Papillary glioneuronal tumor with a high proliferative component and minigemistocytes in a child

Momota, Hiroyuki; Fujii, Masazumi; Tatematsu, Akiko; Shimoyama, Yoshie; Tsujiuchi, Takashi; Ohno, Masasuke; Natsume, Atsushi; Wakabayashi, Toshihiko

doi:10.1111/neup.12123

Cited by 8 publications

(3 citation statements)

References 46 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Only 150 cases of PGNTs are reported up to now in the literature [36,31,17,11,41,15,12,2]. Amongst these, only 41 cases are reported in pediatric population which demonstrates rarity of this pathology in this age group [11,43,44,32,35,29,26,8,1,42,30,33,28,34,20,18,19,14,16,3,39,13,10,38,7,25,9,4,23]. To our 3 knowledge, there is no report of PGNT in a toddler up to now.…”

Section: Background and Importancementioning

confidence: 94%

Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: an odd presentation of a rare pathology

Tavallaii

Keykhosravi

Rezaee

2020

British Journal of Neurosurgery

View full text Add to dashboard Cite

Background and Importance: Papillary glioneuronal tumor is a recently known entity in central nervous system tumors. These benign WHO grade I tumors are mostly seen in young adults. Pediatric PGNT is rare and there is no report of these tumors in toddlers. Headache, nausea/vomiting and seizure are most common clinical symptoms. Acute presentation with focal neurological deficits or loss of consciousness are not amongst the expected presentations. These tumors are typically cystic with enhancing mural nodule. Although case with chronic intermittent microhemorrhages are reported in the literature but overt intra-tumoral hemorrhage is an odd radiological presentation with just one reported case in the literature. Clinical presentation: We present an extremely rare case of PGNT presenting with sudden onset hemiparesis and impaired consciousness due to acute intra-tumoral hemorrhage in a toddler which was surgically treated with favorable outcome. Conclusion: PGNTs can also be seen in very young children even in toddlers. Also, it should be kept in mind that these tumors have potential for overt intra-tumoral hemorrhage and acute presentation with focal neurological deficits mimicking more common pathologies which should be considered to plan optimal patient management.

show abstract

Section: Background and Importancementioning

confidence: 94%

Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: an odd presentation of a rare pathology

Tavallaii

Keykhosravi

Rezaee

2020

British Journal of Neurosurgery

View full text Add to dashboard Cite

show abstract

“…Only 150 cases of PGNTs are reported up to now in the literature [36,31,17,11,41,15,12,2]. Amongst these, only 41 cases are reported in pediatric population which demonstrates rarity of this pathology in this age group [11,43,44,32,35,29,26,8,1,42,30,33,28,34,20,18,19,14,16,3,39,13,10,38,7,25,9,4,23]. To our knowledge, there is no report of PGNT in a toddler up to now.…”

Section: Background and Importancementioning

confidence: 98%

Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: An odd presentation of a rare pathology

Tavallaii

Keykhosravi

Rezaee

2020

Preprint

View full text Add to dashboard Cite

Background and Importance: Papillary glioneuronal tumor is a recently known entity in central nervous system tumors. These benign WHO grade I tumors are mostly seen in young adults. Pediatric PGNT is rare and there is no report of these tumors in toddlers. Headache, nausea/vomiting and seizure are most common clinical symptoms. Acute presentation with focal neurological deficits or loss of consciousness are not amongst the expected presentations. These tumors are typically cystic with enhancing mural nodule. Although case with chronic intermittent microhemorrhages are reported in the literature but overt intra-tumoral hemorrhage is an odd radiological presentation with just one reported case in the literature.Clinical presentation: We present an extremely rare case of PGNT presenting with sudden onset hemiparesis and impaired consciousness due to acute intra-tumoral hemorrhage in a toddler which was surgically treated with favorable outcome.Conclusion: PGNTs can also be seen in very young children even in toddlers. Also, it should be kept in mind that these tumors have potential for overt intra-tumoral hemorrhage and acute presentation with focal neurological deficits mimicking more common pathologies which should be considered to plan optimal patient management.

show abstract

“…We previously reported the rare case of a papillary glioneuronal tumor (PGNT) with high mitotic index and minigemistocytes in a 12‐year‐old boy in Neuropathology . Although PGNT is considered a benign brain tumor, several cases with pathologically and clinically aggressive features have been reported.…”

mentioning

confidence: 99%

Recurrent papillary glioneuronal tumor presenting as a ganglioglioma with the BRAF V600E mutation

Momota

Shimoyama

2015

Neuropathology

Self Cite

View full text Add to dashboard Cite

Recurrent papillary glioneuronal tumor presenting as a ganglioglioma with the BRAF V600E mutationWe previously reported the rare case of a papillary glioneuronal tumor (PGNT) with high mitotic index and minigemistocytes in a 12-year-old boy in Neuropathology. 1Although PGNT is considered a benign brain tumor, several cases with pathologically and clinically aggressive features have been reported. Given the histologic features of malignancy in our reported case, that is, necrosis, microvascular proliferation, minigemistocytes and high Ki-67 proliferative index (14%), we continued careful follow-up by MRI after gross total resection of the tumor. Thus, a small recurrent tumor was observed by MRI at the site of the tumor cavity 26 months after the first operation. The tumor was well demarcated and homogeneously enhanced by gadolinium, and grew gradually over 5 months (Fig. 1A, B). Although it was small and seen as benign, we performed a second surgical resection of the tumor.The histological examination demonstrated proliferation of mature and dysmorphic ganglion cells and microcalcification. Pseudopapillary structures and minigemistocytes that were observed in the first operative specimen were absent ( Fig. 2A, B). Immunohistochemically, the tumor cells were mostly negative for GFAP (6F2, Dako, Glostrup, Denmark; 1:400) but positive for synaptophysin (SY38, Dako; 1:500) (Fig. 2C, D). The cells were positive for neurospecific enolase (BBS/NC/VI-H14, Dako; 1:100) and S-100 (polyclonal, Dako; 1:1000), partially positive for neurofilament (2F11, Dako; 1:1000) and oligodendrocyte transcription factor 2 (Olig2; polyclonal, IBL, Gunma, Japan; 1:300), and negative for CD34 (QBEnd10, Dako; 1:600). The Ki-67 (MIB-1, Dako; 1:300) proliferative index was 2% in the most proliferative area. Although the expression of GFAP was minimal in the tumor, there was a small area with GFAP-positive tumor cells. Therefore, a pathological diagnosis of ganglioglioma was made.Because the pathology was determined to be ganglioglioma, we further investigated for the presence of the BRAF V600E mutation by immunohistochemistry in the first and second surgical specimens, using an anti-BRAF V600E antibody (VE1, Spring Bioscience, Pleasanton, CA, USA; 1:1000). Interestingly, mutated-BRAF protein was observed both in the first and second surgical specimens (Fig. 3A-C). In the first surgical specimen, both the pseudopapillary and desmoplastic areas showed mutated-BRAF expression only in the tumor cells and not in vascular endothelial cells and infiltratelymphocytes. Neuronal and ganglionic tumor cells showed a more intense staining of mutated-BRAF than glial tumor cells. In the second surgical specimen, all the tumor cells of neuronal or ganglionic morphology demonstrated strong mutated-BRAF expression. Based on these findings, we speculated that a neuronal component of the PGNT persisted after the first operation and regrew as a secondary ganglioglioma.The pathogenesis of PGNT is unknown. Myung et al. reported that two patients with PGNT were tested ...

show abstract

Papillary glioneuronal tumor with a high proliferative component and minigemistocytes in a child

Cited by 8 publications

References 46 publications

Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: an odd presentation of a rare pathology

Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: an odd presentation of a rare pathology

Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: An odd presentation of a rare pathology

Recurrent papillary glioneuronal tumor presenting as a ganglioglioma with the BRAF V600E mutation

Contact Info

Product

Resources

About