Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature

Abstract

BACKGROUNDPrimitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family. The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors (PNETs) in extremely rare anatomic locations, the abdominal wall and vulva.CASE SUMMARYCase 1 was a 66-month-old girl with lesions on the abdominal wall; tumor size was about 3.4 cm × 6.1 cm × 2 cm. The patient underwent radical resection of the tumor. After the operation, an alternating vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide (IE) regimen was given for eight cycles, and the patient survived for 66 mo without progression. Case 2 was a 40-month-old girl, with a vulvar lesion; tumor size was about 3.3 cm × 5 cm × 2.5 cm. The tumor was partially resected by surgery. The family left treatment after two cycles of vincristine, pirarubicin, and cyclophosphamide/IE chemotherapy, and the patient died at home six months after surgery.CONCLUSIONPNET is a rare, fast-growing, highly malignant tumor that requires histologic and molecular analyses for exact diagnosis, and multimodal treatment is required to achieve a good prognosis.

Ewing Sarcoma of the Female Genital Tract

“…Treatment is adjuvant chemotherapy per standard Ewing sarcoma protocols administered at other sites, and more than half of patients are treated surgically and/or chemotherapeutically to no evidence of disease after several months to years of surveillance. Unfortunately, about a third succumb to their disease quite rapidly, with relentless progression on therapy and death within <1 year 16–18,22,31,32,34–61 …”

Tumor neuroectodérmico primitivo primario de ovario

“…La quimioterapia se considera uno de los métodos más eficaces para tratar los tumores con metástasis. No obstante, se ha descrito que esta solo es efectiva durante los primeros ciclos, ya que los tumores desarrollan resistencia muy rápidamente (10)(11)(12)(13)(14)(15) . En el caso de los TNPP que aparecen desde tumores de células germi-nales, algunos autores recomiendan el uso de quimioterapia basada en bleomicina, etopósido y cisplatino (16) .…”

Unilateral vulvar swelling in a 7‐year‐old girl

“…Unilateral vulvar enlargement is rare in the pediatric and adolescent population. The differential diagnosis of unilateral vulvar swelling includes lipomas, vascular malformations such as hemangiomas and lymphangiomas, Bartholin gland cysts, primitive neuroectodermal tumors of the vulva, mesenchymal tumors, and Crohn disease (CD) 3,7,8 . Vascular malformations should be excluded with imaging prior to biopsy.…”