Brain tumor imaginig 2 3rd may 02

4,645 views

Published on

2 Comments
7 Likes
Statistics
Notes
No Downloads
Views
Total views
4,645
On SlideShare
0
From Embeds
0
Number of Embeds
6
Actions
Shares
0
Downloads
348
Comments
2
Likes
7
Embeds 0
No embeds

No notes for slide
  • Table 1: World Health Organization proposed new classification of CNS tumors I. Tumors of neuroepithelial tissue A. Astrocytic tumors Astrocytoma Variants: fibrillary, protoplasmic, gemistocytic, mixed Anaplastic (malignant) astrocytoma Glioblastoma Variants: giant cell glioblastoma, gliosarcoma Pilocytic astrocytoma Pleomorphic xanthoastrocytoma Subependymal giant cell astrocytoma B. Oligodendroglial tumors Oligodendroglioma Anaplastic (malignant) oligodendroglioma C. Ependymal tumors Ependymoma Variants: cellular papillary, epithelial, clear cell, mixed Anaplastic (malignant) ependymoma Myxopapillary ependymoma Subependymoma D. Mixed gliomas Mixed oligo-astrocytoma Anaplastic (malignant) oligo-astrocytoma Others E. Choroid plexus papilloma Choroid plexus papilloma Choroid plexus carcinoma F. Neuroepithelial tumors of uncertain origin Astroblastoma Polar spongioblastoma Gliomatosis cerebri G. Neuronal and mixed neuronal-glial tumors Gangliocytoma Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos) Desmoplastic infantile ganglioglioma Dysembryoplastic neuroepithelial tumor Ganglioglioma Anaplastic (malignant) ganglioglioma H. Pineal tumors Pineocytoma Pineoblastoma Mixed pineocytoma/pineoblastoma I. Embryonal tumors Medulloepithelioma Neuroblastoma Variant: ganglioneuroblastoma Ependymoblastoma Retinoblastoma Primitive neuroectodermal tumors (PNET) with multipotential differentiation - neuronal, astrocytic, ependymal, muscle, melanocytic, etc. a. Medulloblastoma Variants: desmoplastic, medullomyoblastoma, melanocytic medulloblastoma b. Cerebral (supratentorial) and spinal PNETs II. Tumors of cranial and spinal nerves Schwannoma (syn: neurilemmoma, neurinoma) Variants: cellular, plexiform, melanotic Neurofibroma Variants: circumscribed (solitary), plexiform, mixed neurofibroma/schwannoma Malignant peripheral nerve sheath tumor (MPNST)(syn: neurogenic sarcoma, anaplastic neurofibroma, malignant schwannoma ) Variants: epithelioid, MPNST with divergent mesenchymal and/or epithelial differentiation, melanotic III. Tumors of the meninges A. Tumors of meningothelial cells 1. Meningioma Histologic types: Meningothelial (syncytial) Transitional/mixed Fibrous (fibroblastic) Psammomatous Angiomatous Microcystic Secretory Clear cell Chordoid Lymphoplasmacyte-rich Metaplastic variants (xanthomatous, myxoid, osseous, chondroid) 2. Atypical meningioma 3. Anaplastic (malignant) meningioma Variants: of a-k above, papillary B. Mesenchymal, non-meningothelial tumors Benign: Osteocartilagenous tumors Lipoma Fibrous histiocytoma Others Malignant Mesenchymal chondrosarcoma Malignant fibrous histiocytoma Rhabdomyosarcoma Meningeal sarcomatosis Others C. Primary melanocytic lesions Diffuse melanosis Melanocytoma Malignant melanoma Variant: meningeal melanomatosis D. Tumors of uncertain origin Hemangiopericytoma Capillary hemangioblastoma IV. Hemopoietic neoplasms Malignant lymphomas Plasmacytoma Granulocytic sarcoma Others V. Germ cell tumors Germinoma Embryonal carcinoma Yolk sac tumor (endodermal sinus tumor) Choriocarcinoma Teratoma Variants: immature, teratoma with malignant transformation Mixed germ cell tumors VI. Cysts and tumor-like lesions Rathke's cleft cyst Epidermoid cyst Dermoid cyst Colloid cyst of the third ventricle Enterogenous cyst (syn: neuroenteric cyst) Neuroglial cyst Other cysts Lipoma Granular cell tumor (syn: choristoma, pituicytoma) Hypothalamic neuronal hamartoma Nasal glial heterotopias VII. Tumors of the anterior pituitary Pituitary adenoma Pituitary carcinoma VIII. Local extensions from regional tumors Craniopharyngioma Variants: adamantinomatous, squamous, papillary Paraganglioma (syn: chemodectoma) Chordoma Variant: chondroid chordoma Chondroma Chondrosarcoma Adenoid cystic carcinoma (syn: cylindroma) Others IX. Metastatic tumors
  • A 45 year old woman with a two year history of infrequent headaches presented with a seizure 4 years ago and was found to have a nonenhancing left posterior temporal and parietal lesion that was stereotactic biopsied and shown to be a low grade anaplastic astrocytoma grade 3. She was subsequently treated with radiotherapy, and later with chemotherapy. Despite treatment, the tumor recurred and radiosurgery was performed six months later. Six months following radiosurgery she developed visual field loss and speech problems. SPECT was normal. Several weeks following surgery, a MRI showed a questionable area of enhancement in the resection bed adjacent to the occipital horn.
  • Case #28: MRI, 8/31/92: Right temporal lobe astrocytoma CC: Episodic confusion HX: This 65 y/o RHM reportedly suffered a stroke on 1/17/92. He presented locally at that time with complaint of episodic confusion and memory loss lasting several minutes per episode. The "stroke" was reportedly verified on MRI scan dated 1/17/92. He was subsequently placed on ASA and DPH. He admitted that there had been short periods(1-2 days duration) since then, during which he had forgotten to take his DPH. However, even when he had been taking his DPH regularly, he continued to experience the spells mentioned above. He denied any associated tonic/clonic movement, incontinence, tongue-biting, HA, visual change, SOB, palpitation, weakness or numbness. The episodes of confusion and memory loss last 1-2 minutes in duration, and have been occurring 2-3 times per week. PMH: Bilateral Hearing Loss of unknown etiology, S/P bilateral ear surgery many years ago. MEDS: DPH and ASA SHX/FHX: 2-4 Beers/day. 1-2 packs of cigarettes per day. EXAM: BP 111/68, P 68BPM, 36.8C. Alert and Oriented to person, place and time, 30/30 on mini-mental status test, Speech fluent and without dysarthria. CN: Left superior quandranopia only. Motor: 5/5 strength throughout. Sensory: unremarkable except for mild decreased vibration sense in feet. Coordination: unremarkable. Gait and station testing were unremarkable. He was able to tandem walk without difficulty. Reflexes: 2+ and symmetric throughout. Flexor plantar responses bilaterally. LAB: Gen Screen, CBC, PT, PTT all WNL. DPH 4.6mcg/ml. Review of outside MRI Brain done 1/17/92 revealed decreased T1 and increased T2 signal in the Right temporal lobe involving the uncus and adjacent hippocampus. The area did not enhance with gadolinium contrast. CXR: 8/31/92: 5 x 6 mm spiculated opacity in apex right lung. EEG: 8/24/92: normal awake and asleep MRI Brain with/without contrast: 8/31/92: Decreased T1 and increased T2 signal in the right temporal lobe. The lesion increased in size and enhances more greatly when compared to the 1/17/92 MRI exam. There is also edema surrounding the affected area and associated mass effect. Neuropsychological testing: Low-average digit symbol substitution, mildly impaired verbal learning, and severely defective delayed recall. There was relative preservation of other cognitive functions. The findings were consistent with left mesiotemporal dysfunction. COURSE: Patient underwent right temporal lobectomy on 9/16/92 following initial treatment with Decadron. Pathologic analysis was consistent with a Grade 2 astrocytoma. GFAP staining positive. Following surgery he underwent 5040 cGy radiation therapy in 28 fractions to the tumor bed.
  • Functional Magnetic Resonance Imaging is a relatively new addition to the brain imaging techniques which allows us to obtain information about brain function non invasively. Generally speaking, there are other MR techniques that also provide functional information. MR angiography displays macroscopic flow in brain arteries and veins, perfusion and diffusion imaging depict semiquantitative tissue perfusion estimates and maps of water diffusion coefficients, respectively, while MR spectroscopy detects specific metabolites and other substances present in brain parenchima. By fMR we mean a specific functional imaging approach targeting the correlate of neural activity associated with the most diverse mental processes. From simple motor, somatic sensory and parceptual function to complex patterns of motor learning, sensory discrimination, memory and attentional processes, this new way of looking at the human brain, still in its infancy, has already done immensurable contributions to our knowledge about it. Differently from Positron Emission Tomography (PET) or Single Photon Emission Tomography (SPECT), no tracer is needed. The intrinsic contrast of the blood is used instead. There exists a coupling between neuronal electrical activity and blood supply, which is mediated by some local vasodilating substances. Among them, nitric oxide (NO) figures as a high potency, fast acting one. Soon after neurons discharge, the local capillary bed receives a higher flow load, bathing the "activated" tissue. Contrary to one would expect, the oxigen contend of the local blood increases, due to a marked increase in delivered arterial blood and a hardly significant increase in oxigen extraction. The consequence is an increase in oxihemoglobin content as compared to deoxihemoglobin levels. What does in mean for imaging purposes? Deoxihemoglobin has paramagnetic properties, while oxihemoglobin is diamagnetic. In the MR context, this means that paramagnetic substances cause a substantial field inhomogeneity, causing loss of signal in the obtained images, what doesn't happen with diamagnet substances. In other words, regions of the brain containing higher oxigenated blood will appear slightly brighter. The problem is that this difference in signal intensity is extremely subtle, being undetectable by simply looking at the images. In order to discern the activated areas, multiple dynamic scans are obtained during alternating task and non-task periods. A statistical analisys is necessary do detect which pixels of the image show significant increase in signal correlated to the task periods. Finally, a coloured map of the activated regions is calculated and overlaid on high resolution images obtained in the same session from the same subject.
  • This patient was operated under local anesthesia with IV sedation.  The surgical navigation system previously described was used to detrmine the incision site for the skin flap and the craniotomy.  Slight widening of the gyri could be observed at the site of the tumor but was not impressive. The surgical navigator was used to confirm the margins of the tumor at the surface, at which point, cortical stimulation was used to locate the motor and sensory cortices.  Stimulation results revealed the lesion to be two gyri behind the motor cortex and that the tumor had indeed pushed the motor and sensory cortices forward.  The lesion was totally removed grossly. Pathology results indicated the tumor to be a low-grade glioma.  The patient tolerated the procedure extremely well and was discharged soon thereafter.
  • Gross brain (left) and corresponding axial contrast-enhanced CT (right). Notice that the right hemisphere is grossly enlarged, but without a focal lesion. There is a diffuse infiltration of the right hemisphere by a low-grade (WHO Grade 2) fibrillary astrocytoma. This appearance is typical of the so-called "gliomatosis cerebri". Notice that although the right thalamus is slightly enlarged (displacement of the internal cerebral veins to left) there is no abnormal enhancement. A lack of enhancement is typical for low-grade diffuse astrocytomas.                      
  • Genetic Profile of the Giant Cell Glioblastoma Aurelia Peraud, Kunihiko Watanabe, Karl Schwechheimer, Yasuhiro Yonekawa, Paul Kleihues, and Hiroko Ohgaki Giant cell glioblastoma is a rare glioblastoma variant characterized by the presence of large, bizarre, multinucleated giant cells. This glioblastoma subtype develops clinically de novo after a short clinical history and contains a high frequency of p53 mutations. In this study, we screened a series of 18 giant cell glioblastomas for additional genetic alterations. PCR-SSCP followed by DNA sequencing revealed PTEN mutations in 5 of 15 tumors (33%). Of these, two mutations were located in exon 5, two mutations in exon 6, and one mutation each in exons 1 and 9. Four mutations were point mutations and two mutations were deletions. One neoplasm contained two PTEN mutations (exons 5 and 6). None of the giant cell glioblastomas showed a homozygous deletion of PTEN or p16, or amplification of MDM2. Immunohistochemically, MDM2 overexpression was either not observed or detected in only a minor fraction of tumor cells. Differential PCR revealed EGFR amplification in only one of 17 tumors (6\%). These results indicate that giant cell glioblastomas occupy a hybrid position, sharing with primary (de novo) glioblastomas a short clinical history, the absence of a less malignant precursor lesion and a 30% frequency of PTEN mutations. With secondary glioblastomas that develop through progression from low-grade astrocytomas, they have in common a younger patient age at manifestation and a high frequency (>70%) of p53 mutations.      
  • Glioblastoma Multiforme Etiology: • The exact etiology is not known but has to do with several mutations in protooncogenes and tumor suppressor genes. • Some appear as a part of several hereditary syndromes such as neurofibromatosis or Turcot's syndrome. Pathogenesis: • The cause of the glioblastoma or any brain tumor is not known but changes or loss of chromosome 17 and inactivation of a tumor suppressor gene, p53, play a role. Thus far, we do not know what precipitates these changes. Epidemiology: • Glioblastomas are the most comman primary brain tumor. • They account for 50% of all gliomas and arise after age 50 in most patients. • Younger patients tend to have a better prognosis than the elderly. • Radiation and chemotherapy appear to extend the life of the patient. General Gross Description: • The glioblastoma multiforme has a multiform or variable appearance with evidence of old and recent hemorrhage (yellowish-brown to red), necrosis and areas of firm tissue. • Usually the glioblastoma is seen as a mass lesion involving a focal area although it may cross the corpus callosum to the other hemisphere or be multifocal. General Microscopic Description: • Microscopically the glioblastoma has many forms as well. • Is a highly cellular tumor with pleomorphic, basophilic nuclei with indistinct cytoplasmic borders or plump pink cytoplasm and a delicate fibrillary background. • Mitoses,necrosis, and capillary endothelial proliferation are common. Clinical Correlations: • The clinical appearance of the glioblastoma is typical for brain tumors in general with a slowly progressive neurological deficit of a focal nature, that is, a slowly progressive hemiparesis of one side of the body. • Prognosis is poor, in that, patients live only 6 month to a year after diagnosis. References: • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 1342-1344. • Poirer J et.al. Manual of basic neuropathology. Philadelphia: Saunders, 1990, pp. 25-26. Synopsis by: M. L. Grunnet M.D.
  • These images illustrate how one superficial GBM had an unusually early presentation as a small lesion. Because the tumor was a subcortical nodule, this mass caused and early presentation with seizures. The small ring-lesion had a rim of hemosiderin, and could mimic a benign process, such as an abscess or hematoma. However, an acute hematoma is usually not surrounded by vasogenic edema (as is seen surrounding this nodule), and an abscess usually does not have such a profound region of T2 shortening (hypointensity - the black rim). After a few months, the lesion had increased in size. Then, dramatically changed its morphology to present as a more "typical" heterogeneous multiloculated and ring enhancing mass on both MR and CT.                            
  • Clinical History: 61-year-old RH white male with history of multiple CVAs with new onset of right sided hemiparesis of one week duration. Diagnosis: Bifrontal butterfly S-shaped lesion consistent with glioblastoma multiforme. Findings: Figure 1: Axial post Gadolinium T1 W1 of the brain reveals a large necrotic butterfly mass involving the corpus callosum with enhancing borders. There is no evidence of herniation. Discussion: A butterfly lesion is a lesion which infiltrates across the corpus callosum. Thus this pathological process spreads from one hemisphere to another. The differential diagnosis of a butterfly lesion includes: glioblastoma multiforme (GBM), lymphoma, and demyelinating process. In this patient's case, the diagnosis was that of a GBM. Symptoms can range from seizures to focal neurologic deficits to symptoms associated with increased intracranial pressure (headaches, nausea, vomiting, decreased visual acuity). Once a mass lesion is suspected, then an MRI with Gadolinium should be performed. This will usually show the classic ring enhancing (associated with angiogenesis occurring at the periphery of the tumor) lesion. However, definitive diagnosis is based on surgical biopsy. Astrocytomas are the most common primary cerebral tumors affecting those in their fifth and sixth decade of life. These tumors represent 50% of all primary intracranial neoplasms. On histologic basis, these tumors are graded as: Grade 1---> low grade, grade II ---> anaplastic, grade III---> GBM. Of all astrocytomas, greater than half are GBM which are the least differentiated and most aggressive. GBM tumors are treated with surgical resection and post-operative radiation. Nonetheless, this malignant process has a poor prognosis with the median survival being 8-10 months. The 1, 2, and 5-year survival rates are 30-44%, 8-12%, and 2.5-5%. The most common cause of death is reoccurrence of tumor at the original site. References: Grossman R, Loftus C. Principles of Neurosurgery . Lippincott-Raven, Philadelphia; 1999. Return to Neuro Imaging Page
  • One of the most "classic" forms of GBM is the "butterfly glioma" illustrated here. Approximately 75% of glioblastomas will "go deep", and infiltrate into and through the corpus callosum, spreading from one hemisphere into the other.   A butterfly lesion is a lesion which infiltrates across the corpus callosum. Thus this pathological process spreads from one hemisphere to another. The differential diagnosis of a butterfly lesion includes: glioblastoma multiforme (GBM), lymphoma, and demyelinating process. In this patient's case, the diagnosis was that of a GBM. Symptoms can range from seizures to focal neurologic deficits to symptoms associated with increased intracranial pressure (headaches, nausea, vomiting, decreased visual acuity). Once a mass lesion is suspected, then an MRI with Gadolinium should be performed. This will usually show the classic ring enhancing (associated with angiogenesis occurring at the periphery of the tumor) lesion. However, definitive diagnosis is based on surgical biopsy. Astrocytomas are the most common primary cerebral tumors affecting those in their fifth and sixth decade of life. These tumors represent 50% of all primary intracranial neoplasms. On histologic basis, these tumors are graded as: Grade 1---> low grade, grade II ---> anaplastic, grade III---> GBM. Of all astrocytomas, greater than half are GBM which are the least differentiated and most aggressive. GBM tumors are treated with surgical resection and post-operative radiation. Nonetheless, this malignant process has a poor prognosis with the median survival being 8-10 months. The 1, 2, and 5-year survival rates are 30-44%, 8-12%, and 2.5-5%. The most common cause of death is reoccurrence of tumor at the original site.
  • WHO Grade IV Cell of Origin: ASTROCYTE Synonyms: GBM, glioblastoma multiforme, spongioblastoma multiforme Common Locations: cerebral hemispheres, occasionally elsewhere (brainstem, cerebellum, cord) Demographics: peak from 45-60 years Histology: grossly heterogeneous, degeneration, necrosis and hemorrhage are common Special Stains: GFAP varies, often present in areas of better differentiation Progression : Can't get any worse. Radiology: Glioblastoma is usually seen as a grossly heterogeneous mass. Ring enhancement surrounding a necrotic center is the most common presentation, but there may be multiple rings. Surrounding vasogenic edema can be impressive, and adds significantly to the mass effect. Signs of recent (methemoglobin) and remote (hemosiderin) hemorrhage are common. Despite it’s apparent demarcation on enhanced scans, the lesion may diffusely infiltrate into the brain, crossing the corpus callosum in 50-75% of cases.
  • Case #101: MRI Brain, 1/26/1993: Pilocytic Astrocytoma in thalamus and caudate. CC: Headache.   Hx: The patient is an 8y/o RHM with a 2 year history of early morning headaches (3:00-6:00AM) intermittently relieved by vomiting only. He had been evaluated 2 years ago and an EEG was "normal" then, but no brain imaging was performed. His headaches progressively worsened, especially in the past two months prior to this presentation. For 2 weeks prior to his 1/25/93 evaluation at UIHC, he would awake screaming. His parent spoke with a local physician who thought this might be due to irritability secondary to pinworms and Vermox was prescribed and arrangements were made for a neurologic evaluation. On the evening of 1/24/93 the patient awoke screaming and began to vomit. This was followed by a 10 min period of tonic-clonic type movements and postictal lethargy. He was taken to a local ER and a brain CT revealed an intracranial mass. He was given Decadron and Phenytoin and transferred to UIHC for further evaluation. MEDS: noted above. PMH: 1)Born at 37.5 weeks gestation by uncomplicated vaginal delivery to a G1P0 mother. Pregnancy complicated by vaginal bleeding at 7 months. Met developmental milestones without difficulty. 2) Frequent otitis media, now resolved. 3) Immunizations were "up to date." FHx: non-contributory. SHx: lives with biologic father and mother. No siblings. In 3rd grade (mainstream) and maintaining good marks in schools. EXAM: BP121/57mmHg HR103 RR16 36.9C MS: Sleepy, but cooperative. CN: EOM full and smooth. Advanced papilledema, OU. VFFTC. Pupils 4/4 decreasing to 2/2. Right lower facial weakness. Tongue midline upon protrusion. Corneal reflexes intact bilaterally. Motor: 5/5 strength. Slightly increased muscle on right side. Sensory. No deficit to PP/VIB noted. Coord: normal FNF, HKS and RAM, bilaterally. Station: Mild truncal ataxia. Tends to fall backward. Reflexes: BUE 2+/2+, Patellar 3/3, Ankles 3+/3+ with 6 beats of nonsustained clonus bilaterally. Gen exam: unremarkable. COURSE: The patient was continued on Dilantin 200mg qd and Decadron 5mg IV q6hrs. Brain MRI, 1/26/93, revealed a large mass lesion in the region of the left caudate nucleus and thalamus which was hyperintense on T2 weighted images. There were areas of cystic formation at its periphery. The mass appeared to enhance on post gadolinium images. there was associated white matter edema and compression of the left lateral ventricle, and midline shift to the right. There was no sign of uncal herniation. He underwent bilateral VP shunting on 1/26/93; and then, subtotal resection (left frontal craniotomy with excision of the left caudate and thalamus with creation of an opening in the septum pellucidum) on 1/28/93. He then received 5040cGy of radiation therapy in 28 fractions completed on 3/25/93. A 3/20/95 neuropsychological evaluation revealed low average intellect on the WISC-III. There were also signs of memory, attention, reading and spelling deficits; and mild right-sided motor incoordination and mood variability. He remained in mainstream classes at school, but his physical and cognitive performance began to deteriorate in 4/95. Neurosurgical evaluation in 4/95 noted increased right hemiplegia and right homonymous hemianopia. MRI revealed tumor progression and he was subsequently placed on Carboplatin/VP-16 (CG 9933 protocol chemotherapy, regimen A). He was last seen on 4/96 and was having difficulty in the 6th grade; he was also undergoing physical therapy for his right hemiplegia.
  • The JPA is a WHO Grade I lesion. The mass is usually sharply circumscribed, with a very narrow zone of microscopic infiltration. The tumor nodule is supplied by capillaries that lack a complete blood-brain-barrier (BBB). The absence of the BBB is the cause of both the contrast enhancement, as well as the source of production of the proteinaceous fluid that accumulates in the "cyst". Because there is no true "lining" the lesions are not "true" cysts. The wall of tissue surrounding the fluid is normal or compressed brain, or may have a mild reactive (not neoplastic) gliosis.    
  • Histology: Alternating dense and loose areas, fusiform "piloid" bipolar astrocytes, microcysts in loose areas may coalesce to form the macroscopic cysts. The presence of nuclear atypia (without mitotic activity) does not convey a worse prognosis. Vascular changes are usually limited to capillary proliferations that may include glomeruloid capillaries and endothelial proliferation. Eosinophilic "Rosenthal fibers" are characteristic. Calcification possible.
  • DISCUSSION: This neuropathologically rather unproblematic case is worth consideration for several reasons. First, it is an unusual finding that a histologically benign tumor is capable of widespread dissemination within the CNS. Second, this feature is unfamiliar to clinicians and even neuropathologists so that presentation of this case leads to a greater awareness of this phenomenon helping to avoid an unnecessary delay of treatment. Low-grade astrocytomas in children exhibit typically benign growth characteristics and have a good prognosis. In contrast to this, a small percentage of these tumors (4 %) manifest widespread dissemination either at presentation or later (2, 4, 5, 7). Astrocytomas with a predelection for cerebral spinal fluid seeding are lesions in close proximity to the ventricles and basal cisterns like in our case. In addition to a periventricular tumor location, operative manipulation has been suggested to play a role in tumor dissemination (5). In the recently published cases of childhood astrocytoma with widely distributed cerebrospinal fluid metastases, presence of tumor dissemination was detected at a presymptomatic stage using MRI as in our case. In such cases, cytologic examination of the cerebrospinal fluid failed to demonstrate the presence of malignant cells (7). As in our case the disseminated tumor nodules showed a strong and diffuse contrast medium enhancement. Because histology of these lesions lacked anaplastic features, enhancement was not interpreted as a sign of malignancy. It might, however, be due to high densities of blood vessels. Our case indeed demonstrated high vascularity in the seeding tissue but not in the initial specimen. The reason for the difference in blood vessel content is unknown. Also unknown are the factors which make benign astrocytomas capable to spread within the cerebrospinal fluid. Moreover, it is not clear whether this ability is restricted to particular neoplastic cell populations. Fig 8B shows aggregates of small tumor cells within the ventricle near the tumor surface but without connection to it suggesting that these cells might be the spreading cell population. They are identical to the cells which form the 'layers' at the ventricular site of the tumor ( Fig. 8A ). Whether these cells differ from the other neoplastic cells molecular genetically has not been determined at present. The prognosis for children with dissemination of a benign astrocytoma is considerably better than that for those with disseminated malignant gliomas (3). Although histological anaplasia is lacking, children have been treated aggressively with radiotherapy and chemotherapy with good response. Under this therapeutic strategy, metastases diminished in size or did not grow further (1, 2, 5, 6). One case in a four-year old girl, however, has been reported in which under chemotherapy and radiation no further growth of tumor spreadings was noted first, whereas later numerous confluent tumor masses developed. She eventually died of central circulatory failure (8). Until now, the patient presented here has showed no progression of disease under polychemotherapy. This case illustrates the importance of recognizing cerebrospinal fluid spread as a possible feature in benign juvenile astrocytomas, which does not inevitably mean a malignant course of the disease. In contrast, if the true benign histopathology of the metastases (or at least of one of the metastatic lesions) has been proven, prognosis is potentially good when treatment with radiation and/or chemotherapy is performed. However, cases with bad outcome have been described. REFERENCES Braun-Fischer A, Romeike BF, Eymann R, Glas B, Riesinger P, Reiche (1997) Pilocytic astrocytoma with subarachnoidal dissemination. Radiologe 38:899-904 Civitello LA, Packer RJ, Rorke LB, Siegal K, Sutton LN, Schut L (1988) Leptomeningeal dissemination of low-grade gliomas in childhood. Neurology 38:562-566 Grabb PA, Albright AL, Pang D (1992) Dissemination of supratentorial malignant gliomas via the cerebrospinal fluid in children. Neurosurgery 30:64-71 Kocks W, Kalff R, Reinhardt V, Grote W, Hilke J (1989) Spinal metastasis of pilocytic astrocytoma of the chiasma opticum. Child´s Nerv Syst 5:118-120 Obana WG, Cogen PH, Davis RL, Edwards MSB (1991) Metastatic juvenile pilocytic astrocytoma. J Neurosurg 75:972-975 Perez MJ, Lorenzo G, Munoz A, Otheo de Tejada E, Maldonado MS, Aparicio JM (1997) Low grade disseminated astrocytoma in childhood. Rev Neurol 25:877-881 Pollack IF, Hurtt M, Pang D, Albright AL (1994) Dissemination of low grade intracranial astrocytomas in children. Cancer 73:2869-2878 Romeike BFM, Niedermayer I, Braun-Fischer A, Graf N, Feiden W (1997) Pilocytic astrocytoma (PA) with subarachnoidal dissemination and „Fahr"-like calcifications after brain irradiation and polychemotherapy. Clin Neuropath 16:282-283 Contributed by Stephan Patt, Nils Haberland, Hagen Graupner, Dieter Schreiber and Rolf Kalff
  • Brain tumor imaginig 2 3rd may 02

    1. 1. Astrocytoma Neuroimaging Dr Deb
    2. 2. I. Tumors of neuroepithelial tissue <ul><li>Astrocytic tumors </li></ul><ul><li>Oligodendroglial tumors </li></ul><ul><li>Ependymal tumors </li></ul><ul><li>Mixed gliomas </li></ul><ul><li>Choroid plexus papilloma </li></ul><ul><li>Neuroepithelial tumors of uncertain origin </li></ul><ul><li>Neuronal and mixed neuronal-glial tumors </li></ul><ul><li>Pineal tumors </li></ul><ul><li>Embryonal tumors </li></ul>
    3. 6. A. Astrocytic tumors <ul><ul><li>Astrocytoma Variants : </li></ul></ul><ul><ul><ul><li>fibrillary, </li></ul></ul></ul><ul><ul><ul><li>protoplasmic, </li></ul></ul></ul><ul><ul><ul><li>gemistocytic, </li></ul></ul></ul><ul><ul><ul><li>mixed </li></ul></ul></ul><ul><ul><li>Anaplastic (malignant) astrocytoma </li></ul></ul><ul><ul><li>Glioblastoma Variants </li></ul></ul><ul><ul><ul><li>giant cell glioblastoma, </li></ul></ul></ul><ul><ul><ul><li>gliosarcoma </li></ul></ul></ul><ul><ul><li>Pilocytic astrocytoma </li></ul></ul><ul><ul><li>Pleomorphic xanthoastrocytoma </li></ul></ul><ul><ul><li>Subependymal giant cell astrocytoma </li></ul></ul>
    4. 7. Astrocytoma <ul><li>Cell of Origin: ASTROCYTE </li></ul><ul><li>Synonyms: diffuse astrocytoma, &quot;ordinary&quot; astrocytoma </li></ul><ul><li>Common Locations: cerebral hemispheres, in children in the pons (&quot;brainstem glioma&quot;) </li></ul><ul><li>Demographics: 4th and 5th decades (30's and 40's) </li></ul><ul><li>Histology: ill-defined, diffusely infiltrating, overrun (trap) neurons, enlargement but not destruction of the invaded structures, mitoses, vascular proliferation, and necrosis are not present </li></ul><ul><li>Special Stains: GFAP+ (Glial Fibrillary Acidic Protein) </li></ul><ul><li>Progression : naturally progress to anaplastic astrocytoma, and then to glioblastoma </li></ul>
    5. 8. Radiology : Astrocytoma <ul><li>The lesion is usually recognized by causing expansion of the infiltrated portion of the cerebrum or brainstem. </li></ul><ul><li>Mass effect may be minimal for the overall size of the signal/attenuation abnormality, </li></ul><ul><li>and the lesion may be surprising large at the time of presentation with minimal symptoms. </li></ul><ul><li>Regions of signal and attenuation change usually represent the neoplasm itself - since the intact blood-brain-barrier does not cause enhancement nor vasogenic edema. </li></ul><ul><li>Hemorrhage is rare, however, calcification can occur. </li></ul><ul><li>The tumor tends to follow white-matter tracts and may cross the corpus callosum, or follow the peduncles to/from the brainstem </li></ul>
    6. 9. Astrocytoma of the Brain • Axial T2 weighted spin-echo image of the head. • There is an intra-axial mass in the left frontal lobe with surrounding vasogenic edema. • There is mass effect on the surrounding structures with compression of the left frontal horn and displacement of the falx to the right.
    7. 11. A 45 year old woman with a two year history of infrequent headaches presented with a seizure 4 years ago and was found to have a nonenhancing left posterior temporal and parietal lesion that was stereotactic biopsied and shown to be a low grade anaplastic astrocytoma grade 3. She was subsequently treated with radiotherapy, and later with chemotherapy. Despite treatment, the tumor recurred and radiosurgery was performed six months later. Six months following radiosurgery she developed visual field loss and speech problems. SPECT was normal. Several weeks following surgery, a MRI showed a questionable area of enhancement in the resection bed adjacent to the occipital horn.
    8. 13. Parietal lobe perfusion defect (HMPAO) with faint focal parietal thallium uptake, consistent with radiation necrosis.
    9. 14. Astrocytoma <ul><li>the tumor completely replaces the basal ganglia and infiltrates profusely into surrounding tissue. </li></ul><ul><li>Note also that this is an expanding intercranial mass. </li></ul><ul><li>The nature of gliomas is such that they infiltrate subtly into surrounding tissue, making them very difficult to remove </li></ul>
    10. 15. Fibrillary Astrocytoma <ul><li>Coronal gross brain section. There is gross expansion of the left cerebral hemisphere. </li></ul><ul><li>This is caused by infiltration of a diffuse low-grade (WHO Grade 2) fibrillary astrocytoma. This is &quot;gliomatosis cerebri&quot;. </li></ul><ul><li>In addition, the superior surface of the expanded hemisphere has a discoloration from a local area of transformation from Grade 2 into a Grade 4 astroctyoma (Glioblastoma Multiforme or GBM). </li></ul><ul><li>This is a natural progression, from low-grade to high-grade. At least 50% of GBM's arise from a pre-existing lower grade lesion </li></ul>
    11. 16. Gemistocytic Astrocytoma
    12. 17. Anaplastic Astrocytoma
    13. 18. <ul><li>CC: Episodic confusion </li></ul><ul><li>HX: This 65 y/o RHM reportedly suffered a stroke on 1/17/92. He presented locally at that time with complaint of episodic confusion and memory loss lasting several minutes per episode. The &quot;stroke&quot; was reportedly verified on MRI scan dated 1/17/92. </li></ul><ul><li>He was subsequently placed on ASA and DPH. </li></ul><ul><li>He admitted that there had been short periods(1-2 days duration) since then, during which he had forgotten to take his DPH. However, even when he had been taking his DPH regularly, he continued to experience the spells mentioned above. </li></ul><ul><li>The episodes of confusion and memory loss last 1-2 minutes in duration, and have been occurring 2-3 times per week. </li></ul><ul><li>PMH: Bilateral Hearing Loss of unknown etiology, S/P bilateral ear surgery many years ago. </li></ul><ul><li>MEDS: DPH and ASA </li></ul><ul><li>SHX/FHX: 2-4 Beers/day. 1-2 packs of cigarettes per day. </li></ul><ul><li>EXAM: BP 111/68, P 68BPM, 36.8C. </li></ul><ul><li>Alert and Oriented to person, place and time, 30/30 on mini-mental status test, Speech fluent and without dysarthria. </li></ul><ul><li>CN: Left superior quandranopia only. </li></ul><ul><li>Motor: 5/5 strength throughout. </li></ul><ul><li>Sensory: unremarkable except for mild decreased vibration sense in feet. Coordination: unremarkable. </li></ul><ul><li>Gait and station testing were unremarkable. He was able to tandem walk without difficulty. </li></ul><ul><li>Reflexes: 2+ and symmetric throughout. Flexor plantar responses bilaterally. </li></ul>
    14. 19. <ul><li>Review of outside MRI Brain done 1/17/92 revealed decreased T1 and increased T2 signal in the Right temporal lobe involving the uncus and adjacent hippocampus. The area did not enhance with gadolinium contrast </li></ul>
    15. 20. <ul><li>MRI Brain with/without contrast: 8/31/92: Decreased T1 and increased T2 signal in the right temporal lobe. The lesion increased in size and enhances more greatly when compared to the 1/17/92 MRI exam. There is also edema surrounding the affected area and associated mass effect. </li></ul>
    16. 21. <ul><li>Neuropsychological testing: Low-average digit symbol substitution, mildly impaired verbal learning, and severely defective delayed recall. There was relative preservation of other cognitive functions. The findings were consistent with left mesiotemporal dysfunction. </li></ul><ul><li>COURSE: Patient underwent right temporal lobectomy on 9/16/92 following initial treatment with Decadron. Pathologic analysis was consistent with a Grade 2 astrocytoma. GFAP staining positive. Following surgery he underwent 5040 cGy radiation therapy in 28 fractions to the tumor bed. </li></ul>
    17. 22. Shown here is the displaced sensorimotor cortex of a patient with a low grade astrocytoma, demonstrated by fMRI
    18. 23. <ul><li>A seventeen year-old boy with numbness in the left foot was examined. </li></ul><ul><li>Sagittal T1, axial T2 (middle), axial FLAIR and axial post-gadolinium (right) MRI revealed a 5cm (anterior-posterior) x 4 cm (right to left) x 3.5 cm (superior-inferior) right parietal lobe mass. </li></ul><ul><li>The imaging characteristics suggested either as  astrocytoma or dysembryoblastic neuroepithelial tumor </li></ul>
    19. 26. Three-dimensional mod of this patient's MRI was reconstructed.  The brain is shown in white, the tumor is shown in green, the vessels are shown in red and the ventricles are shown in light blue.
    20. 27. This model helped visualize the lesion in 3D space. The location of the tumor was confirmed as being in the motor and primary sensory cortex. After discussion with the parents of the  patient, it was decided to perform resection of the lesion under local anesthesia to monitor the patient's motor and sensory functions.
    21. 32. Grade II Astrocytoma
    22. 33. A. Astrocytic tumors <ul><ul><li>Astrocytoma Variants : </li></ul></ul><ul><ul><ul><li>fibrillary, </li></ul></ul></ul><ul><ul><ul><li>protoplasmic, </li></ul></ul></ul><ul><ul><ul><li>gemistocytic, </li></ul></ul></ul><ul><ul><ul><li>mixed </li></ul></ul></ul><ul><ul><li>Anaplastic (malignant) astrocytoma </li></ul></ul><ul><ul><li>Glioblastoma Variants </li></ul></ul><ul><ul><ul><li>giant cell glioblastoma, </li></ul></ul></ul><ul><ul><ul><li>gliosarcoma </li></ul></ul></ul><ul><ul><li>Pilocytic astrocytoma </li></ul></ul><ul><ul><li>Pleomorphic xanthoastrocytoma </li></ul></ul><ul><ul><li>Subependymal giant cell astrocytoma </li></ul></ul>
    23. 34. Anaplastic Astrocytoma <ul><li>WHO Grade III </li></ul><ul><li>Cell of Origin: ASTROCYTE </li></ul><ul><li>Synonyms: </li></ul><ul><li>Common Locations: cerebral hemispheres, brainstem </li></ul><ul><li>Demographics: </li></ul><ul><li>Histology: astrocytes with anaplastic features: increased cellularity, pleomorphism, mitotic activity, and nuclear atypia </li></ul><ul><li>Special Stains: GFAP+ </li></ul><ul><li>Progression : great probability of progression to glioblastoma </li></ul><ul><li>Radiology: Variable appearance for features of enhancement and surrounding edema. Necrosis (ring enhancement) does not occur, and cyst formation is extremely rare. </li></ul><ul><li>COMMENTS: This stage of astrocytoma is the least common, and may represent a short-term intermediate lesion during the transition from Grade II to Grade IV astrocytoma. </li></ul>
    24. 35. <ul><li>Male, 45 years, suffering from a first generalised seizure two weeks ago. </li></ul><ul><li>On MR images, subcortical, slightly hyperintense (T2 WI), ill-delimited lesion in left temporal lobe. </li></ul><ul><li>Heterogeneous and mild contrast enhancement. We firstly read this lesion as a probable aspecific vascular versus inflammatory (infectious?) lesion. </li></ul><ul><li>A control MR, one month later, demonstrates an enlargement of the lesion. </li></ul><ul><li>Final diagnosis after surgery: anaplastic astrocytoma. </li></ul>
    25. 36. A. Astrocytic tumors <ul><ul><li>Astrocytoma Variants : </li></ul></ul><ul><ul><ul><li>fibrillary, </li></ul></ul></ul><ul><ul><ul><li>protoplasmic, </li></ul></ul></ul><ul><ul><ul><li>gemistocytic, </li></ul></ul></ul><ul><ul><ul><li>mixed </li></ul></ul></ul><ul><ul><li>Anaplastic (malignant) astrocytoma </li></ul></ul><ul><ul><li>Glioblastoma Variants </li></ul></ul><ul><ul><ul><li>giant cell glioblastoma, </li></ul></ul></ul><ul><ul><ul><li>gliosarcoma </li></ul></ul></ul><ul><ul><li>Pilocytic astrocytoma </li></ul></ul><ul><ul><li>Pleomorphic xanthoastrocytoma </li></ul></ul><ul><ul><li>Subependymal giant cell astrocytoma </li></ul></ul>
    26. 37. GBM <ul><li>WHO Grade IV </li></ul><ul><li>Cell of Origin: ASTROCYTE </li></ul><ul><li>Synonyms: GBM, glioblastoma multiforme, spongioblastoma multiforme </li></ul><ul><li>Common Locations: cerebral hemispheres, occasionally elsewhere (brainstem, cerebellum, cord) </li></ul><ul><li>Demographics: peak from 45-60 years </li></ul><ul><li>Histology: grossly heterogeneous, degeneration, necrosis and hemorrhage are common </li></ul><ul><li>Special Stains: GFAP varies, often present in areas of better differentiation </li></ul><ul><li>Progression : Can't get any worse. </li></ul>
    27. 38. Radiology: GBM <ul><li>Glioblastoma is usually seen as a grossly heterogeneous mass. </li></ul><ul><li>Ring enhancement surrounding a necrotic center is the most common presentation, but there may be multiple rings. </li></ul><ul><li>Surrounding vasogenic edema can be impressive, and adds significantly to the mass effect. </li></ul><ul><li>Signs of recent (methemoglobin) and remote (hemosiderin) hemorrhage are common. </li></ul><ul><li>Despite it’s apparent demarcation on enhanced scans, the lesion may diffusely infiltrate into the brain, crossing the corpus callosum in 50-75% of cases. </li></ul>
    28. 39. Glioblastoma
    29. 40. <ul><li>Glioblastoma is usually seen as a grossly heterogeneous mass. </li></ul><ul><li>Ring enhancement surrounding a necrotic center is the most common presentation, but there may be multiple rings. </li></ul><ul><li>Surrounding vasogenic edema can be impressive, and adds significantly to the mass effect. </li></ul><ul><li>Signs of recent (methemoglobin) and remote (hemosiderin) hemorrhage are common. </li></ul><ul><li>Despite it’s apparent demarcation on enhanced scans, the lesion may diffusely infiltrate into the brain, crossing the corpus callosum in 50-75% of cases. </li></ul>
    30. 41. Admission scans after first seizure
    31. 42. One month later
    32. 43. Two months later
    33. 44. GBM thalamus <ul><li>This T1W MR after Gadolinium infusion reveals a thalamic mass lesion. </li></ul><ul><li>This is characterized by irregular ring-enhancement surrounding a central non-enhancing region of necrosis. </li></ul><ul><li>Note the shaggy inner-margin of the ring, and the remakable variation in its thickness. </li></ul><ul><li>The small foci of internal enhancement represent islands of living tumor within the regions of necrosis. </li></ul>
    34. 45. GBM <ul><li>61-year-old RH white male with history of multiple CVAs with new onset of right sided hemiparesis of one week duration. </li></ul><ul><li>Axial post Gadolinium T1 W1 of the brain reveals a large necrotic butterfly mass involving the corpus callosum with enhancing borders. There is no evidence of herniation </li></ul>
    35. 46. GBM
    36. 47. GBM Axial Gd Enhanced T1W MR Axial T2W MR
    37. 48. Fusion imaging - <ul><li>Grade IV astrocytoma . (a) Contrast-enhanced axial CT scan . (b) Tc-99m HMPAO SPECT image at the same anatomic level. (c) Fused imag </li></ul>                                                                    
    38. 49. <ul><li>Recurrence and upgrading of a grade IV astrocytoma. (a) T1-weighted contrast-enhanced MR image. (b) F-18 DG SPECT image at the same anatomic level. (c) Fused image before stereotactic biopsy. </li></ul>
    39. 50. GBM - Gross <ul><li>a massive neoplasm in the left hemisphere replacing the ventricle and much of the temporal and parietal lobe. </li></ul><ul><li>Note that it is variegated in color and appearance indicating necrosis and hemorrhage. </li></ul><ul><li>The apparent sharp boundaries of the tumor are deceptive because actually it, like any other glioma, has infiltrated beyond its apparent margins. </li></ul>
    40. 51. GBM Gross <ul><li>A coronal section through the parietal-occipital junction. </li></ul><ul><li>The large variegated mass represents a glioblastoma multiforme - multiform in shape, color, texture and histology. </li></ul>
    41. 52. GBM - Microscopy <ul><li>the typical type of necrosis seen in a glioblastoma surrounded by more dense layer of tumor cells referred to as &quot;pseudopalisading&quot;. Note also in the lower corner a vessel that is thrombosed </li></ul>
    42. 53. GBM- Mesencephelalon <ul><li>There is an irregular red-brown-black lesion of the brainstem, with dramatic enlargement of the affected area. Histologically, this was a glioblastoma. </li></ul>
    43. 55. A. Astrocytic tumors <ul><ul><li>Astrocytoma Variants : </li></ul></ul><ul><ul><ul><li>fibrillary, </li></ul></ul></ul><ul><ul><ul><li>protoplasmic, </li></ul></ul></ul><ul><ul><ul><li>gemistocytic, </li></ul></ul></ul><ul><ul><ul><li>mixed </li></ul></ul></ul><ul><ul><li>Anaplastic (malignant) astrocytoma </li></ul></ul><ul><ul><li>Glioblastoma Variants </li></ul></ul><ul><ul><ul><li>giant cell glioblastoma, </li></ul></ul></ul><ul><ul><ul><li>gliosarcoma </li></ul></ul></ul><ul><ul><li>Pilocytic astrocytoma </li></ul></ul><ul><ul><li>Pleomorphic xanthoastrocytoma </li></ul></ul><ul><ul><li>Subependymal giant cell astrocytoma </li></ul></ul>
    44. 59. Pilocytic Astrocytoma
    45. 60. JPA – WHO Grade 1 <ul><li>Contrast enhanced CT of a juvenile pilocytic astrocytoma (JPA). Notice that there is a characteristic &quot;cyst and mural nodule&quot; morphology. The key feature here is that most of the cyst lining does NOT show contrast enhancement. Notice that this nodule has a central area without enhancement - due to a cyst within the nodule (NOT necrosis). Although the appearance can be variable, both JPA and hemangioblastoma can be suggested when a cerebellar lesion has a &quot;cyst and nodule&quot; appearance. </li></ul>
    46. 61. JPA – WHO - 1 <ul><li>This Gadolinium enhanced T1W MR shows a characteristic &quot;cyst and mural nodule&quot; morphology for a pilocytic astrocytoma (JPA). Notice that part of the cyst lining does NOT enhance. Although the appearance can be variable, both JPA and hemangioblastoma should be suggested when a cerebellar lesion has this &quot;cyst and nodule&quot; appearance . </li></ul>
    47. 62. This coronal T1W Gadolinium enhanced MR shows a cerebellar mass with a &quot;cyst with nodule&quot; morphology.
    48. 63. JPA – WHO –1 <ul><li>This CT scan shows a posterior fossa mass (&quot;Cyst with Nodule&quot;) causing obstructive hydrocephalus with temporal horn dilatation. </li></ul>
    49. 64. JPA – WHO - 1 This is also a pathologically-proven JPA, presenting in a 14 y.o. boy. This one did not &quot;read the book&quot; and the mass, although partially cystic and partially solid, has a grossly heterogeneous morphology - rather than the classic &quot;cyst with nodule&quot; shape. A lesion with this complex configuration could be a malignant glioma with necrosis. However, the cerebellar location and the young age make JPA a more likely possibility.
    50. 65. Pilocytic Astrocytoma <ul><li>This sagittal T1W image demonstrates a large suprasellar pilocytic astrocytoma of the hypothalamus. The mass is heterogeneous, and extends downward into the region of the sphenoid sinus, as well as behind the clivus </li></ul>
    51. 66. JPA - Angiogram <ul><li>Lateral posterior fossa angiogram, subtracted image. Cerebellar pilocytic astrocytoma. Notice how there is vascular displacement - but not neovascularity. Pilocytic astrocytomas enhance because of increased capillary permeability, not because of arterial neovascularity. </li></ul>
    52. 67. JPA – WHO - 1 <ul><li>This gross picture demonstrates a cystic lesion in the left cerebellar hemisphere. There is a thin white line surrounding the cavity - representing compressed cerebellar tissue with gliosis. However, the &quot;cyst lining&quot; is NOT composed of neoplastic tissue, accounting for the absence of enhancement. </li></ul>
    53. 68. JPA - Gross <ul><li>Coronal gross photograph. This juvenile pilocytic astrocytoma shows a well-defined &quot;mural nodule&quot; inside of a &quot;cystic&quot; region. </li></ul>
    54. 69. JPA - Gross <ul><li>This section of cerebellum shows a cystic tumor occupying the central white matter of the cerebellar and part of the dentate nucleus. This is an example of a pilocytic astrocytoma. It is usually very slow growing, occurs most commonly in children and will cause symptoms referable to the cerebellum where it most commonly occurs </li></ul>
    55. 71. JPA- Histology <ul><li>This HE photomicrograph shows some of the classic features of a juvenile pilocytic astrocytoma: a biphasic pattern (alternating dense and loose areas), microcysts, and an absence of aggressive features (no necrosis, no mitoses, no hemorrhage). </li></ul>
    56. 72. Pilocytict Astroctytoma
    57. 74. <ul><li>This 16-year-old young man presented in 1993 with headache, nausea, and vomiting. Magnetic resonance imaging (MRI) study revealed a solitary brain tumor at the trigonum of the right lateral ventricle </li></ul>
    58. 75. <ul><li>No contrast enhancement was noted. The tumor was totally removed in 1994 as demonstrated by the preoperative scan (left) </li></ul><ul><li>and a postoperative scan (right). </li></ul>
    59. 76. <ul><li>MRI in February 1995 showed recurrent tumor tissue lateral to the temporal horn of the right ventricle. In October 1995, tumor recurrence had enlarged in size. A concentric limitation of the visual field occurred seven months later in May 1996. In November 1996 the recurrent tumor tissue demonstrated for the first time contrast enhancement on MRI. </li></ul>
    60. 77. <ul><li>In February of 1998, extensive dissemination of the tumor along the supratentorial ventricular system and the spinal leptomeninges was noted during a routine control MRI scan </li></ul><ul><li>No recurrent tumor tissue was present at the primary site of operation. The patient had no clinical symptoms. Multiple nodules, up to 5 mm I size and markedly contrast enhancing, were found in both lateral ventricles, the III ventricle </li></ul>
    61. 78. Spinal cord dissemination
    62. 79. With MRI criteria they all looked identical. One of them was removed using neuronavigation
    63. 80. An Ommaya reservoir was implanted. The patient recieved systemic chemotherapy with vincristin and carboplatinum, under which tumor nodules had not changed in size by the end of December 1998.
    64. 81. H&E sections of the tumor operated in 1994 showed a moderately cellular glial neoplasm. Some areas were composed of piloid astrocytes and displayed few Rosenthal fibers
    65. 82. Sometimes larger multinuclear cells were present
    66. 83. The regions near the ventricle were composed of loosely arranged piloid cells and large glial cells with one or more nuclei A broad glial fibrillary acidic protein (GFAP)-positive cytoplasm Areas immediately at the ventricle site ('V' in Fig. 3C and Fig. 3D ) showed five or six 'layers' of densely packed smaller astrocytic cells with shorter processes and only slight GFAP-immunoreaction. Mitoses, necroses and endothelial proliferations were absent.
    67. 84. The biopsy of one of the ventricular tumor disseminations in 1998 revealed histological and immunohistochemical features which were very similar with the initial resection specimen. The density of capillaries was however higher
    68. 85. The smaller neoplastic astrocytes which formed a 'layer' at the ventricle site were also demonstrable in the tumor spreadings (Fig. 8A). They could sometimes be found in the ventricular space without connection to the other tumor cells As in 1994, no histologic signs of anaplasia or malignancy could be found. Mitoses were absent. The Ki67-positivity was very low, especially in the densely packed cells in close vicinity to the ventricle (not shown). Cebrospinal fluid cytology revealed no anaplastic cells.
    69. 86. DIAGNOSIS <ul><li>  Pilocytic astrocytoma (WHO grade I) with widespread dissemination in the CNS. The diagnosis was confirmed by the German reference center for brain tumors in Bonn. </li></ul>
    70. 87. A. Astrocytic tumors <ul><ul><li>Astrocytoma Variants : </li></ul></ul><ul><ul><ul><li>fibrillary, </li></ul></ul></ul><ul><ul><ul><li>protoplasmic, </li></ul></ul></ul><ul><ul><ul><li>gemistocytic, </li></ul></ul></ul><ul><ul><ul><li>mixed </li></ul></ul></ul><ul><ul><li>Anaplastic (malignant) astrocytoma </li></ul></ul><ul><ul><li>Glioblastoma Variants </li></ul></ul><ul><ul><ul><li>giant cell glioblastoma, </li></ul></ul></ul><ul><ul><ul><li>gliosarcoma </li></ul></ul></ul><ul><ul><li>Pilocytic astrocytoma </li></ul></ul><ul><ul><li>Pleomorphic xanthoastrocytoma </li></ul></ul><ul><ul><li>Subependymal giant cell astrocytoma </li></ul></ul>
    71. 88. Pleomorphic xanthoastrocytoma <ul><li>WHO Grade II - III </li></ul><ul><li>Cell of Origin: ASTROCYTE (subpial?) </li></ul><ul><li>Common Locations: cerebral hemispheres, usually superficial, often temporal </li></ul><ul><li>Demographics: children and young adults </li></ul><ul><li>Histology: A frightening mixture of unusually pleomorphic cells, ranging from fibrillary to bizarre giant multinucleated cells with intracellular lipid vacuoles (&quot;xanthoma&quot; cells). </li></ul><ul><li>Special Stains: GFAP positive </li></ul><ul><li>Progression : may progress in some cases to Grade III (anaplastic) or even Grade IV (GBM) </li></ul>
    72. 89. Radiology : PXAC <ul><li>Usually a large hemispheric mass, closely related to the cerebral surface. </li></ul><ul><li>May have a disturbingly heterogeneous appearance with cyst formation, variable calcification, and prominent enhancement. </li></ul><ul><li>The superficial location and presentation in childhood are the most helpful features. </li></ul><ul><li>Dural involvement and a &quot;dural tail&quot; are possible </li></ul>
    73. 90. PLEOMORPHIC XANTHOASTROCYTOMA Axial T1W Gd Enhanced MR Axial CT
    74. 91. A. Astrocytic tumors <ul><ul><li>Astrocytoma Variants : </li></ul></ul><ul><ul><ul><li>fibrillary, </li></ul></ul></ul><ul><ul><ul><li>protoplasmic, </li></ul></ul></ul><ul><ul><ul><li>gemistocytic, </li></ul></ul></ul><ul><ul><ul><li>mixed </li></ul></ul></ul><ul><ul><li>Anaplastic (malignant) astrocytoma </li></ul></ul><ul><ul><li>Glioblastoma Variants </li></ul></ul><ul><ul><ul><li>giant cell glioblastoma, </li></ul></ul></ul><ul><ul><ul><li>gliosarcoma </li></ul></ul></ul><ul><ul><li>Pilocytic astrocytoma </li></ul></ul><ul><ul><li>Pleomorphic xanthoastrocytoma </li></ul></ul><ul><ul><li>Subependymal giant cell astrocytoma </li></ul></ul>
    75. 92. <ul><li>Synonyms: Ventricular tumor of Tuberous Sclerosis </li></ul><ul><li>Common Locations: Lateral ventricle, attached to caudate head. </li></ul><ul><li>Demographics: children, young adults </li></ul><ul><li>Histology: Large cells that variously resemble astrocytes, although may express neuronal markers </li></ul><ul><li>Special Stains: GFAP variable (usually present), neuronal markers +/- </li></ul><ul><li>Progression : No </li></ul><ul><li>Radiology: Usually seen with other features of Tuberous Sclerosis. May obstruct f. of Monro and cause hydrocephalus. Enhancement and calcification are both common. </li></ul>Plane Contrast
    76. 93. SUBEPENDYMAL GIANT CELL ASTROCYTOMA T1 Gd T1
    77. 94. Thank You

    ×