Clinical utility of chromogranin a and octreotide in large cell neuro endocrine carcinoma of the uterine corpus

Abstract

Primary neuroendocrine tumors of the female genital tract have been described in the cervix, ovaries and uterus. Large cell neuroendocrine carcinoma (LCNC) of the uterine corpus is the least common and appears to behave the most aggressively. We report a rare case of a large cell neuroendocrine tumor of the endometrium. These tumors are not well characterized, unlike neuroendocrine tumors of the uterine cervix. Consequently, the optimal management remains still unclear. The treatment of our case consisted of surgery, radiotherapy, chemotherapy, and octreotide. Despite the aggressive treatment, the patient died of disease progression 12 months after the initial diagnosis. We discuss the diagnosis, prognosis, and treatment options for LCNC of the genital tract, and potential future therapeutics.

Neuroendocrine Carcinoma of the Endometrium

“…In contrast to the uterine cervix where NECa are frequently TTF-1 positive 37 , we found that TTF-1 positivity in endometrial NECa was rare. Previous studies of endometrial NECa had also failed to demonstrate TTF-1 immunoreactivity in two cases of endometrial SCNECa 21, 41 and one case of LCNECa 10 .…”

“…We also encountered one case that was pankeratin negative and keratin 18 positive according to the outside pathology report. Shahabi et al 10 also reported one case of LCNECa negative for keratin AE1/AE3 but positive for keratin 18. Thus, keratin 18 may be slightly more sensitive than pankeratin in endometrial NECa, although the added value of keratin 18 may not be as great as for undifferentiated carcinomas 33–35 .…”

“…Most studies published on endometrial NECa have demonstrated keratin positivity in tumor cells, but keratin negative cases have been reported 3, 4, 9, 10, 24, 26 and are encountered in routine practice. In our series, 17 of 18 cases were positive with both a sensitive pankeratin cocktail and with keratin 18, most commonly in a patchy or diffuse distribution.…”

Rapidly progressing large-cell neuroendocrine carcinoma arising from the uterine corpus: A case report and review of the literature

“…Cases of LCNEC of the lungs and digestive tract that responded to irinotecan and cisplatin therapy (19) and pulmonary SC carcinoma patients with a significant response to cisplatin and etoposide therapy (20) have been reported. In addition, cases treated with a combination of chemotherapy and an octreotide similar to somatostatin were reported (10). Inhibition of tumor growth by somatostatin analogues has been shown in animal models and human tumor cell lines.…”

“…Its mechanism is reported to be inhibition of secretion of insulin-like growth factor-1, other tumor growth factors and vascularization, and specific direct tumor inhibition through the somatostatin receptor, which is strongly expressed in NETs (10,21). Partial response was observed in a SCNEC of the endometrium treated with octreotide (22), but other cases exhibited tumor enlargement and adverse effects, such as repeated hypo/hyperglycemia (10). Thus, despite these investigations, LCNECs of the endometrium rapidly progress and the prognosis is very poor.…”