Neuropathology Flashcards
- The organism most frequently identified in brain abscesses is
A. Bacteroides
B. Candida
C. Citrobacter
D. Microaerophilic Streptococcus
E. Staphylococcus
A. Bacteroides
B. Candida
C. Citrobacter
**D. Microaerophilic Streptococcus
**E. Staphylococcus
While brain abscesses tend to consist of mixed ora, microaerophilic and
anaerobic st reptococci are the most frequently identi ed organisms in brain
abscesses.1,2
- Mees’ transverse white lines on fingernails
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
Arsenic toxicity (A) can be caused by insecticides. Chronic exposure to arsenic
causes malaise, hyperkeratosis, and pigmentation of the palms and soles, as
well as Mees’ transverse white lines in the ngernails. Arsenic toxicity is treated
with dimercaprol (BAL).
- Psychological dysfunction (“mad as a hatter”)
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
Mercury (C) can be found in contaminated sh and in felt
hat dyes. Mercury poisoning may cause psychological dysfunction (“mad as
a hat ter”) as well as cerebellar signs and renal tubular necrosis. Penicillamine
is the treatment of choice for mercury toxicity;
- Parkinson’s symptoms
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
Mercury (C) can be found in contaminated sh and in felt
hat dyes. Mercury poisoning may cause psychological dysfunction (“mad as
a hat ter”) as well as cerebellar signs and renal tubular necrosis. Penicillamine
is the treatment of choice for mercury toxicity;
- Red blood cell basophilic stippling
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
- Brain levels increased by dimercaprol (BAL)
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
Mercury can be found incontaminated shand in felt hat dyes. Mercury poisoning may cause psychological dysfunction (“mad as a hatter”) as well as cerebellar signs and renal tubular necrosis. Penicillamine is the treatment of choice for mercury toxicity; BALincreases brain levels of mercury and should be avoided.
- Symptoms improve with L-dopa
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
Manganese toxicity primarily a ects miners and is characterized by Parkinson’s-type symptoms. Neuronal loss is observed in the basal ganglia, and symptoms generally respond to L-dopa.3
- Increased urine coproporphyrin
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
causes encephalitis in children, but in adults causes a demyelinating motor polyneuropathy and anemia. Lead toxicity leads to basophilic stippling of the erythrocytes and increases excre- tion of urinary coproporphyrin. Lead toxicity can be treated with EDTA, BAL, andpenicillamine.
- Both penicillamine and BAL are used in treatment
A. Arsenic
B. Lead
C. Mercury
D. Manganese
A. Arsenic
B. Lead
C. Mercury
D. Manganese
Intranuclear
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
- Core composed of a protein
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
- Contains paired helical laments
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
- Immunoreactive for t protein
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
- Revealed with silver stains
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
A. Neuro brillary tangles
B. Neuritic plaques
C. Both
D. Neither
Neuro brillary tangles (A) and neuritic plaques (B) are both intracytoplas- mic; both contain paired helical laments and are revealed with silver stains. The central core of the neuritic plaque (B) is composed of b/A4, not a protein. The neuro brillary tangles (A) are immunoreactive for t protein.
Most meningiomas express immunoreactivity for
A. Cytokeratin
B. Desmin
C. Glial brillary acidic protein (GFAP)
D. S-100 protein
E. Vimentin
A. Cytokeratin
B. Desmin
C. Glial brillary acidic protein (GFAP)
D. S-100 protein
E. Vimentin
Vimentin (E) is an intermediate lament protein and is usually expressed by
meningiomas. Vimentin (E) expression is not terribly useful in meningioma
diagnosis, as the histopathologic di erential diagnostic considerations include
many other tumors that may also be vimentin positive such as carcinomas (positive
for cytokeratins [A]), melanomas (positive for myelin A, HMB45, and S-100
[D]), gliomas (positive for S-100 [D]), and schwannomas (positive for S-100 [D]).
Epithelial membrane antigen (EMA) is also expressed by the majority of meningiomas
and is a re ection of their epithelial character. Metastatic carcinomas
may also express EMA; however, EMA positivity helps to rule out schwannomas,
melanomas, and hemangioblastomas. GFAP (C) staining is generally negative for
meningiomas but has been reported in papillary meningiomas.
Each of the following is true of gangliogliomas except
A. The astrocytes are GFAP positive
B. The ganglion cells are synaptophysin positive
C. They contain neuropeptides
D. They are usually di usely infiltrative
E. They are most common in the temporal lobes
A. The astrocytes are GFAP positive
B. The ganglion cells are synaptophysin positive
C. They contain neuropeptides
D. They are usually di usely infiltrative
E. They are most common in the temporal lobes
Gangliogliomas are usually well circumscribed and can be part ially cystic
(D is false). The other responses regarding gangliogliomas are t rue: the
astrocytes are GFAP posit ive (A), the ganglion cells are synaptophysin positive
(B), they contain neuropeptides (C), and they most commonly occur in the
temporal lobes (E).1,2
Which of the following is not associated with trisomy 13?
A. Holoprosencephaly
B. Hypertelorism
C. Microcephaly
D. Microphthalmia
E. Polydactyly
A. Holoprosencephaly
B. Hypertelorism
C. Microcephaly
D. Microphthalmia
E. Polydactyly
Trisomy 13, Patauʼs syndrome, is associated with hypotelorism, holoprosen- cephaly (A), microcephaly (C), microphthalmia (D), cleft palate, polydac- tyly (E), dextrocardia, and ocular abnormalities. Patients typically survive no more than 9 months. Hypotelorism, not hypertelorism (B), is associated with trisomy 13.
Which of the following is not characteristic of ependymomas?
A. Blepharoplasts in the basal cytoplasm
B. Intermediate filaments that are immunohistochemically identical to glial
laments of astrocytes
C. Perivascular pseudorosettes
D. Surface microvilli
E. True rosette formation
A. Blepharoplasts in the basal cytoplasm
B. Intermediate filaments that are immunohistochemically identical to glial
laments of astrocytes
C. Perivascular pseudorosettes
D. Surface microvilli
E. True rosette formation
Ependymomas are CNS neoplasms that resemble the structure of the brain’s ependyma. The most de nitive evidence of ependymoma is the presence of true rosettes (E), also called “Flexner-Wintersteiner rosettes.” Most ep- endymomas contain perivascular pseudorosettes (C) involving tumor cells surrounding an endothelial-lined lumen. Ependymomas tend to stain for GFAP and vimentin (B), particularly in the perivascular pseudorosettes. On electron microscopy, extensive surface microvilli (D) forming both intra- and extracellular lumens can be seen. Blepharoplasts (ciliary basal bodies) are found in the apical, not basal, cytoplasm (A is false).
For questions 19 to 28, match the vitamin with the description of its de ciency or toxicity. Each response may be used once, more than once, or not at all.
Wernicke’s encephalopathy?
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Korsako ’s psychosis
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Pellagra
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Beriberi
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Seen in rice eaters
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Seen in corn eaters
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Rickets
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Pernicious anemia
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Subacute combined degeneration
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Pseudotumor
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
A. Thiamine
B. Niacin
C. VitaminB12
D. VitaminA
E. VitaminD
Diets heavy in corn lack t ryptophan that is used to synthesize niacin (B);
niacin de ciency causes pellagra—dermat it is, diarrhea, and dementia. Diets
heavy in re ned rice are more likely to lack su cient thiamine (A). Thiamine
de ciency is associated with Wernicke’s encephalopathy and Korsako ’s psychosis,
as seen in chronic alcoholism, and is also associated with beriberi—
characterized by peripheral polyneuropathy, demyelinat ion, and autonomic
dysfunction. Vitamin A (D) toxicit y m ay cause cerebral edema w ith a pseudotumor
presentation. Pernicious anemia can lead to a vitamin B12 (C) de ciency
with megaloblast ic anemia and subacute combined degenerat ion of the spinal
cord. Vitamin D de ciency (E) causes rickets, which is associated with
decreased parathyroid hormone and brit tle bones.3
Which of the following is true of lymphomas (non-Hodgkin’s malignant lymphomas) of the central nervous system (CNS)?
A. All exhibit a di use histologic pattern.
B. Meningeal lesions are more common in primary lymphomas.
C. Most are of T cell lineage.
D. Parenchymal lesions are more common in secondary lymphomas.
E. They are radioresistant.
A. All exhibit a di use histologic pattern.
B. Meningeal lesions are more common in primary lymphomas.
C. Most are of T cell lineage.
D. Parenchymal lesions are more common in secondary lymphomas.
E. They are radioresistant.
Meningeal in ltration is the most common lesion in secondary lymphomas (B is false), and parenchymal lesions are the most common lesion in primary lymphomas (D is false). Most are of B cell lineage and are radiosensitive (Cand E are false). Nodular lymphomas are not seen in the central nervous system (CNS); all show a di use histology (A is true).
Which of the following is not seen in Sturge-Weber syndrome?
A. Cortical arteriovenous malformations
B. Facialnevus
C. Intracortical calci cation
D. Meningeal angioma
E. Seizures
A. Cortical arteriovenous malformations
B. Facialnevus
C. Intracortical calci cation
D. Meningeal angioma
E. Seizures
Sturge-Weber syndrome is characterized by a usually unilateral port-wine nevus (B) that typically involves the orbit or upper eyelid, unilateral menin- geal angioma (D), calci cations con ned to the second and third layers of c e r e b r a l c o r t e x ( C) , a n d s e i z u r e a c t i v i t y ( E ) . T h e a b n o r m a l m e n i n g e a l v e s s e l s are typically veins and are not well-visualized on angiography—a feature that is not consistent with arteriovenous malformations. Arteriovenous malfor- mations (AVMs) are not characteristic of the Sturge-Weber syndrome (A).
Each of the following is true of the cord pathology in pernicious anemia except
A. Demyelination occurs
B. Lumbar levels are most severely affected
C. Lesions may occur in the medulla
D. Vacuolar distention of myelin sheaths occurs
E. Wallerian degeneration occurs
A. Demyelination occurs
B. Lumbar levels are most severely affected
C. Lesions may occur in the medulla
D. Vacuolar distention of myelin sheaths occurs
E. Wallerian degeneration occurs
The demyelination (A), spongiosis (D), and gliosis seen in vitamin B12
de ciency are most common at lower cervical and thoracic levels (Bis false)
Which of the following is associated with progressive multi focal encephalopathy?
A. Bacterial infection
B. Demyelination
C. Increased numbers of oligodendroglial cells
D. Intense in ammatory in ltrate
E. Shrunken oligodendroglial nuclei at the periphery of the lesion
A. Bacterial infection
B. Demyelination
C. Increased numbers of oligodendroglial cells
D. Intense in ammatory in ltrate
E. Shrunken oligodendroglial nuclei at the periphery of the lesion
Progressive multifocal leukoencephalopathy is caused by a papovavirus (no- tably the JCvirus; A is false). Lesions occur mainly in the white matter and consistoffociofmyelinandoligodendroglialcelllosswithminimalin amma- tory in ltrate (Cand D are false). Hyperchromatic enlarged oligodendroglial nuclei are found at the margin of the lesions (E is false). Demyelination is present (B); some early cases were thought to represent atypical MS.1
Which of the following is associated with von Hippel-Lindau disease?
I. Hepatic cysts
II. Hemangioblastoma of the spinal cord
III. Renal cysts
IV. Renal cell carcinoma
A. I, II, III
B. I, III
C. II, IV
D. IV
E. Alloftheabove
A. I, II, III
B. I, III
C. II, IV
D. IV
E. Alloftheabove
v o n H i p p e l - L i n d a u ( V H L) d i s e a s e i s a n a u t o s o m a l d o m i n a n t d i s o r d e r l i n k e d t o t h e VHLgene on chromosome 3—a tumor suppressor gene. The disease is associated with hemangioblastomas of the brain and spinal cord (II), retinal hemangio- blastomas, renal cell carcinomas and renal cysts (III, IV), pheochromocytoma, pancreatic tumors and cysts, hepatic cysts (I), and polycythemia vera
For questions 34 to 38, match the tumor with the description. Each response may be used once, more than once, or not at all.
Antoni A areas ?
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
Schwannomas (B) are characterized by a biphasic cellular pattern composed of compact spindle cells (Antoni Aareas) and loosely arranged stellate cells (An- toni Bareas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear brillar material.
Antoni B areas
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
Schwannomas (B) are characterized by a biphasic cellular pattern composed of compact spindle cells (Antoni Aareas) and loosely arranged stellate cells (An- toni Bareas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear brillar material.
Verocay bodies
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
Schwannomas (B) are characterized by a biphasic cellular pattern composed of compact spindle cells (Antoni Aareas) and loosely arranged stellate cells (An- toni Bareas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear brillar material.
Axons are present between tumor cells
A. Neuro fibroma
B. Schwannoma
C. Both
D. Neither
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
Neuro bromas (A) incorporate the parent nerve and hence have axons in their midst. The plexiform type is considered pathognomonic for neuro bromatosis type 1
The plexiform type is strongly associated with neuro bromatosis type 1
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
A. Neurofibroma
B. Schwannoma
C. Both
D. Neither
Neuro bromas (A) incorporate the parent nerve and hence have axons in their midst. The plexiform type is considered pathognomonic for neuro bromatosis type 1
Which one of the following cerebral metastases has the greatest tendency to hemorrhage?
A. Breast
B. Choriocarcinoma
C. Gastrointestinal (GI) tract
D. Ovarian
E. Prostate
A. Breast
B. Choriocarcinoma
C. Gastrointestinal (GI) tract
D. Ovarian
E. Prostate
Of these choices, choriocarcinoma (B) has the greatest tendency to hem- orrhage. Hemorrhage is also common in melanoma, renal cell carcinoma, colorectal carcinoma (C), and lung carcinoma. Cancers of breast origin (A) are unlikely to hemorrhage.
For questions 40 to 44, match the time period after a cerebral infarct with the histologic appearance. Each response may be used once, more than once, or not at all.
Lipid-laden macrophages first appear ?
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
Macrophages begin to arrive on day 3; by day 5–7 (C)
Fibrillary astrocytes present at the periphery of the lesion
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
gem- istocytic astrocytes begin to appear at the periphery of the lesion, and en- hancement begins to occur on contrasted images. Fibrillary astrocytes do not appear at the periphery of the lesion for more than 3 months (E)
Gemistocytic astrocytes present at the periphery of the lesion
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
lipid-laden macrophages become apparent.Between days 10 and 20 (D)
Polymorphonuclear infiltrate
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
Polymorphonuclear (PMN) leu- kocytes begin to accumulate 24 hours after the insult and PMN accumulation peaks at 48 hours (B)
Neuronal necrosis is first apparent
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
A. 12–24 hours
B. Days 1–2
C. Days 5–7
D. Days 10–20
E. More than 3 months
Irreversible ischemic injury is evident at the cellular level within 6 hours with microvacuolization of the cells and cytoplasmic bulging. Neuronal necrosis becomes apparent within 12–24 hours (A)
Hepatic failure is most closely associated with
A. Endothelial proliferation
B. Gliosis localized to the globus pallidus and hippocampus
C. Gliosis localized to the white matter
D. Alzheimer’stypeIIastrocytes
E. Loss of oligodendroglial cells
A. Endothelial proliferation
B. Gliosis localized to the globus pallidus and hippocampus
C. Gliosis localized to the white matter
D. Alzheimer’stypeIIastrocytes
E. Loss of oligodendroglial cells
Acquired hepatocerebral degeneration is associated with gliosis with a predi- lection for the cortex (C is false). It tends to spare the hippocampus, globus pallidus, and deep folia of the cerebellar cortex (B is false). Widespread hy- perplasia of protoplasmic astrocytes (Alzheimer’s type II astrocytes) is visible in the deep layers of the cerebral and cerebellar cortex and in deep nuclear structures (D)
Each of the following has been associated with central pontine myelinolysis except
A. Alcoholism
B. Severe burns
C. Rapid correction of hyponatremia
D. Serum hyperosmolarity
E. VitaminAexcess
A. Alcoholism
B. Severe burns
C. Rapid correction of hyponatremia
D. Serum hyperosmolarity
E. VitaminAexcess
“The outstanding characteristic of CPM is its invariable association with some other serious, often life threatening disease.” Central pontine myelinolysis is an acute demyelinating condition of the brainstem that has been attributed to rapid correction of hyponatremia. The disorder has been associated with alcoholism (A), severe burns (B), and serum hyperosmolarity (D). The common pathway of all of these disease processes seems to involve either the rapid correction of hyponatremia (C) or severe acute hyperosmolarity (D) (as in burn victims). Vitamin A excess has not been associated with central pontine myelinolysis (E is false)
Rosenthal fibers are associated with
I. Astrocytosis
II. Alexander’s disease
III. Pilocytic astrocytoma
IV. Pick’s disease
A. I, II, III
B. I, III
C. II, IV
D. IV
E. Alloftheabove
A. I, II, III
B. I, III
C. II, IV
D. IV
E. Alloftheabove
Rosenthal bers, eosinophilic masses observed in astrocytic processes, are associated with pilocytic astrocytomas (III, neoplastic), astrocytosis (I), and Alexander’s disease (II, nonneoplastic). Pick’s disease (IV) is associated with Pick bodies, which are round, intracytoplasmic eosinophilic inclusions that are positive with silver stains and with antibodies to tau
Which of the following is not typically seen in neurofibromatosis type 2?
A. Acousticneuromas
B. Café-au-lait spots
C. Cutaneous neuro bromatosis
D. Lisch nodules
E. Plexiform neuro bromas
A. Acousticneuromas
B. Café-au-lait spots
C. Cutaneous neuro bromatosis
D. Lisch nodules
E. Plexiform neuro bromas
Neuro bromas (E) and café-au-lait spots (B) occur less commonly in neuro- bromatosis type 2 (NF-2) than in NF-1. Bilateral acoustic neuromas (A) are
the hallmark of NF-2. Lisch nodules (D) are rare in NF-2.
Which of the following is not associated with hepatic encephalopathy?
A. Thiamine de ciency
B. Asterixis
C. Alzheimer’stypeIIastrocytes
D. Increased serum ammonia
A. Thiamine de ciency
B. Asterixis
C. Alzheimer’stypeIIastrocytes
D. Increased serum ammonia
Asterixis (B) can appear in a variety of metabolic encephalopathies but is most common in hepatic encephalopathy. The serum ammonia (D) level usually exceeds 200 mg/dL. The most striking neuropathologic nding in pa- tients who die in a state of hepatic encephalopathy is the presence of a large amount of large protoplasmic astrocytes with glycogen-containing inclu- sions. These Alzheimerʼs type II astrocytes (C) can be found throughout the deep cerebral cortex, lenticular nuclei, thalamus, substantia nigra, cerebellar cortex, red, dentate, and pontine nuclei. Thiamine de ciency is not associated with hepatic encephalopathy
In amyotrophic lateral sclerosis, the cranial nerve nucleus that typically does not exhibit cell loss is
A. III
B. V
C. VII
D. IX
E. XII
A. III
B. V
C. VII
D. IX
E. XII
The motor nuclei of cranial nerves V (B), VII (C), IX (D), and XII (E) as well as the motor cortex may be a ected.
Which of the following vascular malformations have no intervening brain paren- chyma between blood vessels?
A. Arteriovenous malformations
B. Capillary telangiectasias
C. Cavernous malformations
D. Cryptic arteriovenous malformations
E. Venous angiomas
A. Arteriovenous malformations
B. Capillary telangiectasias
C. Cavernous malformations
D. Cryptic arteriovenous malformations
E. Venous angiomas
Cavernous malformations (cavernous hemangiomas [C]) are composed of large, thin-walled vessels without interposed brain parenchyma. Typically AVMs (Aand D) traverse disordered brain tissue that lies between the abnor- mal vessels. Capillary telangiectasias (B) contain intervening brain paren- chyma; so do developmental venous anomalies (venous angiomas [E]
For questions 52 to 57, match the sites of damage in the axonal transport apparatus with the toxin. Each response may be used once, more than once, or not at all.
Diabetes ??
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
in particular. End-organ glycosylation may disrupt turn- around transport (E), as seen in diabetes.
Vincristine
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
This question focuses on causes of toxic neuropathies. Both vincristine and v i n b l a s t i n e i n t e r f e r e w i t h m i c r o t u b u l e ( A) f u n c t i o n , a l t h o u g h t h r o u g h s l i g h t l y di erent mechanisms.
Mercury
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
Mercury inactivates sulfhydryl groups of enzymes interfering with cellular metabolism and function— translation (D),
Actinomycin D
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
Actinomycin D is an antibiotic produced by streptomyces that is used in cancer therapy. Its phenoxazone ring intercolates with DNA and interferes with DNA transcription (C).
Dinitrophenol
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
Dinitrophenol is thought to disrupt oxidative phos- phorylation (B).
Vinblastine
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
A. Microtubules
B. Oxidative phosphorylation
C. Transcription
D. Translation
E. Turnaround transport
This quest ion focuses on causes of toxic neuropathies. Both vincristine and
vinblastine interfere with microtubule (A) funct ion, although through slightly
di erent mechanisms. Dinit rophenol is thought to disrupt oxidative phosphorylation
(B). Act inomycin D is an antibiotic produced by streptomyces
that is used in cancer therapy. Its phenoxazone ring intercolates with DNA
and interferes with DNA transcription (C). Mercury inact ivates sulfhydryl
groups of enzymes interfering with cellular metabolism and function—
translation (D), in part icular. End-organ glycosylation may disrupt turnaround
transport (E), as seen in diabetes
Catecholamine production can occur in which of the following tumors?
A. Choriocarcinomas
B. Glomus jugulare tumors
C. Oligodendrogliomas
D. Pineocytomas
E. Pleomorphic xanthoastrocytomas
A. Choriocarcinomas
B. Glomus jugulare tumors
C. Oligodendrogliomas
D. Pineocytomas
E. Pleomorphic xanthoastrocytomas
Glomus jugulare tumors (B) originate from foci of paraganglionic tissue around the jugular bulb (they are paragangliomas of the glomus jugulare). These invasive tumors contain neurosecretory granules similar to those in the carotid body. Some of them produce clinically detectable amounts of catecholamine. The most common paraganglioma is that of the adrenal gland and goes by another name: pheochromocytoma. None of the other options listed are known to secrete catecholamines.
The viral inclusions seen in herpes simplex encephalitis are
A. Basophilic
B. CalledCowdrytypeBbodies
C. Found in neurons only
D. Intranuclear
E. Only evident several weeks after the infection
A. Basophilic
B. CalledCowdrytypeBbodies
C. Found in neurons only
D. Intranuclear
E. Only evident several weeks after the infection
The viral inclusions of herpes simplex type 1 (Cowdry type A) are dense, in- tranuclear, eosinophilic bodies found in neurons, astrocytes, and oligoden- drocytes. They are more likely to be found early in the course of the disease
High levels of a-fetoprotein are associated with
A. Endodermal sinus tumors
B. Choriocarcinomas
C. Germinomas
D. Pineoblastomas
E. Teratomas
A. Endodermal sinus tumors
B. Choriocarcinomas
C. Germinomas
D. Pineoblastomas
E. Teratomas
High levels of human chorionic gonadotrophin (HCG) are associated with choriocarcinomas (B), and high levels of a-fetoprotein (AFP) are associated with endodermal sinus tumors (yolk sac tumors [A]). Fifteen percent of germinomas (C) may be associated with increased HCG. Embryonal carcino- mas will show elevations in both AFP and HCG. Teratomas (E) may cause a rise in serum CEAlevels
The most common sites of hypertensive hemorrhage, in decreasing order of fre- quency, are
A. Lobar, putamen, cerebellum, thalamus, pons
B. Putamen, lobar, thalamus, cerebellum, pons
C. Putamen, thalamus, pons, lobar, cerebellum
D. Thalamus, cerebellum, lobar, putamen, pons
E. Thalamus, lobar, putamen, cerebellum, pons
A. Lobar, putamen, cerebellum, thalamus, pons
B. Putamen, lobar, thalamus, cerebellum, pons
C. Putamen, thalamus, pons, lobar, cerebellum
D. Thalamus, cerebellum, lobar, putamen, pons
E. Thalamus, lobar, putamen, cerebellum, pons
The most common sites of hypertensive cerebral hemorrhage are (1) puta- men and internal capsule (50%); (2) lobar hemorrhages of the central white matter of the temporal, parietal, or frontal lobes; (3) thalamus; (4) cerebellar hemisphere; and (5) pons
For questions 62 to 65, match the source of the metastatic brain lesion to the descrip- tion. Each response may be used once, more than once, or not at all.
most common ?
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
Lung (C) metastasis is the most common intracranial metastatic tumor.
Greatest tendency to hemorrhage
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
Choriocarcinoma (B) has the greatest propensity to hemorrhage.
Meningeal involvement is most common.
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
Secondary (metastatic) CNS lymphoma (D) tends to involve the meninges, while primary CNS lymphoma tends to involve the parenchyma. Of the options listed,
Least propensity to involve the brain
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
A. Breast
B. Choriocarcinoma
C. Lung
D. Lymphoma
E. Prostate
prostate (E) has the lowest propensity to metastasize to brain.
for questions 66 to 69, match the mechanism of action to the disease. Each response may be used once, more than once, or not at all.
Botulism?
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
Both botulism and Eaton-Lambert syndrome cause presynaptic inhibition at the neuromuscular junction (A)
Myasthenia gravis
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
Myasthenia gravis is caused by antibodies to acetycholine receptors located on the postsynaptic end-plate (C).
Eaton-Lambert syndrome
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
Tetanus
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
A. Presynaptic inhibition at the neuromuscular junction
B. Inhibition of Renshaw cells
C. Postsynaptic inhibition
albeit via di erent mechanisms. Botuli- num toxin prevents binding of synaptic vesicles to the presynaptic membrane inhibiting acetylcholine release. Eaton-Lambert syndrome is caused by anti- bodies directed against voltage-gated calcium channels located at the presyn- aptic terminal; interference with these voltage-gated Ca21 channels causes decreased release of ACh quanta, as synaptic vesicle binding is a calcium- dependent process. Tetanus toxin causes excitation of agonist and antago- nist muscles by inhibiting the release of glycine from Renshaw cells (B) (similar to strychnine poisoning)
Tuberous sclerosis is most closely associated with
A. Acousticneuromas
B. Cortical calci cation
C. Giant-cell astrocytomas
D. Opticgliomas
E. Renalcysts
A. Acousticneuromas
B. Cortical calci cation
C. Giant-cell astrocytomas
D. Opticgliomas
E. Renalcysts
Tuberous sclerosis is an autosomal dominant condition localized to chromo- som es 9 and 16 that is associated w ith a classic triad of m ental retardation, sei- zures, and adenoma sebaceum. Subependymal giant-cell astrocytomas (C) are present in 15%of cases. Acoustic neuromas (A) are associated with NF-2. Cortical calci cations (B) are associated with Sturge-Weber syndrome.Optic gliomas (D) are associated with NF-1.Renal cysts (E) are associated with VHL disease
For questions 106 to 162,match the gure with the most appropriate response.
A. Rarely multiple
B. Associated with immunosuppression in older men
C. Associated with immunosuppression in younger men
D. Resistant to steroids
A. Rarely multiple
B. Associated with immunosuppression in older men
C. Associated with immunosuppression in younger men
D. Resistant to steroids
This is lymphoma with di use perivascular lymphocytic in ltration into the Virchow-Robin space around a blood vessel. Such lymphomas are often multiple (A is false), respond initially to steroids (D is false), but invariably recur, and are associated with immunosuppression in younger men (C). They also occur in immunocompetent males over 60 years
A. Hirano bodies
B. Inclusion bodies of herpes simplex virus-1 (HSV-1)
C. Lewybodies
D. Pick bodies
E. Rabies
A. Hirano bodies
B. Inclusion bodies of herpes simplex virus-1 (HSV-1)
C. Lewybodies
D. Pick bodies
E. Rabies
This hematoxylin and eosin H&E-stained section represents HSV-1 encepha- litis (B) and shows lymphocytic perivascular cu ng on the main slide. The inset at the top right of the image shows a Cowdry type A body, the eosino- philic intranuclear inclusion with a surrounding halo typical for HSV-1
A. Hirano bodies
B. Inclusion bodies of herpes simplex virus-1 (HSV-1)
C. Lewybodies
D. Pick bodies
E. Rabies
A. Hirano bodies
B. Inclusion bodies of subacute sclerosing panencephalitis (SSPE)
C. Lewybodies
D. Pick bodies
E. Rabies
This H&E-stained section is an example of subacute sclerosing panencepha- litis (B) as is seen sometimes following measles infection (1 in 1,000 cases). The inclusion bodies of HSV (dense, eosinophilic, and surrounded by a clear halo) and SSPE are intranuclear. The inclusion bodies seen in the other responses are intracytoplasmic. Smaller eosinophilic intracytoplasmic inclu- sions may also be seen in SSPE
A. Inclusion bodies of HSV-1
B. Lewybodies
C. Pick bodies
D. Rabies
E. Inclusion bodies of SSPE
A. Inclusion bodies of HSV-1
B. Lewybodies
C. Pick bodies
D. Rabies
E. Inclusion bodies of SSPE
This silver stain shows an example of Pick bodies (C), rounded intracytoplas- micmasses.OnH&Estaining,itmightbedi culttodistinguishcorticalPick bodies from cortical Lewy bodies (B), both of which would appear as round eosinophilic intracytoplasmic inclusions. Lewy bodies of the brainstem and nucleus basalis typically have a halo,which can help with the distinction.Pick bodies are marked with silver stains (Lewy bodies are not). Pick bodies are immunopositive with anti-tau antibodies
A. Inclusion bodies of HSV-1
B. Lewybodies
C. Pick bodies
D. Rabies
E. Inclusion bodies of SSPE
A. Inclusion bodies of HSV-1
B. Lewybodies
C. Pick bodies
D. Rabies
E. Inclusion bodies of SSPE
Th i s i s a n H &E- s t a i n e d s e c t i o n s h o w i n g a n e x a m p l e o f a Le w y b o d y ( n e u r o n a l intracytoplasmic inclusion with an eosinophilic core surrounded by a clear halo [B]), which can be seen in the neurons of the substantia nigra in Parkinson’s disease patients
A. Ganglioglioma
B. Hepatic encephalopathy
C. H S V- 1
D. Parkinson’s disease
E. Normalcortex
A. Ganglioglioma
B. Hepatic encephalopathy
C. H S V- 1
D. Parkinson’s disease
E. Normalcortex
This H&E-stained section shows an example of Alzheimer’s type II astrocytes, large vesicular nuclei,and little visible cytoplasm.These reactive protoplasmic astrocytes are found in hepatic encephalopathy (B) and Wilson’s disease
A. Fibrillary astrocytoma
B. Gemistocytic astrocytoma
C. Glioblastoma multiforme
D. Hem angioblastom a
E. Oligodendroglioma
A. Fibrillary astrocytoma
B. Gemistocytic astrocytoma
C. Glioblastoma multiforme
D. Hem angioblastom a
E. Oligodendroglioma
This H&E-stained section shows an example of a gemistocytic astrocytoma (B). The cells of this variant of astrocytoma have prominent eosinophilic cyto- plasm,short processes,and eccentricnuclei(comprised ofgemistocyticastro- cytes). Gemistocytic astrocytes can be seen in the setting of reactive gliosis, but the crowding and overlapping of cells in this slide support the diagnosis of neoplasm. Gemistocytic astrocytomas tend to behave more aggressively than other WHO grade II astrocytomas; for this reason, gemistocytic astrocytomas are sometimes graded WHO grade III because of gemistocytic features
A. Aneurysmal subarachnoid hemorrhage
B. Bacterial meningitis
C. Contusion
D. H S V- 1
E. Subdural hematoma
A. Aneurysmal subarachnoid hemorrhage
B. Bacterial meningitis
C. Contusion
D. H S V- 1
E. Subdural hematoma
The hemorrhagic appearance of the medial temporal lobe is characteristic of HSV-1 (D)
A. AIDS encephalopathy
B. Giant-cell glioblastoma multiforme (GBM)
C. Hem angioblastom a
D. Creutzfeldt-Jakob disease
E. Progressive multifocal leukoencephalopathy
A. AIDS encephalopathy
B. Giant-cell glioblastoma multiforme (GBM)
C. Hem angioblastom a
D. Creutzfeldt-Jakob disease
E. Progressive multifocal leukoencephalopathy
This H&E-stained section is an example of progressive multifocal encepha- lopathy (E, associated with the JC virus and an immunocompromised state). Demyelination and oligodendroglial cell loss are seen. Residual oligo- dendroglial nuclei (arrows) are large and bizarre
A. AIDS encephalopathy
B. Giant-cell GBM
C. Hem angioblastom a
D. Creutzfeldt-Jakob disease
E. Progressive multifocal leukoencephalopathy
A. AIDS encephalopathy
B. Giant-cell GBM
C. Hem angioblastom a
D. Creutzfeldt-Jakob disease
E. Progressive multifocal leukoencephalopathy
This is a representative H&E-stained section from a patient with Creutzfeldt- Jakob disease (D), a prion disease causing vacuolization and spongiform changes
A. Metastatic disease
B. Neuro bromatosis type 1 (NF-1)
C. NF-2
D. Tuberous sclerosis
E. von Hippel-Lindau disease
A. Metastatic disease
B. Neuro bromatosis type 1 (NF-1)
C. NF-2
D. Tuberous sclerosis
E. von Hippel-Lindau disease
The iris hamartomas (Lisch nodules) of NF-1 (B) are seen in this photograph.
A. HIV encephalopathy
B. Giant-cell GBM
C. Hem angioblastom a
D. Creutzfeldt-Jakob disease
E. Progressive multifocal leukoencephalopathy
A. HIV encephalopathy
B. Giant-cell GBM
C. Hem angioblastom a
D. Creutzfeldt-Jakob disease
E. Progressive multifocal leukoencephalopathy
This Luxolfast blue H&E-stained section is an example ofHIVencephalopathy (A). Microglial nodules with foci of demyelination, neuronal loss, and reac- tive astrocytosis are typical. The characteristic multinucleated giant cell is seen here
A. Anaplastic astrocytoma
B. Ependymoma
C. Ganglioglioma
D. Meningioma
E. Oligodendroglioma
A. Anaplastic astrocytoma
B. Ependymoma
C. Ganglioglioma
D. Meningioma
E. Oligodendroglioma
This is an H&E-stained section from a ganglioglioma (C),which contains both neoplastic neurons and ganglion cells. The nding of abnormal ganglion cells (including binucleate forms) is key to the diagnosis of ganglioglioma
A. Anaplastic astrocytoma
B. Meningioma
C. Normal pituitary gland
D. Oligodendroglioma
E. Pituitary adenoma
A. Anaplastic astrocytoma
B. Meningioma
C. Normal pituitary gland
D. Oligodendroglioma
E. Pituitary adenoma
This is an H&E-stained section of an oligodendroglioma (D). 1p and 19q co-deletions in these tumors are associated with improved progression-free and overall survival. A characteristic “chicken wire” vascular pattern and a monotonous “fried egg” nuclear array are seen
A. Choroid plexus papilloma
B. Ependymoma
C. Medulloblastoma
D. Meningioma
E. Pituitary adenoma
A. Choroid plexus papilloma
B. Ependymoma
C. Medulloblastoma
D. Meningioma
E. Pituitary adenoma
This H&E-stained sections shows dense, hyperchromatic cells that are radially arranged in Homer-Wright rosettes with central granulo brillar material. These ndings are most consistent with a primitive neuroectodermal tumor (medulloblastoma [C]), neuroblastoma, etc.)
A. Choroid plexus papilloma
B. Craniopharyngioma
C. Hem angioblastom a
D. Metastatic tumor
E. Myxopapillary ependymoma
A. Choroid plexus papilloma
B. Craniopharyngioma
C. Hem angioblastom a
D. Metastatic tumor
E. Myxopapillary ependymoma
This H&E-stained section of a myxopapillary ependymoma (E) shows cohesive ependymal cells terminating around perivascular accumulations of mucinous material. Myxopapillary ependymomas tend to occur at the lum (conus medullaris
A. Choroid plexus papilloma
B. Craniopharyngioma
C. Hem angioblastom a
D. Metastatic tumor
E. Myxopapillary ependymoma
A. Choroid plexus papilloma
B. Craniopharyngioma
C. Hem angioblastom a
D. Metastatic tumor
E. Myxopapillary ependymoma
Question 122 shows an H&E-stained section from a craniopharyngioma (B) that demonstrates an adamantinomatous pattern with a basal layer of colum- nar cells separated by loosely arranged stellate cells. Palisading epithelial cells with keratinization and calci cation are prominent. The papillary variant is more often seen in adults and contains papillae of well-di erentiated squa- mous epithelium (not pictured here)
A. Choroid plexus papilloma
B. Craniopharyngioma
C. Hem angioblastom a
D. Metastatic tumor
E. Myxopapillary ependymoma
A. Choroid plexus papilloma
B. Craniopharyngioma
C. Hem angioblastom a
D. Metastatic tumor
E. Myxopapillary ependymoma
An H&E-stained section of hemangioblastoma (C) is pictured here. They are most commonly found in the posterior fossa, and 60%present as a cyst with a mural nodule. Multiple hemangioblastoma are associated with VHL. On H&E staining they are di cult to distinguish from renal cell carcinoma (particularly problematic in VHLpatients who are at risk for renal cell carci- noma also). The diagnostic distinction can be made by immunohistochemis- try. Vacuolated “stromal” cells in a complex capillary network are seen in this photomicrograph
A. Butter yglioma
B. Carbon monoxide poisoning
C. Fatemboli
D. Lipoma
E. Lipofuscin deposition
A. Butter yglioma
B. Carbon monoxide poisoning
C. Fatemboli
D. Lipoma
E. Lipofuscin deposition
This gross anatomical specimen shows an example of a lipoma (D) of the corpus callosum
A. Carbon monoxide poisoning
B. Fatemboli
C. Hallervorden-Spatz disease
D. Miliary tuberculosis
E. Wilson’s disease
A. Carbon monoxide poisoning
B. Fatemboli
C. Hallervorden-Spatz disease
D. Miliary tuberculosis
E. Wilson’s disease
This gross anatomic specimen shows selective necrosis of the globus pallidus, most consistent with carbon monoxide poisoning (A)
A. Ependymoma
B. Glioblastoma
C. Medulloblastoma D. Meningioma
E. Schwannoma
A. Ependymoma
B. Glioblastoma
C. Medulloblastoma D. Meningioma
E. Schwannoma
This is an H&E-stained section showing a section representative of glioblas- toma (B). Either microvascular proliferation or necrosis is required for an astrocytic tumor to qualify as glioblastoma (WHO grade IV). Necrosis with pseudopalisading is well illustrated in this photomicrograph
A. Metastatic disease
B. NF-1
C. NF-2
D. Tuberous sclerosis
E. von Hippel-Lindau disease
A. Metastatic disease
B. NF-1
C. NF-2
D. Tuberous sclerosis
E. von Hippel-Lindau disease
The adenoma sebaceum of tuberous sclerosis (D) is seen in this photograph. Tuberous sclerosis is an autosomal dominant condition linked to chromo- somes 9 and 16 characterized by the classic triad of adenoma sebaceum, seizures, and mental retardation. Patients with tuberous sclerosis are prone to develop cortical tubers, subependymal giant cell astrocytomas, cardiac rhabdomyoma, retinal hamartoma, and renal angiomyolipoma.
A. Anaplastic astrocytoma
B. Medulloblastoma
C. Meningioma
D. Metastatic tumor
E. Oligodendroglioma
A. Anaplastic astrocytoma
B. Medulloblastoma
C. Meningioma
D. Metastatic tumor
E. Oligodendroglioma
This H&E-stained photomicrograph is an example of a meningotheliomatous (syncytial) type of meningioma (C). Apsammoma body is noted as well
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
This H&E-stained section is another example of meningioma (C). Note the prominent whorls
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
This is an H&E-stained section of a neuro broma (D). Bundles of elongated Schwann cells with characteristic wavy nuclei in a loose mucinous or collag- enous matrix are features of the neuro broma
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
Malignant peripheral nerve sheath tumors (B) are composed of spindle cells in fascicles with occasional mitoses and foci of necrosis (H&E).
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
A. Glioblastoma
B. Malignant peripheral nerve sheath tumor
C. Meningioma
D. Neurofibroma
E. Schwannoma
This H&E-stained section shows Verocay bodies, palisading elongated nuclei en- circling anuclear brillary material, which are hallmarks of schwannomas (E).
A. Filum terminale
B. Kidney
C. Notochord
D. Pituitary
E. von Hippel-Lindau disease
A. Filum terminale
B. Kidney
C. Notochord
D. Pituitary
E. von Hippel-Lindau disease
This H&E-stained section shows “physaliphorous” or “bubbly” cells surround- ing pools of mucin, consistent with the diagnosis of chordoma. Chordomas are thought to arise from notochord (C) remnants, usually occurring in the clivus or sacrum.3
A. Alzheimer’s disease
B. H S V- 1
C. Huntington’s disease
D. Parkinson’s disease
E. Pickʼs disease
A. Alzheimer’s disease
B. H S V- 1
C. Huntington’s disease
D. Parkinson’s disease
E. Pickʼs disease
Huntington’s disease (C) is an autosomal dominant hereditary movement disorder that localizes to chromosome 4 and involves a CAG trinucleotide repeat. The clinical syndrome involves choreiform movements of the trunk and upper limbs with subcortical dementia. Atrophy of the head of the caudate with “boxcar”ventricles is characteristic and can be seen in this gross pathologic specimen.1,3
Double arrows correspond to
A. Bacterial meningitis
B. Candidiasis
C. Neuritic plaques
D. Neuro brillary tangles
E. Pick bodies
A. Bacterial meningitis
B. Candidiasis
C. Neuritic plaques
D. Neuro brillary tangles
E. Pick bodies
Neuritic (“senile”) plaques (composed of degenerating nerve cell processes surrounding a central core of amyloid composed of b/A4 protein, double ar- rows [C]) and neuro brillary tangles (single arrows [D]) are seen (silver stain).3
A. Acute disseminated encephalomyelitis
B. Adrenoleukodystrophy
C. Alexander’s disease
D. Krabbe’s disease
E. Metachromatic leukodystrophy
A. Acute disseminated encephalomyelitis
B. Adrenoleukodystrophy
C. Alexander’s disease
D. Krabbe’s disease
E. Metachromatic leukodystrophy
Alexander’s disease (C) is one of the leukodystrophies and is caused by a defect in the GFAP gene leading to hemispheric demyelination and mitochon- drial dysfunction. Numerous Rosenthal bers (eosinophilic material in cell processes, likely from GFAP degradation products) in areas of astrocytosis are noted (H&E). Alexander’s disease is an example of a nonneoplastic process where Rosenthal bers may occur.
A. Amyloid angiopathy
B. Duret’s hemorrhage
C. Glioblastoma
D. Hypertensive hemorrhage
E. Melanoma
A. Amyloid angiopathy
B. Duret’s hemorrhage
C. Glioblastoma
D. Hypertensive hemorrhage
E. Melanoma
Amassive basal ganglia hypertensive hemorrhage (D) is noted on this gross pathologic specimen
A. Central neurocytoma
B. Colloid cysts
C. Glioblastoma
D. Hem angioblastom a
E. Schwannoma
A. Central neurocytoma
B. Colloid cysts
C. Glioblastoma
D. Hem angioblastom a
E. Schwannoma
A dense array of uniform undi erentiated cells with small blue nuclei and perinuclear halos is found in central neurocytomas (A). The ndings are similar to oligodendrogliomas and can be di cult to di erentiate on H&E staining.Centralneurocytomasstainwithsynaptophysinandneuron-speci c enolase (NSE).
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
This Luxol fast blue stain shows demyelination of the posterior columns and ventral spinocerebellar tracts. Corticospinal tracts are also a ected
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
This Luxol fast blue stain reveals a spongiform and gliotic appearance of the cord primarily a ecting the posterior and lateral columns consistent with subacute combined degeneration (E). Subacute combined degeneration occurs in the setting of vitamin B12 de ciency and leads to impaired proprioceptive sense and paraplegia
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
There is an irregular area of coagulative necrosis involving both gray and white matter consistent with radiation myelopathy (D).
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
Well-de ned plaques are seen involving both gray and white matter in this Luxolfastbluestainedsection.These ndingssupportadiagnosisofmultiple sclerosis (C).
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
A. Amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. Multiple sclerosis
D. Radiation myelopathy
E. Subacute combined degeneration
This photomicrograph of a Luxol fast blue stained section shows degeneration of the anterior horn and corticospinal tracts, consistent with amyotrophic lateral sclerosis (A).
A. Gliomatosis cerebri
B. Huntington’s disease
C. Krabbe’s disease
D. Multiple sclerosis
E. Tuberous sclerosis
A. Gliomatosis cerebri
B. Huntington’s disease
C. Krabbe’s disease
D. Multiple sclerosis
E. Tuberous sclerosis
Cortical tubers are seen in the frontal and temporal lobes of this gross pathologic specimen as seen in . 95%of patients with tuberous sclerosis (D). Tuberous sclerosis is an autosomal dominant condition linked to chro- mosomes 9 and 16 characterized by the classic triad of adenoma sebaceum, seizures, and mental retardation. Patients with tuberous sclerosis are prone to develop cortical tubers, subependymal giant cell astrocytomas, cardiac rhabdomyoma, retinal hamartoma, and renal angiomyolipoma.
A. Choroid plexus papilloma
B. Ependymoma
C. Lymphoma
D. Medulloblastoma
E. Meningioma
A. Choroid plexus papilloma
B. Ependymoma
C. Lymphoma
D. Medulloblastoma
E. Meningioma
The histologic appearance of ependymomas is highly variable. Acellular vari- ety with sheetlike growth of oval to polygonal cells arranged in a perivascular pseudorosette is illustrated (H&E). Ependymomas are CNS neoplasms that resemblethestructureofthebrain’sependyma.Themostde nitiveevidence of ependymoma is the presence of true rosettes, also called “Flexner- Wintersteiner rosettes.” Most ependymomas contain perivascular pseudo- rosettes involving tumor cells surrounding an endothelial-lined lumen (as seen here). Ependymomas tend to stain for GFAP and vimentin, particularly in the perivascular pseudorosettes. On electron microscopy extensive surface microvilli forming both intra- and extracellular lumens can be seen.
A. Chordoma
B. Dermoid
C. Metastatic tumor
D. Myxopapillary ependymoma
E. Teratoma
A. Chordoma
B. Dermoid
C. Metastatic tumor
D. Myxopapillary ependymoma
E. Teratoma
Teratomas are the most di erentiated of the germ cell neoplasms and contain elements of all three germ layers: ectoderm, mesoderm, and endoderm. Ca r t i l a g e , m u c i n - p r o d u c i n g e p i t h e l i u m , a n d i m m a t u r e s p i n d l e c e l l s t r o m a a r e all part of this immature teratoma (E)—a low-grade malignancy (H&E)
This patient is most likely to have
A. Advanced age and lobar hemorrhages
B. Alcoholism and prone to falls
C. Port-wine nevus on the face
D. Retinalhamartomas
E. Subungual bromas
A. Advanced age and lobar hemorrhages
B. Alcoholism and prone to falls
C. Port-wine nevus on the face
D. Retinalhamartomas
E. Subungual bromas
Atrophy of the hemisphere and leptomeningeal venous angioma are present in this specimen with Sturge-Weber syndrome. Sturge-Weber syndrome is characterized by a usually unilateral port-wine nevus (C) that typically in- volves the orbit or upper eyelid, unilateral meningeal angioma, calci cations con ned to the second and third layers of cerebral cortex, and seizure activity. Advanced age and lobar hemorrhage (A) are associated with amyloid angi- opathy. Retinal hamartomas (D) and subungual bromas (E) are associated with tuberous sclerosis. Choice B, alcoholism and prone to falls, would be a better answer if the gross specimen showed cerebellar atrophy
A. Acousticneuroma
B. Anaplastic astrocytoma
C. Medulloblastoma
D. Melanoma
E. Meningioma
A. Acousticneuroma
B. Anaplastic astrocytoma
C. Medulloblastoma
D. Melanoma
E. Meningioma
This low-power view (H&E) shows dense Antoni Aareas (with compact spin- dle cells) and looser Antoni Bareas (with stellate cells) consistent with acous- tic neuroma (schwannoma)
A. Carbon monoxide poisoning
B. Cerebral contusions
C. Herpes encephalitis
D. Meningeal carcinomatosis
E. Melanoma
A. Carbon monoxide poisoning
B. Cerebral contusions
C. Herpes encephalitis
D. Meningeal carcinomatosis
E. Melanoma
Melanoma (E) tends to be composed of cells with epitheloid or spindle cell con gurations. Epitheloid cells with melanin inclusions are seen in this H&E-stained section, consistent with the diagnosis of melanoma. Primary CNS melanomas are more likely to be pigmented than metastatic melano- mas, which tend to be amelanotic. Amelanotic metastatic melanomas may be di cult to distinguish from metastatic carcinoma, but this distinction can be made with immunohistochemical stains. The presence of ducts or glands rules out melanoma
A. Acousticneuroma
B. Anaplastic astrocytoma
C. Medulloblastoma
D. Melanoma
E. Meningioma
A. Acousticneuroma
B. Anaplastic astrocytoma
C. Medulloblastoma
D. Melanoma
E. Meningioma
The anaplastic astrocytoma (B, WHO grade III) is a di use tumor with low to moderate cell density and moderate pleomorphism. There may be focal areas of increased cell density and increased pleomorphism; however, it must not contain areas of microvascular proliferation or necrosis (if either of these features are present, the diagnosis is glioblastoma—WHO grade IV). This H&E-stained section shows cellular pleomorphism, hypercellularity, and mitotic activity consistent with the diagnosis of anaplastic astrocytoma
A. Anaplastic astrocytoma
B. Infarct
C. Metachromatic leukodystrophy
D. Multiple sclerosis
E. Radiation necrosis
A. Anaplastic astrocytoma
B. Infarct
C. Metachromatic leukodystrophy
D. Multiple sclerosis
E. Radiation necrosis
This gross anatomical specimen shows a periventricular demyelinating plaque. This nding is consistent with the diagnosis of multiple sclerosis (D)
A. Anaplastic astrocytoma
B. Infarct
C. Metachromatic leukodystrophy
D. Multiple sclerosis
E. Radiation necrosis
A. Anaplastic astrocytoma
B. Infarct
C. Metachromatic leukodystrophy
D. Multiple sclerosis
E. Radiation necrosis
Metachromatic leukodystrophy (C) is caused by a de ciency of arylsulfatase Aleading to the accumulation of sulfatides in lysosomes. Inheritance is auto- somalrecessive—itisthemostcommonoftheleukodystrophies.Largecon u- ent areas of demyelination with U- ber sparing are seen in the H&E-stained photomicrograph and are typical of metachromatic leukodystrophy
A. Epidermoid
B. Lipoma
C. Metastatic tumor
D. Multiple sclerosis
E. Teratoma
A. Epidermoid
B. Lipoma
C. Metastatic tumor
D. Multiple sclerosis
E. Teratoma
A large cerebellopontine angle epidermoid (A) with white aky, keratinous debris is illustrated in this gross specimen. Epidermoid cysts consist of a cyst wallmadeupofstrati edsquamousepitheliumwithoutglandularstructures. The cyst contains desquamated keratin. Dermoid cysts can be distinguished histopathologically from epidermoid cysts based on the presence of hair follicles, adnexal glands, and the beginnings of papillary dermis formation in dermoid cysts. Some authors argue that dermoid cysts may represent benign teratom as.
A. Astrocytoma
B. Lymphoma
C. Melanoma
D. Oligodendroglioma
E. Pituitary adenoma
A. Astrocytoma
B. Lymphoma
C. Melanoma
D. Oligodendroglioma
E. Pituitary adenoma
This is an example of lymphoma (B) with di use perivascular lymphocytic in ltration into the Virchow-Robin space around a blood vessel. Such lym- phomas are often multiple, respond initially to steroids, but invariably recur, and are associated with immunosuppression in younger men.They also occur in immunocompetent males over age 60 years
A. Meningioma
B. Neurfibroma
C. Pilocytic astrocytoma
D. Pleomorphic xanthoastrocytoma
E. Schwannoma
A. Meningioma
B. Neurfibroma
C. Pilocytic astrocytoma
D. Pleomorphic xanthoastrocytoma
E. Schwannoma
Compact fascicles of elongated cells and spongiform foci with stellate forms and microcystic change are noted in this example of pilocytic astrocytoma (C).
A. Aneurysmal subarachnoid hemorrhage
B. Bacterial meningitis
C. Contusion
D. H S V- 1
E. Subdural hematoma
A. Aneurysmal subarachnoid hemorrhage
B. Bacterial meningitis
C. Contusion
D. H S V- 1
E. Subdural hematoma
Extensive bilateral contrecoup contusions of the orbital surfaces and frontal poles are illustrated in this gross pathologic specimen
A. Amyloid angiopathy
B. Arteriovenous malformation
C. Capillary telangiectasia
D. Embolism
E. Venousangioma
A. Amyloid angiopathy
B. Arteriovenous malformation
C. Capillary telangiectasia
D. Embolism
E. Venousangioma
The arterioles of the leptomeninges and super cial cortex are dilated, and amorphous material in ltrates the wall in this H&E-stained section from a patient with amyloid angiopathy (A).
A. Alzheimer’s disease
B. Astrocytoma
C. Huntington’s disease
D. Krabbe’s disease
E. Pick’s disease
A. Alzheimer’s disease
B. Astrocytoma
C. Huntington’s disease
D. Krabbe’s disease
E. Pick’s disease
Pickʼs disease (E) is a form of cerebral degeneration characterized by atrophy of the frontal and temporal lobes involving both the gray and white matter (lobar atrophy). Selective atrophy of the frontal and temporal lobes consistent with Pickʼs disease is noted in this gross pathologic specimen. In Alzheimer’s disease (A), atrophy is more mild and di use. Huntington’s disease (C) is associated with atrophy of the caudate
A. Astrocytoma
B. Glioblastoma
C. Hem angioblastom a
D. Medulloblastoma
E. Metastasis
A. Astrocytoma
B. Glioblastoma
C. Hem angioblastom a
D. Medulloblastoma
E. Metastasis
Hemangioblastoma (C) accounts for ~10% of posterior fossa tumors. The majority of hemangioblastomas are cystic with a mural nodule. In this gross specimen, a vascular mural nodule in the left cerebellar hemisphere and an associated cyst (midline) are consistent with the diagnosis of cerebellar hemangioblastoma.
A. Astrocytoma
B. Glioblastoma
C. Neuro broma
D. Pituitary adenoma
E. Schwannoma
A. Astrocytoma
B. Glioblastoma
C. Neuro broma
D. Pituitary adenoma
E. Schwannoma
This H&E-stained section shows an example of a pituitary adenoma (D). The normal acinar, heterogeneous appearance of the pituitary is replaced by a di use sheet of polygonal cells.2,3
A. Dejerine-Sottas disease
B. Krabbe’s disease
C. Metachromatic leukodystrophy
D. Normal peripheral nerve
E. Charcot-Marie-Tooth disease
A. Dejerine-Sottas disease
B. Krabbe’s disease
C. Metachromatic leukodystrophy
D. Normal peripheral nerve
E. Charcot-Marie-Tooth disease
Asection from normal sural nerve is illustrated
A. Cysticercosis
B. Hemangioblastoma
C. Juvenile pilocytic astrocytoma
D. Renalcellcarcinoma
E. Toxoplasmosis
A. Cysticercosis
B. Hemangioblastoma
C. Juvenile pilocytic astrocytoma
D. Renalcellcarcinoma
E. Toxoplasmosis
The lesion seen here is an example of cysticercosis (A). This specimen was excised from the fourth ventricle. The other responses are incorrect
The photomicrograph seen here
is most consistent with which of the following diagnoses?
A. Centralneurocytoma
B. Dysembryoplastic neuroepithelial tumor
C. Lymphoma
D. Meningioma
E. Schwannoma
A. Centralneurocytoma
B. Dysembryoplastic neuroepithelial tumor
C. Lymphoma
D. Meningioma
E. Schwannoma
This H&E-stained section is an example ofdysembryoplastic neuroepithelial tumor (B). Note the “ oating neuron” in a microcyst surrounded by smaller neurocytic cells. The other answer choices are incorrect
This photomicrograph
is an example of which of the following?
A. Chondrosarcoma
B. Chordoma
C. Glioblastoma
D. Gliosarcoma
E. Schwannoma
A. Chondrosarcoma
B. Chordoma
C. Glioblastoma
D. Gliosarcoma
E. Schwannoma
This H&E-stained photomicrograph shows an example of gliosarcoma (D) with a mosaic pattern of sarcomatous and gliomatous foci. Cells in the glial portion tend to be GFAP positive, while cells in the sarcomatous areas tend to be GFAP negative.
Which of the following is true regarding the lesion seen here?
A. They are usually lled with keratin.
B. They tend to be dorsally located.
C. They tend to occur in the midline.
D. Alloftheabove
E. None of the above
A. They are usually lled with keratin.
B. They tend to be dorsally located.
C. They tend to occur in the midline.
D. Alloftheabove
E. None of the above
The lesion seen here is an example of a neurenteric cyst. Neurenteric cysts likely represent developmental abnormalities involving entrapment of devel- oping foregut tissue in the developing leptomeninges. They have a columnar epithelium that usually produces a mucinous (PAS positive) material into the cyst lumen (A is false). Typically they occur ventrally (B is false), in the midline (C).
