Primitive Neuroectodermal Tumor of the Uterus

Primitive neuroectodermal tumours of the uterus: a case report with cytological correlation and review of the literature

“…Rose et al 5 reported a case of a 17-year-old female with stage IIIC tumour and subsequent follow-up for more than 10 years revealed no evidence of disease. 13 With the exception of the current report where the follow-up interval is short, all the other patients with stage II, III or IV tumours died from tumour progression within 2 years of presentation. On gross examination, these neoplasms appeared as solitary, polypoidal masses filling the endometrial cavity and often showed myometrial invasion.…”

“…Other differential diagnoses to be considered in the paediatric population include rhabdomyosarcoma , neuroblastoma and Ewing's sarcoma. 13 The cytological diagnosis of uterine PNET from cervicovaginal smear remains challenging and, to date, only one such case has been reported. Ward et al 13 described a 17-year-old female presenting with profuse vaginal bleeding and the cervical smear showed a diffuse and monotonous population of small-to medium-sized undifferentiated cells.…”

“…13 The cytological diagnosis of uterine PNET from cervicovaginal smear remains challenging and, to date, only one such case has been reported. Ward et al 13 described a 17-year-old female presenting with profuse vaginal bleeding and the cervical smear showed a diffuse and monotonous population of small-to medium-sized undifferentiated cells. In this case, the cervical smear showed abnormal glandular cells belonging to the endometrial adenocarcinoma component and occasional tubular-like structures lined by columnar cells resembling ependymal rosettes.…”

“…The recognition of PNET in an unexpected site like the uterus is often difficult in spite of the well characterised morphological features and this is probably attributed to a low index of suspicion. Ward et al 13 suggested that with careful evaluation of cytological features, aided by liquid-based Pap smear and material available for immunohistochemical staining, the wide range of differential diagnoses can be narrowed to a few reasonable possibilities.…”

Primitive Neuroectodermal Tumor of the Kidney

“…16 Children and adolescents are most frequently affected, but the disease may present in young adults as well. These tumors usually occur in bone or soft tissue, particularly of the extremities, trunk, head and neck, 5 and only seldom affect the viscera, 15,16 including genitourinary sites 2,12,17,18 and pancreas. PNETs originating in the kidney are extremely rare and, because of their more aggressive behavior, appear to form a unique clinical entity.…”