Primary neuroendocrine carcinoma of the endometrium – a case report

Neuroendocrine carcinomas (NEC) of the female genital tract are uncommon and aggressive. The authors report the single case of primary NEC of the endometrium at their institution. A 54-year-old female presented with post-menopausal bleeding and gross ascites. Histology from endometrial curettings revealed large cell NEC of the endometrium. The patient underwent total hysterectomy, bilateral salpingo-oophorectomy, pelvic and para-aortic lymphadenectomy, and omentectomy with adjuvant chemotherapy for Stage 1A NEC of the endometrium. The disease recurred in the left iliac bone, with invasion to the left iliacus and gluteal muscles, which was treated with radiotherapy. Repeat imaging showed progressive disease with development of skin metastases over the groin region despite treatment. Palliative chemotherapy was commenced. The patient passed away 26 months after her initial surgery. This case highlights the aggressiveness of NEC, with a poor outcome despite an early stage at diagnosis, and the challenges in diagnosis and management of such a rare carcinoma.

Endometrium; Large cell neuroendocrine carcinoma; Neuroendocrine carcinoma.

[1] Nguyen M.L.T., Han L., Minorsa A.M., Bentley-Hibbert S., Pradhana T.S., Pua T.L., et al.: “Rare large cell neuroendocrine tumour of the endometrium: A case report and review of the literature”. Int. J. Surg. Case Rep., 2013, 4, 651.

[2] Shahabi S., Pellicciotta I., Hou J., Graceffa S., Huang G.S., Samuelson R.N., et al.: “Clinical utility of chromogranin A and octreotide in large cell neuroendocrine carcinoma of the uterine corpus”. Rare Tumors, 2011, 3, e41.

[3] Felix A.S., Weissfeld J.L., Stone R.A., Bowser R., Chivukula M., Edwards R.P., et al.: “Factors associated with Type I and Type II endometrial cancer”. Cancer Causes Control, 2010, 21, 1851.

[4] Erhan Y., Dikmen Y., Yucebilgin M.S., Zekioglu O., Mgoyi L., Terek M.C.: “Large cell neuroendocrine carcinoma of the uterine corpus metastatic to brain and lung: case report and review of the literature”. Eur. J. Gynaecol. Oncol., 2004, 25, 109.

[5] Mulvany N.J., Allen D.G.: “Combined large cell neuroendocrine and endometrioid carcinoma of the endometrium”. Int. J. Gynecol. Pathol., 2008, 27, 49.

[6] Albores-Saavedra J., Martinez-Benitez B., Leuvano E.: “Small Cell carcinomas and large cell neuroendocrine carcinomas of the endometrium and cervix: polypoid tumors and those arising in polyps may have a favorable prognosis”. Int. J. Gynecol. Pathol., 2008, 27, 333.

[7] Deodhar K.K., Kerkar R.A., Suryawanshi P., Menon H., Menon S.: “Large cell neuroendocrine carcinoma of the endometrium: an extremely uncommon diagnosis, but worth the efforts”. J. Cancer Res. Ther., 2011, 7, 211.

[8] Makihara N., Maeda T., Nishimura M., Deguchi M., Sonoyama A., Nakamayashi K., et al.: “Large cell neuroendocrine carcinoma originating from the uterine endometrium: a report on magnetic resonance features of 2 cases with very rare and aggressive tumor”. Rare Tumors, 2012, 4, e37.