Surgery for craniopharyngioma

Buchfelder, Michael; Schlaffer, Sven-Martin; Lin, Fei; Kleindienst, Andrea

doi:10.1007/s11102-012-0414-8

Cited by 60 publications

(68 citation statements)

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“…, without involvement of hypothalamic or optical structures), the preferred treatment of choice is an attempt at complete surgical resection with preservation of visual and hypothalamic function [27,28,29]. For unfavorably located tumors too close to or too entangled with the optic nerve and/or hypothalamic structures, controversy exists over whether complete resection should still be attempted or whether a planned limited surgical resection should be performed [2,30,31]. Many authors take a critical view of planned radical resection in these cases because of the risk of surgically induced deficits (mainly hypothalamic) and the high rate of recurrence especially in infants and small children despite apparent complete resection [32,33].…”

Section: Treatment Strategiesmentioning

confidence: 99%

Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

Daubenbüchel

Müller

2015

JCM

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Childhood-onset craniopharyngiomas are partly cystic embryonic malformations of the sellar/parasellar region. The therapy of choice in patients with favorable tumor localization is complete resection with a specific focus on maintaining optical and hypothalamic neuroendocrine functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), a limited hypothalamus-sparing surgical strategy followed by local irradiation is recommended. Involvement and/or surgical lesions of posterior hypothalamic areas cause major neuroendocrine sequelae. The overall survival rates are high (92%) but neuroendocrine disorders such as obesity and metabolic syndrome due to involvement and/or treatment-related hypothalamic lesions have major negative impact on survival and quality of life. Recurrences and progressions are frequent post-surgical events. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007). Childhood-onset craniopharyngioma should be recognized as a chronic disease requiring treatment and constant monitoring of the clinical and quality of life consequences, frequently impaired due to neuroendocrine disorders, by experienced multidisciplinary teams in order to provide optimal care of surviving patients.

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Section: Treatment Strategiesmentioning

confidence: 99%

Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

Daubenbüchel

Müller

2015

JCM

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“…Opublikowano metaanalizy dotyczące porównania skuteczności i powikłań różnych metod leczenia czaszkogardlaka oraz czynników ryzyka nawrotów choroby [11][12][13][14][15]. Zacharia obejmuje swoją analizą 429 pacjentów (dane z bazy SEER z lat 2004-2008), Buchfelder 1260 pacjentów (13 artykułów), Mortini 2087 pacjentów (26 doniesień), Elliot 2955 chorych (35 artykułów), a Yang i Sughrue 422 pacjentów.…”

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“…U 60% pacjentów wykonano totalną resekcję, a częściową u 40% [11-13, 15, 16].Wznowę po totalnej resekcji obserwowano u 21,5% pacjentów. Progresja po częściowej resekcji wzrastała aż do 70% chorych, natomiast zastosowanie radioterapii zmniejszyło częstość progresji do 30% [11,12,14,16]. Śmiertelność okołooperacyjna (do miesiąca po operacji) wynosiła 0-5,4% i była niższa dla zabiegów transfenoidalnych (2,6%) niż kraniotomii (3,1%) [16].…”

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Treatment methods of craniopharyngioma and its complications with a particular emphasis on obesity

Moszczyńska¹,

Szalecki²,

Pasternak-Pietrzak³

2017

Pediatr. Endocrinol.

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StreszczenieMimo że czaszkogardlak jest guzem histopatologicznie łagodnym, jego lokalizacja w okolicy istotnych struktur mózgu powoduje, że leczenie jest trudne i obarczone powikłaniami. Nie ma standardu leczenia czaszkogardlaka u dzieci. Toczy się dyskusja wokół korzyści i ryzyka całkowitej resekcji w stosunku do częściowej z następową radioterapią. Najczęściej stosowaną obecnie metodą radioterapii jest frakcjonowana radioterapia konformalna. Do innych metod radioterapii należą: stereotaktyczna radiochirurgia, terapia protonowa czy radioterapia wewnątrzkawitarna. Powikłania wynikające z obecności guza jak i z zastosowanego leczenia to: niedoczynność przysadki, moczówka prosta, dysfunkcje okulistyczne, neurologiczne oraz zaburzenia podwzgórzowe i neuropoznawcze. Otyłość występuje u 40-50% chorych po leczeniu czaszkogardlaka i nie ma jej skutecznego leczenia farmakologicznego. W przypadku otyłości olbrzymiej z towarzyszącymi ciężkimi powikłaniami u pacjentów po zakończeniu wzrastania należy rozważyć wskazania do zabiegu bariatrycznego. AbstractHowever craniopharyngioma is a histologically benign tumor, its localization around important brain structures makes the treatment process challenging with an increased risk of complications. There is no treatment standard for craniopharyngioma in children. Advantages and risk of total resection relative to the partial resection with subsequent radiotherapy are still discussed. Fractionated stereotactic radiotherapy is currently most used method of radiotherapy. The other methods are: stereotactic radiosurgery, proton radiotherapy, intracavitary radiation therapy. There are the following complications in patients after craniopharyngioma treatment: hypopituitarism, diabetes insipidus, visual disturbances, hypothalamic and neurocognitive dysfunctions. Obesity occurs in 40-50% of patients after craniopharyngioma treatment and there is no effective pharmacological therapy. In the case of morbid obesity with severe complications in patients after growth termination, indications to the bariatric surgery should be taken into consideration. Metody leczenia czaszkogardlaka oraz powikłania ze szczególnym uwzględnieniem otyłości Metody leczenia czaszkogardlaka A. Operacyjna. B. Radioterapia. C. Chemioterapia: wewnątrzkawitarna, ogólna A. Leczenie operacyjne Do metod operacyjnych stosowanych w leczeniu czaszkogardlaka należą: 1) całkowita resekcja guza, 2) niecałkowita resekcja guza: a. prawie totalna resekcja -pozostaje guz o powierzchni <1,5cm 2 ; b. subtotalna resekcja -zmniejszenie guza ≥90% jego objętości, pozostała powierzchnia guza wynosi ≥1,5cm 2 ; c. częściowa resekcja -zmniejszenie objętości guza <90% objętości [1], 3) aspiracja torbieli, 4) biopsja guza.Główne cele terapeutyczne to • maksymalna resekcja guza bez uszkodzenia układu podwzgórzowo-przysadkowego i skrzyżowania nerwów wzrokowych, tętnicy szyjnej i jej gałęzi • zminimalizowanie powikłań leczenia (dysfunkcje endokrynologiczne, zaburzenia okulistyczne i neurologiczne, zaburzenia łaknienia i pamięci) • poprawa jakości ...

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“…Due to their intricate localization, these tumors often affect crucial surrounding brain structures such as the pituitary, hypothalamus, and optic chiasm, resulting in endocrinological disorder and visual impairment. 8,14 Treatment is challenging as radical resection poses the risk of damaging crucial brain structures. 8,31,46 Macroscopic or microscopic tumor residues, which lead to recurrence without further intervention, are treated by postoperative radiation therapy.…”

mentioning

confidence: 99%

“…8,14 Treatment is challenging as radical resection poses the risk of damaging crucial brain structures. 8,31,46 Macroscopic or microscopic tumor residues, which lead to recurrence without further intervention, are treated by postoperative radiation therapy. 15,28 However, subtotal resection and subsequent radiotherapy still result in frequent progressions and recurrences.…”

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confidence: 99%

Drug priming enhances radiosensitivity of adamantinomatous craniopharyngioma via downregulation of survivin

Stache

Bils

Fahlbusch

et al. 2016

FOC

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OBJECTIVE In this study, the authors investigated the underlying mechanisms responsible for high tumor recurrence rates of adamantinomatous craniopharyngioma (ACP) after radiotherapy and developed new targeted treatment protocols to minimize recurrence. ACPs are characterized by the activation of the receptor tyrosine kinase epidermal growth factor receptor (EGFR), known to mediate radioresistance in various tumor entities. The impact of tyrosine kinase inhibitors (TKIs) gefitinib or CUDC-101 on radiation-induced cell death and associated regulation of survivin gene expression was evaluated. METHODS The hypothesis that activated EGFR promotes radioresistance in ACP was investigated in vitro using human primary cell cultures of ACP (n = 10). The effects of radiation (12 Gy) and combined radiochemotherapy on radiosensitivity were assessed via cell death analysis using flow cytometry. Changes in target gene expression were analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Survivin, identified in qRT-PCR to be involved in radioresistance of ACP, was manipulated by small interfering RNA (siRNA), followed by proliferation and vitality assays to further clarify its role in ACP biology. Immunohistochemically, survivin expression was assessed in patient tumors used for primary cell cultures. RESULTS In primary human ACP cultures, activation of EGFR resulted in significantly reduced cell death levels after radiotherapy. Treatment with TKIs alone and in combination with radiotherapy increased cell death response remarkably, assessed by flow cytometry. CUDC-101 was significantly more effective than gefitinib. The authors identified regulation of survivin expression after therapeutic intervention as the underlying molecular mechanism of radioresistance in ACP. EGFR activation promoting ACP cell survival and proliferation in vitro is consistent with enhanced survivin gene expression shown by qRT-PCR. TKI treatment, as well as the combination with radiotherapy, reduced survivin levels in vitro. Accordingly, ACP showed reduced cell viability and proliferation after survivin downregulation by siRNA. CONCLUSIONS These results indicate an impact of EGFR signaling on radioresistance in ACP. Inhibition of EGFR activity by means of TKI treatment acts as a radiosensitizer on ACP tumor cells, leading to increased cell death. Additionally, the results emphasize the antiapoptotic and pro-proliferative role of survivin in ACP biology and its regulation by EGFR signaling. The suppression of survivin by treatment with TKI and combined radiotherapy represents a new promising treatment strategy that will be further assessed in in vivo models of ACP.

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Surgery for craniopharyngioma

Cited by 60 publications

References 51 publications

Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

Treatment methods of craniopharyngioma and its complications with a particular emphasis on obesity

Drug priming enhances radiosensitivity of adamantinomatous craniopharyngioma via downregulation of survivin

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