Emiliya Tosheva, Zhivko Shavalov, Vasil Solunkin, Veselin Miroslavov, Dani Penev, Ivaylo Ivanov, Georgi Korukov, Atanas Yonkov

Advanced neuroendocrine tumor of the appendix with adnexal infiltration: case report and literature review

Introduction

Advanced neuroendocrine tumor of the appendix with adnexal infiltration: case report and literature review. Rare, advanced neuroendocrine tumor of the appendix with adnexal infiltration. This case report details diagnosis, radical surgery, and chemotherapy for this aggressive malignancy.

Abstract

Introduction: Neuroendocrine tumors (NETs) of the appendix are rare lesions, typically discovered incidentally and characterized by an indolent clinical course. High-grade neuroendocrine carcinomas (NECs), however, are exceptional and associated with aggressive behavior, early dissemination, and poor prognosis. Local invasion of adjacent organs, such as the adnexa, is exceedingly uncommon.Case presentation: We report a case of a 71-year-old woman presenting with right-sided abdominal pain and altered bowel habits. Imaging revealed a cecal mass measuring 7.5 cm, associated with an appendiceal mucocele and enlarged para-aortic lymph nodes. Intraoperatively, a locally advanced tumor involving the cecum, retroperitoneum, and right adnexa was identified. A right hemicolectomy with adnexectomy and lymphadenectomy was performed. Histopathology demonstrated a high-grade large-cell neuroendocrine carcinoma of the appendix (pT4N1M1b, LV1, Pn+, R0) with vascular and perineural invasion and multiple nodal metastases. The postoperative course was uneventful.Discussion: Large-cell NEC of the appendix is exceptionally rare and may mimic other ileocecal or adnexal malignancies. Definitive diagnosis requires histopathologic and immunohistochemical confirmation. Radical surgical resection remains the cornerstone of therapy, while systemic platinum-based chemotherapy (cisplatin/carboplatin with etoposide) is recommended for advanced disease. Although prognosis is poor, R0 resection offers the best chance for prolonged survival.Conclusion: This case highlights an unusual manifestation of locally advanced large-cell NEC of the appendix with direct adnexal and retroperitoneal invasion, underscoring the importance of a high index of suspicion and a multidisciplinary approach to diagnosis and management.

Review

The title and abstract present a highly interesting and clinically relevant case report detailing an advanced neuroendocrine tumor (NET) of the appendix with unusual adnexal infiltration. While appendiceal NETs are themselves rare, the described high-grade large-cell neuroendocrine carcinoma (NEC) variant, coupled with extensive local invasion of adjacent organs like the adnexa and retroperitoneum, represents an exceedingly uncommon and aggressive presentation. This case provides valuable insights into the atypical manifestations and challenging management of these aggressive tumors, significantly contributing to the existing sparse literature on this specific entity. The abstract clearly outlines the pertinent clinical details, from the initial presentation of a 71-year-old woman with right-sided abdominal pain and altered bowel habits, through the diagnostic imaging suggestive of a complex mass, to the intraoperative findings confirming advanced local disease. The detailed histopathological confirmation of a high-grade large-cell NEC (pT4N1M1b, LV1, Pn+, R0) with comprehensive staging and description of vascular/perineural invasion is particularly strong. The discussion points, as summarized, effectively highlight the diagnostic challenges, the cornerstone role of radical surgery, and the recommended systemic chemotherapy for advanced disease, offering practical guidance for clinicians facing similar complex cases. This case report effectively underscores the importance of maintaining a high index of suspicion for rare and aggressive pathologies when encountering unusual ileocecal or adnexal masses, and advocates for a multidisciplinary approach. While the abstract strongly conveys the immediate clinical course and management, the full manuscript would ideally delve deeper into the specific immunohistochemical markers used for definitive diagnosis and any detailed long-term follow-up beyond the uneventful postoperative period to further assess the efficacy of the comprehensive treatment. Overall, this well-structured abstract promises a valuable contribution to surgical oncology and pathology literature, and the full manuscript is likely to be highly beneficial for clinicians in navigating such rare and aggressive malignancies.

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