Systemic Treatment of Gastroenteropancreatic Neuroendocrine Carcinoma

Mollazadegan, Kazhan; Welin, Staffan; Crona, Joakim

doi:10.1007/s11864-021-00866-9

Cited by 21 publications

(17 citation statements)

References 91 publications

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“…[16,20,26] The average or median age at the time of diagnosis ranges from 50 to 70 years in different studies. [5][6][7]14,[26][27][28] Our patient was 57 years of age and fell within this age range. As expected, the patient had metastatic disease Approximately two-thirds of the cases were metastatic at the time of diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…In patients with metastatic CRNEC with deficient mismatch repair (dMMR), high microsatellite instability (MSI-H), and BRAF mutation, addition of immune checkpoint inhibitor therapy is recommended. [ 16 , 19 , 26 , 29 , 44 , 49 ] Our patient received such therapy but did not undergo a tumor cell molecular study.…”

Section: Discussionmentioning

confidence: 99%

“…[6,15,16,25] Most patients present with advanced or latestage disease due to the lack of specific symptoms and aggressive nature of the disease. [16,20,26] The average or median age at the time of diagnosis ranges from 50 to 70 years in different studies. [5][6][7]14,[26][27][28] Our patient was 57 years of age and fell within this age range.…”

Section: Discussionmentioning

confidence: 99%

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Metastatic rectal neuroendocrine carcinoma presenting with treatment-refractory immune thrombocytopenia: A case report and literature review

2022

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Rationale: Colorectal neuroendocrine cancer (CRNEC) accounts for < 1% of all colorectal cancers. It presents in advanced stages and carries a risk of poor survival. CRNEC may be diagnosed incidentally on imaging or routine endoscopy. When symptomatic, it may mimic colorectal adenocarcinoma. Syncope and immune thrombocytopenic purpura (ITP) have not previously been reported as clinical presentations. Patient concerns: A 57-year-old man with human immunodeficiency virus infection on treatment, seizure and stroke without residue presented for the evaluation of fall and syncope. Diagnosis: Physical examination revealed tachycardia, swelling, and ecchymosis of his proximal left lower extremity. Laboratory tests showed a new isolated thrombocytopenia of 26,000/mm3. Computed tomography for a trauma survey showed an incidental left posterior rectal wall mass. After hospital admission, his platelet count dropped to 14,000/mm3. A peripheral blood smear revealed low platelet count, no schistocytes or immature cells. ITP at a high risk for bleeding was diagnosed and treated with standard medical therapy but remained refractory. Bone marrow biopsy showed metastatic neuroendocrine carcinoma, likely from the rectum. Interventions: Patient received courses of high-dose dexamethasone and intravenous immunoglobulin. He also receive eleven units of platelet transfusion. A course of rituximab was administered. The platelet count response was suboptimal or short lived with drop to a nadir of 4000/mm3. However, after initiation of Eltrombopag, the thrombocytopenia resolved. Patient was started on etoposide, carboplatin, Atezolizumab. His hospital stay was complicated by neutropenia and sepsis, which was successfully treated. Outcomes: He was discharged to subacute rehab in stable condition. About 4 months later, he was readmitted for severe thrombocytopenia, septic shock, and acute respiratory failure. Despite appropriate treatment, the patient deteriorated and expired. Conclusion: CRNEC is a rare aggressive disease with dismal outcome that lacks standardized treatment. Metastasis to the bone marrow is uncommon and concomitant ITP has not been reported. We report a rare case of rectal neuroendocrine carcinoma metastatic to bone marrow associated with refractory ITP and review the relevant literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Metastatic rectal neuroendocrine carcinoma presenting with treatment-refractory immune thrombocytopenia: A case report and literature review

2022

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“…However, the efficacy and longlasting response of platinum-based chemotherapy (etoposide and cisplatin/carboplatin, EP/EC regimens) is very limited. The reported objective response rate (ORR) ranges from 20% to 40%, with a median progression-free survival (PFS) of 2-6 months and an overall survival (OS) of 6-12 months (7). In the NORDIC NEC study, investigators retrospectively analyzed 305 patients with advanced gastrointestinal NEC and found that the ORR of the first-line platinum-based chemotherapy was 31%, with a median PFS of 4 months (95% confidence interval (CI) 3.1-4.6 months) and a median OS of 11 months (95% CI 9.4-12.6 months) (8).…”

Section: Introductionmentioning

confidence: 99%

First-line treatment of camrelizumab combined with chemotherapy in advanced gastroenteropancreatic neuroendocrine carcinoma: Study protocol for a prospective, multicenter, phase II study

Qian

Chen

et al. 2022

Front. Oncol.

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BackgroundGastroenteropancreatic neuroendocrine carcinoma (GEP-NEC) is a group of rare but highly aggressive malignancies. The standard chemotherapy regimens composed of etoposide and cisplatin/carboplatin (EP/EC) are of limited efficacy. This prospective, multicenter, phase II study is conducted to explore the effectiveness and safety of first-line anti-PD-1 antibody (camrelizumab) combined with chemotherapy in advanced GEP-NEC patients.MethodsPatients with unresectable or metastatic GEP-NEC will receive camrelizumab combined with standard first-line chemotherapy every 3 weeks (camrelizumab 200 mg, administered intravenously on day 1; etoposide 100 mg/m2, administered intravenously on days 1–3; cisplatin 75 mg/m2, administered intravenously on day 1 or carboplatin area under the curve 5 mg/ml per min, administered intravenously on day 1). All patients were naïve to systemic therapy in the advanced setting. The primary endpoint is a 6-month progression-free survival (PFS) rate. The secondary endpoints are objective response rate, PFS, overall survival and adverse reactions.DiscussionThis is the first study to investigate the therapeutic potential of camrelizumab plus chemotherapy for advanced GEP-NEC. It is expected that this trial will propose a new and effective treatment strategy for GEP-NEC in the first-line setting.Clinical Trial RegistrationThis trial is registered at the Chinese Clinical Trial Registry http://www.chictr.org.cn, identifier ChiCTR2100047314.Date of RegistrationJune 12, 2021.

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“…Patients with G3 NETs or Ki-67 < 55% (mostly well-differentiated) were significantly less responsive to treatment than those with NEC or Ki-67 ≥ 55% (mostly poorly differentiated). G3 NET and NEC patients have an objective response rate (ORR) of less than 17% and 35%-70%, a median progression-free survival rate (mPFS) of 2.4-4 mo and 5.0 mo, and mOS of 17 mo and 99 mo, respectively[ 8 - 10 ].…”

Section: Introductionmentioning

confidence: 99%

Immunotherapies for well-differentiated grade 3 gastroenteropancreatic neuroendocrine tumors: A new category in the World Health Organization classification

Xu¹,

Wu²,

Ying³

et al. 2021

WJG

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According to the 2019 World Health Organization (WHO) classification, well-differentiated grade 3 (G3) gastroenteropancreatic (GEP) neuroendocrine tumors (NETs) are a new category of cancer of the digestive system. G3 GEP-NET research and treatment are not as robust as those of lower grade (G1/2) NETs and poorly differentiated neuroendocrine carcinomas (NECs). Previously, the management of high-grade NETs was mainly based on NEC therapies, as high-grade NETs were classified as NECs under the previous WHO classification. Despite this, G3 GEP-NETs are significantly less responsive to platinum-based chemotherapy regimens than NECs, due to their distinct molecular pathogenesis and course of pathological grade transition. Patients with advanced G3 GEP-NETs, who have progressed or are intolerant to chemotherapy regimens such as capecitabine plus temozolomide, have limited treatment choices. Immunotherapy has helped patients with a variety of cancers attain long-term survival through the use of immune checkpoint inhibitors. Immunotherapies, either alone or in combination with other therapies, do not have a clear function in the treatment of G3 GEP-NETs. Currently, the majority of immunotherapy studies, both prospective and retrospective, do not reliably differentiate G3 GEP-NETs from NECs. By contrast, a significant number of studies include non-GEP neuroendocrine neoplasms (NENs). Therefore, there is an urgent need to summarize and evaluate these data to provide more effective therapeutic approaches for patients with this rare tumor. The purpose of this mini-review was to screen and summarize information on G3 GEP-NETs from all studies on NENs immunotherapy.

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Systemic Treatment of Gastroenteropancreatic Neuroendocrine Carcinoma

Cited by 21 publications

References 91 publications

Metastatic rectal neuroendocrine carcinoma presenting with treatment-refractory immune thrombocytopenia: A case report and literature review

Metastatic rectal neuroendocrine carcinoma presenting with treatment-refractory immune thrombocytopenia: A case report and literature review

First-line treatment of camrelizumab combined with chemotherapy in advanced gastroenteropancreatic neuroendocrine carcinoma: Study protocol for a prospective, multicenter, phase II study

Immunotherapies for well-differentiated grade 3 gastroenteropancreatic neuroendocrine tumors: A new category in the World Health Organization classification

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