238 / 366
investigate intracranial cystic lesions that underwent pathologic
examination.
Method:
Between 2010 and 2017, reports of 357 pathologic examination
which were sent by neurosurgeons were retrospectively reviewed and 15
pathologically benign cystic lesions were detected. The histopathological
diagnosis, demographic data and localisations of these lesions were evaluated.
Results:
The most common diagnosis was epidermoid cyst ( 53.3 % (8
patients)). Others were cyst hydatid 20 % (3 patients), colloidal cyst
13.3 % (2 patients), enteriogenous cyst 6.6 % (1 patient), and
glioependent cyst 6.6 % (1 patient) according to the order of frequency.
60 % of the cases were female, 40 % were male. The average age was 28.8
(standart dev.: 24). The most common cysts in children (0
–
18 years) were
hydatid cysts, the most common in adults (> 18 years) were epidermoid cysts.
Hydatid cysts and colloidal cysts were supratentorial and others were
infratentorial.
Conclusion:
While epidermoid cysts should be kept in mind in
infratentorial cystic lesions in adults in preoperative evaluation, hydatid
cyst should be suspected in childhood, especially supratentorial cyst in
endemic regions.
PS-17-001
A rare paediatric case of the cholesterol granuloma in the maxillary
sinus
A. Kilitci
*
, M. Avcu
*
Ahi Evran University Hospital, Dept. of Pathology, Kirsehir, Turkey
Objective:
Cholesterol granuloma(CG) is usually associated with chronic
middle ear disease,it
’
s a rare histological entity in the maxillary sinus
consisting of granulation tissue in which dense masses of cholesterol crys-
tals and provoke foreign body giant cell formation.It
’
s more common in
middle-aged patients. We report a paediatric case of CG in the maxillary
sinus,underlining the rarity and the differential diagnosis.
Method:
A 12-year-old girl complained of chronic nasal obstruction and
headache.Sinus endoscopy revealed a polypoid mass, and enlarged ade-
noids in the nasopharynx.Preoperative diagnosis was sinonasal
polyp.Polypectomy and adenoidectomy were performed.
Results:
Gross examination showed polypoid mass measuring 2x1,4 cm
which had a tan-brownish appearance. Microscopic examination of the
mass demonstrated tissue lined by respiratory epitheliumwith large numbers
of subepithelial cholesterol clefts.Many histiocytes andmultinucleated foreign
body giant cells surrounded the cholesterol clefts. Focal areas showed
degenerated red cells,hemosiderin-laden macrophages,lymphoplasmacytic
cells,fibrosis and mucosal edema.Adenoidectomy material was reported as
adenoiditis and lymphoid tissue hyperplasia.
Conclusion:
Etiological causes has been suggested of maxillary sinus
CG:disturbed ventilation,impaired drainage and hemorrhage into the sinus
with hemolysis and accumulation of cholesterol from red cell
membranes.The mode of therapy is surgical excision. Differantial diagnosis
of CG should contain inflammatory/allergic polyps, mucoceles,sinusal cysts
and tumours.Histopathologic analysis is necessary for the final diagnosis.
PS-17-003
Brain metastasis of a clear cell sarcoma of the kidney: A case report
and review of the literature
C. Marques Pontinha
*
, D. Pereira, R. Cabrera, M. Mafra, D. Forte, A.
Iraneta
*
Central Lisbon Hospital Center, Dept. of Anatomic Pathology, Portugal
Objective:
To report a case and review the literature.
Method:
The MRI of a 16-months-old male child presenting with sei-
zures revealed a lesion at the posterior fossa invading the 4th ventricle. At
2 months of age, he was diagnosed with a Clear Cell Sarcoma of the
Kidney (SIOP stage IV
–
with axillary lymph node and cutaneous metas-
tases) and treated according to a protocol for paediatric high risk renal
tumours, with good response. Almost at the same time bone metastasis
were identified.
Results:
The histopathological examination of the brain lesion identified
a neoplasm composed of uniform, small round cells with a clear appear-
ance, separated by a delicate vascular network, one of the patterns ob-
served in the primary tumour. The neoplastic cells were imunoreactive for
Vimentin, Bcl2 and INI1; as in the renal tumour.
Conclusion:
Clear Cell Sarcoma of the Kidney is an uncommon renal
neoplasm of childhood. It
’
s one of the tumours with most unfavorable
prognosis, with high propensity to metastize to unusual sites. Brain me-
tastasis have only infrequently been published.
PS-17-004
The spectrum of cloacal dysgenesis in one male and three female
fetuses: Case study and review of the literature
S. Stasinopoulou
*
, M. Eleftheriadis, A. Konstantinidou
*
Aretaieio University Hospital, Dept. of Pathology, Athens, Greece
Objective:
To describe features of cloacal dysgenesis, and emphasize on
the extremely rare occurrence of cloacal dysgenesis in males, which may
be prenatally misinterpreted as megacystis.
Method:
We reviewed the postmortem findings and the prenatally de-
tected ultrasonographic findings of four cases of cloacal dysgenesis in one
male and three female fetuses. Fetal autopsies were carried out at the
Department of Pathology of the National and Kapodistrian University
of Athens, Greece. Our observations were compared with a review of
the literature on cases of cloacal dysgenesis.
Results:
All 4 fetuses showed a persistent cloaca (large dilated
intraabdominal cystic formation, communicating with the intestine).
Abnormalities or complete absence of the external genitalia and associ-
ated renal malformations were features noted in both female and male
cases. The gonads were hypoplastic, absent or ectopic in one of the fe-
male cases. Anal atresia was present in all female fetuses, but was not
seen in our male case. Vertebral defects were seen in one of the female
fetuses. Cardiovascular malformations and focal amyoplasia were seen
only in the male fetus. On prenatal ultrasonography all four cases were
diagnosed as cases of megacystis. Dysplastic cystic renal changes were
also detectable by ultrasound. Our findings were overall consistent with
those described in the literature. Tubular hypoplasia of the aorta and focal
amyoplasia of the intestinal wall have not been previously described in
other female or male fetuses with cloacal dysgenesis.
Conclusion:
According to our observations, megacystis detected in male
fetuses on prenatal ultrasound examination may harbor the severe spec-
trum of cloacal dysgenesis, which necessitates a totally different parental
counseling and therapeutic approach.
PS-17-005
Primary endocardial fibroelastosis in an infant with the prenatal
diagnosis of dilated cardiomyopathy
R. Carvalho
*
, C. Pontinha, C. Brandão, C. Trigo, F. Pinto, E. Pinto, M.
Oliveira, J. Fragata
*
Central Lisbon Hospital Center, São José
’
s Hospital, Pathology, Portugal
Objective:
Endocardial fibroelastosis is thought to be a nonspecific reac-
tion against hemodynamic stress, regardless of etiology. It may be clas-
sified as primary (idiopathic) or secondary, when in association with
congenital heart diseases or viral myocarditis.
Tuesday, 5 September 2017, 09:30
–
10:30, Hall 3
PS-17 Paediatric and Perinatal Pathology
Virchows Arch
(
2017
)
471
(
Suppl 1
):
S1
–
S352
S224