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Intracranial mixed vascular malformations (MVMs) are defined as any combination of a developmental venous anomaly (DVA), cerebral cavernous malformation (CCM), capillary telangiectasia (CTG), or arteriovenous malformation (AVM) within a single, contiguous lesion. However, most MVMs described in the literature contain only 2 pathologically discrete malformations; juxtaposition of 3 or more abnormalities in a single lesion remains exceedingly rare. We present the case of a 19-month-old female with new onset focal seizures and a 4-cm right basal ganglia lesion initially believed to be an embryonal neoplasm. She subsequently underwent gross total resection (GTR) of the lesion via a transsylvian-transinsular approach. Intraoperatively, the lesion appeared to be heterogenous and highly vascular, with areas of purplish-gray friable tissue. Pathology confirmed the lesion to be a MVM containing a CCM, CTG, and a DVA. This appears to be the first reported case of such a lesion confirmed on pathology in the literature.

Immature gastric teratoma is an extremely rare embryonal neoplasm. Although teratomas have been identified in most body regions, the sacrococcygeal is the most commonly involved site, followed by cervicofacial, mediastinal, gonadal, and retroperitoneal regions. The stomach is rarely involved, accounting for

According to the World Health Organization, medulloepithelioma belongs to the embryonal neoplasm entity. It is a very rare, highly malignant tumor typically affecting infants and young children. Usually, the tumor arises in the eye or in the central nervous system; a peripheral location has been rarely reported without an established treatment. The recognition and separation of this neoplasm from other differential tumors are mandatory for better understanding of its biology and determination of optimal treatment. This paper reports a case of an ectopic intrapelvic medulloepithelioma with liver metastasis in a 3-year-old girl.

Atypical teratoid/rhabdoid tumor (AT/RT) represents a highly malignant and aggressive embryonal neoplasm of the central nervous system (CNS). Histologically it consists of rhabdoid cells with a varying combination of neuroectodermal, epithelial, and mesenchymal tissue. Histologic features and the poor clinical outcome indicate that this tumor corresponds to World Health Organization grade IV. AT/RT mainly occurs in children 3 years of age and has only been rarely described in adults. It often arises from the posterior fossa of infants and has the tendency to spread through the subarachnoid space. In contrast to pediatric cases, most of the AT/RTs in adults are located in the cerebral hemispheres; infratentorial appearance or localization in the spinal cord is relatively uncommon. Likewise there are only a few reports of AT/RT in the pineal region. We present another interesting case of this tumor occurring in the pineal region of a 19-year-old man and discuss our case in the context of the current literature. The patient is alive 18 months since his initial diagnosis without any signs of tumor relapse.

Atypical teratoid/rhabdoid tumor (AT/RT) is a distinct tumor of young children based on morphological, immunohistochemical, and cytogenetic characteristics. It is included in the World Health Organization classification as grade IV embryonal neoplasm. Sometimes it is difficult to recognize by using only histopathologic criteria. Microscopically, AT/RT contains sheets of rhabdoid cells, primitive neuroectodermal cells, mesenchymal cells, or epithelial cells. The immunohistochemical profile is complex, but the absence of nuclear expression of INI1 has emerged as a critical tool for accurate AT/RT diagnosis.

Neuroblastoma has a notorious reputation among solid tumors of childhood because of its frequently massive and widespread tumor burden. Yet stage for stage, this embryonal neoplasm of the sympathet...

Embryonal tumor with abundant neuropil and true rosettes has been recently defined as a distinct central nervous system embryonal neoplasm, although it was initially regarded as a subtype of central nervous system primitive neuroectodermal tumor. To date 70 cases have been reported. We have performed a literature review and we present 2 new cases. Analysis of the reported data revealed that radiotherapy, tumor excision and high-dose adjuvant chemotherapy with sequential autologous hematopoietic stem cell rescue have a prognostic significance.

Introducción: Los teratomas sacrococcígeos, constituyen una neoplasia que pese a su baja frecuencia, es una de las más comunes en neonatos. Se describe un caso de un feto muerto con hallazgos histopatológicos de un teratoma sacro coccígeo inmaduro.Pacientes y Métodos: Mujer de 26 años de edad en su cuarto embarazo de 32 semanas de gestación, quien presenta amenaza de parto pre término. Se le realiza una ecografía obstétrica que informa la presencia de una masa sacra fetal, e hidrops fetal. Se realizó cesárea y se obtiene un óbito fetal de sexo femenino. El estudio anatomopatológico evidenció un teratoma sacrococcígeo inmaduro. Conclusiones: En los teratomas sacrococcígeos fetales, el desarrollo de hidrops fetal, insuficiencia cardiaca y gran tamaño tumoral son indicadores de mal pronóstico. El diagnóstico prenatal, la planificación del parto, la estabilización perinatal y la resección quirúrgica del tumor, pueden hacer posible la sobre vida en estos pacientes. Salud UIS 2010; 42: 127-131 Introduction: Sacrococcygeal teratomas are neoplasm that despite their low frequency, are one of the most common in newborns. We describe a case of a dead fetus with an immature sacrococcygeal teratoma, its radiological and histological findings. Patients and Methods: A 26 years old woman in the fourth pregnancy of 32 weeks' of gestation, who presented the threat of pre-term birth. We performed obstetric ultrasound reporting the presence of a fetal sacral mass and fetal hydrops. Caesarean section was run and got a female fetal death. Pathologic study showed immature sacrococcygeal teratoma. Conclusions: In fetal sacrococcygeal teratomas, the development of fetal hydrops, heart failure and large tumor size are indicators of poor prognosis. Prenatal diagnosis, planning delivery, the postnatal stabilization period and surgical resection of the tumor, can make survival possible in these patients. Salud UIS 2010; 42: 127-131.

A five days old 3.3 kg infant, preterm male was admitted at the neonatal ward MNH five hours after birth from a peripheral hospital for further management of congenital hernia and general care. Examinations revealed macroglosia, earlobe crease, large exomphalous, small penis, hypospadius and cryptochidism. Investigations portrayed neonatal hypoglycaemia and distended bowel by x-ray. The ultimate diagnosis reached was Beckwith Waidemann Syndrome. The management for such a condition is mainly supportive to maintain euglycaemia, surgical intervention when indicated followed by monitoring the possibility of embryonal neoplasm later by ultrasonography surveillance.

Atypical teratoid/rhabdoid tumor (ATsRT) is a rare tumor and extremely aggressive embryonal neoplasm of the central nervous system. Brain tumors in infant are suggestive of some oncogenic prenatal factors. We report on a case of ATRT in a 4-month-old infant conceived by in vitro fertilization (IVF). Some previous reports have raised a question about the possible relation between IVF and childhood cancer, particularly embryonal tumors. Report of such cases may provide some evidence to identify if there is a real association between congenital tumors and IVF.

International audience; An atypical teratoid/rhabdoid tumor of the central nervous system is an aggressive infantile embryonal neoplasm, usually presenting as an infratentorial and intraparenchymatous lesion. We report on magnetic resonance imaging findings of a 22-month-old boy with a biopsy-proven primary rhabdoid tumor, presenting as a single intracranial supratentorial extra-axial mass. Based on the patient's age and imaging features (perfusion, diffusion magnetic resonance imaging, and magnetic resonance spectroscopy), a diagnosis of atypical teratoid/rhabdoid tumor was more accurate than diagnoses of meningioma and primitive neuroectodermal tumor. Although this entity is relatively rare, it should be considered in the differential diagnosis of dural-based, space-occupying central nervous system lesions.

Rhabdomyosarcoma is the common malignant soft tissue tumor of childhood. Twelve of 67 cases recently reviewed from this institution arose in the bladder or prostate (6 are surviving). Until recently and regardless of the primary site, the reports of successful treatment of this aggressive embryonal neoplasm have been limited to very small series or to isolated cases managed by radical mutilative surgery. Now, combined therapy, as has proven so effective in Wilms' tumor, and utilizing “reasonable surgery” (excision of all gross tumor), tolerable x-ray therapy (Cobalt 60 2-4000 R according to age in 3–4 weeks), and repeated courses of Actinomycin D and Vincristine (9-week cycles for at least a year), is recommended. At Ohio State University the curability rate in all types of rhabdomyosarcoma including all bladder and prostatic lesions has improved spectacularly from 14% prior to 1967 to 71% (corrected survival by life table method) in the last 38 cases treated.