Neuroendocrine Carcinoma of the Anus and Rectum: Patient Characteristics and Treatment Options

Ueberroth, Benjamin E.; Liu, Alex J.; Starr, Jason S.; Hobday, Timothy J.; Ashman, Jonathan B.; Mishra, Nitin; Bekaii‐Saab, Tanios; Halfdanarson, Thorvardur R.; Sonbol, Mohamad Bassam

doi:10.1016/j.clcc.2020.12.001

Cited by 6 publications

(9 citation statements)

References 29 publications

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“…The application of CRT in the treatment of non-metastatic anorectal PDNEC has been growing in the last decade. High disease control rates of up to 93% in this group of patients were observed by several investigators when CRT was used as definitive treatment (Ueberroth et al 2021 ; Voong et al 2017 ; Brieau et al 2015 ). This correlated with our observations in a radiological confirmed LRTC at the disease’s progression time in four patients.…”

Section: Discussionsupporting

confidence: 50%

“…However, while the survival benefit of neoadjuvant chemoradiotherapy (CRT) in rectal adenocarcinomas is well recognised, no high-quality data exist supporting the use of perioperative CRT in anorectal PDNEC. Recently published studies have shown an increasing trend towards using CRT in anorectal PDNEC (Della Torre et al 2021 ; Antelo et al 2020 ; Ueberroth et al 2021 ). However, little is known about the optimal treatment schedules, choice of chemotherapy regimens, sequencing of modalities and the appropriate doses of radiotherapy (RT) or CRT.…”

Section: Introductionmentioning

confidence: 99%

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Treatment of poorly differentiated neuroendocrine carcinomas of rectum and anus with chemoradiotherapy: a single-centre evaluation

Fløe,

Aggerholm-Pedersen,

Tabaksblat

2024

J Cancer Res Clin Oncol

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Purpose Poorly differentiated neuroendocrine carcinoma (PDNEC) of the rectum and anus is a rare disease exhibiting aggressive biological behaviour, even if diagnosed early. Currently, there are no agreed standard treatment approaches and management of locally advanced (LA) and metastatic PDNEC usually follows treatments used in pulmonary neuroendocrine carcinomas because of the similarities with small cell lung cancer. The role of surgery in PDNEC is still debated and the benefit of chemoradiotherapy (CRT) is unknown. This report summarises the experiences of CRT application in anorectal PDNEC in a single Danish institution. Methods All patients with PDNEC treated with concomitant CRT between May 2019 and January 2021 at a University hospital in Denmark were evaluated. Demographics, treatment and survival outcomes were collected and analysed. Results Six patients were identified. Five patients received radiotherapy with 50.4 Gy/28 fractions, and four were eligible for curative resection after the CRT. Distant metastasis was observed in four patients at diagnosis. Two patients with synchronous liver metastases were treated with RFA, and one received a liver resection. The treatment was well tolerated with limited side effects. The median follow-up time was 17 months (range 10–36 months), and the median duration of response was 11.2 months (range 8.1 to 24.2 months). One patient achieved a complete response. Conclusion A multimodal treatment approach with CRT in advanced stages of PDNEC in a highly selected patient group is well tolerated and with a high chance of achieving local control and, combined with surgery, even complete response in a single case.

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Section: Discussionsupporting

confidence: 50%

Section: Introductionmentioning

confidence: 99%

Treatment of poorly differentiated neuroendocrine carcinomas of rectum and anus with chemoradiotherapy: a single-centre evaluation

Fløe,

Aggerholm-Pedersen,

Tabaksblat

2024

J Cancer Res Clin Oncol

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“…Systemic chemotherapy with cisplatin or carboplatin, which has a lower toxicity and equivalent efficacy, plus etoposide (irinotecan with cisplatin optional) for at least 4 to 6 cycles, continued until maximal response, is recommended in all categories of CRNEC cases. [9,14,19,25,[27][28][29]38,[41][42][43][44] Neoadjuvant chemotherapy has been advocated for the purpose of downstaging the cancer because of the concern for a relatively high proportion of positive surgical margins, to improve resection, and to lower the risk of systemic recurrence. Patients with early stage disease treated with surgical resection alone had inferior outcomes compared to those who received neoadjuvant or adjuvant chemotherapy, suggesting that micrometastases contributed to poor surgical outcomes.…”

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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“…8 However, there exists an area of uncertainty with regards to tumors ranging from 10 mm to 20 mm, and the treatment options remain controversial due to the moderate risk of metastasis. 5,9 The National Comprehensive Cancer Network (NCCN) guidelines recommend endoscopic local resection of tumors smaller than 20 mm in size. 10 Chen et al 11 found that endoscopic therapy should be prioritized for G1 stage R-NETs limited to the submucosa.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Optimization of Endoscopic Submucosal Dissection and Endoscopic Mucosal Resection Strategies for Rectal Neuroendocrine Tumors Within 20 mm

Yu,

Zhang,

et al. 2024

The American Surgeon™

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Aims No consensus regarding the optimal endoscopic resection approach for rectal neuroendocrine tumors (R-NETs) measuring 10-20 mm, this study aims to investigate this issue. Methods Patients with R-NETs underwent either endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD). The primary endpoint was the complete resection rate, and the secondary endpoints were surgery-related complications and long-term outcomes. Results 96 patients met the inclusion criteria, 84 patients completed endoscopic resection, and 5 patients were excluded. 79 patients were enrolled and divided into EMR (n = 21) and ESD groups (n = 58). 100% of ESD excisions reached the primary endpoint, while 90.5% of EMR. Endoscopic submucosal dissection can achieve higher R0 rate and lower positive margin rate than EMR. The mean operative time of ESD and EMR was 35.22 ± 8.96 min and 13.14 ± 3.26 min, respectively. The complication rates of ESD and EMR were 3.4% and 4.8%, respectively. For R-NETs between 10 mm and 20 mm, the R0 rate of ESD was significantly higher than that of EMR (100% vs 71.4%, P = .01) and the margin positive rate of ESD was significant lower than that of EMR (4.8% vs 42.9%, P < .05). Both ESD and EMR obtained 100% R0 resection of less than 10 mm R-NET. The median follow-up was 13 months (3-84 months); 1 patient relapsed 25 months after EMR and was re-treated with ESD. Conclusion For R-NETs with a diameter less than 10 mm, both EMR and ESD were safe and effective and EMR is convenient and fast, with advantages. ESD offers superiority for R-NETs between 10 and 20 mm and can be considered as the preferred method.

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Neuroendocrine Carcinoma of the Anus and Rectum: Patient Characteristics and Treatment Options

Cited by 6 publications

References 29 publications

Treatment of poorly differentiated neuroendocrine carcinomas of rectum and anus with chemoradiotherapy: a single-centre evaluation

Treatment of poorly differentiated neuroendocrine carcinomas of rectum and anus with chemoradiotherapy: a single-centre evaluation

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

Optimization of Endoscopic Submucosal Dissection and Endoscopic Mucosal Resection Strategies for Rectal Neuroendocrine Tumors Within 20 mm

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