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OVARIAN NON-SMALL CELL UNDIFFERENTIATED CARCINOMA OF NEUROENDOCRINE TYPE

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OVARIAN NON-SMALL CELL UNDIFFERENTIATED CARCINOMA OF NEUROENDOCRINE TYPE. M Mora*, E Fulcheri*, ML Carcangiu**. *Department of Surgical and Morphological Sciences, Anatomic Pathology Division, University of Genova, Italy. **Department of Pathology, Istituto Nazionale Tumori, Milan, Italy.

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OVARIAN NON-SMALL CELL UNDIFFERENTIATED CARCINOMA OF NEUROENDOCRINE TYPE

M Mora*, E Fulcheri*, ML Carcangiu**.

*Department of Surgical and Morphological Sciences, Anatomic Pathology Division, University of Genova, Italy.

**Department of Pathology, Istituto Nazionale Tumori, Milan, Italy.

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Introduction

Neuroendocrine carcinomas of the ovary include small cell carcinoma of pulmonary type, small cell carcinoma of hypercalcemic type and non-small cell undifferentiated carcinoma of neuroendocrine type (NSCNEC) which is often associated to epithelial-stromal tumours [1-2-3].

A large variety of primary ovarian epithelial-stromal tumours may express neuroendocrine differentiation, with neuroendocrine cells arranged singularly or in solid areas with trabecular and insular growth pattern [2].

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Methods /1

Pelvic mass in a 41 year-old woman with no significant past medical history.

Pelvic ultrasound showed two masses in right ovary, one of cystic appearance of 41 x 40 mm and a second one of solid appearance.

Laboratory test showed normal levels for serum CEA and CA-125, no hypercalcemia or paraneoplastic syndromes were observed.

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Methods /2
  • Right salpingo-oophorectomy was performed and the ovary sent for introperative consultation.
  • After the diagnosis of carcinoma was given, patient underwent:
    • hysterectomy with left salpingo-oophorectomy,
    • omentectomy,
    • pelvic lymph node dissection,
    • multiple biopsies of abdominal side wall and peritoneal ligaments.
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Results

Microscopic investigation revealed that the tumour was composed of a G2 endometroid carcinoma commisted to a solid tumour with neuroendocrine features, consisting of cells of medium to large size with scanty cytoplasm and nuclei with evenly distributed chromatin and occasional large nucleoli.

These latter were arranged in solid sheet, island or nest, with little or no intervening stroma; in other areas they were disposed in single-elements ribbons surrounding endometrioid glands without infiltrating them. No high cytologic atypia and mitotic activity were demonstrated.

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Results
  • Immunohistochemical staining showed:
  • positivity of the neuroendocrine component for:
    • Chromogranin A
    • NSE
    • Synaptophysin
  • positivity of the endometrioid component for:
    • CEA (focally)
    • CA-125
    • EMA
    • ER and PGR receptors
slide12
Conclusion

We concluded that the lesion was a primary ovarian non-small cell carcinoma of neuroendocrine type (NSCNEC) associated to an endometroid carcinoma.

Tumour stage was pT1c/G2/N0/Mx.

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Conclusion

At present in the literature there are 27 previous cases of NSCNEC, 26 of which associated to epithelial-stromal tumours:

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Conclusion

NSCNEC can be misdiagnosed as metastasis of large cell endocrine carcinoma of unknown origin but clinical findings help the pathologist to the correct diagnosis and the association with a epithelial-stromal neoplasia is a clue to the primary nature of the neoplasm.

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References

1] Eichhorn JH, Young RH. Neuroendocrine tumours of the genital tract. Am J Clin Pathol; 2001; 115(Suppl. 1):S94-112 .

2] Scully RE et al. Tumors of the ovary, maldeveloped gonads, fallopian tube and broad ligament, chapter 17. Whashington D.C.: Ed. Armed Forces Institute of Pathology 1996.

3] WHO Classification of Tumours. Pathology&Genetics. Tumours of the Breast and Female Genital Organs. IARCPress, Lyon 2003.

4] Collins R, Cheung A, Ngan H, et al. Primary mixed neuroendocrine and mucinous carcinoma of the ovary. Arch Gynecol Obstet. 1991;248:139–143.

5] Khurana K, Tornos C, Silva E. Ovarian neuroendocrine carcinoma associated with a mucinous neoplasm. Arch Pathol Lab Med. 1994;118:1032–1034.

6] Jones K, Diaz J, Donner L. Neuroendocrine carcinoma arising in an ovarian mucinous cystadenoma. Int J Gynecol Pathol. 1996;15: 167–170.

7] Eichhorn J, Lawrence W, Young R, et al. Ovarian neuroendocrine carcinomas of non-small-cell type associated with surface epithelial adenocarcinomas—A study of .ve cases and review of the literature. Int J Gynecol Pathol. 1996;15:303–314.

8] Chen KT. Composite large-cell neuroendocrine carcinoma and surface epithelial-stromal neoplasm of the ovary. Int J Surg Pathol. 2000;8:169–174.

9] Behnam K, Kabus D, Behnam M. Primary ovarian undi.erentiated non-small cell carcinoma, neuroendocrine type. Gynecol Oncol. 2004;92:372–375.

10] Hirasawa T. Ovarian neuroendocrine carcinoma associated with mucinous carcinoma and teratoma. Nippon Rinsho 2004;62:973–8.

11] Ohira S, Itoh K, Shiozawa T, Horiuchi A, Ono K, Takeuchi H, et al. Ovarian non-small cell neuroendocrine carcinoma with paraneoplastic parathyroid hormone-related hypercalcemia. Int J Gynecol Pathol 2004;23:393–7.

12] Ahmed Z, Aftab K, Kayani N.Ovarian Primary Neuroendocrine Carcinoma of Non-Small Cell type: report of an extremely rare Neoplasm. J Pak Med Assoc. 2005;55:82–84.

13] Duk Choi Y, Shin Lee J, Choi C, Soo Park C, Hee Nam J. Ovarian neuroendocrine carcinoma, non-small cell type, associated with serous carcinoma. Gynecologic Oncology 2007;104: 747–752.

14] Veras E, Deavers MT, Silva EWG, Malpica A. Ovarian Nonsmall Cell Neuroendocrine Carcinoma A Clinicopathologic and Immunohistochemical Study of 11 Cases. Am J Surg Pathol 2007;31:774–782).

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