PrognostIc and Predictive Factors in Unresectable Locally Advanced NEC and MANEC

NCT ID: NCT06400654

Last Updated: 2024-05-06

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 100 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2021-07-05
• Study Completion Date: 2025-12-31

Brief Summary

Extra-pulmonary (EP) poorly differentiated neuroendocrine carcinomas (NECs) represent a rare and aggressive category of neoplasms. Mixed adeno-neuroendocrine carcinomas (MANEC) are a group of rare neoplasms composed by a neuroendocrine (NE) and a non-neuroendocrine (non-NE) component, each representing at least the 30% of the neoplasm.

Considering their rarity, low prevalence and poor prognosis a clear clinical, morphological and biomolecular characterization of these neoplasms has been prevented and a clinical approach universally shared is still lacking.

Detailed Description

Extra-pulmonary (EP) poorly differentiated neuroendocrine carcinomas (NECs) represent a rare and aggressive category of neoplasms. They occur in almost 1 / 100.000 patients and the largest part is represented by gastro-entero-pancreatic (GEP) NECs (39%).

Moreover, mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) represent an even more rare entity, with a crude incidence of 0.1 / 100.000 / year, composed at least by 30% of a neuroendocrine (NE) and a non-neuroendocrine (non-NE) component based on the 2019 World Health Organization (WHO) classification. Focusing on the neuroendocrine counterpart, although the term MiNEN includes both well and poorly differentiated morphologies, the main part of them are represented by high grade neuroendocrine neoplasms categorized as MANECs in previous 2010 WHO classification that is a term still used in clinical practice is still lacking.

While for localized disease, surgery represents the cornerstone and virtually the unique curative approach, patients with metastatic disease are mostly managed with chemotherapy. Although any specific clinical practice guideline by Oncological and / or Neuroendocrine Societies has been yet developed worldwide, based on the clinical and morphological similarity with small and large cell lung NECs, cis / carbo-platinum based chemotherapy, is the most often chemotherapeutic regimen proposed in clinical practice both in NECs and MANECs from each site of origin. Other proposed options include regimens containing 5-fluorouracil/folinic-acid and irinotecan or oxaliplatin which are used also in the treatment of colo-rectal adenocarcinomas. Moreover, considering their rarity, low prevalence and poor prognosis a clear clinical, morphological and biomolecular characterization of these neoplasms has been prevented and a clinical approach universally shared is still lacking.

Therefore, a comprehensive clinical and biological characterization of these neoplasms represents an unmet medical need and a major challenge and could improve the awareness of clinicians in the management of EP-NECs and MANECs.

Conditions

Neuroendocrine Carcinoma

Study Design

• Observational Model Type: CASE_ONLY
• Study Time Perspective: OTHER

Study Groups

unresectable locally advanced neuroendocrine carcinoma

patients with extra-pulmonary, advanced neuroendocrine carcinomas

No interventions assigned to this group

mixed Adeno-neuroendocrine carcinomas

composed at least by 30% of a neuroendocrine (NE) and a non-neuroendocrine (non-NE) component based on the 2019 World Health Organization (WHO) classification

No interventions assigned to this group

Eligibility Criteria

Inclusion Criteria

• Histological diagnosis of unresectable locally advanced or metastatic NEC or MANEC confirmed by an expert-pathologist
• EP-primary site (included unknown primary site
• Age > 18 years
• Signed written informed consent
• Performance status ≤2
• Available tumor tissue (formalin-fixed paraffin-embedded, FFPE) (preferably within 6 months). If the tumor contained in FFPE tissue block cannot be provided in total, sections from this block should be provided that are freshly cut. Preferably, 25 slides should be provided (minimum of 15 slides). If tumor tissue is not available, patients should be willing to undergone to a new biopsy.

Exclusion Criteria

• Diagnosis of well-differentiated NEN (G1, G2, G3)
• Collision tumors
• Cytological diagnosis of NEC or MANEC or not availability of tumor tissue for pathological analysis.
• Concurrent neoplastic disease (e.g. Advanced breast or prostatic cancer in hormonal treatment, hematologic diseases)

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

European Institute of Oncology

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Francesca Spada, MD

Role: PRINCIPAL_INVESTIGATOR

Istituto Europeo di Oncologia

Locations

European Institute of Oncology

Milan, , Italy

Site Status: RECRUITING

Countries

Italy

Central Contacts

Francesca Spada, MD

Role: CONTACT

divisione.gastrointestinale@ieo.it

+390257489258

Cristina Mazzon

Role: CONTACT

cristina.mazzon@ieo.it

Other Identifiers

IEO 1476

Identifier Type: -

Identifier Source: org_study_id