TY - JOUR
T1 - Vulvar fibromatosis
T2 - A clinical enigma
AU - Conroy, Erin M.
AU - Frimer, Marina
AU - Karabakhtsian, Rouzan G.
AU - Gorlick, Richard
AU - Goldberg, Gary L.
N1 - Publisher Copyright:
Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2015/1/3
Y1 - 2015/1/3
N2 - CONCLUSIONS: Desmoid tumors are nonmalignant, locally aggressive neoplasms most common in the 15 to 60 years age group. They are associated with high estrogen states, prior surgical trauma, and Gardner syndrome. Most commonly, desmoid tumors present in the abdominal wall, shoulder, neck, and chest, but can occur anywhere in the body. Given their rarity and lack of definitive therapy, vulvar desmoid tumors can be exceedingly difficult to treat, and are best managed with an interdisciplinary approach.OBJECTIVE: To present a case of recurrent vulvar fibromatosis in an adolescent, discuss the specific difficulties of treating adolescents, and review the literature on available treatment.METHODS: We present a case of recurrent vulvar fibromatosis in a 14-year-old girl, requiring several treatment modalities, including multiple surgeries, radiation therapy, and multiagent chemotherapy. We then discuss management strategies for these tumor types, and specifically examine how tumor location may impact their treatment.RESULTS: Vulvar desmoids are extremely uncommon and they can be disfiguring and cause significant discomfort for women. Initial management of these tumors is surgical excision, yet failed surgery is often followed by other treatment modalities, including radiation, tyrosine kinase inhibitors, nonsteroidal anti-inflammatory drugs, hormonal therapy, and chemotherapy. This case clearly highlights the difficulties in managing these rare tumors, particularly in the adolescent population.
AB - CONCLUSIONS: Desmoid tumors are nonmalignant, locally aggressive neoplasms most common in the 15 to 60 years age group. They are associated with high estrogen states, prior surgical trauma, and Gardner syndrome. Most commonly, desmoid tumors present in the abdominal wall, shoulder, neck, and chest, but can occur anywhere in the body. Given their rarity and lack of definitive therapy, vulvar desmoid tumors can be exceedingly difficult to treat, and are best managed with an interdisciplinary approach.OBJECTIVE: To present a case of recurrent vulvar fibromatosis in an adolescent, discuss the specific difficulties of treating adolescents, and review the literature on available treatment.METHODS: We present a case of recurrent vulvar fibromatosis in a 14-year-old girl, requiring several treatment modalities, including multiple surgeries, radiation therapy, and multiagent chemotherapy. We then discuss management strategies for these tumor types, and specifically examine how tumor location may impact their treatment.RESULTS: Vulvar desmoids are extremely uncommon and they can be disfiguring and cause significant discomfort for women. Initial management of these tumors is surgical excision, yet failed surgery is often followed by other treatment modalities, including radiation, tyrosine kinase inhibitors, nonsteroidal anti-inflammatory drugs, hormonal therapy, and chemotherapy. This case clearly highlights the difficulties in managing these rare tumors, particularly in the adolescent population.
KW - adolescent
KW - chronic illness
KW - fibromatosis
KW - vulvar
UR - http://www.scopus.com/inward/record.url?scp=84920595635&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84920595635&partnerID=8YFLogxK
U2 - 10.1097/MPH.0000000000000261
DO - 10.1097/MPH.0000000000000261
M3 - Article
C2 - 25238226
AN - SCOPUS:84920595635
SN - 1077-4114
VL - 37
SP - 63
EP - 67
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
IS - 1
ER -