3_Papillary_CARANGELO.qxp_- 13/05/15 16:19 Pagina 63
G Chir Vol. 36 - n. 2 - pp. 63-69 March-April 2015
original article
Papillary glioneuronal tumor: case report and review of literature B. CARANGELO1, U. ARRIGUCCI2, A. MARIOTTINI1, L. LAVALLE1, G. MUSCAS1, D. BRANCO1, L. LIPPA1, G. PERI1, M. MUYA1, G. COSTANTINO1, G. TIEZZI1, D. TACCHINI3, A. MATURO4
SUMMARY: Papillary glioneuronal tumor: case report and review of literature. B. CARANGELO, U. ARRIGUCCI, A. MARIOTTINI, L. LAVALLE, G. MUSCAS, D. BRANCO, L. LIPPA, G. PERI, M. MUYA, G. COSTANTINO, G. TIEZZI, D. TACCHINI, A. MATURO Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial sy-
stem, adjacent to the lateral ventricles. In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently. In our report we discuss the clinical, neuroradiological and surgical features. The final result is compared with literature data.
KEY WORDS: Papillary glioneuronal tumor - Mixed glioneuronal tumor - Brain tumor.
Introduction Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial system, adjacent to the lateral ventricles. Well-established examples of glioneuronal tumors include DNET (Dysembryoplastic NeuroEpithelial Tumor), ganglioglioma and desmoplastic infantile ganglioglioma. More recently recognized entities include rosette-forming tumor of fourth ventricle, rosette glioneuronal tumor (RGNT)/ glioneuronal tumor with neuropil-like islands and PGNT (1). In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently.
Department of Neurosurgery, "Le Scotte" University Hospital of Siena, Siena, Italy Department of Neuroradiology and Interventional Neurology, "Le Scotte" University Hospital of Siena, Siena, Italy 3 Department of Pathology, "Le Scotte" University Hospital of Siena, Siena, Italy 4 Department of Surgical Sciences, “Sapienza” University of Rome, Rome, Italy
It was described in approximately 67 cases to date (Table 1). Both CT and MRI studies show PGNTs to be circumscribed supratentorial lesions, extending to the cortex and the white matter, or may be more deeply situated, displacing the ventricular system. Cystic alteration are frequent. Solid components are iso/hypointense in T1 and iso/hyperintense in T2, contrast enhancing in a diffuse, patchy or rimming fashion and may present as mural nodule or plaques within otherwise cystic masses. Calcifications may be seen. Bleeding of lesions are rare but recorded (3), associated with superficial siderosis of neuraxis. Surrounding edema and mass effect are seen in larger lesions. Reported tumor size ranges 1-9 cm (mean 4,5 cm). Hystology showed pseudopapillary pattern consisting of a single layer of astrocytic cells surrounding hyalinized blood cells. Follow-up indicated that this type of tumor had a benign behavior even if recently we found out 5 cases of aggressive PGNT (1, 2, 4, 5, 7, 8).
1
2
Corresponding Author: Biagio Carangelo, e-mail: [email protected] © Copyright 2015, CIC Edizioni Internazionali, Roma
Case report The patient was a 34 yr old woman with complaints of progressive headache started 2 weeks before and more recently confusion, vomiting and dizziness. She had a va63
64 Age/sex 25/F 13/M 30/F 11/M 27/F 36/M 19/F 35/F 52/M 26/F 23/M 18/M 16/M 4/M 75/F 24/F 23/F 14/F 14/F 18/F 28/F 25/M 16/F 16/F 35/M 27/M NA 13/M 67/M 27/M 17/F 15/F 20/F 49/F
Series
Kim et al., (1997) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Bouvier-Labit et al. (2000) Prayson et al. (2000) Broholm et al. (2002) Barnes et al. (2002) Tsukayama et al. (2002) Lamszus et al. (2002) Ebato et al. (2003) Kordek et al. (2003) Borges et al. (2004) Tanaka et al., (2005) Tanaka et al., (2005) Tanaka et al., (2005) Stosic-Opincal et al. (2005) Chen et al. (2006) Chen et al. (2006) Celli et al. (2006) Qi et al. (2006) Vajtal et al. (2006) Ishizawa et al. (2006) Epelbaum et al. (2006) Epelbaum et al. (2006) Buccoliero et al., (2006) Dim et al. (2006) Konya et al. (2006)
TABLE 1 - REVIEW OF LITERATURE.
temporal temporal/sx parietal/sx temporal/sx temporal/dx parietal/sx temporal/sx temporal/sx frontal/dx frontal/sx P-O/sx P-O/sx frontal/sx temporal/sx frontal/sx P-O/sx frontal/dx parietal/sx fronto-parietal/sx occipital 3rd ventricle temporal/dx parietal/dx temporal/dx temporal/dx frontal/sx NA temporal/sx parietal/dx temporal/sx frontal/dx basal ganglia/sx frontal/dx frontal/dx
Localization GTR GTR GTR PR GTR GTR GTR GTR GTR GTR GTR GTR GTR GTR PR GTR NA NA GTR GTR GTR GTR PR GTR GTR GTR NA GTR PR GTR GTR GTR GTR GTR
Type NA yes no yes yes no no yes no no NA no NA NA NA NA NA no NA NA yes no no yes NA NA NA NA NA no no no NA no
NA marked mild marked mild mild mild mild mild mild marked no no mild mild NA mild no no NA mild no no no no no NA no NA marked no mild moderate no
Calcification Perit edema
5/NR solid 45/NR cystic/mural nodule 43/NR cystic/solid 12/NR cystic 18/NR cystic/solid 6/NR cystic/mural nodule 12/NR solid 12/NR cystic/solid 6/NR cystic/mural nodule NA cystic/mural nodule 84/NR cystic/mural nodule 3/NR cystic 6/NR cystic/solid 30/NR cystic/solid 20/NR cystic/mural nodule 4/NR cystic/solid 33/NR cystic/mural nodule NA cystic/solid 56/NR cystic NA NA 23/NR cystic/solid 6/NR cystic/solid 36/NR cystic 13/NR cystic/solid 50/NR cystic/mural nodule 30/NR cystic/solid NA NA 0 cystic/solid 6/R cystic/solid 214/NR cystic 24/NR solid 12/NR cystic/solid hemorrhage 2/NR cystic/solid 12/NR cystic/hemorrhage
Surgery Follow-up NA NA NA NA NA NA NA NA NA NA NA NA NA mild mild NA mild no mild NA NA NA NA no NA mild NA mild NA mild NA NA mild NA
Mass effect adjacent intimate adjacent intimate intimate intimate no no intimate intimate adjacent intimate no intimate intimate no adjacent no intimate no adjacent adjacent adjacent intimate adjacent adjacent NA adjacent NA adjacent adjacent intimate intimate adjacent
Ventricular association ring-like mixed mixed ring-like irregular mixed mixed mild mixed mixed mixed ring-like peripheral/solid enhanced mixed mural nodule absence NA solid enhanced ring-like mixed NA solid enhanced rim, septum mixed NA wall, solid part NA mixed solid enhanced ring-like mixed solid enhanced homogeneous NA
MRI features
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B. Carangelo et al.
60/NR 24/NR 12/NR 8/ER NA NA 32/NR 19/NR NA 2/ER NA 14/NR NA NA NA NA NA ER ER NA 10/NR 8/NR NA NA NA 9/DAD 35/NR 34/NR 48/NR 24/ER NA 30/NR 48/NR
NA no no NA NA NA no no yes NA NA no no no no NA NA NA NA no yes NA NA no Yes no no NA no no NA no no
Mass effect
no NA mild NA mild mild mild mild NA mild no NA no no mild mild no no marked marked NA NA no no no no mild no no no no NA NA NA NA mild mild yes no no no no no no NA NA Moderate Mild Moderate no no Mild no no NA NA no Mild no no NA NA Minimal no no no
Calcification Perit edema
Cystic/solid cystic/mural nodule solid NA cystic/hemorrhage cystic/solid cystic+mural nodule cystic+mural nodule cystic+mural nodule solid NA cystic/solid cystic+solid NA cystic/mural nodule cystic/solid NA cystic cystic Solid cystic/solid cystic NA cystic+mural nodule Solid Cystic Cystic cystic/solid cystic Cystic+solid NA Cystic+solid Cystic
Type adjacent no intimate no no intimate adjacent adjacent adjacent no NA adjacent adjacent no NA intimate NA yes yes no intimate no NA Intimate Intimate Intimate Intimate NA Intimate Intimate NA no no
Ventricular association solid enhanced wall, mural nodule mixed ring-like mixed ring-like mixed multiple rings ring enhancing mixed NA mixed mixed ring-like mural nodule mixed NA mixed mixed mixed mixed mural nodule NA mixed mixed ring-like ring-like mixed ring-like mixed NA mixed mixed
MRI features
Legend: GTR: gross total resection; F-P: fronto-parietal; T-O: temporo-occipital; P-T: parieto-temporal; P-O: parieto-occipital; NA: not available; NR: not evidence of recurrence; ER: evidence of recurrence; DAD: death associated with disease progression.
34/M frontal-parietal/dx GTR 4/M frontal dx PR 41/M T-P-O/dx GTR 74/F frontal PR 38/M frontal/dx GTR 12/M trigone/sx GTR 19/F occipital GTR 9/F temporal GTR 26/F frontal GTR 19/F T-O sx PR NA NA NA 23/M fronto-parietal/sx GTR 15/M pre-central gyrus GTR 17/F frontal/sx GTR 47/M fronto-parietal/sx GTR 25/M temporal/dx GTR 4/M pineal/3°-lateral ventricle NA 13/F frontal GTR 7/F parietal GTR 17/F frontal sx GTR 12/F temporal/dx GTR 27/M temporal/sx GTR 7/F 3° ventricle GTR 23/F parietal dx GTR 27/F lateral ventricle sx PR 75/F P-T dx PR 22/M frontal sx GTR 24/M frontal sx GTR 12/M frontal dx GTR 6/M Iuxaventricular GTR 26/F frontal sx NA 14/M parietal dx GTR 4/M F-P dx PR+RT
Surgery Follow-up
Vaquero et al., (2007) Atri et al. (2007) Radotra et al. (2007) Adam et al., (2007) Adam et al., (2007) Gelpi et al. (2007) Pimentel et al. (2008) Pimentel et al. (2008) Williams et al. (2008) Newton et al., (2008) Faria et al. (2008) Guo et al. (2008) Izycka-Swieszewska et al. (2008) Mahajan et al. (2009) Govindan et al. (2009) Govindan et al. (2009) Husain et al. (2009) Javahery et al., (2009) Javahery et al., (2009) Hema et al (2009) Xiao et al. (2010) Xiao et al. (2010) Phi JK et al (2010) Lavnic et al. (2011) Lavnic et al. (2011) Myung et al., (2011) Myung et al., (2011) Myung et al., (2011) Myung et al., (2011) Agarwal et al., (2012) Agarwal et al., (2012) Agarwal et al., (2012) Agarwal et al., (2012)
Localization
Age/sex
Series
TABLE 1 - REVIEW OF LITERATURE. (continued)
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scular operation for aortic coarctation with stenting in 2003. She denied any use of drugs, alcohol and tobacco. Her family history was significant for a pituitary adenoma in her mother. On physical examination, she was found to have deterioration of conscious level, rigor nucalis, right upper arm paresis (4+/5), aphasia (E3, V4, M6). The patient was referred to the hospital for evaluation and imaging studies. The brain CT scan with and without contrast revealed a bulky, iso-iperdense lesion (50x39x52 mm) in her left basal/polar temporal lobe on post-contrast injection, images showed a round-ring enhancement of the cyst wall with disomogeneous components (Figure 1). MRI - after administration of contrast enhancement, the cystic portion showed heterogeneous enhancement while the solid component a more homogeneous one. Angio-CT and angio-MRI were also mandatory because of the high suspect of a vascular abnormality that was excluded after the performance of these neuroradiological studies (Figures 2, 3). The patient underwent a fronto-temporal craniotomy with macroscopically total removal of tumor. Intraope-
ratively it appears in most part an hemorrhagic lesion (90%) with a minimal solid component (10%). All the samples were analyzed and processed. The final histophatological exam that matched with the diagnosis of PGNT documented the presence of “piloid” areas with associated high vascular proliferation and cystic degeneration. It described papillary zones surrounding a vascular core, the vessels showed marked thickening and hyalinization of wall layers, proliferation index (MIB1)
G Chir Vol. 36 - n. 2 - pp. 63-69 March-April 2015
original article
Papillary glioneuronal tumor: case report and review of literature B. CARANGELO1, U. ARRIGUCCI2, A. MARIOTTINI1, L. LAVALLE1, G. MUSCAS1, D. BRANCO1, L. LIPPA1, G. PERI1, M. MUYA1, G. COSTANTINO1, G. TIEZZI1, D. TACCHINI3, A. MATURO4
SUMMARY: Papillary glioneuronal tumor: case report and review of literature. B. CARANGELO, U. ARRIGUCCI, A. MARIOTTINI, L. LAVALLE, G. MUSCAS, D. BRANCO, L. LIPPA, G. PERI, M. MUYA, G. COSTANTINO, G. TIEZZI, D. TACCHINI, A. MATURO Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial sy-
stem, adjacent to the lateral ventricles. In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently. In our report we discuss the clinical, neuroradiological and surgical features. The final result is compared with literature data.
KEY WORDS: Papillary glioneuronal tumor - Mixed glioneuronal tumor - Brain tumor.
Introduction Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial system, adjacent to the lateral ventricles. Well-established examples of glioneuronal tumors include DNET (Dysembryoplastic NeuroEpithelial Tumor), ganglioglioma and desmoplastic infantile ganglioglioma. More recently recognized entities include rosette-forming tumor of fourth ventricle, rosette glioneuronal tumor (RGNT)/ glioneuronal tumor with neuropil-like islands and PGNT (1). In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently.
Department of Neurosurgery, "Le Scotte" University Hospital of Siena, Siena, Italy Department of Neuroradiology and Interventional Neurology, "Le Scotte" University Hospital of Siena, Siena, Italy 3 Department of Pathology, "Le Scotte" University Hospital of Siena, Siena, Italy 4 Department of Surgical Sciences, “Sapienza” University of Rome, Rome, Italy
It was described in approximately 67 cases to date (Table 1). Both CT and MRI studies show PGNTs to be circumscribed supratentorial lesions, extending to the cortex and the white matter, or may be more deeply situated, displacing the ventricular system. Cystic alteration are frequent. Solid components are iso/hypointense in T1 and iso/hyperintense in T2, contrast enhancing in a diffuse, patchy or rimming fashion and may present as mural nodule or plaques within otherwise cystic masses. Calcifications may be seen. Bleeding of lesions are rare but recorded (3), associated with superficial siderosis of neuraxis. Surrounding edema and mass effect are seen in larger lesions. Reported tumor size ranges 1-9 cm (mean 4,5 cm). Hystology showed pseudopapillary pattern consisting of a single layer of astrocytic cells surrounding hyalinized blood cells. Follow-up indicated that this type of tumor had a benign behavior even if recently we found out 5 cases of aggressive PGNT (1, 2, 4, 5, 7, 8).
1
2
Corresponding Author: Biagio Carangelo, e-mail: [email protected] © Copyright 2015, CIC Edizioni Internazionali, Roma
Case report The patient was a 34 yr old woman with complaints of progressive headache started 2 weeks before and more recently confusion, vomiting and dizziness. She had a va63
64 Age/sex 25/F 13/M 30/F 11/M 27/F 36/M 19/F 35/F 52/M 26/F 23/M 18/M 16/M 4/M 75/F 24/F 23/F 14/F 14/F 18/F 28/F 25/M 16/F 16/F 35/M 27/M NA 13/M 67/M 27/M 17/F 15/F 20/F 49/F
Series
Kim et al., (1997) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Komori et al., (1998) Bouvier-Labit et al. (2000) Prayson et al. (2000) Broholm et al. (2002) Barnes et al. (2002) Tsukayama et al. (2002) Lamszus et al. (2002) Ebato et al. (2003) Kordek et al. (2003) Borges et al. (2004) Tanaka et al., (2005) Tanaka et al., (2005) Tanaka et al., (2005) Stosic-Opincal et al. (2005) Chen et al. (2006) Chen et al. (2006) Celli et al. (2006) Qi et al. (2006) Vajtal et al. (2006) Ishizawa et al. (2006) Epelbaum et al. (2006) Epelbaum et al. (2006) Buccoliero et al., (2006) Dim et al. (2006) Konya et al. (2006)
TABLE 1 - REVIEW OF LITERATURE.
temporal temporal/sx parietal/sx temporal/sx temporal/dx parietal/sx temporal/sx temporal/sx frontal/dx frontal/sx P-O/sx P-O/sx frontal/sx temporal/sx frontal/sx P-O/sx frontal/dx parietal/sx fronto-parietal/sx occipital 3rd ventricle temporal/dx parietal/dx temporal/dx temporal/dx frontal/sx NA temporal/sx parietal/dx temporal/sx frontal/dx basal ganglia/sx frontal/dx frontal/dx
Localization GTR GTR GTR PR GTR GTR GTR GTR GTR GTR GTR GTR GTR GTR PR GTR NA NA GTR GTR GTR GTR PR GTR GTR GTR NA GTR PR GTR GTR GTR GTR GTR
Type NA yes no yes yes no no yes no no NA no NA NA NA NA NA no NA NA yes no no yes NA NA NA NA NA no no no NA no
NA marked mild marked mild mild mild mild mild mild marked no no mild mild NA mild no no NA mild no no no no no NA no NA marked no mild moderate no
Calcification Perit edema
5/NR solid 45/NR cystic/mural nodule 43/NR cystic/solid 12/NR cystic 18/NR cystic/solid 6/NR cystic/mural nodule 12/NR solid 12/NR cystic/solid 6/NR cystic/mural nodule NA cystic/mural nodule 84/NR cystic/mural nodule 3/NR cystic 6/NR cystic/solid 30/NR cystic/solid 20/NR cystic/mural nodule 4/NR cystic/solid 33/NR cystic/mural nodule NA cystic/solid 56/NR cystic NA NA 23/NR cystic/solid 6/NR cystic/solid 36/NR cystic 13/NR cystic/solid 50/NR cystic/mural nodule 30/NR cystic/solid NA NA 0 cystic/solid 6/R cystic/solid 214/NR cystic 24/NR solid 12/NR cystic/solid hemorrhage 2/NR cystic/solid 12/NR cystic/hemorrhage
Surgery Follow-up NA NA NA NA NA NA NA NA NA NA NA NA NA mild mild NA mild no mild NA NA NA NA no NA mild NA mild NA mild NA NA mild NA
Mass effect adjacent intimate adjacent intimate intimate intimate no no intimate intimate adjacent intimate no intimate intimate no adjacent no intimate no adjacent adjacent adjacent intimate adjacent adjacent NA adjacent NA adjacent adjacent intimate intimate adjacent
Ventricular association ring-like mixed mixed ring-like irregular mixed mixed mild mixed mixed mixed ring-like peripheral/solid enhanced mixed mural nodule absence NA solid enhanced ring-like mixed NA solid enhanced rim, septum mixed NA wall, solid part NA mixed solid enhanced ring-like mixed solid enhanced homogeneous NA
MRI features
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60/NR 24/NR 12/NR 8/ER NA NA 32/NR 19/NR NA 2/ER NA 14/NR NA NA NA NA NA ER ER NA 10/NR 8/NR NA NA NA 9/DAD 35/NR 34/NR 48/NR 24/ER NA 30/NR 48/NR
NA no no NA NA NA no no yes NA NA no no no no NA NA NA NA no yes NA NA no Yes no no NA no no NA no no
Mass effect
no NA mild NA mild mild mild mild NA mild no NA no no mild mild no no marked marked NA NA no no no no mild no no no no NA NA NA NA mild mild yes no no no no no no NA NA Moderate Mild Moderate no no Mild no no NA NA no Mild no no NA NA Minimal no no no
Calcification Perit edema
Cystic/solid cystic/mural nodule solid NA cystic/hemorrhage cystic/solid cystic+mural nodule cystic+mural nodule cystic+mural nodule solid NA cystic/solid cystic+solid NA cystic/mural nodule cystic/solid NA cystic cystic Solid cystic/solid cystic NA cystic+mural nodule Solid Cystic Cystic cystic/solid cystic Cystic+solid NA Cystic+solid Cystic
Type adjacent no intimate no no intimate adjacent adjacent adjacent no NA adjacent adjacent no NA intimate NA yes yes no intimate no NA Intimate Intimate Intimate Intimate NA Intimate Intimate NA no no
Ventricular association solid enhanced wall, mural nodule mixed ring-like mixed ring-like mixed multiple rings ring enhancing mixed NA mixed mixed ring-like mural nodule mixed NA mixed mixed mixed mixed mural nodule NA mixed mixed ring-like ring-like mixed ring-like mixed NA mixed mixed
MRI features
Legend: GTR: gross total resection; F-P: fronto-parietal; T-O: temporo-occipital; P-T: parieto-temporal; P-O: parieto-occipital; NA: not available; NR: not evidence of recurrence; ER: evidence of recurrence; DAD: death associated with disease progression.
34/M frontal-parietal/dx GTR 4/M frontal dx PR 41/M T-P-O/dx GTR 74/F frontal PR 38/M frontal/dx GTR 12/M trigone/sx GTR 19/F occipital GTR 9/F temporal GTR 26/F frontal GTR 19/F T-O sx PR NA NA NA 23/M fronto-parietal/sx GTR 15/M pre-central gyrus GTR 17/F frontal/sx GTR 47/M fronto-parietal/sx GTR 25/M temporal/dx GTR 4/M pineal/3°-lateral ventricle NA 13/F frontal GTR 7/F parietal GTR 17/F frontal sx GTR 12/F temporal/dx GTR 27/M temporal/sx GTR 7/F 3° ventricle GTR 23/F parietal dx GTR 27/F lateral ventricle sx PR 75/F P-T dx PR 22/M frontal sx GTR 24/M frontal sx GTR 12/M frontal dx GTR 6/M Iuxaventricular GTR 26/F frontal sx NA 14/M parietal dx GTR 4/M F-P dx PR+RT
Surgery Follow-up
Vaquero et al., (2007) Atri et al. (2007) Radotra et al. (2007) Adam et al., (2007) Adam et al., (2007) Gelpi et al. (2007) Pimentel et al. (2008) Pimentel et al. (2008) Williams et al. (2008) Newton et al., (2008) Faria et al. (2008) Guo et al. (2008) Izycka-Swieszewska et al. (2008) Mahajan et al. (2009) Govindan et al. (2009) Govindan et al. (2009) Husain et al. (2009) Javahery et al., (2009) Javahery et al., (2009) Hema et al (2009) Xiao et al. (2010) Xiao et al. (2010) Phi JK et al (2010) Lavnic et al. (2011) Lavnic et al. (2011) Myung et al., (2011) Myung et al., (2011) Myung et al., (2011) Myung et al., (2011) Agarwal et al., (2012) Agarwal et al., (2012) Agarwal et al., (2012) Agarwal et al., (2012)
Localization
Age/sex
Series
TABLE 1 - REVIEW OF LITERATURE. (continued)
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scular operation for aortic coarctation with stenting in 2003. She denied any use of drugs, alcohol and tobacco. Her family history was significant for a pituitary adenoma in her mother. On physical examination, she was found to have deterioration of conscious level, rigor nucalis, right upper arm paresis (4+/5), aphasia (E3, V4, M6). The patient was referred to the hospital for evaluation and imaging studies. The brain CT scan with and without contrast revealed a bulky, iso-iperdense lesion (50x39x52 mm) in her left basal/polar temporal lobe on post-contrast injection, images showed a round-ring enhancement of the cyst wall with disomogeneous components (Figure 1). MRI - after administration of contrast enhancement, the cystic portion showed heterogeneous enhancement while the solid component a more homogeneous one. Angio-CT and angio-MRI were also mandatory because of the high suspect of a vascular abnormality that was excluded after the performance of these neuroradiological studies (Figures 2, 3). The patient underwent a fronto-temporal craniotomy with macroscopically total removal of tumor. Intraope-
ratively it appears in most part an hemorrhagic lesion (90%) with a minimal solid component (10%). All the samples were analyzed and processed. The final histophatological exam that matched with the diagnosis of PGNT documented the presence of “piloid” areas with associated high vascular proliferation and cystic degeneration. It described papillary zones surrounding a vascular core, the vessels showed marked thickening and hyalinization of wall layers, proliferation index (MIB1)
