scholarly journals Genital Tract Tumors in Proteus Syndrome: Report of a Case of Bilateral Paraovarian Endometrioid Cystic Tumors of Borderline Malignancy and Review of the Literature

2002 ◽  
Vol 15 (2) ◽  
pp. 172-180 ◽  
Author(s):  
Rajeeva R Raju ◽  
William R Hart ◽  
David K Magnuson ◽  
Janet R Reid ◽  
Douglas G Rogers
Keyword(s):  
Genital Tract ◽  
Proteus Syndrome ◽  
Review Of The Literature ◽  
Cystic Tumors ◽  
Borderline Malignancy ◽  
Syndrome Report

scholarly journals Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-6
Author(s):  
Liliana Vasquez ◽  
Mariela Tello ◽  
Ivan Maza ◽  
Monica Oscanoa ◽  
Milagros Dueñas ◽  
...  
Keyword(s):  
Germ Cell ◽  
Genital Tract ◽  
Germ Cell Tumors ◽  
Cystic Tumor ◽  
Proteus Syndrome ◽  
Rare Syndrome ◽  
Epithelial Tumors ◽  
Adnexal Tumor ◽  
Borderline Malignancy ◽  
Asymptomatic Infant

Ovarian and paraovarian neoplasms are uncommon in children, mainly originating from germ cell tumors and, least frequently, epithelial tumors. There is an association between genital tract tumors and Proteus syndrome, a rare, sporadic, and progressive entity, characterized by a postnatal overgrowth in several tissues caused by a mosaic mutation in the AKT1 gene. We describe a 20-month-old asymptomatic infant with Proteus syndrome who developed an endometrioid paraovarian borderline cystic tumor. This is the youngest patient so far reported in the literature with this rare syndrome and an adnexal tumor of borderline malignancy. A total of nine patients have been described with female tract tumors and associated Proteus syndrome, which includes bilateral ovarian cystadenomas and other benign masses. A paraovarian neoplasm is extremely rare in children and could be considered a criterion for Proteus syndrome. Standardized staging and treatment of these tumors are not well established; however, most authors conclude that these neoplasms must be treated as their ovarian counterparts.

Scoliosis Associated with Proteus Syndrome: Report of 2 Cases and Review of the Literature

2020 ◽  
Vol 138 ◽  
pp. 274-283
Author(s):  
Rosa M. Egea-Gámez ◽  
María Galán-Olleros ◽  
Carmen Martínez-González ◽  
Rafael González-Díaz
Keyword(s):  
Proteus Syndrome ◽  
Review Of The Literature ◽  
Syndrome Report

Dorsal Hemi-Lithotomy Position for Femoral Fracture Nailing and the Well Leg Compartment Syndrome. Report of Two Cases, Review of the Literature

Swiss Surgery ◽  
2002 ◽  
Vol 8 (4) ◽  
pp. 193-196 ◽  
Author(s):  
Christodoulou ◽  
Garofalo ◽  
Echeverri ◽  
Pelet ◽  
Mouhsine
Keyword(s):  
Compartment Syndrome ◽  
Femoral Fracture ◽  
Lithotomy Position ◽  
Review Of The Literature ◽  
Revue De La Littérature ◽  
Examen Clinique ◽  
Positionnement Du Patient ◽  
Syndrome Report

Le syndrome de loge sur positionnement prolongé en lithotomie ou en hémilithitomie, est une complication rare en chirurgie. Les dommages neurovasculaires sont souvent permanents. On reporte deux cas d'ostéosynthèse du fémur en position d'hemilithotomie, compliqués d'un syndrome de loge de la jambe controlatérale. Une revue de la littérature sur les 40 cas décrits, jusqu'à ce jour, nous démontre que cette complication est fortement liée au positionnement du patient et à la durée opératoire. Compte tenu du pronostic fonctionnel incertain, une limitation du temps de positionnement en lithotomie doit être recherchée. La surveillance postopératoire doit être rigoureuse et sans hésitation quant à une fasciotomie éventuelle sur simple examen clinique ou après la mesure de la pression dans les loges. Une technique de positionnement sur la table orthopédique est proposée.

Variability in the Proteus syndrome: report of an affected child with progressive lipomatosis

1985 ◽  
Vol 143 (4) ◽  
pp. 320-323 ◽  
Author(s):  
J. M�cke ◽  
H. Willgerodt ◽  
R. K�nzel ◽  
D. Brock
Keyword(s):  
Proteus Syndrome ◽  
Affected Child ◽  
Syndrome Report

The Greig cephalopolysyndactyly syndrome: Report of a family and review of the literature

1985 ◽  
Vol 22 (1) ◽  
pp. 59-68 ◽  
Author(s):  
Thomaz Rafael Gollop ◽  
Luiz Roberto Fontes ◽  
John M. Opitz ◽  
James F. Reynolds
Keyword(s):  
Review Of The Literature ◽  
Greig Cephalopolysyndactyly Syndrome ◽  
Syndrome Report

LINEAR NEVUS SEBACEOUS SYNDROME: REPORT OF TWO CASES AND A REVIEW OF THE LITERATURE

PEDIATRICS ◽  
1973 ◽  
Vol 52 (3) ◽  
pp. 382-387
Author(s):  
Frederick H. Lovejoy ◽  
William E. Boyle
Keyword(s):  
Cerebrospinal Fluid ◽  
Neurologic Disease ◽  
Review Of The Literature ◽  
Cutaneous Lesions ◽  
Neurocutaneous Syndromes ◽  
Nevus Sebaceous ◽  
Cerebrospinal Fluid Protein ◽  
Syndrome Report

Two cases of linear nevus sebaceous syndrome are described and a review of the eleven cases now reported in the literature is undertaken. The first patient has retardation, seizures, and classic ectodermal lesions while the second patient manifests typical cutaneous lesions and only an elevated cerebrospinal fluid protein as evidence of neurologic disease. The rationale for defining the syndrome as an entity distinct from other neurocutaneous syndromes is discussed and a pleomorphic presentation of the syndrome is suggested.

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