaa096. [Epub ahead of print] PubMed PMID: 32365203.
5)

Kühn AL, Satti SR, Eden T, de Macedo Rodrigues K, Singh J, Massari F, Gounis MJ, Puri AS. Anatomic Snuffbox (Distal Radial Artery) and Radial Artery Access for Treatment of Intracranial Aneurysms with FDA-Approved Flow Diverters. AJNR Am J Neuroradiol. 2021 Jan 14. doi: 10.3174/ajnr.A6953. Epub ahead of print. PMID: 33446501.
6)

Al Saiegh F, Chalouhi N, Sweid A, Mazza J, Mouchtouris N, Khanna O, Tjoumakaris S, Gooch R, Shields CL, Rosenwasser R, Jabbour P. Intra-arterial chemotherapy for retinoblastoma via the transradial route: Technique, feasibility, and case series. Clin Neurol Neurosurg. 2020 Apr 6;194:105824. doi: 10.1016/j.clineuro.2020.105824. [Epub ahead of print] PubMed PMID: 32283473.
7)

Chen SH, Brunet MC, Sur S, Yavagal DR, Starke RM, Peterson EC. Feasibility of repeat transradial access for neuroendovascular procedures. J Neurointerv Surg. 2019 Oct 5. pii: neurintsurg-2019-015438. doi: 10.1136/neurintsurg-2019-015438. [Epub ahead of print] PubMed PMID: 31586940.
8)

Shchanitsyn IN, Sharafutdinov MR, Iakubov RA, Larin IV. [Transradial approach in carotid stenting]. Angiol Sosud Khir. 2018;24(2):114-122. Russian. PubMed PMID: 29924782.

Craniopharyngioma (CP)

Craniopharyngioma (CP)

A craniopharyngioma (CP) is an embryonic malformation of the sellar region and parasellar region.

Its relation to Rathke’s cleft cyst (RCC) is controversial, and both lesions have been hypothesized to lie on a continuum of ectodermal cystic lesions of the sellar region.

Craniopharyngiomas frequently grow from remnants of the Rathke pouch, which is located on the cisternal surface of the hypothalamic region. These lesions can also extend elsewhere in the infundibulohypophyseal axis.

These tumors can also grow from the infundibulum or tuber cinereum on the floor of the third ventricle, developing exclusively into the third ventricle.

Jakob Erdheim (1874-1937) was a Viennese pathologist who identified and defined a category of pituitary tumors known as craniopharyngiomas. He named these lesions “hypophyseal duct tumors” (Hypophysenganggeschwülste), a term denoting their presumed origin from cell remnants of the hypophyseal duct, the embryological structure through which Rathke’s pouch migrates to form part of the pituitary gland. He described the two histological varieties of these lesions as the adamantinomatous and the squamous-papillary types. He also classified the different topographies of craniopharyngiomas along the hypothalamus-pituitary axis. Finally, he provided the first substantial evidence for the functional role of the hypothalamus in the regulation of metabolism and sexual functions. Erdheim’s monograph on hypophyseal duct tumors elicited interest in the clinical effects and diagnosis of pituitary tumors. It certainly contributed to the development of pituitary surgery and neuroendocrinology. Erdheim’s work was greatly influenced by the philosophy and methods of research introduced to the Medical School of Vienna by the prominent pathologist Carl Rokitansky. Routine practice of autopsies in all patients dying at the Vienna Municipal Hospital (Allgemeines Krankenhaus), as well as the preservation of rare pathological specimens in a huge collection stored at the Pathological-Anatomical Museum, represented decisive policies for Erdheim’s definition of a new category of epithelial hypophyseal growths. Because of the generalized use of the term craniopharyngioma, which replaced Erdheim’s original denomination, his seminal work on hypophyseal duct tumors is only referenced in passing in most articles and monographs on this tumor.

Jakob Erdheim should be recognized as the true father of craniopharyngiomas 1).

Craniopharyngioma epidemiology.

Its relation to Rathke’s cleft cyst (RCC) is controversial, and both lesions have been hypothesized to lie on a continuum of cystic ectodermal lesions of the sellar region.

It grows close to the optic nervehypothalamus and pituitary gland.

Craniopharyngioma Classification.

Genetic and immunological markers show variable expression in different types of CraniopharyngiomaBRAF is implicated in tumorigenesis in papillary Craniopharyngioma (pCP), whereas CTNNB1 and EGFR are often overexpressed in adamantinomatous Craniopharyngioma (aCP) and VEGF is overexpressed in aCP and Craniopharyngioma recurrence. Targeted treatment modalities inhibiting thesepathways can shrink or halt progression of CP. In addition, Epidermal growth factor receptor tyrosine kinase inhibitors may sensitize tumors to radiation therapy. These – drugs show promise in medical management and neoadjuvant therapy for CP. Immunotherapy, including anti-interleukin 6 (IL-6) drugs and interferon treatment, are also effective in managing tumor growth. Ongoing – clinical trials in CP are limited but are testing BRAF/MET inhibitors and IL-6 monoclonal antibodies.

Genetic and immunological markers show variable expression in different subtypes of CP. Several current molecular treatments have shown some success in the management of this disease. Additional clinical trials and targeted therapies will be important to improve CP patient outcomes 2).

Craniopharyngioma natural history.

see Craniopharyngioma Clinical Features.

see Craniopharyngioma Diagnosis.

Rathke’s cleft cyst.


ependymomapilocytic astrocytomachoroid plexus papilloma (CPP), craniopharyngiomaprimitive neuroectodermal tumor (PNET), choroid plexus carcinoma (CPC), immature teratomaatypical teratoid rhabdoid tumor (AT/RT), anaplastic astrocytoma, and gangliocytoma.


Compared with craniopharyngiomas, sellar gliomas presented with a significantly lower ratio of visual disturbances, growth hormone deficiencies, lesion cystic changes, and calcification. Sellar gliomas had significantly greater effects on the patients’ mentality and anatomical brain stem involvement 3).

Simultaneous sellar-suprasellar craniopharyngioma and intramural clival chordoma, successfully treated by a single staged, extended, fully endoscopic endonasal approach, which required no following adjuvant therapy is reported 4).

see Craniopharyngioma treatment

see Craniopharyngioma outcome

Craniopharyngioma: Surgical Treatment.

Craniopharyngioma Selected Works.

see Craniopharyngioma case series.

see Craniopharyngioma case reports.

Craniopharyngioma Videos


1)

Pascual JM, Rosdolsky M, Prieto R, Strauβ S, Winter E, Ulrich W. Jakob Erdheim (1874-1937): father of hypophyseal-duct tumors (craniopharyngiomas). Virchows Arch. 2015 Jun 19. [Epub ahead of print] PubMed PMID: 26089144.
2)

Reyes M, Taghvaei M, Yu S, Sathe A, Collopy S, Prashant GN, Evans JJ, Karsy M. Targeted Therapy in the Management of Modern Craniopharyngiomas. Front Biosci (Landmark Ed). 2022 Apr 20;27(4):136. doi: 10.31083/j.fbl2704136. PMID: 35468695.
3)

Deng S, Li Y, Guan Y, Xu S, Chen J, Zhao G. Gliomas in the Sellar Turcica Region: A Retrospective Study Including Adult Cases and Comparison with Craniopharyngioma. Eur Neurol. 2014 Dec 18;73(3-4):135-143. [Epub ahead of print] PubMed PMID: 25531372.
4)

Iacoangeli M, Rienzo AD, Colasanti R, Scarpelli M, Gladi M, Alvaro L, Nocchi N, Scerrati M. A rare case of chordoma and craniopharyngioma treated by an endoscopic endonasal, transtubercular transclival approach. Turk Neurosurg.2014;24(1):86-9. doi: 10.5137/1019-5149.JTN.7237-12.0. PubMed PMID: 24535799.
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