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Publicly Available Published by De Gruyter July 9, 2018

Symptomatic Rathke cleft cyst in paediatric patients – clinical presentations, surgical treatment and postoperative outcomes – an analysis of 38 cases

  • Monika Prokop-Piotrkowska EMAIL logo , Elżbieta Moszczyńska , Paweł Daszkiewicz , Marcin Roszkowski and Mieczysław Szalecki

Abstract

Background:

Rathke cleft cysts (RCC) are benign, epithelium-lined intrasellar and/or suprasellar cysts believed to originate from the remnants of the Rathke pouch. The aim of this study was to analyse the symptoms and surgical outcome of patients with the diagnosis of RCC verified in a histopathological examination of the postoperative material.

Methods:

The study is a retrospective analysis of 38 cases of children who underwent a neurosurgical treatment due to RCC at the Children’s Memorial Health Institute in Warsaw, Poland, between 1994 and 2015.

Results:

At diagnosis, the mean age was 13 years and 8 months (6 years and 11 months–17 years and 10 months, sex ratio was 1:0.9 with a female prevalence). The most common symptoms were the following: headache (50%), hypothyroidism (50%), short stature and/or decreased growth velocity (47%), delayed puberty and menstrual abnormalities (37%), diabetes insipidus or polydipsia and polyuria (26%), adrenal dysfunction (26%), sleepiness and general weakness (13%) and visual disturbances (11%). Due to the gravity of symptoms and size of the lesion, all the patients underwent a surgical treatment. All but one were successful (one patient died due to postoperative neurosurgical complications). The most common postoperative complications were the following: adenohypopituitarism (67%) and diabetes insipidus (45%).

Conclusions:

RCC can present with serious symptoms that significantly deteriorate patients’ quality of life. Despite a successful neurosurgical treatment in most of the analysed cases, patients required long-term pharmacological treatment.

Introduction

Rathke cleft cysts (RCC) are epithelium-lined intrasellar and/or suprasellar cysts containing mucoid material, which are benign, nonneoplastic lesions that originate from the remnants of the Rathke pouch [1], [2].

They are one of the most common lesions in the sellar region, found in 13–33% of autopsies, and they are diagnosed in 1% of all intracranial lesions [3], [4]. However, although its prevalence in adults is rather high, it is much rarer in children [5], [6], [7]. In one study, among patients younger than 15 years old who underwent magnetic resonance imaging (MRI) due to epilepsy, psychomotor retardation or headache, pituitary cysts suggestive of RCCs were observed in 1.2% [8]. It has been suggested that the incidence of these benign lesions is rising due to the increased utilisation of advanced imaging techniques in paediatric patients [9], [10].

Mostly, they are asymptomatic findings [8]; however, depending on their size and localisation, they can present with a wide spectrum of symptoms. The most common symptoms in children are headache, hypopituitarism, central precocious puberty and visual disturbances [5], [6], [7], [9], [10], [11], [12], [13], [14].

There is no literature known to the authors regarding the genetic background of RCC or any reports of RCC in siblings besides a case report of a patient with tetrasomy X and RCC and a case report of two twin sisters with von Hippel-Lindau disease type 2C both of whom diagnosed with RCC [15], [16]. However, only one of the twins had an operation performed, making the histopathological examination possible; diagnosis of the other twin was made on the basis of an MR examination [16].

This study is a retrospective analysis of symptoms and surgical outcome of children treated at the Children’s Memorial Health Institute due to RCC as well as an analysis of long-term follow-up data.

Materials and methods

A total number of 38 patients who underwent an operation due to RCC at the Children’s Memorial Health Institute in Warsaw, Poland, from December 1994 to April 2015, were taken into consideration in this retrospective, clinical study.

Enrolled patients were children and adolescents, whose mean age at diagnosis was 13 years and 8 months (±3.27 standard deviation [SD], 6 years and 11 months–17 years and 10 months) (Figure 1). The sex ratio was 1:0.9 (there were 20 girls and 18 boys in the group). Two female patients were twins, who had different symptoms and the diagnosis was made at a different age (with the difference of nearly 4 years).

Figure 1: Age of patients at diagnosis.
Figure 1:

Age of patients at diagnosis.

Although there are patients under observation in our institute with a pituitary lesion found in the MRI scan that is suspected to be RCC, only patients who underwent an operation and had a histopathological examination made were taken into consideration in this study (Figure 2).

Figure 2: (A and B) Haemotoxylin and eosin (H&E) stain. The histopathology is consistent with Rathke cleft cyst.
Figure 2:

(A and B) Haemotoxylin and eosin (H&E) stain. The histopathology is consistent with Rathke cleft cyst.

All the surgical interventions were performed at the Children’s Memorial Health Institute in Warsaw, all but one by the same neurosurgeon.

Medical data of the patients were collected from the Children’s Memorial Health Institute in Warsaw and analysed retrospectively. To collect the follow-up information, the authors contacted the patients or their parents using the personal data available in the medical documentation. They were contacted by phone and asked to answer the author’s survey.

It was possible to obtain the follow-up data with 31 out of 37 patients (84%).

At the moment of the survey (June–July 2016), the average age of the patients was 24 years (±6.19 SD, 21 of them were adults and 10 patients were below 18 years). The age ranged from 9 years and 9 months to 31 years and 10 months.

The follow-up period was on average 8 years and 1 month long (±5.26 SD, 1 year and 1 month – 16 years and 9 months), which was calculated using the time since the first operation.

Results

Neuroradiological findings

The average diameter of the cysts was 16.6 mm (±6.12 SD, 8–32 mm, the largest dimension of the lesion was taken into consideration). Unfortunately for four patients, no data on the size of the lesion was available.

One patient had the lesion defined as suprasellar (3%), 20 patients as intrasellar (53%) and 17 patients as intra- and suprasellar (45%) (Figure 3). In 11 cases, the cyst was described as compressing the optic chiasm (29%).

Figure 3: MR image of Rathke cleft cyst.
Figure 3:

MR image of Rathke cleft cyst.

In 16% of the cases, calcifications were observed either in brain imaging or intraoperatively.

Symptoms and endocrinological findings

The most common symptoms before each operation were the following: headache (50%), hypothyroidism (50%), short stature and/or decreased growth velocity (47%), delayed puberty and/or menstrual abnormalities (37%), diabetes insipidus or polydipsia and polyuria (26%), adrenal insufficiency (26%), sleepiness and general weakness (13%), visual disturbances (11%).

These symptoms are visualised in the diagram (Figure 4) as a dark blue field.

Figure 4: Deficits in hormonal axes before and after operation.
Figure 4:

Deficits in hormonal axes before and after operation.

Surgery

Due to the large diameter of the cysts and serious symptoms, significantly deteriorating the quality of patients’ life, all the patients in our study were qualified for surgical treatment of the lesion. All these patients presented with more than one clinical symptom as above or symptoms of chiasmal compression and at least one other clinical symptom.

Twenty-nine patients underwent a transsphenoidal operation (76%) and in nine cases of large lesions extending to suprasellar cistern, a craniotomy was performed (24%). All but one were successful (one patient died due to postoperative persistent cerebrospinal fluid [CSF] liquorrhea, severe pneumocephalus with brain herniation 2 months after the operation).

Total resection of the cyst was achieved in 26 patients (68%).

In the case of asymptomatic, incidentally found sellar cysts, the decision to control it in brain imaging is the most common one, even if the diameter of the cyst is significant. It has also been observed that even big lesions found in young children tend to diminish in size over time, especially during puberty (author’s observation, unpublished data).

Postoperative morbidity

The most common preoperative symptom, which was a headache, resolved in all the cases directly after the operation. Although one patient now suffers from strong head and back pain, there is no sign of relapse of the cyst after observation via MRI.

Multiple pituitary hormone deficiency (MPHD), defined as a deficiency of two or more pituitary hormones [17], was diagnosed in 19 out of 31 patients after the neurosurgical treatment (62%), which is more than the number of patients with symptoms of MPHD before the operation: 21 out of 38 patients (55%).

Six patients did not need any hormonal treatment at all (19%) and two of them are not under endocrinological care anymore. Among the other 25 patients who need pharmacological treatment, 21 (67% of the entire group) need supplementation of the thyroid axis, 15 (48%) of the adrenal axis, 14 (45%) suffer from diabetes insipidus and obtain adequate treatment, 11 (35%) need supplementation of sex hormones and eight (26%) are treated with growth hormone (Figure 4 – light blue field). Endocrinological problems in light of most of the hormonal axes worsened after the operation: 17% more patients had hypothyroidism, 22% more patients had adrenal insufficiency, 19% in the case of diabetes insipidus, but, on the other hand, 21% less patients showed short stature and/or decreased growth velocity and 2% less patients had insufficiency of sex hormones axis.

Three patients (10%) have subjective ophthalmological problems (losses of the visual field and in one case atrophy of the optic nerve). Before the operation, visual disturbances were noticed in four patients (11%), but in three of them these problems resolved postoperatively. In two patients, visual disturbances were noticed after the neurosurgical operation for the first time.

Follow-up

In four patients (11%), a reoperation due to progressive enlargement of RCC was necessary, which was conducted after 13, 23, 61 and 133 months after the first operation (on average 57.5 months, which is 4 years and 10 months, ±54.42 SD). In all of these cases, the first operation was subtotal; in one case it was only an evacuation of the fluid with a biopsy of the cyst wall.

Considering the metabolic problems, the average body mass index is 22.08 kg/m2 (±5 SD, 14.32–33 kg/m2). Three of our patients are obese (10%), six are overweight (19%), which is less than that in the Polish population in general (for age groups of 15–19 years and 20–29 years) [18]. On the other hand, six patients are underweight (19%).

Regarding education, 10 out of 14 patients over 25 years have higher education. Among patients younger than 25 years, 10 are following a regular school career, four finished secondary school and entered work market, two have an individual education and one interrupted their education during secondary school.

Among patients older than 25 years (14 patients), 12 work and are financially independent (85%) and five of them are employed or self-employed in a career related to their education (41%). One of the patients is still provided for by his parents and social benefits, but besides RCC he suffers from epilepsy, urological, nephrological and gastrological disorders, is hearing impaired and mute.

Eight out of 14 patients over 25 years have a life partner (57%) and two of them have children (25%). Two female patients had problems with getting pregnant, one succeeded and is now pregnant with a second child, another one has not succeeded yet, but in this case, there are andrological problems with the partner.

Discussion

In our study, a relatively big group of patients were analysed in consideration of symptoms and effectiveness of treatment of RCC. Symptoms at diagnosis were similar to those described by different authors [5], [6], [7], [9], [10], [11], [12]. Detailed analysis of symptoms found in RCC in children according to previous reports is presented below (Table 1). The most frequent symptom in our study was a headache, reported by half of the patients. In prior studies, it was reported by 15–85% of patients (on the average 50.2%) [5], [6], [7], [9], [10], [12], [13], [14], [19]. As it is a very common complaint in the general population, it might not be obvious that it was caused by the mass effect of the lesion; however, in our study, it resolved in all the cases after the operation. Observed hormonal deficits at diagnosis were also similar to those mentioned by other authors (Table 1).

Table 1:

Summary of prior studies on paediatric RCC.

Author, yearNo. of patientsAge, year, median, rangeClinical symptoms at the moment of diagnosisCyst size, mm, median, rangeSurgical treatment, %Recurrence rate, %Follow-up, months
Headache, %Visual disturbances, %Adenohypopituitarism, %Diabetes insipidus, %Other, %
Shepard et al. (2018) [11]2413 (1–18)794844a9 (3–19)291458 (8–180)
Jung et al. (2017) [10]919.7 (1.2–18)21.33.3233.3NAb10.1 (±3.7)/15 (±4.1)c7.7024
Hayashi et al. (2016) [5]1112.2 (5–18)8218451818d19 (8–33)732512–152
Oh et al. (2014) [9]349.7 (4–18)15041035e6.2 (1–17)00f12
Müller et al. (2012) [12]1410.2 (3.3–15.2)237NAgNANA3.4 (2.3–6.5) cm3100h0/76i86.4 (8.4–338.4)
Jahangiri et al. (2011) [6]1416 (3–18)507.1457NA12 (7–20)1001438
Lim and Yang (2010) [13]4410.1 (0.1–18.2)45.513.643% endocrine dysfunctionNANA34.1NA15.6 (0.1–95)
Katavetin et al. (2010) [14]1314 (7–17)851515/023j12.2 (7–15)312521 (6–60)
Zada et al. (2009) [7]1013 (2–17)801060020k13.6 (8–18)100l1034
Our study3813.6 (6.9–17.8)5011682613d16.6 (8–32)1001197
  1. NA, not applicable. aPrecocious puberty. bCentral precocious puberty in 39.5%, hyperprolactinemia in 2% of patients. cNon-invasive/invasive treatment. dActivity loss, fatigue, anorexia. eCentral precocious puberty. fA follow-up MRI was performed in 35% of patients after 12 months. gGrowth retardation in 57% of patients. hComplete resection in 36%. i0% of relapses after complete resection. 76% of progression after incomplete resection. jHyperprolactinemia in two cases and precocious puberty in one case. kMeningitis, weight gain. lComplete cyst drainage in 90%.

A significant clinical problem is how to distinguish RCC from craniopharyngioma (CP), adenomas (especially its cystic presentation), cyst of pars intermedia, arachnoid cyst and dermoid cyst. In brain imaging, RCC is suspected when there is a sharply demarcated cystic lesion situated posterior to the anterior pituitary lobe with a homogenous signal intensity [8]. On T1-weighted imaging, two-thirds of lesions are hyperintense and one-third of them are hypointense. On T2-weighted imaging a quarter are isointense, a quarter are hypointense and the rest are hyperintense [4]. RCC and CP have the same embryological aetiology, similar characteristics in brain imaging and, in some cases, there are diagnostic problems in the histopathological examination. Thus, it is even suggested that there is a spectrum of changes between RCC and CP [20], [21], [22], [23].

There have been studies conducted on the paediatric population comparing the characteristics of these lesions [5], [12]. Müller et al. stated that RCC was characterised by smaller intrasellar masses that led to severe pituitary deficiencies without hypothalamic sequelae, such as obesity [12]. In our group of patients, no symptoms suggesting hypothalamic involvement were observed. Nonetheless, only 53% of lesions were purely intrasellar and the rest were both intra- and suprasellar or, in one case, a strictly suprasellar mass.

Hayashi et al. described that RCCs are relatively small, of regular, oval shape and hypointense on T2-WI MRI in 55% of cases [5]. In their study, in 36% of the cases RCCs were located in the suprasellar region and they concluded that these lesions cannot be definitely differentiated from CP by preoperative imaging [5]. What is more, they found no calcifications in RCC while they were observed in all the cystic CP [5]. In our study, however, in 16% of the cases calcifications were observed in brain imaging or intraoperatively. In a study by Shepard et al., on MRI, RCCs are described as often hyperintense on T2-weighted images with either a hyper- or a hypointense appearance on T1-weighted images depending upon the cyst content that typically lack contrast enhancement and do not have evidence of calcification [11].

Choi et al. concluded in their study performed among adult patients that characteristics such as superiorly lobulated shape, third ventricle compression by superior tumour extension, mixed solid and cystic characteristics and reticular enhancement of the solid portion were more common in CPs (p<0.017). On the other hand, an ovoid shape, a small tumour volume, cystic characteristics and no or thin cyst wall enhancement were more common in RCCs (p<0.017) [24].

Although RCC is defined as a lesion in the pituitary region, there is also a case report of RCC in the prepontine cistern diagnosed in a 14-year-old girl [25].

According to the approach of specialists (radiologists and neurosurgeons) in our institute, a definite diagnosis of RCC can be confirmed only by an examination of the postoperative material by a pathologist. Thus, only patients who underwent an operation and had this examination made were taken into consideration in this study.

An area for future exploration is a frozen section during surgery, which could help differentiate RCC from CP, thus allowing modification of the extent of the operation. In our material, we did not perform such examinations.

It is important to underline that even with an experienced pathologist, a misdiagnosis is possible. In two of our patients, initially, a diagnosis of CP was made (two twin patients), but it was changed later to RCC after re-examination.

Nonetheless, a histopathological examination gives a more accurate diagnosis than a clinical or radiological one. Clinical assessment is not of much significance for diagnosis as it has been proven by different authors that symptoms can be varied and sometimes contradictory to what one would expect. Namely, there are reports on precocious puberty in girls with RCC [10], [14], [26] but in our group, no such symptoms were observed. Katavetin et al. suggested that female preponderance (5.5:1 in this study) and presentation only in patients who achieved puberty (in all, 13 cases, of which four were confirmed histopathologically) was significant for patients with RCC [14], but in our study the sex ratio was 1:0.9 (there were 20 girls and 18 boys in the group) and 10 patients presented with late onset of puberty. Female patients were also predominant (64 vs. 27) in a study performed by Jung et al., although, similarly, only a small number of patients had the diagnosis made on the basis of a histopathological examination [10].

Surgical resection of the cyst in the case of severe symptoms and considerably big lesions observed in the presented material seems to be the best treatment option. Although it does not alleviate the endocrinological symptoms and in many cases, pituitary function deteriorates postoperatively, there is a high risk of a neoplastic lesion and it is impossible to exclude CP with certainty on the basis of brain imaging. This approach was also shared by Müller et al. who concluded in their study that the treatment of choice is radical surgery with the intention to preserve the integrity of hypothalamic structures and that regular monitoring is obligatory due to a high rate of relapse/progressions, which in their study occurred exclusively during the first 2 years after the primary surgery [12]. What is stressed in another study by this author is that gross total resection is not recommended in the case of sellar masses presenting with hypothalamic involvement in order to prevent further hypothalamic damage and thereby hopefully increase the long-term outcome [27]. According to Hayashi et al., standard partial excision of the cyst wall and complete aspiration of the cyst contents are considered to result in total resolution of RCC while minimising complications, but on the other hand, they conclude that the decision to observe an incidentally found RCC without hormonal deficits and no chiasmal compression seems to be a reasonable one [5]. Other authors also share the approach that small, asymptomatic cysts believed to be RCC can be managed with non-invasive treatments [6], [9].

In a recent study by Jung et al., a conservative approach with follow-up MRI was preferred in cases of silent RCCs or smaller cysts with mild symptoms, and surgery was preferable for larger cysts with definite pressure symptoms. They demonstrated that most cases of RCCs without pressure symptoms remain the same or decrease in size over time. Only 7.7% of the patients in their study were operated, all by transsphenoidal microscopic surgery, either to alleviate pressure symptoms or to investigate the possibility of malignancy because of the increasing size of the RCC [10].

In another study published lately, by Shepard et al., it is underlined that the majority of conservatively managed paediatric patients with RCCs will have spontaneous headache resolution, although, in most of the patients, cyst fenestration alleviates this symptom [11]. They suggest that RCC fenestration may be indicated in patients who develop visual field deficits, show radiological evidence of chiasmal compression or have medically refractory headaches. They also reported spontaneous RCC regression in a substantial number of individuals; thus, they conclude that in the absence of optic compression, visual field deficit or diagnostic uncertainty, many paediatric cases of RCC can be managed conservatively. They recommend annual MRI studies and clinical visits to assess RCC progression and new symptom development [11].

In the literature, there are no reports of perioperative mortality in RCC [2], besides a study by Daubenbüchel et al. who examined cases of paediatric CP and RCC with hydrocephalus and/or hypothalamic involvement. In their study, 23 out of 177 patients died during the follow-up, but a majority of these patients were diagnosed with CP, not RCC [27].

There is also a vivid discussion on the indications for surgical treatment of RCC in the adult population.

In a meta-analysis performed by Mendelson et al., the authors evaluated the overall recurrence rate of RCC after a transsphenoidal operation in 1151 adult patients with symptomatic RCC at 12.5% and found no significant difference between less aggressive interventions like cyst drainage with wall biopsy and more aggressive – subtotal or gross total resection, while aggressive resection had a much higher rate of postoperative endocrine dysfunction [28].

In another study, 75 cases of adults with radiologically, incidentally diagnosed RCC were analysed and it was demonstrated that the majority of the lesions remained unchanged or decreased in size over time. The authors concluded that, in the absence of pressure symptoms, it is reasonable to manage patients with RCCs conservatively [3].

However, there are also voices in favour of endonasal endoscopic transsphenoidal surgery (ETSS), which provides excellent clinical and endocrinologic improvement according to Ratha et al. [29]. They believe that radical excision does not necessarily result in endocrinological impairment and may have a better impact on recurrence and cyst resolution [29]. Also, in a study by Fukui et al. ETSS was effectively and safely performed on patients with severe headaches (which can develop even from small RCCs) to relieve their headaches [30]. Kuan et al. also concluded in their study that the transnasal, transsphenoidal surgical approach is safe and effective in the surgical decompression of RCC [31]. Also, a meta-analysis performed by Altuwaijri et al. demonstrates that the resection of RCC in patients presenting with headache is associated with headache resolution [33].

The most significant advantage of our study is the number of enrolled patients, all of whom had the diagnosis confirmed by a histopathological examination, which contributes to the biggest study group described in the available literature so far and has a relatively long follow-up duration. Disadvantages of this study are as follows: its retrospective character and lack of preoperative examination of all the hormonal axis in all the patients. In most of the cases, laboratory tests were performed according to the symptoms.

Further multi-institutional, prospective studies on a bigger number of patients are required to investigate the factors differentiating RCC from CP preoperatively and determine the best treatment choice for the patient.


Corresponding author: Monika Prokop-Piotrkowska, MD, Department of Endocrinology and Diabetology, Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland

  1. Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  2. Research funding: None declared.

  3. Employment or leadership: None declared.

  4. Honorarium: None declared.

  5. Competing interests: The funding organisation(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.

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Received: 2017-12-22
Accepted: 2018-06-11
Published Online: 2018-07-09
Published in Print: 2018-08-28

©2018 Walter de Gruyter GmbH, Berlin/Boston

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