ArticlePDF AvailableLiterature Review

Extracranial propagation of glioblastoma with extension to pterygomaxillar fossa

Authors:

Abstract and Figures

Glioblastoma multiforme is a highly malignant primary brain tumor that shows marked local aggressiveness, but extracranial spread is not a common occurrence. We present an unusual case of recurrent glioblastoma in 54-year old male that spread through the scull base to the ethmoid and sphenoid sinuses, to the orbita, pterygomaxillar fossa, and to the neck. A 54-year old male underwent left temporal resection because of brain tumor of his left temporal lobe. Operation was followed by external beam radiation combined with temozolomide. The tumor recurred eight months after first surgery. The patient developed swelling of left temporal region, difficult swallowing and headache. MRI of head showed recurrent tumor, which invaded orbita, ethmoid and sphenoid sinuses, nasal cavity, pterygomaxillar fossa. The patient died ten months after initial diagnosis of glioblastoma multiforme, and two months after his second operation. The aggressive surgical operation helped to downsize the tumor mass as much as possible, but did not prolonged significantly the life or improved the life quality of the patient. The current literature is reviewed, and the diagnostic approaches as well as therapeutic options are discussed.
Content may be subject to copyright.
CAS E REP O R T Open Access
Extracranial propagation of glioblastoma with
extension to pterygomaxillar fossa
Damir Tomac
1
, Darko Chudy
1
, Smiljka Lambaša
2
, Iva Topić
3
, Gordan Grahovac
1*
and Arijana Zoric
4
Abstract
Background: Glioblastoma multiforme is a highly malignant primary brain tumor that shows marked local
aggressiveness, but extracranial spread is not a common occurrence. We present an unusual case of recurrent
glioblastoma in 54-year old male that spread through the scull base to the ethmoid and sphenoid sinuses, to the
orbita, pterygomaxillar fossa, and to the neck.
Methods: A 54-year old male underwent left temporal resection because of brain tumor of his left temporal lobe.
Operation was followed by external beam radiation combined with temozolomide. The tumor recurred eight
months after first surgery. The patient developed swelling of left temporal region, difficult swallowing and
headache. MRI of head showed recurrent tumor, which invaded orbita, ethmoid and sphenoid sinuses, nasal cavity,
pterygomaxillar fossa.
Results: The patient died ten months after initial diagnosis of glioblastoma multiforme, and two months after his
second operation.
Conclusions: The aggressive surgical operation helped to downsize the tumor mass as much as possible, but did
not prolonged significantly the life or improved the life quality of the patient. The current literature is reviewed,
and the diagnostic approaches as well as therapeutic options are discussed.
Background
Glioblastoma multiforme is a highly malignant primary
brain tumor. The median survival with therapy is
approximately 9-12 months[1].Glioblastomashows
marked local aggressiveness, but extracranial spread is
not a common occurrence. It is believed that dura pro-
vides excellent protection against infiltration by malig-
nant tumors. Improvement of treatment options and
survival time led to increase of extracranial recurrence
of glioblastoma. Most commonly glioblastomas metas-
tases are to the lungs, lymph nodes, liver, and bones [2].
We report an exceptional case of glioblastoma multi-
forme spreading extracranially to the orbita, ethmoid
and sphenoid sinuses, nasal cavity, pterygomaxillar fossa,
and neck.
Case Report
A 54-year old Caucasian male presented to Department
of Neurosurgery complaining of severe headaches,
dizziness, and dysarthria that lasted for 2 weeks. His
personal and family history was unremarkable, and his
Karnofsky score was 90. Upon admission MSCT showed
a hypodense lesion in the left temporal lobe. After initial
analysis MRI of the head was scheduled, which showed
a ring-enhancing lesion in the left temporal lobe.
(Figure 1) We preformed left temporal osteoplastic
craniotomy, and tumor was removed along with the sur-
rounding normal brain tissue (Figure 2). Regression of
dysarthria was noticed after the operation, and his Kar-
nofsky score was 100 at discharge. Histopathological
analysis confirmed diagnosis of glioblastoma, gradus IV
according to WHO. After discharge patient was sent for
oncological evaluation.
The patient received radiotherapy in daily factions of 2
Gy given 5 days per week for 6 weeks, for total 60 Gy
plus continuous daily temozolomide (75 mg per square
meter of body-surface area per day). Following with the
six cycles of the adjuvant temozolomide therapy (150 to
200 mg per square meter for 5 days).
Three months after operation control MSCT showed
no signs of tumor. Eight months after the first operation
* Correspondence: ggrahov@mef.hr
1
Department of Neurosurgery, Clinical Hospital Dubrava, Zagreb, Croatia
Full list of author information is available at the end of the article
Tomac et al.World Journal of Surgical Oncology 2011, 9:53
http://www.wjso.com/content/9/1/53 WORLD JOURNAL OF
SURGICAL ONCOLOGY
© 2011 Tomac et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons
Attribution License (http://creative commons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, pro vided the original work is properly cited.
MRI was performed and revealed a tumor that involved
middle cranial fossa with extension to the left orbita,
ethmoid and sphenoid sinuses, nasal cavity, and pterygo-
maxillar fossa (Figure 3, Figure 4). The patient had Kar-
nofsky score of 70 at second admission when he was
transferred to Department of Neurosurgery. Left tem-
poral recraniotomy and reduction of intracranial tumor
and tumor in pterygomaxillar fossa was performed.
Zygoma and left side of the mandible were resected.
Parotid gland and masseter muscle were used for defect
Figure 1 Axial T1 weighted contrast enhanced MRI image demonstrating ring-enhanced lesion of the left temporal lobe
Tomac et al.World Journal of Surgical Oncology 2011, 9:53
http://www.wjso.com/content/9/1/53
Page 2 of 6
reconstruction. Histological analysis showed glioblas-
toma multiforme with invasion of bone, muscles, and
blood vessels (Figure 5, Figure 6 and Figure 7). The
patient died two months after second operation. The
patient and the family declined any other oncology
treatment because the Karnofsky score at discharge was
40, which rapidly deteriorated after discharge form the
hospital. Autopsy was not performed.
Despite malignant nature of glioblastoma multiforme,
extracranial metastases are rare [3]. Glioblastoma are
prevented from metastasing by the relatively impassable
dura, tough basal membrane around intracerebral blood
vessels, and lack of true lymphatics in the brain[4].
Local dissemination to the scalp, face, and neck usually
occurs after operations and failure of closure of the
dura, or after shunt operations. Such procedures can
facilitate tumor cells to enter vascular system, extracra-
nial lymphatic system, or directly enter the peritoneum
in the setting of a ventriculoperitoneal shunt. Direct
bone invasion, which might interfere with local dural
blood supply resulting in dural necrosis, is also possible.
According to pathohistological findings in our patient,
Figure 2 Axial contrast enhanced MSCT of the head showing no signs of tumor.
Tomac et al.World Journal of Surgical Oncology 2011, 9:53
http://www.wjso.com/content/9/1/53
Page 3 of 6
we believe that extracranial spread was caused by
angioinvasion and invasion of the bone of the scull base.
Similarly, surgery may also have made metastases more
likely by simply prolonging the life of the patient [5].
Although rare, there are cases of glioblastoma multi-
forme with extracranial metastases in the absence of
previous craniotomies [2,6]. Various mechanisms of
spontaneous transdural spread have been described. The
tumor can extend through the perivascular or dural slit,
the increase of the intracranial pressure over a long per-
iod of time will allow the cerebral cortex to insinuate
itself wherever possible through the dura, or transdural
extension may originate by infiltration of tumor cells
into the previously herniated normal brain substance
[7]. The tumor can also pass through the dura mater by
way of cranial or spinal nerves [8], or the dura can be
directly destroyed by the tumor.
Conclusions
In summary, the extra cranial spread of glioblastoma
multiforme is a rare occurrence. In this report we pre-
sented unusual case of extra cranial spread of glioblas-
toma multiforme after resection and concomitant
radiotherapy with chemotherapy. The tumor showed
Figure 3 Axial T1-weighted contrast enhanced MRI image demonstrates extra cranial portion of the tumor extended to the sphenoid
and ethmoid sinuses, nasal cavity, and orbit.
Tomac et al.World Journal of Surgical Oncology 2011, 9:53
http://www.wjso.com/content/9/1/53
Page 4 of 6
aggressive clinical behavior after standard treatment in
very short period of time. The recurrent tumor extended
to the orbit, ethmoid and sphenoid sinuses, and to the
pterygomaxillar fossa. During second surgery we could
observe the adherent brain tumor mass to the temporal
dura. The tumor destructed the floor of the skull base;
the dura mater was absent from the floor of the skull
base. The pathological findings revealed invasion of the
bone of the skull base, and underlying masticator mus-
cles in the infratemporal fossa. Due to intraoperative
and histological findings we believe that the tumor
spread with direct invasion of the dura and underlying
scull base and mastication muscles. In the later stage of
disease tumor showed angioinvasion. Aggressive opera-
tion can downsize the tumor mass but the life quality
may not be improved significantly.
Consent
Written informed consent was obtained from the patient
for publication of this Case report and any accompanying
Figure 4 Sagittal T1-weighted contrast enhanced MRI image demonstrates intracranial and extra cranial portion of the tumor
extended to the sphenoid and ethmoid sinuses, nasal cavity, orbit, pterygomaxillar fossa, and neck.
Tomac et al.World Journal of Surgical Oncology 2011, 9:53
http://www.wjso.com/content/9/1/53
Page 5 of 6
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
List of abbreviations
MRI: magnetic resonance imaging; MSCT: multi slice computed tomography;
WHO: World health organization
Author details
1
Department of Neurosurgery, Clinical Hospital Dubrava, Zagreb, Croatia.
2
Department of Pathology, Clinical Hospital Dubrava, Zagreb, Croatia.
3
Department of Otorhinolaryngology, Head and Neck Surgery, Clinical
Hospital Center Zagreb, Croatia.
4
Rudjer Boskovic Institute, Division for
Molecular Medicine, Laboratory of Molecular Oncology, Zagreb, Croatia.
Authorscontributions
DT collected the data, analyzed data and wrote the paper, DC gave
conceptual design and edited the paper, SL gathered pathological pictures
and interpreted them, IT supervised and edited the paper, GG wrote the
paper, AZ supervised the paper end edited the paper. All authors read and
approved the final manuscript.
Competing interests
All authors declare that they do not have any financial or non-financial
competing interests in relation in relation to this manuscript.
Received: 20 December 2010 Accepted: 19 May 2011
Published: 19 May 2011
References
1. Taha M, Ahmad A, Wharton S, Jellinek D: Extra-cranial metastasis of
glioblastoma multiforme presenting as acute parotitis. Br J Neurosurg
2005, 19:348-351.
2. Pasquier B, Pasquier D, NGolet A, Panh MH, Couderc P: Extraneural
metastases of astrocytomas and glioblastomas: clinicopathological study
of two cases and review of literature. Cancer 1980, 45:112-125.
3. Ates LE, Bayindir C, Bilgic B, Karasu A: Glioblastoma with lymph node
metastases. Neuropathology 2003, 23:146-149.
4. Wallace CJ, Forsyth PA, Edwards DR: Lymph node metastases from
glioblastoma multiforme. AJNR Am J Neuroradiol 1996, 17:1929-1931.
5. Rubinstein LJ: Development of extracranial metastases from a malignant
astrocytoma in the absence of previous craniotomy. Case report. J
Neurosurg 1967, 26:542-547.
6. Anzil AP: Glioblastoma multiforme with extracranial metastases in the
absence of previous craniotomy. Case report. J Neurosurg 1970, 33:88-94.
7. Sanerkin NG: Transdural spread of glioblastoma multiforme. J Pathol
Bacteriol 1962, 84:228-233.
8. Orita T, Nishizaki T, Furutani Y, Aoki H: Extradural nasal and orbital
extension of malignant glioma. Case report. Surg Neurol 1989, 31:395-399.
doi:10.1186/1477-7819-9-53
Cite this article as: Tomac et al.: Extracranial propagation of
glioblastoma with extension to pterygomaxillar fossa. World Journal of
Surgical Oncology 2011 9:53.
Submit your next manuscript to BioMed Central
and take full advantage of:
Convenient online submission
Thorough peer review
No space constraints or color figure charges
Immediate publication on acceptance
Inclusion in PubMed, CAS, Scopus and Google Scholar
Research which is freely available for redistribution
Submit your manuscript at
www.biomedcentral.com/submit
Figure 5 Tissue sections showing a glioblastoma multiforme
with invasion in muscle tissue (hematoxylin-eosin stain, ×400).
Figure 6 Tissue sections showing a glioblastoma multiforme
with bone invasion (hematoxylin-eosin stain, ×400).
Figure 7 Tissue sections showing a glioblastoma multiforme
with angioinvasion (hematoxylin-eosin stain, ×400).
Tomac et al.World Journal of Surgical Oncology 2011, 9:53
http://www.wjso.com/content/9/1/53
Page 6 of 6
... showed that 43.5% of patients had a pathological fracture at presentation, while 56.5% of patients developed a pathological fracture during treatment. [2] Additionally, 34.6% of the fractures were displaced whereas 65.4% were undisplaced. [2] Scully et al., reported no statistical correlation on either timing of fracture development or fracture displacement on outcome measures like a local recurrence and mortality rates. ...
... [2] Additionally, 34.6% of the fractures were displaced whereas 65.4% were undisplaced. [2] Scully et al., reported no statistical correlation on either timing of fracture development or fracture displacement on outcome measures like a local recurrence and mortality rates. [6] Additionally, there are no studies in the literature which have looked at the effect of tumor size and hematoma size on clinical outcomes such as local and distant recurrence rates. ...
... [1] There have, however, been reports of gliomas invading other skull base regions. [2] Macroscopically, extradural extension of gliomas has been described to occur through perivascular or dural slits; along the cranial nerves; or via., direct dural destruction. [3] The latter route is the implicated mechanism following radiation therapy or a previous surgery. ...
... [2][3][4] Tumorous cells can infiltrate and destroy the dura. 5 The tumour can also migrate across the dura mater through the cranial nerve foramina. 6 7 All of these mechanisms may lead to an invasion of the skull base with infiltration of bones, neighbouring soft tissues, paranasal sinuses, orbit and infratemporal fossa. 1 8 9 Learning points ► Glioblastoma is a tumour that can spread extracranially through the dura mater. ...
... Extracranial GBM extensions to the neck, ethmoidal, maxillary and sphenoid sinuses, orbit, pterygomaxillar and nasal fossa [10][11][12][13][14][15], parotid gland [16], and retroauricular region [17] have been reported. Only four primary gliosarcoma case reports are described in the literature with transcranial (intradural to extradural) penetration into the region of the infratemporal fossa [18][19][20][21]. ...
Article
Full-text available
Only four primary gliosarcoma case reports are described in the literature with transcranial (intradural to extradural) penetration into the region of the infratemporal fossa. This is the first report of a primary glioblastoma (GBM) that evolved into secondary or post-treatment gliosarcoma without evidence of a second de novo tumor and with extension into the left pterygomaxillary fossa.
... органах [12]. Экстракраниальный метастаз ГБ может сопровождаться прорастанием твердой мозговой оболочки, особенно в областях прилегания к естественным отверстиям черепа и синусам [11,13]. Клетки ГБ могут распространяться различными путями: по волокнам проводящих путей головного мозга [14][15][16][17], ликворной системе [18] и реже гематогенным и контактным путями [7][8][9]. ...
Article
Introduction - since the 1990s, the literature has described cases of glioblastoma metastases with the development of foci located at a distance from the primary tumor. However, the pathogenesis of this process remains unclear until the end. This focus is believed to result, on the one hand, from tumor metastasis from the primary site and, on the other hand, from multifocal growth. This article presents a literature review and a description of clinical observations of patients with glioblastoma metastases. The study included 6 patients (1 female and 5 males) with brain glioblastomas who received treatment at the Burdenko Neurosurgical Institute (5 patients) and the Department of Neurosurgery of the Research Center of Neurology (1 patient) in the period from 2010 to 2014. Neurophysiological control was used if the tumor was localized near the eloquent cortical areas and pathways; 4 of 6 patients were operated on using the methods of intraoperative fluorescence diagnosis (5-ALA agent - Alasens). Four patients had metastases within one hemisphere, two had metastases in the contralateral hemisphere in the period of 5 to 18 months after the first operation. The primary tumor site was located near the ventricular system in two patients. In one patient, the lateral ventricle was opened during the first operation. In another patient, the prepontine cistern was opened during the first operation. In two patients, the primary tumor site was located at a distance from the lateral ventricles, however, the tumor was located near them during recurrence. Based on metabolic navigation, fluorescence of the tumor was observed in the four patients during both the first and repeated operations. The close relationship between primary glioblastomas and metastases and the cerebrospinal fluid circulation pathways may confirm the fact of dissemination of tumor cells with cerebrospinal fluid flow. In our opinion, there should be an increased suspicion of the possibility for metastases of glioblastomas that are closely associated with the cerebrospinal fluid circulation pathways. Metabolic navigation with 5-ALA is effective both during primary surgery in patients with glioblastomas and during resection of glioblastoma metastases.
Article
Glioblastoma multiforme is the most common primary brain tumor. It is locally aggressive but rarely spreads outside the central nervous system. We present an unusual case of a 57-year-old woman who had presented 1 year after surgical resection and adjuvant therapy, with evidence of recurrent tumor invading through the skull base into the orbital apex, masticator, and pterygoid space. We have also reviewed all available case reports on local invasion of glioblastoma that occurred in absence of treatment and recurrence that developed away from the initial surgical location.
Article
Full-text available
Many developing countries are lagging behind in reporting epidemiological data for individual central nervous system (CNS) tumors. This paper aimed to elicit patterns for the epidemiology of individual World Health Organization (WHO) classified CNS tumors in countries registered by WHO as "developing". Cyber search was carried out through 66 cancer networks/registries and 181 PubMed published papers that reported counts of CNS tumors for the period of 2009-2012. The relationship between the natural log of incidence Age Standardized Rate (ASR) reported by Globocan and Latitude/ Longitude was investigated. Registries for 21 countries displayed information related to CNS tumors. In contrast tends for classified CNS tumor cases were identified for 38 countries via 181 PubMed publications. Extracted data showed a majority of unclassified reported cases [PubMed (38 countries, 45.7%), registries (21 countries, 96.1%)]. For classified tumors, astrocytic tumors were the most frequently reported type [PubMed (38 countries, 1,245 cases, 15.7%), registries (21 countries, 627 cases, 1.99%]. A significant linear regression relationship emerged between latitudes and reported cases of CNS tumors. Previously unreported trends of frequencies for individually classified CNS tumors were elucidated and a possible link of CNS tumors occurrence with geographical location emerged.
Article
Full-text available
We present an unusual case of extracranial metastasis of glioblastoma multiforme (GBM) to the parotid gland and cervical lymph nodes. The patient had previously undergone two craniotomies to debulk a left frontal GBM, followed by radiotherapy. After the second craniotomy, while waiting for chemotherapy, the patient was re-admitted with a short history of a painful swelling of his left parotid gland. The initial diagnosis was infective parotitis; however, as there was no improvement with broad-spectrum antibiotics, CT was undertaken, which revealed a mass in the parotid gland with a necrotic centre and enlarged cervical lymph nodes. Parotid gland biopsy revealed a parotid GBM metastasis. This case illustrates how GBM behaves in an aggressive manner even outside the CNS. A brief review of the literature and of the theories, which might explain the extra-neural metastasis of this tumour is also presented.
Article
A rare case of extradural nasal and orbital extension of malignant glioma is presented. The development of malignant changes was observed during 10 years. The mode of transdural extension was via the olfactory nerve, where it was directly destructive in all four cases that have been reported to date. The biological malignancy of transdurally extending glioma is also described.
Article
Two cases of intracranial gliomas with extraneural metastases are described. Case 1, studied with biopsy material only, was a left malignant astrocytoma from the area of the rolandic fissure with right cervical lymph nodes metastases in a 43-year-old man. Case 2 was a left temporal malignant astrocytoma in a 21-year-old woman. Fifteen days after craniotomy, a left submandibular lymph node metastasis appeared. Forty days after surgery, a ventriculoperitoneal shunt was performed. Fifty-four days after surgery, the patient died. Autopsy revealed three liver metastases. Our review of the literature consists of 72 autopsy cases with extraneural deposits. Those metastases occurred mainly in adults (63/72) and among men (46/72). The primary glioma was supratentorial in 67 cases. Metastases were mainly pulmonary and pleural. The majority of patients (82.8%) died within 2 years after onset of symptoms. In 8 of the cases, metastasis developed without any craniotomy and in 8 other cases, through a shunt.
Article
A rare case of extradural nasal and orbital extension of malignant glioma is presented. The development of malignant changes was observed during 10 years. The mode of transdural extension was via the olfactory nerve, where it was directly destructive in all four cases that have been reported to date. The biological malignancy of transdurally extending glioma is also described.
Article
Two cases of intracranial gliomas with extraneural metastases are described. Case 1, studied with biopsy material only, was a left malignant astrocytoma from the area of the rolandic fissure with right cervical lymph nodes metastases in a 43-year-old man. Case 2 was a left temporal malignant astrocytoma in a 21-year-old woman. Fifteen days after craniotomy, a left submandibular lymph node metastasis appeared. Forty days after surgery, a ventriculoperitoneal shunt was performed. Fifty-four days after surgery, the patient died. Autopsy revealed three liver metastases. Our review of the literature consists of 72 autopsy cases with extraneural deposits. Thos metastases occurred mainly in adults (63/72) and among men (46/72). The primary glioma was supratentorial in 67 cases. Metastases were mainly pulmonary and pleural. The majority of patients (82.8%) died within 2 years after onset of symptoms. In 8 of the cases, metastasis developed without any craniotomy and in 8 other cases, through a shunt.
Article
Extraneural metastases from glioblastoma multiforme are rare. Spread to the extracranial head and neck may be evident on routine follow-up images of the original lesion. We present two cases, one with documented metastatic adenopathy in the head and neck from glioblastoma and the other with probable metastatic disease in a lymph node in which biopsy was not performed, and discuss probable mechanisms of extraneural extension of this tumor.
Article
Metastatic spread of malignant astrocytomas is rare and documented in very few patients with this tumor. Both pathologists and clinicians may confront more of these cases as the patients live longer. We present a 40-year-old-man with glioblastoma multiforme metastasizing to the supraclavicular lymph node after surgery. The tumor was located at the parietal convexity and several lymphadenopathies occured after surgery.
Article
Korman, Ruth Z. (Elmira College, Elmira, N.Y.) and David T. Berman. Genetic transduction with staphylophage. J. Bacteriol. 84:228–236. 1962.—Transduction by crude lysates obtained by ultraviolet-light induction of phage 53 lysogenic derivatives of Staphylococcus aureus NCTC 8511 has been confirmed. A defined medium and sugar-differential medium were developed, permitting demonstration of transmission of sugar-fermentation loci. The range of transmissible markers has been extended to include free-coagulase production as well as antibiotic-resistance markers. A rare class of heterogeneous streptomycin-resistant transformed clones was obtained. These were assumed to be heterogenotic.