PMID- 9500772
OWN - NLM
STAT- MEDLINE
DCOM- 19980324
LR  - 20220331
IS  - 0147-5185 (Print)
IS  - 0147-5185 (Linking)
VI  - 22
IP  - 3
DP  - 1998 Mar
TI  - Primary cutaneous Ewing's sarcoma: immunophenotypic and molecular cytogenetic 
      evaluation of five cases.
PG  - 310-8
AB  - Cutaneous small blue cell tumors are relatively uncommon and include primary 
      lesions of either adnexal or neuroendocrine differentiation, as well as 
      metastatic disease. Extraosseous Ewing's sarcoma/malignant primitive 
      neuroectodermal tumor (MPNET) rarely may occur as a primary, superficially based 
      neoplasm in children and young adults. We describe a series of five cases of 
      Ewing's sarcoma/malignant primitive neuroectodermal tumor occurring as a primary 
      cutaneous malignancy supported diagnostically both by immunohistochemical stains 
      and fluorescence in situ hybridization (FISH). All five cases occurred as a 
      solitary dermal nodule and were located in the lower extremities (3 cases), the 
      axilla (1 case), and the flank (1 case). Three of the cases were clinically 
      polypoid. Four of the five patients were female, and age at presentation ranged 
      form 8 to 50 years of age (median, 18 years). All five tumors consisted of 
      nodular proliferations of monomorphous, small blue cells with round, vesicular 
      nuclei, and scant to moderate cytoplasm that were uniformly immunoreactive for 
      the CD99 cell surface glycoprotein in a characteristic membranous pattern. 
      Fluorescence in situ hybridization analysis of paraffin-embedded tissue revealed 
      that three of four tumors were positive for a chromosomal translocation involving 
      the EWS locus at 22q12, seen in more than 90% of cases of Ewing's 
      sarcoma/malignant primitive neuroectodermal tumor. One case was not analyzable. 
      All five patients were treated using local excision, and two patients 
      additionally received postoperative chemotherapy and radiotherapy. Clinical 
      follow-up is available in three cases (median duration, 33 months) and to date 
      none has shown evidence of either local recurrence or metastasis. Because similar 
      cases reported in the literature have likewise had favorable clinical courses 
      after excision, primary cutaneous Ewing's sarcoma/malignant primitive 
      neuroectodermal tumor may represent a clinically favorable subset of this 
      otherwise highly aggressive neoplasm.
FAU - Hasegawa, S L
AU  - Hasegawa SL
AD  - Department of Pathology, Brigham & Women's Hospital, Harvard Medical School, 
      Boston, Massachusetts 02115, USA.
FAU - Davison, J M
AU  - Davison JM
FAU - Rutten, A
AU  - Rutten A
FAU - Fletcher, J A
AU  - Fletcher JA
FAU - Fletcher, C D
AU  - Fletcher CD
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Am J Surg Pathol
JT  - The American journal of surgical pathology
JID - 7707904
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - *Chromosomes, Human, Pair 22
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - Sarcoma, Ewing/*genetics/*pathology/physiopathology
MH  - Skin Neoplasms/*genetics/*pathology/physiopathology
MH  - *Translocation, Genetic
EDAT- 1998/03/21 00:00
MHDA- 1998/03/21 00:01
CRDT- 1998/03/21 00:00
PHST- 1998/03/21 00:00 [pubmed]
PHST- 1998/03/21 00:01 [medline]
PHST- 1998/03/21 00:00 [entrez]
AID - 10.1097/00000478-199803000-00005 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 1998 Mar;22(3):310-8. doi: 10.1097/00000478-199803000-00005.