96. Jahrestagung der Deutschen Gesellschaft für Pathologie e. V ...

nic markers such as myf4 and MyoD1 are a helpful and sometimes indispensable
diagnostic tool for the correct diagnosis of rhabdomyosarcoma.
SA-P-070
Angiofibromyxoma of the umbilical cord
A .M . Müller 1 , U . Gembruch 2 , M . Vogel 3
1 University Bonn, Department of Pediatric Pathology, Bonn, 2 University
Bonn Medical Center, Dept . of Obstetrics and Prenatal Therapy, Bonn, 3 University
of Leipzig, Institute of Pathology
Aims. Tumours of the umbilical cord are extremely rare. Differential diagnosis
includes hemangiomas, teratomas, abdominal wall defects and
vascular lesions like hematoma and thrombosis. Hemangiomas are by
far the most common umbilical cord tumours.
Methods. In a 35-year-old woman in the 25th week of gestation a tumour
of the umbilical cord was ultrasonographically diagnosed. Ultrasound
displayed a significantly broadened umbilical cord with hyperechogenic
areas and increased whartons’ jelly and arterio-venous fistulas. Apart
from a slight cardiomegaly fetal heart was regular. In week 38 a healthy
girl was born by cesarean.
Results. Histologically the placenta showed several chorangiomas. The
umbilical cord displayed an angiofibromyxom with – taking into consideration
ultrasound findings – intratumoral arterio-venous malformations.
Conclusions. Angiofibromyxoma with intratumoral arteriovenous malformations
is a very rare subtype of hemangiomatous lesion of the umbilical
cord. Umbilical cord is formed between the sixth and eighth week
of gestation by the approximation of the omphalomesenteric duct resp
yolk sac and the allantoic duct within the body stalk. The two arteries
and one vein derive from the allantoic vessels. Hence cord hemangiomas
are recognized as arising from omphalomesenteric or allantoic vessels.
As they often occur together with the more common chorangiomas –
like in our case – an underlying congenital predisposition to vascular
neoplasms has to be discussed.
SA-P-071
Pregnancy luteoma – an uncommon ovarian tumor: association
with intrauterine growth retardation (IUGR)?
C . Jayasinghe1 , T . Ameziane2 , C . Auerbach2 , A .M . Müller3 1Gummersbach Hospital, Institute of Pathology, Gummersbach,
2St . Elisabeth Hospital Bonn, Department of Obstetrics and Gynecology,
Bonn, 3University Bonn, Department of Pediatric Pathology, Bonn
Aims. Pregnancy luteomas are rare benign lesions of the ovary induced
by hormonal alterations during pregnancy. They present as unilateral
or bilateral tumorous masses and are usually incidental findings during
imaging or caesarean section. Most of the patients are asymptomatic.
However, virilization of the mother occurs in one third of cases and virilization
of the female fetus is found with two thirds of virilized mothers.
Pregnancy luteomas regress postpartum spontaneously, therefore
conservative treatment is sufficient. Nevertheless pregnancy luteomas
are challenging, particularly for clinicians, as they can mimic malignant
ovarian tumors.
Methods. We present the case of a 30-year-old primigravida with beta
thalassemia major who underwent caesarean section at week 37 because
of pathological CTG. A hypotrophic girl was delivered with normal
Apgar score. Both mother and daughter showed no endocrine abnormalities.
Intraoperative both ovaries were enlarged and multicystic. One
ovary was resected for histopathologic examination.
Results. Histomorphology of the ovary displayed nodules of luteinized
cells and muliple luteinized ovarian cysts within an edematous stroma,
typical histological findings of a pregnancy luteoma. Placental examination
revealed signs of insufficiency.
Conclusions. Pregnancy luteoma is a rare ovarian lesion which can macroscopically
be misinterpreted as malignancy. Awareness of this entity
can avoid unnecessary adnexectomy in young patients as pregnancy
luteomas regress spontaneously. Although hyperandrogenism can be
associated with IURG it is hardly probable that in the present case fetal
hypotrophy was due to pregnancy luteoma because the level of secreted
androgens was not high enough to cause manifest hyperandrogenism.
Instead IUGR in our case is more likely attributable to placental insufficiency
and/or beta thalassemia major.
SA-P-072
Infantile digital fibromatosis (IDF) – A case report and review of
the literature
U . Titze1 , R . Rödl2 , G . Köhler1 1University Hospital Münster, Gerhard-Domagk-Institute for Pathology,
Münster, 2University Hospital Münster, Department of General and Tumor
Orthopedics, Münster
Aims. We report on a 7 months old male infant who received excisionbiopsy
of a rapidly growing dermal tumor from the 2nd toe of the right
side.
Methods. Histological, immunohistological and ultrastructural examination
revealed typical findings of an infantile digital fibromatosis
(WHO: inclusion body fibromatosis).
Results. Infantile digital fibromatosis (IDF) is a rare, distinctive benign
fibroblastic/myofibroblastic tumor of infancy typically arising in the digits
of the hands or feet. Characteristic morphological findings in the
proliferating spindled cells are characteristic rounded eosinophilic paranuclear
inclusions.
Conclusions. Etiology of IDF remains uncertain. Despite of its benign
behavior, local recurrence was seen in up to 60% of cases after surgical
therapy. Local installations of corticosteroids do not reduce the size of
these lesions. Current management of IDF recommends avoiding surgical
intervention, as spontaneous involution is the rule.
SA-P-073
Male fetus with ectrodactyly ectodermal dysplasia clefting (EEC)
syndrome
F . Fronhoffs1 , S . Detering2 , S . Gerlach1 , C . Berg3 , M . Born4 , A .M . Müller1 1 2 University Bonn Medical Center, Institute of Pathology, Bonn, University
Bonn Medical Center, Institute of Pathology, Bonn, 3University Clinic of Cologne,
Department of Prenatal Medicine and Ultrasound, Köln, 4University Bonn Medical Center, Institute of Radiology, Bonn
Aims. Characteristics of the ectrodactyly ectodermal dysplasia clefting
(EEC) syndrome, first described by Rüdiger et al in 1970, are ectrodactyly,
dysplasia of skin, its adnexal structures and/or teeth as well as orofacial
clefts. Its exact prevalence is unknown. Until now, about 300 cases
have been reported in literature. In more than 90% of all cases, a missense
mutation in the gene TP63 can be detected.
Methods. 33-year-old mother, gravida 1, para 1. Proof of bilateral complete
cleft of lip and palate and ectrodactyly of both feet and hands and
tentative sonographic diagnosis of complex cloacal persistence and malformation.
Confirmation of EEC-syndrome by genetic testing. Feticide
in 24th+4 week of gestation.
Results. Male fetus, appropriate for gestational age, with bilateral complete
cleft of lip and palate accompanied by deformation of the nasal apex.
Ectrodactyly of both feet and hands. Right hand with five metacarpals (I,
III–V regular, II shortened) and agenesis of phalanges II and III. At the
left hand only a rudimentary anlage of digitus II but regularly formed
digitus I and III–V. Right foot with five metacarpals but shortened metacarpal
II. Left foot with five regularly shaped metacarpal bones, but only
four phalanges (I and III–V), i.e. missing second toe. Time-adequate development
of the nails. Histologically, in the skin biopsy only very few
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