Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal central nervous system tumor commonly affecting children <3 years of age. It roughly constitutes 1%-2% of all pediatric central nervous system tumors. Recent data show that it is the most common malignant central nervous system tumor in children <6 months of age. Management of this aggressive tumor is associated with a myr...

The rare pediatric embryonal tumors retinoblastoma, medulloblastoma and neuroblastoma derive from neuroectodermal tissue and share similar histopathological features despite different anatomical locations and diverse clinical outcomes. As metabolism can reflect genetic and histological features, we investigated whether the metabolism of embryonal tumors reflects their similar histology, shared ...

Askin's tumor is a primitive neuroectodermal tumor developing from the soft tissues of the chest wall. Its diagnosis approach is complex and requires a multidisciplinary team. Given the rarity of this entity, no regimen has been validated in the literature. We report two cases of Askin's tumor with a major response to polychemotherapy and surgical resection in one case. These cases show that tr...

Primitive neuroectodermal tumors (PNETs) are small round-cell tumors that often show neuroectodermal differentiation. They can be either central or peripheral. Central PNETs are derived from the neural tube predominantly involving the brain or spinal cord. Peripheral PNETs (pPNETs) are derived from the neural crest involving the sympathetic nervous system, bone, or soft tissue [1]. The most com...

A neoplastic mass compressing the left cerebellar hemisphere and hindbrain was observed at trimming in a 3&frac12;-year-old male cynomolgus monkey from a control dose group. Microscopically, the neoplastic mass was nonencapsulated, invasive, and showed two morphological patterns. The predominant area consisted of densely packed undifferentiated, polygonal to spindle cells arranged in vague shee...

Neuroblastoma (NBL) is a neuroectodermal tumor derived from neural crest cells. The biological and clinical behavior of NB is extremely heterogenous. We here report a newborn who presented as 4S NBL with a massive hepatomegaly resulting in IVC syndrome.

Primary primitive neuroectodermal tumors (PNETs) are rare and high-grade malignant tumors that mostly occur in children and young adults. The most common sites are the trunk, limbs, and retroperitoneum. Herein, we present a case of a PNET involving the cervix uteri in a 27-year-old woman. The lesion showed characteristic histologic features of a PNET and was positive for the immunohistochemical...

Primitive neuroectodermal tumors (PNETs) are small round cell malignant tumors classified as part of the Ewing’s sarcoma family of neoplasms, which represents approximately 1% of all sarcomas1. Predominantly occurring in soft tissues along the extremities, they have also been reported in a variety of organs such as kidney, urinary bladder, testis, ovary, uterus, heart and lung2, 3. Pancreatic P...

Melanotic neuroectodermal tumor of infancy is a rare congenital neoplasm involving the head and neck in young patients. The clinical assessment, histologic diagnosis, and management is reviewed, with an emphasis on different treatment alternatives in two new case reports.

BACKGROUND : Primitive peripheral neuroectodermal tumors (PNETs) of the uterus are rare. Recent data have demonstrated improved response rates with adjuvant chemotherapy. CASE : A 26-year-old, gravida 2, para 1, Filipina female underwent an emergent cesarean section for fetal indications. Intraoperative findings were remarkable for a soft tissue mass in the lower uterine segment. Histologic f...