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Sambataro D, Bellavia S, Di Mattia P, Centonze D, Emmanuele C, Bonasera A, Caputo G, Quattrocchi AMO, Vinci E, Gebbia V, Valerio MR. Combined Neuroendocrine Carcinoma and Hepatocellular Carcinoma of the Liver: Systematic Literature Review Suggests Implementing Biological Characterization to Optimize Therapeutic Strategy. Cancers (Basel) 2025; 17:1074. [PMID: 40227579 PMCID: PMC11988019 DOI: 10.3390/cancers17071074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2025] [Revised: 03/11/2025] [Accepted: 03/19/2025] [Indexed: 04/15/2025] Open
Abstract
BACKGROUND Mixed neuroendocrine-non-neuroendocrine tumors (MINEN) of the liver are exceptionally rare, with limited data available regarding their clinical behavior, pathogenesis, and optimal management. The coexistence of hepatocellular carcinoma (HCC) and neuroendocrine carcinoma (NEC) within the liver presents diagnostic and therapeutic challenges. METHODS A systematic literature search was conducted on PubMed, identifying cases of primary mixed HCC and NEC in the liver. The search adhered to PRISMA guidelines, and relevant studies were critically analyzed. A total of 45 documented cases were reviewed, focusing on patient demographics, clinical characteristics, treatment strategies, and outcomes. RESULTS Most patients (90%) were male, with a median age of 66.5 years. Hepatitis B or C infection was present in 74% of cases, and liver cirrhosis was reported in 38%. The combined type was the most frequently observed histological pattern (65%). Treatment modalities varied, including transarterial chemoembolization (TACE), radiofrequency ablation (RFA), surgery, and systemic therapies. The median overall survival was 10 months, highlighting the aggressive nature of these tumors. CONCLUSIONS Given the rarity and poor prognosis of hepatic MINEN tumors, multidisciplinary management is essential. Advanced molecular profiling may offer insights into tumor biology and potential therapeutic targets. Future research should explore novel systemic therapies, including immune checkpoint inhibitors, to improve patient outcomes.
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Affiliation(s)
- Daniela Sambataro
- Medical Oncology Unit, Umberto I Hospital, 94100 Enna, Italy; (A.B.); (G.C.); (A.M.O.Q.); (E.V.)
- Department of Medicine and Surgery, Kore University, 94100 Enna, Italy; (P.D.M.); (V.G.)
| | - Sandro Bellavia
- Pathology Unit, Umberto I Hospital, 94100 Enna, Italy; (S.B.); (C.E.)
| | - Paolo Di Mattia
- Department of Medicine and Surgery, Kore University, 94100 Enna, Italy; (P.D.M.); (V.G.)
- Surgery Unit, Umberto I Hospital, 94100 Enna, Italy;
| | | | - Carmela Emmanuele
- Pathology Unit, Umberto I Hospital, 94100 Enna, Italy; (S.B.); (C.E.)
| | - Annalisa Bonasera
- Medical Oncology Unit, Umberto I Hospital, 94100 Enna, Italy; (A.B.); (G.C.); (A.M.O.Q.); (E.V.)
| | - Giuseppe Caputo
- Medical Oncology Unit, Umberto I Hospital, 94100 Enna, Italy; (A.B.); (G.C.); (A.M.O.Q.); (E.V.)
| | | | - Ernesto Vinci
- Medical Oncology Unit, Umberto I Hospital, 94100 Enna, Italy; (A.B.); (G.C.); (A.M.O.Q.); (E.V.)
| | - Vittorio Gebbia
- Department of Medicine and Surgery, Kore University, 94100 Enna, Italy; (P.D.M.); (V.G.)
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Mubeen B, Eapen M, Sudhindran S, Krishna Haridas N. Mixed Neuroendocrine Non-Neuroendocrine Tumor (MINEN) of the Liver: Report of Two Cases and Review of the Literature. Turk Patoloji Derg 2025; 41:21-29. [PMID: 39268996 PMCID: PMC11831964 DOI: 10.5146/tjpath.2024.13492] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2024] [Accepted: 08/12/2024] [Indexed: 09/15/2024] Open
Abstract
OBJECTIVE To highlight two cases mixed neuroendocrine non-neuroendocrine tumors (MINEN) of the liver and to review the literature till date. To present two cases of MINEN of the liver diagnosed in our centre with clinical & diagnostic workup, the treatment modalities, and follow up. Extensive review of the literature and compilation of the presentation and treatment modalities used in those cases. CASE REPORTS Thirty-three cases of MINEN of the liver have been reported till date including ours. Our cases presented as incidental masses in liver during workup for other symptoms. AFP levels were normal in both cases but PIVKA (Protein induced by vitamin K absence) levels were increased. Resection was done in one of the cases while the other patient had to undergo transplantation. A diagnosis of MINEN was made on H&E, and confirmed on IHC. One patient was unfit for systemic chemotherapy whereas the other patient received cisplastin and etoposide based chemotherapy. Both patients developed metastasis on follow up but are still alive after 12-15 months. CONCLUSION MINEN is an uncommon tumor of the liver with a poor prognosis as shown by the few studies available. Recurrence and distant metastases are often described even after complete resection and the course is fatal. The role of adjuvant chemotherapy following surgical resection is not fully elucidated. Mean survival in the cases reported ranged from 1 month to 33 months. However, no significant differences were seen in the clinicopathologic profile of the cases described so far. Further multiinstitutional studies and follow up will help to further characterize this subtype for appropriate treatment.
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Affiliation(s)
- Basharat Mubeen
- Department of Pathology, Amrita Institute of Medical Sciences, Kerela, India
| | - Malini Eapen
- Department of Pathology, Amrita Institute of Medical Sciences, Kerela, India
| | - S. Sudhindran
- Department of Surgical Gastroenterology, Amrita Institute of Medical Sciences, Kerela, India
| | - Nikhil Krishna Haridas
- Department of Clinical Hematology, Stem Cell Transplantation and Medical Oncology, Amrita Institute of Medical Sciences, Kerela, India
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Gao X, Wang H, Niu Z, Liu M, Kong X, Sun H, Ma C, Zhu H, Lu J, Zhou X. Case report: mixed large-cell neuroendocrine and hepatocellular carcinoma of the liver. Front Oncol 2024; 13:1309798. [PMID: 38264742 PMCID: PMC10803416 DOI: 10.3389/fonc.2023.1309798] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2023] [Accepted: 12/18/2023] [Indexed: 01/25/2024] Open
Abstract
Background Cases of large-cell neuroendocrine carcinoma (NEC) concomitant hepatocellular carcinoma (HCC) are very rare. Based on the microscopic characteristics, mixed HCC-NEC tumors can be divided into collision type and combined type. We report a patient with both collision and combined type HCC-NEC tumor at the same time. Case presentation A 58-year-old man with hepatitis B and cirrhosis was found to have two masses in segment 5 and segment 8 of the liver, respectively. Preoperative imaging diagnosis was primary liver cancer. Indocyanine green retention test (ICG R15) <10% suggested that the patient can tolerate surgery. Partial hepatectomy was performed under the guidance of 3D reconstruction. Postoperative pathology showed that most of the tumors in S5 were large-cell neuroendocrine carcinoma (90%), and a small part were hepatocellular carcinoma (10%). The tumor in S8 of the liver was diagnosed as HCC combined with immunohistochemistry. After surgery, the patient underwent genetic testing, which indicated mutations in TP53 gene. The test of immune markers of the sample suggest that the patient may benefit little from immune checkpoint inhibitor therapy. The cisplatin and etoposide chemotherapy protocol to the patient following their surgery. Eight month later after the operation, Enhanced CT showed there was no recurrence or metastasis of the tumor. Conclusion The case at hand augments the understanding of HCC-NEC mixed tumors, offering pivotal insights into their precise diagnosis and treatment modalities. Furthermore, we document a favorable prognosis, marked by an absence of recurrence signs thus far-a rarity in comparable instances. This enlightenment stands to facilitate the handling of ensuing cases and enhance patient prognoses.
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Affiliation(s)
- Xin Gao
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
| | - Heng Wang
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
| | - Zheyu Niu
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
- Department of Clinical Research, Qilu Synva Pharmaceutical Co. Ltd., Dezhou, China
- Department of Physiology and Pathophysiology, School of Basic Medical Sciences, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, China
| | - Meng Liu
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Xiaohan Kong
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Hongrui Sun
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Chaoqun Ma
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Huaqiang Zhu
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Jun Lu
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Xu Zhou
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
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Mixed Primary Hepatocellular Carcinoma and Hepatic Neuroendocrine Carcinoma: Case Report and Literature Review. MEDICINA (KAUNAS, LITHUANIA) 2023; 59:medicina59020418. [PMID: 36837619 PMCID: PMC9959776 DOI: 10.3390/medicina59020418] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/17/2023] [Revised: 02/15/2023] [Accepted: 02/17/2023] [Indexed: 02/23/2023]
Abstract
Mixed hepatocellular carcinoma with neuroendocrine carcinoma (HCC-NEC) is extremely rare, comprising about 0.46% of primary hepatic tumors. A 63-year-old man who was a chronic alcoholic presented with a nine-centimeter-sized hepatic mass. His serum alpha-fetoprotein and protein induced by vitamin K antagonist-II levels were 22,815 ng/mL and 183 mAU/mL, respectively. The patient underwent a right hemihepatectomy, including the middle hepatic vein. The tumor consisted of poorly differentiated HCC (20%) and large- and small-cell-type NEC (80%) components as per the pathological examination. Immunohistochemically chromogranin and synaptophysin were positive in the areas of NEC and negative in the areas of HCC. Adjuvant chemotherapy with a combination of cisplatin and etoposide was administered after surgery. At postoperative 5 months, the patient complained of right flank pain, and CT showed a new mass measuring 7.3 cm in the right adrenal gland. Postoperatively, after 6.5 months, more recurred masses were noted on the posterior aspect of the right kidney and both lungs. Although the regimen was changed from etoposide to irinotecan, additional recurred masses were developed in the liver, lung, and brain. He passed away 12 months after the surgery. After reviewing and analyzing previous literature, the 1 and 2 year overall survival rates are 57.3 and 43.6%, respectively, and the 1 and 2 year disease-free survival rates are 36.2 and 29.0%, respectively. Mixed HCC-NEC is a very rare tumor, and the surgical outcome is poor.
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Jeng KS, Huang CC, Chung CS, Chang CF. Liver collision tumor of primary hepatocellular carcinoma and neuroendocrine carcinoma: A rare case report. World J Clin Cases 2022; 10:13129-13137. [PMID: 36569002 PMCID: PMC9782927 DOI: 10.12998/wjcc.v10.i35.13129] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/20/2022] [Revised: 11/08/2022] [Accepted: 11/22/2022] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND Hepatocellular carcinoma (HCC) can occasionally develop with other non-HCC cell types, either in a combined type or collision type. A collision tumor is defined as two histopathologically distinct tumors of the same organ lacking a clear transition zone. Hepatic collision tumors are rare. Among them, “hepatocellular carcinoma-hepatic neuroendocrine carcinoma” (HCC-NEC) collision tumors are especially rare and information about them is rarely published.
CASE SUMMARY A 48-year-old man with typical findings of HCC underwent consecutive therapies, including radiofrequency ablation and embolization prior to resection. Diagnosis of the HCC-NEC collision tumor in the right liver and another HCC in the left liver was established following surgical resection. The patient displayed NEC metastasis following resection and succumbed to septicemia after 2 more rounds of chemotherapy. To our knowledge, this is the 25th reported case of mixed HCC-NEC tumor. The rarity of HCC-NEC collision tumors and the absence of diagnostic criteria make it difficult to differentiate this condition from simple liver tumors, especially in patients with chronic liver disease.
CONCLUSION Our case highlights the difficulty in accurately diagnosing HCC-NEC in the absence of histological evidence. The prognosis is poor for this condition, although ultrasound-guided liver biopsy can be helpful to establish a prompt diagnosis. Further accumulation of such cases could help establish an accurate diagnosis earlier. Early discovery of NEC may allow for better treatment strategies and better prognoses.
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Affiliation(s)
- Kuo-Shyang Jeng
- Department of General Surgery, Far Eastern Memorial Hospital, New Taipei 220, Taiwan
| | - Chun-Chieh Huang
- Department of Radiology, Far Eastern Memorial Hospital, New Taipei 220, Taiwan
| | - Chen-Shuan Chung
- Department of Internal Medicine, Far Eastern Memorial Hospital, New Taipei 220, Taiwan
| | - Chiung-Fang Chang
- Department of Medical Research, Far Eastern Memorial Hospital, New Taipei 220, Taiwan
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Tang J, Zhang LJ, Kang M, Huang R, Shu HY, Wei H, Zou J, Pan YC, Ling Q, Shao Y. AFP and CA-125 as an accurate risk factor to predict eye metastasis in hypertension patients with liver carcinoma: A STROBE-compliant article. Front Genet 2022; 13:1010903. [PMID: 36199582 PMCID: PMC9527270 DOI: 10.3389/fgene.2022.1010903] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2022] [Accepted: 08/30/2022] [Indexed: 11/28/2022] Open
Abstract
Purpose: In this study, we analyzed the differences between hypertension patients with ocular metastasis of liver cancer and those with metastases to other sites, the correlation between history of HBV and liver cancer metastasis, and independent risk factors for ocular metastasis. Methods: We used treatment records from 488 patients with metastases of primary liver cancer from August 2001 to May 2015, divided into two groups based on metastatic sites: OM (ocular metastasis) and NOM (non-ocular, other sites of metastasis) groups. The Student’s t-test and Chi-square test were used to assess the significance of differences between the groups and define the relationship between history of HBV and ocular metastasis of liver cancer. Binary logistic regression analysis was used to identify indicators of ocular metastasis of liver cancer and receiver operating curve (ROC) analyses to estimate their diagnostic value. Results: No significant differences in sex, age, tumor stage, pathological type, or treatment were identified between the OM and NOM groups, while the prevalence of HBV was higher in the former than that in latter. Binary logistic regression demonstrated that AFP and CA-125 were independent indicators of liver metastasis (both p < 0.001). ROC curve analyses generated cut-off values for AFP and CA-125 of 957.2 ng/ml and 114.25 U/ml, respectively, with corresponding AUC values of 0.739 and 0.810. The specificity of the combination of AFP and CA-125 was higher than either factor separately. Discussion: To explore the diagnostic value of AFP and CA125 in predicting the development of ocular metastases of hypertensive patients with liver cancer, which will help us to diagnose the occurrence and development of the disease more accurately and make the best clinical diagnosis and treatment measures.
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Affiliation(s)
- Jing Tang
- Department of Oncology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Li-Juan Zhang
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Min Kang
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Rong Huang
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Hui-Ye Shu
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Hong Wei
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Jie Zou
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Yi-Cong Pan
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Qian Ling
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
| | - Yi Shao
- Department of Ophthalmology, The First Affiliated Hospital of Nanchang University, Jiangxi Center of National Ocular Disease Clinical Research Center, Nanchang, Jiangxi, China
- *Correspondence: Yi Shao,
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Quinn E, Miller N, Capanegro J, Smith T. Small cell neuroendocrine carcinoma presenting as inguinal hernia: A case report. Ann Med Surg (Lond) 2021; 65:102310. [PMID: 33981426 PMCID: PMC8082203 DOI: 10.1016/j.amsu.2021.102310] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2021] [Revised: 04/09/2021] [Accepted: 04/12/2021] [Indexed: 12/05/2022] Open
Abstract
Introduction One of the most common surgical procedures performed annually is inguinal hernia repair. Inguinal hernias are traditionally known to be caused by a weakening in the abdominal wall and precipitated by increased intraabdominal pressure. Recently, intra-abdominal cancer producing the increased intraabdominal pressure, along with metastasis directly into the inguinal canal, have been identified in more studies as causes of inguinal hernias. Presentation of case This case focuses on a unique presentation of small-cell neuroendocrine carcinoma presenting as an inguinal hernia. Discussion This patient's rapid demise and advanced metastatic disease upon presentation is alarming, but his advanced disease process presenting as a routine inguinal hernia is noteworthy. Upon literature analysis, the number of advanced disease processes – most notably cancer – presenting as hernias is significant. Conclusion This case emphasis the importance of perioperative screening, and presents the question, should hernias indicate further workup in the appropriate, at-risk patient populations.
Small cell neuroendocrine carcinoma is a highly malignant, poorly defined cancer with a wide variety of metastasis sites. The clinical presentation of intra-abdominal cancer within inguinal hernia has mounting supporting evidence. Late metastatic small cell lung carcinoma developed in inguinal hernia from an increase in intra-abdominal pressure. Further screening for malignancies in those who present for an evaluation of a hernia may be merited.
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Affiliation(s)
- Erina Quinn
- Lake Erie College of Osteopathic Medicine, Bradenton, United States
| | - Natalie Miller
- Lake Erie College of Osteopathic Medicine, Bradenton, United States
| | - James Capanegro
- Lake Erie College of Osteopathic Medicine, Bradenton, United States
| | - Travis Smith
- Lake Erie College of Osteopathic Medicine, Bradenton, United States
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Nakano A, Hirabayashi K, Yamamuro H, Mashiko T, Masuoka Y, Yamamoto S, Ozawa S, Nakagohri T. Combined primary hepatic neuroendocrine carcinoma and hepatocellular carcinoma: case report and literature review. World J Surg Oncol 2021; 19:78. [PMID: 33726764 PMCID: PMC7968236 DOI: 10.1186/s12957-021-02187-5] [Citation(s) in RCA: 18] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2020] [Accepted: 03/04/2021] [Indexed: 12/14/2022] Open
Abstract
Background Hepatocellular carcinoma (HCC) can grow in a mosaic pattern, often combined with various non-hepatocellular cells. However, HCC combined with a neuroendocrine carcinoma (NEC) component is rarely reported, and its clinical features, origin, diagnosis, and behavior have not been established. In the literature, mixed HCC–NEC tumors are categorized as either collision type or combined type, depending on their microscopic features. Here, we report a patient with a combined-type HCC–NEC tumor. Case presentation An asymptomatic 84-year-old woman was found to have a solid mass in the right lobe of the liver. Laboratory and radiologic examinations showed typical findings of HCC, including arterial-phase enhancement, and portal- and delay-phase washout. She was treated by partial laparoscopic hepatectomy of segment 5. Pathological examination showed that the tumor was predominantly HCC, partly admixed with an NEC component. A transitional zone between the HCC and NEC tissues was also observed. The tumor was finally diagnosed as a combined-type primary mixed NEC–HCC tumor. After the preoperative diagnosis, the patient underwent somatostatin receptor scintigraphy to detect the primary NEC lesion, but no accumulation was found in any other part of her body. She has been free of recurrence for 9 months since the surgery. Conclusion Mixed HCC–NEC tumors are extremely rare, and correct diagnosis requires multidisciplinary collaboration. The accumulation of further cases is needed to help understand the exact pathology, diagnosis, and treatment of this disease.
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Affiliation(s)
- Akira Nakano
- Department of Digestive Surgery, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan.
| | - Kenichi Hirabayashi
- Department of Pathology, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan
| | - Hiroshi Yamamuro
- Department of Radiology, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan
| | - Taro Mashiko
- Department of Digestive Surgery, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan
| | - Yoshihito Masuoka
- Department of Digestive Surgery, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan
| | - Seiichiro Yamamoto
- Department of Digestive Surgery, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan
| | - Soji Ozawa
- Department of Digestive Surgery, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan
| | - Toshio Nakagohri
- Department of Digestive Surgery, Tokai University Hospital, Shimokasuya 143, Isehara, Kanagawa, 259-1143, Japan
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Mixed hepatocellular carcinoma-neuroendocrine carcinoma—A diagnostic and therapeutic challenge. CURRENT PROBLEMS IN CANCER: CASE REPORTS 2020. [DOI: 10.1016/j.cpccr.2020.100020] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
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Mao JX, Teng F, Sun KY, Liu C, Ding GS, Guo WY. Two-in-one: A pooled analysis of primary hepatic neuroendocrine carcinoma combined/collided with hepatocellular carcinoma. Hepatobiliary Pancreat Dis Int 2020; 19:399-403. [PMID: 32359724 DOI: 10.1016/j.hbpd.2020.03.012] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/08/2019] [Accepted: 03/23/2020] [Indexed: 02/05/2023]
Affiliation(s)
- Jia-Xi Mao
- Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Navy Medical University, Shanghai 200003, China
| | - Fei Teng
- Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Navy Medical University, Shanghai 200003, China
| | - Ke-Yan Sun
- Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Navy Medical University, Shanghai 200003, China
| | - Cong Liu
- Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Navy Medical University, Shanghai 200003, China
| | - Guo-Shan Ding
- Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Navy Medical University, Shanghai 200003, China
| | - Wen-Yuan Guo
- Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Navy Medical University, Shanghai 200003, China.
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Xin Q, Lv R, Lou C, Ma Z, Liu GQ, Zhang Q, Yu HB, Zhang CS. Primary hepatic neuroendocrine carcinoma coexisting with distal cholangiocarcinoma: A case report and review of the literature. Medicine (Baltimore) 2020; 99:e20854. [PMID: 32590784 PMCID: PMC7328923 DOI: 10.1097/md.0000000000020854] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022] Open
Abstract
INTRODUCTION Although primary hepatic neuroendocrine carcinomas, whose prognostic mechanisms remain unclear, are rare, coexistence of neuroendocrine carcinomas and other tumors is rarer. In this report, we describe a unique case of coexistence between primary hepatic neuroendocrine carcinoma and a distal cholangiocarcinoma in the pancreas. PATIENT CONCERNS A 64-year-old woman with a history of diabetes, but none of hepatitis, was admitted to hospital because of intermittent epigastric distension and pain discomfort for more than 1 month aggravated 1 day. A contrast-enhanced computed tomography (CT) scan of the upper abdomen and abdominal magnetic resonance imaging (MRI) revealed a thickening of the bile duct wall in the middle and lower segment of common bile duct and the corresponding lumen is narrow and low-density tumors with ring enhancement (1.83 cm × 1.9 cm) in lobi hepatis dexte. DIAGNOSIS Primary neuroendocrine carcinoma of the liver was diagnosed to be coexisting with a distal cholangiocarcinoma, which had invaded the pancreas. Immunohistochemical examination revealed that the neoplastic cells strongly expressed chromogranin A, synaptophysin, and CD56 proteins. The tumor cells did not express HepPar-1, glypican-3, S-100, CK7, and CK19 in the liver tumor. A distal bile duct in pancreatic tissues shows the characteristics of typical bile duct carcinoma, as an invasion of carcinoma is also seen in the pancreatic tissues. Gastrointestinal endoscopy, chest and abdominal CT, abdominal MRI, and positron emission tomography (PET)-CT were used to exclude metastatic neuroendocrine tumors of the liver. INTERVENTIONS Resection of the pancreas-duodenum, the right anterior lobe of the liver, and regional lymph nodes was performed in patients. OUTCOMES The patient had survived for 5 months after the operation. CONCLUSION A unique case of a coexistence of primary hepatic neuroendocrine carcinoma and a distal cholangiocarcinoma, which had invaded the pancreas. No treatment guidelines are established for the treatment of the unique case.
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Affiliation(s)
- Qi Xin
- Department of Pathology, Third Central Hospital of Tianjin, Tianjin Third Central Hospital affiliated to Nankai University, Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases
- Tianjin Key Laboratory of Brain Science and Neural Engineering, Academy of Medical Engineering and Translational Medicine, Tianjin University
| | | | - Cheng Lou
- Department of Hepatobiliary Surgery, Tianjin third central hospital, Tianjin Third Central Hospital affiliated to Nankai University, Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases
| | - Zhe Ma
- Department of Pathology, Third Central Hospital of Tianjin, Tianjin Third Central Hospital affiliated to Nankai University, Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases
| | - Gui-Qiu Liu
- Department of Pathology, Third Central Hospital of Tianjin, Tianjin Third Central Hospital affiliated to Nankai University, Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases
| | - Qin Zhang
- Department of Pathology, Third Central Hospital of Tianjin, Tianjin Third Central Hospital affiliated to Nankai University, Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases
| | - Hai-Bo Yu
- Kidney Disease and Blood Purification Treatment Department, the Second Hospital of Tianjin Medical University, Tianjin, China
| | - Chuan-Shan Zhang
- Department of Pathology, Third Central Hospital of Tianjin, Tianjin Third Central Hospital affiliated to Nankai University, Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases
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Yılmaz DB, Bayramoğlu Z, Ünay G, Ayık E, Başsorgun Cİ, Elpek GÖ. Incidental Collision Tumor of Hepatocellular Carcinoma and Neuroendocrine Carcinoma. J Clin Transl Hepatol 2018; 6:339-344. [PMID: 30271748 PMCID: PMC6160311 DOI: 10.14218/jcth.2017.00076] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/29/2017] [Revised: 04/03/2018] [Accepted: 04/06/2018] [Indexed: 02/05/2023] Open
Abstract
The composite tumors of the liver are very rare, including the coexistence of HCC (hepatocellular carcinoma) with NEC (neuroendocrine carcinoma). The rare occurrence of these tumors necessitates more reported cases in order to fully understand their clinical characteristics, behaviors and treatments. Herein is described an incidental collision tumor of HCC-NEC, along with a review of the literature focusing on their clinicopathological findings and prognosis. The tumor presented here was found incidentally in the hepatectomy specimen of a 56-year-old man who had undergone liver transplantation for rapidly progressive liver failure because of alcoholic hepatitis and cirrhosis. Imaging and laboratory examinations did not demonstrate tumor-related findings. During macroscopic examination, two sharply defined and distinctive areas (1.7 cm and 0.6 cm dimension respectively) were detected among the cirrhotic nodules. The characteristic histopathological features and immunohistochemical findings allowed a diagnosis of HCC-NEC to be made. There was no evidence of recurrence and metastasis after 10 months following surgery. The present case and review revealed that these tumors are frequently found in older ages and males. Although serum markers are valuable in the discrimination of malignant tumors, their absence cannot completely rule out composite HCC-NEC. Diagnosis requires a comprehensive histopathological evaluation together with immunohistochemistry. The NEC component might influence the treatment strategy and eventually the outcome of the patient. In conclusion, the rare occurrence of HCC-NEC and the lack of diagnostic clinical signs and symptoms do not exclude their consideration in the differential diagnosis of liver tumors, especially in patients with the chronic liver disease.
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Affiliation(s)
- Düriye Betül Yılmaz
- Department of Pathology, Akdeniz University Medical School, Konyaalti/Antalya, Turkey
| | - Zeynep Bayramoğlu
- Department of Pathology, Akdeniz University Medical School, Konyaalti/Antalya, Turkey
| | - Gülşah Ünay
- Department of Pathology, Akdeniz University Medical School, Konyaalti/Antalya, Turkey
| | - Erdem Ayık
- Department of Pathology, Akdeniz University Medical School, Konyaalti/Antalya, Turkey
| | | | - Gülsüm Özlem Elpek
- Department of Pathology, Akdeniz University Medical School, Konyaalti/Antalya, Turkey
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Kwon HJ, Kim JW, Kim H, Choi Y, Ahn S. Combined Hepatocellular Carcinoma and Neuroendocrine Carcinoma with Ectopic Secretion of Parathyroid Hormone: A Case Report and Review of the Literature. J Pathol Transl Med 2018; 52:232-237. [PMID: 29794961 PMCID: PMC6056365 DOI: 10.4132/jptm.2018.05.17] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2018] [Accepted: 05/15/2018] [Indexed: 12/19/2022] Open
Abstract
Primary combined hepatocellular carcinoma (HCC) and neuroendocrine carcinoma is a rare entity, and so is hypercalcemia due to ectopic parathyroid hormone (PTH) secretion by tumor. A 44-year old man with hepatitis B virus associated chronic liver disease presented with a hepatic mass. Hemihepatectomy discovered the mass as combined HCC and poorly differentiated cholangiocarcinoma. During adjuvant chemoradiation therapy, he presented with nausea, and multiple systemic metastases were found. Laboratory tests revealed hypercalcemia with markedly elevated PTH and neuron specific enolase. Parathyroid scan showed normal uptake in parathyroid glands, suggestive of ectopic PTH secretion. Subsequently, immunohistochemistry of neuroendocrine marker was performed on the primary lesion, and confirmed the neuroendocrine differentiation in non-HCC component. The patient died 71 days after surgery. This report may suggest the possibility of ectopic PTH secretion by neuroendocrine carcinoma of hepatic origin causing hypercalcemia. Caution for neuroendocrine differentiation should be exercised when diagnosing poorly differentiated HCC.
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Affiliation(s)
- Hyun Jung Kwon
- Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Ji-Won Kim
- Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Haeryoung Kim
- Department of Pathology, Seoul National University Hospital, Seoul, Korea
| | - YoungRok Choi
- Department of Surgery, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Soomin Ahn
- Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Korea
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