Carcinomatosis meníngea secundaria a melanoma metastásico: reporte de un caso / Meningeal carcinomatosis secondary to metastatic melanoma: a case report

Resumen La carcinomatosis meníngea es una entidad poco frecuente, que puede formar parte de la historia natural de muchos procesos neoplásicos. Se presenta habitualmente con síntomas poco específicos, como cefalea, cambios en la conducta o alteraciones motoras y sensitivas. A continuación, presentamos el caso de una paciente con carcinomatosis meníngea por melanoma metastásico y su evolución clínica.

La carcinomatosis meníngea es una entidad poco frecuente, que puede formar parte de la historia natural de muchos procesos neoplásicos. Se presenta habitualmente con síntomas poco específicos, como cefalea, cambios en la conducta o alteraciones motoras y sensitivas. A continuación, presentamos el caso de una paciente con carcinomatosis meníngea por melanoma metastásico y su evolución clínica.

Isolated Medical Rectus Palsy as an Initial Presentation of Non Hodgkin Lymphoma

Introduction: Cranial nerves, leptomeninges and cavernoussinus are often involved in lymphomas. Isolated oculomotornerve palsy as the first manifestation of a lymphoma is rare,particularly when none of its other manifestations are initiallyidentified.Case report: A 31 year old man with no known co-morbidities,came with complaints of acute onset of blurring of visionand drooping of the right eyelid. Neurological examinationrevealed isolated right medial rectus palsy with dilated pupiland ptosis of right eyelid, suggesting complete oculomotornerve palsy. CT Brain, CT Angiogram and CSF study werenormal.Chest X Ray showed mediastinal widening and CECTThorax showed anterior mediastinal mass. CT guided biopsysuggested lymphoproliferative disorder. IHC was diagnosticfor Thymoma. The mass was resected. Ten days later, thepatient returned, with worsening of his symptoms. Neurologicexamination showed third, fourth and sixth cranial nervepalsy. IHC of the resected specimen revealed high gradeB cell Lymphoblastic Lymphoma. Repeat CSF analysisshowed leptomeningeal involvement. Patient was started onchemotherapy for high grade lymphoma and his extra ocularmovements improved. One week later, he developed bilateralfacial palsy and Left CN IX, X Palsy. Chemotherapy wascontinued until he was discharged at request. Patient was lostto follow up.Conclusion: This atypical presentation of NHL can bringabout a delay in the diagnosis due to the variability of itspresenting symptoms and wide differential diagnosis. Thusearly diagnosis and aggressive management is essential.

Síndrome De Sturge-Weber: Reporte De Un Caso Y Revisión De La Literatura / Sturge-Weber Syndrome: A Case Report And Literature Review

Resumen El Síndrome de Sturge-Weber es un trastorno poco común del desarrollo neuroectodérmico, caracterizado por un angioma facial tipo nevus flammeus y una angiomatosis leptomeníngea, con frecuencia ipsilateral al nevus. Este síndrome predispone a calcificaciones, atrofia cerebral y convulsiones refractarias. Propósito: En este artículo se realiza una revisión de la literatura sobre el Síndrome de Sturge-Weber y se reporta el caso de un paciente de 18 meses de edad diagnosticado con esta patología que ingresa a urgencias por presentar cuadro febril de tres días y comienzo de convulsiones tónico clónicas localizadas en hemicuerpo derecho refractarias al tratamiento convencional; en esta revisión se resalta la importancia del diagnóstico y manejo oportuno al igual que un adecuado seguimiento. Desarrollo: se realizaron búsquedas en las bases de datos PubMed, Science Direct y Scielo, confirmando que aún se desconocen algunos aspectos de esta patología, sin embargo, con el descubrimiento de la mutación somática de GNAQ hay un amplio campo para próximas investigaciones. Hallazgos y conclusiones: Es importante en el ejercicio médico no pasar de alto las lesiones angiomatosas que posean una ubicación trigeminal en los recién nacidos, con el fin de establecer un diagnóstico oportuno e intentar conseguir un mejor desarrollo a futuro.

Abstract Sturge-Weber Syndrome is a rare developmental neuroectodermical disorder. It is characterized by a facial port-wine stain and a leptomeningeal angiomata, frequently localized ipsilateral to the facial port-wine stain. This syndrome predisposes either to brain atrophy, calcifications and refractory seizures. In this paper a Sturge-Weber Syndrome literature review was made and a 18 month aged child case with this diagnosis is reported. He was admitted to the emergency department of a local hospital with a history of three days of fever and tonic-clonic seizures localized on the right side and refractory to conventional treatment. This review highlights the importance of an early diagnosis and an appropriate follow up. To carry out this review a search in PubMed, Science Direct and Scielo databases was done, confirming that there are some issues about this disorder that are still unknown. However, with the GNAQ somatic mutation discovery, there is an open field for new researches. It is very important in medical practice not to understimate a facial port-wine stain over trigeminal territory in newborns in order to make an early diagnosis and try to achieve a better future neurodevelopment.

Clinical characteristics of 15 glioma cases with leptomeninges and spinal cord metastases / 中国肿瘤临床

Objective:To retrospectively analyze and summarize the clinical characteristics of 15 glioma cases that led to leptomenin-ges and spinal cord metastases in Department of Glioma, Beijing Shijitan Hospital, Capital Medical University since 2011. Methods:A total of 15 cases were considered, including 5 patients with World Health Organization gradeⅡ, 6 patients with gradeⅢ, and 4 pa-tients with gradeⅣ. One patient had a tumor at the brain stem, two patients had tumors at the spinal cords, and the other patients had tumors at the hemispheres. One case received biopsy, 4 cases received subtotal resection, and 10 cases received complete resec-tion. Results: Symptoms included low back pain, sensory and motor dysfunction, incontinence, and seizures. After the metastases spread to the cerebrospinal region, patients were treated with chemotherapy, whole spine radiotherapy, intrathecal chemotherapy, and target therapy. The median time of leptomeninges and spinal cord metastasis dissemination appearance was 10 months (1.5-80 months) since surgery. The median overall survival time of the 15 patients was 20 months (9-83 months), and the median survival time was 6 months (2-48 months) after leptomeninges and spinal cord metastases. Conclusion:The prognosis of glioma patients with lepto-meninges and spinal cord metastases was poor, and a proportion of the patients who received appropriate treatment might have a better survival.

Primary Intracranial Leptomeningeal Melanomatosis

Primary intracranial malignant melanoma is a very rare and highly aggressive tumor with poor prognosis. A 66-year-old female patient presented a headache that had been slowly progressing for several months. A large benign pigmented skin lesion was found on her back. A brain MRI showed multiple linear signal changes with branching pattern and strong enhancement in the temporal lobe. The cytological and immunohiostochemical cerebrospinal fluid examination confirmed malignant melanoma. A biopsy confirmed that the pigmented skin lesion on the back and the conjunctiva were benign nevi. We report a case of primary intracranial malignant melanoma and review relevant literatures.

Primary Meningeal Melanocytoma in the Thoracic Spine: A Case Report

Meningeal melanocytoma is a rare benign melanotic tumor arising from melanocytic cells in the leptomeninges. Preoperative differential diagnosis of meningeal melanocytoma from other melanotic tumors is difficult based on magnetic resonance imaging (MRI). Definitive diagnosis of meningeal melanocytoma from other melanotic tumors is done on the basis of histopathological and immunohistochemical analyses. The prognosis of this tumor is not always favorable with occasional local recurrence, especially in cases of subtotal gross resection. The authors report on a case of a 58-year-old man who presented with a melanocytoma located at the T7-8 level. The patient underwent on a total surgical excision for successful control of the tumor. During a follow-up period of 2 years, the patient presented no signs of recurrence. We emphasize the importance of discrimination of meningeal melanocytoma from other melanotic tumors to facilitate appropriate treatment. Complete resection is the treatment of choice for spinal melanocytoma. Radiation therapy should be reserved for those cases in which complete resection is not possible or in which there is recurrence.

Extramedullary B Lymphoblastic Crisis of CML, Presenting as a Leptomeningeal Tumor: A Case Report

We report here on a rare case of a patient who presented with an extramedullary B lymphoblastic crisis as an initial manifestation of chronic myelogenous leukemia (CML). A 71-year-old man visited the emergency room due to suddenly developed dysarthria and right side weakness. Emergency craniotomy was done under the presumptive diagnosis of subdural hemorrhage. During the operation, a poorly demarcated firm mass was identified in the leptomeningeal space. Microscopically, the majority of the tumor was composed of monotonous immature cells with blast morphology, and these cells were immunoreactive for TdT, CD34, CD10 and CD20, indicating the precursor B-cell phenotype. The peripheral area of the tumor consisted of myeloid cells in various stages of maturation, and these cells were reactive for myeloperoxidase, chloroacetate esterase, CD43 and CD15. FISH analysis using the LSI bcr-abl dual color probe showed gene fusion signals in both the B-lymphoblasts and myeloid cells. The peripheral blood and bone marrow findings were consistent with CML with no evidence of a blast crisis. Cytogenetic study of the bone marrow demonstrated the 46, XY, t(9;22)(q34;q11) chromosome. A diagnosis of extramedullary B lymphoblastic blast crisis in a patient with Philadelphia chromosome-positive CML was made. Despite treatment, the patient died 3 months after he was diagnosed.

Primary Leptomeningeal Glioblastomatosis Detected in Cerebrospinal Fluid Cytology: A Case Report / 대한세포병리학회지

Primary leptomeningeal glioblastomatosis is a rare and fatal tumor of the central nervous system, the condition is characterized by diffuse infiltration of the tumor in the meninges without evidence of primary tumor within the brain or spinal cord. We reported an unusual case of leptomengial glioblastomatosis, which was detected by the consecutive cerebrospinal fluid (CSF) cytology with application of immunohistochemistry, in addition to its cytologic findings. A healthy 21 year old man, who was enlisted in the army, presented with a stuporous mental state and diffuse enhancement of meninges without evidence of primary mass lesion in the brain and spinal cord on magnetic resonance imaging(MRI). CSF cytology showed small loose clusters of tumor cells with single cells and lymphocytes. The tumor showed variable pleomorphism with coarse chromatin, irregular nuclear membranes and multi lobated nuclei. On immunohistochemical staining, the tumor cells were founded to be positive for GFAP. In conjunction with radiologic findings, brain biopsy confirmed the diagnosis of leptomenigeal glioblastomatosis. The use of immunohistochemistry is helpful in confirming CSF cytologic diagnosis in patients with primary leptomeningeal glioblastomatosis.

A Case of Primary Intracranial Malignant Melanoma

The primary malignant melanoma arising in the leptomeninges of the central nervous system is extremely rare. We report a case of malignant melanoma, apparently thought to be originated from leptomeninges, with diffuse neoplastic process widely spreaded over the leptomeninges of 42 years old man, who had suffered severe headache, nausea and left hemiparesis. This patient is free of the symptoms for six months after operation. The literature on this subject is briefly reviewed.

Two Cases of Primary Intracranial Melanoma

Primary malignant melanoma arising from the leptomeninges is a rare entity. We report two cases of primary intracranial melanoma developed in one aged 65 years female and the other 70 years male. One case died 2 years after the operation, and the other case is still living more than 1 year after operation. Both cases seems to have a relatively long survival. The literature on this subject is briefly reviewed.

An Experimental Study about the Effect of Urokinase Injected into the Ventricle on Intraventricular Hemorrhage

For the reduction of morbidity, such as an acute hydrocephalus, following intraventricular hemorrhage, we attempted to produce a lysis of the experimental intraventricular hemorrhage by a direct intraventricular injection of fibrinolytic agent. Urokinase was used as the fibrinolytic activator. Sixty-four adult rabbits were used in this study. The animals were divided into 5 groups to investigate the effect of urokinase in different time interval of urokinase injection. Intraventricular hemorrhage was made by an injection of 0.3cc of autogenous venous blood. Group I was the control group in which intraventricular injection of blood or urokinase was only done. In Group II and III urokinase was injected into the ventricule 30 minutes and 2 hours after the blood injection. In Group IV urokinase was injected into the ventricle at the same time of the blood injection. In Group V urokinase was injected into the ventricle at the same time of the blood injection, and then the urokinase injection was repeated 24 hours later. The animals of each group were sacrificed on the 1st, 2nd, 3rd and 7th day successively after the experimental procedures. The brains were examined to observe the outcome of intraventricular hematoma with urokinase injection and the consequent changes of the ventricular system grossly and microscopically. The results were as follows : The duration of the remaining hematoma in the ventricles and basal cisterns was 7 days in both the control and the urokinase injection groups equally. In the group of repeated urokinase injection the duration of remaining hematoma in the ventricular system was shortened to 5 days after the blood injection. Upon the ventricular dilatation, the blood injection control group showed moderate dilatation persistently for 1 week ; from minimal to moderate dilatations were found in the urokinase injection groups. In the microscopic examination there were no definite abnormal changes on the ventricular walls and leptomeninges throughout 1 week in the urokinase injection control group. The group of repeated urokinase injection revealed mild flattening and denudation of the ependyma of the ventricular system than the group of single urokinase injection.