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Yolk sac tumor of the vagina.Saudi
Med J. 2007 Jul;28(7):1125-6.
Malignant
germ-cell tumors GCT are rare tumors of childhood accounting for less
than 3% of pediatric malignancies. Endodermal sinus tumors EST form
the most common histologic subtype of malignant GCT. The vagina is an
extremely rare site for GCTs. An 8-month-old female was admitted with
a short history of vaginal bleeding, and a mass protruding from the
vagina. A mass was palpable anteriorly on rectal examination. Computed
tomography showed a tumor mass posterior to the bladder. A biopsy
revealed a vaginal EST. The serum alpha-fetoprotein was elevated.
Vaginohysterectomy was carried out. She was subsequently referred to
the oncologist for further management.
Intrarenal
pure yolk sac tumor: an extremely rare entity.Int
J Surg Pathol. 2007 Apr;15(2):204-6.
Yolk sac tumor
(endodermal sinus tumor) is a malignant germ cell tumor that usually
arises in the gonads. Extragonadal germ cell tumors are rare and have
been described in case reports. We report a pure intrarenal yolk sac
tumor in a 1-year-old boy who presented with a huge abdominal mass and
was operated for suspected Wilms tumor. The tumor exhibited
histopathologic and immunohistochemical features identical to those of
an endodermal sinus tumor of gonadal origin. The purpose of this
report is to add a rare tumor to the differential diagnosis of
pediatric renal neoplasms.
Endodermal
sinus tumor of the infant vagina treated exclusively with
chemotherapy.J
Pediatr Hematol Oncol. 2006
Nov;28(11):768-71.
Endodermal
sinus tumor (EST) of the vagina is a rare malignancy usually diagnosed
before 3 years of age. Historically, the approach to therapy has
included radical surgical resection, with adjuvant irradiation, and
chemotherapy. An infant presented with vaginal bleeding, imaging
evidence of a vaginal mass and an elevated alpha-fetoprotein level.
Examination under anesthesia with vaginal biopsies confirmed the
diagnosis of an EST (yolk sac) tumor of the vagina. After 5 cycles of
chemotherapy, the alpha-fetoprotein had normalized and repeat vaginal
biopsies for suspected residual disease was negative for malignancy.
To allow preservation of sexual and reproductive function,
chemotherapy as a sole modality of treatment for EST should be
considered.
Cytomorphology
of yolk sac tumor of the liver in fine-needle aspiration: a pediatric
case.Diagn
Cytopathol. 2006 Jun;34(6):421-3.
Yolk sac
tumors (YST) in extragonadal locations are rare. Cytologic diagnosis
of YST on fine-needle aspiration (FNA) smears may be a challenge to
the cytopathologist. Further neo-adjuvant therapy may be based on
cytologic diagnosis making accuracy important. We studied FNA material
from a hepatic mass in a pediatric patient to further define the
cytomorphologic features of hepatic YST. Features include large
pleomorphic balls of tumor cells with high nuclear to cytoplasmic
ratios, papillary or microglandular pattern of growth, cytoplasmic and
intranuclear vacuoles, and prominent nucleoli. Positive
immunohistochemical studies included alpha-fetoprotein, cytokeratin
AE1/AE3, and CAM 5.2, which are useful in supporting the diagnosis. We
report a pediatric patient in whom the diagnosis of hepatic YST was
made by cytologic, histologic, and immunohistochemical studies. The
subsequent liver biopsy was consistent with the FNA diagnosis. Our
findings may further help to characterize the cytomorphologic features
of this rare lesion.
Endodermal
sinus tumor of the vagina.
Indian J
Pediatr. 2005;72(9):797-8.
Malignant germ
cell tumors are rare tumors of childhood accounting less than 3% of
pediatric malignancies, and endodermal sinus tumor (EST) is the most
common histological subtype. The vagina is an extremely rare site for
germ cell tumors (GCT). A one-year female was admitted with history of
bleeding per vagina. She had pallor and a mass was palpable anteriorly
on rectal examination. Magnetic Resonance Imaging (MRI) showed a tumor
mass at the junction of cervix and vagina and biopsy was suggestive of
a vaginal EST. The serum alpha fetoprotein (AFP) was elevated. She
underwent vaginohysterectomy. The tumor was excised in toto and
patient received four courses of cisplatin, etoposide and bleomycin.
At one year of follow up, patient was symptom free and serum AFP
remained at normal level. EST of the vagina is a rare, highly
malignant GCT that exclusively involves children less than 3 years of
age. It is both locally aggressive and capable of metastasis. Even
though more conservative surgery is advised to maintain sexual and
reproductive function, at times, radical surgery is mandatory
depending on the infiltration of the tumor to the surrounding
structures. Simple tumor excision is not sufficient, as residual cells
induce recurrence and make chemotherapy ineffective. The serum AFP
level is a useful marker for diagnosis and monitoring the recurrence
of vaginal EST in infants.
Cytodiagnosis
of yolk sac tumor.Indian
J Pediatr. 2004 Oct;71(10):939-42.
To analyze
cytomorphologic features of yolk sac tumors of childhood. Four cases
of pediatric yolk sac tumor (YST), diagnosed by fine needle aspiration
cytology were reviewed (1998-2002). Age of patients ranged from 1(1/2)
to 5 years. Three cases presented clinically with an intra-abdominal
mass while one case presented with a testicular mass. Fine needle
aspirates had been obtained directly as well as under radiologic
(USG/CT) guidance. Smears were stained with H & E and Papanicolaou
stain. In all cases values of serum alpha-fetoprotein and hCG were
available preoperatively. Histopathologic diagnosis was correlated
with cytologic findings in all the cases. Cytologic examination showed
richly cellular smears with a combination of morphological patterns.
Characteristically, tumor cells were arranged in papillary groups,
tight cell clusters and formed acinar structures. Cells showed
enlarged, moderately pleomorphic, hyperchromatic nuclei and moderate
amount of cytoplasm, some of which displayed cytoplasmic vacuolation,
displacing the nuclei eccentrically. Preoperatively, serum
alpha-fetoprotein level was raised in all cases. Histopathology
confirmed the cytologic diagnosis. Yolk sac tumor is common among the
germ cell tumors of pediatric age group which presents a spectrum of
cytomorphologic features having important differences with other germ
cell neoplasm, e.g. embryonal carcinoma. Clinicoradiologic features
and tumor markers are additionally helpful for an accurate cytologic
diagnosis.
Intracardiac
yolk sac tumor and dysrhythmia as an etiology of pediatric syncope.Pediatrics.
2004 Apr;113(4):e374-6.
This report
describes a new etiology of pediatric syncope. Epilepsy, brain
anomalies, infection, electrolyte abnormalities, and trauma are
commonly identified etiologies of seizures in the pediatric
population. We report here a child with third-degree heart block and
right ventricular outflow tract obstruction related to an intracardiac
tumor presenting with syncope and seizure-like activity.
Echocardiography revealed a large (3 x 8-cm) intracardiac mass filling
the right atrium, extending across the tricuspid valve into the right
ventricle and crossing the atrial septum into the left atrium,
extending into the left ventricular outflow tract. She underwent
emergent cardiopulmonary bypass with removal of the majority of the
tumor mass, clearing both the left and right ventricular outflow
tracts of obstruction and repairing the tricuspid valve. Postoperative
cardiac conduction remained blocked and required permanent pacing. The
initial serum alpha-fetoprotein level was grossly elevated, and the
tumor showed characteristic histopathologic features of a yolk sac
tumor. Four years after the completion of her chemotherapy, she
remains clinically well, with no evidence of recurrent tumor by
echocardiography or radiographic studies, and her alpha-fetoprotein
remains in the normal range. The clinical manifestations of tumor
infiltration of the heart with complete heart block resulting in loss
of consciousness with tonic-clonic movements are detailed. Although
rare, cardiac syncope has multiple known causes and should be
suspected in any patient with sudden loss of consciousness and pallor.
In the pediatric population, cardiac rhythm disturbances are typically
the result, rather than the cause, of acute cardiac emergencies.
Pediatricians should be aware of depressed cardiac output and
dysrhythmias as etiologies of new-onset syncope. Evaluation should
include a cardiac assessment with electrocardiogram to exclude a
life-threatening arrhythmia as a potential cause.
Primary yolk
sac tumor of the rectum.Cancer
Invest. 2000;18(7):597-601.
Extragonadal
germ cell tumors are well recognized in men but have rarely been
reported in women. Reports have primarily focused on the pediatric
population and have suggested a poor prognosis for extragonadal yolk
sac tumors. A 23-year-old woman with a yolk sac tumor arising in the
rectum is described. A review of the English-language literature
(MEDLINE 1966-1998) regarding extragonadal germ cell tumors in females
is provided. Treatment with four courses of cisplatin, etoposide, and
bleomycin was followed by surgical resection of the involved area. No
residual tumor was identified. She remains disease free 3.5 years
later. Previous reports are limited by the small number of patients,
focus on the pediatric population, and treatment before the
availability of cisplatin. Extragonadal germ cell tumors in women are
extremely rare but can be successfully treated with aggressive
chemotherapy and surgery similar to testis cancer.
Transcription
factor GATA-4 is expressed in pediatric yolk sac tumors.Am
J Pathol. 1999 Dec;155(6):1823-9.
Yolk sac
tumors (YSTs) are malignant tumors that occur in the gonads of
children and young adults, and at extragonadal sites in young
children. The histological features of YSTs are variable and can be
superimposed on other germ cell tumor histologies. Malignant
endodermal cells within YSTs express alpha-fetoprotein, which can be
detected in tumor tissue or serum. However, additional markers of
endoderm differentiation would be beneficial for the classification of
these tumors. Transcription factor GATA-4 regulates the
differentiation and function of murine yolk sac endoderm, and its
expression correlates with proliferation and cell survival in certain
tissues. To see whether GATA-4 plays a role in human YSTs, we surveyed
its expression in human germ cell tumors and cell lines. Northern
analysis demonstrated expression of GATA-4 mRNA in four human germ
cell tumor lines exhibiting yolk sac endoderm differentiation. GATA-4
protein was detected in eight of nine pediatric YSTs by
immunohistochemistry. Three of five immature teratomas exhibited
GATA-4 in neural blastematous cells and in cylindrical epithelium,
whereas all 16 mature teratomas were devoid of GATA-4. We conclude
that GATA-4 is a clinically useful marker of human YSTs and speculate
that it may play a role in the maintenance of the malignant phenotype.
Testicular
yolk sac tumors in children.Zhonghua
Yi Xue Za Zhi (Taipei). 1999
Feb;62(2):92-7.
BACKGROUND:
Testicular tumors in children are uncommon, comprising about 1% of
pediatric malignancies. Yolk sac tumor is the most common malignant
testicular tumor in children. Because yolk sac tumor in children is
rarely seen, its treatment has been controversial. We reviewed the
records of 15 children with testicular yolk sac tumor treated at our
hospital in order to evaluate optimal management and treatment
outcome. METHODS: From February, 1981, to August, 1996, 15 children
with testicular yolk sac tumor were treated. Mean patient age at
diagnosis was 15.8 months (range, 7-22 months). Fourteen patients
presented with stage I disease and one presented with stage III
disease. Mean follow-up was 88 months (range, 2-156 months). RESULTS:
All 15 patients received radical inguinal orchiectomy as initial
treatment. Serum alpha-fetoprotein (AFP) concentrations were measured
in 14 stage I patients preoperatively and were elevated in all of
them. During follow-up, the one stage III patient died of the disease.
Of the remaining 14 patients, two (14.3%) had recurrence with elevated
AFP at three months and 10 months postorchiectomy, respectively. These
patients were managed with cisplatin-based combination chemotherapy.
To date, they are both alive with no further recurrence, and AFP
concentrations returned to normal after chemotherapy. Overall, of the
15 patients with testicular yolk sac tumor, 14 (93.3%) survived
without disease. CONCLUSIONS: Our results suggest that testicular yolk
sac tumor in children is a tumor with a favorable prognosis. Serum AFP
concentration is extremely useful in diagnosis and monitoring of
treatment response. Radical inguinal orchiectomy alone seems adequate
for patients with stage I disease if serum AFP concentrations return
to normal postoperatively. Cisplatin-based combination chemotherapy
should be administered in patients with tumor recurrence or
metastasis.
Immature
teratomas in children: pathologic considerations: a report from the
combined Pediatric Oncology Group/Children's Cancer Group.Am
J Surg Pathol. 1998 Sep;22(9):1115-24.
Pediatric germ
cell tumors (n = 135) with a major component of immature teratoma (IT)
registered on Pediatric Oncology Group/Children's Cancer Group
treatment protocols from 1990 to 1995 were reviewed. Sixty cases were
pure IT with no malignant component and 75 were mixed tumors with a
major component of IT. Foci of yolk sac tumor (YST) were present in
all 75 mixed tumors; additional malignant components were present in
15. The IT component was as follows: 47% grade 3, 29% grade 2, 24%
grade 1. There were no significant correlations between tumor grade
and patient age by specific subsets or overall (all p > 0.10).
Significant correlations were detected between stage and the presence
of foci of YST (p = 0.0145) and grade and the presence of foci of YST
(p < 0.001). Serum alpha-fetoprotein concentrations were elevated at
diagnosis in 96% of ovarian tumors with foci of YST and were mildly
elevated (< 60 ng/dL) in only 16% of tumors without YST. Overall 2- to
6-year survival rate was 96% and was related to the presence of YST.
Central pathologic review revealed aspects of morphologic diagnosis
that were most frequently misinterpreted by contributing pathologists.
These included the classification of differentiating tissues as
immature and the failure to recognize two well-differentiated patterns
of YST (the hepatoid pattern resembling fetal liver and the
well-differentiated glandular pattern resembling fetal lung or
intestine). Such foci were often overlooked. The authors conclude that
the presence of microscopic foci of YST, rather than the grade of IT,
per se, is the only valid predictor of recurrence in pediatric IT at
any site.
Clinical
experience with conservative surgery for vaginal endodermal sinus
tumor.J
Pediatr Surg. 1996 Feb;31(2):219-22.
Two cases of
vaginal endodermal sinus tumor (EST), a rare pediatric malignancy,
were managed with conservative surgery followed by adjuvant
chemotherapy. The first case involved a 9-month-old girl with EST of
the vagina, who was treated with a partial vaginectomy and VAC regimen
(vincristine, actinomycin D, cyclophosphamide) during a 2-year period.
The serum alpha-fetoprotein (AFP) level returned to normal after
excision of the tumor, and it remained normal throughout the treatment
period. There was no evidence of disease 30 months after diagnosis.
The second case involved an 8-month-old girl with EST of the vagina,
who was treated with local excision of the mass through a vaginotomy.
The VAC regimen was administered, but the serum AFP level remained
elevated. A follow-up abdominopelvic computed tomography scan, taken 4
months after the operation, showed local recurrence of the tumor. The
VAC regimen was then changed to a BEP regimen (bleomycin, etoposide,
cisplatin). The serum AFP level returned to normal after 2 courses of
the new regimen, and no tumor was visible on the follow-up magnetic
resonance imaging study. For vaginal EST, primary conservative surgery
and adjuvant chemotherapy are attractive measures to preserve both
reproductive and sexual function. The extent of conservative surgery
requires at least a partial vaginectomy. Simple tumor excision may not
be adequate to achieve cure or to prevent local recurrence, even with
adjuvant chemotherapy. The serum AFP level is useful for diagnosing
and monitoring vaginal EST in the infant.
Detection of
chromosome aberrations in paraffin sections of seven gonadal yolk sac
tumors of childhood.Hum
Genet. 1995 Dec;96(6):644-50.
Yolk sac
tumors are the most frequent kind of malignant pediatric germ cell
tumor and may have a fundamentally different pathogenesis than adult
germ cell tumors. Since few cytogenetic studies have been performed so
far, in situ hybridization was applied to interphase cell nuclei of
seven gonadal yolk sac tumors of childhood in routine
paraffin-embedded tissue sections. The panel of chromosome-specific
DNA probes was selected on the basis of their relevance in adult germ
cell tumors and consisted of five DNA probes specific for the (peri)centromeric
regions of chromosomes 1, 8, 12, 17 and/or X and/or one DNA probe
specific for the subtelomeric region of chromosome 1 (p36.3). As in
adult germ cell tumors, all pediatric gonadal yolk sac tumors had an
increased incidence of numerical chromosome aberrations. All tumors
showed an overrepresentation of at least three chromosomes. Gains of
chromosome 12, which is highly specific in adult germ cell tumors,
were diagnosed in six pediatric gonadal yolk sac tumors. The DNA
indices determined in the paraffin-embedded tumor material correlated
well with the in situ hybridization findings. A chromosome was either
over- or underrepresented, compared with the corresponding DNA
indices, in only a few cases. The short arm of chromosome 1 in adult
germ cell tumors is often involved in structural aberrations. In
pediatric germ cell tumors, the short arm of chromosome 1 is also a
nonrandom site of structural aberrations. Moreover, the presence of a
deletion at 1p36.3 in four out of five tumors suggests that the loss
of gene(s) in this region is an important event in the pathogenesis of
gonadal yolk sac tumors of childhood.
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