Large cell neuroendocrine carcinoma with sarcomatous changes of the endometrium: a case report with immunohistochemical studies and molecular genetic study of KIT and PDGFRA.

Abstract

The author herein reports a very rare case of large cell neuroendocrine carcinoma (LCNEC) with sarcomatous changes of the endometrium. A 40-year-old woman was admitted to our hospital because of abnormal uterine bleeding. Gynecologic examination and imaging modalities revealed a polypoid tumor of the uterine corpus. Uterine curettage biopsy revealed a sarcomatous undifferentiated carcinoma. Simple hysterectomy, salpingo-oophorectomy, extensive lymph node dissection, and omentectomy were performed. The patient was diagnosed as having FIGO stage Ib (T1N0M0) carcinoma, and adjuvant chemotherapy was performed. The patient is now alive 16 months after the operation. Pathologically, a polypoid tumor measuring 3x2x2 cm(3) was found in the uterine corpus. Histologically, the tumor consisted of relatively large-sized carcinoma cells without differentiation. The tumor cells have abundant cytoplasm and prominent nucleoli. It was composed of a spindle cell component (40%) and an epithelioid component (60%). A gradual transition between the two was recognized. Immunohistochemically, both elements showed the same immunophenotypes. The carcinoma cells were positive for cytokeratin, vimentin, CA125, CD34, estrogen receptor, progesterone receptor, p53 protein, Ki-67 antigen (80%), synaptophysin, CD56, KIT, and PDGFRA. They were negative for epithelial membrane antigen, CEA, desmin, S100 protein, melanosome, alpha-smooth muscle actin, chromogranin, and neuron-specific enolase. A molecular genetic analysis revealed no mutations of KIT (exons 9, 11, 13, and 17) and PDGFRA (exons 12 and 18) genes. The final diagnosis was LCNEC with sarcomatous changes.

• Authors: Tadashi Terada
• Journal: Pathology, research and practice (Pathol Res Pract) Vol. 206 Issue 6 Pg. 420-5 (Jun 15 2010) ISSN: 1618-0631 [Electronic] Germany
• PMID: 20189318 (Publication Type: Journal Article)
• Copyright: 2010 Elsevier GmbH. All rights reserved.

Chemical References

• Antineoplastic Agents
• Biomarkers, Tumor
• Stem Cell Factor
• Receptor, Platelet-Derived Growth Factor alpha

Topics

• Antineoplastic Agents (therapeutic use)
• Biomarkers, Tumor (analysis)
• Carcinoma, Large Cell (genetics, pathology, therapy)
• Carcinoma, Neuroendocrine (genetics, pathology, therapy)
• Chemotherapy, Adjuvant
• Combined Modality Therapy
• Endometrial Neoplasms (genetics, pathology, therapy)
• Female
• Gynecologic Surgical Procedures
• Humans
• Immunohistochemistry
• Middle Aged
• Neoplasm Staging
• Polymerase Chain Reaction
• Receptor, Platelet-Derived Growth Factor alpha (genetics)
• Stem Cell Factor (genetics)