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.

Pterional craniotomy

Pterional craniotomy

The resulting bone flap is centered over the depression of the sphenoid ridge. Approximately 33% of the craniotomy is anterior to the anterior margin of temporalis muscle insertion, ≈ 66% is posterior.

With the craniotome, starting at the frontal burr hole the craniotomy is taken anteriorly across the anterior margin of the superior temporal line, staying as low as possible on the orbit (to obviate having to rongeur bone, which is unsightly on the forehead). The distance “B” from the medial extent of the craniotomy to the frontal burr hole is 3 cm for anterior circulation aneurysms. For the approaches to skull base (e.g. Dolenc approach), distance “B” is larger and takes the opening to ≈ the mid orbit. Then from point “B,” a sharp superior turn is made and the opening is taken back to point “A.” The height (“H”) of the craniotomy needs to be only ≈ 3 cm for aneurysms of the Circle of Willis, and slightly larger (≈ 5 cm) for the middle cerebral artery aneurysms. Minimal exposure of the temporal cortex is necessary for aneurysms of the skull base region. For large flaps (e.g. for tumors), “H” is made larger to expose more temporal lobe.


Frontotemporal craniotomy under regional anesthesia during awake craniotomies provides better pain control, a reduction in opioid use, and less somnolence in the early postoperative period 1)


Frontotemporal craniotomy, also known as “pterional craniotomy” (PC), provides an optimal microscopic exposure and a wide open working space for manipulation of intracranial structures, and it has been widely used in the field of neurosurgery for treatment of lesions in the anterior and posterior circulations 2).

The pterional craniotomy provides wide, multidirectional access to the anterior and middle cranial fossae as well as many structures of the interpeduncular fossae.

Other frontotemporal craniotomies derived from the pterional 3) 4) and supraorbital 5) craniotomies, as are the combined epi- and subdural approach with anterior clinoid removal 6) 7) and the orbitozygomatic extension of the pterional craniotomy 8) 9).

The pterional craniotomy is well established for microsurgical clipping of most anterior circulation aneurysms. The incision and temporalis muscle dissection impacts postoperative recovery and cosmetic outcomes.

The minipterional (MPT) craniotomy offers similar microsurgical corridors, with a substantially shorter incision, less muscle dissection, and a smaller craniotomy flap.


Although pterional craniotomy and its variants are the most used approaches in neurosurgery, few studies have evaluated their precise indications.

da Silva et al., evaluated the pterional (PT), pretemporal (PreT), and orbitozygomatic (OZ) approaches through quantitative measurements of area, linear, and angular exposures of the major intracranial vascular structures.

Eight fresh, adult cadavers were operated with the PT, followed by the PreT, and ending with the OZ approach. The working area, angular exposure of vascular structures and linear exposure of the basilar artery were measured.

The OZ approach presented a wider area (1301.3 ± 215.9 mm2) with an increase of 456.7 mm2 compared with the PT and of 167.4 mm2 to the PreT (P = 0.011). The extension from PT to PreT and OZ increases linear exposure of the basilar artery. When comparing the PreT and OZ, they founded an increase in the horizontal and vertical angle to the bifurcation of the ipsilateral middle cerebral artery (P = 0.005 and P = 0.032, respectively), horizontal angle to the basilar artery tip (P = 0.02), and horizontal angle to the contralateral ICA bifurcation (P = 0.048).

The OZ approach offered notable surgical advantages compared with the traditional PT and PreT regarding to the area of exposure and linear exposure to basilar artery. Regarding angle of attack, the orbital rim and zygomatic arch removal provided quantitatively wider exposure and increased surgical freedom. A detailed anatomic study for each patient and surgeon experience must be considered for individualized surgical approach indication 10).

see pterional approach.

Pterional craniotomy complications.

Left pterional craniotomy for thrombectomy and clipping of ruptured left MCA giant aneurysm

Mini-pterional craniotomy


1)

Bojaxhi E, Louie C, ReFaey K, Gruenbaum SE, Leone BJ, Bechtel P, Barbosa MP, Chaichana KL, Quinones-Hinojosa A. Reduced Pain and Opioid Use in the Early Postoperative Period in Patients Undergoing a Frontotemporal Craniotomy under Regional vs General Anesthesia. World Neurosurg. 2021 Jun;150:e31-e37. doi: 10.1016/j.wneu.2021.02.009. Epub 2021 Mar 5. Erratum in: World Neurosurg. 2022 Apr 19;163:2. PMID: 33684585.
2)

Kang HJ, Lee YS, Suh SJ, Lee JH, Ryu KY, Kang DG. Comparative analysis of the mini-pterional and supraorbital keyhole craniotomies for unruptured aneurysms with numeric measurements of their geometric configurations. J Cerebrovasc Endovasc Neurosurg. 2013 Mar;15(1):5–12.
3)

Yasargil MG: Microneurosurgery. Stuttgart: Georg Thieme, 1984, Vol I
4)

Yasargil MG, Fox JL, Ray MW: The operative approach to aneurysms of the anterior communicating artery, in Krayenbül H (ed): Advances and Technical Standards in Neurosurgery. Wien: Springer-Verlag, 1975, Vol 2, pp 114–170
5)

Jane JA, Park TS, Pobereskin LH, et al: The supraorbital approach: technical note. Neurosurgery 11:537–542, 1982
6)

Dolenc VV: A combined epi-and subdural direct approach to carotidophthalmic artery aneurysms. J Neurosurg 62:667–672, 1985
7)

Dolenc VV, Yasargil MG: Anatomy and Surgery of the Cavernous Sinus. Wien: Springer-Verlag, 1989
8)

Fujitsu K, Kiwabara T: Zygomatic approach for lesions in the interpeduncular cistern. J Neurosurg 62:340–343, 1985
9)

Hakuba A, Liu S, Nishimura S: The orbitozygomatic infratemporal approach: a new surgical technique. Surg Neurol 26: 271–276, 1986
10)

da Silva SA, Yamaki VN, Solla DJF, Andrade AF, Teixeira MJ, Spetzler RF, Preul MC, Figueiredo EG. Pterional, Pretemporal, and Orbitozygomatic Approaches: Anatomic and Comparative Study. World Neurosurg. 2019 Jan;121:e398-e403. doi: 10.1016/j.wneu.2018.09.120. Epub 2018 Sep 26. PubMed PMID: 30266695.

Insular Cavernous Malformation

Insular Cavernous Malformation

Surgical management of cavernous malformation (CM) of the insula consists of total resection of the lesion and the surrounding gliosis to avoid or reduce seizures. When located in the dominant hemisphere, an awake craniotomy with intraoperative mapping reduces the risk of functional damage. The insula is covered by the operculum and has a relationship with the middle cerebral artery and its branches that run along its lateral cortical surface. Therefore high expertise is required to manage the exposure of the insula and its complex anatomy.

Insular Cavernous Malformation Classification.

https://www.neurosurgicalatlas.com/cases/insular-cavernous-malformation


A video of Burkhardt et al. demonstrated the microsurgical resection of a de novo CM adjacent to a previously treated high-grade AVM and clipping of a middle cerebral artery (MCA) aneurysm. A 70-yr-old male with history of radiosurgery for AVM presented with aphasia and confusion. Preoperative angiography showed complete occlusion of the AVM. MRI showed multiple cystic lesions suspicious for radiation-induced necrosis and CM. IRB approval and patient consent was obtained. A pterional craniotomy was performed with transsylvian exposure of the insula. The radiated feeding arteries were followed to the occluded AVM nidus. A CM was noted deep to this candelabra of the MCA vessels, which were mobilized to access and resect the CM. A small incision was made in this insular cortex underneath the malformation circumferentially freeing it of adhesions. The sclerotic AVM nidus was circumferentially dissected and removed en bloc. Thorough exploration of the resection cavity revealed no residual CM or AVM nidus. Attention was then turned to the M2-MCA bifurcation aneurysm, which was occluded with a straight clip. Postoperative imaging confirmed complete CM resection. The patient recovered from his aphasia. This case demonstrates the management of a radiation-induced de novo CM following treatment of a high-grade AVM. Radiographic follow-up for radiosurgically treated AVM is needed to rule out long-term complications. Bleeding from a de novo CM mimics bleeding from residual AVM nidus, requiring careful angiographic evaluation 1).


A video of Norat et al. illustrated the use of a trans-Sylvian, trans-sulcal approach to resect a deep insular/basal ganglia cavernous malformation in a young patient. The use of the neuronavigation is essential for success in these types of operation as this tool limits the surgeon’s footprint in eloquent brain. Unlike superficial lesions where the removal of hemosiderin stained brain is possible and often safe, resection of deep-seated lesions requires the surgeon to distinguish between hemosiderin-stained brain and residual cavernous malformation. This task is not simple, and residual cavernous malformation is the most common reason for re-bleed in patients who have undergone surgery. Resection of symptomatic cavernous malformations in deep locations can be performed safely, but outcomes are heavily influenced by proper patient selection and surgeon experience. In patients with multiple cerebral cavernous malformations, such as the one in this case, genetic testing should be performed 2).


A video of Vigo et al. demonstrated the surgical management of a large left insular CM. A 29-year-old female with multiple CM and 7 years of partial seizures and recent onset of short memory loss. Neuroimaging showed a large left insular and planum polare CM with important mass effect and hemorrhage signs. The patient consented to surgery, and an awake pretemporal craniotomy was carried out with continuous motor evoked potential monitoring. No language function was localized in the superior temporal gyrus; therefore corticectomy of the middle portion was performed to expand the operative corridor. The vessel manipulation during wide opening of the sylvian fissure increased the risk of postoperative vasospasm and blood drain into the surgical field. The CM was exposed and completely removed without functional damage. The patient recovered from surgery without complications, and no seizures occurred at 2 months’ follow-up. Postoperative imaging showed complete removal of the CM 3).

A study included patients affected by iCMs and referred to the Senior Author (FA). All cases were divided in 2 groups, according to a mainly pial growth pattern (exophytic group) or a subcortical one (endophytic group). Endophytic iCM was further subdivided in 3 subgroups, based on the insular gyri involved. According to this classification, each patient underwent a specific additional neuroimaging investigation and surgical evaluation.

Results: A total of 24 patients were included. In the surgical group, trans-sylvian (TS) approach was used in 6 patients with exophytic or Zone I endophytic iCMs. The transcortical (TC) approach with awake monitoring was used in 6 cases of Zone II endophytic vascular lesions. Both TS and trans-intraparietal sulcal (TIS) approach were used for 3 cases of Zone III endophytic iCM. At follow-up, 3 patients were fully recovered from a transient speech impairment while a permanent morbidity was observed in one case.

Conclusions: ICMs represent a single entity with peculiar clinical and surgical aspects. The proposed iCM classification focuses on anatomical and functional concerns, aiming to suggest the best pre-operative work-up and the surgical evaluation 4).

A 25-yr-old female presented with an acute-onset right homonymous hemianopsia. Neuroimaging revealed a large left insular CM, adjacent to the posterior limb of IC. After obtaining IRB approval and patient consent, a left pterional craniotomy with a wide distal Sylvian fissure split was completed. Using neuronavigation, an insular entry point was chosen for corticectomy. The CM was opened with subsequent hematoma evacuation and intracapsular resection technique. Inspection of the cavity revealed remnants anteromedially near the IC, which were removed meticulously, mobilizing the CM away from the IC. Postoperative MRI demonstrated gross total resection of the CM. The patient was discharged home on postoperative day 5 with persistent homonymous hemianopia.This case describes the use of a transsylvian-transinsular approach to access deep lesions with the shortest surgical distance and minimal cortical transgression. A wide Sylvian fissure split exposes the M2 MCA and accesses a safe insular zone, keeping the most eloquent structures deep to the lesion in the surgical corridor. This approach can safely expose vascular pathologies in the insular region without the risk of injury to overlying eloquent frontal and temporal lobes, even in the dominant hemisphere 5).


1)

Burkhardt JK, Gandhi S, Tabani H, Benet A, Lawton MT. Left Transsylvian-Transinsular Approach for Radiation-Induced Cavernous Malformation: 3-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Aug 1;17(2):E62-E63. doi: 10.1093/ons/opy357. PubMed PMID: 30418603.
2)

Norat P, Yagmurlu K, Park MS, Kalani MYS. Keyhole, Trans-Sylvian, Trans-Sulcal Resection of an Insular Cerebral Cavernous Malformation: 2-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Jul 1;17(1):E18. doi: 10.1093/ons/opy326. PubMed PMID: 30496497.
3)

Vigo V, Zanabria Ortiz R, Paganelli SL, da Costa MDS, Campos Filho JM, Chaddad-Neto F. Awake Craniotomy for Removal of Left Insular Cavernous Malformation. World Neurosurg. 2019 Feb;122:209. doi: 10.1016/j.wneu.2018.10.220. Epub 2018 Nov 9. PubMed PMID: 30415050.
4)

Fioravanti A, Elia A, Morandini A, Valtulina C, Bertuccio A. Anatomo-functional evaluation for management and surgical treatment of insular cavernous malformation: a case series. Acta Neurochir (Wien). 2022 Jan 23. doi: 10.1007/s00701-021-05089-3. Epub ahead of print. PMID: 35066681.
5)

Mascitelli J, Gandhi S, Wright E, Lawton MT. Transsylvian-Transinsular Approach for an Insular Cavernous Malformation Resection: 3-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Feb 1;16(2):50. doi: 10.1093/ons/opy155. PubMed PMID: 29905877.
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