scholarly journals Primary hepatic neuroendocrine carcinoma diagnosed by needle biopsy: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yusuke Seki ◽  
Hiroki Sakata ◽  
Toshimasa Uekusa ◽  
Hirokazu Momose ◽  
Satomi Yoneyama ◽  
...  

Abstract Background Primary hepatic neuroendocrine carcinomas (NECs) are extremely rare. The rate of recurrence after resection is extremely high, and the prognosis is poor. It is debatable whether chemotherapy or surgical resection is the optimal initial treatment for primary hepatic NECs. Therefore, selecting an appropriate therapeutic approach for patients with primary hepatic NECs remains clinically challenging. We present a case of primary hepatic NEC in a patient who developed recurrence after undergoing surgical resection. Case presentation A 78-year-old man with bone metastases of prostate cancer was referred to our department because of a solitary 66-mm tumor in the left lateral segment of the liver, which was detected on annual follow-up by computed tomography after prostate resection. A biopsy and preoperative diagnostic workup identified the lesion as a primary hepatic neuroendocrine carcinoma; therefore, left lateral segmentectomy was performed. Immunohistochemically, the tumor was positive for chromogranin A, synaptophysin, and CD 56, and the Ki-67 index was 40%. This neuroendocrine carcinoma was classified as a large cell type. Adjuvant chemotherapy with carboplatin + etoposide was initially administered a month after surgery. However, lymph node recurrence occurred 4 months after surgery, and the patient died of systemic metastases 15 months after surgical resection. Conclusions Due to the lack of availability of abundant quantities of relevant, high-quality data, there is no standard therapy for primary hepatic NECs. Selecting the most appropriate treatment for patients depending on several factors, such as the stage and differentiation of a tumor and a patient’s performance status and clinical course, is consequently preferred. More cases need to be studied to establish the best treatment strategy for primary hepatic NEC.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 4015-4015 ◽  
Author(s):  
Halfdan Sorbye ◽  
Staffan Welin ◽  
Seppo W. Langer ◽  
Lene Weber Vestermark ◽  
Nanna Holt ◽  
...  

4015 Background: Gastrointestinal poorly differentiated neuroendocrine carcinoma (GI-NEC) are aggressive tumors with Ki-67>20% and usually metastatic at diagnosis. Knowledge about GI-NEC is limited. We retrospectively reviewed clinical data to identify predictive and prognostic markers for advanced GI-NEC patients. Methods: Epidemiological, biochemical, histopathological, treatment and survival data were registered for advanced GI-NEC patients diagnosed during 2000-2009 at 12 Nordic university hospitals. Results: 305 patients were included. Palliative chemotherapy was given to 252 patients, median survival was 11 months. Response rate to 1st-line chemotherapy was 31%, 33% had stable disease. Ki-67<55% was by ROC analyses the best cut-off value concerning correlation to response rate. Response rate to platinum-based chemotherapy was lower in patients with Ki-67<55% (14% vs.44%, p<0.001). Response rate for 84 patients given 2nd-line chemotherapy was 18%, whereas 33% achieved SD. The most important negative prognostic factors for survival were poor performance status, primary colorectal tumors, and elevated baseline platelets or lactate dehydrogenase (LDH) levels. Patients with Ki-67<55% had longer median survival (15 months) than patients with Ki-67>55% (10 months) (p<0.001). Survival and response rates did not differ between the different platinum chemotherapy schedules (cisplatin-based vs. carboplatin-based) or morphology subtypes. 53 patients received best supportive care only with a median survival of 1 month. Conclusions: This is, to our knowledge, the largest study reporting patient and tumor characteristics, treatment and survival in advanced GI-NEC. Performance status, location of primary tumor and blood levels of platelets and LDH were the strongest prognostic factors for survival. Patients with Ki-67<55% had significantly longer survival than patients with higher Ki-67, but were less responsive to platinum-based chemotherapy. Our data indicate that to consider all GI-NEC as one single disease entity may not be correct.


2016 ◽  
Vol 11 (11) ◽  
pp. S284
Author(s):  
Maria Cattoni ◽  
Eric Vallieres ◽  
Lisa M. Brown ◽  
Amir A. Sarkeshik ◽  
Stefano Margaritora ◽  
...  

Chemotherapy ◽  
2021 ◽  
pp. 1-7
Author(s):  
Ken Naganuma ◽  
Hisao Imai ◽  
Ou Yamaguchi ◽  
Kosuke Hashimoto ◽  
Tomoe Akagami ◽  
...  

<b><i>Background:</i></b> Large-cell neuroendocrine carcinoma (LCNEC) of the lung is a rare tumor with an aggressive clinical course. However, there is limited knowledge of its treatment strategy. This retrospective study aimed to assess the efficacy and safety of anti-programed death-1 (PD-1) blockade monotherapy in previously treated advanced LCNEC. <b><i>Methods:</i></b> Eleven patients with previously treated advanced LCNEC who received immune checkpoint inhibitor monotherapy between January 2015 and November 2020 were retrospectively analyzed for efficacy and safety. <b><i>Results:</i></b> Of a total of 11 patients (median [range] age, 66 [37–79] years; 8 men [73%] and 3 women [27%]), 8 patients had performance status (PS) 0–1 [73%] and 3 patients had PS 2 [27%]; 9 patients received 1 prior chemotherapy [82%] and 2 patients received 2 prior chemotherapies [18%]. The median follow-up duration was 4.6 months. Although PD-1 blockade was administered at median cycles of 3 (range, 1–12), overall response rate, median progression-free survival, and median overall survival were 9.1%, 2.7 months, and 4.6 months, respectively. Any adverse events were observed in 9 patients (82%), including 1 patient with grade 3 pneumonitis as a serious adverse event. <b><i>Conclusion:</i></b> Anti-PD-1 blockade monotherapy as a subsequent line for previously treated advanced LCNEC exhibited usefulness and tolerability and was identified as a valid treatment option.


2020 ◽  
Vol 38 (4_suppl) ◽  
pp. TPS635-TPS635
Author(s):  
Robert A. Ramirez ◽  
Marc Ryan Matrana ◽  
Suma Satti ◽  
Ryan Patrick Griffin ◽  
Brianne A Voros ◽  
...  

TPS635 Background: Combination chemotherapy (CTx) is the mainstay of treatment for patients with advanced high-grade gastroenteropancreatic neuroendocrine carcinoma (GEPNECs) and large cell neuroendocrine carcinomas (LCNECs) of the lung. Pembrolizumab (PEM) is a humanized antibody to the programmed cell death receptor (PD-1), which blocks the interaction with its cognate ligands, PD-L1 and PD-L2. PEM blocks the protective mechanism of cancer cells and allows the immune system to destroy them. Combination Ctx and immunotherapy has shown efficacy in other malignancies including small cell lung cancer. The purpose of this study is to test the efficacy, safety, and tolerability of combination CTx with PEM in patients with high-grade GEPNEC or LCNEC of the lung who are CTx naïve. Methods: This is an open label, phase II, single institution, multi-site trial using PEM in combination with either cisplatin or carboplatin and etoposide in patients with high grade GEPNECs or LCNEC of the lung who are CTx naïve. Patients with a histologic diagnosis of a GEPNECs with a Ki-67 of 55% or higher or a LCNEC of the lung will be eligible. Patients must be metastatic or unresectable; chemotherapy naïve; have at least one measurable lesion per RECIST 1.1, have an ECOG performance score of 0-1; and have a predicted life expectancy > 3 months. Approximately 36 GEPNEC and 6 LCNEC of the lung patients will be enrolled. Patients will receive PEM 200mg IV in combination with cisplatin 80 mg/m2 or carboplatin AUC 6 on day 1 and etoposide 100mg/m2 on days 1-3 of a 21-day cycle. Tumor response will be assessed by CT scan every 6 weeks using RECIST 1.1. Those patients who have responsive or stable disease after 4-6 cycles of platinum-based CTx will move to maintenance PEM every 3 weeks for up to 2 years. The primary endpoint will be progression free survival. Results: This study is open to enrollment. Clinical trial information: NCT03901378.


2019 ◽  
Vol 153 (2) ◽  
pp. 165-174 ◽  
Author(s):  
Marina K Baine ◽  
John H Sinard ◽  
Guoping Cai ◽  
Robert J Homer

Abstract Objectives The aim of this study was to devise reproducible biopsy criteria for distinguishing pulmonary large cell neuroendocrine carcinoma (LCNEC) from non-small cell lung carcinoma (NSCLC). Methods Tissue microarrays of LCNEC and NSCLC were generated from resection specimens and used as biopsy surrogates. They were stained for neuroendocrine markers, Ki-67, napsin-A, and p40, and independently analyzed by standardized morphologic criteria by four pathologists. Tumors were scored based on morphology, neuroendocrine marker expression, and Ki-67 proliferative index. Results The average total score for LCNEC was significantly higher than for NSCLC (5.65 vs 0.51, P &lt; .0001). Utilizing a cutoff score of 4 or higher showed 100% sensitivity and 99% specificity for LCNEC diagnosis, with an excellent agreement among four pathologists (98%). Conclusions The proposed semiquantitative approach based on a combination of specific morphologic and immunophenotypic features may be a useful tool for biopsy diagnosis of LCNEC.


2010 ◽  
Vol 1 (2) ◽  
pp. 99-102 ◽  
Author(s):  
Duncan F Hanby ◽  
Andrew McWhorter ◽  
Eric Wallace ◽  
Eugene A Woltering ◽  
Anthony Harton ◽  
...  

Abstract Objective To describe three patients with nonsinonasal neuroendocrine carcinoma (NSNEC) of the head and neck and present a systematic review of literature. Study design Retrospective chart review Methods Three patients with pathologically proven NSNEC of the head-neck who presented to our institution were identified. Relevant demographic, clinic-pathological, and radiological data was recorded. A web-based search was conducted to identify relevant scientific literature on “neuroendocrine carcinoma (NEC) of the head and neck” and a systematic review of literature is presented. Results Two female and one male patient aged 44 to 66 years presented to our service with NEC of the supraglottis (2/3), and of the thymus (1/3). Diagnosis was confirmed with immunohistochemical staining such as Ki-67, synaptophysin, chromogranin, and also with octreotide scanning. The first case, a T4bN0M0 large cell NEC of the thymus with tracheal invasion received conservative airway management and emergent radiotherapy. The second case, moderately differentiated NEC of the supraglottis was managed with a laser supraglottic laryngectomy followed by adjuvant treatment. The third case, a laryngeal NEC was treated with chemoradiotherapy. Conclusions NSNEC of the head and neck are extremely rare tumors with variability in clinical presentation that present challenges in diagnosis and treatment planning. Consideration of the histological subtype and staining characteristics of the individual tumor, accurate diagnosis, and classification of the tumor is vital in order to tailor therapeutic intervention.


2019 ◽  
Vol 37 (4_suppl) ◽  
pp. 197-197
Author(s):  
Zhenyu Lin ◽  
Haihong Wang ◽  
Dejun Zhang ◽  
Dandan Yu ◽  
Tao Zhang

197 Background: To evaluate the prognostic factors in patients with gastrointestinal neuroendocrine carcinoma (WHO G3) (GI-NEC) and develop a nomogram to predict 1-year overall survival (OS). Methods: We performed a retrospective analysis of 122 patients with GI-NEC. Nomogram for 1-year OS was created as visualizations of Cox proportional hazards regression models and internally validated by use of bootstrap and cross-validation. We assessed nomogram model performance by examining overall accuracy (Brier score), calibration (calibration plot), and discrimination (Harrell C index). Results: The median survival was 9 months in 122 patients, and 51 (41.8%) patients died in the development cohort. A 1-year OS was chosen because 82% of the patients who died from disease did so within 1 year. Multivariable analysis identified prognostic factors including performance status, stage, Ki-67 index and LDH, all of which were included in the final model. In the development cohort, the Harrell C index for overall survival was 0·887 (95% CI 0·810-0·964), and calibration curves showed adequate calibration (judged by eye) of the observed and predicted probabilities. Conclusions: Prognostic factors were used to develop nomograms for 1-year OS for GI-NEC. The nomogram can be offered to clinicians to improve their abilities to assess patient prognosis, strengthen the prognosis-based decision making, enhance patient stratification, and inform patients in the clinic.


2017 ◽  
Vol 12 (1) ◽  
pp. S707-S708
Author(s):  
Maria Cattoni ◽  
Eric Vallieres ◽  
Lisa Brown ◽  
Amir Sarkeshik ◽  
Stefano Margaritora ◽  
...  

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S64-S65
Author(s):  
M Bhatt ◽  
B Al-Khafaji

Abstract Introduction/Objective Large cell neuroendocrine carcinoma (LCNEC) of the colon is an exceedingly rare and aggressive neuroendocrine carcinoma. These tumors are associated with a poor prognosis, as they are commonly diagnosed in advanced stages with distant metastases. We present a case of a patient with colonic LCNEC with carcinomatosis peritonei who underwent surgical resection and hyperthermic intraperitoneal chemotherapy. Methods/Case Report The patient is a 49-year-old Caucasian male who presented with several months history of occasional hematochezia, constipation, 40-pound unintentional weight loss, and reflux. An initial diagnosis of adenocarcinoma of colorectal origin was rendered. Subsequently, the patient underwent a low-anterior resection with hyperthermic intraperitoneal chemotherapy (HIPEC). Histological evaluation of the rectosigmoid lesion demonstrated sheets of malignant epithelial cells with scant amphophilic cytoplasm, large pleomorphic vesicular nuclei, prominent nucleoli, and numerous mitotic figures. The tumor invaded the entire colonic wall and extended to the visceral peritoneum with extensive small vessel lymphovascular and perineural invasion with widespread involvement of the diaphragm, spleen, falciform ligament, and omentum. Immunohistochemical stains showed the tumor to be diffusely positive for CK20, CDX2, and synaptophysin; while negative for CK7, PAP, PSA, chromogranin, and p40. These findings are consistent with the diagnosis of a large cell neuroendocrine carcinoma. In addition, CD31 highlighted lymphatic spaces that were extensively filled with tumor cells. One-month post-operative, the patient remains in critical but stable condition with features of acute hypoxic respiratory failure and possible metastatic disease to the lung associated with pleural effusion. Results (if a Case Study enter NA) NA Conclusion Colonic large cell neuroendocrine carcinoma (LCNEC) is a rare, highly aggressive neuroendocrine carcinoma that frequently presents with distant metastases. Clinical awareness of the entity with early diagnosis and surgical resection remains the essential initial step. Utilizing immunohistochemistry stains to further characterize the tumor is critical to reach the correct diagnosis. Accumulating appropriate clinical data will aid in the establishment of future treatment protocols.


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