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.

Invasive pituitary adenoma

Invasive pituitary adenoma

Invasive pituitary adenomas are benign pituitary tumors that infiltrate the dura matercranial bone, or sphenoid sinus. Gross invasion at the time of operation is observed in up to 35 % of pituitary adenoma1) 2) 3).

Clinically nonfunctioning pituitary adenoma (NFPA) is a very common type of intracranial tumor, which can be locally invasive and can have a high recurrence rate.

Atypical or aggressive pituitary adenomas are tumors that rapidly increase in size and may invade into the suprasellar or parasellar regions. They are characterized by a Ki-67 nuclear labeling index greater than 10 %.

Invasive pituitary adenoma molecular markers.

They can be presented as Non-pulsatile exophthalmos.

Infrequently they produce cerebrospinal fluid rhinorrhea.

Invasive pituitary adenomas and pituitary carcinomas are clinically indistinguishable from pituitary adenoma until identification of metastases.

Invasive pituitary adenoma treatment.

Aggressive pituitary adenomas (APAs) are pituitary tumors that are refractory to standard treatments and carry a poor prognosis.

A 57-year-old man presented with visual deterioration and bitemporal hemianopsiaMRI of the brain demonstrated a sellamass suspected to be pituitary macroadenoma with a displacement of the stalk and optic nerve impingement. The patient underwent stereotactic endoscopic transsphenoidal resection of the mass. Postoperative MRI demonstrated gross total resectionPathology revealed a sparsely granulated corticotroph adenoma with malignant transformationImmunohistochemistry showed a loss of expression of MLH1 and PMS2 in the tumor cells. Proton therapy was recommended given an elevated Ki67 index and p53 positivity. Before radiotherapy, there was no radiographic evidence of residual tumor. Temozolomide therapy was initiated after surveillance MRI showed recurrence at 16 months postoperatively. However, MRI demonstrated marked progression after 3 cycles. Next-generation sequencing using the MSK-IMPACT platform identified somatic mutations in MLH1 Y548lfs*9 and TP53 R337C. Immunotherapy with ipilimumab/nivolumab was initiated, and MRI demonstrated no residual tumor burden 34 months postoperatively.

APA is a tumor with frequent recurrence and a short median expected length of survival. Shah et al. demonstrated the utility of immunotherapy in a single case report of APA, with complete resolution of recurrent APA and improved survival compared with a life expectancy 4).


1)

Oruçkaptan HH, Senmevsim O, Ozcan OE, Ozgen T. Pituitary adenomas: results of 684 surgically treated patients and review of the literature. Surg Neurol. 2000;53:211–219.
2)

Scheithauer BW, Kovacs KT, Laws ER, Jr, Randall RV. Pathology of invasive pituitary tumors with special reference to functional classification. J Neurosurg. 1986;65:733–744.
3)

Selman WR, Laws ER, Scheithauer BW, et al. The occurrence of dural invasion in pituitary adenomas. J Neurosurg. 1986;64:402–407.
4)

Shah S, Manzoor S, Rothman Y, Hagen M, Pater L, Golnik K, Mahammedi A, Lin AL, Bhabhra R, Forbes JA, Sengupta S. Complete Response of a Patient With a Mismatch Repair Deficient Aggressive Pituitary Adenoma to Immune Checkpoint Inhibitor Therapy: A Case Report. Neurosurgery. 2022 May 13. doi: 10.1227/neu.0000000000002024. Epub ahead of print. PMID: 35544035.

Microvascular decompression for trigeminal neuralgia outcome

Microvascular decompression for trigeminal neuralgia outcome

Microvascular decompression (MVD) is the most effective long-term surgical treatment for trigeminal neuralgia (TN) patients. The risk factors for poor pain control following MVD surgery are not fully understood.


A significant proportion of patients with significant neurovascular compression fail to achieve long-term pain relief after technically successful surgery. Neuroimaging using magnetic resonance imaging (MRI) provides a non-invasive method to generate objective biomarkers of eventual response to TN surgery 1).


Younger patients with TN had worse long-term pain outcomes following MVD. Additional factors associated with postoperative recurrence included poor preoperative pain control (BNI score > IV) and multivessel compression. Furthermore, SCA combined with PV was confirmed to be associated with a worse outcome 2).


Not all patients with TN manifest unequivocal neurovascular compression (NVC). Furthermore, over time patients with an initially successful MVD manifest a relentless rate of TN recurrence.

It does not achieve 100 % cure rate. Re-exploration of the posterior fossa may carry increased risk over first-time MVD and is not always successful, so other treatments are needed.


Age itself does not seem to represent a major contraindication of microvascular decompression for typical trigeminal neuralgia 3).

Patients 60 yr of age and older have significantly better long-term pain outcomes following MVD than younger patients 4).


1)

Wang Z, Zhao Z, Song Z, Wang Y, Zhao Z. The application of magnetic resonance imaging (MRI) for the prediction of surgical outcomes in trigeminal neuralgia. Postgrad Med. 2022 May 3:1-7. doi: 10.1080/00325481.2022.2067612. Epub ahead of print. PMID: 35503235.
2)

Shi J, Qian Y, Han W, Dong B, Mao Y, Cao J, Guan W, Zhou Q. Risk factors for outcomes following microvascular decompression for trigeminal neuralgia. World Neurosurg. 2020 Jan 17. pii: S1878-8750(20)30100-5. doi: 10.1016/j.wneu.2020.01.082. [Epub ahead of print] PubMed PMID: 31958591.
3)

Mastronardi L, Caputi F, Rinaldi A, Cacciotti G, Roperto R, Scavo CG, Stati G, Sufianov A. Typical Trigeminal Neuralgia: Comparison of Results between Patients Older and Younger than 65 Operated on with Microvascular Decompression by Retrosigmoid Approach. J Neurol Surg A Cent Eur Neurosurg. 2019 Aug 29. doi: 10.1055/s-0039-1693126. [Epub ahead of print] PubMed PMID: 31466107.
4)

Bick SK, Huie D, Sneh G, Eskandar EN. Older Patients Have Better Pain Outcomes Following Microvascular Decompression for Trigeminal Neuralgia. Neurosurgery. 2019 Jan 1;84(1):116-122. doi: 10.1093/neuros/nyy011. PubMed PMID: 29562363.
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