An Unusual Dermoid Cyst of the Pineal Region: Case Report in a Child

Introduction The pineal gland is a common location for intracranial germ cells, but dermoids are not commonly observed in this area. In the present paper, we discuss the clinical and radiological features as well as the treatment and outcome of this cyst in a 6-year- old child with a pineal dermoid cyst. Case Presentation The patient presented with chronic headache 6 months before admission in 2018. On the first admission, an enhanced lesion with a small cyst was detected in brain imaging. Magnetic resonance imaging (MRI) of the brain at follow-up (2 months after the first presentation) showed enlargement of the cyst size with compression on the adjacent structures. Radical excision of the tumor was performed after the endoscopic biopsy due to pressure exerted on the adjacent structures. Conclusion Dermoid cyst should be considered as a differential diagnosis for enhanced lesions of the pineal region.

Tumor pineal resuelto por Abordaje Infratentorial Supracerebeloso Endoscópico. Reporte de un caso y revisión de la literatura / Pineal tumor resolved by Endoscopic Supracerebellar Infratentorial Approach. Case report and literature review

Introducción: Los tumores de la región pineal constituyen un grupo heterogéneo de lesiones localizadas en la porción posterior del tercer ventrículo bajo el esplenio del cuerpo calloso y encima de la lámina cuadrigémina. Dentro de estos, los tumores del parénquima pineal constituyen un grupo frecuente. En estas lesiones no existe actualmente un protocolo establecido debido a la ausencia de grandes series. Se sugiere una combinación de cirugía y radioterapia con o sin quimioterapia. El tratamiento de elección es la resección total, sin embargo, muchas veces no es posible por dificultades técnicas. En este contexto, la cirugía con visualización endoscópica puede contribuir a lograr este objetivo. Descripción del caso: Se presenta un paciente masculino de 22 años de edad con lesión de región pineal e hidrocefalia obstructiva triventricular al que se le practicó una tercerventriculostomía endoscópica y un abordaje infratentorial supracerebeloso con total visualización endoscópica. Se describe la técnica quirúrgica y se realiza un análisis crítico de la literatura actualizada. Conclusiones: Los tumores de la región pineal constituyen un reto terapéutico. La resección total es el principal objetivo en lesiones de grado intermedio o bajo de malignidad. El abordaje infratentorial supracerebeloso con total visualización endoscópica es efectivo en la resección quirúrgica de estas lesiones al mejorar la visualización y minimizar la retracción cerebelosa

Introduction: Pineal region tumors are a variable group of lesions located in the posterior wall of the third ventricle under the corpus callous splenium and above the tectal plate. Pineal gland tumors are frequent. There is not a standard protocol in these lesions due the lack of large series. A combination of surgery and chemo therapy or radiotherapy are recommended. The complete surgical resection is the treatment of choice. However, usually it is not possible to accomplished this goal due to technical limitations. In this scenario, the endoscopic visualization could contribute to archive the goal. Patient characteristics: A 22 years old young male patient with a pineal region lesion and obstructive hydrocephalus is presented. A third ventriculostomy was performed and an infratentorial supracerebelous approach with fully endoscopic visualization. The surgical technique is described and a critical review of literature is performed. Conclusions: Pineal region tumors represents a therapeutic challenge. Total removal is the most important objective in intermediate or low-grade lesions. The infratentorial supracerebelous approach with full endoscopic visualization is effective and improve the visualization while reducing the cerebellar retraction.

An Unusual Variant of Anlage Tumor of Pineal Region in an Infant

A 9-month-old male child was brought with complaints of increasing head size for 2 months, increasing lethargy and vomiting for the last 2 days. Radiology revealed a heterogeneously enhancing, globular lesion in the pineal region with hydrocephalus. Near total excision of the tumor was carried out. The histopathological examination of the lesion showed heterogenous elements in the form of mature neuroepithelial and ectomesenchymal tissue. The pathology and radiology of this unusual lesion is discussed with relevant review of literature.

Cerebellar Mutism After Pineal Tumour Excision: A Case Report.

Cerebellar mutism syndrome (CMS) was first described by Rekate et al in 1985. This syndrome is a common complication of posterior fossa surgery in children with range of 11-29% and usually manifests as diminished speech, hypotonia, and ataxia. The cause is due to bilateral pertubation of the dentate nuclei and their efferent pathways by edema, perfusional defects, axonal damage or metabolic disturbances. Other rare causes of CMS like acute subdural hematoma of the posterior fossa, head injury, brainstem glioma surgery, meningitis and basilar artery occlusion have also been reported. CMS after supracerebellar resection of the pineal tumor is a very rare with very few cases reported. We report such a case in a 10- year old boy who underwent excision of a pineal tumor through the infratentorial supracerebellar route.

Cyst of Pineal Gland

With advances in MR imaging, reports of pineal cyst have become more common. In contrast to pineal neoplasm, a pineal cyst is usually asymptomatic and is detected incidentally. We reviewed six cases of pineal cyst diagnosed between 1991 and 1996. Only one patient presented with syncopal attack due to compression of the midbrain. MRI typically reveals a cystic mass with a mean diameter of 1.66cm and shows faint rim-like contrast enhancement. Since it shows the anatomic relationship of the cyst to the aquedect, sagittal MRI is the most useful diagnost test. In the one symptomatic patient, we performed suboccipital cramiectomy and removed the cyst. It contained clear fluid and was easily removed. Surgery can be considered only when the lesion produces symptoms due to compression of the quadrigeminal plate, aqueduct, or midbrain. The long term behavior of these lesions is, however, unknown, so only with follow-up for long time will appropriate management techniques become apparent.

Radiotherapy of Pineal and Ectopic Pineal Tumors / 대한치료방사선과학회지

From December 1984 to February 1990, 16 patients with tumors of pineal and suprasellar location were treated with radiation therapy. Tissue diagnoses were obtained before radiation therapy in 5 patients and 11 were irradiated without histologic confirmation. Initial treatments for these patients were craniospinal plus boost primary irradiation(six), whole brain plus boost primary irradiation(nine), primary tumor site irradiation(one). The 5 year actuarial survival rate is 71%. Three cases with elevated beta-human chorionic gonadotropin (HCG) responded favorably to radiation, but pineal tumors with elevated alpha-fetoprotein(AFP) did not respond well. Spinal metastasis developed in 2 cases (2/15) with elevated AFP : one received prophylactic spinal irradiation, another did not. Our studies suggest that more aggressive treatment would be necessary in patient with elevated AFP and in this patient, radiation therapy may be initiate without pathologic confirmation. From the result of our study, routine use of prophylactic spinal irrdiation for all patients with pineal region tumor is not indicated and use of prophylactic spinal irrdiation is considered for the patients with positive craniospinal fluid cytology, meningeal seeding, disease extension along the ventricular wall and biopsy proven germinoma

Treatment of Pineal Region Tumors and CNS Germ Cell Tumors ; Evolution of Treatment Policy and Results

A retrospective analysis has been made of 40 patients with pineal region tumors and CNS germ cell tumors who were treated at Yonsei University Hospital, Department of Radiation Oncology between 1971 and 1985. A tissue diagnosis was obtained before radiotherapy in 19 patients and 21 patients were irradiated without histological diagnosis. Among 19 biopsy-proven cases, 14 were germinomas, 2 were teratomas, and the others were two pineocytomas and one pineoblastoma. In the earlier period, every attempt was made to obtain a tissue pathology by either stereotaxic biopsy or open craniotomy before irradiation. However, in recent years, with the advent of CT scan, a trial radiotherapy with a modest dose of 20 Gy in 2 weeks was attempted in cases of highly suspected germinomas by CT scan findings. Further management after trial radiation depended on the radiation response shown on the follow-up CT scan and tumor marker study. Radiation fields varied from a small local field to whole brain or entire neuroaxis irradiation. Most patients received 40-50 Gy to the primary tumor site and 20-30 Gy to the neuroaxis. Twenty-nine of the total 40 patients are alive without of disease 22-144 months after treatment and the overall 5-year recurrence-free survival rate was 74.4%, Univariate analysis of prognostic factors at presentation showed that tumor type was highly correlated with outcome. Two of fourteen biopsy-proven germinomas and none of nine presumed germinomas by trial radiation recurred. On the other hand, five of six patients who showed poor response to trial radiation died of uncontrolled disease and only one patient with elevated AFP in serum and CSF was salvaged by chemotherapy. On the basis of the results of this study, application of trial radiation therapy without tissue biopsy is well justified as a treatment modality in a suspected germinoma by CT scan finding. Aggressive combined modality approaches with surgery, radiotherapy and chemotherapy need to be investigated to improve results in radioresistant tumors.