Cerebral Malignant Tumors with Ependymal and Choroidal Differentiation in Two Siblings

Neurosurgery ◽  
1988 ◽  
Vol 22 (5) ◽  
pp. 928-933 ◽  
Author(s):  
David G. Munoz ◽  
Robert Griebel ◽  
Bohdan Rozdilsky ◽  
David George
Keyword(s):  
Choroid Plexus ◽  
Posterior Fossa ◽  
Cerebral Hemisphere ◽  
Malignant Tumors ◽  
Rare Tumor ◽  
Choroid Plexus Epithelium ◽  
Left Cerebral Hemisphere ◽  
Inherited Predisposition ◽  
History Of ◽  
Immunocytochemical Studies

Abstract Two siblings in a family without a history of phacomatosis or cerebral tumors developed malignant tumors in the posterior fossa at age 28 months and in the left cerebral hemisphere at age 15 months, respectively. Dual ependymal and choroid plexus epithelium differentiation was established by histological, ultrastructural, and immunocytochemical studies. The development of this rare tumor in siblings suggests an inherited predisposition, a common environmental insult, or both.

scholarly journals BT-05 A CASE OF GLIOMATOSIS CEREBRI WITH TOTAL SPINAL CORD EXTENSION

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii37-ii37
Author(s):  
Yasuyoshi Chiba ◽  
Osamu Takemoto ◽  
Junji Yamada ◽  
Takaki Matsumura
Keyword(s):  
Spinal Cord ◽  
Cerebral Hemisphere ◽  
Tumor Volume ◽  
Spinal Cord Tumor ◽  
Gliomatosis Cerebri ◽  
Eye Enucleation ◽  
Left Cerebral Hemisphere ◽  
First Occurrence ◽  
History Of ◽  
Important Disease

Abstract INTRODUCTION Although gliomatosis cerebri is no longer a pathological diagnostic name, it is still an important disease state as a gliomatosis cerebri growth pattern. Here we report a case of gliomatosis cerebri originating from the left cerebral hemisphere that developed whole spinal cord. CASE A 10-year-old boy. He has a history of 13q-syndrome and left retinoblastoma. Left eye enucleation and chemotherapy (modified 98A1) have been performed. MRI in July 2017 showed no abnormalities, but in September 2018, he developed epilepsy. MRI revealed a gliomatosis cerebri that spreads extensively in the left cerebral hemisphere. Biopsy revealed anaplastic astrocytoma (MIB-1 LI; 22%, IDH1 / 2; WT, TERT C228T mutation positive) and IMRT (59.4Gy) and temozolomide (Stupp regimen) were performed in December 2018. In June 2019, neck pain developed. Head and neck MRI revealed that the tumor in the head increased lightly, and there was no suspicion of tumor growth in the brainstem, but the tumor progressed to the entire spinal cord. Therefore, radiation therapy was started from the lower brainstem that had not been irradiated last time to the entire spinal cord, and administration of bevacizumab was started. DISCUSSION Based on the single cell origin theory, the left hemisphere tumor and spinal cord tumor should be continuous. Since gliomatosis cerebri is visualized on MRI only after the tumor volume has increased and edema has occurred, it may appear as if there is no tumor in between. The spinal cord MRI was not taken, so it is only speculation, but it seems that tumor cells had probably infiltrated the spinal cord from the beginning, and it seems that it gradually increased because it was not irradiated. Considering the possibility of remote invasion as in this case, it is necessary to consider taking MRI of spinal cord at the first occurrence.

Organization of tight junctions in the choroid plexus epithelium

1978 ◽  
Vol 36 (2) ◽  
pp. 336-337
Author(s):  
B. Van Deurs ◽  
J. K. Koehler
Keyword(s):  
Choroid Plexus ◽  
Present Report ◽  
Freeze Fracture ◽  
Barrier Properties ◽  
Cell Junctions ◽  
Structural Basis ◽  
Apical Surface ◽  
Choroid Plexus Epithelium ◽  
Solid Nitrogen ◽  
Special Composition

The choroid plexus epithelium constitutes a blood-cerebrospinal fluid (CSF) barrier, and is involved in regulation of the special composition of the CSF. The epithelium is provided with an ouabain-sensitive Na/K-pump located at the apical surface, actively pumping ions into the CSF. The choroid plexus epithelium has been described as “leaky” with a low transepithelial resistance, and a passive transepithelial flux following a paracellular route (intercellular spaces and cell junctions) also takes place. The present report describes the structural basis for these “barrier” properties of the choroid plexus epithelium as revealed by freeze fracture.Choroid plexus from the lateral, third and fourth ventricles of rats were used. The tissue was fixed in glutaraldehyde and stored in 30% glycerol. Freezing was performed either in liquid nitrogen-cooled Freon 22, or directly in a mixture of liquid and solid nitrogen prepared in a special vacuum chamber. The latter method was always used, and considered necessary, when preparations of complementary (double) replicas were made.

scholarly journals Endometriosis Mimicking a Cecum Mass with Complete Bowel Obstruction: An Infrequent Cause of Acute Abdomen

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Gabriel A. Molina ◽  
Darwin R. Ramos ◽  
Alberto Yu ◽  
Patricio A. Paute ◽  
Paul S. Llerena ◽  
...  
Keyword(s):  
Intestinal Obstruction ◽  
Bowel Obstruction ◽  
Preoperative Diagnosis ◽  
Malignant Tumors ◽  
Signs And Symptoms ◽  
Final Diagnosis ◽  
Intestinal Involvement ◽  
History Of ◽  
Ovarian Abscess ◽  
Fertile Women

Endometriosis is a common entity among fertile women which unfortunately manifests through variable symptomatology. Intestinal involvement in endometriosis is quite common and can simulate several diseases such as Crohn’s disease, appendicitis, tubo-ovarian abscess, or malignant tumors. Intestinal obstruction due to endometriosis is rare, and preoperative diagnosis is difficult because the signs and symptoms are nonspecific and can be easily confused. In the case of patients without a history of endometriosis, diagnosis is further complicated. We present a case of a 41-year-old female patient. She presented to the emergency room with complete bowel obstruction and a mass in the cecum. Surgery was decided, and the patient underwent full recovery. Endometriosis was the final diagnosis for the observed condition.

scholarly journals Ovarian epithelioid angiosarcoma complicating pregnancy: a case report and review of the literature

2021 ◽  
Vol 49 (5) ◽  
pp. 030006052110196
Author(s):  
Xiaotong Peng ◽  
Zhi Duan ◽  
Hongling Yin ◽  
Furong Dai ◽  
Huining Liu
Keyword(s):  
Adverse Events ◽  
Soft Tissues ◽  
Malignant Tumors ◽  
Cystic Teratoma ◽  
Abdominal Distension ◽  
Epithelioid Angiosarcoma ◽  
History Of ◽  
New Ideas ◽  
The Right ◽  
Clinical Awareness

Epithelioid angiosarcoma is a rare and highly aggressive soft tissue angiosarcoma most commonly arising in the deep soft tissues. Given that abundant vascular cavities anastomose with each other, most angiosarcomas prone to metastasis recur quickly, and the overall prognosis is poor. We report a 25-year-old woman at 24 weeks’ gestation who presented with a 1-month history of abdominal distension. Ultrasonography suggested a mass in the right adnexa, and she underwent two operations owing to uncontrolled intraperitoneal bleeding with progressive anemia. The right ovarian tumor and right adnexa were removed successively. Biopsy yielded a diagnosis of primary epithelioid angiosarcoma with mature cystic teratoma. The patient died from uncontrolled progressive bleeding 1 week after the second operation. This case revealed that epithelial angiosarcoma is a highly malignant endothelial cell tumor. The results of surgery and chemoradiotherapy tend to be poor, and the recurrence rate is high. The purpose of this study is to raise clinical awareness of epithelial angiosarcoma and its adverse events and to provide new ideas for the treatment of these adverse events. Immunohistochemical staining of pathological specimens can facilitate diagnosis. Pregnancy with malignant tumors may lead to rapid disease progression, extensive lesions, and a poor prognosis.

scholarly journals T-Lymphocytes Traffic into the Brain across the Blood-CSF Barrier: Evidence Using a Reconstituted Choroid Plexus Epithelium

PLoS ONE ◽  
2016 ◽  
Vol 11 (3) ◽  
pp. e0150945 ◽  
Author(s):  
Nathalie Strazielle ◽  
Rita Creidy ◽  
Christophe Malcus ◽  
José Boucraut ◽  
Jean-François Ghersi-Egea
Keyword(s):  
T Lymphocytes ◽  
Choroid Plexus ◽  
Choroid Plexus Epithelium ◽  
The Brain

scholarly journals Primary Occipital Ewing’s Sarcoma with Subsequent Spinal Seeding

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Ali Alqahtani ◽  
Roaa Amer ◽  
Eman Bakhsh
Keyword(s):  
Posterior Fossa ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Bone Cancer ◽  
Surgical Excision ◽  
Good Prognosis ◽  
Bone Lesions ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Whole Spine

Ewing’s sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing’s sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT) imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI) showed cerebrospinal fluid (CSF) seeding from the L5 to the S4 vertebrae. Primary cranial Ewing’s sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing’s sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing’s sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

scholarly journals Posterior Fossa Arachnoid Cyst Masking a Delayed Diagnosis of Hyperparathyroidism in a Child

2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
B. Dhamija ◽  
D. Kombogiorgas ◽  
I. Hussain ◽  
G. A. Solanki
Keyword(s):  
Differential Diagnosis ◽  
Primary Hyperparathyroidism ◽  
Posterior Fossa ◽  
Arachnoid Cyst ◽  
Hospital Admissions ◽  
Delayed Diagnosis ◽  
Visual Disturbance ◽  
Organ Damage ◽  
Presenting Symptoms ◽  
History Of

Background. Primary hyperparathyroidism in childhood is a very rare entity, often being diagnosed late after the onset of its presenting symptoms. It most commonly affects patients in their fourth decade of life and beyond. The inclusion of primary hyperparathyroidism in the differential diagnosis is necessary when evaluating patients presenting with nonspecific symptoms such as polyuria, fatigue, weight loss, abdominal pain, nausea, and vomiting.Methods. We report the case of an eleven-year-old girl presenting with three years history of headaches, visual disturbance, along with episodes of emotional lability. Neuroimaging confirmed a large posterior fossa arachnoid cyst. It was decided to manage this lesion conservatively with surveillance. Only after further hospital admissions with recurrent loss of consciousness, dizziness, and nausea to add to her already existing symptoms, a full biochemical and endocrine assessment was performed to look for more specific causes for her presentation. These pointed to a diagnosis of primary hyperparathyroidism.Conclusions. The inclusion of primary hyperparathyroidism in the differential diagnosis should be considered when evaluating paediatric patients presenting with nonspecific (neurological, gastrointestinal, and renal) symptoms in order to establish a prompt diagnosis of the disorder and to avoid severe complications of prolonged hypercalcaemia and end-organ damage.

A discussion of apraxia, aphasia, and gestural language

1984 ◽  
Vol 246 (6) ◽  
pp. R884-R887
Author(s):  
N. Helm-Estabrooks
Keyword(s):  
Sign Language ◽  
Cerebral Hemisphere ◽  
Communication Disorder ◽  
Left Cerebral Hemisphere ◽  
Language Behavior ◽  
Normal Language ◽  
Symbolic Gestures ◽  
The Many ◽  
The Relationship ◽  
The Brain

It is understood that damage to the left cerebral hemisphere in adulthood may result in syndromes of language disturbances called the aphasias. The study of these syndromes sheds light on normal language processes, the relationship between language behavior and the brain, and how best to treat aphasic individuals. Aphasia, for some, is a central communication disorder affecting all symbolic behavior in all modalities (i.e., speech, writing, and gesture). Difficulty producing symbolic gestures on command is called apraxia. Others view aphasia as a manifestation of a motor-sequencing disorder affecting all gestural systems including those required for speech movements. These divergent theories of the underlying nature of aphasia can be tested through examination of deaf individuals who use sign language before onset of aphasia. Poizner et al. [Am. J. Physiol. 246 (Regulatory Integrative Comp. Physiol. 15): R868-R883, 1984] studied three such patients with different aphasia syndromes: one patient had a nonsymbolic, motor-sequencing disorder; one had a gestural apraxia; and one had neither. These findings force the conclusion that neither the symbolic nor motor-sequencing theory of aphasia can account for the many varieties of that disorder.

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