Sabato 27 ottobre 2012 - Pacini Editore

PoStER
invaded focally adjacent tunica albuginea but no epididymus,
tunica vaginalis, spermatic cord and scrotum skin.
Specimens were fixed in 10% buffered formalin and embedded
in paraffin. Paraffin sections were stained with hematoxilyn and
eosin for histological analysis. For immunohistochemical studies,
sections were incubated with cytokeratin cocktail (Ck AE1/AE3),
low molecular weight cytokeratin (Ck35BetaH11), PLAP, CD30,
CD-117 (c-Kit), AlphaFetoProtein (AFP) and Beta-Human Chorionic
Gonadotropin (BetaHCG).
On low-power microscopic examination, tumour showed a reticular-microcystic
pattern, characterized by irregular loose spaces and
anastomosing thin cords and tubules lined by flat or cuboidal cells.
Also, papillary clusters of cells and many Schiller-Duval bodies
were present. Tumoral lymphovascular invasion occurred. All
tumor cells are positive for Ck AE1/AE3, Ck35BetaH11 and AFP,
negative for CD30, PLAP, CD117 and BetaHCG. No other germ
cell components are associated with this tumor. So, our diagnostic
conclusion was “Pure Yolk Sac Tumour of adult testis”.
Discussion. Yolk Sac Tumour (or Endodermal Sinus Tumour)
is a testicular non-seminomatous germ cell tumour (NSGCT)
characterized by numerous patterns that recapitulate the yolk sac,
allantois and extraembrionic mesenchyme.
In the testis YST is seen in two distinct age groups, infants and
young children (birth to 5 years) and postpubertal males.
In young children it is almost always seen in pure form and it accounts
for 75-80% of all childhood testicular neoplasms.
In adults, it is seen in approximately 40% of NSGCT and the
pure form is very rare because it usually occurs as a component
of mixed germ cell tumours.
Alfa-Fetoprotein levels are elevated in 90 percent of cases.
On gross examination, the enlarged testis contains a poorly defined,
lobulated, white-gray or gray-yellow tumor ranging in size
from 2 to 6 cm in diameter. It may be focally cystic or a solid
mass with variable consistency, and haemorrhage and necrosis
may be present. The cut surface often has a mucinous texture.
Microscopically, the kay to the recognition of YST is the simultaneous
presence of myriad histologic patterns. The reticularmicrocystic
pattern is most common. The most distinctive pattern
is the one forming Schiller-Duval bodies, considered a hallmark
of YST, in which a central fibrovascular core is surrounded by
malignant cuboidal to columnar epithelioid cells. Other variations
include macrocystic, papillary, glandular-alveolar, solid, myxomatous,
polyvesicular vitelline, hepatoid and enteric patterns.
Intracellular and extracellular hyaline Pas-positive globules are
characteristic of yolk sac differentiation.
Pediatric tumors are not associated with ITGCN (Intratubular
Germ Cell Neoplasia); in contrast, almost all adult tumors with
yolk sac tumor occurring as a component of MGCT (Mixed Germ
Cell Tumors) have ITGCN. Tumor cells are positive for AFP, CK
AE1/AE3, CK35BetaH11 and negative for CD30, CD117, PLAP
and BetaHCG.
Conclusions. There are two groups of prognosis predictive factors
for yolk sac tumor of testis:
clinical (age, clinical stage, blood levels of AFP) and morphologic
(histologic patterns, lymphovascular invasion, pure and
mixed forms) criteria.
Age does not appear to be prognostically important even though
patients less than 2 years old have the best prognosis.
Clinical stage of disease at initial presentation and degree of AFP
elevation are important prognostic factors.
Histologic patterns of YST are not prognostic value.
Lymphovascular invasion is associated with a worse prognosis.
In adults with yolk sac differentiation as a part of NSGCT, the
prognosis varies with the stage of disease, but its presence does
not appear to affect outcome adversely when current therapeutic
modalities are used.
Since pure YST in adults is very rare, little is known about its
behavior at this time.
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references
1 Kaplan GW, Cromie WC, Kelalis PP. Prepubertal yolk sac testicular
tumours: Report of the Testicular Tumor Registry. J. Urol
1988;140:1109-12.
2 Montserrat Orri V, López-Bonet E, Garijo G, et al. Pure yolk sac
tumor of the testis in adults: report of a case. Actas Urol Esp
1996;20:659-61.
3 Pinkerton CR. Malignant germ cell tumours in childhood . Eur J Cancer
1997;33:895-901.
5 Foster RS, Hermans B, Bihrle R, et al. Clinical stage I Pure Yolk Sac
Tumor of the testis in adults has different clinical behavior than juvenile
yolk sac tumor. J Urol 2000;164:1943-4.
6 Terenziani M, Piva L, et al., Clinical stage I non-seminomatous germ
cell tumors of the testis in chilhood and adolescence: an analysis of 31
cases”. J Pediatr Hematol Oncol 2002;24:454-8.
7 Medica M, Germinale F, Giglio M, et al. Adult testicular pure yolk sac
tumor. Urol Int 2001;67:94-6.
8 Denfeng Cao, Humphrey PA. Yolk sac tumor of the testis . The Journal
of Urology 2001;19.
9 Wada S, Yoshimura R, Nishisaka N, et al. Primary retroperitoneal
pure yolk sac tumor in an adult man. Scand J Urol Nephrol
2001;35:515-7.
10 Pohl HG, Shukla AR, Metcalfe PD, et al. Prepubertal testis tumors:
Actual prevalence rate of histological types. J Urol 2004;172:2370-2.
11 Eble JN, Sauter G, Epstein JI. Sesterhenn “Tumours of the Urinary
System and Male Genital Organs. WHO Blue Books, 2004: 237-240.
12 Ulbright TM. Germ cell tumors of the gonads: a selective review emphasizing
problems in differential diagnosis, newly appreciated, and
controversial issues! Mod Pathol 2005;18(Suppl. 2):S61-79.
13 Dadali Mumtaz, Sunay Melih, et al. Pure yolk sac tumor of testis in an
adult patient: case report. Turkish Journal of Urology 2010;01.
Leiomyomatosis peritonealis disseminata:
pregnancy, oral contraception and myomectomy,
three peculiar features in a single case
M. Onorati, P. Uboldi, G. Petracco, S. Romagnoli * , M.M. Amidani
** , F. Di Nuovo
Pathology Unit, Garbagnate Milanese, AO “G. Salvini” Garbagnate Milanese,
Italy; * Dept. Health Sciences, University of Milan Medical School,
Pathology Unit, AO S. Paolo, Milan, Italy; ** Gynecological Unit, Garbagnate
Milanese, AO “G. Salvini” Garbagnate Milanese, Italy
Introduction. Leiomyomatosis peritonealis disseminata (LPD) is
an unusual smooth muscle tumor of unknown origin. It is characterized
by the presence of multiple nodules of varying sizes
on the abdominal and pelvic peritoneum, grossly mimicking disseminated
carcinoma. The tumor was first described in 1952 by
Wilson and Peale. Taubert et al. (1965) named it leiomyomatosis
peritonealis disseminata. It appears during reproductive age, especially
with pregnancy or, more often, in cases of long exposure
to oral contraceptive agents. According to the hormonal hypothesis,
Tavassoli and Norris postulated that the pathogenesis of LPD
involves subperitoneal mesenchymal stem cells that undergo
metaplasia, being the process promoted by hormonal stimulation.
LPD has been also found in patients with endometriosis, suggesting
that both of them derive from the same cell of origin, the
submesothelial multipotential mesenchymal cells. The hormonal
theory is supported by experimental studies that have shown
a development of metaplasia of mesenchymal stem cells and
subsequent leiomyomatous peritoneal lesions after a prolonged
administration of high doses of estrogens. At the same time, it
has been shown that the nodules can be reduced by decreasing
the estrogen level with gonadotropin-releasing hormone agonists
(GnRh agonists) or aromatase inhibitors. In recent years, some
authors reported LPD in women after abdominal miomectomy
or abdominal hysterectomy. Approximately, 113 cases have been
published in literature so far, less than 50 reported in pregnant
women. We report an unusual case of a 36 year old pregnant
woman with leiomyomatosis peritonealis diffusa, a past history
of miomectomy and use of oral contraception for three years.
Decidual areas were present both in the tumour and in the sinus of