Journal of Neurological Sciences [Turkish] 31:(2)# 40; , Case Report

Transcription

1 Journal of Neurological Sciences [Turkish] 31:(2)# 40; , Case Report Giant Rathke Cleft Cysts: Report of Two Cases Aydın CANPOLAT 1, Osman AKDEMIR 1, Hakan DUMAN 1, Türker KARANCI 2, Şahin YÜCELI 3, Harun ÖZLÜ 1, Emre ALTUNRENDE 1, Mehmet Osman AKÇAKAYA 1 1 Taksim Education and Research Hospital, Neurosurgery, İstanbul, Türkiye 2 Medicana Hospital, Neurosurgery, İstanbul, Türkiye 3 Neon Hospital, Neurosurgery, Erzincan, Türkiye Summary Rathke cleft cysts, known to result from defects during the embryological development period, may remain asymptomatic until adulthood. Herein we report 34 and 62 years old two female patients with Rathke cleft cysts, who admitted with visual problems and headache. The different clinical behaviors and the treatment options which may vary according to the lesion size of the radiologically diagnosed cysts were discussed in the light of the current literature. Key words: Embryology, Rathke cleft cyst Özet Dev Rathke Kleft Kistleri. İki Olgu Sunumu Embriyonel gelişim döneminin bir defekti sonucu ortaya çıktığı bilinen Rathke Kleft Kisti, erişkin döneme kadar asemptomatik olarak kalabilir. Farklı klinik davranışları olan, radyolojik olarak tanı konulan ve lezyonun boyutlarına göre değişen tedavi seçenekleri tercih edilebilen, görme bozukluğu ve başağrısı ile başvuran 34 ve 62 yaşlarındaki iki bayan olgumuzu literatür bilgileri ışığında inceledik. Anahtar Kelimeler: Embriyoloji, rathke kleft kisti INTRODUCTION A Rathke cleft cyst is the remnant of pharyngeal ductus, primitively originating from the oral cavity (11). It is rarely seen in the sellar-suprasellar regions and is generally small and asymptomatic. The larger lesions, through the effect of compression, may cause different clinical symptoms and hormonal disturbances, primarily visual problems (6,9,14). In autopsy series, it occurs at a rate of approximately 20%. The rate of occurrence is more in the fourth and the fifth decades and the female to male ratio is approximately 3:1 (13). CASE PRESENTATION Case I A 34-year-old female patient referred to the clinic with complaints of headache and a decrease in visual acuity of the left eye. Upon physical examination, visual acuity was counting fingers at 1 meter and left temporal hemianopia was detected. Upon fundus examination, optic atrophy was detected on the left side. No hormonal 377

2 disturbances were seen in the laboratory examination. A cystic lesion filling the sella and the suprasellar cistern, eroding the clivus, enlarging towards the sphenoid sinus, and posteriorly flattening the hypophysis by superiorly displacing the third ventricle and the optic chiasm, was seen in cranial MRI (Figure 1 and 2). The initial diagnosis was thought to be a Rathke cleft cyst and craniopharyngioma or cystic hypophysis adenoma was also considered in differential diagnosis. After patient provided informed consent, she was operated on and cyst drainage and partial excision of the cyst wall were performed with a frontotemporal craniotomy. A significant improvement in the visual acuity of the patient was observed after the operation. The histological diagnosis was reported as a Rathke cleft cyst from the lining of the wall of the lesion with low, single-layered cilia columnar epithelium (Figure 3a, 3b). Case II A 62-year-old female patient was referred to the clinic with complaints of a decrease in visual acuity in both eyes, more prominent in the last month, and headache. Upon physical examination, visual acuity in both eyes was reported as counting fingers at 2 meters, and bitemporal hemianopia was detected. No hormonal disturbances were found in laboratory examination. A 35x30x20 mm cystic lesion, filling the suprasellar cistern, anteriorly producing an indentation towards the third ventricle, extending towards the anterior fossa along the interhemispheric fissure, anteriorly enlarging the sella, and causing both anterior cerebral arteries to be springy, was observed (Figure 4, 5 and 6). It was initially believed to be a Rathke cleft cyst, and in the differential diagnosis, was believed to be a craniopharyngioma or cystic hypophysis adenoma. After the patient provided informed consent, she was operated on and cyst drainage and partial excision of the cyst wall were performed with a frontotemporal craniotomy. A significant improvement in the visual acuity of the patient was observed after the operation and the histopathological diagnosis reported a Rathke cleft cyst, similar to the first case, which was lined by cilia columnar epithelium with mucinous cytoplasm inside (Figure 7a, 7b). Figure 1: The sellar-suprasellar mass in T2 weighted sagittal MR in the first case 378

3 Figure 2: The cystic mass in T2 weighted axial MR in the first case Figure 3a (H&E x200) and Figure 3b (H&E x100): The cyst wall lined by single layered columnar epithelium on histopathological examination 379

4 Figure 4: The cystic mass in T2 weighted axial MR imaging in the second case Figure 5: The cystic mass in T2 weighted axial MR in the second case 380

5 Figure 6: The cystic mass in T2 weighted coronal MR in the second case Figure 7a (H&E x400) and Figure 7b (H&E x200): The single layer ciliated columnar epithelium with mucinous cytoplasm lining the cyst wall in the histopathological examination of the second case 381

6 DISCUSSION The craniopharyngeal ductus is divided into the anterior lobe, pars intermedia and the pars tuberalis, and disappearing in the early fetal period. The Rathke cleft cyst is formed as a result of a defect that occurs in the regression of this ductus during transformation into adenohypophysis and neurohypophysis (11). These lesions were first defined by German anatomist Martin Heinrich Rathke (12). A symptomatic Rathke cleft cyst (RCC) forms in approximately 6-10% of sellar-suprasellar lesions. Preoperatively, it is difficult to differentiate these lesions from other pathologies of the pituitary region, such as pituitary adenoma, craniopharyngioma, or arachnoid cysts (6,11). In light of the generally accepted knowledge, craniopharyngiomas are rarely seen in the intrasellar compartment, whereas RCC is more frequently seen (6). The anterior disposition of the pituitary gland in RCC is seen more frequently than other pituitary pathologies (6,11). As shown in the study of Shin et al., the average size of a craniopharyngioma is greater than 20 mm at a rate of 80%, whereas the frequency of the occurrence of RCC of the same size is 20% (13). In many reported series, it appears in patients around years old on average and it is seen more frequently in females. As the hormonal disturbances cause symptoms earlier in females, it is thought that it is diagnosed more frequently in females. Rathke cysts that are generally small and asymptomatic sometimes cause symptoms such as endocrine dysfunction, headaches, and visual disturbances. Headache is the most frequently seen symptom and it is seen at a rate of 55-81% (9,14). Acute pain episodes may mimic pituitary apoplexy. Pituitary dysfunction is seen at a rate of 17-81% and occurs as a result of chronic compression of the cystic lesion or inflammation caused by the rupture of the cyst. Hyperprolactinemia is most frequently seen. Hypocortisolism, hypothyroidism, hypogonadism, growth hormone deficiency, and rarely, panhypopituitarism, can be seen (9). Diabetes insipidus is seen more frequently in cases of hypophysitis caused by inflammation of the cyst content (7,9). Visual disturbances in symptomatic cases of RCC are seen at a rate of 19-35% due to chiasmopathy (9). Syncope and pathological laughing attacks caused by giant RCC were reported in the literature (2,4). In these cases, brain stem compression and its effect on pontomedullary reflex activity causing the laugh reflex, which is controlled by the anterior and posterior corticobulbar descending pathways, are thought to be responsible (2). Histopathologically, RCC is composed of mucin secreting, ciliated, cuboid, or columnar Goblet cells. It is suggested that cysts showing squamous metaplasia demonstrate more aggressive behaviors and their recurrence is more frequent (10). In our cases, the histopathological diagnosis was completed by observing the abovementioned findings. In both cases, there were no clinical findings of endocrine dysfunction. At times, clinical attacks may be seen as a result of hemorrhage inside the cyst, the leaking of the cystic fluid to the intrasellar or parasellar regions (12). The visual loss, persistent headache, no response to treatment, and serious hormonal insufficiency causing the enlargement of the cyst are the important factors determining the surgical decision (5,9). Surgical options may include transcranial and transsphenoidal approaches. With the development of the endoscope technology, extended endoscopic endonasal transsphenoidal approach may also be performed for suprasellar lesions. Jahangiri et al presented a series of patients with Rathke cleft cysts managed via endonasal transsphenoidal approach microscopically or endoscopically (8). They reported better results by means of complete removal and 382

7 visual outcomes for endoscopic surgery compared to microscopic surgery (8). As reported as a valuable tool in the surgical management of sellar, para- and suprasellar lesions, endoscopes provide better illumination, larger surgical view and with the use of angled lens endoscopes an opportunity to look around the corner (3). They minimize manipulation to neural and vascular structures and provides surgical maneuverability without brain retraction. However a greater risk of cerebrospinal fluid (CSF) fistulas and difficulties of hemorrhage control from intracranial vessels were listed as the disadvantages of this technique (3). The most accepted management in surgical treatment is drainage and partial excision of the cyst wall through the transsphenoidal approach. The transcranial approach is recommended in suprasellar, largely expanded, or recurrent cases, and in conditions in which the pituitary gland show anterior-inferior extension (1,5,14). The transcranial approach may lead to complications like postoperative diabetes insipitus, new onset hypopituitarism, injury to optic apparatus, meningitis, CSF leakage and motor deficits. However this approach provides better manipulation of the neural and vascular structures. In both of our cases, the patients were operated by transcranial approach and drainage of the cyst, cystocysternal marsupialization and partial excision of the cyst wall was performed. Although recurrence is seen less in cases in which radical cyst resection is completed, the risk of occurrence of CSF fistula and pituitary dysfunction increases (14). In a series of Kim et al., the recurrence was reported as 5% as result of a 77 months' follow-up (9). The recurrent cyst formation is seen more frequently within the first 5-6 years. The factors thought to be responsible from this condition are contrast enhancement in MRI, small extension of the cyst wall resection, the presence of a previous residual mass, inflammatory changes, squamous metaplasia, alcohol use in the previous surgery, the large size of the cyst preoperatively, the use of lipid graft, and the presence of transitional histology (14). It is recommended to follow-up on the cases with MRI at least for 10 years. CONCLUSION Rathke cleft cyst, one of the pathologies of sellar-suprasellar region, occurs as a result of a developmental defect seen in the embryological period. It is clinically presented by the size of the mass and its compression effect on the neighboring structures. While transsphenoidal approach was defined more frequently in its surgical treatment, transcranial surgery is primarily preferred in some situations. Correspondence to: Osman Akdemir osakdemir@yahoo.com Received by: 19 July 2013 Revised by: 08 January 2014 Accepted: 12 April 2014 The Online Journal of Neurological Sciences (Turkish) This e-journal is run by Ege University Faculty of Medicine, Dept. of Neurological Surgery, Bornova, Izmir-35100TR as part of the Ege Neurological Surgery World Wide Web service. Comments and feedback: editor@jns.dergisi.org URL: Journal of Neurological Sciences (Turkish) Abbr: J. Neurol. Sci.[Turk] ISSNe

8 REFERENCES 1. Cakabay M, Iplikçioğlu AC, Gökduman CA, Dinc C, Sav A. Rathke Kleft Kistleri; klinik, hormonal özellikler, patoloji, cerrahi tedavi. Türk Nöroşirurji Dergisi 2006; 16: Cao Z, Lv J, Ding Z, Du H. Pathological laughter in a patient with Rathke cleft cyst. J Clin Neurosci 2008; 15: Ceylan S, Koc K, Anik I. Extended endoscopic approaches for the midline skull base lesions. Neurosurg Rev 2009; 32(3): Chuang CC, Chen YL, Jung SM, Pai PC. A giant retroclival Rathke's cleft cyst. J Clin Neurosci 2010; 17: Ciric I, Farhat H. Diagnosis and treatment of Rathke cleft cysts: a perspective. World Neurosurg 2012; 78: Donowan JL, Nesbit GM. Distinction of masses involving the sella and suprasellar space: specificity of imaging features. AJR Am J Roentgenol 1996; 167: Hama S, Arita K, Tominaga A, Yoshikawa M, Eguchi K, Sumida M, Inai K, Nishisaka T, Kurisu K. Symptomatic Rathke's cleft cyst coexisting with central diabetes insipidus and hypophysitis: case report. Endocr J 1999; 46: Jahangiri A, Potts M, Kunwar S, Blevins L, El-Sayed IH, Aghi MK. Extended endoscopic endonasal approach for suprasellar Rathke's cleft cysts. J Clin Neurosci 2013 Dec 2. doi: /j.jocn [Epub ahead of print] 9. Kim E. Symptomatic Rathke cleft cyst: clinical features and surgical outcomes. World Neurosurg 2012; 78: Post KD. Rathke's cleft cysts: unanswered questions. World Neurosurg 2012; 78: Raper DM, Michael Beser M. Clinical features, management and recurrence of symptomatic Rathke cleft cyst. Clinical Study.J Clinl Neursci 2009; 16: Sade B,Albrecht S, Assimakopoulos P, Vézina JL, Mohr G. Management of Rathke cleft cysts. Surg Neurol 2005; 63: Shin JL, Asa SL, Woodhouse LJ, Smyth HS, Ezzat S. Cystic lesions of the pituitary:clinicopathological features distinguishing craniopharyngioma, Rathke cleft cyst and arachnoid cyst. J Clin Endocrinol Metab 1999; 84: Zhong W, You C, Jiang S, Huang S, Chen H, Liu J, Zhou P, Liu Y, Cai B. Symptomatic Rathke cleft cyst. J Clin Neurosci 2012; 19: