Krista J Childress1, John Hicks2, Hao Wu3, Mary L Brandt4, Oluyemisi A Adeyemi-Fowode5. 1. Department of Pediatrics, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas; Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas. Electronic address: kchildre@bcm.edu. 2. Department of Pediatrics, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas; Department of Pathology, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas. 3. Department of Pathology, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas. 4. Michael E. DeBakey Department of Surgery, Division of Pediatric Surgery, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas. 5. Department of Pediatrics, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas; Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas.
Abstract
BACKGROUND: Vulvar masses are rare in prepubertal girls. Lipoblastomas are benign adipose tumors that arise from embryonic white fat and occur almost exclusively in infants and children. CASE: An 18-month-old female infant presented with a 2-cm mobile mass in the left labia majora. Imaging and examination revealed normal prepubertal gynecologic structures and a 4.5 cm fatty mass in the left labia. Surgical excision revealed a 3.8 cm well circumscribed adipose tissue mass consistent with maturing lipoblastoma on microscopic examination. Cytogenetic analysis revealed 79,XXX [7]/46,XX[13], a near-triploid clone. SUMMARY AND CONCLUSION: The differential diagnosis of vulvar masses in children should include lipoblastoma. Although preoperative imaging has limited ability to differentiate lipomatous tumors, magnetic resonance imaging is the modality of choice for evaluating tumor extension and for surgical planning. Treatment is complete surgical excision with close follow-up for at least 5 years because of the high recurrence rate.
BACKGROUND: Vulvar masses are rare in prepubertal girls. Lipoblastomas are benign adipose tumors that arise from embryonic white fat and occur almost exclusively in infants and children. CASE: An 18-month-old female infant presented with a 2-cm mobile mass in the left labia majora. Imaging and examination revealed normal prepubertal gynecologic structures and a 4.5 cm fatty mass in the left labia. Surgical excision revealed a 3.8 cm well circumscribed adipose tissue mass consistent with maturing lipoblastoma on microscopic examination. Cytogenetic analysis revealed 79,XXX [7]/46,XX[13], a near-triploid clone. SUMMARY AND CONCLUSION: The differential diagnosis of vulvar masses in children should include lipoblastoma. Although preoperative imaging has limited ability to differentiate lipomatous tumors, magnetic resonance imaging is the modality of choice for evaluating tumor extension and for surgical planning. Treatment is complete surgical excision with close follow-up for at least 5 years because of the high recurrence rate.
Authors: Isabel Bada Bosch; Agustín Del Cañizo; Minia Campos-Domínguez; Javier Ordoñez; María Dolores Blanco Verdú; María Fanjul; Laura Pérez-Egido; Juan Carlos de Agustín Journal: European J Pediatr Surg Rep Date: 2022-04-19