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1.
Endocr J ; 71(3): 285-293, 2024 Mar 28.
Artículo en Inglés | MEDLINE | ID: mdl-38281757

RESUMEN

Pituitary xanthogranulomatomas (XG) are a rare pathological entity caused by accumulation of lipid laden macrophages and reactive granuloma formation usually triggered by cystic fluid leakage or hemorrhage. Our aim was to compare clinical characteristics and presenting features of patients with secondary etiology of XG and those with no identifiable founding lesion (primary -"pure" XG) in order to gain new insights into this rare pituitary pathology. In a retrospective review of 714 patients operated for sellar masses, at tertiary center, we identified 16 (2.24%) with histologically confirmed diagnosis of pituitary XG over the period of 7 years (2015-2021). Patients were further analyzed according to XG etiology: "pure"- XG (n = 8) with no identifiable founding lesion were compared to those with histological elements of pituitary tumor or cyst - secondary XG (n = 8). We identified 16 patients (11 male), mean age 44.8 ± 22.3 years, diagnosed with pituitary XG. Secondary forms were associated with Ratke's cleft cyst (RCC, n = 2) and pituitary adenoma (PA, n = 6). The most common presenting features in both groups were hypopituitarism (75%), headache (68.5%) and visual disturbances (37.5%). Predominance of male sex was noted (males 68.75%, females 31.25%), especially in patients with primary forms. Patients with primary pituitary XG were all males (p = 0.0256) and more frequently affected by panhypopituitarism (87.5% vs. 25%, p = 0.0406) compared to patients with secondary causes. Hyperprolactinemia was noted in pituitary tumor group with secondary etiology only (p = 0.0769). Majority of lesions were solid on magnetic resonance imaging - MRI (81.25%). Distinct clinical phenotype was observed dependent on the etiology of XG.


Asunto(s)
Quistes del Sistema Nervioso Central , Quistes , Enfermedades de la Hipófisis , Neoplasias Hipofisarias , Xantomatosis , Femenino , Humanos , Masculino , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/epidemiología , Enfermedades de la Hipófisis/epidemiología , Hipófisis/diagnóstico por imagen , Hipófisis/patología , Imagen por Resonancia Magnética , Quistes del Sistema Nervioso Central/complicaciones , Quistes/patología , Granuloma/complicaciones , Granuloma/patología , Xantomatosis/epidemiología , Xantomatosis/patología
2.
Neurosurg Rev ; 47(1): 253, 2024 Jun 03.
Artículo en Inglés | MEDLINE | ID: mdl-38829433

RESUMEN

PURPOSE: The study intends to clarify the optimal endoscopic endonasal surgical strategy for symptomatic Rathke's cleft cysts (RCCs). METHODS: We retrospectively analyzed patients with RCCs that underwent EEA surgery. The strategy for surgical and reconstruction method selection was presented. Patients were split into groups of fenestration open or closed. Pre- and postoperative symptoms, imaging, ophthalmologic, and endocrinologic exams were reviewed. The incidence of complications and the recurrence rates were determined. RESULTS: The 75 individuals were all received primary operations. The fenestration closed group contained 32 cases, while the fenestration open group contained 43 cases. The median follow-up period was 39 months. The three primary complaints were headache (n = 51, 68.00%), vision impairment (n = 45, 60.00%), and pituitary dysfunction (n = 16, 21.33%). Of the 51 patients with preoperative headaches, 48 (94.12%) reported improvement in their symptoms following surgery. Twenty-three out of 45 patients (51.11%) experienced an improvement in visual impairment. Pituitary dysfunction was found improved in 14 out of 16 individuals (87.50%). There was no discernible difference in the rate of symptom alleviation between both groups. There were three patients (3/75, 4.00%) had cyst reaccumulation. One of them (1/75, 1.33%), which needed reoperation, was healed using pterional approach. In term of complications, cerebral infections occurred in two patients (2/75, 2.67%). Both of them recovered after antibiotic treatment. No postoperative cerebrospinal fluid rhinorrhea occurred. One patient (1/75, 1.33%) in the open group experienced epistaxis. There was no persistent hypopituitarism or diabetes insipidus (DI). Analysis of headache related factors showed that the presence of wax like nodules was related to it. CONCLUSION: RCC was successfully treated with endoscopic endonasal surgery with few problems when the fenestration was kept as open as feasible. Preoperative identification of T2WI hypointense nodules may be a potential reference factor for surgical indication.


Asunto(s)
Quistes del Sistema Nervioso Central , Humanos , Masculino , Quistes del Sistema Nervioso Central/cirugía , Quistes del Sistema Nervioso Central/complicaciones , Femenino , Adulto , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven , Adolescente , Neuroendoscopía/métodos , Anciano , Complicaciones Posoperatorias/epidemiología , Neoplasias Hipofisarias/cirugía , Cefalea/etiología , Procedimientos Neuroquirúrgicos/métodos
3.
Pituitary ; 26(4): 393-401, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37227614

RESUMEN

PURPOSE: Large Rathke's cleft cysts (LRCCs) and cystic craniopharyngiomas (CCPs) arise from the same embryological origin and may have similar MR presentations. However, the two tumors have different management strategies and outcomes. This study was designed to evaluate the clinical and imaging findings of LRCCs and CCPs, aiming to evaluate their pretreatment diagnosis and outcomes. METHODS: We retrospectively enrolled 20 patients with LRCCs and 25 patients with CCPs. Both tumors had a maximal diameter of more than 20 mm. We evaluated the patients' clinical and MR imaging findings, including symptoms, management strategies, outcomes, anatomic growth patterns and signal changes. RESULTS: The age of onset for LRCCs versus CCPs was 49.0 ± 16.8 versus 34.2 ± 22.2 years (p = .022); the following outcomes were observed for LRCCs versus CCPs: (1) postoperative diabetes insipidus: 6/20 (30%) versus 17/25 (68%) (p = .006); and (2) posttreatment recurrence: 2/20 (10%) versus 10/25 (40%) (p = .025). The following MR findings were observed for LRCCs versus CCPs: (1) solid component: 7/20 (35%) versus 21/25 (84%) (p = .001); (2) thick cyst wall: 2/20 (10%) versus 12/25 (48%) (p = .009); (3) intracystic septation: 1/20 (5%) versus 8/25 (32%) (p = .030); (4) snowman shape: 18/20 (90%) versus 1/25 (4%) (p < .001); (5) off-midline extension: 0/0 (0%) versus 10/25 (40%) (p = .001); and (6) oblique angle of the sagittal long axis of the tumor: 89.9° versus 107.1° (p = .001). CONCLUSIONS: LRCCs can be differentiated from CCPs based on their clinical and imaging findings, especially their specific anatomical growth patterns. We suggest using the pretreatment diagnosis to select the appropriate surgical approach and thus improve the clinical outcome.


Asunto(s)
Quistes del Sistema Nervioso Central , Craneofaringioma , Neoplasias Hipofisarias , Humanos , Niño , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Craneofaringioma/patología , Neoplasias Hipofisarias/patología , Estudios Retrospectivos , Quistes del Sistema Nervioso Central/patología , Imagen por Resonancia Magnética
4.
Pituitary ; 26(6): 696-707, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37878234

RESUMEN

OBJECTIVE: This paper assesses the clinical and imaging characteristics, histopathological findings, and treatment outcomes of patients with Rathke's cleft cyst (RCC), as well as identifies potential risk factors for preoperative visual and pituitary dysfunction, intraoperative cerebrospinal fluid (CSF) leak, and recurrence. Through analyzing these factors, the study aims to contribute to the current understanding of the management of RCCs and identify opportunities for improving patient outcomes. METHODS: We performed a retrospective analysis of 45 RCC patients between ages 18-80 treated by Endoscopic Endonasal Approach (EEA) and cyst marsupialization between 2010 and 2022 at a single institution. RESULTS: The median patient age was 34, and 73% were female. The mean follow-up was 70 ± 43 months. Preoperative visual impairment correlated with cyst diameter (OR = 1.41, 95% CI = 1.07 to 1.85, p-value = 0.01) and older age (OR = 1.06, 95% CI = 1.01 to 1.11, p-value = 0.02). Intraoperative CSF leaks were 11 times more likely for cysts ≥ 2 cm (OR = 11.3, 95% CI = 1.25 to 97.37, p-value = 0.03), with the odds of leakage doubling for every 0.1 cm increase in cyst size (OR = 1.41, 95% CI = 1.08 to 1.84, p-value = 0.01). Preoperative RCC appearing hypointense on T1 images demonstrated significantly higher CSF leak rates than hyperintense lesions (OR = 122.88, 95% CI = 1.5 to 10077.54, p-value = 0.03). Preoperative pituitary hypofunction was significantly more likely in patients with the presence of inflammation on histopathology (OR = 20.53, 95% CI = 2.20 to 191.45, p-value = 0.008 ) and T2 hyperintensity on magnetic resonance imaging (MRI) sequences (OR = 23.2, 95% CI = 2.56 to 211.02, p-value = 0.005). Notably, except for the hyperprolactinemia, no postoperative improvement was observed in pituitary function. CONCLUSION: Carefully considering risk factors, surgeons can appropriately counsel patients and deliver expectations for complications and long-term results. In contrast to preoperative visual impairment, preoperative pituitary dysfunction was found to have the least improvement post-surgery. It was the most significant permanent complication, with our data indicating the link to the cyst signal intensity on T2 MR and inflammation on histopathology. Earlier surgical intervention might improve the preservation of pituitary function.


Asunto(s)
Carcinoma de Células Renales , Quistes del Sistema Nervioso Central , Quistes , Enfermedades de la Hipófisis , Femenino , Humanos , Masculino , Quistes del Sistema Nervioso Central/cirugía , Quistes del Sistema Nervioso Central/patología , Quistes/cirugía , Quistes/complicaciones , Inflamación/complicaciones , Estudios Retrospectivos , Factores de Riesgo , Trastornos de la Visión/etiología , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años
5.
Endocr Pract ; 29(1): 60-68, 2023 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-36270609

RESUMEN

Pituitary Incidentalomas (PI) are pituitary lesions serendipitously identified on imaging. PIs have become common in clinical practice because of increased use of imaging and radiologic advances. The most frequently incidentally detected lesions in adults are pituitary adenomas, followed by cystic lesions, and rarely other types of tumors and infiltrative and inflammatory disorders. Biochemical screening for hyperprolactinemia and acromegaly is needed in all patients with PI, whereas testing for hyposecretion is recommended for lesions larger than 6.0 mm. Most PIs are small nonfunctioning adenomas or cysts, which can be conservatively managed. For larger lesions, a multidisciplinary approach including endocrinology, neurosurgery, and neuro-ophthalmology is required. For incidentally detected lactotroph, somatotroph, and corticotroph adenomas, disease-specific management guidelines apply. Prospective studies are needed to enhance our understanding of the long-term course and response to treatment.


Asunto(s)
Adenoma Hipofisario Secretor de ACTH , Acromegalia , Adenoma , Neoplasias Hipofisarias , Adulto , Humanos , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/terapia , Adenoma/patología , Hipófisis/patología
6.
Acta Neurochir (Wien) ; 165(8): 2277-2282, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37046123

RESUMEN

BACKGROUND: The most common presenting symptom in patients with both small and large Rathke cleft cysts (RCC) is headache (H/A). It is well established that patients with large RCC can have significant symptomatic improvement after cyst drainage. However, patients with small RCC (≤ 1 cm) are rarely operated on, even if they present with debilitating H/A. It is not well understood whether resection of these smaller RCCs can lead to durable H/A resolution. METHODS: A retrospective search of our institutional database for sub-centimeter RCCs presenting with intractable H/A and treated with an endoscopic endonasal approach was carried out. A detailed H/A questionnaire as well as patient chart review was conducted to assess the long-term outcome of these patients after surgical intervention. RESULTS: Ten consecutive patients with 11 endonasal surgeries met inclusion criteria. Eight responded to the questionnaire. The median cyst diameter was 6 mm (IQR 3-9). Median preoperative H/A duration was 12 months (range 2 months-15 years). H/As occurred on average for 20 days per month and all required analgesics for symptomatic control for more than 15 of these 20 days. Half of the patients also had to miss work because of H/A. Average preoperative H/A intensity was 8.7 (scale 0-10) compared with postoperative scores of 2.9 at one month, 1.6 at 3 months, and 0.9 at 1 year. There were no permanent endocrinological or other surgical complications. After a median follow-up of 2 years, one patient had radiographic and symptomatic recurrence which resolved after re-operation. CONCLUSIONS: Endoscopic fenestration of sub-centimeter RCCs provides a safe and durable treatment for patients with intractable H/A.


Asunto(s)
Carcinoma de Células Renales , Quistes del Sistema Nervioso Central , Quistes , Neoplasias Renales , Humanos , Estudios Retrospectivos , Cefalea/etiología , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Quistes del Sistema Nervioso Central/cirugía , Quistes del Sistema Nervioso Central/complicaciones , Quistes/diagnóstico por imagen , Quistes/cirugía , Quistes/complicaciones , Resultado del Tratamiento
7.
Acta Neurochir (Wien) ; 165(8): 2267-2276, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37160776

RESUMEN

BACKGROUND: Both intrasuprasellar and suprasellar Rathke cleft cysts (RCCs) have suprasellar components, and we aimed to explore their clinical features and surgical outcomes. METHOD: Patients with surgically treated intrasuprasellar or suprasellar RCCs were retrospectively analyzed. All patients with intrasuprasellar RCCs were treated with the standard endoscopic endonasal approach (EEA, group I); the patients with suprasellar RCCs received the extended EEA (group II) or supraorbital keyhole approach (SKA, group III) according to the relevant indications. A surgical strategy of maximal safe resection aiming to protect neuroendocrine function was adopted. In addition, patients (distinguished from the above 3 groups) who had aggressive resection of suprasellar RCC were also enrolled for comparison of different surgical strategies. RESULTS: A total of 157 patients were eligible, including 121 patients with intrasuprasellar RCCs in group I, 19 patients with suprasellar RCCs in group II, and 17 patients with suprasellar RCCs in group III. Preoperatively, the patients with suprasellar RCC (groups II and III) more commonly presented with visual dysfunction, diabetes insipidus (DI), and hyperprolactinemia than the patients with intrasuprasellar RCCs (all p<0.05). A higher incidence of hypopituitarism and a larger diameter were observed for intrasuprasellar RCCs (both p<0.05). Postoperatively, group II had a higher rate of new-onset DI, hyponatremia, and recurrence than group I (all p<0.025) and similar outcomes to group III. For suprasellar RCCs, comparison of the maximal safe resection vs. aggressive resection (supplementary patients: 14 with extended EEA, 12 with SKA) showed similar improvement and recurrence, with higher rates of DI and hyponatremia with the latter strategy (all p<0.05). CONCLUSIONS: Suprasellar RCC is associated with more complicated preoperative presentations, intricate postoperative complications, and frequent recurrence compared with intrasuprasellar RCC. Under rational indications, both extended EEA and SKA achieve satisfactory outcomes. The strategy of maximal safe resection is recommended for greatest functional preservation.


Asunto(s)
Carcinoma de Células Renales , Quistes del Sistema Nervioso Central , Diabetes Insípida , Hiponatremia , Neoplasias Renales , Neoplasias Hipofisarias , Humanos , Estudios Retrospectivos , Diabetes Insípida/complicaciones , Quistes del Sistema Nervioso Central/cirugía , Quistes del Sistema Nervioso Central/complicaciones , Resultado del Tratamiento , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/complicaciones
8.
Clin Endocrinol (Oxf) ; 96(1): 54-61, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34724249

RESUMEN

OBJECTIVE: Rathke's cleft cysts are benign, embryological remnants in the pituitary gland. The majority of them are small and asymptomatic but a few may become large, and cause mass effects, pituitary hormone deficiencies and visual impairment. Recommendations for the follow-up of Rathke's cleft cysts vary since data on the natural history are sparse. PATIENTS AND DESIGN: Data at diagnosis and at 1, 5 and 10 years for patients with a Rathke's cleft cyst (434 at diagnosis, 317 females) were retrieved from the Swedish Pituitary Registry. Cysts ≤3 mm in diameter were excluded from the study. MEASUREMENTS: Data included demographics, cyst size, pituitary function, visual defects and surgery. RESULTS: The mean age at diagnosis was 45 years. In patients with cysts <10 mm in diameter (n = 204) 2.9% had pituitary hormone deficiencies and 2% had visual field impairments. Cyst size did not progress during the 5 years. Cysts with a diameter of ≥10 mm that were not operated (n = 174) decreased in size over the years (p < .01). Pituitary hormone deficiencies and visual impairments were more frequent (18% and 5.7%, respectively) but were stable over time. Transphenoidal surgery was performed in 56 patients of whom 51 underwent surgery before the 1-year follow-up. The mean cyst diameter at diagnosis was 18 mm (range: 9─30 mm), 36% had pituitary hormone deficiency, 45% had visual field defects and 20% had impaired visual acuity. One year after surgery 60% had no cyst remnants, 50% had a pituitary deficiency, 26% had visual field defects and 12% had impaired visual acuity. No major changes were observed after 5 years. Twelve of the operated patients had a follow-up at 10 years, in eight the cyst remnants or recurrences increased in size over time (p < .05). CONCLUSIONS: Rathke's cleft cysts with a size less than 10 mm rarely grow and our results indicate that radiological follow-up can be restricted to 5 years. In contrast, progression of postoperative remnants or recurrent cysts is more likely and require long-term follow-up.


Asunto(s)
Quistes del Sistema Nervioso Central , Neoplasias Hipofisarias , Quistes del Sistema Nervioso Central/cirugía , Femenino , Humanos , Recurrencia Local de Neoplasia , Hipófisis/cirugía , Neoplasias Hipofisarias/cirugía , Sistema de Registros , Suecia , Resultado del Tratamiento
9.
Neuroradiology ; 64(9): 1755-1761, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35357532

RESUMEN

PURPOSE: T2 hypointense signal at the posterior edge of the adenohypophysis (T2HSPA) on magnetic resonance imaging (MRI) is incidentally encountered. We aimed to investigate the prevalence and morphology of T2HSPA and their relationship to age. METHODS: A total of 212 cases between 3 and 88 years old were examined. Sagittal T2-weighted image (T2WI) was evaluated for the presence of T2HSPA, which classified by its morphology into two types (belt-like or nodal). The Wilcoxon rank sum test and chi-square test were used to evaluate the differences between the groups. The T2HSPA was extracted by ImageJ software and measured as a cross-sectional area (CSA) quantitatively by threshold setting. We examined the relationship between CSA of T2HSPA and age, and Spearman's correlation coefficients were used for statistical analysis. RESULTS: Of the 212 cases, 80 (37.7%) were identified with T2HSPA. The groups with T2HSPA were significantly younger than the groups without it (p = .01). Groups with belt-like T2HSPA were significantly younger than the groups with nodal T2HSPA (p = .01). There was a weak negative correlation between CSA of T2HSPA and age (p = .02). CONCLUSION: T2HSPAs were incidentally detected in 37.7% of all cases, tended to be more common in younger cases, and their morphology was related to age. They seem to have little clinical significance as they tend to decrease in size with age.


Asunto(s)
Quistes del Sistema Nervioso Central , Adenohipófisis , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Quistes del Sistema Nervioso Central/patología , Niño , Preescolar , Humanos , Imagen por Resonancia Magnética/métodos , Persona de Mediana Edad , Adenohipófisis/patología , Prevalencia , Adulto Joven
10.
Pituitary ; 25(4): 684-686, 2022 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-35781174

RESUMEN

Rathke cleft cysts (RCC) arise from the remnants of Rathke's pouch, a structure that is midline in the pituitary. Therefore, an off-midline location on imaging is a finding that is often used to distinguish Rathke cleft cysts from pituitary adenomas, with RCCs being more commonly in the midline than in a purely lateral position. Given the rarity of these laterally oriented RCCs, the incidence and behavior of RCCs that are purely lateral have not been described in current literature. Retrospective investigation was performed through review of patient records of 122 patients who underwent surgical resection for RCCs. RCCs were classified as purely lateral or midline. Lateral RCCs were found have significantly higher rates of recurrence relative to RCCs in the midline group (p = .04). Although limited by statistical power due to the low amount of lateral RCCs (n = 13/122), our findings suggest that there may be an association between lateral RCC location and cyst recurrence.The orcid 0000-0002-2949-227 of author (Manish K. Aghi) is changed to 0000-0002-2949-2227. Kindly check and confirm.The correct orcid of Manish K. Aghi is 0000-0002-2949-2227.


Asunto(s)
Carcinoma de Células Renales , Quistes del Sistema Nervioso Central , Neoplasias Renales , Neoplasias Hipofisarias , Quistes del Sistema Nervioso Central/complicaciones , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Quistes del Sistema Nervioso Central/cirugía , Humanos , Neoplasias Renales/complicaciones , Imagen por Resonancia Magnética/métodos , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/cirugía , Estudios Retrospectivos
11.
Pituitary ; 25(2): 258-266, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34807360

RESUMEN

OBJECTIVES: The primary objective was to investigate the clinical presentation, hormonal dysfunction, imaging characteristics and natural history of RCCs that were managed conservatively. Secondary objective was to identify factors associated with cyst progression. METHODS: A retrospective review of patients with the clinical diagnosis of RCC-identified from word search from radiology reports that were followed up from January 1999 to March 2019 was performed. The demographics, clinical data, radiological features and outcomes were reviewed and analyzed. RESULTS: 105 patients were identified with a median follow up of 6 years. 68 patients (64.8%) were managed conservatively from diagnosis till last follow up while 37 patients (35.2%) underwent surgery, with 26 operated at time of diagnosis and 11 operated upon monitoring. For patients managed conservatively from diagnosis till last follow up, incidental finding was the most common presentation. 19.1% had either one or more axes of hormonal dysfunction, with hypogonadism and hypocortisolemia being the commonest ones. Imaging features were variable. 66.2% of patients had T2W hyperintensity on MRI. Pathognomonic feature of intracystic nodule was present in only 14.7% of patients. Among the 79 patients with repeated MRI imaging (68 from conservative group and 11 from surgical group), 32.9% of patients developed cyst progression while 67.1% had either static disease or regression in size of RCC. Median time to progression of cyst was 14 months. Longer median follow up duration and presence of pituitary stalk displacement at presentation were associated with cyst progression. Only one patient developed new endocrine dysfunction. CONCLUSION: 2/3 of the RCCs had static disease or even regression in the size of the cyst. They rarely gave rise to additional endocrine dysfunction by adopting observant approach. Cyst progression was demonstrated in 1/3 of patients. Conservative treatment remained a reasonable treatment for patients without significant symptoms.


Asunto(s)
Quistes del Sistema Nervioso Central , Neoplasias Hipofisarias , Quistes del Sistema Nervioso Central/complicaciones , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Quistes del Sistema Nervioso Central/terapia , Humanos , Imagen por Resonancia Magnética , Hipófisis , Neoplasias Hipofisarias/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
12.
Am J Otolaryngol ; 43(5): 103519, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35690516

RESUMEN

Rathke's cleft cysts (RCCs) are sellar or suprasellar cystic lesions arising from the remnants of the embryological Rathke's pouch. When symptomatic, RCCs are usually treated surgically via marsupialization. Free mucosal graft (FMG) repair has shown promise in decreasing recurrence versus marsupialization alone. The authors present a case report with operative video of a patient with visual and endocrinological symptoms with a RCC treated with FMG following marsupialization. A search of the PubMed database from July 1997 through April 2022 was conducted using the terms 'Rathke's cleft cyst', 'Rathke's cleft cyst management', 'Rathke's cleft cyst repair', 'mucosal graft', 'mucosal coupling', 'transsphenoidal', and 'endoscopic endonasal'. Clinical and pathological aspects of the case presented were compared with information obtained from literature review. A 25-year-old female presented with a six-year history of amenorrhea, one-year history of anemia, headaches, and progressive visual loss. Imaging revealed an RCC. The patient underwent surgical treatment via an endoscopic endonasal approach. Marsupialization was achieved with placement of an FMG to help prevent restenosis. Post-operative examination revealed that the patient's OD vision returned to normal, although her OS vision was only slightly improved. Our review of the English literature resulted in 35 full-length articles that were published between 1997 and 2022. The literature suggests that FMG prevents scar formation by allowing for adequate epithelialization, thus decreasing the recurrence rate and being well tolerated by patients. Our findings support the utility of the FMG as a superior surgical treatment option for RCC management.


Asunto(s)
Carcinoma de Células Renales , Quistes del Sistema Nervioso Central , Quistes , Neoplasias Renales , Adulto , Carcinoma de Células Renales/cirugía , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Quistes del Sistema Nervioso Central/cirugía , Quistes/cirugía , Femenino , Humanos , Neoplasias Renales/cirugía , Procedimientos Neuroquirúrgicos/métodos
13.
Br J Neurosurg ; : 1-7, 2022 Jun 27.
Artículo en Inglés | MEDLINE | ID: mdl-35762111

RESUMEN

BACKGROUND: Purely suprasellar and some complex intra-suprasellar Rathke cleft cysts (RCCs) are commonly treated via extended endonasal endoscopic approach or traditional transcranial approach. The feasibility of the trans-eyebrow supraorbital keyhole approach (TSKA) for RCCs was evaluated in this retrospective study. METHODS: A cohort of 16 patients (11 females and 5 males) with RCC was surgically treated via TSKA between January 2013 and November 2021. The medical data and follow-up results were analyzed. RESULTS: Eight patients had a purely suprasellar RCC, and 8 patients had an intra-suprasellar RCC with most of pituitary gland occupying the sellar floor. The mean maximal size of the cysts was 15.4 (range 7-29) mm. Postoperatively, complete cyst drainage was achieved in 15 (93.7%) patients. Preoperative headache, visual dysfunction, and hypopituitarism improved in 12 (100.0%), 3 (75.0%) and 3 (75.0%) patients, respectively, and hyperprolactinemia normalized in all patients. Except 2 (12.5%) cases of transient diabetes insipidus (TDI), no other complications were observed. During the mean follow-up period of 41.0 (range 4-102) months, 1 (6.3%) radiological recurrence was found. CONCLUSIONS: For the treatment of purely suprasellar and some intra-suprasellar RCCs with most of the pituitary occupying the sellar floor, the endoscopic TSKA has the advantage of the minimal invasiveness, excellent visualization of the intrasellar compartment, no additional damage to the underlying pituitary gland, and no risk of CSF leakage. TDI or DI is relative commonly found in treatment of these RCCs. During surgery, care should be taken to avoid damage to the surrounding structures, including the pituitary stalk.

14.
Pituitary ; 24(5): 787-796, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34046806

RESUMEN

PURPOSE: Rathke cleft cysts (RCC) are a target for transsphenoidal surgery when symptomatic; however, the surgical techniques and outcomes continue to be of interest. Our goal is to highlight nuances in diagnosis, surgical philosophy and techniques, and outcomes. METHODS: We retrospectively reviewed a single surgeon experience in a contemporary series of RCC from April 2008 through December 2019. The clinical presentation, radiographic studies, intraoperative findings, histopathological diagnosis, postoperative complications, and outcome data were recorded and analyzed. RESULTS: A total of 131 endoscopic endonasal operations were performed on 116 patients (30 male, 86 female), with an average age of 39 years. We identified 102 patients without prior surgery, and 14 patients with prior surgery elsewhere. Postoperative complications included transient diabetes insipidus (DI) (n = 23, 20%), permanent DI (n = 5, 4%), SIADH (n = 6, 5%), epistaxis (n = 6, 5%), postoperative infection (n = 3, 2.5%), postoperative CSF leak (n = 1, < 1%), intrasellar hematoma (n = 1, < 1%), and stroke (n = 1, < 1%). Visual improvement was observed in 74% (n = 31/42) of patients presenting with visual loss, and headache improvement in 86% (n = 80/93). Overall, the recurrence rate in our cohort was 11%, with a lower recurrence rate seen in males (p = 0.02). The median follow-up duration was 15.2 months (interquartile range 1.7-52.3) CONCLUSION: Our results reflect the philosophy of a conservative surgical approach, designed to preserve normal pituitary function, and avoid DI, while achieving the maximal feasible resection. Evacuation of the cyst contents and meticulous sampling of the cyst wall when possible for histopathological diagnosis are crucial. The postoperative relief of symptoms and signs is gratifying, as are the overall rates of recurrence and outcomes.


Asunto(s)
Quistes del Sistema Nervioso Central , Quistes , Neurocirugia , Neoplasias Hipofisarias , Adulto , Quistes del Sistema Nervioso Central/diagnóstico , Quistes del Sistema Nervioso Central/cirugía , Quistes/diagnóstico , Quistes/cirugía , Femenino , Humanos , Masculino , Procedimientos Neuroquirúrgicos , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/cirugía , Estudios Retrospectivos
15.
Pituitary ; 24(3): 374-383, 2021 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-33433887

RESUMEN

PURPOSE: Pituitary abscesses (PAs) are a rare clinical entity which may arise from normal pituitary tissue or underlying lesions within the gland. Rathke's cleft cysts (RCCs) are not commonly associated with the development of PA. METHODS: Retrospective chart review of three patients with PAs within RCCs at a single university center and review of the literature. RESULTS: Three cases are reported. The first case presented with fever and headache and a history of prior surgery due to RCC and a recent respiratory tract infection. The second case had a history of recent skin infections and presented with sudden onset headache and hypopituitarism. In the third case, chronic visual field impairment prompted an ophthalmologic evaluation resulting in a diagnosis of an adenoma and an infected RCC. In all three cases, an endoscopic endonasal approach was performed to drain infected tissue and allowed microbiological identification of gram-positive cocci, followed by treatment with antibiotics for at least three weeks. Cases in the literature are scarce and the diagnosis is usually made intraoperatively due to non-specific manifestations and imaging. PAs arising from underlying pituitary lesions are less common than primary PAs. Differential diagnosis should include pituitary apoplexy, hypophysitis and other cystic lesions. CONCLUSION: PAs occurring in RCCs are infrequent. Clinical manifestations are commonly subacute, without septic symptoms. Imaging is usually non-specific. Preoperative diagnosis is infrequent and a broad differential diagnosis should be considered. Empirical antimicrobial therapy should be initiated and adjusted after obtaining cultures to reduce the rate of recurrence and improve clinical outcomes.


Asunto(s)
Carcinoma de Células Renales , Quistes del Sistema Nervioso Central , Neoplasias Renales , Enfermedades de la Hipófisis , Neoplasias Hipofisarias , Absceso , Quistes del Sistema Nervioso Central/diagnóstico , Quistes del Sistema Nervioso Central/cirugía , Cefalea , Humanos , Enfermedades de la Hipófisis/diagnóstico , Neoplasias Hipofisarias/diagnóstico , Estudios Retrospectivos
16.
Endocr Pract ; 27(11): 1128-1132, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-33965583

RESUMEN

OBJECTIVE: To evaluate the response of incidentally discovered pituitary cysts to growth hormone (GH) treatment. METHODS: A retrospective chart review was performed of children with pituitary cysts on magnetic resonance imaging (MRI) over a 5-year period. Records and images were reviewed, and the results were analyzed using descriptive statistics. Children with pituitary cysts who received GH treatment were compared with those without. RESULTS: We identified 109 children with pituitary cysts, 24 were treated with GH therapy. The average age was 8.5 ± 5.1 years. Children whose initial MRI scan was to evaluate growth hormone deficiency were more commonly male and non-Hispanic White compared with those with scans for other indications (male, 18 of 24 vs 35 of 85, P = .003; White, 23 of 24 vs 58 of 85, P = .004). Among patients who received GH treatment, 12 had follow-up MRI. Six had no change in cyst size and 6 had a decrease in cyst size. We observed no difference in the likelihood of cyst growth between those who received GH and those who did not (0 of 12 cysts with GH vs 1 of 15 cysts without GH showed growth at follow-up). No patient had neurologic deficits attributable to the pituitary cyst at any time. CONCLUSION: In a single-institution, retrospective study, we find no evidence of growth in pituitary cysts in response to GH therapy.


Asunto(s)
Quistes del Sistema Nervioso Central , Quistes , Hormona de Crecimiento Humana , Neoplasias Hipofisarias , Adolescente , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Quistes del Sistema Nervioso Central/tratamiento farmacológico , Niño , Preescolar , Quistes/diagnóstico por imagen , Quistes/tratamiento farmacológico , Hormona del Crecimiento , Hormona de Crecimiento Humana/uso terapéutico , Humanos , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos
17.
Endocr J ; 68(3): 269-279, 2021 Mar 28.
Artículo en Inglés | MEDLINE | ID: mdl-33087628

RESUMEN

Rathke's cleft cyst (RCC) is a common incidental tumor in the hypothalamic-pituitary region. Some reports have shown that the clinical symptoms and endocrine functions of symptomatic RCCs are temporarily improved by glucocorticoid administration. However, it is still unknown whether glucocorticoid treatment is effective for symptomatic RCCs according to long-term observations. In this study, we describe the long-term clinical outcomes of two cases of glucocorticoid-treated biopsy-proven secondary hypophysitis caused by RCCs. We summarize the symptoms, imaging findings, and endocrine evaluations of two symptomatic RCC patients with concomitant hypophysitis before and after prednisolone treatment. In both evaluated cases, visual impairments and altered endocrine parameters were present due to chiasm and stalk compression; these outcomes improved after shrinkage of RCCs in response to prednisolone administration, and partial recovery of anterior pituitary hormone secretion was observed. However, in both cases, the deficits in anterior pituitary hormone secretion recurred, possibly due to persistent inflammatory infiltration in the RCCs and pituitary glands. After relapse of hypophysitis, anterior hormone secretion did not fully recover. In our cases of secondary hypophysitis caused by RCCs, prednisolone administration had an early effect of cyst shrinkage, followed by partial improvements in clinical symptoms and pituitary functions. However, long-term observation showed that prednisolone treatment did not contribute to complete improvement in anterior pituitary hormone dysfunction.


Asunto(s)
Quistes del Sistema Nervioso Central/tratamiento farmacológico , Glucocorticoides/uso terapéutico , Hipofisitis/tratamiento farmacológico , Hipopituitarismo/tratamiento farmacológico , Neoplasias Hipofisarias/tratamiento farmacológico , Prednisolona/uso terapéutico , Fármacos Antidiuréticos/uso terapéutico , Quistes del Sistema Nervioso Central/complicaciones , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Quistes del Sistema Nervioso Central/patología , Desamino Arginina Vasopresina/uso terapéutico , Femenino , Terapia de Reemplazo de Hormonas , Humanos , Hidrocortisona/uso terapéutico , Hipofisitis/etiología , Hipopituitarismo/etiología , Persona de Mediana Edad , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología
18.
Endocr J ; 68(8): 943-952, 2021 Aug 28.
Artículo en Inglés | MEDLINE | ID: mdl-33814485

RESUMEN

Although Rathke's cleft cysts (RCCs) are common sellar/parasellar lesions, studies examining pituitary function in patients with nonsurgical RCC are limited. This study aimed to clarify the importance of RCCs, including small nonsurgical ones, as a cause of hypopituitarism by determining the prevalence of pituitary hormone secretion impairment and its relationship to cyst/tumor size in patients with RCC and in those with nonfunctioning pituitary adenoma (NFA). We retrospectively investigated the basal levels of each anterior pituitary hormone, its responses in the stimulation test(s), and cyst/tumor size in patients with RCC (n = 67) and NFA (n = 111) who were consecutively admitted to our hospital for endocrinological evaluation. RCCs were much smaller than NFAs (median height, 12 vs. 26 mm). The prevalence of gonadotropin, PRL, and GH secretion impairment in RCC was lower in comparison to NFA (19% vs. 44%, 34% vs. 61%, and 24% vs. 46%, respectively), whereas the prevalence of TSH and ACTH secretion impairment was comparable (21-27% and 17-24%, respectively). A significant positive relationship between cyst/tumor size and number of impaired hormones was observed in both groups, but smaller cysts could cause hormone secretion impairment in RCC. Stimulation tests suggested that most hormone secretion impairment was attributable to the interrupted hypothalamic-pituitary axis in both groups. Therefore, RCC, even small ones, can cause pituitary dysfunction. Different mechanisms may underlie hypothalamic-pituitary interruption in RCC and NFA.


Asunto(s)
Adenoma/fisiopatología , Quistes del Sistema Nervioso Central/fisiopatología , Adenohipófisis/fisiopatología , Neoplasias Hipofisarias/fisiopatología , Adenoma/sangre , Adulto , Anciano , Anciano de 80 o más Años , Quistes del Sistema Nervioso Central/sangre , Femenino , Hormona Liberadora de Gonadotropina/sangre , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Hipofisarias/sangre , Estudios Retrospectivos , Tirotropina/sangre , Adulto Joven
19.
Arkh Patol ; 83(6): 60-70, 2021.
Artículo en Ruso | MEDLINE | ID: mdl-34859988

RESUMEN

In the Turkish saddle area, there is a wide variety of pathological processes, the vast majority of which present as tumors of various origins (up to 90%). For a clear morphological verification of the diagnosis, it is proposed to use a diagnostic algorithm that includes the stages of differential diagnosis of normal adenohypophysis and neurohypophysis with tumors in the anterior and posterior lobes of the pituitary gland, non-pituitary origin neoplasms, as well as with non-tumor pathological processes (inflammation, cystic masses, and hyperplasia). For morphological diagnosis, histochemical and immunohistochemical methods are recommended using various staining techniques (silver impregnation, periodic acid Schiff reaction) of tissue specimens and antibody panels (pituitary hormones, low-molecular cytokeratins, pituitary transcription factors, neuroendocrine markers, etc.).


Asunto(s)
Adenoma , Neoplasias Hipofisarias , Algoritmos , Diagnóstico Diferencial , Humanos , Queratinas , Hipófisis , Neoplasias Hipofisarias/diagnóstico
20.
Zh Vopr Neirokhir Im N N Burdenko ; 85(6): 111-118, 2021.
Artículo en Ruso | MEDLINE | ID: mdl-34951768

RESUMEN

Central diabetes insipidus is a neuroendocrine disorder caused by disturbances in antidiuretic hormone release. The last one is responsible for fluid and electrolyte balance regulation. The most common cause of diabetes insipidus is resection of sellar-suprasellar tumors followed by damage to hypothalamic nuclei responsible for antidiuretic hormone release, disruption of antidiuretic hormone transportation from hypothalamus or its release by neurohypophysis. According to various data, postoperative diabetes insipidus occurs in 13-30% of cases. The highest risk of diabetes insipidus is observed after resection of craniopharyngioma, Rathke's cleft cyst and ACTH-releasing pituitary microadenoma. This review is devoted to prevalence and predictors of diabetes insipidus after resection of sellar-suprasellar tumors.


Asunto(s)
Quistes del Sistema Nervioso Central , Craneofaringioma , Diabetes Insípida Neurogénica , Diabetes Mellitus , Neoplasias Hipofisarias , Craneofaringioma/cirugía , Diabetes Insípida Neurogénica/epidemiología , Diabetes Insípida Neurogénica/etiología , Humanos , Neoplasias Hipofisarias/cirugía , Prevalencia
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