9364/3 Peripheral neuroectodermal tumor



Definitions

Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

A small round cell tumor with neural differentiation arising from the soft tissues or bone 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

A high-grade, primitive, round cell tumour of neuroectodermal phenotype arising from the soft tissues or bone
2
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Renal peripheral neuroectodermal tumour is a rare malignancy occurring in all age groups, with a predilection for males. It forms sizeable, inhomogeneous masses which may replace almost the entire kidney. Haemorrhage and necrosis are common
3
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



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Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

A small round cell tumor with neural differentiation arising from the soft tissues or bone.



Skin
ICD-O-3 topography code: C44
ICD10: C44
           

A small round cell tumor with neural differentiation arising from the soft tissues or bone.



Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Peripheral neuroectodermal tumour (PNET) is an embryonal tumour composed of undifferentiated or poorly differentiated neuroepithelial cells that arises outside of the central nervous system. PNETs are usually mulitlobulated lesions of variable appearance. They may contain solid areas, sinusoidal appearing areas with cystic spaces, and Homer Wright rosettes. The neoplastic cells are typically small and characterized by minimal cytoplasm, ill defined cell borders and hyperchromatic nuclei with finely granular chromatin and small nucleoli. The chromosome translocation t(11;22)(q24;q12) is found in approximately 90% of cases.
Primary peripheral neuroectodermal tumour is rare in the vulva. It occurs in children and women of reproductive age and presents as a subcutaneous mass
4
Click to access Pubmed
Takeshima N, Tabata T, Nishida H, Furuta N, Tsuzuku M, Hirai Y, Hasumi K (2001)
Peripheral primitive neuroectodermal tumor of the vulva: report of a case with imprint cytology.
Acta Cytol 45: 1049-52



5
Click to access Pubmed
Vang R, Taubenberger JK, Mannion CM, Bijwaard K, Malpica A, Ordonez NG, Tavassoli FA, Silver SA (2000)
Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction.
Int J Gynecol Pathol 19: 103-9



6
Click to access Pubmed
Rekhi B, Qureshi S, Basak R, Desai SB, Medhi S, Kurkure P, Menon S, Maheshwari A, Jambhekar NA (2010)
Primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report.
J Med Case Reports 4: 88



7
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Vagina
ICD-O-3 topography code: C52
ICD10: C52
           

A small round cell tumor with neural differentiation arising from the soft tissues or bone.



Bone
ICD-O-3 topography code: C40-C41
ICD10: C40-C41
           

A small round cell tumor with neural differentiation arising from the soft tissues or bone.