Teratoma of the nervous system: A case series

Algahtani, Hussein; Shirah, Bader; Abdullah, Ahad; Bazaid, Abdulrahman

doi:10.1016/j.neucir.2017.03.004

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The ventricular system is prominent representing hydrocephalus mainly affecting the lateral and third ventricles." ] ] ] "textoCompleto" => "IntroductionTeratomas, a common form of non-germinomatous germ cell tumor, are composed of multiple tissues foreign to the organ or site from which they arise with a histological representation of all three germ cell layers (ectoderm, mesoderm, and endoderm).<a class="elsevierStyleCrossRef" href="#bib0065">1</a> Teratomas commonly present during infancy and childhood with males being affected more often than females. Teratomas account for 3% of all pediatric tumors with the major site affected being the sacrococcygeal regions and the gonads.<a class="elsevierStyleCrossRef" href="#bib0070">2</a> In this study, we report three cases of intracranial teratomas with an interesting clinical course, neuroradiology, and outcome. In addition, we review the literature and convey important messages to the neuroscience community regarding issues related to the management of these rare tumors.Case report 1 (pineal gland teratoma)A 10-year-old male presented to our hospital with a 7-month history of headache, vomiting, and decreased vision in the left eye. The course was progressive, and a few days prior to seeking medical advice, he experienced a decrease in the level of consciousness. The patient was a product of a full term pregnancy and uncomplicated vaginal delivery. At birth, his Apgar score, head circumference, weight, and fontanels were all normal. His milestones were normal with no remarkable medical and surgical abnormalities. He completed all scheduled vaccinations. There was a family history of a brain tumor in the paternal grandmother. CT of the brain (without contrast) revealed a mass in the pineal region measuring 1.3×1.5cm with calcification and possible fat components. The ventricular system was prominent representing hydrocephalus mainly affecting the lateral and third ventricles (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Magnetic Resonance Imaging (MRI) of the brain was done, which revealed a heterogeneous pineal region mass measuring 1.4×1.3cm with internal fat signal intensity and calcifications. He was diagnosed with pineal region tumor with obstructive hydrocephalus. Tumor markers in both serum and cerebrospinal fluid (CSF) were negative. A right ventriculoperitoneal shunt was inserted with an improvement of both headache and vision. He was discharged home and recommended to follow up and repeat the MRI in three months. Unfortunately, he missed his appointment, and a few months later, he was presented again to the hospital with a chronic headache that increased during the day with fatigue. His overall performance at school was deteriorating with associated sleep disturbance. He had a history of marked weight loss, polyphagia, morning sickness, and vomiting with intermittent ptosis. There was no history of seizures or weakness. On examination during this admission, fundus examination showed papilledema. He was clinically fully conscious, oriented, with full extraocular movements and normal motor and gait examinations. MRI of the brain showed interval increase in the size of the tumor measuring 5×3cm with extensive venous congestion and cerebellar swelling with no hydrocephalus observed (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). He was admitted for craniotomy and tumor debulking, but the surgery was limited to bony decompression and duroplasty due to severe cerebellar engorgement, which made it impossible to reach the pineal region using the supracerebellar infratentorial approach. All measures to reduce tension during surgery did not help the engorgement, which was related to venous compression of the deep veins by the tumor. He recovered well postoperatively and treated with a course of steroid and Mannitol, which made resection of the tumor by endoscopic third ventriculostomy achievable. The pediatric oncology department was involved for follow-up and adjuvant radiotherapy. He received six cycles of chemotherapy; Ifosfamide and Etoposide (VP-16). He is still alive and receiving treatment.<elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia>Case report 2 (hemispheric cerebral teratoma)A 2-year-old male, a case of cow milk allergy and Rickets, presented to the emergency department with a 2-day history of vomiting associated with a decreased oral intake and progressive right eye squint for two weeks. During the initial physical examination, he was awake and alert with a Glasgow Coma Scale (GCS) of 15/15 and was found to have bradycardia with a heart rate of 60–70 beats per minute. The pupils were reacting briskly to light with mild abducens nerve palsy on the right side. Computed Tomography (CT) of the brain revealed a left hemispheric mass with an internal area of calcification and hemorrhage (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). MRI showed that the mass measured 9.9×7.2cm with extensions into the left side and posteriorly occupying the cranial-caudal diameter. There was a significant mass effect and midline shift of around 1.4cm and hydrocephalus (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>). The patient developed vomiting and was electively intubated, and an emergency mass excision was performed. The mass was showing changes consistent with left hemispheric teratoma with an internal area of calcification and hemorrhage. During surgery and while debulking the tumor, the patient bled profusely which necessitated the administration of several blood units. Unfortunately, the patient developed cardiac arrest, and cardiopulmonary resuscitation (CPR) was performed. CPR continued for about one hour with no success. The cardiac arrest and unsuccessful CPR were then found to be due to the increased potassium level up to 13.8mmol/L.<elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia>Case report 3 (posterior fossa teratoma)A 5-year-old male presented one year ago to a local hospital with a three months history of headache, vomiting, and irritability that was progressive in course. One month prior to seeking medical advice, he became obtunded and stopped responding to questions and verbal commands. A CT scan was performed and revealed a cystic posterior fossa lesion (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a>). The patient was then urgently referred to our hospital. He was a product of a full term pregnancy and uncomplicated vaginal delivery. His Apgar score, head circumference, weight, and fontanels at birth were all normal. His milestones were normal until the time of presentation. His medical, surgical, and family history were unremarkable, and vaccinations were all up to date. His vital signs were normal on examination in our emergency department. His level of consciousness was altered, but he was able to open his eyes spontaneously and obey simple commands. His pupils were equal and reactive, and extraocular muscle and gaze examination revealed paralysis of upward gaze. The remaining cranial nerves, motor functions, and coordination were normal. He was admitted to the pediatric intensive care unit, and an emergency extraventricular drain was inserted. On the 2nd day, he developed a generalized tonic–clonic seizure before his surgery and was started on lorazepam and phenytoin therapy. Three days later, a suboccipital craniotomy and resection of the posterior fossa tumor were carried out with no complications. Intraoperatively, the tumor was found to be composed of a large loop of intestine with teeth and hair-like structures and was removed completely. The pathology department diagnosed him as a case of mature posterior fossa teratoma. Eight days later from his admission, a ventriculoperitoneal shunt was inserted due to the hydrocephalus and high intracranial pressure on monitoring. 6 weeks later, in the outpatient department, his parents reported no significant complaints, and the neurological examination was normal. The parents expressed their satisfaction regarding his progress and management plan. One year later, an MRI of the brain showed significant improvement of the communicating hydrocephalus with no evidence of tumor recurrence. Six years later, a brain CT was performed and showed significant improvement with no evidence of tumor recurrence (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a>).<elsevierMultimedia ident="fig0025"></elsevierMultimedia>DiscussionGerm cell tumors are uncommon pediatric brain tumors accounting for 1–3%, but for unknown reasons, they are more common in Japan and South East Asia (around 10%). Although rare in infancy and adulthood, teratomas are the commonest congenital brain tumors and also in the first two years of life. They account for up to 50% of fetal brain tumors, 33% of neonatal intracranial tumors, and only 2–4% of intracranial tumors in people less than 15 years of age.<a class="elsevierStyleCrossRef" href="#bib0070">2</a> Of all the germ cell tumors, only the mature teratoma is considered to be benign. Although intracranial teratomas may occur anywhere in the neuroaxis, they are usually located in the midline region, in or around the pineal gland, third ventricular region, and suprasellar region.<a class="elsevierStyleCrossRef" href="#bib0075">3</a>The presenting signs and symptoms of teratoma are variable and related to the size and localization of the tumor.<a class="elsevierStyleCrossRef" href="#bib0080">4</a> Tumors in the pineal region typically present with signs of obstructive hydrocephalus as a result of increased intracranial pressure. About 25–50% of patients with pineal region tumors have ophthalmological abnormalities and somnolence and 25% have ataxia, seizures, and behavioral changes. Parinaud's syndrome (vertical gaze palsy) that is caused by involvement of the adjacent midbrain structures, specifically the rostral interstitial nucleus of medial longitudinal fasciculus, affects about 50%. Other less common manifestations include endocrinopathies and disturbances in the sexual development.<a class="elsevierStyleCrossRef" href="#bib0085">5</a> Tumors of the suprasellar region typically present with hypothalamic/pituitary axis dysfunction including diabetes insipidus, delayed sexual development, isolated growth hormone deficiency, hypopituitarism, and precocious puberty. Other less common signs include ophthalmological abnormalities (e.g. bilateral temporal hemianopsia due to compression of the optic chiasm).<a class="elsevierStyleCrossRef" href="#bib0090">6</a>The diagnosis of intracranial teratomas is based on clinical signs and symptoms, neuroimaging, tumor markers, CSF cytology, and histological assessment. Assay of serum and CSF for α-fetoprotein and beta human chorionic gonadotropin are currently routine in the assessment of suspected teratoma prior to surgery and typically show an elevation of either oncoproteins.<a class="elsevierStyleCrossRef" href="#bib0095">7</a> Both CT and MRI are sensitive in intracranial teratomas. The CT or MRI features of teratomas in the intracranial cavity are non-specific, but a diagnosis can be suspected if the lesion shows intratumoral cysts admixed with calcified regions, heterogeneous density, and foci of low-attenuation similar to fat.<a class="elsevierStyleCrossRef" href="#bib0100">8</a> Although the tumors in our cases were located in the pineal gland, posterior fossa, and cerebral hemisphere, other reported sites of origin include the hypothalamus, ventricles, cavernous sinus, suprasellar region, and cerebellum.<a class="elsevierStyleCrossRef" href="#bib0100">8</a>The management of teratomas usually depends on the histological findings from the biopsy. Total surgical resection is considered the gold standard for the treatment of intracranial teratomas.<a class="elsevierStyleCrossRef" href="#bib0105">9</a> Management of teratomas in the pineal glands includes ventriculoperitoneal shunts and endoscopic third ventriculostomy. Performing endoscopic third ventriculostomy may be the treatment of choice of obstructive hydrocephalus, and some experts would argue that endoscopic third ventriculostomy and biopsy should be performed as the initial management for a pineal mass.<a class="elsevierStyleCrossRef" href="#bib0110">10</a> The follow-up period after resection may be as long as ten years due to a possible metachronous occurrence. In addition, a combination of chemotherapy and radiation therapy are often used.<a class="elsevierStyleCrossRef" href="#bib0115">11</a> Mature teratoma is more resistant to chemotherapy and radiotherapy than any other germ cell tumor. It is not unusual to operate in a “second look” surgery for a mature teratoma because residual tumor is frequent after chemotherapy and radiotherapy. The prognosis is dependent on location, size, and surgical experience.<a class="elsevierStyleCrossRef" href="#bib0120">12</a>ConclusionThe present cases in our series are interesting examples of intracranial teratoma in terms of location of the tumor and neuroimaging findings. Total surgical resection is the treatment of choice for intracranial teratomas. However, preoperative, intraoperative, and postoperative medical care and good preparation for surgery are of paramount importance. This includes acid–base balance, electrolyte disturbances, and preparation for an unexpected large amount of blood loss. Surgical experience is a prerequisite for a good clinical outcome. Delay in surgical intervention may complicate the course of the disease with progressive enlargement of tumors and development of complication including hydrocephalus. Using endoscopic surgical techniques may emerge as the preferred intervention option as compared to other traditional methods. We recommend the establishment of a national and international registry for intracranial tumors.Conflict of interestThe authors declare that they have no conflicts of interest." 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Intracranial teratomas are very rare. In this study, we report three cases of intracranial teratomas with an interesting clinical course, neuroradiology, and outcome. In addition, we review the literature and convey important messages to the neuroscience community regarding issues related to the management of these rare tumors. The present cases are interesting examples of intracranial teratoma in terms of location of the tumor and neuroimaging findings. Delay in surgical intervention may complicate the course of the disease with progressive enlargement of tumors and development of complication including hydrocephalus. Using endoscopic surgical techniques may emerge as the preferred intervention option as compared to other traditional methods. We recommend the establishment of a national and international registry for intracranial tumors." ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "El teratoma es un tipo frecuente de tumor de células germinales compuesto por múltiples tejidos ajenos a la zona en que aparecen con una representación histológica de las 3 capas de células germinales. Los teratomas intracraneales son muy poco frecuentes. En este estudio, se presentan 3 casos de teratomas intracraneales con una evolución clínica, neurorradiología y resultado interesantes. Además, se revisa la bibliografía y se difunde un mensaje importante a la comunidad de las neurociencias respecto a los problemas relacionados con el tratamiento de estos tumores raros. Estos casos son ejemplos interesantes de teratoma intracraneal en términos de localización de los hallazgos tumorales y de neuroimagen. El retraso en la intervención quirúrgica puede complicar la evolución de la enfermedad con la ampliación progresiva de los tumores y el desarrollo de complicaciones, especialmente hidrocefalia. El uso de técnicas quirúrgicas endoscópicas aparece como la opción de intervención preferida en comparación con otros métodos tradicionales. Se recomienda el establecimiento de un registro nacional e internacional de tumores intracraneales." ] ] "multimedia" => array:5 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1182 "Ancho" => 1600 "Tamanyo" => 226184 ] ] "descripcion" => array:1 [ "en" => "CT of the brain (without contrast) showing a mass in the pineal region measuring 1.3×1.5cm with calcification and possible fat components. 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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">CT of the brain &#40;without contrast&#41; showing a mass in the pineal region measuring 1&#46;3<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>1&#46;5<span class="elsevierStyleHsp" style=""></span>cm with calcification and possible fat components&#46; The ventricular system is prominent representing hydrocephalus mainly affecting the lateral and third ventricles&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Teratomas&#44; a common form of non-germinomatous germ cell tumor&#44; are composed of multiple tissues foreign to the organ or site from which they arise with a histological representation of all three germ cell layers &#40;ectoderm&#44; mesoderm&#44; and endoderm&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">1</span></a> Teratomas commonly present during infancy and childhood with males being affected more often than females&#46; Teratomas account for 3&#37; of all pediatric tumors with the major site affected being the sacrococcygeal regions and the gonads&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">2</span></a> In this study&#44; we report three cases of intracranial teratomas with an interesting clinical course&#44; neuroradiology&#44; and outcome&#46; In addition&#44; we review the literature and convey important messages to the neuroscience community regarding issues related to the management of these rare tumors&#46;</p><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report 1 &#40;pineal gland teratoma&#41;</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 10-year-old male presented to our hospital with a 7-month history of headache&#44; vomiting&#44; and decreased vision in the left eye&#46; The course was progressive&#44; and a few days prior to seeking medical advice&#44; he experienced a decrease in the level of consciousness&#46; The patient was a product of a full term pregnancy and uncomplicated vaginal delivery&#46; At birth&#44; his Apgar score&#44; head circumference&#44; weight&#44; and fontanels were all normal&#46; His milestones were normal with no remarkable medical and surgical abnormalities&#46; He completed all scheduled vaccinations&#46; There was a family history of a brain tumor in the paternal grandmother&#46; CT of the brain &#40;without contrast&#41; revealed a mass in the pineal region measuring 1&#46;3<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>1&#46;5<span class="elsevierStyleHsp" style=""></span>cm with calcification and possible fat components&#46; The ventricular system was prominent representing hydrocephalus mainly affecting the lateral and third ventricles &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Magnetic Resonance Imaging &#40;MRI&#41; of the brain was done&#44; which revealed a heterogeneous pineal region mass measuring 1&#46;4<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>1&#46;3<span class="elsevierStyleHsp" style=""></span>cm with internal fat signal intensity and calcifications&#46; He was diagnosed with pineal region tumor with obstructive hydrocephalus&#46; Tumor markers in both serum and cerebrospinal fluid &#40;CSF&#41; were negative&#46; A right ventriculoperitoneal shunt was inserted with an improvement of both headache and vision&#46; He was discharged home and recommended to follow up and repeat the MRI in three months&#46; Unfortunately&#44; he missed his appointment&#44; and a few months later&#44; he was presented again to the hospital with a chronic headache that increased during the day with fatigue&#46; His overall performance at school was deteriorating with associated sleep disturbance&#46; He had a history of marked weight loss&#44; polyphagia&#44; morning sickness&#44; and vomiting with intermittent ptosis&#46; There was no history of seizures or weakness&#46; On examination during this admission&#44; fundus examination showed papilledema&#46; He was clinically fully conscious&#44; oriented&#44; with full extraocular movements and normal motor and gait examinations&#46; MRI of the brain showed interval increase in the size of the tumor measuring 5<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>3<span class="elsevierStyleHsp" style=""></span>cm with extensive venous congestion and cerebellar swelling with no hydrocephalus observed &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; He was admitted for craniotomy and tumor debulking&#44; but the surgery was limited to bony decompression and duroplasty due to severe cerebellar engorgement&#44; which made it impossible to reach the pineal region using the supracerebellar infratentorial approach&#46; All measures to reduce tension during surgery did not help the engorgement&#44; which was related to venous compression of the deep veins by the tumor&#46; He recovered well postoperatively and treated with a course of steroid and Mannitol&#44; which made resection of the tumor by endoscopic third ventriculostomy achievable&#46; The pediatric oncology department was involved for follow-up and adjuvant radiotherapy&#46; He received six cycles of chemotherapy&#59; Ifosfamide and Etoposide &#40;VP-16&#41;&#46; He is still alive and receiving treatment&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Case report 2 &#40;hemispheric cerebral teratoma&#41;</span><p id="par0015" class="elsevierStylePara elsevierViewall">A 2-year-old male&#44; a case of cow milk allergy and Rickets&#44; presented to the emergency department with a 2-day history of vomiting associated with a decreased oral intake and progressive right eye squint for two weeks&#46; During the initial physical examination&#44; he was awake and alert with a Glasgow Coma Scale &#40;GCS&#41; of 15&#47;15 and was found to have bradycardia with a heart rate of 60&#8211;70 beats per minute&#46; The pupils were reacting briskly to light with mild abducens nerve palsy on the right side&#46; Computed Tomography &#40;CT&#41; of the brain revealed a left hemispheric mass with an internal area of calcification and hemorrhage &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; MRI showed that the mass measured 9&#46;9<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>7&#46;2<span class="elsevierStyleHsp" style=""></span>cm with extensions into the left side and posteriorly occupying the cranial-caudal diameter&#46; There was a significant mass effect and midline shift of around 1&#46;4<span class="elsevierStyleHsp" style=""></span>cm and hydrocephalus &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; The patient developed vomiting and was electively intubated&#44; and an emergency mass excision was performed&#46; The mass was showing changes consistent with left hemispheric teratoma with an internal area of calcification and hemorrhage&#46; During surgery and while debulking the tumor&#44; the patient bled profusely which necessitated the administration of several blood units&#46; Unfortunately&#44; the patient developed cardiac arrest&#44; and cardiopulmonary resuscitation &#40;CPR&#41; was performed&#46; CPR continued for about one hour with no success&#46; The cardiac arrest and unsuccessful CPR were then found to be due to the increased potassium level up to 13&#46;8<span class="elsevierStyleHsp" style=""></span>mmol&#47;L&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Case report 3 &#40;posterior fossa teratoma&#41;</span><p id="par0020" class="elsevierStylePara elsevierViewall">A 5-year-old male presented one year ago to a local hospital with a three months history of headache&#44; vomiting&#44; and irritability that was progressive in course&#46; One month prior to seeking medical advice&#44; he became obtunded and stopped responding to questions and verbal commands&#46; A CT scan was performed and revealed a cystic posterior fossa lesion &#40;<a class="elsevierStyleCrossRef" href="#fig0025">Fig&#46; 5</a>&#41;&#46; The patient was then urgently referred to our hospital&#46; He was a product of a full term pregnancy and uncomplicated vaginal delivery&#46; His Apgar score&#44; head circumference&#44; weight&#44; and fontanels at birth were all normal&#46; His milestones were normal until the time of presentation&#46; His medical&#44; surgical&#44; and family history were unremarkable&#44; and vaccinations were all up to date&#46; His vital signs were normal on examination in our emergency department&#46; His level of consciousness was altered&#44; but he was able to open his eyes spontaneously and obey simple commands&#46; His pupils were equal and reactive&#44; and extraocular muscle and gaze examination revealed paralysis of upward gaze&#46; The remaining cranial nerves&#44; motor functions&#44; and coordination were normal&#46; He was admitted to the pediatric intensive care unit&#44; and an emergency extraventricular drain was inserted&#46; On the 2nd day&#44; he developed a generalized tonic&#8211;clonic seizure before his surgery and was started on lorazepam and phenytoin therapy&#46; Three days later&#44; a suboccipital craniotomy and resection of the posterior fossa tumor were carried out with no complications&#46; Intraoperatively&#44; the tumor was found to be composed of a large loop of intestine with teeth and hair-like structures and was removed completely&#46; The pathology department diagnosed him as a case of mature posterior fossa teratoma&#46; Eight days later from his admission&#44; a ventriculoperitoneal shunt was inserted due to the hydrocephalus and high intracranial pressure on monitoring&#46; 6 weeks later&#44; in the outpatient department&#44; his parents reported no significant complaints&#44; and the neurological examination was normal&#46; The parents expressed their satisfaction regarding his progress and management plan&#46; One year later&#44; an MRI of the brain showed significant improvement of the communicating hydrocephalus with no evidence of tumor recurrence&#46; Six years later&#44; a brain CT was performed and showed significant improvement with no evidence of tumor recurrence &#40;<a class="elsevierStyleCrossRef" href="#fig0025">Fig&#46; 5</a>&#41;&#46;</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">Germ cell tumors are uncommon pediatric brain tumors accounting for 1&#8211;3&#37;&#44; but for unknown reasons&#44; they are more common in Japan and South East Asia &#40;around 10&#37;&#41;&#46; Although rare in infancy and adulthood&#44; teratomas are the commonest congenital brain tumors and also in the first two years of life&#46; They account for up to 50&#37; of fetal brain tumors&#44; 33&#37; of neonatal intracranial tumors&#44; and only 2&#8211;4&#37; of intracranial tumors in people less than 15 years of age&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">2</span></a> Of all the germ cell tumors&#44; only the mature teratoma is considered to be benign&#46; Although intracranial teratomas may occur anywhere in the neuroaxis&#44; they are usually located in the midline region&#44; in or around the pineal gland&#44; third ventricular region&#44; and suprasellar region&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The presenting signs and symptoms of teratoma are variable and related to the size and localization of the tumor&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a> Tumors in the pineal region typically present with signs of obstructive hydrocephalus as a result of increased intracranial pressure&#46; About 25&#8211;50&#37; of patients with pineal region tumors have ophthalmological abnormalities and somnolence and 25&#37; have ataxia&#44; seizures&#44; and behavioral changes&#46; Parinaud&#39;s syndrome &#40;vertical gaze palsy&#41; that is caused by involvement of the adjacent midbrain structures&#44; specifically the rostral interstitial nucleus of medial longitudinal fasciculus&#44; affects about 50&#37;&#46; Other less common manifestations include endocrinopathies and disturbances in the sexual development&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">5</span></a> Tumors of the suprasellar region typically present with hypothalamic&#47;pituitary axis dysfunction including diabetes insipidus&#44; delayed sexual development&#44; isolated growth hormone deficiency&#44; hypopituitarism&#44; and precocious puberty&#46; Other less common signs include ophthalmological abnormalities &#40;e&#46;g&#46; bilateral temporal hemianopsia due to compression of the optic chiasm&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">6</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The diagnosis of intracranial teratomas is based on clinical signs and symptoms&#44; neuroimaging&#44; tumor markers&#44; CSF cytology&#44; and histological assessment&#46; Assay of serum and CSF for &#945;-fetoprotein and beta human chorionic gonadotropin are currently routine in the assessment of suspected teratoma prior to surgery and typically show an elevation of either oncoproteins&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">7</span></a> Both CT and MRI are sensitive in intracranial teratomas&#46; The CT or MRI features of teratomas in the intracranial cavity are non-specific&#44; but a diagnosis can be suspected if the lesion shows intratumoral cysts admixed with calcified regions&#44; heterogeneous density&#44; and foci of low-attenuation similar to fat&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">8</span></a> Although the tumors in our cases were located in the pineal gland&#44; posterior fossa&#44; and cerebral hemisphere&#44; other reported sites of origin include the hypothalamus&#44; ventricles&#44; cavernous sinus&#44; suprasellar region&#44; and cerebellum&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">8</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The management of teratomas usually depends on the histological findings from the biopsy&#46; Total surgical resection is considered the gold standard for the treatment of intracranial teratomas&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">9</span></a> Management of teratomas in the pineal glands includes ventriculoperitoneal shunts and endoscopic third ventriculostomy&#46; Performing endoscopic third ventriculostomy may be the treatment of choice of obstructive hydrocephalus&#44; and some experts would argue that endoscopic third ventriculostomy and biopsy should be performed as the initial management for a pineal mass&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">10</span></a> The follow-up period after resection may be as long as ten years due to a possible metachronous occurrence&#46; In addition&#44; a combination of chemotherapy and radiation therapy are often used&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">11</span></a> Mature teratoma is more resistant to chemotherapy and radiotherapy than any other germ cell tumor&#46; It is not unusual to operate in a &#8220;second look&#8221; surgery for a mature teratoma because residual tumor is frequent after chemotherapy and radiotherapy&#46; The prognosis is dependent on location&#44; size&#44; and surgical experience&#46;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">12</span></a></p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusion</span><p id="par0045" class="elsevierStylePara elsevierViewall">The present cases in our series are interesting examples of intracranial teratoma in terms of location of the tumor and neuroimaging findings&#46; Total surgical resection is the treatment of choice for intracranial teratomas&#46; However&#44; preoperative&#44; intraoperative&#44; and postoperative medical care and good preparation for surgery are of paramount importance&#46; This includes acid&#8211;base balance&#44; electrolyte disturbances&#44; and preparation for an unexpected large amount of blood loss&#46; Surgical experience is a prerequisite for a good clinical outcome&#46; Delay in surgical intervention may complicate the course of the disease with progressive enlargement of tumors and development of complication including hydrocephalus&#46; Using endoscopic surgical techniques may emerge as the preferred intervention option as compared to other traditional methods&#46; We recommend the establishment of a national and international registry for intracranial tumors&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conflict of interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Teratoma is a common form of germ cell tumors composed of multiple tissues foreign to the site in which arise with a histological representation of all three germ cell layers&#46; Intracranial teratomas are very rare&#46; In this study&#44; we report three cases of intracranial teratomas with an interesting clinical course&#44; neuroradiology&#44; and outcome&#46; In addition&#44; we review the literature and convey important messages to the neuroscience community regarding issues related to the management of these rare tumors&#46; The present cases are interesting examples of intracranial teratoma in terms of location of the tumor and neuroimaging findings&#46; Delay in surgical intervention may complicate the course of the disease with progressive enlargement of tumors and development of complication including hydrocephalus&#46; Using endoscopic surgical techniques may emerge as the preferred intervention option as compared to other traditional methods&#46; We recommend the establishment of a national and international registry for intracranial tumors&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El teratoma es un tipo frecuente de tumor de c&#233;lulas germinales compuesto por m&#250;ltiples tejidos ajenos a la zona en que aparecen con una representaci&#243;n histol&#243;gica de las 3 capas de c&#233;lulas germinales&#46; Los teratomas intracraneales son muy poco frecuentes&#46; En este estudio&#44; se presentan 3 casos de teratomas intracraneales con una evoluci&#243;n cl&#237;nica&#44; neurorradiolog&#237;a y resultado interesantes&#46; Adem&#225;s&#44; se revisa la bibliograf&#237;a y se difunde un mensaje importante a la comunidad de las neurociencias respecto a los problemas relacionados con el tratamiento de estos tumores raros&#46; Estos casos son ejemplos interesantes de teratoma intracraneal en t&#233;rminos de localizaci&#243;n de los hallazgos tumorales y de neuroimagen&#46; El retraso en la intervenci&#243;n quir&#250;rgica puede complicar la evoluci&#243;n de la enfermedad con la ampliaci&#243;n progresiva de los tumores y el desarrollo de complicaciones&#44; especialmente hidrocefalia&#46; El uso de t&#233;cnicas quir&#250;rgicas endosc&#243;picas aparece como la opci&#243;n de intervenci&#243;n preferida en comparaci&#243;n con otros m&#233;todos tradicionales&#46; Se recomienda el establecimiento de un registro nacional e internacional de tumores intracraneales&#46;</p></span>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">CT of the brain showing a large intra-axial heterogeneous left hemispheric mass with extensive internal areas of calcification occupying the left parietal lobe with an involvement of the left occipital and temporal lobes causing obliteration of the ventricular system and left to right midline shift of about 1&#46;5<span class="elsevierStyleHsp" style=""></span>cm&#46;</p>"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">CT scan of the brain showing a cystic posterior fossa lesion causing acute hydrocephalus preoperatively &#40;the upper raw&#41; and significant improvement with no evidence of tumor recurrence six years postoperatively &#40;the lower raw&#41;&#46;</p>"
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