Clinical Neurology and Neurosurgery 121 (2014) 27–29
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
A giant Suprasellar Rathke cleft cyst with psychiatric manifestations: Case report Chih-Hsiang Liao a , Chun-Fu Lin a,c , Kai-Lin Huang b,c , Min-Hsiung Chen a,c , Sanford P.C. Hsu a,c,∗ , Yang-Hsin Shih a,c a
Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan, ROC Department of Psychiatry, Taipei Veterans General Hospital, Taipei, Taiwan, ROC c National Yang Ming University School of Medicine, Taipei, Taiwan, ROC b
a r t i c l e
i n f o
Article history: Received 5 November 2013 Received in revised form 29 January 2014 Accepted 9 March 2014 Available online 19 March 2014 Keywords: Craniotomy Hypocortisolism Mood disorder Rathke cleft cyst Transsphenoidal surgery
1. Introduction Rathke cleft cyst (RCC) is a benign, cystic remnant of the craniopharyngeal duct, typically located in the sellar region [1]. Purely suprasellar RCC situated above a normal sella is rare [2]. The common clinical presentations of symptomatic RCCs include headache, visual impairment, and endocrine disturbance. Descriptions of psychiatric manifestations associated with RCCs were exceedingly rare [2–8], and their potential neuropsychiatric significance may be underestimated. In this report, the authors presented the case of a giant suprasellar RCC with psychiatric manifestations which resolved spontaneously after surgical intervention. 2. Case report A 39-year-old married woman (155 cm, 44.7 kg, BMI: 18.6 kg/m2 , non-smoking) has two children and manages a hairdressing salon. She was robust and cheerful (premorbid
∗ Corresponding author at: Department of Neurosurgery, The Neurological Institute, Taipei Veterans General Hospital, No. 201, Section 2, Shih-Pai Road, Taipei 112, Taiwan, ROC. Tel.: +886 2 2875 7587; fax: +886 2 2822 5072. E-mail addresses: [email protected], [email protected], [email protected] (S.P.C. Hsu). http://dx.doi.org/10.1016/j.clineuro.2014.03.011 0303-8467/© 2014 Elsevier B.V. All rights reserved.
personality: positive, outgoing). She had neither systemic diseases nor the history of alcoholism or substance use. There were no past psychiatric history and psychiatric family history. No regular medications were taken. About 4 months before presentation, she started to have depressed moods. She was demotivated at her work, showed apathy to her husband and the family, and quit exercising. She felt tired easily, had headaches, insomnia, and suicidal ideation. Due to poor appetite, she lost 5 kg of body weight in a 2-months period. Visual blurring was also noticed, but there were no visual and auditory hallucinations. She was brought to the psychiatry clinic for help. The thyroid hormone test did not reveal hypothyroidism. According to the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision, the initial impression included major depressive disorder and affective psychosis. The score of Beck Depression Inventory-II (BDI-II) was 21 (moderate depression). The Wechsler Adult Intelligence Scale-III (WAIS-III) showed VIQ = 83, PIQ = 67, and FIQ = 75. The Millon Clinical Multiaxial Inventory-III (MCMI-III) showed histrionic personality. Deanxit (Flupenthixol 0.5 mg/Melitracen 10 mg, twice daily) was prescribed, and the reeducative individual psychotherapy was arranged. The treatment with Deanxit had continued for 2 months before her presentation to our neurosurgical department. She responded well to Deanxit in the first month of treatment. However, her headaches persisted, which woke her up at nights. The headaches
28
C.-H. Liao et al. / Clinical Neurology and Neurosurgery 121 (2014) 27–29
Fig. 1. Pre- and post-operative brain MRI of a giant suprasellar Rathke cleft cyst. The sagittal/coronal T1-weighted (A and B) and axial T2-weighted (C) images revealed a lobulated cystic lesion at suprasellar region with retroclival extension. (C). One year after the operation, resolution of the abnormalities was shown on the sagittal/coronal T1-weighted (D and E) and axial T2-weighted (F) images.
could be relieved partially by acetaminophen and non-steroid antiinflammatory drugs. She went to the neurology clinic for help. She mentioned about poor memory, urinary incontinence, and visual blurring. The mini-mental state examination revealed cognitive impairment (poor memory, abnormal abstraction, and impaired calculation). The physical examinations showed anisocoria with decreased light reflex and temporal anopsia of the left eye, hyperreflexia, positive Babinski sign, and unsteady gait. The brain CT was arranged initially by the neurologist in the clinic, but the patient missed it for some personal reason. About one month after her visit to the neurology clinic, the patient was sent to our emergency department because she had an episode of syncope with head injury. For evaluation of the head injury, the brain CT incidentally revealed a large tumor in the suprasellar area, leading to obstructive hydrocephalus and brainstem compression. The brain MRI showed a suprasellar cystic lesion, about 5.7 cm × 3.7 cm × 4.8 cm in size, causing severe mass effect (Fig. 1A–C). The lesion contained mixed high signals on T1- and T2-weighted series. No enhancement of the tumor was found after contrast medium injection. The brain MRI demonstrated distinct interfaces between the lesion and the pituitary gland. The differential diagnoses included RCC, epidermoid cyst, and enteric cyst. The pre-operative examinations also revealed hypocortisolemia (0.66 g/dL, blood sample drawn in the morning) and compressive optic neuropathy. Other laboratory findings, including the serum levels of electrolytes, pituitary and thryoird hormones, were within normal limits. The patient underwent the left-sided pretemperoal approach for tumor removal (see Fig. 2 and supplementary video). Total excision of the tumor was achieved. The histopathological study revealed a cystic lesion characterized by the fibrotic wall with ciliated cuboidal to low columnar lining epithelial cells and proteinaceous material in the cystic lumen, which was compatible with the diagnosis of RCC. Her visual field and acuity improved immediately after the operation. There was no post-operative diabetes insipidus. She was discharged on post-operative day 3. In regular follow-ups, the patient’s psychiatric manifestations resolved spontaneously, and
the serum cortisol level returned to normal. One year after the operation, no residual tumor or recurrence was noticed. (Fig. 1D–F) The patient maintained her active life without any medications. In the latest follow-up, one year and five months after the operation, the score of the BDI-II was zero (no depression) and the results of the personality assessment were similar to that of the pre-operative exams. Supplementary video related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.clineuro.2014.03.011. 3. Discussion RCC is a benign cystic lesion, usually arising at the interface between the anterior and posterior lobes of the pituitary. RCC with sufficient size usually cause visual or endocrine disorders by compressing the visual apparatus, pituitary gland, or hypothalamus. Hypocortisolism was common in patients with symptomatic RCCs [2–8]. Impairment of the hypothalamic–pituitary–adrenal axis causing hypocortisolism may result in mood disorders [9–11], but descriptions of psychiatric manifestations associated with RCCs were exceedingly rare in the literature [2–8]. In this report, the patient’s psychiatric manifestations might be associated with hypocortisolism. She responded well to antidepressants, which could be explained by that antidepressants were associated with normalization of altered glucocorticoid-mediated inhibitory feedback [9]. Nevertheless, the symptoms caused by the mass effect of RCC warranted surgical intervention. Depression is often associated with somatic symptoms [12]. However, if the patient’s mood improves after treatment, but somatic symptoms remain or even worsen, clinicians should be aware of organic mood disorder. Symptomatic RCCs were generally managed by transsphenoidal surgery (TSS) [2–8]. Compared with sellar-based RCCs, RCCs with a suprasellar component were associated with higher rates of incomplete excision, recurrence, and postoperative endocrine morbidity, and their symptoms less frequently improved with TSS [2]. However, aggressive resection of the capsule of RCC was associated with
C.-H. Liao et al. / Clinical Neurology and Neurosurgery 121 (2014) 27–29
29
Fig. 2. Intraoperative images of the left-sided pretemporal approach. (A) The bulging tumor stretched the left optic nerve before decompression. (B) The tumor was deflated through the space between the left optic nerve and the left ICA. The tumor content was yellowish milk-like liquid. (C) After partial decompression, the neurovascular structures surrounding the tumor were visualized. (D) During the dissection around the brainstem, the right oculomotor nerve was exposed. ACA, anterior cerebral artery; CNII, optic nerve; CNIII, oculomotor nerve; ICA, internal carotid artery; PCP, posterior clinoid process; R’t, right.
iatrogenic diabetes insipidus in TSS [3,4]. In this case, the authors chose an open surgery due to the location (purely suprasellar) and the tumor size (giant). The authors removed the tumor completely, preserved the pituitary gland/stalk, and kept the surrounding neurovascular structures intact. The authors did not favor simple cyst content removal and wall marsupialization in an open surgery [13], because the inflammation from RCC might spread to adjacent pituitary tissues causing irreversible damages [14], and the high recurrence rate of RCC after surgical treatment was observed, especially after a partial removal [4]. 4. Conclusion RCC can manifest psychiatric symptoms which may resolve spontaneously after the surgical intervention. Open surgery is a viable option for a giant suprasellar RCC with critical neurovascular involvement. Instead of simple cyst content removal and marsupialization of the cyst wall, gross total tumor removal is suggested in an open surgery. Funding/COI statement Nil. References [1] Kanter AS, Sansur CA, Jane Jr JA, Laws Jr ER. Rathke’s cleft cysts. Front Horm Res 2006;34:127–57. [2] Potts MB, Jahangiri A, Lamborn KR, Blevins LS, Kunwar S, Aghi MK. Suprasellar Rathke cleft cysts: clinical presentation and treatment outcomes. Neurosurgery 2011;69:1058–68.
[3] Aho CJ, Liu C, Zelman V, Couldwell WT, Weiss MH. Surgical outcomes in 118 patients with Rathke cleft cysts. J Neurosurg 2005;102:189– 93. [4] Kim JE, Kim JH, Kim OL, Paek SH, Kim DG, Chi JG, et al. Surgical treatment of symptomatic Rathke cleft cysts: clinical features and results with special attention to recurrence. J Neurosurg 2004;100:33–40. [5] Higgins DM, Van Gompel JJ, Nippoldt TB, Meyer FB. Symptomatic Rathke cleft cysts: extent of resection and surgical complications. Neurosurg Focus 2011;31:E2. [6] Lillehei KO, Widdel L, Astete CA, Wierman ME, Kleinschmidt-DeMasters BK, Kerr JM. Transsphenoidal resection of 82 Rathke cleft cysts: limited value of alcohol cauterization in reducing recurrence rates. J Neurosurg 2011;114:310–7. [7] Wait SD, Garrett MP, Little AS, Killory BD, White WL. Endocrinopathy, vision, headache, and recurrence after transsphenoidal surgery for Rathke cleft cysts. Neurosurgery 2010;67:837–43, discussion 843. [8] Benveniste RJ, King WA, Walsh J, Lee JS, Naidich TP, Post KD. Surgery for Rathke cleft cysts: technical considerations and outcomes. J Neurosurg 2004;101:577–84. [9] Raison CL, Miller AH. When not enough is too much: the role of insufficient glucocorticoid signaling in the pathophysiology of stress-related disorders. Am J Psychiatry 2003;160:1554–65. [10] Thomsen AF, Kvist TK, Andersen PK, Kessing LV. The risk of affective disorders in patients with adrenocortical insufficiency. Psychoneuroendocrinology 2006;31:614–22. [11] Hori H, Teraishi T, Sasayama D, Hattori K, Hashikura M, Higuchi T, et al. Relationship of temperament and character with cortisol reactivity to the combined dexamethasone/CRH test in depressed outpatients. J Affect Disord 2013;147:128–36. [12] Haug TT, Mykletun A, Dahl AA. The association between anxiety, depression, and somatic symptoms in a large population: the HUNT-II study. Psychosom Med 2004;66:845–51. [13] Chuang CC, Chen YL, Jung SM, Pai PC. A giant retroclival Rathke’s cleft cyst. J Clin Neurosci: Off J Neurosurg Soc Australas 2010;17:1189– 91. [14] Hama S, Arita K, Nishisaka T, Fukuhara T, Tominaga A, Sugiyama K, et al. Changes in the epithelium of Rathke cleft cyst associated with inflammation. J Neurosurg 2002;96:209–16.
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
A giant Suprasellar Rathke cleft cyst with psychiatric manifestations: Case report Chih-Hsiang Liao a , Chun-Fu Lin a,c , Kai-Lin Huang b,c , Min-Hsiung Chen a,c , Sanford P.C. Hsu a,c,∗ , Yang-Hsin Shih a,c a
Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan, ROC Department of Psychiatry, Taipei Veterans General Hospital, Taipei, Taiwan, ROC c National Yang Ming University School of Medicine, Taipei, Taiwan, ROC b
a r t i c l e
i n f o
Article history: Received 5 November 2013 Received in revised form 29 January 2014 Accepted 9 March 2014 Available online 19 March 2014 Keywords: Craniotomy Hypocortisolism Mood disorder Rathke cleft cyst Transsphenoidal surgery
1. Introduction Rathke cleft cyst (RCC) is a benign, cystic remnant of the craniopharyngeal duct, typically located in the sellar region [1]. Purely suprasellar RCC situated above a normal sella is rare [2]. The common clinical presentations of symptomatic RCCs include headache, visual impairment, and endocrine disturbance. Descriptions of psychiatric manifestations associated with RCCs were exceedingly rare [2–8], and their potential neuropsychiatric significance may be underestimated. In this report, the authors presented the case of a giant suprasellar RCC with psychiatric manifestations which resolved spontaneously after surgical intervention. 2. Case report A 39-year-old married woman (155 cm, 44.7 kg, BMI: 18.6 kg/m2 , non-smoking) has two children and manages a hairdressing salon. She was robust and cheerful (premorbid
∗ Corresponding author at: Department of Neurosurgery, The Neurological Institute, Taipei Veterans General Hospital, No. 201, Section 2, Shih-Pai Road, Taipei 112, Taiwan, ROC. Tel.: +886 2 2875 7587; fax: +886 2 2822 5072. E-mail addresses: [email protected], [email protected], [email protected] (S.P.C. Hsu). http://dx.doi.org/10.1016/j.clineuro.2014.03.011 0303-8467/© 2014 Elsevier B.V. All rights reserved.
personality: positive, outgoing). She had neither systemic diseases nor the history of alcoholism or substance use. There were no past psychiatric history and psychiatric family history. No regular medications were taken. About 4 months before presentation, she started to have depressed moods. She was demotivated at her work, showed apathy to her husband and the family, and quit exercising. She felt tired easily, had headaches, insomnia, and suicidal ideation. Due to poor appetite, she lost 5 kg of body weight in a 2-months period. Visual blurring was also noticed, but there were no visual and auditory hallucinations. She was brought to the psychiatry clinic for help. The thyroid hormone test did not reveal hypothyroidism. According to the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision, the initial impression included major depressive disorder and affective psychosis. The score of Beck Depression Inventory-II (BDI-II) was 21 (moderate depression). The Wechsler Adult Intelligence Scale-III (WAIS-III) showed VIQ = 83, PIQ = 67, and FIQ = 75. The Millon Clinical Multiaxial Inventory-III (MCMI-III) showed histrionic personality. Deanxit (Flupenthixol 0.5 mg/Melitracen 10 mg, twice daily) was prescribed, and the reeducative individual psychotherapy was arranged. The treatment with Deanxit had continued for 2 months before her presentation to our neurosurgical department. She responded well to Deanxit in the first month of treatment. However, her headaches persisted, which woke her up at nights. The headaches
28
C.-H. Liao et al. / Clinical Neurology and Neurosurgery 121 (2014) 27–29
Fig. 1. Pre- and post-operative brain MRI of a giant suprasellar Rathke cleft cyst. The sagittal/coronal T1-weighted (A and B) and axial T2-weighted (C) images revealed a lobulated cystic lesion at suprasellar region with retroclival extension. (C). One year after the operation, resolution of the abnormalities was shown on the sagittal/coronal T1-weighted (D and E) and axial T2-weighted (F) images.
could be relieved partially by acetaminophen and non-steroid antiinflammatory drugs. She went to the neurology clinic for help. She mentioned about poor memory, urinary incontinence, and visual blurring. The mini-mental state examination revealed cognitive impairment (poor memory, abnormal abstraction, and impaired calculation). The physical examinations showed anisocoria with decreased light reflex and temporal anopsia of the left eye, hyperreflexia, positive Babinski sign, and unsteady gait. The brain CT was arranged initially by the neurologist in the clinic, but the patient missed it for some personal reason. About one month after her visit to the neurology clinic, the patient was sent to our emergency department because she had an episode of syncope with head injury. For evaluation of the head injury, the brain CT incidentally revealed a large tumor in the suprasellar area, leading to obstructive hydrocephalus and brainstem compression. The brain MRI showed a suprasellar cystic lesion, about 5.7 cm × 3.7 cm × 4.8 cm in size, causing severe mass effect (Fig. 1A–C). The lesion contained mixed high signals on T1- and T2-weighted series. No enhancement of the tumor was found after contrast medium injection. The brain MRI demonstrated distinct interfaces between the lesion and the pituitary gland. The differential diagnoses included RCC, epidermoid cyst, and enteric cyst. The pre-operative examinations also revealed hypocortisolemia (0.66 g/dL, blood sample drawn in the morning) and compressive optic neuropathy. Other laboratory findings, including the serum levels of electrolytes, pituitary and thryoird hormones, were within normal limits. The patient underwent the left-sided pretemperoal approach for tumor removal (see Fig. 2 and supplementary video). Total excision of the tumor was achieved. The histopathological study revealed a cystic lesion characterized by the fibrotic wall with ciliated cuboidal to low columnar lining epithelial cells and proteinaceous material in the cystic lumen, which was compatible with the diagnosis of RCC. Her visual field and acuity improved immediately after the operation. There was no post-operative diabetes insipidus. She was discharged on post-operative day 3. In regular follow-ups, the patient’s psychiatric manifestations resolved spontaneously, and
the serum cortisol level returned to normal. One year after the operation, no residual tumor or recurrence was noticed. (Fig. 1D–F) The patient maintained her active life without any medications. In the latest follow-up, one year and five months after the operation, the score of the BDI-II was zero (no depression) and the results of the personality assessment were similar to that of the pre-operative exams. Supplementary video related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.clineuro.2014.03.011. 3. Discussion RCC is a benign cystic lesion, usually arising at the interface between the anterior and posterior lobes of the pituitary. RCC with sufficient size usually cause visual or endocrine disorders by compressing the visual apparatus, pituitary gland, or hypothalamus. Hypocortisolism was common in patients with symptomatic RCCs [2–8]. Impairment of the hypothalamic–pituitary–adrenal axis causing hypocortisolism may result in mood disorders [9–11], but descriptions of psychiatric manifestations associated with RCCs were exceedingly rare in the literature [2–8]. In this report, the patient’s psychiatric manifestations might be associated with hypocortisolism. She responded well to antidepressants, which could be explained by that antidepressants were associated with normalization of altered glucocorticoid-mediated inhibitory feedback [9]. Nevertheless, the symptoms caused by the mass effect of RCC warranted surgical intervention. Depression is often associated with somatic symptoms [12]. However, if the patient’s mood improves after treatment, but somatic symptoms remain or even worsen, clinicians should be aware of organic mood disorder. Symptomatic RCCs were generally managed by transsphenoidal surgery (TSS) [2–8]. Compared with sellar-based RCCs, RCCs with a suprasellar component were associated with higher rates of incomplete excision, recurrence, and postoperative endocrine morbidity, and their symptoms less frequently improved with TSS [2]. However, aggressive resection of the capsule of RCC was associated with
C.-H. Liao et al. / Clinical Neurology and Neurosurgery 121 (2014) 27–29
29
Fig. 2. Intraoperative images of the left-sided pretemporal approach. (A) The bulging tumor stretched the left optic nerve before decompression. (B) The tumor was deflated through the space between the left optic nerve and the left ICA. The tumor content was yellowish milk-like liquid. (C) After partial decompression, the neurovascular structures surrounding the tumor were visualized. (D) During the dissection around the brainstem, the right oculomotor nerve was exposed. ACA, anterior cerebral artery; CNII, optic nerve; CNIII, oculomotor nerve; ICA, internal carotid artery; PCP, posterior clinoid process; R’t, right.
iatrogenic diabetes insipidus in TSS [3,4]. In this case, the authors chose an open surgery due to the location (purely suprasellar) and the tumor size (giant). The authors removed the tumor completely, preserved the pituitary gland/stalk, and kept the surrounding neurovascular structures intact. The authors did not favor simple cyst content removal and wall marsupialization in an open surgery [13], because the inflammation from RCC might spread to adjacent pituitary tissues causing irreversible damages [14], and the high recurrence rate of RCC after surgical treatment was observed, especially after a partial removal [4]. 4. Conclusion RCC can manifest psychiatric symptoms which may resolve spontaneously after the surgical intervention. Open surgery is a viable option for a giant suprasellar RCC with critical neurovascular involvement. Instead of simple cyst content removal and marsupialization of the cyst wall, gross total tumor removal is suggested in an open surgery. Funding/COI statement Nil. References [1] Kanter AS, Sansur CA, Jane Jr JA, Laws Jr ER. Rathke’s cleft cysts. Front Horm Res 2006;34:127–57. [2] Potts MB, Jahangiri A, Lamborn KR, Blevins LS, Kunwar S, Aghi MK. Suprasellar Rathke cleft cysts: clinical presentation and treatment outcomes. Neurosurgery 2011;69:1058–68.
[3] Aho CJ, Liu C, Zelman V, Couldwell WT, Weiss MH. Surgical outcomes in 118 patients with Rathke cleft cysts. J Neurosurg 2005;102:189– 93. [4] Kim JE, Kim JH, Kim OL, Paek SH, Kim DG, Chi JG, et al. Surgical treatment of symptomatic Rathke cleft cysts: clinical features and results with special attention to recurrence. J Neurosurg 2004;100:33–40. [5] Higgins DM, Van Gompel JJ, Nippoldt TB, Meyer FB. Symptomatic Rathke cleft cysts: extent of resection and surgical complications. Neurosurg Focus 2011;31:E2. [6] Lillehei KO, Widdel L, Astete CA, Wierman ME, Kleinschmidt-DeMasters BK, Kerr JM. Transsphenoidal resection of 82 Rathke cleft cysts: limited value of alcohol cauterization in reducing recurrence rates. J Neurosurg 2011;114:310–7. [7] Wait SD, Garrett MP, Little AS, Killory BD, White WL. Endocrinopathy, vision, headache, and recurrence after transsphenoidal surgery for Rathke cleft cysts. Neurosurgery 2010;67:837–43, discussion 843. [8] Benveniste RJ, King WA, Walsh J, Lee JS, Naidich TP, Post KD. Surgery for Rathke cleft cysts: technical considerations and outcomes. J Neurosurg 2004;101:577–84. [9] Raison CL, Miller AH. When not enough is too much: the role of insufficient glucocorticoid signaling in the pathophysiology of stress-related disorders. Am J Psychiatry 2003;160:1554–65. [10] Thomsen AF, Kvist TK, Andersen PK, Kessing LV. The risk of affective disorders in patients with adrenocortical insufficiency. Psychoneuroendocrinology 2006;31:614–22. [11] Hori H, Teraishi T, Sasayama D, Hattori K, Hashikura M, Higuchi T, et al. Relationship of temperament and character with cortisol reactivity to the combined dexamethasone/CRH test in depressed outpatients. J Affect Disord 2013;147:128–36. [12] Haug TT, Mykletun A, Dahl AA. The association between anxiety, depression, and somatic symptoms in a large population: the HUNT-II study. Psychosom Med 2004;66:845–51. [13] Chuang CC, Chen YL, Jung SM, Pai PC. A giant retroclival Rathke’s cleft cyst. J Clin Neurosci: Off J Neurosurg Soc Australas 2010;17:1189– 91. [14] Hama S, Arita K, Nishisaka T, Fukuhara T, Tominaga A, Sugiyama K, et al. Changes in the epithelium of Rathke cleft cyst associated with inflammation. J Neurosurg 2002;96:209–16.
