A study on clinical outcomes of Rathke's cleft cyst in patients managed conservatively.

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.

Surgery versus conservative care for Rathke's cleft cyst.

BACKGROUND: Rathke's cleft cysts are benign cystic lesions of the sellar region, which may cause headache, pituitary deficiencies and visual disturbances from mass effect. Their management is not standardized yet. This study is about establishing a consensus for medical care of RCC. MATERIAL AND METHODS: We performed a retrospective observational study of all patients that were diagnosed or followed for RCC between 2008 and 2018 (11 years), in the neurosurgical and the adult endocrine departments of our institution. The study's average time length of follow-up is 72.9 months (from 2 to 385 months). RESULTS: The 57 included patients were divided into 2 groups: group A, which included 39 patients that were conservatively managed and group B, which included 18 surgically treated patients. Group A showed either an improvement or a spontaneous resolution of headaches in 56.1% of the cases (P<0.01); a resolution of hyperprolactinemia in 70% of the cases (P=0.21); and of hypogonadism, ACTH deficiency, growth hormone deficiency in 100% of the cases. There was no spontaneous improvement of visual disturbances (P<0.01) or diabetes insipidus (P=0.29) during follow-up. Regarding group B, surgery allowed improvement or complete resolution of headaches in 60% of the cases; visual troubles in 100% of the cases (P<0.01); and hyperprolactinemia in 100% of the cases. Pituitary deficiencies were not improved by surgery. CONCLUSIONS: This study offers guidance in decision-making regarding the management of RCC patients. Surgery is particularly suitable for treating visual disturbances caused by RCC. Regular follow-up is more appropriate than surgery concerning headaches, hyperprolactinemia, endocrine disruptions and diabetes insipidus.

Outcome of nonneoplastic pituitary cysts during conservative monitoring and after surgery-a SwissPit study.

BACKGROUND: Nonneoplastic cysts of the pituitary are common incidental findings; however, best management remains controversial as they are often asymptomatic but eventually may cause symptoms. The aims of this study are to describe the course of conservative and surgical approaches, to assess timing and results of surgery, and to identify predictors for growth. METHODS: This retrospective study reviewed medical records from the Swiss Pituitary registry. Fifty patients (68% females; median 44 years old) fulfilled the criteria for inclusion. Three cohorts were defined: a conservative group (n = 28), a group who initially needed surgery (n = 18), and a group who had surgery during follow-up (n = 4). Transsphenoidal cyst evacuation was used in 95%; 68% had intraoperative MRI. All patients had standardized neuroradiological, endocrinological, and ophthalmological follow-up (mean 44 (7-151) months). RESULTS: Conservative follow-up of 30 (6-120) months showed cyst growth in 16% (4%/year) and spontaneous shrinkage in 19% (8%/year). Cyst-volumes changed - 0.95 to 1.45 cm3 per year. The probability of needing surgery was 5% per year. Larger cysts (> 1.4 cm3) and T1-hypo-/T2-hyper cyst contents were associated with higher probability for growth. Postoperatively, no remnant was seen in 82% during a follow-up of 53 months. Visual field deficiencies improved in 83%. Hypopituitarism recovered in up to 88%. But for a smaller complication rate, the outcome was not influenced by the use of the intraoperative MRI. CONCLUSIONS: Asymptomatic nonneoplastic pituitary cysts may be monitored; many lesions may shrink with time. Larger or T1-hypo-/T2-hyper cysts have higher growth rates. If indicated, surgery for nonneoplastic sellar cysts is a safe and efficient.

Prevalence and characteristics of sellar masses in the city of Al Ain, United Arab Emirates: 2010 to 2016.

BACKGROUND: The prevalence of sellar masses (SMs) is reported in Europe and North America but only limited data are available from the Middle East and North Africa (MENA) region. OBJECTIVES: Assess the prevalence and characteristics of SMs in Al Ain city, United Arab Emirates. DESIGN: Retrospective, descriptive multicenter study. SETTING: Three endocrine centers in Al Ain. PATIENTS AND METHODS: All patients diagnosed with SMs in the city of Al Ain, Emirate of Abu Dhabi, between 2011 and 2016 were evaluated. Cases were identified using ICD 9 and 10 codes and demographic and clinical data were collected. The prevalence rate was calculated for patients alive and residing in Al Ain city until 31 December 2016. MAIN OUTCOME MEASURES: Clinical presentations and prevalence rate. SAMPLE SIZE: 272. RESULTS: The mean (SD) age on presentation was 40.8 (14.3) years (range: 6-114 years, median: 40.0). The 170 (61.8%) females and 128 (46.5%) were native citizens of the United Arab Emirates. Two hundred and forty five (90%) patients had pituitary adenomas (PAs) while 27 (10%) had non-pituitary sellar lesions. The four most common SMs were prolactinoma (n=139, 51.1%), nonfunctioning adenoma (NFA) (n= 69, 25.4%), somatotroph adenoma (n=32, 11.8%) and craniopharyngioma (n=15, 5.5%). Patients with prolactinoma, corticotroph adenoma, and Rathke's cleft cyst had small sellar masses (<1 centimeter) while the majority of patients with other SMs had macroadenomas. Hypogonadism and growth hormone deficiency was present in 41.8% and 20.5% of the patients, respectively. Of 268 patients with available data, 82 patients underwent surgery while 25 patients received radiotherapy. At the end of 2016, 197 patients were residing in Al Ain city. The overall prevalence of SMs was 25.7/100 000 with PAs constituting most of these masses (n=177) for a prevalence of 23.1/100 000. CONCLUSIONS: This is the first study of SMs in the United Arab Emirates and the MENA region. Prolactinoma and NFA were the two most common SMs. Further studies are needed to explore the reasons for the lower prevalence of SMs in our region compared with other countries. LIMITATIONS: Retrospective design, relatively small sample size. CONFLICT OF INTEREST: None.

Novel Use of Biodissolvable Stent in Treatment of Recurrent Rathke Cleft Cyst.

BACKGROUND: Rathke cleft cysts (RCCs) are benign cysts arising from the pars intermedia as a result of incomplete obliteration of the Rathke pouch during development of the pituitary gland. The most common presenting symptoms are headaches, visual disturbances, and endocrinopathies. Recurrence of RCCs after surgical treatment is a well-known phenomenon after surgery with reported recurrence rates as high as 30%. Various methods have been employed to reduce the rate of recurrence. Complete cyst wall resection has been associated with increased rates of perioperative cerebrospinal fluid leak, diabetes insipidus, and carotid injury, while inconsistently demonstrating reduced recurrence rates. Marsupialization, in which the cyst cavity is widely exposed and left open with or without a fat graft suspension, has similarly shown increased morbidity without clear improvement in outcomes. We report here the use of a steroid-eluting sinus stent to maintain patency of the cyst opening. CASE DESCRIPTION: A 39-year-old female presented with a symptomatic RCC. She underwent 4 different surgeries including cyst wall resection, marsupialization, and fat graft placement. She developed short-term symptomatic and radiographic recurrence within 3 months of each surgery. She then underwent placement of a steroid-eluting sinus stent. At 3 months, the patient remained symptom free, without radiographic recurrence and with patent cyst fenestration on nasal endoscopy. CONCLUSIONS: Recurrent RCCs are challenging to manage. Strategies to reduce recurrence are typically associated with higher risk and varying success. Stent placement represents a simple, low-risk method of potentially maintaining patency of cyst fenestration.

Cystic lesions of the sellar-suprasellar region - diagnosis and treatment.

The differentiation of cystic lesions located in the sellar-suprasellar region is a significant problem in clinical practice because of the similarities in their clinical, radiological, and even histopathological picture. Arriving at the right diagnosis is vital for taking appropriate therapeutic decisions. The most frequent clinical manifestation of lesions located in the sellar-suprasellar region is headache. It often co-exists with symptoms of anterior pituitary gland insufficiency or hyperprolactinaemia caused by compression of the pituitary stalk. Diabetes insipidus, obe-sity, mental disorders, and circadian rhythm disorders may be associated with lesions penetrating the suprasellar space. It is extremely important to rule out the possible coexistence of pituitary microadenoma and Rathke's cleft cyst, which became possible with the use of ¹¹C-methionine positron emission tomography/computed tomography (C-MET PET/CT). Reports from literature indicate that pituitary microadenoma may coexist with Rathke's cleft cyst in 10% of patients. Cystic lesions of the sellar-suprasellar region should also be differentiated from a cystic pituitary adenoma or abscess. The first-choice therapy in symptomatic cystic lesions of the sellar-suprasellar region is neurosurgery, which usually relieves headache and improves vision impairment, while less frequently restores normal pituitary function. In suprasellar lesions, neurosurgery may trig-ger or aggravate pre-existing symptoms of damage to the hypothalamus. Patients undergoing neurosurgery for cystic lesions located in the sellar-suprasellar region should be monitored for a few years due to their high recurrence rate, potential malignant transformation of these lesions, and possible adenoma development through metaplasia. The advent of targeted therapy of the BRAF/MEK pathway is associated with new therapeutic opportunities for patients with craniopharyngiomas.

Spontaneous Cystic Arteriovenous Malformation-A Novel Treatment Alternative.

BACKGROUND: Brain arteriovenous malformation (BAVM) is a life-threatening vascular congenital malformation due to the risk of intracerebral hemorrhage. The formation of a spontaneous cyst within a BAVM, without history of hemorrhage or gamma knife surgery, is rare and has an unknown pathophysiology. We suggest a novel theory of spontaneous cystic BAVM formation, subsequently treated with endovascular embolization followed by surgery with a favorable long-term outcome. METHODS: Review of the literature using PUBMED database and comparison between clinical presentation, diagnostic imaging studies, and treatment options. RESULTS: The high flow causing a shear stress over the drainage vein may be responsible for venous ectasia and thus incrementing hydrostatic intranidal pressure causing a serous effusion that develops into a pseudocyst. We hypothesize that hemodynamic factors may be responsible for both origin and growth of such cystic lesions. CONCLUSIONS: Endovascular treatment allowed us to achieve cyst volume reduction by managing its unique flow characteristics. To our knowledge, this is the first case report of combined treatment with endovascular and surgical approach of a cystic BAVM.

Leukoencephalopathy, cerebral calcifications, and cysts: A clinical case involving a long-term follow-up and literature review.

BACKGROUND: Leukoencephalopathy, cerebral calcifications, and cysts (LCC) is a rare disease that was first reported by Labrune in 1996. A case of adult-onset LCC was successfully followed up for a long period. CASE PRESENTATION: A 30-year-old female presented with visual field disturbance and seizure on several occasions. Radiographic images revealed multiple supratentorial cysts and calcifications in the bilateral nucleus basalis and cerebella. Aspiration, Ommaya reservoir placement, and nodule removal were performed for the responsible cysts, and the patient had a good postoperative course. DISCUSSION: A tiny, strongly enhanced nodule was identified before cyst formation on her radiographic images. Thus, cyst growth may be related to nodule microbleeding. According to our review, if the responsible cyst is located on the noneloquent area, surgical removal of the cyst should be considered. However, if the responsible cyst is located on the eloquent area, the nodule should be first removed because nodules can bleed and enlarge cysts. CONCLUSION: Careful follow-up is needed, especially for cysts with a strongly enhanced nodule.

Cerebroretinal microangiopathy with calcifications and cysts: A case report.

RATIONAL: Cerebroretinal microangiopathy with calcifications and cysts (CRMCC) is believed to be an autosomal recessive genetic disease, with disorders in multisystem organs. Its characteristic neurological disorders manifested on neuroimaging are a triad of leukoencephalopathy, intracranial calcifications, and parenchymal cysts. In this paper, we report a CRMCC patient with multisystem involvement, focusing on the neuroimaging features, to get a better understanding of the rare disease and improve our diagnostic ability. PATIENT CONCERNS: The 23-year-old female patient firstly presented with an adolescence onset of ophthalmological manifestations. Four years later, hematological and neurological disorders occurred, the latter of which demonstrated a relatively slow progression in the following 7 years preceding her presentation to our hospital. INTERVENTIONS: During hospitalization, disorders involving digestive, cardiovascular and respiratory systems were also detected. In addition, a more comprehensive depiction of neurological disorders on neuroimaging was also obtained. DIAGNOSES: On the basis of multiple system disorders and the detection of mutations in conserved telomere maintenance component 1(CTC1) gene, a diagnosis of CRMCC was made. OUTCOMES: After supportive therapy during her 4-week hospitalization, the patient's general condition improved and was released from the hospital. LESSONS: CRMCC could be primarily diagnosed with the aid of its multiple system disorders and remarkable neuroimaging features. Cerebral micro hemorrhages determined by the combination of CT and T2-weighted magnetic resonance images in our case could provide some additional information for diagnosis. Furthermore, several other associated disorders were depicted for the first time in our case, expanding the clinical spectrum of CRMCC.

Leukoencephalopathy with cerebral calcification and cysts: Cases report and literature review.

BACKGROUND: Leukoencephalopathy with calcifications and cysts (LCC) is a rare disease in which parenchymal cysts and calcifications within a widespread leukoencephalopathy can cause a broad spectrum of neurological symptoms. We present cases with adult LCC and discuss previously described entities in relevant literature. CASE PRESENTATION: Two cases of adult-onset LCC confirmed by clinical presentations, typical neuroimaging and neuropathological findings are reported. LITERATURE REVIEW: A detailed search of all relevant reports published in the English language between 1996 and 2015 via PubMed (http://www.ncbi.nlm.nih.gov/pubmed) was performed, with "Leukoencephalopathy", "cerebral calcifications" and "cysts" as keywords. Including the current cases, we summarized the clinical presentations, neuroimaging features, biopsy features, and genetic features of 38 LCC patients. CONCLUSION: Our findings suggested that LCC could be diagnosed by clinical presentations, neuroimaging and gene detection, and biopsy might not be necessary. Therefore, we propose a diagnostic flow chart for neuroimaging in leukoencephalopathy, cerebral calcifications and cysts.

Intraparenchymal infiltration of Rathke's cleft cysts manifesting as severe neurological deficits and hypopituitarism: 2 case reports.

BACKGROUND: Rathke's cleft cysts generally remain asymptomatic throughout life, but a few patients may suffer severe neurological and/or endocrinological deficits. The symptoms include visual disturbances caused by compression of the optic chiasm, and severe endocrinological deficits caused by repeated intracystic hemorrhage or leakage of cyst content. However, no case of Rathke's cleft cyst has infiltrated into neuroglial tissue with marked cerebral edema. CASE PRESENTATION: Two patients presented with non-infectious re-deterioration of Rathke's cleft cysts with intraparenchymal infiltration and marked cerebral edema, to ipsilateral hypothalamus in one case and to the bilateral frontal lobes in the other. Both patients were surgically treated by extended transsphenoidal surgery, and showed remarkable improvement with postoperative pulse-dose steroid therapy, including disappearance/shrinkage of abnormal enhanced lesion and cerebral edema on magnetic resonance imaging. Histological examination disclosed significant squamous metaplasia in epithelia and marked infiltration of inflammatory cells into the pituitary gland and neuroglial tissues. Most infiltrated cells were lymphocytes and plasma cells, thought to indicate the involvement of long-term underling inflammatory processes in this phenomenon. CONCLUSION: Long-term subclinical inflammation may be the mechanism of this extraordinary aggressive clinical course. Postoperative steroid administration should be reduced prudently, and careful follow-up imaging is essential in cases of Rathke's cleft cyst with abnormal histological findings.

Late-onset leukoencephalopathy with cerebral calcifications and cysts: case report and review of the literature.

BACKGROUND: Leukoencephalopathy with calcifications and cysts (LCC or Labrune disease) is a relatively recently defined and exceptionally rare disease in which parenchymal cysts and calcifications within a widespread leukoencephalopathy can cause a broad spectrum of neurological symptoms. The cause of the disease is unknown. Manifestation is usually in childhood or adolescence, while onset in adulthood has been described in 19 cases. CASE PRESENTATION: Here we report a case of an adult-onset LCC of a Caucasian woman who became symptomatic at age 70 as confirmed by typical neuroimaging and neuropathological findings. After resection of left mesioparietal space-occupying cystic brain tissue the patient has so far remained clinically stable during one year of follow-up with a continuous treatment with glucocorticosteroids. CONCLUSION: To our knowledge this report of a patient who became symptomatic at age 70 represents the oldest age-at-onset case of LCC described so far.

Pediatric Suprasellar Tumors.

The various childhood suprasellar tumors, while pathologically distinct, present similar clinical and surgical challenges as a result of their common anatomic location. These lesions are in close proximity to or may invade the optic nerve and chiasm, pituitary gland and infundibulum, hypothalamus, and third ventricle, leading to presenting features including visual field loss, impairment in visual acuity, endocrine dysfunction, and hydrocephalus. Though many suprasellar lesions are relatively benign in pathology, treatment may be complicated by high surgical morbidity resulting from damage to the hypothalamic-pituitary axis. Here we review the most frequent pediatric lesions occurring in the suprasellar region: craniopharyngioma, chiasmatic glioma, germ cell tumor, Rathke cleft and arachnoid cysts, pituitary adenoma, and histiocytosis. This review outlines both common presenting features and differentiating aspects of these lesions. It also includes classic radiographic presentations and treatment considerations for each lesion.

Spontaneous Shrinkage of a Suprasellar Arachnoid Cyst Diagnosed with Prenatal Sonography and Fetal Magnetic Resonance Imaging: Case Report and Review of the Literature.

Arachnoid cysts (AC's) are congenital anomalies that occur within the cerebrospinal fluid (CSF) cisterns and major cerebral fissures. Suprasellar AC's comprise 5-12.5% of all the lesions. Despite being commonly reported, their etiology and pathogenesis still remain unclear. In this report, we presented an unusual case of spontaneous shrinkage of a suprasellar AC that had been incidentally diagnosed during a routine prenatal sonographic examination. To our knowledge, only three cases of spontaneous shrinkage of suprasellar AC have been reported in the literature. In detail of the manuscript, fetal and postnatal radiological images were presented and the possible mechanisms were discussed with a review of the literature.

Conservative treatment of cysts of the cavum septum pellucidum presenting in childhood: report of 3 cases.

Cavum septum pellucidum (CSP) cysts are relatively rare. The most common presenting symptom is headache, which is thought to be secondary to elevated intracranial pressure. Many CSP cysts are treated surgically; conservative treatment is seldom recommended. The authors describe 3 cases of pediatric CSP cysts that were managed without surgery. The patients ranged in age from 5 months to 8 years old. Two presented with headaches, which were associated with mild ventricular enlargement in 1 case. Over the course of 5-15 months, 2 cysts became markedly reduced in size, and in one of these 2 cases a substantial reduction in ventricle size was also observed. At last follow-up, all 3 children were asymptomatic. The authors note that CSP cysts are often associated with headaches. In the absence of hydrocephalus, they recommend conservative management with clinical and radiological follow-up.

[Reversible porencephalic cyst related to shunt dysfunction]. / Cavidad porencefálica reversible secundaria a disfunción valvular.

INTRODUCTION: The surgical treatment of hydrocephalus is one of the most commonly procedures of modern pediatric neurosurgical practice, and cerebrospinal fluid shunts are the main means of treatment of hydrocephalus, being independent of site of obstruction and ventricular shunt complications are frequent, especially in children. The most common complications are shunt obstructions, mechanical disconnections, overdrainage and infection. CASE REPORT: A 7.5-year-old girl with ventriculoperitoneal shunt presented with intermittent headache. A head computed tomography scan revealed a cyst located in right temporal lobe. After ventriculoperitoneal shunt revision, a gradual resolution of the cyst was demonstrated on computed tomography. CONCLUSIONS: Cerebrospinal fluid porencephaly is a rare postoperative complication of a ventricular shunt that results from dysfunction of the distal catheter, especially in children with taut ventricles.

TITLE: Cavidad porencefalica reversible secundaria a disfuncion valvular.Introduccion. El tratamiento quirurgico de la hidrocefalia es uno de los procedimientos quirurgicos mas habituales en la neurocirugia pediatrica, y las derivaciones ventriculoperitoneales constituyen una herramienta fundamental en el tratamiento de la hidrocefalia tanto infantil como del adulto. Las complicaciones de las valvulas son relativamente frecuentes, sobre todo en la poblacion pediatrica, y, entre estas, las mas habituales incluyen: las obstrucciones, las desconexiones, el hiperdrenaje y las infecciones. Caso clinico. Niña de 7,5 años, portadora de una valvula ventriculoperitoneal, que presentaba cefalea intermitente. Un estudio con tomografia computarizada demostro una lesion quistica temporal derecha. Tras la revision valvular, la tomografia computarizada evidencio la reduccion del quiste. Conclusiones. La formacion de una cavidad porencefalica es una complicacion poco frecuente. Se relaciona con problemas en el cateter distal en pacientes con ventriculos dilatados y de las que existen escasas referencias en la bibliografia.

Incidence of pineal gland cyst and pineoblastoma in children with retinoblastoma during the chemoreduction era.

PURPOSE: To report on the frequency of cysts and tumors of the pineal gland in patients with retinoblastoma. DESIGN: Observational retrospective case control study. SETTING: Institutional. study population: Four hundred eight patients treated for retinoblastoma from January 2000 to January 2012 at Wills Eye Institute, Philadelphia, Pennsylvania, USA. OBSERVATION PROCEDURE: Magnetic resonance imaging (MRI) features of the pineal gland were evaluated in all patients with retinoblastoma. Characteristics of patients with pineal cysts and pineoblastoma were reviewed. MAIN OUTCOME MEASURES: Comparison of frequency of pineal gland cyst and pineoblastoma in children managed with systemic chemoreduction vs other methods. RESULTS: Of 408 patients, treatment included systemic chemoreduction in 252 (62%) and nonchemoreduction methods in 156 (38%). Overall, 34 patients (8%) manifested pineal gland cyst and 4 (1%) showed pineoblastoma. Of all 408 patients, comparison (chemoreduction vs nonchemoreduction) revealed pineal cyst (20/252 vs 14/156, P = .7) and pineoblastoma (1/252 vs 3/156, P = .1). The pineal cyst (n = 34) (mean diameter 4 mm) was asymptomatic (n = 34), followed conservatively (n = 34), and with minimal enlargement (n = 2, 9%) but without progression to pineoblastoma. The cyst was found in 22 germline and 12 nongermline patients (P = .15). Among the 4 patients with pineoblastoma, all had germline mutation and 2 had family history of retinoblastoma. Among all patients with family history of retinoblastoma (n = 45), 2 (4%) developed pineoblastoma. The pineoblastoma was asymptomatic in 2 patients and symptomatic with vomiting and headache in 2 patients. The mean interval from date of retinoblastoma detection to pineal cyst was 2 months (median 2, range 0-8 months) and to pineoblastoma was 27 months (median 28, range 7-46 months). Management included aggressive chemotherapy and radiotherapy, with 2 survivors. CONCLUSIONS: Pineal gland cyst was incidentally detected in 8% of retinoblastoma patients, causing no symptoms, and without progression to pineoblastoma. Pineoblastoma was detected in 1% of patients and fewer patients who received systemic chemotherapy developed pineoblastoma, possibly indicating a systemic protective effect.

Primary intracranial and spinal hydatidosis: a retrospective study of 21 cases.

OBJECTIVE: To analyse the epidemiological characteristics, clinical symptoms, radiological aspects, treatments, and outcomes of primary central nervous system (CNS) hydatidosis and compare our results with those observed for secondary intracranial hydatidosis. PATIENTS AND METHODS: We retrospectively reviewed 21 cases of primary CNS hydatid cysts operated on at the First Affiliated Hospital of Xinjiang Medical University between 1996 and 2010. RESULTS: Of the 21 primary cases, the vast majority were intracranial hydatidosis patients (20 cases, 95·24%). Only one patient had spinal hydatidosis. Unlike previously published reports, we found that intracranial hydatid cysts were more common in adults (80·96%) than in children (19·04%), with a slight male predominance (M/F = 1·1). All symptoms, including vomiting, nausea, and focal neurological signs, resulted from the increased intracranial pressure, which was closely associated with the cyst location. For the spinal hydatidosis patient, the primary symptom of back pain was indicative of spinal cord compression syndrome. All cysts in the 21 primary cases were pathologically similar. The recurrence percentage was 28% over 12 years. Two patients with multiple intracranial hydatid cysts died due to foramen magnum herniation. CONCLUSION: Despite imaging and therapeutic advances, CNS hydatidosis remains difficult to treat, and severe complications and the high incidence of recurrence result in unsatisfactory outcomes.