• Along with neurofibroma, schwannoma constitutes one of the two most common benign peripheral nerve sheath tumors. (medscape.com)
  • [ 12 ] There have also been reports of nerve sheath tumors exhibiting histologic hybrid features of schwannoma and soft-tissue perineurioma. (medscape.com)
  • 6, 11] This finding adds credence to a neural crest origin because elevated VMA has been reported in neuroblastoma, ganglioneuroblastoma, pheochromocytoma, and other neural crest tumors. (medscape.com)
  • Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of newborn infants. (medscape.com)
  • Some of the terms applied to this lesion included pigmented ameloblastoma, retinal anlage tumor, melanotic progonoma, melanotic adamantinoma, and pigmented epulis of infancy. (medscape.com)
  • In 1966, Borello and Gorlin reported a case with high urinary excretion of vanillylmandelic acid (VMA), suggesting a neural crest origin, and they proposed the term melanotic neuroectodermal tumor of infancy. (medscape.com)
  • 6] Since then, numerous histochemical, immunohistochemical, electron microscopic, and tissue culture studies have supported the neural crest origin and confirmed the preferred term of melanotic neuroectodermal tumor of infancy. (medscape.com)
  • Several patients with melanotic neuroectodermal tumor of infancy (MNTI) have demonstrated a high urinary excretion of VMA. (medscape.com)
  • Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm and approximately 485 cases have been reported in the literature to date worldwide. (medscape.com)
  • 13] A few cases of melanotic neuroectodermal tumor of infancy (MNTI) have been reported in adults, notably, a 23-year-old man, a 39-year-old woman, and a 67-year-old woman. (medscape.com)
  • Although melanotic neuroectodermal tumor of infancy (MNTI) is classified as a benign lesion, it is often clinically worrisome because of its rapid onset and alarming local growth rate. (medscape.com)
  • More than 90% of cases of melanotic neuroectodermal tumor of infancy (MNTI) occur in the head and neck region, with a striking predilection for the anterior portion of the maxilla. (medscape.com)
  • Melanotic neuroectodermal tumor of infancy presents as a rapidly growing bluish mass on the anterior aspect of the maxilla. (medscape.com)
  • Consider clinical, radiographic, laboratory, and histologic findings when establishing a proper differential diagnosis for melanotic neuroectodermal tumor of infancy (MNTI). (medscape.com)
  • It is generally accepted that in embryogenesis, the Schwann cells are derived from the neural crest and are of neuroectodermal origin. (medscape.com)
  • The first section will cover those tumors that characteristically are associated with the pediatric population. (abdominalkey.com)
  • Comparative RNA-seq analysis aids in diagnosis of a rare pediatric tumor. (beds.ac.uk)
  • 3, 4, 5] These terms reflected theories of suspected origin from the odontogenic apparatus, the pigmented anlage of the retina, or the sensory neuroectodermal tissues. (medscape.com)
  • Wilms tumor is believed to arise from embryonic tissues called nephrogenic rests that fail to undergo normal involution ( 7 ). (abdominalkey.com)
  • The tumor is usually circumscribed by a pseudocapsule formed of compressed renal and perirenal tissues. (abdominalkey.com)
  • Ewing tumors are most common in bone but can also form in soft tissue. (dana-farber.org)
  • Blau syndrome Chronic infantile neurologic cutaneous and articular syndrome Familial cold urticaria (familial cold autoinflammatory syndrome) Familial Mediterranean fever Hyper-IgD syndrome Majeed syndrome Muckle-Wells syndrome TNF receptor associated periodic syndrome (familial Hibernian fever, TRAPS, tumor necrosis factor receptor associated periodic syndrome) Chronic blistering cutaneous conditions have a prolonged course and present with vesicles and bullae. (wikipedia.org)
  • Patients with Beckwith-Wiedemann syndrome and Denys-Drash syndrome have an increased risk of developing Wilms tumor ( 5 , 6 ). (abdominalkey.com)
  • Wiedemann-Beckwith syndrome is related to abnormalities on chromosome 11p15 and characterized by multiple craniofacial anomalies, abdominal wall defects, and tumors of the genitourinary tract, liver, adrenal gland, and central nervous system among other abnormalities. (abdominalkey.com)
  • The presence of a noninvasive tumor next to a peripheral nerve suggests the diagnosis of neurilemmoma. (medscape.com)
  • The same age differential is noted with respect to the more common odontogenic tumors (eg, ameloblastoma, odontoma, adenomatoid odontogenic tumor, calcifying epithelial odontogenic tumor, ameloblastic fibroma, odontogenic myxoma, odontogenic fibroma). (medscape.com)
  • A tumor (abnormal growth of tissue) of the nervous system. (beds.ac.uk)
  • The purpose of this chapter is to familiarize the reader with the tumor types encountered in the human rather than to provide a comprehensive diagnostic reference, which is better handled in more comprehensive textbooks and monographs. (abdominalkey.com)
  • In this chapter, these will be covered using an approach that has become a standard one in dealing with this group of tumors. (abdominalkey.com)
  • 12] There have also been reports of nerve sheath tumors exhibiting histologic hybrid features of schwannoma and soft-tissue perineurioma. (medscape.com)
  • Other than histologic and cytogenetic analysis, no specific laboratory tests exist for diagnosing soft-tissue tumors. (medscape.com)
  • Because prognosis is primarily dependent on the disease stage rather than the histologic tumor type, evaluation of local and distant extent is pivotal in the ultimate management of soft-tissue sarcoma. (medscape.com)
  • Along with neurofibroma, schwannoma constitutes one of the two most common benign peripheral nerve sheath tumors. (medscape.com)
  • Of note, the benign nerve sheath tumors are classified as World Health Organization (WHO) grade I on the basis of their benign cytologic features, in contrast to the malignant counterparts, which are WHO grade III or IV. (medscape.com)
  • Angiography to evaluate any vascular involvement by soft tissue tumors has essentially been replaced by MRI. (medscape.com)
  • Early tissue diagnosis is the most important component of multimodality treatment for soft-tissue tumor. (medscape.com)
  • Although MRI alone suffices for most patients, the information obtained from CT and MRI of the primary tumor occasionally may be complementary. (medscape.com)