Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships

Zada, Gabriel; Lin, Ning; Ojerholm, Eric; Ramkissoon, Shakti; Laws, Edward R.

doi:10.3171/2010.2.focus09318

Cited by 232 publications

(199 citation statements)

References 98 publications

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“…The low recovery rates are thought to be associated with an inflammatory process caused by the cyst contents, which may result in irreversible damage to the adjacent hypophysis. 7,8,12,25,33,43,54 Our results show that preoperative headache and visual dysfunction were resolved in 85% and 95% of patients, and both rates compare favorably with the findings of other studies. However, we achieved better resolution in the patients with hypopituitarism and DI than other series of similar sample size (55% and 65%, respectively).…”

Section: Outcome Analysissupporting

confidence: 80%

Individualized surgical strategies for Rathke cleft cyst based on cyst location

Fan¹,

Peng²,

Qi³

et al. 2013

JNS

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R athke cleft cysts (RCCs) are regarded as benign cystic lesions derived from a remnant of the Rathke pouch. They are common findings at autopsy, with a reported incidence of 5% to 33%, and account for 6%-10% of sellar lesions. 3,9,17,32,39,46,49 Although mostly asymptomatic, some RCCs can become sufficiently large to cause compressive effect on surrounding structures, resulting in neurological and endocrine disturbances. As a result, surgical intervention is recommended for symptomatic or at-risk patients.Traditionally, the 2 most common surgical approaches are the endonasal transsphenoidal route and the standard craniotomy. The endonasal approach is typically used for sellar lesions, whereas the craniotomy is indicated for suprasellar lesions. 2,11,38,40 Owing to its reduced invasiveness Department of Neurosurgery, Nanfang Hospital, Southern Medical University, Guangzhou, ChinaObject. An assessment regarding both surgical approaches and the extent of resection for Rathke cleft cysts (RCCs) based on their locations has not been reported. The aim of this study was to report the results of a large series of surgically treated patients with RCCs and to evaluate the feasibility of individualized surgical strategies for different RCCs.Methods. We retrospectively reviewed 87 cases involving patients with RCCs (16 intrasellar, 50 intra-and suprasellar, and 21 purely suprasellar lesions). Forty-nine patients were treated via a transsphenoidal (TS) approach, and 38 were treated via a transcranial (TC) approach (traditional craniotomy in 21 cases and supraorbital keyhole craniotomy in 17). The extent of resection was classified as gross-total resection (GTR) or subtotal resection (STR) of the cyst wall. Patients were thus divided into 3 groups according to the approach selected and the extent of resection: TS/STR (n = 49), TC/STR (n = 23), and TC/GTR (n = 15).Results. Preoperative headaches, visual dysfunction, hypopituitarism, and diabetes insipidus (DI) resolved in 85%, 95%, 55%, and 65% of patients, respectively. These rates did not differ significantly among the 3 groups. Overall, complications occurred in 8% of patients in TS/STR group, 9% in TC/STR group, and 47% in TC/GTR group, respectively (p = 0.002). Cerebrospinal fluid (CSF) leakage (3%), new hypopituitarism (9%), and DI (6%) were observed after surgery. All CSF leaks occurred in the endonasal group, while the TC/GTR group showed a higher rate of postoperative hypopituitarism (p = 0.7 and p < 0.001, respectively). It should be particularly noted that preoperative hypopituitarism and DI returned to normal, respectively, in 100% and 83% of patients who underwent supraorbital surgery, and with the exception of 1 patient who had transient postoperative DI, there were no complications in patients treated with supraorbital surgery. Kaplan-Meier 3-year recurrence-free rates were 84%, 87%, and 86% in the TS/STR, TC/STR, and TC/GTR groups, respectively (p = 0.9).Conclusions. It is reasonable to adopt individualized surgical strategies for RCCs based on cyst location....

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Section: Outcome Analysissupporting

confidence: 80%

Individualized surgical strategies for Rathke cleft cyst based on cyst location

Fan¹,

Peng²,

Qi³

et al. 2013

JNS

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“…34,38,40 The histopathological similarities between ACP and odontogenesis and frequently cystic odontogenic tumors such as ameloblastoma and calcifying odontogenic cyst has also been recognized, but the molecular biology of these similarities and links remains relatively underexplored. 59 …”

Section: Molecular Pathology Of Tumor Cystsmentioning

confidence: 99%

“…Cyst fluid has a so-called "motor oil" appearance and is rich in cholesterol, with variable necrotic and inflammatory debris. 59 Several studies have begun to investigate the molecular biology underlying cyst formation, as existing intracystic therapies have been largely empirical in their mechanism of action. 7 It has long been recognized that leakage of cystic fluid can lead to local inflammation, and this has been modeled by injection of cystic fluid into the brain of rats, which induces an inflammatory response and an increase in GFAP expression.…”

Section: Molecular Pathology Of Tumor Cystsmentioning

confidence: 99%

Molecular pathology of adamantinomatous craniopharyngioma: review and opportunities for practice

Apps¹,

Martinez-Barbera²

2016

FOC

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Since the first identification of CTNNB1 mutations in adamantinomatous craniopharyngioma (ACP), much has been learned about the molecular pathways and processes that are disrupted in ACP pathogenesis. To date this understanding has not translated into tangible patient benefit. The recent development of novel techniques and a range of preclinical models now provides an opportunity to begin to support treatment decisions and develop new therapeutics based on molecular pathology. In this review the authors summarize many of the key findings and pathways implicated in ACP pathogenesis and discuss the challenges that need to be tackled to translate these basic science findings for the benefit of patients.

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“…While a minority display papillary-squamous histology, which is associated with the pathognomonic BRAF V600E mutation and which has been reported to respond to targeted anti-BRAF and anti-MEK1/2 inhibition, most cases are of adamantinomatous histology, which does not have a known therapeutically amenable driver. 1,2,21,39 The treatment of craniopharyngioma is primarily centered on excision, with gross-total resection (GTR) the gold standard treatment, although recent evidence supports comparable control rates with subtotal resection and adjuvant radiotherapy. 35 However, cyst enlargement after radiotherapy may cause symptomatic progression requiring treatment.…”

mentioning

confidence: 99%

Endoscopic endonasal versus open transcranial resection of craniopharyngiomas: a case-matched single-institution analysis

Moussazadeh

Prabhu

Bander

et al. 2016

FOC

104

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OBJECTIVE The authors compared clinical and radiological outcomes after resection of midline craniopharyngiomas via an endoscopic endonasal approach (EEA) versus an open transcranial approach (TCA) at a single institution in a series in which the tumors were selected to be equally amenable to gross-total resection (GTR) with either approach. METHODS A single-institution retrospective review of previously untreated adult midline craniopharyngiomas was performed. Lesions were evaluated by 4 neurosurgeons blinded to the actual approach used to identify cases that were equally amenable to GTR using either an EEA or TCA. Radiological and clinical outcome data were assessed. RESULTS Twenty-six cases amenable to either approach were identified, 21 EEA and 5 TCA. Cases involving tumors that were resected via a TCA had a trend toward larger diameter (p = 0.10) but were otherwise equivalent in preoperative clinical and radiological characteristics. GTR was achieved in a greater proportion of cases removed with an EEA than a TCA (90% vs 40%, respectively; p = 0.009). Endoscopic resection was associated with superior visual restoration (63% vs 0%; p < 0.05), a decreased incidence of recurrence (p < 0.001), lower increase in FLAIR signal postoperatively (−0.16 ± 4.6 cm3 vs 14.4 ± 14.0 cm3; p < 0.001), and fewer complications (20% vs 80% of patients; p < 0.001). Significantly more TCA patients suffered postoperative cognitive loss (80% vs 0; p < 0.0001). CONCLUSIONS An EEA is a safe and effective approach to suprasellar craniopharyngiomas amenable to GTR. For this select group of cases, the EEA may provide higher rates of GTR and visual improvement with fewer complications compared with a TCA.

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Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships

Cited by 232 publications

References 98 publications

Individualized surgical strategies for Rathke cleft cyst based on cyst location

Individualized surgical strategies for Rathke cleft cyst based on cyst location

Molecular pathology of adamantinomatous craniopharyngioma: review and opportunities for practice

Endoscopic endonasal versus open transcranial resection of craniopharyngiomas: a case-matched single-institution analysis

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