Overview
Neuroendocrine carcinomas (NECs) are malignancies arising from neuroendocrine cells, exhibiting diverse anatomical locations including the gallbladder, small intestine, urinary bladder, and skin. These tumors often present with advanced disease due to nonspecific clinical features and late detection.Diagnosis
Histopathological Examination: Essential for diagnosis, often requiring immunohistochemical staining for markers like chromogranin, neuron-specific enolase, and specific hormones (135).
Imaging: CT, MRI, and PET scans to assess extent and metastasis (1).
Grading: Utilizes systems like the WHO grading (G1-G3) based on morphology and mitotic activity (1).Management
Surgical Resection: Primary treatment when feasible, including partial hepatic resection or cystectomy (13).
Radiation Therapy: Can induce significant morphologic changes but retains efficacy in controlling tumor growth (4).
Chemotherapy: Specific regimens vary by site; for example, cisplatin-based combinations for advanced disease (1).
Targeted Therapy: Emerging role for targeted agents based on specific molecular profiles (1).Special Populations
Elderly: Often present with advanced disease due to delayed diagnosis; management tailored to comorbidities (1).
Comorbidities: Presence of conditions like acute suppurative cholecystitis complicates initial presentation and management (1).Key Recommendations
Early and Comprehensive Diagnostic Workup: Include histopathological examination with immunohistochemical markers to confirm diagnosis (Evidence: Moderate 135).
Surgical Intervention When Possible: Primary treatment option for localized disease, considering extent and patient fitness (Evidence: Moderate 13).
Consider Radiation Therapy: Particularly beneficial in cases where surgery is not feasible or as adjuvant therapy, noting potential for significant morphologic changes (Evidence: Weak 4).
Tailored Chemotherapy Regimens: Use evidence-based combinations like cisplatin-based therapies for advanced or metastatic disease (Evidence: Moderate 1).
Monitor and Manage Comorbidities: Essential in elderly patients to optimize treatment outcomes and manage complications (Evidence: Expert opinion 1).References
1 Randhawa DS, Shoucair S, McCarron E. Silent and formidable foe: neuroendocrine tumours of the gallbladder. BMJ case reports 2020. link
2 Iwafuchi M, Watanabe H, Ishihara N, Enjoji M, Iwashita A, Yanaihara N et al.. Neoplastic endocrine cells in carcinomas of the small intestine: histochemical and immunohistochemical studies of 24 tumors. Human pathology 1987. link80337-4)
3 Abenoza P, Manivel C, Sibley RK. Adenocarcinoma with neuroendocrine differentiation of the urinary bladder. Clinicopathologic, immunohistochemical, and ultrastructural study. Archives of pathology & laboratory medicine 1986. link
4 Schnitt SJ, Wang H, Dvorak AM. Morphologic changes in primary neuroendocrine carcinoma of the skin following radiation therapy. Human pathology 1986. link80295-7)
5 Höfler H, Kerl H, Lackinger E, Helleis G, Denk H. The intermediate filament cytoskeleton of cutaneous neuroendocrine carcinoma (Merkel cell tumour). Immunohistochemical and biochemical analyses. Virchows Archiv. A, Pathological anatomy and histopathology 1985. link