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PS-16-001
Histopathologic pattern of ependymomas at an African tertiary
hospital
M. Nweke
*
*
University College Hospital, Pathology, Ibadan, Nigeria
Objective:
To determine the histopathologic pattern of Ependymomas at
the University College Hospital, Ibadan Nigerian over a seventeen-year
period. Ependymomas make up 19.5 % of paediatric Central Nervous
System tumours at our institution.
Method:
H & E Slides of all patients with clinical and radiological fea-
tures of intracranial space occupying lesions were reviewed over a
seventeen-year period
Results:
There were 31 histologically diagnosed cases of
Ependymomas. 21 (67.7 %) were males and 10 (32.3 %) were
females with a ratio of 2.1:1. Median age of the patients was
19.6 years. Age range spanned 10 months
–
60 years. Grade I
(Myxopapillary) Ependymomas accounted for 3 cases (9.7 %),
Grade II Ependymomas accounted for 17 cases (54.8 %) while
Grade III (Anaplastic) Ependymomas accounted for the remaining
11 cases (35.5 %). Among the grade II cases, the tannycytic
variant was the most common -16 cases (94.1 %). Only 1 case
of the papillary variant which accounted for (5 %) of Grade II
Ependymomas. Majority of Ependymomas were supratentorial in
location (90 %), located in various areas including Cerebral pa-
renchyma, Posterior Cranial Fossa, Thalamus. The remainder
(10 %) were located infra-tentorially in the Conus Medullaris
and Cervical Spine.
Conclusion:
Ependymomas in our environment show a male predomi-
nance and involve mostly adult patients.
PS-16-002
A case of CNS embryonal tumour with rhabdoid features and pre-
served SMARCB1/INI1 and SMARCA4/BRG1 nuclear expression
J. Pinheiro
*
, R. Silva, M. Honavar
*
Centro Hospitalar São João, Pathology, Porto, Portugal
Objective:
Atypical teratoid/rhabdoid tumour (AT/RT) is a malignant
CNS embryonal tumour composed predominantly of poorly differentiat-
ed elements, frequently including rhabdoid cells. Most are associated with
inactivation of SMARCB1/INI1 or SMARCA4/BRG1 genes. We present
a case of a CNS embryonal tumour with rhabdoid features, with preserved
SMARCB1/INI1 and SMARCA4/BRG1 expression.
Method:
An 18 months-old male child was admitted with prostration,
alimentary refusal and intolerance to being in prone position. On MRI, a
large left supratentorial lesion was seen, with cystic-necrotic areas and
calcifications. He was submitted to surgery.
Results:
On histological examination, a highly cellular tumour of
“
small
round blue cells
”
was seen with distinct rhabdoid cells. Areas of neuropil
with ganglion cells and mesenchymal differentiation were also observed.
However, on the immunohistochemistry, expression of SMARCB1/INI1
and SMARC/BRG1 were found to be preserved. At surgery, apparent
total gross resection was thought to have been achieved and was followed
by adjuvant radiotherapy and chemotherapy. Four years after surgery, the
patient is alive, with no evidence of disease progression.
Conclusion:
AT/RT is usually associated with loss of SMARCB1/INI1 or
rarely SMARCA4/BRG1. Embryonal tumours with rhabdoid morpholo-
gy and preserved expression of this genes are exceedingly rare. The
presence of a component with ganglionic differentiation is also uncom-
mon.
PS-16-003
Melanocytomas of the central nervous system: A short study of three
clinically different cases and their histopathological particularities
A.-C. Lisievici
*
, M. Lisievici, D. Pasov, C. Cocosila, V. Ciubotaru, L.
Tataranu
*
Bagdasar-Arseni Hospital, Bucharest, Romania
Objective:
Primary melanocytic neoplasms of the CNS are rare tumours
with reported incidence of about 1 in 10 million. Our study consists of 3
cases, diagnosed during a 1 year period, 2015 to 2016. We will try to
empathise the similarities and differences of these three tumours regard-
ing histopathological features as well as clinical presentation.
Method:
The cases were diagnosed based on classical HE slides,
correlated with neuroimaging and clinical data. Our results were later
confirmed by immunohistochemestry.
Results:
Three patients, two male and one female, aged 47, 63 and
26 were operated for tumours located in the craniospinal junction,
pituitary and temporal lobe respectively. While the first patient pre-
sented location specific symptoms, the second had been diagnosed
with a nonfunctional pituitary macroadenoma 14 years prior which
was believed to have recurred. The third patient was being followed
up for a thalamic cavernoma when a second lesion was observed.
Histopathologically all tumours were heavily pigmented and, while
the first presented an overall spindled morphology, the other two
were epithelioid. While nuclear features were sometimes difficult
to examine due to melanin deposits, none of the tumours had nu-
clear atypia or mitotic figures. The last case showed limited brain
invasion but proliferation index remained low.
Conclusion:
Though uncommon, melanocytic tumours affect a wide age
range. Derived from leptomeningeal melanocytes, they usually involve
the brain base. As benign tumours, prognosis is favorable yet the presence
of certain features (the term
“
intermediate grade melanocytic neoplasm
”
was proposed) require close follow-up.
PS-16-004
Histiocytic spinal mass with Rosai-Dorfman immunophenotypic pat-
tern in a patient with H-syndrome
D. Motevalli
*
, N. Kamalian
*
Tehran University, Dept. of Pathology, Iran
Objective:
H-syndrome is a rare autosomal recessive multisystemic
disease with clinical features including hearing impairment, hyper-
pigmentation, hyperglycemia, short stature, hypogonadism, hallux
valgus and phalangeal flexure contractures and hypertrichosis.
Method:
A 26-year-old Iranian boy with parents of a consanguineous
marriage presented with progressive weakness of lower extremities since
few months prior to this admission. On examination, Arcus senilis, bilat-
eral sensorineurial hearing impairment, bilateral cervical lymphadenopa-
thy, generalized cutaneous hyperpigmented plaques, hallux valgus and
phalangeal flexure contracture in both upper and lower extremities and
short stature (H: 157, Wt:37Kg) were noted. Past medical history was
significant for Type 1/ Insulin-dependent diabetes mellitus which caused
CKD and another removed histiocytic lesion on hand with pathologic
diagnosis of histiocytic lesion. Thoracic and lumbospinal MRI showed
a homogenous enhancing extradural soft tissue in thoracic canal (mainly
T6-T8 level) with cord compression.
Results:
In histolopathological examination, the lesion is composed of
large histiocytes, some plasma cells, and scattered lymphoid aggregates.
Immunohistochemical staining revealed positivity of histiocytes for
CD68 and S100 and negativity for CD1a.
Tuesday, 5 September 2017, 09:30
–
10:30, Hall 3
PS-16 Neuropathology
Virchows Arch
(
2017
)
471
(
Suppl 1
):
S1
–
S352
S213