CNS Pathology case based MCQs-2

CASE : The spinal MRI of a 22-year old male with neurofibromatosis type-2 showed a hyperintense lesion. The lesion was resected and histopathologic section is shown in image.

Question 1: What is the diagnosis?

A. Medulloblastoma
B. Ependymoma
C. Retinoblastoma
D. Central neurocytoma

Question 2: Which of the following statements is true regarding this tumor?

A. They are predominantly supratentorial
B. Posterior fossa tumors in adults have a good prognosis
C. Spinal tumors have a bad prognosis
D. IDH 1 and 2 mutations are present in 100% cases.

Question 3: Identify the variant of the tumor shown in the image below.

A. Tancytic
B. Myxopapillary
C. Subependymoma
D. Anaplastic

Question 4: Which of the following statements is true regarding tancytic ependymoma?

A. GFAP negative
B. Abundant rosettes
C. Can be confused with pilocytic astrocytoma
D. WHO Grade 1

Question 5: Identify the FALSE statement regarding the variant of ependymoma shown below

Image credit- http://www.twitter.com/DrAldehyde

A. WHO Grade 1
B. Arises from filum terminale
C. Aggressive clinical course
D. Mucin stains positive

Question 6: TRUE statement regarding recent molecular classification (WHO CNS 2021 5th edition ) of ependymoma is

A. Spinal ependymomas with MYCN have a good prognosis
B. ZFTA Fusion- favorable prognosis
C. YAP Fusion- poor prognosis
D. Spinal ependymomas with NF 2 mutation- favorable prognosis

ANSWER- Question 1

Correct answer is B- Ependymoma

Ependymomas are characterized by perivascular rosettes and ependymal rosettes which have an empty central lumen

Types of rosettes
ANSWER- Question 2

CORRECT ANSWER IS B

🐱 Majority of of Ependymomas are infratentorial.

🐱Posterior fossa tumors in children have a poor prognosis (PF-A)- associated with loss of H3K27me, whereas Posterior fossa tumors in adults have a god prognosis (PF-B)- H3K27me is retained.

🐱 Spinal ependymomas have a good prognosis, whereas a small number of ependymomas associated with MYCN have a poor outcome.

🐱IDH 1 and 2 are associated with astroctyomas and oligodendroglomas but nor ependymomas.

ANSWER-Question 3

CORRECT ANSWER IS A

Diagnosis is TANCYTIC EPENDYMOMA, 🐱 Tancytes are cells with fibrillary processes which line ventricles along with ependymal cells.

Types of tancytes
ANSWER – Question 4

Correct They are GFAP positive 🐱Confused with pilocytic astrocytoma because of the prominent fibrillary projections 🐱It is a GRADE II Ependymoma 🐱 Ependymal rosettes and pseudorosettes are scanty in tanytic ependymoma, which makes diagnosis challenging.

ANSWER- Question 5

Correct answer is C
💀Diagnosis is myxopapillary ependymoma
💀It is a Grade II Tumor according to latest WHO
💀It is common in adults and arises from the filum terminale
💀It is clinically indolent ( nor aggressive)
💀Stains for Mucin is positive.

Mucin stains positive in myxopapillary ependymoma
ANSWER – Question 6

Correct answer is D

Spinal ependymomas with NF2 mutation has a good prognosis

Summary of latest molecular updates in ependymoma

Differences between Kimura disease and Angiolymphoid hyperplasia with eosinophilia

Kimura disease and Angiolymphoid hyperplasia with eosinophilia (ALHE) are often confused and need to be differentiated. Here are a few differences.

1.AGE

Kimura’s disease is a chronic inflammatory condition of unknown cause that affects young to middle-aged patients, most often males of Asian descent. Whereas, ALHE is more common in females.

2.LOCATION

Kimura disease patients usually have a mass in the head and neck region with involvement of subcutaneous tissue, soft tissue, salivary glands, and single or multiple regional lymph nodes. ALHE also commonly involves the head and neck region, particularly behind the ears.

3.HISTOLOGY

Key histologic features of kimura disease includes florid follicular hyperplasia that may contain a proteinaceous precipitate (IgE in a follicular dendritic network pattern) and vascularization of the germinal centers. The interfollicular areas show prominent high endothelial venules with a mixture of lymphocytes, plasma cells, eosinophils, and mast cells. Follicle lysis is often present, and eosinophilic abscesses are characteristic within germinal centers as well as in the paracortex.

ALHE on the other hand, is a vascular neoplasm characterized by the proliferation of blood vessels lined by plump endothelial cells with abundant eosinophilic cytoplasm, imparting a hobnail appearance. This lesion is part of the spectrum of what have been called histiocytoid or epithelioid hemangiomas, and is a low-grade vascular tumor. There is a dense, mixed inflammatory cell infiltrate consisting of lymphocytes, plasma cells, and eosinophils.

4. IMMUNOHISTOCHEMISTRY

Reticular pattern of IgE is seen in KIMURA disease. However, immunohistochemistry in ALHE is positive for CD 31, CD 34 and Factor VIII in the vascular component.

5.PERIPHERAL BLOOD EOSINOPHILIA

Peripheral blood examination shows eosinophilia and increased serum IgE levels in kimura disease but not so much in ALHE.

FIND A QUICK SUMMARY BELOW

REVIEW QUESTION

1. A 20- year- old Asian male has eosinophilia and high levels of IgE with cervical lymphadenopathy. Excisional biopsy of the lymph node demonstrates follicular hyperplasia, intense eosinophilia and eosinophilic microabscesses. h e most likely diagnosis is:

A. Kimura disease

B. Kikuchi- Fujimoto disease

C. Eosinophilic leukemia

D. Hypereosinophilic syndrome

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Answer is A- Kimura disease like Kikuchi disease is seen more ot en in the Asian population. h e etiology is not known. h is is a chronic inl ammatory disorder of the subcutaneous tissue and af ects regional lymph nodes. h e cervical area is the most common site to be involved. Histology of the af ected lymph nodes demonstrates follicular hyperplasia, eosinophilia with eosinophilic microabscesses and ini ltration of the germinal centers. Increase in vessels may also be seen. Warthin Finkeldey giant cells may also be present.

2. All are true about kimura disease except

A. Affects asians

B. Affects females

C. Peripheral eosinophilia is present

D. Elevated levels of IgE are seen

CLICK HERE TO REVEAL ANSWER

Answer is B- Kimura disease is more common in males.