Sabato 27 ottobre 2012 - Pacini Editore

COmuNiCaziONi ORali
progress, recur or (rarely) undergo malignant transformation.
About 103 documented cases were found in the literature: LPD
patients are mainly females (n. 102) in the reproductive age. We
present a case of LPD in a 32 year-old woman with a previous
history of laparoscopic myomectomy.
Material and methods. A 32-year-old female patient was admitted
to Siena University Hospital Department of Surgery since she
presented with abdominal and pelvic pain. Physical examination
revealed a firm hypogastric mass of 5-cm diameter and multiple
nodules of abdominal wall. Abdominal ultrasound examination
demonstrated the presence of numerous roundish peritoneal lesions
of varying sizes reaching the muscular plane. Past medical
history revealed a laparoscopic myomectomy. Exploration laparotomy
showed innumerable, firm, pale-grey, smooth nodules
on the surface of the omentum, parietal peritoneum, colon and
rectum muscle. Excision of several of the masses was performed.
Surgical specimens were formalin-fixed and paraffin-embedded.
From each block, 4-µm thick sections were obtained for histology
and immunohistochemistry. The following antibodies where
checked: cytokeratin AE1/AE3, vimentin, caldesmon, smooth
muscle actin, HHF-35, oestrogen and progesteron receptors,
Mib-1.
Results. On macroscopic examination all the lesions were similar.
They were solid, firm and whitish and ranged in size from
10 to 75 mm in maximum diameter. Nodules presented smooth
external aspects and pale, whorled cut surface. Pinpoint hemorrhages
were found but there was no macroscopic evidence of
necrosis. Microscopic examination showed bundles of spindleshaped
smooth muscle cells, without atypia, necrosis, or mitosis.
The nodules were embedded in fat tissue, and were mostly well
circumscribed. Some lesions showed extensive hyaline degeneration
and foci of calcification. Immunostaining revealed positivity
of neoplastic cells for smooth muscle actin (SMA), desmin, HHF-
35, oestrogen and progesteron receptors, and negativity for cytokeratins.
Proliferative index (Mib-1) was about 8%. Cytological
examination of the peritoneal liquid revealed only reactive mesothelial
cells and foamy histiocytes. The final diagnosis was LPD.
Discussion. Leiomyomatosis peritonealis disseminata is a rare
condition which is known to often simulate intra-abdominal
carcinomatosis. Firstly described by Wilson and Peale in 1952,
103 cases have been reported so far. The pathogenesis remains
obscure although the associations with pregnancy, prolonged oral
contraceptive use, subserosal uterine leiomyomata, functional
granulosa cell tumors and endometriosis indicate hyperestrogenic
states as a causal factor. Suggested pathogenic mechanisms
include smooth muscle metaplasia of the subcoelomic mesenchymal
stem cells (the so-called pluripotent Mullerian stem cells)
which are distributed throughout the subperitoneal mesenchyma
or a fibrosing reaction to ectopic omental deciduosis caused by
hormonal imbalance or excess. In case of individual predisposition
and hormonal stimulation, Mullerian stem cells proliferate
along lines of myofibrous differentiation. However, this hypothesis
does not explain four cases in postmenopausal women
without hormonal treatment and one case in a male patient. The
identification of luteinizing hormone (LH) receptor in LPD nodules
from a post-menopausal woman has suggested that the typical
postmenopausal increase in LH levels might plays a role in the
pathogenesis of the condition. In males, etiological mechanisms
are still obscure. Malignant transformation of LPD is uncommon
and only ten cases have been described so far. Malignancy
is thought to be more common in LPD without exogenous or
increased endogenous exposure and in tumours without expression
of oestrogen or progesteron receptors. Due to the rarity of
the condition, there are no definite guidelines on its management,
however, it has been suggested that more aggressive approaches
should be followed in higher risk groups.
Our patient was treated with laparoscopic surgery of many of the
greater nodules. Eight weeks after surgery she is well.
305
references
Al-Talib A, Tulandi T. Pathophysiology and possible iatrogenic cause
of leiomyomatosis peritonealis disseminata. Gynecol Obstet Invest
2010; 69:239-44.
Carvalho FM, Carvalho JP, Pereira RM, et al. Leiomyomatosis Peritonealis
Disseminata Associated with Endometriosis and Multiple
Uterus-Like Mass: Report of Two Cases. Clin Med Insights Case Rep
2012;5:63-8.
Jeyarajah S, Chow A, Lloyd J, et al. Follow-up in patients with disseminated
peritoneal leiomyomatosis: a report of an unusual, high-risk
case. BMJ Case Rep 2009 (Epub apr 2009).
Nappi C, Di Spiezio Sardo A, Mandato VD, et al. Leiomyomatosis peritonealis
disseminata in association with Currarino syndrome? BMC
Cancer 2006,6:127.
A potentially dangerous misdiagnosis: a rare case
of placental mesenchymal dysplasia associated
with perinatal viral infection and fetal death
M. Basciu2 , F. Moltrasio2 , F.M. Bosisio2 , P. Vergani3 , M. Verderio3
, V. Lucchini1 , M.S. Cuttin1 1 Department of Pathology, Azienda Ospedaliera San Gerarndo, Monza,
Italy; 2 Department of Pathology, Department of Surgical Science, Università
di Milano-Bicocca, Milano, Italy and Azienda Ospedaliera San
Gerardo, Monza, Italy; 3 Department of Obstetrics, Azienda Ospedaliera
San Gerardo, Monza, Italy
Placental mesenchymal dysplasia (PMD) is a disease with
unknown etiology characterized by volumetric increase with
cystic villi and dilated vessels. In literature less than 100 cases
has been described; about 20% of these are in association with
the Beckwith-Wiedeman syndrome, while another half are
associated with IUGR. Other associations are preeclampsia,
maternal hypertension, polyhydramnios, microsomia, omphalocele
and kidney abnormalities. Besides, congenital hemangiomas,
vascular amartoma and hepatic mesenchymal amartomas
have been described in some of this fetuses. 82% of cases show
normal female karyotype and in 43% of cases fetal or neonatal
death occurs.
We will describe the case of a placenta and a fetus of a 32-yearsold
which arrived at our observation with the diagnosis of PMD
and PROM. PMD was suspected on ultrasonography of the second
trimester.
The macroscopic placental alterations were: increase of weight
(1004 gr); presence on about half of fetal surface of a skein of
dilated and tortuous vessels of variable gauge, with sometimes
aneurysmal aspect and macroscopic evidence of thrombosis; pronounced
cord lenght (68 cm) with marked twisting; the presence
on the cut-surface of hemorrhagic and pseudocystic areas with
diffused aspects of vesicular chorionic villi.
The histology of the placental parenchyma was characterized by
a double villi population: one with increased villous core size
and hydropic-like features caused by loose stromal connective
tissue containing numerous vessels with thickened walls, without
evidence of circumferential trophoblast growth. The second
population preserved architecture and showed stromal oedema.
There was evidence of important vascular thrombosis.
The fetus, a female with normal karyotype (46, XX), macroscopically
presented a normal development corresponding to 30 weeks
of gestational age, without malformations and with obvious maceration
aspects (G2 sec. Potter).
On elbows cutis there were few vesicles; cephalohematoma was
present and on examination of visceral pleural and epicardial
surface hemorrhagic petechiae with pericardial blood effusion (2
cc) were found.
The histologic findings of the fetus were lung microhemorrhages
with horny material in the saccular cavity, and hepatic and adrenal
necrosis associated with cytopathic cellular changes suggestive
for viral infection such as nuclear inclusions.
The conclusive diagnosis was hemorrhagic fetal distress with