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PubMedDate not supplied

Abdominal metastases of pediatric brain tumors via ventriculo-peritoneal shunts.

Internal drainage of cerebrospinal fluid (CSF) to the abdominal cavity via a ventriculo-peritoneal shunt (VPS) is a procedure that is commonly used for the treatment of obstructive hydrocephalus. As this condition is often caused by brain tumors blocking the natural CSF pathways, a VPS, as an artificial anastomosis, can provide the means for tumor cells to be spread with the CSF. A review of the literature reveals 35 VPS-related abdominal metastases from pediatric brain tumors; 17 in patients aged 0-9 (group A) and 18 in patients aged 10-18 years (group B); the mean age of male patients was 10.5, and that of female patients, 7 years. The male-to-female ratio was 1.9 (group A 1.1, group B 3.5), and the mean interval between shunt operation and diagnosis of metastases, 16.7 months (group A 11.6, group B 22.6 months; boys 21.6, girls 7.5 months). During the observation period, 22/30=73.3% of the patients died (group A 13/15=86.7%, group B 9/15=60%; boys 13/21=61.9%, girls 9/9=100%); their mean survival time after shunting was 18.7 months (group A 15.7, group B 23.1 months; boys 25.5, girls 9 months). The four most common sources of metastases were germinomas (9 cases=25.7%; group A none, group B 9), medulloblastomas (8 cases=22.9%, group A 7, group B 1), endodermal sinus tumors (5 cases=14.3%, group A 1, group B 4), and astrocytomas (4 cases=11.4%, group A 4, group B none). Metastases via VPS are rare, but should be considered as a possible complication and mode of systemic spread in children with primary intracranial malignancy. They have a more favorable prognosis in boys and in the second decade of life.

Adolescent↗

PubMed1996

Perinatal management of a lingual teratoma.

BACKGROUND: Teratomas of the head and neck are rare and occur almost exclusively in neonates. Prenatal diagnosis of these tumors allows for a carefully planned delivery that maintains an open airway and potentially improves perinatal outcome. We report the perinatal management of a huge intrapharyngeal and intra-oral teratoma that had a broad connection to the base of the tongue. CASE: An anterior neck mass (5 x 5 cm) in an otherwise normal-appearing fetus was detected at 19 weeks' gestation. The mass increased in size over the next 10 weeks to 8 x 6.8 x 4.3 cm. Marked fetal head deflexion was noted along with concomitant hydramnios. The mother had spontaneous rupture of membranes with preterm labor at 29 weeks' gestation. A 1860-g male neonate was delivered by classical cesarean delivery. A tracheostomy was performed in the delivery room for ventilation after the upper airway could not be accessed by bronchoscopy. Histologic examination after surgical excision confirmed a congenital teratoma with immature neuroectodermal tissue and alpha-fetoprotein-bearing endodermal sinus tumor components with exclusively polyvesicular vitelline characteristics. The origin of the pharyngeal mass was the base of the tongue. CONCLUSION: The prenatal diagnosis of a pharyngeal teratoma should prompt a careful delivery plan to optimize perinatal outcome.

Adult↗

PubMed2001

Weaver syndrome with neuroblastoma and cardiovascular anomalies.

We report on an infant with Weaver syndrome, neoplasia and cardiovascular anomalies. Stage 4S neuroblastoma underwent spontaneous resolution. Three neoplasms have been reported in Weaver syndrome: another stage 4S neuroblastoma [Muhonen and Menezes, 1990: J Pediatr 116:596-599], an ovarian endodermal sinus tumor [Derry et al., 1999: J Med Genet 36:725-728], and a sacrococcygeal teratoma [Kelly et al., 2000: Am J Med Genet 95:492-495]. No case was associated with cardiovascular anomalies. Our patient had VSD and PDA, and although several other patients with Weaver syndrome have had cardiovascular anomalies, they were shown not to have neoplasia.

Abnormalities, Multiple↗

PubMed1976

Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases.

Fifty-eight immature ovarian teratomas were studied. Neoplams with other germ cell elements (endodermal sinus tumor, choriocarcinoma, and dysgerminoma) were excluded so that the clinical and pathologic features of "pure" immature teratomas could be defined and correlated with the prognosis. The primary tumors and their metastatic growths were graded from 0 to 3. Forty were stage I; nine, stage II; and nine, stage III. The size and stage of teratomas were related to survival, but it was the grade of the primary tumor that best determined the likelihood of extraovarian spread, and it was the grade of the metastases that related best to the subsequent course. Actuarial survival was 63% at 5 years and also at 10 years. Regardless of the grade of the primary tumor, only one of six with grade 0 metastases progressed, and that neoplasms may not have been adequately sampled. Two of five neoplasms having grade 1 metastases did not progress, and two of six patients with grade 2 metastatic growths were living after relatively long intervals. All seven patients with grade 3 metastases died with tumor, none surviving more than 2.1 years. Survival of patients with grade 1, 2, and 3 neoplasms was 81, 60, and 30% respectively. The importance of adequate sampling of primary tumor and metastases for estimating prognosis and determining therapy is stressed.

Adnexa Uteri↗

PubMed1985

Chemotherapy-associated Raynaud's phenomenon in a two-year-old girl.

Raynaud's phenomenon occurred in a 2-year-old girl 17 months after starting multiagent chemotherapy for an endodermal sinus tumor of the vagina. Symptoms began 2 months after receiving her final dose of bleomycin, and gradually resolved over a 6-month period despite continuing vinblastine. Chemotherapy-associated Raynaud's phenomenon appears to be unusual in young children, although mild episodes of vasospasm may not be reported by children or their parents. Specific questions regarding symptoms of Raynaud's phenomenon should be asked of all children receiving vinblastine and bleomycin therapy.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2006

Immune thrombocytopenia following successful treatment of cancer in children.

A predisposition to developing immune thrombocytopenia (ITP) has not been reported in survivors of childhood cancer. We report a case series of childhood cancer survivors who developed an isolated thrombocytopenia in the presence of a normocellular bone marrow. Five children, two with endodermal sinus tumors and three with acute lymphoblastic leukemia, developed ITP at a median of 4 years (range: 0.2-8 years) after completion of therapy. We suggest the association of ITP in survivors of childhood malignancy may not be co-incidental as chemotherapy may cause persistent immune dysfunction.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1993

Five different histological subtypes of germ cell malignancies in an XY female.

A case of a 19-year-old female with 46 XY gonadal dysgenesis and five different histological subtypes of germ cell malignancies is described. Both adnexa were removed, preserving the uterus. Pathology revealed gonadoblastoma with dysgerminoma differentiation present in both gonads and in the left gonad mature teratoma, embryonal carcinoma, and endodermal sinus tumor were identified as well. She received no adjuvant treatment and has remained well 30 months after diagnosis.

Adnexa Uteri↗

PubMed1998

Primary yolk sac tumor of the endometrium: a case report and review of the literature.

A 49-year-old woman was admitted with abnormal vaginal bleeding of 5 months duration. Hysteroscopy showed polypoid formations in the uterine cavity. Biopsy of a polyp revealed an anaplastic malignant tumor. A total hysterectomy with bilateral salpingo-oophorectomy and iliac lymphadenectomy were performed. A polypoid soft white-yellowish tumor originated from the endometrium. The biopsies plus sections of the operative specimen were histologically similar and displayed typical features of yolk sac tumor (YST) with tubulo-papillary pattern. Schiller-Duval bodies and PAS-positive, diastase-resistant, hyaline droplets were present. There was no neoplastic involvement of the cervix, ovaries, and iliac lymph nodes. Tumor cells and hyaline globules were strongly immunoreactive with alpha-fetoprotein antibody. The patient refused chemotherapy and was subsequently treated by external radiotherapy. The patient remained free of disease 28 months after surgery. There are only four additional cases of primary YST of the endometrium reported in literature. On the basis of the small number of cases reported, such tumors appear to have clinical and pathological features similar to their ovarian counterparts.

Biomarkers, Tumor↗

PubMed2006

Vaginal discharge and bleeding in girls younger than 6 years.

PURPOSE: Persistent unexplained vaginal discharge or bleeding in the pediatric population may be the only manifestation of a serious underlying medical or social problem. Therefore, these symptoms require careful and complete evaluation to identify the primary pathology accurately. We retrospectively reviewed charts of patients who presented for evaluation of persistent vaginal discharge or bleeding to determine if noninvasive imaging was a sensitive means of screening for gynecological pathology. MATERIALS AND METHODS: The records of 24 girls younger than 6 years who presented with vaginal discharge or bleeding were reviewed retrospectively. All patients were evaluated with noninvasive imaging, a pelvic examination while under anesthesia, vaginoscopy and cystoscopy. RESULTS: Noninvasive imaging was useful in identifying 5 of 7 vaginal foreign bodies. However, noninvasive imaging identified only 2 of 6 malignancies. These malignancies consisted of rhabdomyosarcoma (3 patients) and endodermal sinus tumor (3). Two girls also had benign vaginal mullerian papillomas that were not identified by noninvasive imaging. Noninvasive imaging did not aid in the diagnosis of sexual abuse. CONCLUSIONS: Based on these data, we recommend that all girls younger than 6 years who present with persistent vaginal discharge or bleeding be evaluated with pelvic examination while under anesthesia, to be followed by vaginoscopy and cystoscopy if no readily identifiable pathology is found by simple genital examination alone, regardless of the results of noninvasive imaging studies.

Child, Preschool↗

PubMed2004

Adjuvant therapy of completely resected dysgerminoma with carboplatin and etoposide: a trial of the Gynecologic Oncology Group.

OBJECTIVE: This study was designed to evaluate the effect of adjuvant chemotherapy with carboplatin and etoposide in patients with completely resected stage IB-III dysgerminoma. METHODS: Eligible patients were treated with three courses of carboplatin 400 mg/m(2) on day 1 plus etoposide 120 mg/m(2) on days 1, 2, and 3 every 4 weeks for three courses. RESULTS: Forty-two patients were entered on this trial, of whom 39 were eligible. No patient suffered a recurrence of dysgerminoma, but one patient ultimately died of lung adenocarcinoma. One patient was excluded on pathology review (elements of endodermal sinus tumor were present) developed recurrent tumor and died despite further therapy. As expected, the regimen was well tolerated. Median follow-up of surviving patients is 7.8 years (range: 2.86 months to 10.92 years). CONCLUSION: The regimen used in this study is an alternative to cisplatin, etoposide, and bleomycin (BEP) for selected patients for whom minimizing toxicity (particularly neuropathy) is critical or for whom reduction in the number of treatment days is important.

Adolescent↗

PubMed1999

Yolk sac tumor of the stomach with an adenocarcinomatous component: a case report with immunohistochemical analysis.

A 56-year-old male treated for a gastric yolk sac tumor with an adenocarcinomatous component is described. A mixed area of reticular and glandular neoplastic components was morphologically identified in this tumor. Immunohistochemically, the yolk sac tumor expressed alpha-fetoprotein (AFP), placental alkaline phosphatase (PLAP), and cytokeratin, but was negative for carcinoembryonic antigen (CEA). The adenocarcinoma was positive for CEA and cytokeratin, partially positive for PLAP, and negative for AFP. In the mixed area, the tumor cells were positive for cytokeratin, weakly expressed AFP and PLAP, and sporadically stained for CEA both in the reticular and glandular components. This area was identified as a transitional area of the yolk sac tumor and adenocarcinoma. These findings demonstrate that the yolk sac and adenocarcinomatous components are closely related. It also suggests that the tumor arose from multipotential neoplastic mucosal epithelial cells with both yolk sac and gastric mucosal phenotypes.

Adenocarcinoma↗

PubMed1987

Extracellular hyaline bodies are basement membrane accumulations.

Diverse hyaline bodies occurring in various diseases were investigated immunocytochemically with antibodies to type IV collagen and laminin. These hyaline bodies included those found predominantly extracellularly in adenoid cystic carcinomas, endodermal sinus tumors, Spitz nevi, lichen planus, diabetic glomerular nodular sclerosis, and odontogenic cysts, and those found predominantly intracellularly in alcoholic liver disease, Kaposi's sarcoma, and malignant fibrous histiocytoma. The hyaline bodies found in the first six diseases displayed intense immunoreactivity for the basement membrane components, type IV collagen, and laminin, whereas the hyaline bodies occurring in the latter three diseases were unreactive. These findings suggest that a common histogenesis of the extracellular hyaline body is the stimulatory effect of an altered growth state (hyperplasia, neoplasia, or endocrinopathy) on basement membrane synthesis in cells that normally produce a basement membrane.

Basement Membrane↗

PubMed1999

Mixed malignant germ cell tumor of the fallopian tube.

Immature teratomas of the fallopian tube are exceedingly rare with only three reported cases in the English literature. Reported here is a case of primary mixed malignant germ cell tumor of the fallopian tube composed of immature teratoma and yolk sac tumor.

Adult↗

PubMed1983

Ovarian masses in the child and adolescent.

Over a five-year period, we treated 16 children ranging in age from 8 weeks to 16 years for ovarian masses. The most frequent clinical manifestation was abdominal pain and/or mass. Ultrasonography correctly localized the lesion and delineated its nature in nine of ten cases. Operation in 15 showed teratoma (six benign, two malignant), endodermal sinus tumor (two), dysgerminoma (two), follicular cyst (two), and leukemic ovarian infiltrate (one). Adnexal torsion was present in three. Three children had tumor metastases at initial operation. Resection of the tumor with conscious preservation of uninvolved pelvic structures was performed if possible. For unresectable tumor, debulking was undertaken. Multiagent chemotherapy was used for all malignant tumors. Survival was 94%. The proper therapy for ovarian lesions in children requires familiarity with the unique nature of these lesions, as well as consideration of future endocrine and reproductive function.

Adnexal Diseases↗

PubMed2005

Mucinous carcinoid as an unusual manifestation of endodermal differentiation in ovarian yolk sac tumors.

We present, for the first time, two yolk sac tumors (YST) in women 37 and 18 years of age, one with a typical parietovisceral pattern and the other with a glandular pattern, which were associated with extensive areas of mucinous carcinoid (MC). The tumor in the first case had numerous nodules of tubulopapillary YST that merged with well-differentiated MC. This patient responded well to chemotherapy. The tumor in the second case consisted of an AFP-positive glandular YST, with a glandulopapillary pattern closely resembling fetal lung type adenocarcinoma, coexisting with an AFP-negative, cytokeratin 20-positive, atypical MC; transitional areas between the two components were also seen. In the material from the recurrences and metastases; however, no YST was present, the atypical MC having become the predominant component including areas that had become carcinomatous. There was a poor response to various chemotherapeutic regimens. AFP levels became negative during the course of disease paralleling the disappearance of the YST component and the overgrowth of an increasingly anaplastic MC. The patient died 1 year after diagnosis. We think that, in these cases, MC represented an unusual form of endodermal differentiation of the YST. It is important to differentiate the yolk sac and carcinoid components due to their different responses to chemotherapy and to evaluate the possibility of mucinous carcinoid developing into a highly aggressive carcinoma.

Adolescent↗

PubMed2007

Alpha-fetoprotein-producing serous carcinoma of the uterus metastasizing to the ovaries, mimicking primary ovarian yolk sac tumor: a case report and review of the literature.

In the uterus, most alpha-fetoprotein (AFP) producing neoplasms belong to the categories of malignant mixed muellerian tumor, hepatoid carcinoma, and yolk sac tumor. We describe the case of a 44-year-old woman who presented with vaginal bleeding, pelvic mass, and preoperative elevated AFP serum level, clinically suggestive of a primary ovarian yolk sac tumor. However, histological examination revealed a uterine AFP-producing papillary serous carcinoma, which has metastasized to the ovaries. Upon review of the literature on primary endometrial neoplasms with AFP production, 2 categories with possibly different histogenesis and biological behavior become evident: the primary yolk sac tumor of the uterus in young patients (range, 24-49 years; mean, 34 years) and the common high grade endometrial carcinoma with yolk sac dedifferentiation or aberrant AFP production in elderly patients (range, 55-69 years; mean, 63.7 years). In addition to being only the second case of uterine AFP-producing papillary serous carcinoma, this case is unusual for its clinical and radiological presentation as well as the relatively young age of the patient.

Adult↗

PubMed1983

Computed tomography in the evaluation of the gluteal region.

The role of computed tomography (CT) in the evaluation of the gluteal region was assessed. Six cases of gluteal masses were studied preoperatively by CT; several were also studied with conventional radiographic methods, including barium enema, cystogram, and intravenous urogram. Our case material included an epithelioid sarcoma, Ewing's sarcoma, endodermal sinus tumor, cystic hygroma, neurofibromatosis, and a normal variant. The conventional radiologic studies were normal or demonstrated nonspecific soft tissue density mass effect. By comparison, CT, with its cross-sectional imaging capability, provided unique diagnostic information. CT depicted the presence and origin of a mass, provided tissue characterization, and showed the extent of the lesion, often demonstrating the gluteal mass as an extension of an intrapelvic lesion. CT was valuable in monitoring tumor response to therapy, detecting recurrences, and excluding normal variants as the cause for a gluteal mass. The information provided by CT was important in treatment planning.

Adolescent↗

PubMed1992

Pineal region masses: differential diagnosis.

Most pineal region masses are malignant germ cell neoplasms that occur in young male patients. The most common is a germinoma, which is a homogeneous mass with signal intensity and attenuation similar to those of gray matter; the mass engulfs a densely calcified pineal gland. Teratomas are multilocular heterogeneous masses containing lipid areas. Other types of pineal region masses include choriocarcinoma, endodermal sinus tumor, and embryonal carcinoma. Pineal parenchymal neoplasms are usually either pineocytomas or pineoblastomas, which may "explode" preexisting pineal calcifications. Unlike the germinomas, they have no sexual predilection and may be seen in patients who are 20 years of age or older. Although the correct histologic diagnosis may be suggested with a careful evaluation of the morphologic features, attenuation, and signal intensity characteristics, very few of these tumors have a truly pathognomonic imaging pattern. Thus, histologic verification is necessary for most pineal region masses that appear to be neoplastic.

Brain Diseases↗