Current Treatment Strategies and Future Directions for Extrapulmonary Neuroendocrine Carcinomas

Stelwagen, J.; Vries, Elisabeth G.E. de; Walenkamp, Annemiek

doi:10.1001/jamaoncol.2020.8072

Cited by 22 publications

(30 citation statements)

References 79 publications

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“…Although NEN is an uncommon malignant tumor, the incidence of NEN has gradually increased over the past decades owing to continuous improvement in diagnostic methods and improved awareness of the disease[ 2 , 15 ]. The most common site of metastasis in NENs is the liver (40%-93%) followed by bone (12%-20%) and lung (10.8%)[ 16 ]. Metastatic NEN in bone marrow is extremely rare and most reported cases are NECs[ 4 - 8 , 11 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

Bone marrow metastatic neuroendocrine carcinoma with unknown primary site: A case report and review of the literature

Shi¹,

Deng²,

Jin³

2022

World J Clin Cases

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BACKGROUND Metastatic neuroendocrine carcinoma (NEC) of bone marrow is uncommon. Here, we report a case of bone marrow metastatic NEC with an unknown primary site. CASE SUMMARY A 73-year-old Chinese woman was admitted to our hospital because marked chest distress and asthma lasting 1 d on March 18, 2018. She was initially diagnosed with pulmonary infection, cardiac insufficiency, thrombocytopenia and severe anemia. Following treatment with antibiotic therapy, diuresis and blood transfusion, the patient’s symptoms greatly improved. After bone marrow examinations, the patient was diagnosed with bone marrow metastatic NEC, bone marrow necrosis (BMN) and secondary myelofibrosis (MF). Further imaging workup did not show the primary tumor, we presumed that the primary site might regress spontaneously or merely be unexplored due to lack of positron emission tomography with gallium peptide. Everolimus (10 mg/d) was added to the treatment and the best supportive and symptomatic therapies were also administered. Unfortunately, the patient’s condition continued to deteriorate and she died on May 15, 2018. CONCLUSION Bone marrow invasion of NEC is rare and our patient who suffered from bone marrow metastatic NEC as well as secondary BMN and MF had an extremely poor prognosis. Bone marrow biopsy plays an important role in the diagnosis of solid tumors invading bone marrow.

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Section: Discussionmentioning

confidence: 99%

Bone marrow metastatic neuroendocrine carcinoma with unknown primary site: A case report and review of the literature

Shi¹,

Deng²,

Jin³

2022

World J Clin Cases

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“…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%

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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“…However, due to the large size of the tumor, there was a risk in performing surgery, and we opted for systemic treatment instead. Owing to the lack of any definitive therapy for LCNEC, the standard first line of treatment for these patients remains platinum/etoposide-based adjuvant chemotherapy with curative surgery, based on the available literature ( 3 , 16 ). A recent retrospective analysis of 50 patients with GEP MiNEN showed a median overall survival of 31 months (1–104 months) with chemotherapy using platinum/etoposide, palliative chemotherapy, and 5-fluorouracil based chemotherapy, supporting the use of chemotherapy for such cases ( 17 ).…”

Section: Discussionmentioning

confidence: 99%

“…For the treatment of EP-NECs, EP and IP remain the standard first-line chemotherapy regimens ( 10 ). Furthermore, other combinations of drugs such as 5-fluorouracil, leucovorin, oxaliplatin combination regimen (FOLFOX), folinic acid, 5-fluorouracil and irinotecan (FOLFIRI), or capecitabine plus temozolomide (CAPTEM) can also be used ( 16 ). A multicenter, retrospective study showed a promising effect of oxaliplatin-based chemotherapy for the treatment of patients with gastroenteropancreatic NETs, with a disease control rate of 80% and a PFS of 8 months ( 19 ).…”

Section: Discussionmentioning

confidence: 99%

The efficacy of oxaliplatin, surufatinib, and camrelizumab on neuroendocrine carcinoma: a case report and literature review

Wang¹,

Hu²,

Su³

et al. 2022

Ann Transl Med

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Background: Extra-pulmonary neuroendocrine carcinomas (EP-NECs) are rare, accounting for ~1/100,000 of NECs, aggressive neoplasms and poor prognosis. Sometimes, a non-neuroendocrine component is also accompanying these EP-NECs. Curative surgery is suggested for early stage patients while system chemotherapy and locoregional radiotherapy are considered for advanced inoperable disease.Nonetheless, there was lack of standard second-line treatment strategy. Herein, we report a case of NEC involving a large cell neuroendocrine carcinoma (LCNEC) and adenocarcinoma of the gallbladder treated with a surufatinib-containing regimen in the second-line treatment setting and establish the efficacy of this regimen in the treatment of EP-NECs.Case Description: A 58-year-old male presented with symptoms such as distension in the upper right abdomen and a palpable mass. The abdominal magnetic resonance imaging (MRI) scan showed a giant soft tissue mass in the left lobe of the liver, and liver biopsy suggested LCNEC with a non-neuroendocrine (NNE) component. Based on the available literature, a first-line therapy of oxaliplatin + gemcitabine + camrelizumab + apatinib was started initially; however, there was rapid tumor progression. Thus, a second line of treatment was started, where apatinib was replaced with surufatinib, which was given along with oxaliplatin and camrelizumab and continued for seven complete cycles. The patient was re-examined with MRI, which showed a significant decrease in tumor size. And a partial response was achieved. Main adverse events included hand and foot numbness, hypertension, proteinuria, hematuria, and hyperthyroidism.The patient underwent surgery after the second line of treatment and the post-operative pathology report revealed the presence of LCNEC and adenocarcinoma of the gallbladder. Two months later, re-examination result showed no tumor recurrence.Conclusions: As yet, the criteria strategy for unresectable EP-NECs to improve survival outcomes is scarce. EP-NECs are badly in need of effective second-line therapy to carry out survival benefits after resistance to first-line regimen. The case report demonstrated that a surufatinib-containing regimen including oxaliplatin and camrelizumab could be an effective treatment strategy for the second-line treatment of EP-NECs. Furthermore, this strategy is well tolerated and treatment-related toxicity are manageable.More clinical trials are warranted to further confirm the efficacy.

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Current Treatment Strategies and Future Directions for Extrapulmonary Neuroendocrine Carcinomas

Cited by 22 publications

References 79 publications

Bone marrow metastatic neuroendocrine carcinoma with unknown primary site: A case report and review of the literature

Bone marrow metastatic neuroendocrine carcinoma with unknown primary site: A case report and review of the literature

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

The efficacy of oxaliplatin, surufatinib, and camrelizumab on neuroendocrine carcinoma: a case report and literature review

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