The great neurosurgical masquerader: 3 cases of desmoplastic infantile ganglioglioma

Alan R. Cohen Division of Pediatric Neurosurgery, Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland

Search for other papers by Alan R. Cohen in
jns
Google Scholar
PubMed
Close
 MD
Full access

Desmoplastic infantile ganglioglioma (DIG) is a rare, distinctive, supratentorial neoplasm with a generally favorable prognosis. Clinical, radiographic, and pathologic features can sometimes mimic those of a malignant tumor and other serious intracranial disorders. The author describes his experience with 3 cases of DIG, each of which initially masqueraded as another neurological disease with a very different prognosis. Case 1 was an infant boy referred for evaluation of a hemorrhagic infarction at birth. Case 2 was an infant girl referred for evaluation of a holohemispheric malignant neoplasm. Case 3 was an infant girl referred for evaluation of an intracranial mass believed to be a subdural empyema or possible sarcoma. In each case the lesion was resected and found to be a WHO grade I DIG. Each child has had a benign postoperative course. DIG can be mistaken for other serious neurological conditions including malignant neoplasm, cerebral infarction, and infection. It is prudent to consider this rare, low-grade resectable tumor in the differential diagnosis of atypical intracranial masses of childhood, as the impact on prognosis can be profound. The author discusses management strategies for DIG, including a role for molecular sequencing.

ABBREVIATIONS

CBC = complete blood count; DIA = desmoplastic infantile astrocytoma; DIG = desmoplastic infantile ganglioglioma; GFAP = glial fibrillary acidic protein; NAA = N-acetylaspartate.

Desmoplastic infantile ganglioglioma (DIG) is a rare, distinctive, supratentorial neoplasm with a generally favorable prognosis. Clinical, radiographic, and pathologic features can sometimes mimic those of a malignant tumor and other serious intracranial disorders. The author describes his experience with 3 cases of DIG, each of which initially masqueraded as another neurological disease with a very different prognosis. Case 1 was an infant boy referred for evaluation of a hemorrhagic infarction at birth. Case 2 was an infant girl referred for evaluation of a holohemispheric malignant neoplasm. Case 3 was an infant girl referred for evaluation of an intracranial mass believed to be a subdural empyema or possible sarcoma. In each case the lesion was resected and found to be a WHO grade I DIG. Each child has had a benign postoperative course. DIG can be mistaken for other serious neurological conditions including malignant neoplasm, cerebral infarction, and infection. It is prudent to consider this rare, low-grade resectable tumor in the differential diagnosis of atypical intracranial masses of childhood, as the impact on prognosis can be profound. The author discusses management strategies for DIG, including a role for molecular sequencing.

Things are seldom what they seem; Skim milk masquerades as cream.

— Sir William Schwenck Gilbert and Sir Arthur Seymour Sullivan

H.M.S. Pinafore, May 25, 1878

Desmoplastic infantile ganglioglioma (DIG) is an uncommon, superficial, supratentorial neoplasm that typically carries a favorable prognosis. The clinical presentation is usually seizures or progressive macrocephaly.9 On MRI, DIGs are often enhancing, cystic, and solid masses that frequently involve more than one lobe of the brain. The clinical, radiographic, and pathologic features of DIG can sometimes mimic those of more aggressive neoplasms or other serious intracranial diseases. The author describes his experience with 3 cases of DIG, each of which initially masqueraded as another neurological disorder with a vastly different prognosis. Pitfalls in the diagnosis of this tumor are discussed.

Case Reports

Case 1

History and Examination

This 8-month-old infant boy was referred for evaluation of an abnormal MR image. He was born via normal spontaneous vaginal delivery to a healthy 26-year-old primigravida at term following an uncomplicated pregnancy. His birth weight was 8 pounds 12 ounces. He developed focal seizures on the first day of life characterized by clonic activity on the right upper and lower extremities. He was awake and alert with a mild right hemiparesis. CT and MRI performed at an outside hospital on days 3 and 5 of life, respectively, showed a left posterior temporal hemorrhagic lesion (Fig. 1A and B). MR angiography showed no vascular abnormality. Laboratory analysis showed homozygosity for the serum methylene tetrahydrofolate reductase mutation. The rest of the screening laboratory test results were normal, including complete blood count (CBC), electrolytes, prothrombin time, partial thromboplastin time, serum homocysteine, protein C, protein S, antithrombin III, factor V Leiden, antiphospholipid antibody, lupus anticoagulant, prothrombin 20210A, and plasminogen activator inhibitor 1.

FIG. 1.
FIG. 1.

Case 1. Upper Row: Left posterior temporal hemorrhagic lesion interpreted at the referring hospital as a venous infarction at initial presentation. Noncontrast head CT on day 3 of life (A) and T2-weighted axial MRI on day 5 of life (B). Middle Row: Preoperative contrast-enhanced axial (C) and sagittal (D) T1-weighted MR images at age 8 months showing a large, heterogeneously enhancing, left posterior temporal mass. Lower Row: Postoperative contrast-enhanced axial (E) and sagittal (F) T1-weighted axial MR images 1 day after surgery, confirming gross-total tumor resection.

The clinical impression at the outside hospital was that he had sustained a hemorrhagic cerebral infarction. His seizures were controlled with phenobarbital and his motor examination improved except for mild weakness of the right lower extremity. Repeat MRI 5 months later was interpreted as consistent with an evolving hemorrhagic infarction.

Operation and Postoperative Course

The child was referred to us for a second opinion at 8 months of age at the request of his mother. He was alert with mild right lower-extremity weakness. MRI showed a left temporal heterogeneously enhancing mass (Fig. 1C and D). MR spectroscopy showed an elevated choline peak and a depressed N-acetylaspartate (NAA) level. There was no restriction of diffusion. Our impression was that the lesion represented a neoplasm. We removed it through a left temporal craniotomy. At surgery the mass was firm, kidney-shaped, moderately vascular, partially extraaxial, relatively well-circumscribed, and adherent to the meninges (Fig. 2). A gross-total resection was performed. Pathology showed a whorled pattern with ganglion cells in a background of spindle-shaped astrocytes. There were areas of focal necrosis and vascular proliferation. Immunohistochemistry supported the finding of a mixed glial neuronal neoplasm. The pathologic diagnosis was WHO grade I DIG (Fig. 3). MRI confirmed the tumor resection (Fig. 1E and F). The patient experienced an uneventful postoperative course. His seizures have remained well-controlled. Fifteen years postoperatively he is functioning well as an honor-roll student in the 9th grade with an interest in photography.

FIG. 2.
FIG. 2.

Case 1. A: Operative exposure of a large, firm, left temporal tumor that was adherent to the meninges. Anterior is to the right. B: Resected tumor. Figure is available in color online only.

FIG. 3.
FIG. 3.

Case 1. Photomicrograph showing DIG, WHO grade I, with ganglion cells in a background of spindle-shaped astrocytes. H & E, original magnification ×200. Figure is available in color online only.

Case 2

History and Examination

This 2-month-old infant girl was referred for evaluation of an abnormal MRI. She was born to a healthy G2P1 (2 pregnancies, 1 birth) mother at 38 weeks gestation via Caesarean section, with a birth weight of 8 pounds 11 ounces. At 18 weeks gestation she was diagnosed with trisomy 21 by fetal ultrasound and a maternal cell-free DNA blood test. At 30 weeks gestation fetal ultrasound showed a large left hemisphere brain tumor. At 32 weeks gestation fetal MRI confirmed the ultrasound findings and suggested a hemispheric malignant tumor (Fig. 4A). The family was counseled that the child would not survive and were advised to make funeral arrangements.

FIG. 4.
FIG. 4.

Case 2. A: Fetal FIESTA sagittal MRI performed at 32 weeks gestation demonstrating a large, heterogeneous, left hemisphere mass. B–D: Preoperative postcontrast fast spoiled gradient echo axial, sagittal, and coronal MR images at birth, demonstrating a large, enhancing, solid and cystic left hemisphere tumor.

Shortly after birth the child was sent home with hospice care. She was lethargic with poor oral intake and required nasogastric tube feedings for several weeks. Repeat MRI showed a very large enhancing cystic and solid tumor of the left hemisphere (Fig. 4B–D). By 2 months she had no further deterioration and began taking oral feedings without difficulty. She came to us for a second opinion. On physical examination she was alert with an occipitofrontal circumference of 39 cm. The anterior fontanelle was open and flat. She had upward slanting of the palpebral fissures, low-set ears, a short neck, and a small mouth. Her eyes tracked and moved conjugately and her limbs moved well with mild hypotonia. Repeat MRI showed a large enhancing cystic and solid left hemisphere mass with some reduction in size of the solid component and an increase in size of the cysts.

Operation and Postoperative Course

Because her clinical examination looked better than her neuroimaging, we elected to explore the mass through a left parietal craniotomy. The lesion was circumscribed, tan in color, moderately firm, and adherent to the meninges with several cysts (Fig. 5A). A gross-total resection was performed. MRI 1 day after surgery showed postoperative changes (Fig. 5B–E). Pathology showed a glioneuronal tumor (WHO grade I) with astrocytic proliferation, intratumoral neurons, and no mitoses or vascular proliferation. The patient had an uneventful postoperative course. Three-and-a-half years after surgery she is a happy youngster in preschool, speaking several words, sitting independently, standing with assistance, and riding her tricycle. She has some weakness and increased tone in her right upper extremity and some difficulty with balance, for which she receives physical and occupational therapy. Follow-up MRI has shown no evidence of residual tumor.

FIG. 5.
FIG. 5.

Case 2. A: Operative exposure of a large, left parietal, solid and cystic tumor adherent to the meninges. Anterior is to the right. B–E: Axial T2-weighted (B and C), T1-weighted contrast-enhanced (D), and sagittal T1-weighted contrast-enhanced (E) MR images obtained 1 day after surgery, confirming tumor resection. Figure is available in color online only.

Case 3

History and Examination

This 9-month-old previously healthy infant girl was referred to us for evaluation following a 30-minute focal seizure. This consisted of clonic movements of her left upper and lower extremities that developed after she awakened from an uncharacteristically long nap in the afternoon. She was born at term to a healthy G3P2 (3 pregnancies, 2 births) mother via vaginal delivery following an uncomplicated pregnancy, except for placental abruption. Her developmental milestones were normal. By parental choice she had not been immunized. Her neurological examination after the seizure was normal, with an occipitofrontal circumference of 44 cm, a soft anterior fontanelle, equally reactive pupils, and no focal deficits. Laboratory investigation showed a normal CBC. Cranial CT without contrast showed marked edema involving the right frontal and parietal lobes. Levetiracetam administration was begun.

On the 2nd hospital day the patient was intubated in preparation for cranial MRI. She spiked a high fever to 40.5°C. Her skin was cool and clammy and she was agitated and thrashing. Repeat CBC was abnormal with a white blood cell count of 3.2, hemoglobin 7.8 g/dl, and platelet count of 125. Chest radiography showed no infiltrates. There was concern for sepsis, and vancomycin and ceftriaxone were administered. Preoperative T2-weighted axial MRI showed marked right hemispheric edema (Fig. 6A–C). Preoperative postcontrast T1-weighted images demonstrated an avidly enhancing right frontoparietal parasagittal mass (Fig. 6D–F). The lesion did not restrict diffusion. The differential diagnosis included subdural empyema, sarcoma, or other malignant brain tumor.

FIG. 6.
FIG. 6.

Case 3. Pre- and postoperative MR images. A–C: Preoperative axial T2-weighted images showing marked right hemispheric edema. D–F: Preoperative contrast-enhanced axial, sagittal, and coronal images showing a solid, enhancing, right parietal parasagittal tumor with edema. G–I: Postoperative contrast-enhanced axial, sagittal, and coronal images obtained 4 months after surgery showing no residual enhancing tumor.

Operation and Postoperative Course

She underwent emergency right frontoparietal parasagittal craniotomy with administration of perioperative dexamethasone. Her brain was extremely swollen. There was a large, whitish, firm, woody, partially extraaxial tumor adherent to the dura. A subtotal resection was performed as the brain was under significant pressure despite measures taken to control the hemispheric edema. Postoperative MRI showed substantial improvement in the mass effect and edema. CT of the chest, abdomen, and pelvis performed on the day after surgery demonstrated consolidation in both lungs, and antibiotics were continued for pneumonia. She improved gradually over the next week clinically and neurologically except for mild weakness of her left upper extremity.

The pathologic diagnosis was WHO grade I DIG (Fig. 7). The tumor was moderately cellular with a conspicuous storiform pattern and rare mitoses. Immunohistochemical staining showed strong glial fibrillary acidic protein (GFAP) expression as well as OLIG2 positivity in a subset of cells. Synaptophysin labeled rare dysplastic neurons and ganglion cells. There was a low Ki-67 proliferation index of 2%. Molecular next-generation sequencing showed a novel BRAF V600delinsDL mutation that has not been previously described.

FIG. 7.
FIG. 7.

Case 3. Histopathological images showing a WHO grade I DIG. A: Image showing moderate cellularity with elongated astrocytes in a conspicuous storiform pattern. H & E, original magnification ×100. B: Higher magnification view. H & E, original magnification ×200. C: GFAP immunostaining showing abundance of elongated astrocytes. Original magnification ×200. D: Synaptophysin immunostaining labeling rare dysplastic ganglion cells. Original magnification ×200. Figure is available in color online only.

Ten days after the first operation we reoperated and performed a near-total resection of the tumor, leaving small segments adherent to the lateral wall of the superior sagittal sinus. No cysts were identified at either operation. The patient made an excellent recovery and ultimately regained full strength in all extremities. She had no further seizures and was independently ambulatory 3 months following surgery at age 1 year. Follow-up MRI 5 months after surgery showed postoperative changes with resolution of the edema and enhancement (Fig. 6G–I). She remains well and is clinically stable 8 months following surgery.

Discussion

Background

DIG is a rare, distinctive, supratentorial neuroepithelial tumor with a generally favorable prognosis. DIG was first described by VandenBerg and colleagues in 1987, who reported 11 patients, all under the age of 18 months.42 The tumors were superficial in location, large in size, and firm in consistency. Pathologically they were characterized by divergent astrocytic and ganglionic differentiation and intense desmoplasia. The term desmoplasia comes from desmos (Gr. knot) and plasis (Gr. formation) to describe the powerful fibrous leptomeningeal reaction of this tumor. It is classified as grade I by the WHO.

DIG bears a striking similarity to the superficial cerebral astrocytoma described by Taratuto et al. in 1984. They characterized 6 cases of solid and cystic meningocerebral astrocytoma attached to the dura, all in infants under the age of 1 year.39 This tumor differs from DIG only by its absence of neuronal divergence. Currently the superficial cerebral astrocytoma is called desmoplastic infantile astrocytoma (DIA), and the WHO classifies DIG and DIA together as “mixed neuronal-glial” tumors, WHO grade I.23

DIG is a rare tumor with an incidence of 0.5%–1% of all CNS tumors; only approximately 60 cases have been reported as of 2017.29 The incidence in infants in the first year of life is higher, although it is still an uncommon lesion.7 DIG usually presents with progressive macrocephaly or seizures, with a median duration of symptoms of 3 months.5,9–12,17,18,22 The tumor is most commonly located in the parietal, frontal, or temporal lobe with a predilection for the frontoparietal region.41 Multilobar occurrence is common, with up to 60% of reported cases involving more than one hemisphere.34 The solid portion of DIG abuts the meninges and tends to be firm but is rarely calcified.24

A similar tumor in older patients was first described by Kuchelmeister et al., who named it noninfantile desmoplastic ganglioglioma, and reported 2 cases in 1993.20 In a retrospective review of “DIGs” by Hummel and colleagues, 23% of the cases were considered noninfantile, occurring in patients older than 2 years.17 Other investigators have reported similar findings.15,30,33

Radiology

Radiologically, DIG tends to be a large, cystic, and solid supratentorial neoplasm.28 On MRI the cystic component appears hypointense on T1-weighted images and hyperintense on T2. The solid cortical component is isointense on T1- and T2-weighted images and abuts the meninges with avid, homogeneous enhancement.10,25,28,35 It is rare to see the tumor adjacent to the cerebral ventricles. DIG-related metabolic changes have been reported on MR spectroscopy, including reduction of NAA and elevation of choline with no alteration of myoinositol in relation to the creatine peak.4

While the tumor is usually both cystic and solid, it can sometimes appear as a solitary solid mass. Tseng et al. described chronologic changes in a 3-month-old infant girl with seizures and a right temporal DIG that on presentation appeared as a solid enhancing mass on MRI.40 Due to the family’s initial reluctance to pursue surgery (the child had no other symptoms) she was followed with surveillance MR images that showed the evolution of a typical cystic component over a period of months, before the tumor was ultimately removed.

On MRI, DIG can mimic multiple low- and high-grade neoplasms; these include pleomorphic xanthoastrocytoma, supratentorial ependymoma, ganglioglioma, and primitive neuroectodermal tumor.28,33 Because of its large size DIG has been mistaken for a number of malignant neoplasms.

Pathologic Findings

Pathologic features of DIG include a neuronal component that stains with synaptophysin and a glial component that stains with GFAP. The tumor can be moderately cellular but usually displays low MIB-1 and Ki-67 labeling activity, supporting its benign nature.31 There is a background of desmoplasia that can be brought out on reticulin stain and a variable degree of eosinophilic granular bodies.1,17

Just as DIG can mimic more malignant tumors on neuroimaging studies it can also suggest a more ominous process on microscopy. Some tumors may contain clusters of hypercellularity with primitive-looking cells, mitotic figures, and foci of necrosis that do not necessarily portend a worse prognosis.3,32

Molecular Profiling

Recently, DIG and DIA tumors have been evaluated with genome-wide DNA analysis. Although the BRAF V600E mutation has been identified frequently in other low-grade gliomas, previous studies found this mutation to occur infrequently in DIG. Gessi and colleagues found no BRAF V600E mutations in 10 cases of DIG and only 1 mutation in 4 cases of DIA.13 Of note, the one tumor that harbored the mutation was located in the fourth ventricle, an atypical site for DIG or DIA. Greer et al. examined archival material for the BRAF mutation from 6 cases of DIG/DIA and found it to be absent in 5. A single DIG was found to harbor the V600D mutation.14 Koelsche and colleagues examined 18 cases of DIG/DIA and found the V600E mutation in 2 of 16 cases of DIG and 1 of 2 cases of DIA.19 Chatterjee et al. analyzed 8 cases of DIG/DIA and found the BRAF V600E mutation in 4 of them;8 all 4 with the mutation were noninfantile tumors in patients aged 10–14 years. Of note, though, the mutation was not identified in any of the infantile cases of DIG/DIA.

In contrast, a recent study by Wang et al. found the BRAF V600 mutation to occur much more frequently in DIG and DIA tumors.43 In a multiinstitutional collaboration they identified somatic BRAF gene mutations in 7 (44%) of 16 confirmed cases of DIG or DIA. Four of these had the BRAF V600E mutation and 3 had the BRAF V600D mutation.

Blessing and colleagues recently identified a novel BRAF mutation in a 3-month-old infant girl with a large cystic and solid right-temporal DIG.6 Comprehensive molecular tumor profiling found a unique BRAF deletion-insertion at codons 600–604. The child developed progressive leptomeningeal brainstem dissemination 3 months after near-total tumor resection that was refractory to vincristine and carboplatin chemotherapy. Initiation of treatment with the BRAF-MEK inhibitors, dabrafenib and trametinib, led to a reduction in both residual tumor burden and leptomeningeal disease. This suggests a possible oncological role for molecular profiling and targeted therapy for select cases of DIG.

In our series, we performed a next-generation sequencing analysis on the tumor in case 3. We found a unique BRAF mutation that changed codon V600 to aspartic acid (D) plus a new amino acid leucine (L) at codon V601. This mutation has not been previously described. By nomenclature, the deletion/insertion is V600delinsDL (personal communication, Dr. Liam Chen, 2018). The prognostic significance of this finding in our case, and of the mutations found in other cases of DIG, is not yet clear but as Blessing et al.6 showed, it may play an important role in developing targeted molecular therapy for incompletely resected tumors.

Clinical Heterogeneity

Although DIG is generally associated with a favorable prognosis, the tumor can sometimes demonstrate a malignant course. In a retrospective review, Hummel et al. emphasized the clinical heterogeneity of this tumor, noting that 40% of DIG cases required additional treatment including chemotherapy, radiation, or further surgery, and 15% developed leptomeningeal dissemination or died.17 Taranath and colleagues described 2 infants with DIG who had cerebral and spinal metastases and called into question the benign nature of this tumor.38 Other investigators have reported DIG and DIA associated with leptomeningeal dissemination and a malignant course.6,9,16,26,27,34 This emphasizes the need to follow these patients closely, even after gross-total resection. It also emphasizes the importance of making an accurate pathologic diagnosis, because the clusters of primitive-appearing cells in some benign DIGs can mimic other malignant tumors, while clusters of primitive cells in other cases may actually represent true malignant tumors.

DIG has also been reported to undergo malignant transformation. Loh and colleagues reported the unusual case of a 2-year-old girl who underwent gross-total resection of a DIG with no adjuvant therapy. She subsequently developed a glioblastoma at the same operative site at age 5.21 The patient was alive without recurrence 14 years later.

It is noteworthy that DIG can also undergo postoperative tumor regression. Takeshima et al. reported involution of the tumor over a period of months in 2 patients, a 9-month-old boy and a 2-month-old girl following subtotal tumor resection, and suggested this was possibly attributable to apoptosis.36 Tamburrini and colleagues operated on a 9-month-old infant with a cystic and solid temporoparietooccipital DIG and had to leave a nodule that was adherent to the deep cerebral veins. Nine months later MRI demonstrated complete disappearance of the nodule and the child remained tumor-free 11 years later.37 We also observed involution of a DIG remnant in our case 3, in which a segment of tumor that we left adjacent to the superior sagittal sinus disappeared on follow-up MRI after several months.

The Great Masquerader

DIG can be mistaken for a malignant neoplasm clinically, radiographically, and even pathologically. It can mimic anaplastic astrocytoma, glioblastoma, ependymoma, sarcoma, primitive neuroectodermal tumor, and atypical teratoid-rhabdoid tumor. Tamburrini et al. reported a 2-month-old infant girl who underwent staged subtotal removal of a large right frontotemporoparietal tumor with the pathologic diagnosis of anaplastic astrocytoma. After 6 cycles of chemotherapy the child underwent staged reoperation and total resection of the tumor, although this time the pathology was DIG. Because of the discrepancy in the pathologic diagnoses, the original histology was reviewed and found to actually be DIG. No recurrence was noted at follow-up 2 years later.37 This report underscores a difficulty in making the pathologic diagnosis of DIG, as regions of primitive-appearing cells and mitotic figures can mimic a more aggressive neoplasm. Another example highlighting the pitfalls in pathologic diagnosis was reported by Alghamdi and colleagues.2 A 5-day-old infant boy underwent resection of a large left hemisphere cystic and solid tumor that turned out to be a DIG. The tumor stained for desmin and mimicked a pleomorphic rhabdomyosarcoma on morphology and immunohistochemistry.2

Management Strategy

The author suggests a management strategy for DIG that focuses on maximal safe surgical resection, either gross-total or near-total. Histological diagnosis should be made by an experienced neuropathologist. Molecular and genetic characterization should be performed with particular attention to possible mutations at codon 600 of the BRAF gene. Patients should be closely followed clinically along with surveillance MRI, even after gross-total resection, as recurrences have been reported. Some residual nodules of tumor may involute over time. Symptomatic recurrences should be treated with repeat resection when possible. As we learn more about the prognostic significance of specific gene mutations, personalized medicine with targeted molecular therapies may play a role.

Conclusions

DIG is a rare tumor of childhood that can be a great masquerader. It can be mistaken for other neurological disorders including malignant neoplasms, stroke, and infection. In our series, case 1 was initially diagnosed as a venous infarction, case 2 was originally believed to be a hemispheric malignant tumor, and case 3 was considered to be a subdural empyema or an intracranial sarcoma. In each case, the lesion was actually a low-grade and resectable tumor with a favorable prognosis. It is essential to consider DIG in the differential diagnosis of atypical intracranial masses of infancy, as the impact on prognosis can be profound.

Acknowledgments

The author is grateful to the neuropathologists who diagnosed the cases in this series: Mark L. Cohen, MD, Ali G. Saad, MD, Fausto J. Rodriguez, MD, and Liam Chen, MD, PhD.

Disclosures

The author reports no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

References

  • 1

    Alexiou GA, Stefanaki K, Sfakianos G, Prodromou N: Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature. Pediatr Neurosurg 44:422425, 2008

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Alghamdi S, Castellano-Sanchez A, Brathwaite C, Shimizu T, Khatib Z, Bhatia S: Strong desmin expression in a congenital desmoplastic infantile ganglioglioma mimicking pleomorphic rhadomyosarcoma: a case report including ultrastructural and cytogenetic evaluation and review of the literature. Childs Nerv Syst 28:21572162, 2012

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Bächli H, Avoledo P, Gratzl O, Tolnay M: Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. Childs Nerv Syst 19:359366, 2003

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Balaji R, Ramachandran K: Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. Childs Nerv Syst 25:497501, 2009

  • 5

    Bianchi F, Tamburrini G, Massimi L, Caldarelli M: Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review. Childs Nerv Syst 32:18331838, 2016

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Blessing MM, Blackburn PR, Balcom JR, Krishnan C, Harrod VL, Zimmermann MT, et al.: Novel BRAF alteration in desmoplastic infantile ganglioglioma with response to targeted therapy. Acta Neuropathol Commun 6:118, 2018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Cerdá-Nicolás M, Lopez-Gines C, Gil-Benso R, Donat J, Fernandez-Delgado R, Pellin A, et al.: Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features. Histopathology 48:617621, 2006

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Chatterjee D, Garg C, Singla N, Radotra BD: Desmoplastic non-infantile astrocytoma/ganglioglioma: rare low-grade tumor with frequent BRAF V600E mutation. Hum Pathol 80:186191, 2018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, et al.: Desmoplastic infantile ganglioglioma: a potentially malignant tumor? Am J Surg Pathol 26:15151522, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Duffner PK, Burger PC, Cohen ME, Sanford RA, Krischer JP, Elterman R, et al.: Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurgery 34:583589, 1994

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Gelabert-Gonzalez M, Serramito-García R, Arcos-Algaba A: Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. Neurosurg Rev 34:151158, 2010

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Geramizadeh B, Kamgarpour A, Moradi A: Desmoplastic infantile ganglioglioma: Report of a case and review of the literature. J Pediatr Neurosci 5:4244, 2010

  • 13

    Gessi M, Zur Mühlen A, Hammes J, Waha A, Denkhaus D, Pietsch T: Genome-wide DNA copy number analysis of desmoplastic infantile astrocytomas and desmoplastic infantile gangliogliomas. J Neuropathol Exp Neurol 72:807815, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Greer A, Foreman NK, Donson A, Davies KD, Kleinschmidt-DeMasters BK: Desmoplastic infantile astrocytoma/ganglioglioma with rare BRAF V600D mutation. Pediatr Blood Cancer 64:e26350, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Gundamaneni SK, Ganesh CV, Mahadevan A, Madhugiri VS, Sasidharan GM, Kumar VR: Non-infantile desmoplastic cerebellar ganglioglioma in a patient with multiple congenital anomalies: a rare association. Pediatr Neurosurg 49:105109, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16

    Hoving EW, Kros JM, Groninger E, den Dunnen WF: Desmoplastic infantile ganglioglioma with a malignant course. J Neurosurg Pediatr 1:9598, 2008

  • 17

    Hummel TR, Miles L, Mangano FT, Jones BV, Geller JI: Clinical heterogeneity of desmoplastic infantile ganglioglioma: a case series and literature review. J Pediatr Hematol Oncol 34:e232e236, 2012

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Kesavadas C, Sonwalker H, Thomas B, Gupta AK, Radhakrishnan VV: Atypical MRI appearance of desmoplastic infantile ganglioglioma. Pediatr Radiol 35:10241026, 2005

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Koelsche C, Sahm F, Paulus W, Mittelbronn M, Giangaspero F, Antonelli M, et al.: BRAF V600E expression and distribution in desmoplastic infantile astrocytoma/ganglioglioma. Neuropathol Appl Neurobiol 40:337344, 2014

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Kuchelmeister K, Bergmann M, von Wild K, Hochreuther D, Busch G, Gullotta F: Desmoplastic ganglioglioma: report of two non-infantile cases. Acta Neuropathol 85:199204, 1993

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21

    Loh JK, Lieu AS, Chai CY, Howng SL: Malignant transformation of a desmoplastic infantile ganglioglioma. Pediatr Neurol 45:135137, 2011

  • 22

    Lönnrot K, Terho M, Kähärä V, Haapasalo H, Helén P: Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol 68:304308, 2007

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23

    Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al.: The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 131:803820, 2016

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24

    Mallucci C, Lellouch-Tubiana A, Salazar C, Cinalli G, Renier D, Sainte-Rose C, et al.: The management of desmoplastic neuroepithelial tumours in childhood. Childs Nerv Syst 16:814, 2000

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    Martin DS, Levy B, Awwad EE, Pittman T: Desmoplastic infantile ganglioglioma: CT and MR features. AJNR Am J Neuroradiol 12:11951197, 1991

  • 26

    Milanaccio C, Nozza P, Ravegnani M, Rossi A, Raso A, Gambini C, et al.: Cervico-medullary desmoplastic infantile ganglioglioma: an unusual case with diffuse leptomeningeal dissemination at diagnosis. Pediatr Blood Cancer 45:986990, 2005

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 27

    Narayan V, Savardekar AR, Mahadevan A, Arivazhagan A, Appaji L: Unusual occurrence of multifocal desmoplastic infantile astrocytoma: a case report and review of the literature. Pediatr Neurosurg 52:173180, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 28

    Nikas I, Anagnostara A, Theophanopoulou M, Stefanaki K, Michail A, Hadjigeorgi Ch: Desmoplastic infantile ganglioglioma: MRI and histological findings case report. Neuroradiology 46:10391043, 2004

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 29

    Pasquale S, Dam A, Kelly C, Schubert R, Melville L: Progressive disordered movements in an infant leads to rare diagnosis. Clin Pract Cases Emerg Med 1:5355, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30

    Romero-Rojas AE, Diaz-Perez JA, Lozano-Castillo A: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J 26:649654, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31

    Rout P, Santosh V, Mahadevan A, Kolluri VR, Yasha TC, Shankar SK: Desmoplastic infantile ganglioglioma—clinicopathological and immunohistochemical study of four cases. Childs Nerv Syst 18:463467, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 32

    Rushing EJ, Rorke LB, Sutton L: Problems in the nosology of desmoplastic tumors of childhood. Pediatr Neurosurg 19:5762, 1993

  • 33

    Samkari A, Alzahrani F, Almehdar A, Algahtani H: Desmoplastic infantile astrocytoma and ganglioglioma: case report and review of the literature. Clin Neuropathol 36:3140, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Setty SN, Miller DC, Camras L, Charbel F, Schmidt ML: Desmoplastic infantile astrocytoma with metastases at presentation. Mod Pathol 10:945951, 1997

  • 35

    Sperner J, Gottschalk J, Neumann K, Schörner W, Lanksch WR, Scheffner D: Clinical, radiological and histological findings in desmoplastic infantile ganglioglioma. Childs Nerv Syst 10:458463, 1994

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 36

    Takeshima H, Kawahara Y, Hirano H, Obara S, Niiro M, Kuratsu J: Postoperative regression of desmoplastic infantile gangliogliomas: report of two cases. Neurosurgery 53:979984, 2003

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 37

    Tamburrini G, Colosimo C Jr, Giangaspero F, Riccardi R, Di Rocco C: Desmoplastic infantile ganglioglioma. Childs Nerv Syst 19:292297, 2003

  • 38

    Taranath A, Lam A, Wong CK: Desmoplastic infantile ganglioglioma: a questionably benign tumour. Australas Radiol 49:433437, 2005

  • 39

    Taratuto AL, Monges J, Lylyk P, Leiguarda R: Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer 54:25052512, 1984

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Tseng JH, Tseng MY, Kuo MF, Tseng CL, Chang YL: Chronological changes on magnetic resonance images in a case of desmoplastic infantile ganglioglioma. Pediatr Neurosurg 36:2932, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    VandenBerg SR: Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 3:275281, 1993

  • 42

    VandenBerg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, et al.: Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 66:5871, 1987

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Wang AC, Jones DTW, Abecassis IJ, Cole BL, Leary SES, Lockwood CM, et al.: Desmoplastic infantile ganglioglioma/astrocytoma (DIG/DIA) are distinct entities with frequent BRAFV600 mutations. Mol Cancer Res 16:14911498, 2018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • Collapse
  • Expand
  • Case 1. Upper Row: Left posterior temporal hemorrhagic lesion interpreted at the referring hospital as a venous infarction at initial presentation. Noncontrast head CT on day 3 of life (A) and T2-weighted axial MRI on day 5 of life (B). Middle Row: Preoperative contrast-enhanced axial (C) and sagittal (D) T1-weighted MR images at age 8 months showing a large, heterogeneously enhancing, left posterior temporal mass. Lower Row: Postoperative contrast-enhanced axial (E) and sagittal (F) T1-weighted axial MR images 1 day after surgery, confirming gross-total tumor resection.

  • Case 1. A: Operative exposure of a large, firm, left temporal tumor that was adherent to the meninges. Anterior is to the right. B: Resected tumor. Figure is available in color online only.

  • Case 1. Photomicrograph showing DIG, WHO grade I, with ganglion cells in a background of spindle-shaped astrocytes. H & E, original magnification ×200. Figure is available in color online only.

  • Case 2. A: Fetal FIESTA sagittal MRI performed at 32 weeks gestation demonstrating a large, heterogeneous, left hemisphere mass. B–D: Preoperative postcontrast fast spoiled gradient echo axial, sagittal, and coronal MR images at birth, demonstrating a large, enhancing, solid and cystic left hemisphere tumor.

  • Case 2. A: Operative exposure of a large, left parietal, solid and cystic tumor adherent to the meninges. Anterior is to the right. B–E: Axial T2-weighted (B and C), T1-weighted contrast-enhanced (D), and sagittal T1-weighted contrast-enhanced (E) MR images obtained 1 day after surgery, confirming tumor resection. Figure is available in color online only.

  • Case 3. Pre- and postoperative MR images. A–C: Preoperative axial T2-weighted images showing marked right hemispheric edema. D–F: Preoperative contrast-enhanced axial, sagittal, and coronal images showing a solid, enhancing, right parietal parasagittal tumor with edema. G–I: Postoperative contrast-enhanced axial, sagittal, and coronal images obtained 4 months after surgery showing no residual enhancing tumor.

  • Case 3. Histopathological images showing a WHO grade I DIG. A: Image showing moderate cellularity with elongated astrocytes in a conspicuous storiform pattern. H & E, original magnification ×100. B: Higher magnification view. H & E, original magnification ×200. C: GFAP immunostaining showing abundance of elongated astrocytes. Original magnification ×200. D: Synaptophysin immunostaining labeling rare dysplastic ganglion cells. Original magnification ×200. Figure is available in color online only.

  • 1

    Alexiou GA, Stefanaki K, Sfakianos G, Prodromou N: Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature. Pediatr Neurosurg 44:422425, 2008

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Alghamdi S, Castellano-Sanchez A, Brathwaite C, Shimizu T, Khatib Z, Bhatia S: Strong desmin expression in a congenital desmoplastic infantile ganglioglioma mimicking pleomorphic rhadomyosarcoma: a case report including ultrastructural and cytogenetic evaluation and review of the literature. Childs Nerv Syst 28:21572162, 2012

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Bächli H, Avoledo P, Gratzl O, Tolnay M: Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. Childs Nerv Syst 19:359366, 2003

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Balaji R, Ramachandran K: Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. Childs Nerv Syst 25:497501, 2009

  • 5

    Bianchi F, Tamburrini G, Massimi L, Caldarelli M: Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review. Childs Nerv Syst 32:18331838, 2016

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Blessing MM, Blackburn PR, Balcom JR, Krishnan C, Harrod VL, Zimmermann MT, et al.: Novel BRAF alteration in desmoplastic infantile ganglioglioma with response to targeted therapy. Acta Neuropathol Commun 6:118, 2018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Cerdá-Nicolás M, Lopez-Gines C, Gil-Benso R, Donat J, Fernandez-Delgado R, Pellin A, et al.: Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features. Histopathology 48:617621, 2006

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Chatterjee D, Garg C, Singla N, Radotra BD: Desmoplastic non-infantile astrocytoma/ganglioglioma: rare low-grade tumor with frequent BRAF V600E mutation. Hum Pathol 80:186191, 2018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, et al.: Desmoplastic infantile ganglioglioma: a potentially malignant tumor? Am J Surg Pathol 26:15151522, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Duffner PK, Burger PC, Cohen ME, Sanford RA, Krischer JP, Elterman R, et al.: Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurgery 34:583589, 1994

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Gelabert-Gonzalez M, Serramito-García R, Arcos-Algaba A: Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. Neurosurg Rev 34:151158, 2010

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Geramizadeh B, Kamgarpour A, Moradi A: Desmoplastic infantile ganglioglioma: Report of a case and review of the literature. J Pediatr Neurosci 5:4244, 2010

  • 13

    Gessi M, Zur Mühlen A, Hammes J, Waha A, Denkhaus D, Pietsch T: Genome-wide DNA copy number analysis of desmoplastic infantile astrocytomas and desmoplastic infantile gangliogliomas. J Neuropathol Exp Neurol 72:807815, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Greer A, Foreman NK, Donson A, Davies KD, Kleinschmidt-DeMasters BK: Desmoplastic infantile astrocytoma/ganglioglioma with rare BRAF V600D mutation. Pediatr Blood Cancer 64:e26350, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Gundamaneni SK, Ganesh CV, Mahadevan A, Madhugiri VS, Sasidharan GM, Kumar VR: Non-infantile desmoplastic cerebellar ganglioglioma in a patient with multiple congenital anomalies: a rare association. Pediatr Neurosurg 49:105109, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16

    Hoving EW, Kros JM, Groninger E, den Dunnen WF: Desmoplastic infantile ganglioglioma with a malignant course. J Neurosurg Pediatr 1:9598, 2008

  • 17

    Hummel TR, Miles L, Mangano FT, Jones BV, Geller JI: Clinical heterogeneity of desmoplastic infantile ganglioglioma: a case series and literature review. J Pediatr Hematol Oncol 34:e232e236, 2012

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Kesavadas C, Sonwalker H, Thomas B, Gupta AK, Radhakrishnan VV: Atypical MRI appearance of desmoplastic infantile ganglioglioma. Pediatr Radiol 35:10241026, 2005

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Koelsche C, Sahm F, Paulus W, Mittelbronn M, Giangaspero F, Antonelli M, et al.: BRAF V600E expression and distribution in desmoplastic infantile astrocytoma/ganglioglioma. Neuropathol Appl Neurobiol 40:337344, 2014

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Kuchelmeister K, Bergmann M, von Wild K, Hochreuther D, Busch G, Gullotta F: Desmoplastic ganglioglioma: report of two non-infantile cases. Acta Neuropathol 85:199204, 1993

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21

    Loh JK, Lieu AS, Chai CY, Howng SL: Malignant transformation of a desmoplastic infantile ganglioglioma. Pediatr Neurol 45:135137, 2011

  • 22

    Lönnrot K, Terho M, Kähärä V, Haapasalo H, Helén P: Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol 68:304308, 2007

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23

    Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al.: The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 131:803820, 2016

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24

    Mallucci C, Lellouch-Tubiana A, Salazar C, Cinalli G, Renier D, Sainte-Rose C, et al.: The management of desmoplastic neuroepithelial tumours in childhood. Childs Nerv Syst 16:814, 2000

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    Martin DS, Levy B, Awwad EE, Pittman T: Desmoplastic infantile ganglioglioma: CT and MR features. AJNR Am J Neuroradiol 12:11951197, 1991

  • 26

    Milanaccio C, Nozza P, Ravegnani M, Rossi A, Raso A, Gambini C, et al.: Cervico-medullary desmoplastic infantile ganglioglioma: an unusual case with diffuse leptomeningeal dissemination at diagnosis. Pediatr Blood Cancer 45:986990, 2005

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 27

    Narayan V, Savardekar AR, Mahadevan A, Arivazhagan A, Appaji L: Unusual occurrence of multifocal desmoplastic infantile astrocytoma: a case report and review of the literature. Pediatr Neurosurg 52:173180, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 28

    Nikas I, Anagnostara A, Theophanopoulou M, Stefanaki K, Michail A, Hadjigeorgi Ch: Desmoplastic infantile ganglioglioma: MRI and histological findings case report. Neuroradiology 46:10391043, 2004

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 29

    Pasquale S, Dam A, Kelly C, Schubert R, Melville L: Progressive disordered movements in an infant leads to rare diagnosis. Clin Pract Cases Emerg Med 1:5355, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30

    Romero-Rojas AE, Diaz-Perez JA, Lozano-Castillo A: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J 26:649654, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31

    Rout P, Santosh V, Mahadevan A, Kolluri VR, Yasha TC, Shankar SK: Desmoplastic infantile ganglioglioma—clinicopathological and immunohistochemical study of four cases. Childs Nerv Syst 18:463467, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 32

    Rushing EJ, Rorke LB, Sutton L: Problems in the nosology of desmoplastic tumors of childhood. Pediatr Neurosurg 19:5762, 1993

  • 33

    Samkari A, Alzahrani F, Almehdar A, Algahtani H: Desmoplastic infantile astrocytoma and ganglioglioma: case report and review of the literature. Clin Neuropathol 36:3140, 2017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Setty SN, Miller DC, Camras L, Charbel F, Schmidt ML: Desmoplastic infantile astrocytoma with metastases at presentation. Mod Pathol 10:945951, 1997

  • 35

    Sperner J, Gottschalk J, Neumann K, Schörner W, Lanksch WR, Scheffner D: Clinical, radiological and histological findings in desmoplastic infantile ganglioglioma. Childs Nerv Syst 10:458463, 1994

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 36

    Takeshima H, Kawahara Y, Hirano H, Obara S, Niiro M, Kuratsu J: Postoperative regression of desmoplastic infantile gangliogliomas: report of two cases. Neurosurgery 53:979984, 2003

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 37

    Tamburrini G, Colosimo C Jr, Giangaspero F, Riccardi R, Di Rocco C: Desmoplastic infantile ganglioglioma. Childs Nerv Syst 19:292297, 2003

  • 38

    Taranath A, Lam A, Wong CK: Desmoplastic infantile ganglioglioma: a questionably benign tumour. Australas Radiol 49:433437, 2005

  • 39

    Taratuto AL, Monges J, Lylyk P, Leiguarda R: Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer 54:25052512, 1984

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Tseng JH, Tseng MY, Kuo MF, Tseng CL, Chang YL: Chronological changes on magnetic resonance images in a case of desmoplastic infantile ganglioglioma. Pediatr Neurosurg 36:2932, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    VandenBerg SR: Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 3:275281, 1993

  • 42

    VandenBerg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, et al.: Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 66:5871, 1987

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Wang AC, Jones DTW, Abecassis IJ, Cole BL, Leary SES, Lockwood CM, et al.: Desmoplastic infantile ganglioglioma/astrocytoma (DIG/DIA) are distinct entities with frequent BRAFV600 mutations. Mol Cancer Res 16:14911498, 2018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 2221 157 0
Full Text Views 279 43 14
PDF Downloads 258 41 11
EPUB Downloads 0 0 0