Central lobe tumor

Central lobe tumor

Akeret et al., reviewed prospectively collected clinical and imaging data of all patients operated on a supratentorial intraparenchymaprimary brain tumor at the Neuroscience Center Zurichbetween January 2009 and December 2016. The effect of tumor histology, anatomical location and white matter infiltration on seizure prevalence and semiology were assessed using uni- and multivariate analyses.

Of 678 included patients, 311 (45.9%) presented with epileptic seizures. Tumor location within the central lobe was associated with higher seizure prevalence (OR 4.67, 95% CI: 1.90-13.3, p = .002), especially within the precentral gyrus or paracentral lobule (100%). Bilateral extension, location within subcortical structures and invasion of deeper white matter sectors were associated with a lower risk (OR 0.45, 95% CI: 0.25-0.78; OR 0.10, 95% CI: 0.04-0.21 and OR 0.39, 95% CI: 0.14-0.96, respectively). Multivariate analysis revealed the impact of a locationwithin the central lobe on seizure risk to be highly significant and more relevant than histopathology (OR: 4.79, 95% CI: 1.82-14.52, p = .003). Seizures due to tumors within the central lobe differed from those of other locations by lower risk of secondary generalization (p < .001).

Topographical lobar and gyral location, as well as extent of white matter infiltration impact seizure risk and semiology. This finding may have a high therapeutic potential, for example regarding the use of prophylactic antiepileptic therapy1).


Brain mapping with direct electrical stimulation is usefull when the tumor is located near or has infiltrated the central lobe.

To analyze the surgical findings with direct electrical stimulation of the cortex and white matter under general anesthesia during surgery for brain tumors related to the central lobe.

We studied 42 patients operated on from June 2000 to June 2003. We analyzed surgical findings and details of brain mapping.

The mean value of the intensity of the stimulus was greater among those who presented motor deficit prior to surgery (p = 0.0425) and edema on MRI (p = 0.0468) or during anesthesia with continuous propofol (p = 0.001).

The functional mapping of the central lobe may be influenced by severe motor deficit, edema on MRI and propofol’s anesthesia 2).

1)

Akeret K, Serra C, Rafi O, Staartjes VE, Fierstra J, Bellut D, Maldaner N, Imbach LL, Wolpert F, Poryazova R, Regli L, Krayenbühl N. Anatomical features of primary brain tumors affect seizure risk and semiology. Neuroimage Clin. 2019 Jan 25;22:101688. doi: 10.1016/j.nicl.2019.101688. [Epub ahead of print] PubMed PMID: 30710869.
2)

Brainer-Lima PT, Brainer-Lima AM, Brandt CT, Carneiro GS, Azevedo HC. [Intraoperative mapping of motor areas during brain tumor surgery: electrical stimulation patterns]. Arq Neuropsiquiatr. 2005 Mar;63(1):55-60. Portuguese. PubMed PMID: 15830066.

5 aminolevulinic acid fluorescence guided resection of spinal tumor

Multiple studies have attempted to evaluate the utility of 5-ALA-aided resection of spinal neoplasms.

Wainwright et al., from the Westchester Medical CenterTohoku University Hospital, reviewed the existing literature on the use of 5-ALA and PpIXfluorescence as an aid to resection of primary and secondary spinal neoplasms by searching the PUBMED and EMBASE database for records up to March 2018. Data was abstracted from all studies describing spinal neurosurgical uses in the English language.

In the reviewed studies, the most useful fluorescence was observed in meningiomas, ependymomas, drop metastases from cerebral gliomas, and spinal hemangiopericytoma, which is consistent with applications in cerebral neoplasms.

The available literature is significantly limited by a lack of standardized methods for measurement and quantification of 5-ALA fluorescence. The results of the reviewed studies should guide future development of rational trial protocols for the use of 5-ALA guided resection in spinal neoplasms1).


Three hours before the induction of anesthesia, 5-ALA was administered to patients with different intra- and extradural spinal tumors. In all patients a neurosurgical resection or biopsy of the spinal tumor was performed under conventional white-light microscopy. During each surgery, the presence of Protoporphyrin IX fluorescence was additionally assessed using a modified neurosurgical microscope. At the end of an assumed gross-total resection (GTR) under white-light microscopy, a final inspection of the surgical cavity of fluorescing intramedullary tumors was performed to look for any remaining fluorescing foci. Histopathological tumor diagnosis was established according to the current WHO classification.

Fifty-two patients with 55 spinal tumors were included in this study. Resection was performed in 50 of 55 cases, whereas 5 of 55 cases underwent biopsy. Gross-total resection was achieved in 37 cases, STR in 5, and partial resection in 8 cases. Protoporphyrin IX fluorescence was visible in 30 (55%) of 55 cases, but not in 25 (45%) of 55 cases. Positive PpIX fluorescence was mainly detected in ependymomas (12 of 12), meningiomas (12 of 12), hemangiopericytomas (3 of 3), and in drop metastases of primary CNS tumors (2 of 2). In contrast, none of the neurinomas (8 of 8), carcinoma metastases (5 of 5), and primary spinal gliomas (3 of 3; 1 pilocytic astrocytoma, 1 WHO Grade II astrocytoma, 1 WHO Grade III anaplastic oligoastrocytoma) revealed PpIX fluorescence. It is notable that residual fluorescing tumor foci were detected and subsequently resected in 4 of 8 intramedullary ependymomas despite assumed GTR under white-light microscopy.

In this study, 5-ALA-PpIX fluorescence was observed in spinal tumors, especially ependymomas, meningiomas, hemangiopericytomas, and drop metastases of primary CNS tumors. In cases of intramedullary tumors, 5-ALA-induced PpIX fluorescence is a useful tool for the detection of potential residual tumor foci 2).


A study included 10 patients who underwent surgical resection of an intramedullary ependymoma. Nine patients were orally administered 5-ALA (20 mg/kg) 2 hours before the induction of anesthesia. 5-ALA fluorescence was visualized with an operating microscope. Tumors were removed in a standardized manner with electrophysiological monitoring. The extent of resection was evaluated on the basis of intraoperative findings and postoperative magnetic resonance imaging. Histopathological diagnosis was established according to World Health Organization 2007 criteria. Cell proliferation was assessed by Ki-67 labeling index.

5-ALA fluorescence was positive in 7 patients (6 grade II and 1 grade III) and negative in 2 patients (grade II). Intraoperative findings were dichotomized: Tumors covered by the cyst were easily separated from the normal parenchyma, whereas tumors without the cyst appeared to be continuous to the spinal cord. In these cases, 5-ALA fluorescence was especially valuable in delineating the ventral and cranial and caudal margins. Ki-67 labeling index was significantly higher in 5-ALA-positive cases compared with 5-ALA-negative cases. All patients improved neurologically or stabilized after surgery.

5-ALA fluorescence was useful for detecting tumor margins during surgery for intramedullary ependymoma. When combined with electrophysiological monitoring, fluorescence-guided resection could help to achieve maximum tumor resection safely 3).

References

1)

Wainwright JV, Endo T, Cooper JB, Tominaga T, Schmidt MH. The role of 5-aminolevulinic acid in spinal tumor surgery: a review. J Neurooncol. 2018 Dec 29. doi: 10.1007/s11060-018-03080-0. [Epub ahead of print] Review. PubMed PMID: 30594965.
2)

Millesi M, Kiesel B, Woehrer A, Hainfellner JA, Novak K, Martínez-Moreno M, Wolfsberger S, Knosp E, Widhalm G. Analysis of 5-aminolevulinic acid-induced fluorescence in 55 different spinal tumors. Neurosurg Focus. 2014 Feb;36(2):E11. doi: 10.3171/2013.12.FOCUS13485. PubMed PMID: 24484249.
3)

Inoue T, Endo T, Nagamatsu K, Watanabe M, Tominaga T. 5-aminolevulinic acid fluorescence-guided resection of intramedullary ependymoma: report of 9 cases. Neurosurgery. 2013 Jun;72(2 Suppl Operative):ons159-68; discussion ons168. doi: 10.1227/NEU.0b013e31827bc7a3. PubMed PMID: 23149963.

Spinal primitive neuroectodermal tumor

Epidemiology

Primary spinal primitive neuroectodermal tumor (PNET) of the central nervous system has a low incidence. The intraspinal case is very rare. Around 30 cases have been reported so far 1).

Outcome

Spinal PNETs, like their cranial counterparts, are aggressive tumors and patients with these tumors typically have short survival times despite maximal surgery, chemotherapy, and radiation. Because no standard management guidelines exist for treating these tumors, a multitude of therapeutic strategies have been employed with varying success 2)

Case series

Ma et al. from the Beijing Tiantan Hospital summarized the cases of primary spinal PNET available in the database of the institute, either intramedullary or extramedullary cases. Then they did literature review of the same disease.

There were eight cases of primary spinal PNET available in there database, with one intramedullary case and seven extramedullary cases. Surgical resection was performed. The histology diagnosis was PNET. Peri-operative image examinations of the whole central nervous system (CNS) were performed to exclude tumors other than spinal cord origin. Then during literature review, 33 reports of the disease were included. The pre-operative diagnosis rate was low. The disease had a high recurrence rate and poor prognosis given available treatment 3).

Case reports

A 14-year-old teenage girl had suffered from progressive left upper back pain with bilateral lower legs weakness and numbness for 1 year. After treatment, left neck mass was noted 3 years later.

Initially, magnetic resonance imaging (MRI) showed neurogenic tumor involving intradural extramedullary space of T5-T10. Pathology report showed PNET (World Health Organization grade IV) featuring lobules of neoplastic cells with round regular nuclei, high nucleus-to-cytoplasm ratio, and fibrillary cytoplasm. At the time of tumor recurrence, chest MRI then showed recurrent tumor at T2-T3 level of the epidural space with right neural foramina invasion. Brain MRI showed extensive bilateral calvarial metastases and leptomeningeal metastases in the right frontoparietal regions. Bone scan showed multiple bone metastases.

T-spine tumor removal and adjuvant radiotherapy (RT) to T-spine tumor bed were performed in the initial treatment. After clinical tumor recurrence, tumor removal was done again. She then received chemotherapy followed by whole brain irradiation with hippocampal sparing with 35 gray in 20 fractions.

After treatment, follow-up images showed that the disease was under control. There was no neurological sequela. She has survived more than 7 years from diagnosis and more than 4 years from recurrence to date 4).


A 5-year-old Moroccan boy, who presented with torticollis for 1 month. Computed tomography scan and Magnetic resonance imaging of the cervical spine revealed an extradural, dumbbell-shaped mass with extra-spinal extension at the left C1-C6 level. Multiple biopsy specimens were obtained. Histological examination revealed a highly cellular neoplasm composed of diffuse sheets of tumor cells having monomorphic, round to oval, finely vesicular nuclei. Immunohistochemical findings confirmed the diagnosis of intraspinal peripheral primitive neuroectodermal tumor.

After this illustrative case, they reviewed the literature on clinicopathological and therapeutic aspects. In practice, it is important to consider the diagnosis of peripheral primitive neuroectodermal tumor in children and adolescents with an apparent soft-tissue mass located in the spine 5).


A 60-year-old female, which presented clinically as an intraspinal tumor, causing symptoms of lower back pain, numbness and pain in the right lower extremity. The patient underwent tumorectomy. Following primary therapy, the symptoms of spinal cord compression were relieved. The patient underwent several courses of radiotherapy following surgery but refused to continue with chemotherapy. After a further four months, the tumors recurred and the patient succumbed to the disease 6).


A two years old female child presented with weakness both lower limbs. Preoperative MRI of the spine and paravertebral regionIso – hyper intense posterior placed extradural lesion, non contrast enhancing from D11-L2 levels with cord compression D9 to L3 laminectomy done. Granulation tissue found from D11 to L2. with cord compression. The granulation tissue removed in toto. The pathological findings were consistent with PNET. Post operative neurological improvement was minimal. Cranial screening ruled out any intracranialtumour. Hence a diagnosis of primary spinal PNET was made. A review of the literature shows that only 19 cases of primary intraspinal PNETs have been reported to date and the present case extradural in location. Primary intraspinal PNETs are rare tumors and carry a poor prognosis 7).


Multimodal therapy of an intramedullary cervical primitive neuroectodermal tumor in an adult. 8).


A 22-year-old woman with history of severe progressive neck pain, without radiation, associated with paresthesia in the right arm, and palpable right posterior cervical mass. Neurological examination showed increased reflexes in all four limbs, bilateral Hoffman’s sign, right Babinski’s sign, and right hemi-hypoesthesia. Neuroimaging revealed a right posterior cervical lesion with heterogeneous contrast enhancement extending to the neural foramina of the atlas and axis. Patient underwent microsurgical removal of the lesion, and histopathological and immunohistochemical analysis confirmed the diagnosis of peripheral primitive PNET (pPNET). The patient had adjuvant treatment with radiotherapy and chemotherapy. After twelve months, neuroimaging showed no signs of tumor regrowth and the patient had no neurological deficits. However, three months later, the patient developed hydrocephalus and cerebrospinal fluid (CSF) was positive for neoplastic cells. No other treatment was administered and the patient died.

pPNET is a rare malignant tumor with poor prognosis, although promising results with multimodal treatment-surgery, radiotherapy, and chemotherapy. Diagnosis requires immunohistochemical analysis, with identification of neuronal differentiation markers 9).


A 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery 10).


A female who presented at age 21 with diffuse involvement of the lower spinal cord. After biopsy and successful treatment with radiation and chemotherapy, she recurred 10 years later with disease in her cerebellum. She was reinduced with chemotherapy and subsequently received high-dose chemotherapy with autologous stem cell support. She is alive and free of disease 11 years after her initial presentation. This represents the longest survival ever documented for a primary spinal PNET 11).


A 15-year-old girl who presented with gradual onset, over 1 month, of upper back pain and bilateral lower leg weakness. A thoracic spine MRI showed a dumbbell-shaped epidural mass at T2-4 with right paraspinal and posterior mediastinal extension. Surgical resection of the epidural tumor for decompression was performed. The pathologic examination revealed a PNET. Primary spinal PNETs typically have a poor prognosis and optimal therapy has not yet been defined. Surgical resection, with the combination of chemo-radiotherapy or radiotherapy, leads to better outcomes. However, primary epidural PNETs may be classified as a subtype of spinal PNETs because they are free from intrathecal invasion. For these patients, surgery alone and surgery combined with radiotherapy or chemo-radiotherapy remain controversial. Our patient received surgery alone and, 1y ear later, has experienced no local recurrence within the epidural space but the mediastinal part of the tumor has enlarged 12).


A 45-year-old man with a peripheral primitive neuroectodermal tumour arising in the cervical spine. Alexander et al., believed this to be the first report of this type of tumour in the cervical spine 13).


A 25-year-old male patient presented with an extremely rare primary spinal peripheral primitive neuroectodermal tumor (pPNET) manifesting as acutely progressive paraparesis and back pain. Neuroimaging and intraoperative examination showed that the tumor was confined to the epidural space of the thoracic spine. The patient was treated successfully by gross total resection of the tumor followed by chemotherapy and local radiotherapy. The present case illustrates the unexpected occurrence and important differential diagnosis of primary epidural pPNET of the thoracic spine in young patients presenting with progressive paraparesis and back pain 14).


A 29-year-old male with a dumbbell-shaped pPNET at the T9-10 spine level, including details of his examination, surgical procedures applied, histological and genetic findings, and his subsequent treatment. They discussed the clinical course, the pathology and treatment for this disease, the surgical approach to thoracic dumbbell tumors and reviewed the literature. This is the first report of a case of a dumbbell-shaped intradural and spinal peripheral PNET 15).


A 54-year-old woman who presented with quadriplegia and bladder and bowel dysfunction. The patient had suffered symptoms of neck pain for 1 month and left shoulder weakness for 10 days. Magnetic resonance imaging of the cervical spine revealed an intramedullary mass extending from C-2 to C-5 with an exophytic component in the adjacent left subarachnoid space. Multiple biopsy specimens were obtained, and a partial excision was performed. Histological examination revealed nodular growth and neuronal differentiation, with a striking resemblance to desmoplastic medulloblastoma. A positron emission tomography scan did not reveal uptake at any site. These findings confirmed the diagnosis of a primary intramedullary PNET. Postoperatively, the patient was given craniospinal radiotherapy with a radiation boost to the tumor bed 16).

References

1) , 3)

Ma J, Ma S, Yang J, Jia G, Jia W. Primary spinal primitive neuroectodermal tumor: A single center series with literature review. J Spinal Cord Med. 2018 Dec 18:1-9. doi: 10.1080/10790268.2018.1547862. [Epub ahead of print] PubMed PMID: 30561250.
2)

Ellis JA, Rothrock RJ, Moise G, McCormick PC 2nd, Tanji K, Canoll P, Kaiser MG, McCormick PC. Primitive neuroectodermal tumors of the spine: a comprehensive review with illustrative clinical cases. Neurosurg Focus. 2011 Jan;30(1):E1. doi: 10.3171/2010.10.FOCUS10217. Review. PubMed PMID: 21194274.
4)

Chen F, Chiou SS, Lin SF, Lieu AS, Chen YT, Huang CJ. Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases: A case report. Medicine (Baltimore). 2017 Nov;96(46):e8658. doi: 10.1097/MD.0000000000008658. PubMed PMID: 29145292; PubMed Central PMCID: PMC5704837.
5)

Khmou M, Malihy A, Lamalmi N, Rouas L, Alhamany Z. Peripheral primitive neuroectodermal tumors of the spine: a case report and review of the literature. BMC Res Notes. 2016 Sep 9;9(1):438. doi: 10.1186/s13104-016-2246-5. Review. PubMed PMID: 27613377; PubMed Central PMCID: PMC5016941.
6)

Meng XT, He SS. Primitive neuroectodermal tumor in the spinal canal: A case report. Oncol Lett. 2015 Apr;9(4):1934-1936. Epub 2015 Jan 27. PubMed PMID: 25789071; PubMed Central PMCID: PMC4356409.
7)

Venkataraman S, Pandian C, Kumar SA. Primary spinal primitive neuroectodermal tumour – a case report. Ann Neurosci. 2013 Apr;20(2):80-2. doi: 10.5214/ans.0972.7531.200211. PubMed PMID: 25206019; PubMed Central PMCID: PMC4117112.
8)

Coumans JV, Walcott BP, Nahed BV, Oh KS, Chi AS. Multimodal therapy of an intramedullary cervical primitive neuroectodermal tumor in an adult. J Clin Oncol. 2012 Jan 10;30(2):e15-8. doi: 10.1200/JCO.2011.38.6474. Epub 2011 Dec 5. PubMed PMID: 22147740.
9)

Cabral GA, Nunes CF, Melo JO Jr, Guimarães RD, Gonçalves MB, Rodrigues RS, Correa JL, Teixeira OM Jr, Klescoski J Jr, Lapenta MA, Landeiro JA. Peripheral primitive neuroectodermal tumor of the cervical spine. Surg Neurol Int. 2012;3:91. doi: 10.4103/2152-7806.99938. Epub 2012 Aug 21. PubMed PMID: 23050205; PubMed Central PMCID: PMC3463148.
10)

Harbhajanka A, Jain M, Kapoor SK. Primary spinal intramedullary primitive neuroectodermal tumor. J Pediatr Neurosci. 2012 Jan;7(1):67-9. doi: 10.4103/1817-1745.97631. PubMed PMID: 22837786; PubMed Central PMCID: PMC3401662.
11)

Gollard RP, Rosen L, Anson J, Mason J, Khoury J. Intramedullary PNET of the spine: long-term survival after combined modality therapy and subsequent relapse. J Pediatr Hematol Oncol. 2011 Mar;33(2):107-12. doi: 10.1097/MPH.0b013e3181f84b7f. PubMed PMID: 21228722.
12)

Chang SI, Tsai MC, Tsai MD. An unusual primitive neuroectodermal tumor in the thoracic epidural space. J Clin Neurosci. 2010 Feb;17(2):261-3. doi: 10.1016/j.jocn.2009.05.018. Epub 2009 Dec 29. PubMed PMID: 20036552.
13)

Alexander HS, Koleda C, Hunn MK. Peripheral Primitive Neuroectodermal Tumour (pPNET) in the cervical spine. J Clin Neurosci. 2010 Feb;17(2):259-61. doi: 10.1016/j.jocn.2009.05.020. Epub 2009 Dec 29. PubMed PMID: 20036553.
14)

Kiatsoontorn K, Takami T, Ichinose T, Chokyu I, Tsuyuguchi N, Ohsawa M, Ohata K. Primary epidural peripheral primitive neuroectodermal tumor of the thoracic spine. Neurol Med Chir (Tokyo). 2009 Nov;49(11):542-5. PubMed PMID: 19940407.
15)

Hrabálek L, Kalita O, Svebisova H, Ehrmann J Jr, Hajduch M, Trojanec R, Kala M. Dumbbell-shaped peripheral primitive neuroectodermal tumor of the spine–case report and review of the literature. J Neurooncol. 2009 Apr;92(2):211-7. doi: 10.1007/s11060-008-9744-9. Epub 2008 Dec 3. Review. PubMed PMID: 19050994.
16)

Jain A, Jalali R, Nadkarni TD, Sharma S. Primary intramedullary primitive neuroectodermal tumor of the cervical spinal cord. Case report. J Neurosurg Spine. 2006 Jun;4(6):497-502. PubMed PMID: 16776362.
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