An Extremely Rare Gynecologic Tumor; Pure Large-cell Neuroendocrine Carcinoma of the Endometrium: A Case Report

Oadi N. Shrateh^1, , Ali N. Obeidat¹, Maysam Hamarsheh², Mohand Abulihya³ and Mashhour Naasan²

¹Faculty of Medicine, Al-Quds University, Jerusalem, Palestine

²Department of Obstetrics and Gynecology, Al-Istishari Arab Hospital, Ramallah, Palestine

³Department of pathology, Al-Istishari Arab Hospital, Ramallah, Palestine

Cite this paper

Oadi N. Shrateh, Ali N. Obeidat, Maysam Hamarsheh, Mohand Abulihya and Mashhour Naasan. An Extremely Rare Gynecologic Tumor; Pure Large-cell Neuroendocrine Carcinoma of the Endometrium: A Case Report. American Journal of Medical Case Reports. 2022; 10(9):224-226. doi: 10.12691/AJMCR-10-9-3

Abstract

Introduction: Pure large cell neuroendocrine carcinoma (LCNEC) of the endometrium is a rare and inadequately researched gynecologic malignancy with an aggressive course. Our knowledge of pure LCNEC of the endometrium is restricted to case reports. Herein, we describe a very rare case of pure LCNEC of the endometrium. Case presentation: A 70-year-old female presented with post-menopausal uterine bleeding and abdomino-pelvic pain. A sufficient sample endometrial biopsy showed benign endometrial tissue. We performed total abdominal hysterectomy with bilateral salpingo-oophorectomy and the final histopathology and immunohistochemistry established the diagnosis of a pure type of LCNEC of the endometrium (positive for Synaptophysin, CD56, focal Pan-CK, P53, and OCT3/4). Conclusion: A pure LCNEC of the endometrium is extremely uncommon and is often mistaken for a poorly differentiated endometrial carcinoma. Pure LCNEC of the endometrium is a difficult diagnosis to establish. That is why, high clinical suspicion index is required along with performing histological and immunohistochemical testing. Although rare, it is very important to include pure LCNEC of the endometrium in the differential diagnosis of abnormal uterine bleeding and/or abdomino-pelvic pain, especially in the postmenopausal sitting.

Keywords

pure large cell neuroendocrine carcinoma, endometrial cancer, abnormal uterine bleeding, Synaptophysin, CD56

Copyright

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References

[1]	Tsou MH, Tan TD, Cheng SH, Chiou YK. “Small cell carcinoma of the uterine cervix with large cell neuroendocrine carcinoma component”. Gynecologic oncology. 1; 68(1): 69-72. Jan 1998.
[2]	Gardner GJ, Reidy-Lagunes D, Gehrig PA. “Neuroendocrine tumors of the gynecologic tract: A Society of Gynecologic Oncology (SGO) clinical document”. Gynecologic oncology. 1; 122(1): 190-8. Jul 2011
[3]	Eichhorn JH, Young RH. “Neuroendocrine tumors of the genital tract”. Pathology Patterns Reviews. 1; 115(suppl_1): S94-112. Jun 2001.
[4]	Erhan Y, Dikmen Y, Yucebilgin MS, Zekioglu O, Mgoyi L, Terek MC. “Large cell neuroendocrine carcinoma of the uterine corpus metastatic to brain and lung: case report and review of the literature”. European journal of gynaecological oncology. 1; 25(1): 109-12. Jan 2004.
[5]	Kulesza B, Litak J, Mazurek M, Nogalski A. “Initial factors affecting 6-month outcome of patients undergoing surgery for acute post-traumatic subdural and epidural hematoma”. Folia Medica.; 62(1): 94-104. Mar 2020.
[6]	Nguyen ML, Han L, Minors AM, Bentley-Hibbert S, Pradhan TS, Pua TL, Tedjarati SS. “Rare large cell neuroendocrine tumor of the endometrium: a case report and review of the literature”. International Journal of surgery case reports. 1; 4(8): 651-5. Jan 2013.
[7]	Oliva E, Carcangiu ML, Carinelli SG, Ip P, Loening T, Longacre TA, Nucci MR, Prat J, Zaloudek CJ. Blaustein's Pathology of the Female Genital Tract, Springer US, 2018.  In WHO classification of tumours of female reproductive tract (pp. 135-1473). Lyon: IARC Press.
[8]	Shahabi S, Pellicciotta I, Hou J, Graceffa S, Huang GS, Samuelson RN, Goldberg GL. “Clinical utility of chromogranin A and octreotide in large cell neuro endocrine carcinoma of the uterine corpus”. Rare tumors. 11; 3(4): 129-31. Oct 2011.
[9]	Makihara N, Maeda T, Nishimura M, Deguchi M, Sonoyama A, Nakabayashi K, Kawakami F, Itoh T, Yamada H. “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. 25; 4(3): 118-21. Jul 2012.
[10]	Hiroshima K, Mino-Kenudson M. “Update on large cell neuroendocrine carcinoma”. Translational lung cancer research.; 6(5): 530. Oct 2017.
[11]	Matsumoto H, Nasu K, Kai K, Nishida M, Narahara H, Nishida H. “Combined large-cell neuroendocrine carcinoma and endometrioid adenocarcinoma of the endometrium: a case report and survey of related literature”. Journal of Obstetrics and Gynaecology Research.; 42(2): 206-10. Feb 2016.
[12]	Burkeen G, Chauhan A, Agrawal R, Raiker R, Kolesar J, Anthony L, Evers BM, Arnold S. “Gynecologic large cell neuroendocrine carcinoma: a review”. Rare tumors.; 12: 2036361320968401. Oct 2020.
[13]	Travis WD, Brambilla E, Nicholson AG, Yatabe Y, Austin JH, Beasley MB, Chirieac LR, Dacic S, Duhig E, Flieder DB, Geisinger K. “The 2015 World Health Organization classification of lung tumors: impact of genetic, clinical and radiologic advances since the 2004 classification”. Journal of thoracic oncology. 1; 10(9): 1243-60. Sep 2015.
[14]	Jenny C, Kimball K, Kilgore L, Boone J. “Large cell neuroendocrine carcinoma of the endometrium: a report and review of the literature”. Gynecologic Oncology Reports. 1; 28: 96-100. May 2019.