DEFINITION AND PATHOGENESIS
- Neuroectodermal origin
IMPORTANCE
- Rare
CLINICAL FEATURES
- Most commonly involves the trunk
RADIOLOGIC FEATURES
GROSS PATHOLOGY
- Well-circumscribed purple mass
HISTOLOGIC FEATURES
- Small round cells
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Extraskeletal Ewing sarcoma
- Rhabdomyosarcoma
DISEASE COURSE AND TREATMENT
- Metastases to bone, bone marrow, LN, lung
- Chemotx + wide resection
SPECIAL CONSIDERATION
-
CONGENITAL PERIPHERAL NEUROEPITHELIOMA
-
- Has been reported as a hand mass in an infant
REFERENCES
Bolen JW, Thorning D: Peripheral neuroepithelioma: a light and electron microscopic study. Cancer 46:2456-62,1980.
Cavazzana AO, Niufo V, Roberts J, Triche TJ: Peripheral neuroepithelioma: a light microscopic, immunocytochemical, and ultrastrctural study. Mod Pathol 5:71-8,1992.
Erdmann D, Brown RE, Rumbolo PM: Congenital neuroepithelioma in an infant hand. J Hand Surg 21B:117-21,1996.
Llombart-Bosch A, Terner-Lacombe MJ, Peydro-Olaya A, Contesso G: Peripheral neuroectadermal sarcoma of soft tissue (peripheral neuroepithelioma): a pathologic study of ten cases with differential diagnosis regarding other small, round-cell sarcomas. Hum Pathol 20:273-80,1989.
Pappo AS, Douglass EC, Meyer WH, Marina N, Parham DM: Use of HBA 71 and Anti-2-microglobulin to distinguish peripheral neuroepithelioma from neuroblastoma. Hum Pathol 24;880-5,1993.
Silliman CC, Mierau GW, Strain JD, : Peripheral neuroepithelioma of the soft tissues: a retrospective analysis of 15 pediatric patients. Am J Ped Hematol/Oncol 15:299-305,1993.
Voss BL, Pysher TJ, Humphrey GB: Peripheral neuroepithelioma in childhood. Cancer 54:3059-64,1984.