Leptomeningeal Spread Research Articles

MicroRNA-182 promotes leptomeningeal spread of non-sonic hedgehog-medulloblastoma

The contribution of microRNAs to the initiation, progression, and metastasis of medulloblastoma (MB) remains poorly understood. Metastatic dissemination at diagnosis is present in about 30% of MB patients, and is associated with a dismal prognosis. Using microRNA expression profiling, we demonstrate that the retinal miR-183-96-182 cluster on chromosome 7q32 is highly overexpressed in non-sonic hedgehog MBs (non-SHH-MBs). Expression of miR-182 and miR-183 is associated with cerebellar midline localization, and miR-182 is significantly overexpressed in metastatic MB as compared to non-metastatic tumors. Overexpression of miR-182 in non-SHH-MB increases and knockdown of miR-182 decreases cell migration in vitro. Xenografts overexpressing miR-182 invaded adjacent normal tissue and spread to the leptomeninges, phenotypically reminiscent of clinically highly aggressive large cell anaplastic MB. Hence, our study provides strong in vitro and in vivo evidence that miR-182 contributes to leptomeningeal metastatic dissemination in non-SHH-MB. We therefore reason that targeted inhibition of miR-182 may prevent leptomeningeal spread in patients with non-SHH-MB.

Primary brain T-cell lymphoma in an HTLV-1 serologically positive male

Primary central nervous system lymphoma (PCNSL) is a subgroup of extranodal non-Hodgkin lymphoma usually due to B-cells. The incidence of T-cell PCNSL is 1–4% in Western countries. Human T-lymphotropic virus (HTLV-1) causes tropical spastic paraparesis/myelopathy and adult T-cell leukemia/lymphoma. We describe the extremely rare occurrence of T-cell PCNSL in a 29year old HTLV-1 carrier. Additional unusual features of the case included the patient's young age and normal cerebrospinal fluid cytological findings, without leptomeningeal spread. Given the long latency between HTLV-1 infection and disease manifestation, more such cases may be diagnosed in the future. We recommend that every patient with T-cell PCNSL be screened for HTLV-1.

Pineocytoma with diffuse dissemination to the leptomeninges

Pineal parenchymal tumors are rare. Of the three types of pineal parenchymal tumors, pineocytomas are the least aggressive and are not known to diffusely disseminate. In this paper, we report the successful treatment of a case of pineocytoma with diffuse leptomeningeal relapse following initial stereotactic radiotherapy. A 39-year-old female presented with headaches, balance impairment, urinary incontinence, and blunted affect. A pineal mass was discovered on magnetic resonance imaging (MRI). A diagnosis of pineocytoma was established with an endoscopic pineal gland biopsy, and the patient received stereotactic radiotherapy. Ten years later, she developed diffuse leptomeningeal dissemination. The patient was then successfully treated with craniospinal radiation therapy. Leptomeningeal spread may develop as late as 10 years after initial presentation of pineocytoma. Our case demonstrates the importance of long-term follow-up of patients with pineal parenchymal tumors following radiation therapy, and the efficacy of craniospinal radiation in the treatment of leptomeningeal dissemination.

Resolution of extensive leptomeningeal metastasis and clinical spinal cord compression from breast cancer using weekly docetaxel chemotherapy

Metastatic breast cancer to the leptomeninges is a late event in the disease course and is associated with significant morbidity and a grave prognosis. Treatment typically involves direct intrathecal injection of chemotherapy into the cerebrospinal fluid compartment since systemic chemotherapy penetrates poorly to the central nervous system. Here we report an interesting clinical observation involving a patient presenting with leptomeningeal spread of breast cancer causing extensive spinal cord compression with obliteration of the subarachnoid space, thus precluding the use of direct intrathecal chemotherapy. We administered systemic chemotherapy using weekly docetaxel with complete radiographic resolution of her disease and recovery from clinical spinal cord compression. While this is a single clinical observation, weekly administration of docetaxel in this circumstance may have been associated with improved drug "escape" into the central nervous system and better antitumor effect. Because leptomeningeal disease is typically a late event in metastatic breast cancer, resistance to therapeutic intervention may reflect intrinsically resistant disease in the setting of extensive prior therapy rather than a routine problem with systemic drug delivery to the CNS. Studying patterns of disease relapse in patients who had received adjuvant weekly taxanes may provide insights into this hypothesis.

Symptomatic spinal cord metastasis from cerebral oligodendroglioma

Spinal subarachnoid spread is not uncommon in brain oligodendrogliomas; on the other hand, symptomatic involvement of the spinal cord and cauda is very rare, with only 16 reported cases. We report the case of a 41-year-old man who underwent resection of a low-grade frontal oligodendroglioma 4 years previously. He was again observed because of bilateral sciatic pain followed by left leg paresis. A spine MRI showed an intramedullary T12-L1 tumor with root enhancement. At operation, an intramedullary anaplastic oligodendroglioma with left exophytic component was found and partially resected. Two weeks later, a large left frontoparietal anaplastic oligodendroglioma was diagnosed and completely resected. The patient was neurologically stable for 8 months and died 1 year after the spinal surgery because of diffuse brain and spinal leptomeningeal spread. The review of the reported cases shows that spinal symptomatic metastases can occur in both low-grade and anaplastic oligodendrogliomas, even many years after surgery of the primary tumor; however, they exceptionally occur as first clinical manifestation or as anaplastic progression. The spinal seeding represents a negative event leading to a short survival.

Atypical teratoid/rhabdoid tumor of the brain – A case with three secondary implants

AbstractAtypical teratoid/rhabdoid (AT/RT) of the brain is a rare malignant pediatric neoplasm that usually affects children around age two. This report is about a male patient, six months of age, who presented with seizures and decreased level of consciousness, progressing to numbness, oculomotor nerve palsy and tetra-spasticity. CT scan showed left cerebellopontine angle tumor. Magnetic resonance imaging (MRI) showed a solid lesion, hypointense on T1W and heterogeneous hyperintense on T2W, enhanced after contrast infusion, accompanied by three solid lesions, rounded and irregular in the left cerebellar hemisphere, occipito-parietal transition and right occipital lobe with no evidence of leptomeningeal spread, forming secondary implants. We conducted a biopsy, which confirmed AT/ RT. Chemotherapic treatment was ineffective and the patient passed away quickly. In the literature review we found about 300 cases reported, as it is, therefore, extremely rare tumor with a mean age at diagnosis of 29 months. It can affect any area of the central nervous system, mainly infratentorial, being its main differential diagnosis medulloblastomas. The findings of secondary implantation without leptomeningeal spread seen in this patient are described in only one other case in literature. The clinical features usually are neurological deficits, seizures and coma. Its genesis stems from mutations in the gene hSNF5/INI1 on chromosome 22. Neuroimaging studies don’t reveal radiological pattern suggesting AT/RT and the diagnosis is possible only through a biopsy with immunohistochemical analysis. The available treatments are ineffective, and death is the outcome, with 84% in six months.

8704 ORAL SOX8 as a Molecular Marker of Oligodendroglial Tumours

Background: Bevacizumab (BV) has shown activity in recurrent glioblastoma (GBM), both alone and in association with chemotherapeutic agents. Few data are available on the combination of bevacizumab and nitrosoureas, that represent the standard cytotoxic option at recurrence. Fotemustine (FTM) is a nitrosourea with elevated lipophilic properties, and demonstrated activity in recurrent GMBs as single agent. Methods: In this phase II study patients with GBM recurrent after surgery, radiation therapy, and concomitant/adjuvant temozolomide were elegible. The treatment consisted of an induction phase with BV at 10mg/kg intravenously on day 1 and 15 and fotemustine (FTM) at 75mg/m intravenously on day 1 and 8, followed after 3 week interval by a maintenance phase with BV at 10mg/kg i.v. and FTM 75mg/ m every 3 weeks until tumour progression or unacceptable toxicity. Patients had undergone clinical and MRI assessment 1 month after the start of treatment and thereafter every 2 months. The primary endpoint was progression-free survival at 6 months (PFS6), whereas secondary endpoints were response rate (RR), based on RANO criteria, progression-free (PFS) and overall survival (OS), and safety. Results: From April 2008 until November 2010, 54 patients (males 35, females 19, median age 57) were enrolled. PFS6, PFS12 and mPFS were 44%, 21% and 5.29 months respectively. mOS was 9.13 months with 77.4% and 31% of patients surviving at 6 and 12 months respectively. Response rates were as follows: 2CR (4%), 24 PR (44%), 22 SD (41%) and 6 PD (11%). A significant neurological improvement was observed in 57% of patients, being steroids reduced or interrupted in 64%. 44/54 (81%) patients have progressed and patterns of progression were local in 29/44 (66%), multicentric 10/44 (23%), gliomatosis 3/44 (6%) and isolated leptomeningeal spread 2/44 (5%). 12/54 (22%) patients with grade III/IV piastrinopenia/leukopenia discontinued fotemustine, whereas 4/54 (7.4%)discontinued bevacizumab (1 stroke, 1 intratumoral haemorrhage, 1 GI perforation and 1 pulmonary embolism. Conclusions: Combination of bevacizumab and fotemustine in glioblastomas recurrent after standard radiotherapy + temozolomide is safe and promising. The analysis of MGMT methylation status is ongoing.

Gliosarcoma Metastatic to the Leptomeninges and Dura

We describe a rare case of a patient with left frontotemporal gliosarcoma, which metastasized through the cerebrospinal fluid (CSF) to the leptomeninges and pachymeninges. Pathologically confirmed, magnetic resonance imaging-visible leptomeningeal spread of gliosarcoma via the CSF has not been previously reported.

Primary Intracranial Sarcomatoid Carcinoma Arising From a Recurrent/Residual Epidermoid Cyst of the Cerebellopontine Angle

Primary intracranial squamous cell carcinomas (SCCs) are rare and mostly associated with an intracranial epidermoid or dermoid cyst. Sarcomatoid carcinoma is a rare biphasic tumor composed of both carcinomatous and sarcomatous components and has not previously been reported as a primary intracranial tumor. Here, we present a case of a 60-year-old man with a primary intracranial sarcomatoid carcinoma, arising from the remnants of the previously resected epidermoid cyst in the cerebellopontine angle. The resected material had portions of an epidermoid cyst lined by normal and dysplastic squamous epithelia and invasive keratinizing SCC. This area was in continuity with areas of highly pleomorphic, anaplastic sarcomatoid cells. Brisk mitotic activity and extensive areas of necrosis were found. On immunohistochemical staining, the cells of the conventional SCC were positive for cytokeratin 5/6, pancytokeratin, epithelial membrane antigen, p63, and p53. The sarcomatoid cells were diffusely and strongly positive for vimentin, p53, smooth muscle actin, and, focally, muscle-specific actin. Occasional sarcomatoid cells coexpressed cytokeratin 5/6, pancytokeratin, p63, and S100 protein. The patient subsequently developed leptomeningeal spread and died 4 months after the second surgery. This rare entity expands the morphologic spectrum encountered in primary intracranial carcinoma.

Fulminant chemical ventriculomeningitis following intrathecal liposomal cytarabine administration

Intrathecal liposomal cytarabine (ITLC) has shown prolonged time to neurological deterioration in some patients with leptomeningeal spread of breast cancer. We describe a patient with fulminant chemical ventriculomeningitis leading to cerebral edema and death following ITLC administration. A 56-year-old Caucasian female developed progressive headache, depressed level of consciousness and adventitious movements immediately following ITLC. Neurological examination was normal prior to injection. This progressed to loss of all brainstem function and a head CT scan demonstrated diffuse cerebral edema. Due to the absence of neurological function on examination, the family opted to withdraw care. To our knowledge we report the first patient with fulminant chemical ventriculomeningitis leading to cerebral edema and death following ITLC administration. Adjunctive glucocorticoids are recommended. Treatment options are limited and complicated by the liposomal formulation of intrathecal cytarabine.

Phase II trial of bevacizumab with fotemustine in recurrent glioblastoma: Final results of a multicenter study of AINO (Italian Association of Neuro-oncology).

2027 Background: Bevacizumab has shown activity in recurrent glioblastoma (GBM), and few data are available on the combination of bevacizumab and nitrosoureas, that represent the standard cytotoxic option. Fotemustine (FTM) is a nitrosourea with elevated lipophilic properties. Methods: In this phase II study patients with GBM recurrent after surgery, radiation therapy, and concomitant/adjuvant temozolomide were elegible. The treatment consisted of an induction phase with BV at 10 mg/kg intravenously on day 1 and 15 and fotemustine (FTM) at 75mg/m2intravenously on day 1 and 8, followed after 3 week interval by a maintenance phase with BV at 10 mg/kg i.v. and FTM 75mg/ m2 every 3 weeks until tumor progression or unacceptable toxicity. Patients had undergone clinical and MRI assessment 1 month after the start of treatment and thereafter every 2 months. The primary endpoint was progression-free survival at 6 months (PFS6), whereas secondary endpoints were response rate (RR), based on RANO criteria, progression-free (PFS) and overall survival (OS), and safety. Results: From April 2008 until November 2010, 54 patients (males 35, females 19, median age 57) were enrolled. PFS6, PFS12 and mPFS were 44%, 21% and 5.29 months respectively. mOS was 9.13 months with 77.4% and 31% of patients surviving at 6 and 12 months respectively. Response rates were as follows: 2CR (4%), 24 PR (44%), 22 SD (41%) and 6 PD (11%). A significant neurological improvement was observed in 57 % of patients, being steroids reduced or interrupted in 64%. 44/54 (81%) patients have progressed and patterns of progression were local in 29/44 (66%), multicentric 10/44 (23%), gliomatosis 3/44 (6%) and isolated leptomeningeal spread 2/44 (5%). 12/54 (22%) patients with grade 3/4 piastrinopenia/leukopenia discontinued fotemustine, whereas 4/54 (7.4%)discontinued bevacizumab (1 stroke, 1 intratumoral haemorrhage, 1 GI perforation and 1 pulmonary embolism). Conclusions: Combination of bevacizumab and fotemustine in glioblastomas recurrent after standard radiotherapy + temozolomide is safe and promising. The analysis of MGMT methylation status is ongoing.

Clinicopathological features of breast cancers predict the development of leptomeningeal metastases: a case-control study

The incidence of leptomeningeal metastases (LM) in patients with breast cancer (BC) is increasing as a result of increased screening and improved patient survival. However, the median survival time after diagnosis of LM is between 5 weeks (without any treatment) and 5 months (for aggressively treated patients). In an attempt to identify clinicopathological risk factors for LM, we carried out a case-control study of 100 women with BC. Fifty patients with BC and LM were enrolled and an additional 50 patients with BC and no CNS metastases including leptomeningeal spread were selected as controls. Patients who had developed LM were selected between December 2006 and August 2008. The control group was matched for: age at diagnosis, year of diagnosis, and initiation of chemotherapy at BC diagnosis. The ILC type (P = 0.03), ER-negative (P = 0.01) and PR-negative status (P = 0.03), and initial M+ status at BC diagnosis (P = 0.008) tended to be more frequent in LM patients. These characteristics should lead to early appropriate assessments being performed in this targeted population when a neurological complaint appears, in order to detect LM as soon as possible.

Use of T2 signal intensity of cerebellar neoplasms in pediatric patients to guide preoperative staging of the neuraxis

The question of whether to obtain routine or selective preoperative imaging of the neuraxis in pediatric patients with cerebellar neoplasms remains a controversial topic. Staging of the neuraxis is generally considered beneficial in patients with neoplasms associated with an elevated risk of leptomeningeal dissemination (LD). When these studies are obtained preoperatively, there is a decrease in the number of false-positive images related to debris in the immediate postoperative period. Additionally, knowledge of the extent of spread has the potential to affect the risk/benefit analysis of aggressive resection. Although the majority of pediatric neurosurgeons surveyed choose to obtain selective preoperative imaging of the neuraxis in cases of cerebellar neoplasms "with findings suggestive of high-grade pathology," an evidence-based protocol in the literature is lacking. The goal of this study was to assess radiological characteristics of tumors with an elevated risk of LD and identify a method to help guide preoperative imaging of the neuraxis. The authors first reviewed the literature to gain an appreciation of the risk of LD of pediatric cerebellar neoplasms based on underlying histopathology and/or grade. Available evidence indicates preoperative imaging of the neuraxis in patients with Grade I tumors to be of questionable utility. In contrast, evidence suggested that preoperative imaging of the neuraxis in patients with Grades II-IV neoplasms was clinically warranted. The authors then evaluated an extensive base of neuroradiological literature to identify possible MR imaging and/or CT findings with the potential to differentiate Grade I from higher-grade neoplasms in pediatric patients. They analyzed the preoperative radiological findings in 50 pediatric patients who had undergone craniotomy for resection of cerebellar neoplasms at Vanderbilt Children's Hospital since 2003 with reference to 7 chosen radiological criteria. Logistic regression models were fit using radiological features to determine the best predictors of Grades II-IV tumors. Receiver operating characteristic methods were used to identify diagnostic properties of the best predictors. The relative T2 signal intensity (RT2SI), an indirect measure of the water content of the solid component of the tumor, was best able to identify neoplasms with an elevated risk of LD. An RT2SI value of 0.71 was selected by the authors as the best operating point on the curve. Of the 31 neoplasms retrospectively designated as hypointense T2-weighted lesions (RT2SI ≤ 0.71), 30 (97%) were Grade II or higher. All medulloblastomas, ependymomas, and high-grade (Grades III and IV) neoplasms were hypointense T2-weighted lesions. Of the 19 T2-weighted hyperintense neoplasms (RT2SI > 0.71), 16 (84%) were Grade I and 3 were Grade II. Measurement of the RT2SI can help predict Grade II-IV tumors at an elevated risk of leptomeningeal spread and guide staging of the neuraxis. Pediatric patients with cerebellar neoplasms found to have an RT2SI of less than or equal to 0.71 are recommended for neuraxis imaging prior to surgery.

Rhabdoid glioblastoma in a child: case report and literature review

Rhabdoid glioblastoma is a rare type of glioblastoma characterized by cells resembling rhabdomyoblasts. Several reports have identified its aggressive clinical course and the pathological differences from other primary brain tumors. We report a case of rhabdoid glioblastoma in a 12-year-old boy who presented with headache and harbored a 70-mm solid tumor in the left temporal lobe. The tumor was surgically excised, but early tumor recurrence and leptomeningeal spread developed, and the patient died of the disease 4.9 months after surgery. Histologically, the tumor contained two distinct patterns of glioblastoma and rhabdoid cells with necrosis and hemorrhage. Immunohistochemical analysis revealed that both cells were positive for glial fibrillary acid protein, vimentin, and INI1, which is consistent with the reported diagnosis of rhabdoid glioblastoma. Genetic studies confirmed no loss of the INI1 gene and identified hemizygous deletion of the CDKN2A gene. We review reported cases of rhabdoid glioblastoma and summarize the clinical, radiological, and histological features.

Diseminación leptomeníngea de un astrocitoma pilocítico cervical en el adulto: publicación de un caso y revisión de la literatura

BACKGROUND. The rarest location of pilocytic astrocytoma is intramedullary. Gliomas represent up to 24 - 30% of intramedullary tumors in adulthood and are second only after ependymomas. Leptomeningeal dissemination through cerebrospinal fluid is unusual and occurs predominantly in medulloblastomas, ependymoblastomas, central neuroblastomas, ependymomas, germ cell tumors and high-grade gliomas. The majority of spinal cord gliomas reporting metastasis were anaplastic astrocytomas or glioblastomas multiforme and relatively few were low-grade gliomas. The incidence of leptomeningeal spread of low-grade tumors is rare. A rare cranial extension of brain leptomeningeal dissemination in an intramedullary pilocytic astrocytoma during adulthood is reported. CASE REPORT. A 51 year-old-man with a recurrent intramedullary mass at C5-C7 level operated 4 times with all pathological anatomy reports describing the lesion as Pilocytic Astrocytoma developed, after 15 years from the diagnosis, visual hallucinations and his level of consciousness worsened to Glasgow coma score 13/15. The MRI showed highly enhanced cranial and spinal leptomeninges and paquimeninges with a micro nodular-granulomatous aspect associated with intense affectation of basal cisterns, subarachnoid spaces and convexity of both cerebral hemispheres suggestive of leptomeningeal spread of the spinal mass. The patient expired after three days. CONCLUSION. Leptomeningeal spread is a rare phenomenon and when it happens usually doesn't change the primary tumor's behavior. In our case the aggressiveness could be explained by a potential malignization of the primary tumor that it was not documented because of the partial resections from the lasts surgeries or instead the tumor was actually a monomorphous pilomyxoid tumor.

Preoperative diffuse leptomeningeal spread in a medulloblastoma: Paraplegia following surgery for posterior fossa and call for newer management protocols

Management of patients with medulloblastoma presenting with multiple metastasis in subarachnoid space preoperatively is unclear. An 11–year-old boy presented with vermian medulloblastoma with an unusually long segment cervico-dorsal lesion and suprasellar lesion. The child underwent a posterior fossa craniotomy (prone position) and excision of vermian mass. He developed paraplegia in the immediate postoperative period, the possible causes for which are being discussed. Besides, treatment options for patients presenting with disseminated disease preoperatively have been highlighted. Preoperative chemotherapy to downstage such lesions may be tried and such complications could be avoided.

Symptomatic spinal metastases of intracranial glioblastoma: clinical characteristics and pathomechanism relating to GFAP expression

To demonstrate clinical characteristics of symptomatic spinal metastases of intracranial glioblastoma multiforme (GBM) and different spreading mechanisms relating to astrocytic cell differentiation, we present an extraordinary case of a 47-year-old patient with rapid progressive paraplegia due to coincident intramedullary and leptomeningeal dissemination of a supratentorial GBM. Serial biopsies of the intracranial, leptomeningeal, and intramedullary GBM lesions of our patient were analyzed for glial fibrillary acidic protein (GFAP). Furthermore, we present 19 additional cases of intracranial GBM with symptomatic spinal seeding, identified through literature review. GFAP expression was high in intracranial and intramedullary tumors, but low in leptomeningeal dissemination of our patient. Mean patient age was 45 years. Mean interval between identification of spinal metastases and death was 4.5 months. Mean overall survival was 18.6 months. Location of symptomatic spinal metastases was more frequently leptomeningeal (14 cases) than intramedullary (7 cases). The case presented herein supports the hypothesis of higher incidence of low GFAP expression in GBM cells in leptomeningeal manifestations after primary intracranial GBM. Because of the proposed tendency for early leptomeningeal spread from primary tumors with low astrocytic differentiation (low GFAP expression), patients with these tumors should be followed more closely to identify leptomeningeal tumor progression early on. Early identification of leptomeningeal spread could enable these patients to benefit from radiation therapy before they develop severe neurological deficits, which might translate into longer acceptable quality of life for these mostly young patients. This is an important finding, but further prospective studies are needed to verify our observations.

Reconsidering the lifetime deferral of blood donation by men who have sex with men

Although leptomeningeal spread (LMS) of primary CNS tumors in children has been well documented in the literature, it has rarely been reported in children with low-grade gliomas. Between 1975 and 1985, 6 of 162 children (3.7%) with low-grade gliomas treated at Children9s Hospital of Philadelphia had LMS. LMS was present at diagnosis of the original tumor in one patient, was the first sign of relapse in one patient, occurred simultaneously with local relapse in two patients, and after local relapse in two patients. Pathology of the original tumor was low-grade astrocytoma in five and low-grade oligodendroglioma in one. Primary tumor site was cervical cord in three, chiasm in one, frontal lobe in one, and cerebellum in one. All of the children with LMS had undergone surgical treatment at the time of diagnosis of the primary tumor; four had total resections at some point in their course. Three of the six patients died; three are still alive after treatment with radiation therapy and/or chemotherapy. The longest survival to date has been 3¹/₂ years after diagnosis of LMS. We compared clinical characteristics of these six patients with 131 children with low-grade tumors without dissemination treated at our institution during the same time period. LMS, although relatively infrequent, does occur in children with low-grade gliomas, especially spinal cord tumors. LMS may occur at any time during illness and diagnosis may be difficult unless LMS is suspected. Treatment, at times, results in clinical improvement and considerable disease control.

Brain metastases in esophageal carcinoma.

e14609 Background: Brain metastases (BM) are uncommon (< 2%) in esophageal carcinoma (EC). With our anecdotal experience suggesting this occurrence to be more frequent, a retrospective review was undertaken to investigate this pattern of spread in EC. Methods: Patients (pts) with EC or gastroesophageal junction (GEJ) cancer, and BM were identified from the RPCI tumor registry from Jan, 1998 to July, 2009. Demographic, clinical, and treatment (Rx) data were retrieved. Results: Of 1167 pts with EC/GEJ cancer, 60 pts (5.1%) with BM were included (2 pts excluded due to concomitant lung cancer and vertebral metastases). Median age 65 yrs (range 26-83); male (n = 55); white (n = 57), black (n = 3). Most EC/GEJC were adenocarcinomas (n = 56). Site of the primary was distal in 90% and mid-esophagus in 10%. Clinical stage at presentation was I (n = 2), IIa (n = 3), IIb (n = 5), III (n = 10), IV (n = 38). 46 pts (78%) developed BM only, 8 pts (14%) had leptomeningeal spread only and 5 pts had both. Seven pts (12%) ...

Diffuse central nervous system protoplasmic astrocytoma

Protoplasmic astrocytoma is an extremely rare form of grade II low grade glioma which usually presents as a discrete mass lesion. We describe a 3-year-old female with diffuse protoplasmic astrocytoma with parenchymal involvement and leptomeningeal spread. This tumour proved extremely difficult to diagnose and followed a progressive course. Three superficial biopsies did not give the diagnosis and this was only confirmed 8 months from presentation from a larger fourth biopsy taken deeper from the cerebellum. To our knowledge this case represents the distinct presentation of protoplasmic astrocytoma presenting as extensive diffuse meningeal disease.