Tumor Flashcards
Choroid plexus carcinomas are most commonly found in patients with which of the following hereditary syndromes?
Answers:
A. Von-Hippel-Lindau syndrome
B. Neurofibromatosis type I
C. Tuberous sclerosis Syndrome
D. Li-Fraumeni syndrome
E. Neurofibromatosis type II
Li-Fraumeni syndrome
Discussion:
Choroid plexus tumors are a rare intraventricular neoplasm originating from the choroid plexus.
They are predominantly found in children and are located in the lateral ventricle. The WHO
classifies these tumors into 3 subtypes: grade 1 choroid plexus papilloma (CPP), grade 2 atypical
CPP, and grade 3 choroid plexus carcinoma (CPC). CPCs occurs mainly in children, with a median
age of 3 years.
Li-Fraumeni syndrome (LFS) is an autosomal dominant disorder caused by a germline mutation in
the TP53 tumor suppressor gene. Patients with LFS are at risk for developing several types of
intracranial malignancies, including choroid plexus carcinomas, gliomas, primitive neuroectodermal
tumors, and medulloblastomas. These tumors manifest at an earlier age in patients with LFS, with
a mean of 25 years.
Tuberous sclerosis complex (TSC) is associated with benign hamartomas of the brain, eyes, heart,
lung, kidney, liver, and skin. It is of autosomal dominant inheritance due to mutations in the TSC1
or TSC2 gene. The characteristic brain tumor in TSC is subependymal giant cell astrocyotoma
(SEGA).
Neurofibromatosis type I is an autosomal dominant disorder resulting from a mutation of the NF1
tumor-suppressor gene on chromosome 17q. It is associated with low grade gliomas of the optic
nerve and brainstem.
Neurofibromatosis type II is an autosomal dominant disorder resulting from a mutation of the NF2
gene on chromosome 22. It is associated with bilateral vestibular schwannomas.
von Hippel Lindau syndrome is an autosomal dominant disorder that is associated with
hemangioblastomas.
References:
Orr BA, Clay MR, Pinto EM, Kesserwan C. An update on the central nervous system
manifestations of Li-Fraumeni syndrome. Acta Neuropathol. 2020 Apr;139(4):669-687. doi:
10.1007/s00401-019-02055-3. Epub 2019 Aug 30. PMID: 31468188.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/31468188
Vijapura C, Saad Aldin E, Capizzano AA, Policeni B, Sato Y, Moritani T. Genetic Syndromes
Associated with Central Nervous System Tumors. Radiographics. 2017 Jan-Feb;37(1):258-280.
doi: 10.1148/rg.2017160057. Epub 2016 Dec 2. PMID: 27911673.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/27911673
A 48-year-old man is evaluated because of severe right leg pain and urinary frequency. Physical examination shows a positive straight-leg raising test on the right. Urodynamic studies show no abnormalities. MR imaging of the lumbar spine depicting a mass lesion is shown in Figures 1-4. Laminectomy for excision is performed. Based on the histopathology shown in Figure 5, which of the following is the most likely pathologic diagnosis?
Answers:
A. Astrocytoma
B. Schwannoma
C. Paraganglioma
D. Ependymoma
E. Neurofibroma
Paraganglioma
Discussion:
The correct answer is Paraganglioma. Paragangliomas are neuroendocrine tumors which mainly
arise in the adrenal gland and, less commonly, in various extra-adrenal locations. Paragangliomas
of the central nervous system are uncommon, with the vast majority presenting in the cauda
equina region of the spinal cord.
MR imaging typically shows a well-circumscribed hypo- or isointense intradural extramedullary
lesion on T1-weighted sequences and a hyperintense lesion on T2-weighted sequences. Strong
homogenous enhancement of the tumor is typically seen on contrasted sequences. The presence
of serpentine, congested, ectatic vessels and a low signal intensity rim (“cap sign”) are considered
diagnostically helpful clues but not unique to paragangliomas.
Histological features include “zellballen” appearance, or a nesting pattern, with trabeculae and
cords of cells within thin fibrovascular stroma. The predominant cell type is the chief cell, which is
round to polygonal in shape and possesses central round to oval nuclei with finely stippled
chromatin and inconspicuous nucleoli. Cytoplasm is usually eosinophilic and finely granular. The
second cell type is the sustentacular or supporting cell, which is spindle-shaped.
Pathologically, ependymomas are well-circumscribed tumors characterized by perivascular
pseudorosettes, true ependymal rosettes, and fibrillar areas. Schwannomas are characterized by a
dumbbell shaped appearance on MR imaging with biphasic hypercellular Antoni A areas and
myxoid hypocellular Antoni B areas. Nuclear palisading around fibrillary processes, known as
Verocay bodies, can also be seen in the cellular areas. Neurofibromas demonstrate Schwann cells
and fibroblasts and contain a rich network of collagen fibers. Astrocytomas of the spine are
intramedullary lesions and would not exhibit this MR imaging appearance.
References:
Mishra T, Goel NA, Goel AH. Primary paraganglioma of the spine: a clinicopathological study of
eight cases. Journal of Craniovertebral Junction and Spine. 2014 Jan;5(1):20.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/25013343/
Binkley W, Vakili ST, Worth R. Paraganglioma of the cauda equina: case report. Journal of
Neurosurgery. 1982 Feb 1;56(2):275-9.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/27298894/
IDH mutant oligodendrogliomas are characterized by which of the following other genetic finding?
Answers:
A. H3 G34-mutant
B. H3 K27-altered
C. 1p/19q co-deleted
D. MYB- or MYBL-1 altered
E. MYCN-amplified
1p/19q co-deleted
Discussion:
Oligodendroglial tumors have an annual incidence rate of around 1,000 new cases in the United
States. They most commonly occur between 25 and 45 years of age. Oligodendroglioma is
genetically defined as a tumor confirmed to harbor either an IDH1 or IDH2 mutation along with codeletion of chromosome arms 1p and 19q.
In contrast to IDH-mutant astrocytomas, oligodendrogliomas tend to have retained ATRX
expression and lack accumulation of p53, since 1p/19q co-deletion is mutually exclusive with TP53
and ATRX alterations. Some oligodendrogliomas have CDKN2A/B deletion, which often predicts a
more aggressive clinical course when present. MYBL-1 mutations are seen in pediatric gliomas of
the optic pathway. H3K27 mutations are characteristic of pediatric midline gliomas. H3 G34-mutant
high-grade gliomas are rare and newly recognized infiltrating gliomas of the cerebral hemispheres.
References:
Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, Hawkins C, Ng HK, Pfister
SM, Reifenberger G, Soffietti R. The 2021 WHO classification of tumors of the central nervous
system: a summary. Neuro-oncology. 2021 Aug 2;23(8):1231-51.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/34185076/
Smith HL, Wadhwani N, Horbinski C. Major Features of the 2021 WHO Classification of CNS
Tumors. Neurotherapeutics. 2022 May 16:1-4.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/35578106/
A 30-year-old woman with a family history of brain tumors presents with recurrent vomiting. MR imaging shows an enhancing mass at the obex. The intraoperative photograph shown depicts the gross appearance of the lesion at surgery (T = cerebellar tonsils, M = medulla). The lesion seen in
the image is the typical gross appearance of which of the following?
Answers:
A. Hemangioblastoma
B. Pilocytic astrocytoma
C. Ependymoma
D. Choroid plexus papilloma
E. Medulloblastoma
Hemangioblastoma
Discussion:
The correct answer is hemangioblastoma. Macroscopically, hemangioblastomas are wellcircumscribed, highly vascular red nodules which may be found in the in walls of a cyst. Pilocytic
astrocytomas are soft, grey, discrete masses with an accompanying cyst. Posterior fossa
ependymomas are grey tumors with areas of necrosis and hemorrhage; they may originate from
the floor or the roof of the 4th ventricle and fill the entire lumen of the ventricle. Choroid plexus
papillomas are well-circumscribed, cauliflower-like masses which lie within the ventricular lumen.
Medulloblastomas vary macroscopically; they may be soft or firm in texture with discrete or poorly
circumscribed margins.
References:
Wind, J. J. et al. Long-term outcome after resection of brainstem hemangioblastomas in von
Hippel-Lindau disease. J Neurosurg 114, 1312–8 (2010).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/20932100/
Weil, R. J., Lonser, R. R., Devroom, H. L., Wanebo, J. E. & Oldfield, E. H. Surgical management of
brainstem hemangioblastomas in patients with von Hippel—Lindau disease. J Neurosurg 98,
95–105 (2003).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/12546357/
A 7-year-old boy is evaluated because of headache and diplopia. MR imaging shows a posterior fossa mass, and a craniotomy is performed for resection of the tumor. The photomicrograph (H & E stain) shown is a representative sample of the tumor. Based on imaging and the histopathology
shown, which of the following is the most likely diagnosis?
Answers:
A. Medulloblastoma
B. Ependymoma
C. Metastatic carcinoma
D. Pilocytic astrocytoma
E. Glioblastoma
Medulloblastoma
Discussion:
The correct answer is Medulloblastoma. Medulloblastoma is the most common malignant pediatric
brain tumor that accounts for approximately 25% of all intracranial neoplasms and 50% of posterior
fossa tumors in this age group. The majority of medulloblastomas arise in children with a median
age of 9 years, and have a peak in incidence between the ages of 3 and 7 years. However, a
second peak is seen in adults. The 5-year overall survival for medulloblastoma is approximately
75%. Hematoxylin and eosin-stained histological section shows a sheet of small round blue cells
with hyperchromatic nuclei, consistent with medulloblastoma. Pilocytic astrocytomas are typically
characterized by Rosenthal fibers and eosinophilic granular bodies. Metastatic carcinomas can
have variable appearances depending on their site of origin and are not characterized by small
round blue cells. Ependymomas are well-circumscribed tumors characterized by perivascular
pseudorosettes, true ependymal rosettes, and fibrillar areas. Glioblastomas are characterized by
cellular pleomorphism, nuclear atypia, mitotic activity, and microvascular proliferation with areas of
pseudopalisading necrosis and vascular thrombosis.
References:
Orr BA. Pathology, diagnostics, and classification of medulloblastoma. Brain Pathology. 2020
May;30(3):664-78.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/32239782/
Suk Y, Gwynne WD, Burns I, Venugopal C, Singh SK. Childhood Medulloblastoma: An Overview.
Medulloblastoma. 2022:1-2.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/34978683/
An increase in the level of which of the following substances in urine is most definitive for making the diagnosis of neuroblastoma?
Answers:
A. Homovanillic acid
B. Ketones
C. Chitotriosidase
D. Homocysteine
E. Oxalate Crystals
Homovanillic acid
Discussion:
Neuroblastoma is the most common solid tumor to occur in infants. These tumors can occur in a
variety or anatomic locations, but generally arise from the adrenal glands. Neuroblastomas can
also occur in the chest, abdomen, or spine. The malignant cells here are of neural crest
differentiation and in 90% of cases have elevated serum and urinary levels of catecholamines and
their metabolites, including dopamine, Homovanillic acid (HVA), and Vanillylmandelic acid (VMA).
Ketones can be found in the urine of patients who are dehydrated.
Homocysteine is typically seen in the urine of patients with Homocystinuria.
Chitotriosidase has been found in the urine of patients with Gaucher’s disease.
Oxalate Crystals in the urine can be seen in patients with kidney stones or after ingestion of
ethylene glycol.
References:
Strenger et al. Diagnostic and prognostic impact of urinary catecholamines in neuroblastoma
patients. Pediatr Blood Cancer. 2007 May;48(5):504-9. doi: 10.1002/pbc.20888
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/16732582/
Kushner et al. Neuroblastoma: a disease requiring a multitude of imaging studies. J Nucl Med .
2004 Jul;45(7):1172-88
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/15235064/
Which of the following factors is most likely to accurately predict overall survival for patients with brain metastases?
Answers:
A. Karnofsky performance status
B. Presence of liver metastases
C. Less than 3 brain metasases
D. Tumor size <3cm
E. Home oxygen use
Karnofsky performance status
Discussion:
The correct answer is Karnofsky performance status. Each of these factors can have an impact on
predicting survival in metastatic cancer. However, the impact of number of brain metastases, size
of the brain metastases, distant site involvement, and use of home oxygen are cancer type
specific. Of the factors mentioned, only the Karnofsky performance status (KPS) applies to all
metastatic tumor types regardless of primary site of disease. The KPS is a score that describes a
person’s functional level of independence that ranges from as low as 0 (dead) to 100 (healthy, no
signs or symptoms of disease). Scores are given in 10-point increments, with lower scores
corresponding to more severe impact of the disease.
References:
Sperduto PW, Chao ST, Sneed PK, et al. Diagnosis-specific prognostic factors, indexes, and
treatment outcomes for patients with newly diagnosed brain metastases: a multi-institutional
analysis of 4,259 patients. Int J Radiat Oncol Biol Phys. 2010 Jul 1;77(3):655-61.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/19942357/
Gaspar LE, Mehta MP, Patchell RA, et al. The role of whole brain radiation therapy in the
management of newly diagnosed brain metastases: a systematic review and evidence-based
clinical practice guideline. J Neurooncol. 2010;96(1):17-32.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/19960231/
Medulloblastomas associated with Gorlin syndrome are most likely to have which of the following germline mutations?
Answers:
A. PTCH1
B. MSH2
C. Myc
D. p53
E. CTNNB1
PTCH1
Discussion:
The correct answer is PTCH1. Gorlin syndrome is caused by a germ line mutation in the PTCH1
gene and can result in the formation of hemispheric medulloblastomas of the nodular/desmoplastic
type. Biallelic mutations in the mismatch repair gene MSH2 causes mismatch repair cancer
syndrome, also known as Turcot syndrome, which is associated with CNS and intestinal
neoplasias. Germline mutations in p53 cause the autosomal dominant disorder Li-Fraumeni
syndrome, which causes a variety of tumors including sarcomas, brain tumors, breast cancer and
leukemias. WNT subgroup/group 1 medulloblastomas are associated with point mutations of
CTNNB1. Group medulloblastomas are associated with amplification of Myc.
References:
Taylor, M. D., Mainprize, T. G. & Rutka, J. T. Molecular Insight into Medulloblastoma and Central
Nervous System Primitive Neuroectodermal Tumor Biology from Hereditary Syndromes: A Review.
Neurosurgery 47, 888 (2000).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/11014429/
Farndon, P. A., Mastro, R. G. D., Kilpatrick, M. W. & Evans, D. R. G. Location of gene for Gorlin
syndrome. Lancet 339, 581–582 (1992).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/1347096
Choroid plexus carcinomas are most commonly found in patients with which of the following hereditary syndromes?
Answers:
A. Li-Fraumeni syndrome
B. Tuberous sclerosis Syndrome
C. Neurofibromatosis type I
D. Von-Hippel-Lindau syndrome
E. Neurofibromatosis type II
Li-Fraumeni syndrome
Discussion:
Choroid plexus tumors are a rare intraventricular neoplasm originating from the choroid plexus.
They are predominantly found in children and are located in the lateral ventricle. The WHO
classifies these tumors into 3 subtypes: grade 1 choroid plexus papilloma (CPP), grade 2 atypical
CPP, and grade 3 choroid plexus carcinoma (CPC). CPCs occurs mainly in children, with a median
age of 3 years.
Li-Fraumeni syndrome (LFS) is an autosomal dominant disorder caused by a germline mutation in
the TP53 tumor suppressor gene. Patients with LFS are at risk for developing several types of
intracranial malignancies, including choroid plexus carcinomas, gliomas, primitive neuroectodermal
tumors, and medulloblastomas. These tumors manifest at an earlier age in patients with LFS, with
a mean of 25 years.
Tuberous sclerosis complex (TSC) is associated with benign hamartomas of the brain, eyes, heart,
lung, kidney, liver, and skin. It is of autosomal dominant inheritance due to mutations in the TSC1
or TSC2 gene. The characteristic brain tumor in TSC is subependymal giant cell astrocyotoma
(SEGA).
Neurofibromatosis type I is an autosomal dominant disorder resulting from a mutation of the NF1
tumor-suppressor gene on chromosome 17q. It is associated with low grade gliomas of the optic
nerve and brainstem.
Neurofibromatosis type II is an autosomal dominant disorder resulting from a mutation of the NF2
gene on chromosome 22. It is associated with bilateral vestibular schwannomas.
von Hippel Lindau syndrome is an autosomal dominant disorder that is associated with
hemangioblastomas.
References:
Orr BA, Clay MR, Pinto EM, Kesserwan C. An update on the central nervous system
manifestations of Li-Fraumeni syndrome. Acta Neuropathol. 2020 Apr;139(4):669-687. doi:
10.1007/s00401-019-02055-3. Epub 2019 Aug 30. PMID: 31468188.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/31468188
Vijapura C, Saad Aldin E, Capizzano AA, Policeni B, Sato Y, Moritani T. Genetic Syndromes
Associated with Central Nervous System Tumors. Radiographics. 2017 Jan-Feb;37(1):258-280.
doi: 10.1148/rg.2017160057. Epub 2016 Dec 2. PMID: 27911673.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/27911673
Which of the following tumors is most likely to seed into the subarachnoid space?
Answers:
A. Subependymoma
B. Myxopapillary Ependymoma
C. Olfactory Neuroblastoma
D. Medulloblastoma
E. Solitary fibrous tumor/hemangiopericytoma
Medulloblastoma
Discussion:
Medulloblastoma has evidence of dissemination throughout the craniospinal axis in 10-35% of
patients at first diagnosis. As a result, craniospinal irradiation with a boost to the tumor bed is
often employed post-operatively. All patients with suspected medulloblastomas should undergo
craniospinal imaging at first diagnosis (preferably pre-operatively, as post-operative changes can
make interpretation of spinal imaging more difficult to evaluate small drop metastases).
Myxopapillary ependymomas are usually well circumscribed lesions that arise within the filum
terminale. These tumors can disseminate, but this usually occurs if they are not resected en bloc
or if their capsule is violated during surgical resection.
Subependymomas are low grade tumors that typically do not disseminate throughout the
subarachnoid space.
Olfactory Neuroblastomas typically are locally aggressive with low chance of CSF dissemination.
Solitary fibrous tumor/hemangiopericytomas are locally aggressive tumors that have a predilection
for recurrence. These tumors can metastasize but generally do not spread throughout the
neuraxis.
References:
Kocakaya et al. Chemotherapy increases long-term survival in patients with adult
medulloblastoma–a literature-based meta-analysis. Neuro Oncol. 2016 Mar;18(3):408-16. doi:
10.1093/neuonc/nov185
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/26359208/
Cho et al. Integrative genomic analysis of medulloblastoma identifies a molecular subgroup that
drives poor clinical outcome. J Clin Oncol. 2011 Apr 10;29(11):1424-30. doi:
10.1200/JCO.2010.28.5148. Epub 2010 Nov 22
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/21098324/
A 35-year-old man is evaluated because of severe back and left leg pain. MR imaging of a mass lesion is shown in Figures 1-4. Surgical resection is performed. Based on the MR images and histopathology shown in Figure 5, which of the following is the most likely diagnosis?
Answers:
A. Adenoid cystic carcinoma
B. Chordoma
C. Schwannoma
D. Myxopapillary ependymoma
E. Paraganglioma
Myxopapillary ependymoma
Discussion:
The correct answer is Myxopapillary ependymoma. Myxopapillary ependymoma is a rare subtype
of ependymoma that is classified as WHO grade 1 and has benign biological characteristics. It is
mainly derived from ependymal cells located in the central duct of the terminal filament and
accounts for about 13% of all ependymomas, with an incidence of
0.08 cases per 100,000 individuals. The overall prognosis of Myxopapillary ependymoma is
favorable, with an overall 10-year survival rate of 97%.
Myxopapillary ependymoma occurs more commonly at the level of the conus medullaris. It typically
shows an iso- or hypointense signal on T1 weighted MRI and hyperintense signal on T2 weighted
MRI. It usually enhances with contrast, commonly homogenous enhancement but can show
heterogenous enhancement patterns. Histologically, it demonstrates fibrovascular cores, rimmed
by cuboidal to columnar cells sending fibrillary cytoplasmic processes forming pseudorosettes
along with the presence of hyaline globules, and myxoid material.
Paragangliomas are characterized by homogeneous contrast enhancement with serpiginous
vascularity on imaging with a nesting pattern with trabeculae and cords of cells within a
fibrovascular stroma. While schwannomas can occur at the level of the conus medullaris,
histologically they are characterized by biphasic hypercellular Antoni A areas and myxoid
hypocellular Antoni B areas as well as nuclear palisading around fibrillary processes, known as
Verocay bodies, within the cellular areas. Adenoid cystic carcinoma is a salivary gland tumor
composed of ductal and myoepithelial cells with dark angulated nuclei and scant cytoplasm, and
would be highly unlikely to metastasize to the intradural space. Chordomas are expansile,
lobulated, intraosseous masses that demonstrate infiltrative borders with epithelioid cells
demonstrating abundant, clear to eosinophilic cytoplasm that gives it a soap bubble appearance.
References:
Fan F, Zhou J, Zheng Y, Liu S, Tang Z, Wang Y. Clinical Features, Treatments, and Prognostic
Factors of Spinal Myxopapillary Ependymoma. World Neurosurgery. 2021 May 1;149:e1105-11.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/33412325/
Choudhury M, Rautela A, Nain M. Myxopapillary ependymoma: Lesser known cytomorphologic
features. Journal of Cytology/Indian Academy of Cytologists. 2015 Jan;32(1):42.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/25948946/
Which of the following tumors is most commonly seen in patients with tuberous sclerosis?
Answers:
A. Hemangioblastoma
B. Subependymal giant cell astrocytomas
C. Meningioma
D. Optic glioma
E. Vestibular schwannoma
Subependymal giant cell astrocytomas
Discussion:
The correct answer is subependymal giant cell astrocytoma (SEGA). Tuberous sclerosis is an
autosomal dominant neurocutaneous syndrome that presents in infants and children and is
characterized by hypopigmented skin lesions, seizures, and hamartomas within the brain and
kidneys. Tuberous sclerosis is caused by mutations in the TSC1 or TSC2 genes, which cause
dysfunction in hamartin and tuberin, respectively. Subependymal giant cell astrocytomas are a
common feature of tuberous sclerosis. Hemangioblastomas are associated with von Hippel-Lindau
syndrome. Meningiomas can be seen as a part of Neurofibromatosis Type 2, as can bilateral
vestibular schwannomas. Optic gliomas can be seen with Neurofibromatosis Type 1.
References:
Goh S, Butler W, Thiele EA. Subependymal giant cell tumors in tuberous sclerosis complex.
Neurology. 2004 Oct 26;63(8):1457-61.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/15505165/
Randle SC. Tuberous Sclerosis Complex: A Review. Pediatr Ann. 2017 Apr 1;46(4):e166-e171.
doi: 10.3928/19382359-20170320-01. PMID: 28414398.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/28414398/
Pseudorosettes are most commonly associated with which of the following?
Answers:
A. Pilocytic Astrocytoma
B. Vestibular Schwannoma
C. Ependymoma
D. Meningiomas
E. Medulloblastoma
Ependymoma
Discussion:
Ependymomas have been increasingly found to have molecular and genetic profiles that dictate
their proclivity to recur and response to surgery and subsequent chemotherapy and/or radiation.
Nevertheless, these tumors, which can occur within the posterior fossa, in the supratentorial region
or spinal cord, have classic histologic features. These are glial based neoplasms that arise from
the ependyma. Often, cells form radiating processes around blood vessels within the tumor that
are termed “perivascular pseudorosettes;” this is considered one of the histologic hallmarks of
these neoplasms. Tumor cells can also form “true rosettes” when they form rosettes with a true
lumen (no intervening blood vessel).
Pilocytic astrocytoma – These tumors are comprised of loosely associated tissue with microcytic
changes that contain stellate appearing astrocytes, often with elongated “piloid” processes,
eosinophilic granular bodies (EGBs) and Rosenthal fibers.
Vestibular schwannomas, like all schwannomas, classically are described to have two distinct
regions on pathology: Antoni A fibers (regions of dense cellularity) and Antoni B fibers (loose
reticular regions). Verocay bodies (regions in Antoni A fibers) are also features of vestibular
schwannomas and are characterized by parallel sheets of Schwann cells (have sheets of nuclear
palisading and eosinophilic cytoplasm).
Medulloblastomas – These are a small blue cell tumors that can contain Homer Wright Rosettes.
Homer Wright Rosettes occur in tumors of neural differentiation where tumor cells group around a
central neuropil. Primitive Neuroectodermal Tumors (PNETs), Olfactory Neuroblastoma, and
Pineoblastoma also have been reported to have Homer Wright Rosettes
Meningiomas - Pathologic appearance of these tumors can vary depending on the histologic
subtype, but typically are characterized by uniform cells that have the tendency to form whorls on
histopathology. Psamomma bodies are also seen (regions of laminated calcification).
References:
Louis et al: The 2021 WHO Classification of Tumors of the Central Nervous System: a summary.
Neuro Oncol . 2021 Aug 2;23(8):1231-1251. doi: 10.1093/neuonc/noab106
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/34185076/
Pajtler et al. Molecular Classification of Ependymal Tumors across All CNS Compartments,
Histopathological Grades, and Age Groups. Cancer Cell. 2015 May 11; 27(5): 728–743.
Pubmed Web link: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4712639/
A 35-year-old woman is evaluated for a several-year history of headaches and subjective intermittent blurry vision. She reports urinary frequency and nocturia. Physical examination shows no abnormalities. Pituitary hormones are normal. She undergoes an endonasal transsphenoidal approach with resection of a suprasellar cystic mass. The pathologic slides are shown. Which of the following is the most likely diagnosis?
Answers:
A. Rathke’s cleft cyst
B. Adamantinomatous craniopharyngioma
C. Optic nerve gliomas
D. Epidermoid Cyst
E. Pituitary macroadenoma
Epidermoid Cyst
Discussion:
The correct answer is epidermoid cyst. Epidermoid cysts are characterized by dry, flaky keratin
debris which is contained by a cyst wall composed of keratinized stratified squamous epithelium
with a granular layer, findings consistent with those shown in Figure 2. Adamantinomatous
craniopharyngiomas may have stratified squamous or adamantanoid epithelium with aggregates of
wet keratin, but do not exhibit the cystic appearance seen in the images above. Histologically,
adenomas appear as monomorphic cells in a sheet-like growth pattern with abundant cytoplasm.
Rathke’s cleft cysts have a simple columnar or cuboidal epithelium with ciliated or mucinous goblet
cells, and do not exhibit the stratified squamous epithelium seen in the images above. Optic nerve
gliomas are pilocytic astrocytomas and thus are characterized by densely cellular regions
alternating with loose, cystic regions, Rosenthal fibers, and eosinophilic granular bodies.
References:
McCormack, E. P., Cappuzzo, J. M., Litvack, Z., Almira-Suarez, M. I. & Sherman, J. S. Suprasellar
Epidermoid Cyst Originating from the Infundibulum: Case Report and Literature Review. Cureus
10, e3226 (2018).
Pubmed Link: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6263494/
Zada, G., Lin, N., Ojerholm, E., Ramkissoon, S. & Laws, E. R. Craniopharyngioma and other cystic
epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological
relationships. Neurosurg Focus 28, E4 (2010).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/20367361/
A 21-year-old woman presents with visual field loss and headache. A T1-weighted axial MR image with contrast (Figure 1) shows an enhancing mass. The H & E stain (Figure 2), and STAT6 stain (Figure 3) are shown. Which of the following is the most likely diagnosis?
Answers:
A. Paraganglioma
B. Solitary fibrous tumor/hemangiopericytoma
C. Dural-based breast cancer metastasis
D. Schwannoma
E. Meningioma
Solitary fibrous tumor/hemangiopericytoma
Discussion:
The correct answer is solitary fibrous tumor (SFT), previously known as a hemangiopericytoma.
On H&E stains, SFTs are highly cellular tumors with dilated, staghorn-type vessels. The NAB2-
STAT6 gene fusion is the cytogenetic hallmark of SFTs. Immunohistochemical analysis for STAT6
serves as a robust surrogate for the presence of NAB2-STAT6. Meningiomas have a wide range of
histologic features including spindle cell components, psammoma bodies, and whorls. They stain
negative for STAT6 and positive for epithelial membrane antigen (EMA). Schwannomas have a
biphasic histologic appearance with areas of closely packed tumor cells and regions where they
are more loosely arranged (Antoni A and B, respectively). They stain positive for S-100.
Paragangliomas consist of uniform round cells arranged in small nests surrounded by a dense
capillary network. They stain positive for S-100 and synaptophysin.
References:
Louis, D. N. et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a
summary. Neuro-oncology 23, 1231–1251 (2021).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/34185076/
Doyle, L. A., Vivero, M., Fletcher, C. D., Mertens, F. & Hornick, J. L. Nuclear expression of STAT6
distinguishes solitary fibrous tumor from histologic mimics. Modern Pathol 27, 390–395 (2014).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/24030747/
The lesion in the photomicrograph shown was resected from the right frontal lobe of a previously healthy 48-year-old man. Preoperative MR imaging and CT scans showed a nonenhancing lesion with a few pockets of calcification. Which of the following genetic findings is most likely to be found
in this tumor?
Answers:
A. KIAA1549:BRAF fusions
B. Amplification of EGFR
C. Codeletions of chromosomes 1p and 19q.
D. Mutations in TSC1
E. Mutations in H3 K27
Codeletions of chromosomes 1p and 19q.
Discussion:
The correct answer is codeletions of chromosomes 1p and 19q. Histologically, oligodendrogliomas
are characterized by the presence of small, round, closely packed cells with monotonous nuclei,
perinuclear clearing, and a network of thick walled, branching blood vessels. On MRI imaging,
oligodendrogliomas are often hypointense compared to grey matter on T1 and hyperintense on
T2. Sometimes the signal can be heterogeneous due to calcification, cystic degeneration, and
hemorrhage.
The integration of molecular data has created a more objective framework for diagnosing gliomas.
Molecularly, oligodendrogliomas are defined by mutations in isocitrate dehydrogenase type 1 or
type 2 and codeletion of chromosomes 1p and 19q. Amplifications in EGFR may be seen in
glioblastomas. Mutations in H3 K27 are associated with diffuse midline gliomas. KIAA1549:BRAF
fusions are a molecular hallmark of pilocytic astrocytomas. Mutations in TSC1 cause tuberous
sclerosis syndrome, which can lead to the formation of subependymal giant cell astrocytomas.
References:
Lonser RR, Glenn GM, Walther M, et al. von Hippel-Lindau disease. Lancet.
2003;361(9374):2059-2067.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/12814730/
Shanbhogue KP, Hoch M, Fatterpaker G, Chandarana H. von Hippel-Lindau disease: review of
genetics and imaging. Radiol Clin North Am. 2016;54(3):409-422.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/27153780/
For the tumor specimen shown, the positive immunohistochemical stains for vimentin (Figure 1) and epithelial membrane antigen (Figure 2) are most consistent with which of the following pathologic entities?
Answers:
A. Schwannoma
B. Chordoma
C. Meningioma
D. Dermoid cysts
E. Neurofibroma
Meningioma
The correct answer is meningioma. Meningiomas are characterized by positive staining for
vimentin (stain for fibroblasts) and patchy staining for epithelial membrane antigen (EMA, marker
for epithelial cells). Chordomas are expansile, lobulated, intraosseous masses that demonstrate
infiltrative borders with epithelioid cells demonstrating abundant, clear to eosinophilic cytoplasm
that gives it a soap bubble appearance. They are not characterized by vimentin or EMA staining.
Neurofibromas are diffusely S100 immunoreactive (marker for Schwann cells and melanocytes)
due to entrapped axons within the tumor. Dermoid cysts are not characterized by either of these
immunostains. Schwannomas are characterized by negative EMA staining with diffuse S-100
positivity.
References:
Schnitt SJ, Vogel H. Meningiomas. Diagnostic value of immunoperoxidase staining for epithelial
membrane antigen. Am J Surg Pathol. 1986 Sep;10(9):640-9. doi:
10.1097/00000478-198609000-00006. PMID: 2428264.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/2428264/
Mawrin C, Perry A. Pathological classification and molecular genetics of meningiomas. J
Neurooncol. 2010 Sep;99(3):379-91. doi: 10.1007/s11060-010-0342-2. Epub 2010 Sep 1. PMID:
20809251.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/20809251/
Based on the H & E-stained photomicrographs shown (Figures 1 and 2), which of the following is the most likely pathologic diagnosis of this tumor that was resected from the cerebellum?
Answers:
A. Glioblastoma
B. Medulloblastoma
C. Schwannoma
D. Ependymoma
E. Pilocytic astrocytoma
Pilocytic astrocytoma
Discussion:
The correct answer is pilocytic astrocytoma. Pilocytic astrocytomas are characterized by compact
fibrillary areas (Figure 1), Rosenthal fibers (Figure 2), a biphasic appearance, and eosinophilic
granular bodies. Ependymomas are typified by rosettes, which are not present in this image.
Glioblastomas are characterized by hypercellularity, nuclear atypia, and pseudopalisading
necrosis. Medulloblastomas are much more hypercellular with small, round blue cells. The gross
appearance of schwanomma is a well circumscribed mass with degenerative changes and variable
mixture of compact spindled (Antoni A) areas and hypocellular, microcystic (Antoni B) areas rich in
macrophages and collagen fibers. A well-formed collagenous capsule is a consistent finding in
schwanomma, as well as hyalinized vessels.
References:
Fernandez C, Figarella-Branger D, Girard N, et al. Pilocytic astrocytomas in children: prognostic
factors–a retrospective study of 80 cases. Neurosurgery. 2003 Sep;53(3):544-53; discussion
554-5.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/12943571/
Collins, V. Peter, David TW Jones, and Caterina Giannini. “Pilocytic astrocytoma: pathology,
molecular mechanisms and markers.” Acta neuropathologica 129.6 (2015): 775-788.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/25792358/
A 27-year-old woman is evaluated because of confusion and headache that have progressed over the past nine months. The tumor is resected via an interhemispheric approach. On the basis of the MR and pathologic images shown, which of the following is the most likely diagnosis?
Answers:
A. Choroid plexus papilloma
B. Subependymoma
C. Lymphoma
D. Central neurocytoma
E. Glioblastoma
Subependymoma
Discussion:
The correct answer is subependymoma. Histologically, subependymomas are characterized by
clusters of isomorphic nuclei in a dense fibrillary background with microcystic changes, as
demonstrated in the image. Subependymomas occur most commonly in the fourth ventricle
followed by the lateral ventricles. Radiographically, subependymomas are T1 iso- to hypointense to
white matter with T2-hyperintensity with areas of heterogeneity. The histology shown is not
consistent with a glioblastoma where areas of necrosis, microvascular proliferation,
hypercellularity, and nuclear atypia would be expected. Additionally, an intraventricular location for
a glioblastoma would be extremely uncommon. Lymphoma is characterized by small, round blue
cells on histology. Choroid plexus papillomas have classic papillary projections which are not seen
in the slide. Central neurocytoma is characterized by monomorphic cells with round, regular nuclei
and clear cytoplasm.
References:
Varma A, Giraldi D, Mills S, Brodbelt AR, Jenkinson MD. Surgical management and long-term
outcome of intracranial subependymoma. Acta Neurochir (Wien). 2018 Sep;160(9):1793-1799
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/29915887/
Rushing EJ, Cooper PB, Quezado M, et al. Subependymoma revisited: clinicopathological
evaluation of 83 cases. J Neurooncol. 2007 Dec;85(3):297-305.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/17569000/
A 3-year-old boy presents with delayed milestones, paralysis of upward gaze, and head circumference over the 98th percentile. A preoperative T1-weighted sagittal MR image with contrast and a pathologic specimen are shown. Which of the following is the most likely diagnosis?
Answers:
A. Medulloblastoma
B. Vein of Galen malformation
C. Epidermoid cyst
D. Glioblastoma
E. Teratoma
Teratoma
Discussion:
The correct answer is teratoma. The patient presented with Parinaud’s syndrome, which localizes
to the tectum of the midbrain, which classically is compressed by pineal tumors. The most common
pineal region tumors are those of pineal origin (pineal cyst, pineocytomas, pineal parenchymal
tumors of intermediate differentiation, and pineoblastomas) or germ cell origin (teratoma,
germinoma, etc.). On imaging, teratomas are heterogeneously contrast enhancing with areas of T1
and T2 mixed intensity due to the areas of fat and calcifications within it. The tumor is in the
general vicinity of a vein of Galen malformation, but this presentation and the gross appearance of
the tumor would not occur with a vein of Galen malformation (which is a vascular pathology).
Medulloblastoma and epidermoid would both be extremely uncommon in this location. Moreover,
epidermoid cysts only exhibit thin peripheral contrast enhancement with pronounced DWI
restriction. Glioblastoma would be uncommon in this location and at this age. Therefore, teratoma
is the best answer.
References:
Dahiya S, Perry A. Pineal tumors. Adv Anat Pathol. 2010 Nov;17(6):419-27.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/20966647/
Woodward PJ, Sohaey R, Kennedy A, Koeller KK. From the archives of the AFIP: a
comprehensive review of fetal tumors with pathologic correlation. Radiographics. 2005 JanFeb;25(1):215-42.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/15653597/
A 40-year-old woman with a history of intravenous drug use presents with progressive thoracic myelopathy. Thoracic MR imaging of a mass lesion is shown (Figures 1-4). Operative resection of the lesion is performed. The MR images and histopathology shown in Figure 5 are most consistent
with which of the following pathologic processes?
Answers:
A. Glioblastoma
B. Metastatic carcinoma
C. Spinal abscess
D. Thoracic disc
E. Ependymoma
Ependymoma
Discussion:
The correct answer is Ependymoma. In the United States, 1000–2000 adults are diagnosed with
ependymoma each year. In adults, 75% of ependymomas occur in the spinal canal. Patients are
usually 30–40 years old with men and women being affected equally.
Common symptoms of thoracic spinal ependymoma include back pain, spasticity in the lower
extremities, gait ataxia, sensory loss, and paresthesia. MRI imaging of spinal cord ependymoma
will demonstrate T2 hyperintensity and distinct borders with local cord expansion. T1 sequences
with contrast will show peripheral or heterogenous enhancement with non-enhancing hypointense
portions that can be cystic change, necrosis, or hemorrhage. Histopathologic examination will
demonstrate high cellularity, perivascular rosettes (pseudorosettes), and true rosettes. Spinal
abscesses typically are not found in the spinal cord but in the epidural space and can cause cord
compression with associated cord signal change, which is not seen here. It is highly unusual to
find a solitary intramedullary metastasis with no other site of disease, which makes this answer an
unlikely choice based on this history and imaging. Thoracic discs can cause spinal cord
compression with associated cord signal change, but there are no signs of degenerative disc
pathology on the patient’s imaging; moreover, resection of cord signal change due to a disc
herniation would not be appropriate. Intramedullary glioblastomas would be expected to have a
heterogeneous pattern of enhancement with microscopy demonstrating microvascular
proliferation, pseudopalisading necrosis, and vascular thrombosis in the setting of nuclear atypia
and pleomorphism.
References:
Celano E, Salehani A, Malcolm JG, Reinertsen E, Hadjipanayis CG. Spinal cord ependymoma: a
review of the literature and case series of ten patients. Journal of Neuro-oncology. 2016
Jul;128(3):377-86.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/27154165/
Neumann JE, Spohn M, Obrecht D, Mynarek M, Thomas C, Hasselblatt M, Dorostkar MM, Wefers
AK, Frank S, Monoranu CM, Koch A. Molecular characterization of histopathological ependymoma
variants. Acta neuropathologica. 2020 Feb;139(2):305-18.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/31679042/
A 27-year-old man undergoes resection of an intraventricular mass. A biopsy of the mass is done, and the tissue stains positively for synaptophysin and neuron-specific enolase. A photomicrograph (H&E stain) is shown. Which of the following is the most likely diagnosis?
Answers:
A. Medulloblastoma
B. Neurocytoma
C. Oligodendroglioma
D. Choroid plexus papilloma
E. Lymphoma
Neurocytoma
Discussion:
An intraventricular mass that has cells with scant cytoplasm and prominent nuclei that stains for
synaptophysin and neuron-specific enolase is a central neurocytoma. This can resemble an
oligodendroglioma, but those are rarely intraventricular and do not stain for synaptophysin and
neuron-specific enolase. Lymphoma and medulloblastoma can be periventricular or
intraventricular, but they do not stain for synaptophysin and neuron-specific enolase. Additionally,
they are much more hypercellular on H&E and are characterized by small round blue cells.
Choroid plexus papilloma is an intraventricular tumor, but the histologic appearance is different
than the image shown as there are prominent papillary processes in choroid plexus papillomas.
References:
Louis DN, Reifenberger G, Brat DJ, Ellison DW. Tumors: introduction and neuro-epithelial tumors.
In: Love S, Louis DN, Ellison DW, eds. Greenfield’s Neuropathology. 8th ed. London: Edward
Arnold Ltd; 2008: 1821-2000.
Karthigeyan, Madhivanan, Kirti Gupta, and Pravin Salunke. “Pediatric central neurocytoma: a short
series with literature review.” Journal of Child Neurology 32.1 (2017): 53-59.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/27664193/
A 79-year-old man presents with progressive low back pain. He is noted to have no focal neurological deficits, but he does exhibit focal point tenderness over the T12 region. Serum calcium level is 14 mg/dL. A post-contrast T1-weighted sagittal MR image of the lumbar spine is shown (Figure 1). The low-power H & E stain of a CT-guided needle biopsy specimen is shown
(Figure 2). Which of the following is the most likely diagnosis?
Answers:
A. Multiple myeloma
B. Vertebral hemangioma
C. Chordoma
D. Prostate cancer metastasis
E. Myxopapilary ependymoma
Multiple myeloma
Discussion:
The correct answer is multiple myeloma. Laboratory abnormalities seen in multiple myeloma
include elevations in serum calcium, creatinine, free light chains, B2 microglobulins and protein
electrophoresis levels. Histologically, multiple myeloma is characterized by clusters, nodules, or
sheets of monomorphic blue cells with clock face nuclei and abundant cytoplasm with a
perinuclear halo.
Chordomas are expansile, lobulated, intraosseous masses that demonstrate infiltrative borders
with epithelioid cells demonstrating abundant, clear to eosinophilic cytoplasm that gives it a soap
bubble appearance. In the spine, they often occur in the sacrum, are not multifocal, and
histologically are very different than the image shown. Vertebral hemangiomas with aggressive
behavior are best diagnosed with MRI; they present with low signal intensity on T1-weighted and
high signal intensity on T2-weighted MRI. Non-contrast CT scan images can show vertebral body
with trabecular thickening and intervening decreased density giving the characteristic “polka dot,”
“salt and pepper,” or “corduroy cloth/jail bar” appearance, with a decrease in the overall density of
the vertebral body due to the presence of fatty marrow. Myxopapillary ependymomas are
characterized by well-differentiated cuboidal tumor cells radially oriented around vascularized
myxoid cores and are intradural lesions that do not involve the bone. While prostate cancer does
metastasize to the bone and cause blastic lesions, this would be associated with elevated prostate
specific antigen (PSA) levels and none of the levels mentioned above.
References:
Sukpanichnant, S. et al. Diagnostic criteria and histologic grading in multiple myeloma: Histologic
and immunohistologic analysis of 176 cases with clinical correlation. Hum Pathol 25, 308–318
(1994).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/8150462/
Cowan, A. J. et al. Diagnosis and Management of Multiple Myeloma. Jama 327, 464–477 (2022).
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/35103762/
A 55-year-old man presents with progressive pain in the upper back and right chest wall. Cardiac and pulmonary examinations show no abnormalities. MR imaging of a mass in the right chest is shown in Figures 1-6. Resection is performed via a right far lateral extra-cavitary operative approach. Based on the MR images and the histopathology shown in Figure 7, which of the
following is the most likely diagnosis?
Answers:
A. Spinal metastasis
B. Chordoma
C. Osteosarcoma
D. Vertebral hemangioma
E. Giant cell bone tumor
Vertebral hemangioma
Discussion:
The correct answer is Vertebral hemangioma. Vertebral hemangiomas are benign vascular lesions
of the spine. They are the most common benign tumors of the spine; autopsy studies report
hemangiomas of the axial skeletons in 10% to 12% of cases. They are typically found incidentally
during investigations for other spinal pathology. Symptomatic vertebral hemangiomas are rare and
often present with back pain with or without radicular pain.
Vertebral hemangiomas with aggressive behavior are best diagnosed with MRI; they present with
low signal intensity on T1-weighted and high signal intensity on T2-weighted MRI. Non-contrast CT
scan images can show vertebral body with trabecular thickening and intervening decreased
density giving the characteristic “polka dot,” “salt and pepper,” or “corduroy cloth/jail bar”
appearance, with a decrease in the overall density of the vertebral body due to the presence of
fatty marrow. Treatment of symptomatic lesions depends on location and symptomatology, but
often consists of a combination of endovascular embolization, surgical resection, and/or
radiotherapy.
None of the other options listed demonstrate the CT imaging characteristics described above.
Osteosarcomas typically are invasive and destructive intraosseous masses with cortical
permeation and adjacent soft tissue invasion with significant cellular atypia on microscopy. Spinal
metastases can demonstrate lytic or blastic appearances on MR depending on their site of origin.
Giant cell bone tumors are locally aggressive bone tumors located at the end of long bones and
very rarely encountered within the spine. They demonstrate eccentrically located, osteolytic lesions
with well-defined, non-sclerotic margins with enhancement of the solid component. Pathologically,
they are characterized by osteoclastic giant cells with a variable number of nuclei, areas of
hemorrhage, foamy macrophages, and cortical bone erosion or destruction. Chordomas are
expansile, lobulated, intraosseous masses that demonstrate infiltrative borders with epithelioid
cells demonstrating abundant, clear to eosinophilic cytoplasm that gives it a soap bubble
appearance.
References:
Schrock WB, Wetzel RJ, Tanner SC, Khan MA. Aggressive hemangioma of the thoracic spine.
Journal of radiology case reports. 2011;5(10):7.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/22470764/
Qiu B, Joo P, Ajabnoor R, Boyce B, Mesfin A. Surgical management of aggressive hemangiomas
of the spine. Journal of Clinical Neuroscience. 2020 Aug 1;78:67-72.
Pubmed Web link: https://pubmed.ncbi.nlm.nih.gov/32620473/
A 24-year-old man presents with headaches. MR imaging shows a nonenhancing, focally calcified lesion with mild edema centered at the cortical gray-white junction in the right frontal cortex. A histologic section with H & E staining is shown. Which of the following is the most likely diagnosis?
Answers:
A. Atypical teratoid/rhabdoid tumor
B. Pilocytic astrocytoma
C. Meningioma
D. Oligodendroglioma
E. Dysembryoplastic neuroepithelial tumor (DNET)
Oligodendroglioma
Discussion:
The correct answer is oligodendroglioma. All of the answers listed are parenchymal tumors that
can be associated with intratumoral calcifications. Of the answers provided, the only one that
demonstrates perinuclear halos (fried egg appearance) as seen on the H&E stain is
oligodendroglioma. Pilocytic astrocytomas are characterized by compact fibrillary areas, Rosenthal
fibers, a biphasic appearance, and eosinophilic granular bodies. Atypical teratoid/rhabdoid tumors
are characterized by an abundance of small, round blue cells. Dysembryoplastic neuroepithelial
tumors demonstrate glioneural elements with intervening floating axons, scattered astrocytes, and
rare mitotic figures. Meningiomas have a variety of histologic subtypes with each demonstrating
different findings on H&E; however, none of them would have the perinuclear halos shown above.
References:
Jenkinson MD, du Plessis DG, Smith TS, Joyce KA, Warnke PC, Walker C. Histological growth
patterns and genotype in oligodendroglial tumours: correlation with MRI features. Brain. 2006
Jul;129(Pt 7):1884-91. doi: 10.1093/brain/awl108. Epub 2006 May 2. PMID: 16670176.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/16670176/
Wesseling P, van den Bent M, Perry A. Oligodendroglioma: pathology, molecular mechanisms and
markers. Acta Neuropathol. 2015 Jun;129(6):809-27. doi: 10.1007/s00401-015-1424-1. Epub 2015
May 6. PMID: 25943885; PMCID: PMC4436696.
Pubmed Link: https://pubmed.ncbi.nlm.nih.gov/25943885
