Clinical management of a unique case of PNET of the uterus during pregnancy, and review of the literature

Abstract

Rationale:PNETs (primitive neuroectodermal tumors) are a family of highly malignant neoplasms characterized by small round cells of neuroepithelial origin. They usually involve bone and soft tissues, and have a higher incidence in childhood.Patient concerns:In this case report, we describe the obstetric and oncological outcome of a huge mass diagnosed as a leiomyoma in a 39-year-old pregnant woman who complained of low back pain, dysuria, and urinary frequency at 22 weeks of gestation.Diagnoses:During the 25th week of pregnancy, the patient was referred to our hospital at night with severe anemia and suspected hemoperitoneum. She underwent an emergency caesarean section, delivering a female fetus weighing 400 g, with an Apgar score of 7 at 1 minute and 9 at 5 minutes.Intervention:During surgery, we found a huge uterine sarcoma-like metastatic tumor, invading the pelvic peritoneum and parametria bilaterally; the adnexae seemed disease-free. We performed a type B radical hysterectomy, bilateral salpingo-oophorectomy, pelvic peritonectomy, omentectomy, appendectomy, and excision of a bulky lymph node. Seven days after delivery, staging computed tomography (CT) scan demonstrated a large lombo-aortic lymph node compressing the left renal vein and we completed debulking with a second surgery, including diaphragmatic peritonectomy and excision of a huge lymph node by lombo-aortic lymphadenectomy, requiring partial reconstruction of an infiltrated renal vein.Outcome:Ten days after the second surgery, echo-color Doppler showed a regular microcirculation in the left kidney. The patient was discharged after 10 days, and the baby after 1 month, both in good health.Histological examination revealed a uterine body cPNET (central primitive neuroectodermal tumor) orienting the clinical management toward chemotherapy with cisplatin and etoposide.Lessons:PNETs are aggressive neoplasms, usually diagnosed at an advanced stage. Due to their low incidence, universally accepted guidelines are still unavailable. Radical surgery leaving no macroscopic residual disease is mandatory in advanced stages. A good fertility-sparing procedure can be performed only in young women at early stages of disease, when the wish for childbearing is not yet fulfilled.

Ewing Sarcoma of the Female Genital Tract

“…Rare reports are associated with pregnancy. [31][32][33] Occasionally, immunohistochemistry is the sole adjunct to morphology, wherein intense membranous CD99 is an invariable staple, bolstered by positivity for any of FLI1 (highly nonspecific and not recommended for this purpose) and (more recently) NKX2.2. Variant morphology is infrequently reported in gynecologic Ewing sarcoma, manifested in a subset of cells that otherwise display conventional small round cell appearance: spindle cell fascicles, rhabdoid/plasmacytoid cytology, cytoplasmic clearing, and "adamantinoma-like" epithelial differentiation.…”

“…Primary site of disease is most heavily represented by the uterine corpus and cervix, followed by the vulvovaginal tract, and finally, the upper genital tissues (ovary>fallopian tube>broad ligament). Rare reports are associated with pregnancy 31–33 . Occasionally, immunohistochemistry is the sole adjunct to morphology, wherein intense membranous CD99 is an invariable staple, bolstered by positivity for any of FLI1 (highly nonspecific and not recommended for this purpose) and (more recently) NKX2.2.…”

Primary primitive neuroectodermal tumor of the cervix confirmed with molecular analysis in a pregnant woman: A case report and literature review

“…PNET belongs to a group of small round cell tumors that are most commonly found in the central nervous system. In the female reproductive tracts, the ovaries are the most common sites ( De Nola et al, 2018 ). To date, only 26 cases of cervical PNET have been reported in the English literature ( Table 1 ).…”