33 / 366
OFP-06-009
Undifferentiated (embryonal) sarcoma of the liver: An acute
presentation
J. Tavares
*
, C. Quadros, R. Luis, D. López-Presa
*
Hospital de Santa Maria, CHLN, Pathology Dept., Lisbon, Portugal
Objective:
The undifferentiated (embryonal) sarcoma of the liver
(UESL) represents 9
–
15 % of the paediatric primary malignant tumours
of the liver, being the third most common hepatic malignancy in this
epidemiological group; the majority presents in children from 6 to
15 years old. We report one of a total of two UESL cases diagnosed in
the southern Portuguese tertiary care institutions within 10 years, in a
patient whose age was higher than anticipated.
Method:
A 17 year-old female referred to the emergency room with
severe abdominal pain; preliminary imaging studies revealed liver rupture
and hemorrhage stemming from a 19 × 16 × 12 cm solid lesion with
cystic areas in the right hepatic lobe. The patient underwent embolization
of the right branch of the hepatic artery and, a few days later, partial
hepatectomy.
Results:
Multiple tumour fragments were received, the largest measuring
approximately 7 cm in greatest dimension. Grossly, the cut surface was
glistening, with hemorrhagic cystic areas. Histologically, a malignant
spindle-cell tumour was observed with morphologic and immunohisto-
chemical features that led to the diagnosis of UESL.
Conclusion:
UESL is associated with poor prognosis only avertible by
complete surgical resection and adjuvant chemotherapy. In our case the
tumour relapsed leading to a fatal outcome 67 days after the surgery.
OFP-06-010
Persistent localised interstitial lung emphysema associated with
CPAP therapy: A case report
I. Franckevica
*
, I. Melderis, R. Zarina
*
Children
’
s Clinical Hospital, Dept. of Pathology, Riga, Latvia
Objective:
Pulmonary interstitial emphysema (PIE) is abnormal collec-
tion of gases inside the connective tissue of the peribronhovascular tissue,
interlobular septa and visceral pleura. It is not an uncommon finding in
premature infants who need respiratory support by mechanical ventila-
tion. (Bhojani et al, 2008). Only few cases of spontaneous PIE in unven-
tilated patients are described (Bawa et al; 2014)
Method:
We presented an immature baby with persistent PIE on contin-
uous positive airway pressure (CPAP) therapy.
Results:
A 30 week gestation male infant from twin pregnancy was born
in a vaginal delivery. He was placed on nasal CPAP which was stopped in
3rd day of life and renewed again in the 5th day of life. No PIE was found
in X-ray investigation. In the 20th day of life symptoms of sepsis was
developed and patient suddenly died. In the autopsy was found cystic
lesion of the upper lobe of the left lung. Histological investigation showed
appearance of interstitial cysts lined with multinucleated giant cells con-
sistent with persistent PIE.
Conclusion:
1. Localized PIE can occur for children treated with CPAP
therapy only. 2. Localized PIE can predispose patient to worse prognosis
even without appearance of pneumothorax and pneumomediastinum.
OFP-06-011
Microcephaly beyond the Zika virus: A two-center study with dis-
cussion of etiopathogenesis
D. Pinto
*
, A. Braga, A. Barata, P. Borralho, M. Ferreira, L. Monteiro
*
CHLO, EPE, Anatomia Patológica, Portugal
Objective:
Microcephaly doesn
’
t have a consensual definition.
Classically it has been defined as a head circumference more than 2
standard deviations (SD) below average. However, this criterion can
’
t
be used alone for diagnosis, as if it were, 2.3 % of the general population
would be microcephalic. We aim to characterize this malformation in the
context of fetal autopsies.
Method:
We consulted 6 years of data from two centers. Cases with micro-
cephaly were identified and analyzed according to: maternal and obstetric
history, fetal pathology and etiopathogenesis.
Results:
In 2565 autopsies, 31 cases (1.2 %) of microcephaly were identified.
29,1 % were considered severe (3 SD below average). M:F ratio was 3:5.
Average gestational age was 25w + 3d. 38,7 % were medical interruptions
and 35,5 % were intrauterine deaths. Median maternal age was 32 years;
12,9 % had history of previous abortion and more than 20 % had accompa-
nying maternal pathology. Most cases were associated with chromosomal or
genetic abnormalities and in two cases a history of maternal Zika virus infec-
tion (ZVI) was identified.
Conclusion:
Recently, greater attention has been given to microcephaly as-
sociated with ZVI. However, microcephaly has variable etiologies, which our
study highlights. Early echographic detection and a complete fetal autopsy are
fundamental, enabling a proper etiological diagnosis and genetic counseling.
OFP-06-012
Rare case of bifid cardiac apex associated with incidental neuroblas-
toma described in a first trimester fetus using 7Tesla post mortem-
MRI and conventional autopsy
A.-M. T. Domsa
*
, C. Albu, A. Staicu, F. Stamatian
*
Cluj-Napoca, Romania
Objective:
Bifid cardiac apex is an unusual congenital anomaly de-
scribed in marine mammals and rarely encountered in humans, being
reported in less than 10 cases worldwide. We describe a case of bifid
cardiac apex, discovered at the post mortem examination of a 13 weeks
fetus.
Method:
The pregnancy resulted after the artificial insemination of a
36 years old patient, diagnosed with hereditary thrombophilia. The spon-
taneous abortion occurred at 13 weeks of gestatinon, after adequate track-
ing of the pregnancy. The fetus was analyzed postmortem, firstly imag-
istic using a 7 Tesla Magnetic Resonance Imaging scanner and after by
stereomicroscopic conventional autopsy.
Results:
Both postmortem examinations revealed the heart in situs solitus
with a bifid apex having a notch of 0,82 mm. The four chambers and the
great vessels origin presented normal aspect, but the aortic arch was
narrowed in the preductal level. Microscopic examination of the organs
also revealed a neuroblastoma arising in the left adrenal gland, with local
invasion and metastasis in the contralateral kidney, mediastinum and
placental villi. The umbilical cord had normal histology, without tumoural
emboli in the examined sections.
Conclusion:
We present the first case of bifid cardiac apex identified in a
first trimester fetus.
OFP-07-001
Immunohistochemical expression and prognostic significance of PD-
L1 and PD-1 in Ewing sarcoma family of tumours (ESFT)
A. Llombart Bosch
*
, I. Machado, J. A. Lopez Guerrero, K. Scotlandi, P. Picci
*
University of Valencia, Medical School, Dept. of Pathology, Spain
Objective:
Ewing sarcoma family of tumours (ESFT) are aggressive neo-
plasms with scant tumour-infiltrating lymphocytes. We analyzed the
Monday, 4 September 2017, 17:15
–
19:15, G109
OFP-07 Joint Session: Soft Tissue and Bone Pathology /
Nephropathology
Virchows Arch
(
2017
)
471
(
Suppl 1
):
S1
–
S352
S19