We report a rare case of symptomatic Rathke's cleft cyst with thick calcified wall. Brain CT scans revealed a large cystic mass with round thick calcified wall. In this case, we selected the pterional approach instead of transsphenoidal approach due to the possibility of cystic craniopharyngioma. Histopathologically, it was calcified Rathke's cleft cyst with focal epithelial metaplasia. This case illustrates that calcification of the suprasellar cyst does not always suggest craniopharyngioma and the calcification pattern of Rathke's cleft cyst is different from that of the craniopharyngioma.
Brain ; Central Nervous System Cysts ; Craniopharyngioma ; Metaplasia

Symptomatic Rathke's cleft cysts may be confined to the sella turcica or extended upward into suprasellar area. Like the more common cystic tumor of this region, the craniopharyngioma, they produce symptoms by compression of the surrounding structures. We report a rare case of a symptomatic Rathke's cleft cyst. The importance of the intraoperative differentiation between the cyst and a craniopharyngioma is discussed.
Central Nervous System Cysts ; Craniopharyngioma ; Sella Turcica

A Rathke's cleft cyst (RCC) is a benign pituitary cyst derived from the remnant of Rathke's pouch, and usually presents as an intrasellar lesion with varying degrees of suprasellar extension. However, to date, a description of a primary prepontine RCC with no intrasellar component has not been reported. The author describes an exceptional case of a symptomatic RCC located behind the sella turcica in a 41-year-old woman who presented with severe headache. The author also provides an embryological hypothesis of the development of an ectopic RCC, with a special emphasis on radiologic characteristics.
Adult ; Central Nervous System Cysts ; Female ; Headache ; Humans ; Sella Turcica

Intracranial germinomas are malignant neoplasms arising from remnants of primitive germ cells that have failed to migrate to the genital crest during embryonic life. They are usually solid tumors, but focal cysts may occasionally be found within them and are thought to represent proteinaceous fluid. Cystic degeneration of the whole tumor, however, that is caused by intratumoral hemorrhage, has not yet been reported. We present a case of cystic degeneration of solid suprasellar germinoma. During surgery, cyst content was shown to be liquefied hematoma, and bleeding of the tumor had occurred. The exact time of intratumoral hemorrhage in the presented case is unknown, but since there had been no episode of apoplexy, we believe that degeneration occurred chronically. The patient's postoperative course was uneventful. On the basis of our findings we suggest that in diagnosing suprasellar cystic tumors, the possibility of germinomas must be taken into consideration and that spontaneous cystic degeneration of suprasellar germinoma can occur as a result of tumor bleeding.
Central Nervous System Cysts ; Germ Cells ; Germinoma* ; Hematoma ; Hemorrhage ; Stroke

OBJECTIVE: To assess the value of chromosomal microarray analysis (CMA) for the diagnosis of fetuses with anomalies of the central nervous system (CNS) and summarize the outcome of the pregnancies and follow-up. METHODS: A total of 636 fetuses from June 2014 to December 2020 who were referred to the Prenatal Diagnosis Center of Nanjing Drum Tower Hospital due to abnormal CNS prompted by ultrasound were selected as the research subjects. Based on the ultrasound findings, the fetuses were divided into ventricular dilatation group (n = 441), choroid plexus cyst group (n = 41), enlarged posterior fossa group (n = 42), holoprosencephaly group (n = 15), corpus callosum hypoplasia group (n = 22), and other anomaly group (n = 75). Meanwhile, they were also divided into isolated (n = 504) and non-isolated (n = 132) groups based on the presence of additional abnormalities. Prenatal samples (amniotic fluid/chorionic villi/umbilical cord blood) or abortus tissue were collected for the extraction of genomic DNA and CMA assay. Outcome of the pregnancies and postnatal follow-up were summarized and subjected to statistical analysis. RESULTS: In total 636 fetuses with CNS anomalies (including 89 abortus tissues) were included, and 547 cases were followed up. The overall detection rate of CMA was 11.48% (73/636). The detection rates for the holoprosencephaly group, ACC group, choroid plexus cyst group, enlarged posterior fossa group, ventricular dilatation group and other anomaly group were 80% (12/15), 31.82% (7/22), 19.51% (8/41), 14.29% (6/42), 7.48% (33/441) and 9.33% (7/75), respectively. Compared with the isolated CNS anomaly group, the detection rate for the non-isolated CNS anomaly group was significantly higher (6.35% vs. 31.06%) (32/504 vs. 41/132) (χ² = 62.867, P < 0.001). Follow up showed that, for 52 fetuses with abnormal CMA results, 51 couples have opted induced labor, whilst 1 was delivered at full term with normal growth and development. Of the 434 fetuses with normal CMA results, 377 were delivered at full term (6 had developmental delay), and 57 couples had opted induced labor. The rate of adverse pregnancy outcome for non-isolated CNS abnormal fetuses was significantly higher than that of isolated CNS abnormal fetuses (26.56% vs. 10.54%) (17/64 vs. 39/370) (χ² = 12.463, P < 0.001). CONCLUSION Fetuses with CNS anomaly should be tested with CMA to determine the genetic cause. Most fetuses with negative CMA result have a good prognosis, but there is still a possibility for a abnormal neurological phenotype. Fetuses with CNS abnormalities in conjunct with other structural abnormalities are at increased risk for adverse pregnancy outcomes.
Female ; Pregnancy ; Humans ; Holoprosencephaly ; Prenatal Diagnosis/methods* ; Central Nervous System ; Fetus/abnormalities* ; Nervous System Malformations/genetics* ; Microarray Analysis ; Central Nervous System Diseases ; Cysts ; Chromosome Aberrations ; Ultrasonography, Prenatal/methods*

Fetal abnormalities in central nervous system are now recognized more often with the increasing use of high-resolution prenatal sonography. Arachnoid cysts, because of their compression of adjacent brain tissue, may require neurosurgical intervention but otherwise they have a good long- term prognosis. The differential diagnosis of intracranial lesions is important to allow accurate counselling and also to optimize neonatal management. We report the two cases of fetal arachnoid cyst diagnosed with prenatal ultrasonography
Arachnoid Cysts ; Arachnoid* ; Brain ; Central Nervous System ; Diagnosis, Differential ; Prenatal Diagnosis ; Prognosis ; Ultrasonography ; Ultrasonography, Prenatal*

Rathke's cleft cysts are believed to be derived from remnants of Rathke's pouch, a dorsal invagination of the stomodeum. Although these cysts are characteristically small, asymptomatic and intrasellar in location, they occasionally provoke symtoms with enlargement to compress surrounding structures. It is characteristically lined by stratified squamous epithelium with keratinization on a layer of connective tissue. The cells of the anterior pituitary lobe, from which pituitary adenomas develop, are also formed by the proliferation of the anterior wall of Rathke's pouch. Thus, Rathke's cleft cyst and pituitary adenomas are considered to have a common ancestry. We report a rare case in which the preoperative diagnosis was pituitary adenoma, but the pathologic diagnosis was a combination of a Rathke's cleft cyst and a coincidental pituitary adenoma.
Adenoma* ; Central Nervous System Cysts ; Connective Tissue ; Diagnosis ; Epithelium ; Pituitary Neoplasms*

Adult ; Central Nervous System Cysts ; diagnosis ; surgery ; Craniopharyngioma ; diagnosis ; surgery ; Female ; Humans ; Stroke ; diagnosis ; surgery

OBJECTIVE: The indications and optimal surgical treatments for intracranial cysts are controversial. In the present study, we describe long-term clinical and neuroimaging results of surgically treated intracranial cysts in children. The goal of this study is to contribute to the discussion of the debate. METHODS: This study included 110 pediatric patients that underwent surgeries to treat intracranial cysts. Endoscopic cyst fenestrations were performed in 71 cases, while craniotomies and cyst excisions (with or without fenestrations) were performed in 30 patients. Cystoperitoneal shunts were necessary for nine patients. Long-term results were retrospectively assessed with medical and neuroimaging records. RESULTS: Clinical and radiological improvement was reported in 87.3% and 92.8% of cases, respectively, after endoscopic neurosurgery, and in 93.3% and 100% using open microsurgery whereas 88.9% and 85.7% after shunt operation. There were no statistical differences in clinical outcomes (p=0.710) or volume reductions (p=0.177) among the different surgeries. There were no mortalities or permanent morbidities, but complications such as shunt malfunctions, infections, and subdural hematomas were observed in 56% of the patients that had shunt operations. A total of 13 patients (11.8%) underwent additional surgeries due to recurrences or treatment failures. The type of surgery performed did not influence the recurrence rate (p=0.662) or the failure rate (p=0.247). CONCLUSION: Endoscopic neurosurgeries are less invasive than microsurgeries and are at least as effective as open surgeries. Thus, given the advantages and complications of these surgical techniques, we suggest that endoscopic fenestration should be the first treatment attempted in children with intracranial cysts.
Arachnoid Cysts ; Central Nervous System Cysts ; Child ; Craniotomy ; Hematoma, Subdural ; Humans ; Microsurgery ; Neuroendoscopy ; Neuroimaging ; Neurosurgery ; Recurrence ; Retrospective Studies ; Treatment Failure

Rathke cleft cysts (RCCs) are benign epithelial lesions of the sellar and suprasellar region. Most RCCs remain clinically silent throughout an individual's life. Symptomatic patients with RCCs manifest headaches, endocrinopathies, and visual disturbances secondary to parasellar extension. Rarely, RCCs can present in a manner similar to pituitary tumor apoplexy. One such case is reported herein. This 53-year-old man presented with acute headache, vomiting, and partial hypopituitarism. Surgical drainage of the cyst contents via a transnasal route confirmed the diagnosis of RCC and pituitary infarction.
Central Nervous System Cysts ; Diagnosis ; Drainage ; Headache ; Humans ; Hypopituitarism ; Infarction ; Middle Aged ; Pituitary Apoplexy ; Pituitary Neoplasms ; Stroke* ; Vomiting