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Liu Z, Yang F, Hao Y, Jiang Q, Zhang Y, Zheng Q, Jiang Y, Lu J, Gao H. Case report: Surgical treatment and literature review of a recurrent case of glucagonoma. Front Oncol 2024; 14:1437102. [PMID: 39252948 PMCID: PMC11381282 DOI: 10.3389/fonc.2024.1437102] [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: 05/23/2024] [Accepted: 07/31/2024] [Indexed: 09/11/2024] Open
Abstract
A 40-year-old male patient was admitted due to abdominal distension and discomfort in the upper abdomen persisting for three days. Enhanced CT of the upper abdomen revealed an irregularly dense soft tissue area in the body and tail of the pancreas, approximately 7.6 × 3.1 cm in size, with blurred boundaries, and indistinct separation from the splenic artery and vein. Multiple liver lesions of varying sizes and slightly lower densities were also observed. Liver tumor biopsy considering a neuroendocrine tumor G2, combined with the medical history, led to a diagnosis of pancreatic neuroendocrine tumor G2 with liver metastasis. Physical examination showed mild tenderness in the upper abdomen but no other significant positive signs. During treatment, the patient developed multiple red papular rashes around the mouth, on both lower limbs, and the perineum, accompanied by itching. The glucagon level was 1138.3 pg/L. The patient underwent resection of the pancreatic body and tail, splenectomy, partial liver tumor resection, and cholecystectomy. Within five days post-surgery, the skin lesions began to crust and flake off. On the 14th day post-surgery, the serum glucagon level was rechecked at 136.4 pg/L. As of April 2024, progression of liver lesions was noted, with no significant skin symptoms during the period.
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Affiliation(s)
- Zhipeng Liu
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Faji Yang
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Yijie Hao
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Qirong Jiang
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Yisu Zhang
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
| | - Qixuan Zheng
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
| | - Yupeng Jiang
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
| | - Jun Lu
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
| | - Hengjun Gao
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong First Medical University, Jinan, China
- Department of Hepatobiliary Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China
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Zhan SP. Necrolytic migratory erythema caused by pancreatic hyperglycemia with emphasis on therapeutic and prognosis: A case report. World J Clin Cases 2024; 12:5404-5409. [PMID: 39156090 PMCID: PMC11238691 DOI: 10.12998/wjcc.v12.i23.5404] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2024] [Revised: 05/10/2024] [Accepted: 06/13/2024] [Indexed: 07/05/2024] Open
Abstract
BACKGROUND With the incidence of pancreatic diseases increasing year by year, pancreatic hyperglycemia, as one of the common complications, is gradually gaining attention for its impact on the skin health of patients. CASE SUMMARY This was the case of an elderly female with clinical manifestations of necrolytic migratory erythema, "three more and one less," diabetes mellitus, hypertension, anemia, hypoproteinemia, and other syndromes, which had been misdiagnosed as eczema. Abdominal computed tomography showed a pancreatic caudal space-occupying lesion, and the magnetic resonance scanning of the epigastric region with dynamic enhancement and diffusion-weighted imaging suggested a tumor of the pancreatic tail, which was considered to be a neuroendocrine tumor or cystadenoma. The patient was referred to a more equipped hospital for laparoscopic pancreatic tail resection. Post-surgery diagnosis revealed a neuroendocrine tumor in the tail of the pancreas. To date, the patient's general condition is good, and she is still under close follow-up. CONCLUSION Necrolytic migratory erythema can be induced by endocrine system tumors or endocrine metabolic abnormalities, with complex clinical manifestations, difficult diagnosis, and easy misdiagnosis by dermatologists. The initial treatment principles in dermatology include symptomatic supportive therapy and effective drugs to relieve skin lesions. After clarifying the etiology of glucagonoma, comprehensive treatment in collaboration with endocrinologists, general surgeons, and oncologists can help provide individualized treatment for patients and improve their prognosis.
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Affiliation(s)
- Shi-Ping Zhan
- Department of Dermatology, General Hospital of the Yangtze River Shipping, Wuhan Brain Hospital, Wuhan 430000, Hubei Province, China
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Yu S, Ge M, Zhang C, Chen L, Zhao L. Diagnosis and comprehensive treatment of a glucagonoma in a patient with residual intrahepatic metastases postoperatively: A case report and literature review. Oncol Lett 2024; 27:202. [PMID: 38516680 PMCID: PMC10955682 DOI: 10.3892/ol.2024.14336] [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: 07/08/2023] [Accepted: 02/01/2024] [Indexed: 03/23/2024] Open
Abstract
Glucagonomas are rare neuroendocrine neoplasms of the pancreas with malignant potential. At present, their epidemiology is not entirely clear, so clinicians are not well versed, lacking any consensus on diagnosis or comprehensive treatment. The present study reports the case of a 32-year-old woman hospitalized for recurrent glossitis, perioral dermatitis and necrolytic migratory erythema (NME) of both lower limbs. Imaging studies revealed a low-density nodule (~2 cm) in the tail of the pancreas, as well as multiple space-occupying hepatic lesions. Surgical intervention was then selected, and distal pancreatectomy, splenectomy and palliative metastasectomies were performed. Tissue examination subsequently confirmed a primary pancreatic neuroendocrine tumor (grade 2), metastatic to the liver. The NME resolved postoperatively, aided by intramuscular injections of long-acting release octreotide (30 mg) every 28 days. A series of three percutaneous ablative treatments (microwave ablation) were also undertaken within a 2-year period, targeting the liver metastases. The present condition of the patient is good, with no cutaneous relapse to date. Palliative metastasectomies, in conjunction with ablative treatments and combination somatostatin analog (SSA) use, are unique aspects of this case that, to the best of our knowledge, have yet to be documented in the literature. Surgical palliation may benefit patients with liver involvement and prolong their survival time. Likewise, ablative treatments and SSA injections delivered together not only address hepatic spread, but also control hormone-related symptoms, having a positive impact on prognosis. As glucagonomas are so rare, there is no real agreement on their management. The present study aims to guide clinical practice by adding further to the available data.
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Affiliation(s)
- Shenghui Yu
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of China Medical University, Shenyang, Liaoning 110001, P.R. China
| | - Muxi Ge
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of China Medical University, Shenyang, Liaoning 110001, P.R. China
| | - Cheng Zhang
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of China Medical University, Shenyang, Liaoning 110001, P.R. China
| | - Lang Chen
- Department of Pancreatic and Biliary Surgery, The First Hospital of China Medical University, Shenyang, Liaoning 110001, P.R. China
| | - Lei Zhao
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of China Medical University, Shenyang, Liaoning 110001, P.R. China
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Sandru F, Carsote M, Albu SE, Valea A, Petca A, Dumitrascu MC. Glucagonoma: From skin lesions to the neuroendocrine component (Review). Exp Ther Med 2020; 20:3389-3393. [PMID: 32905095 PMCID: PMC7465236 DOI: 10.3892/etm.2020.8966] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2020] [Accepted: 05/15/2020] [Indexed: 02/06/2023] Open
Abstract
Glucagonoma is a hormonally active rare pancreatic neuroendocrine tumour causing an excess of glucagon. This is a narrative review based on a multidisciplinary approach of the tumour. Typically associated dermatosis is necrolytic migratory erythema (NME) which is most frequently seen at disease onset. Insulin-dependent diabetes mellitus, depression, diarrhoea, deep vein thrombosis are also identified, as parts of so-called 'D' syndrome. Early diagnosis is life saving due to potential aggressive profile and high risk of liver metastasis. NME as paraneoplastic syndrome may be present for months and even years until adequate recognition and therapy; it is remitted after successful pancreatic surgery. Thus the level of practitioners' awareness is essential. If surgery is not curative, debulking techniques may improve the clinical aspects and even the outcome in association with other procedures such as embolization of hepatic metastasis; ablation of radiofrequency type; medical therapy including chemotherapy, targeted therapy with mTOR inhibitors such as everolimus, PRRT (peptide receptor radiotherapy), and somatostatin analogues (including combinations of medical treatments). Increased awareness of the condition involves multidisciplinary practitioners.
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Affiliation(s)
- Florica Sandru
- Department of Dermatology, ‘Elias’ Emergency University Hospital, 125100 Bucharest, Romania
- Department of Dermatology, ‘Carol Davila’ University of Medicine and Pharmacy, 020021 Bucharest, Romania
| | - Mara Carsote
- Department of Endocrinology,‘C.I. Parhon’ National Institute of Endocrinology, 011863 Bucharest, Romania
- Department of Endocrinology, ‘Carol Davila’ University of Medicine and Pharmacy, 020021 Bucharest, Romania
| | - Simona Elena Albu
- Department of Gynecology, Emergency University Hospital, 050098 Bucharest, Romania
- Department of Gynecology, ‘Carol Davila’ University of Medicine and Pharmacy, 020021 Bucharest, Romania
| | - Ana Valea
- Department of Endocrinology, Clinical County Hospital, 400000 Cluj-Napoca, Romania
- Department of Endocrinology, ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania
| | - Aida Petca
- Department of Dermatology, ‘Elias’ Emergency University Hospital, 125100 Bucharest, Romania
| | - Mihai Cristian Dumitrascu
- Department of Gynecology, Emergency University Hospital, 050098 Bucharest, Romania
- Department of Gynecology, ‘Carol Davila’ University of Medicine and Pharmacy, 020021 Bucharest, Romania
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Shen C, He J, Le X, Zheng L, Cao D. Application of Somatostatin, Chemotherapy Combined with TAE in Heterogeneous Glucagonoma Presented with Necrolytic Migratory Erythema. Onco Targets Ther 2019; 12:11339-11344. [PMID: 31920326 PMCID: PMC6929939 DOI: 10.2147/ott.s237634] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2019] [Accepted: 12/07/2019] [Indexed: 02/05/2023] Open
Abstract
Glucagonoma, a rare neuroendocrine tumor of the pancreas, which is often misdiagnosed because of non-characteristic clinical manifestations. In addition, the treatment has not been well established for this disease so far. We here report a case of glucagonoma previous misdiagnosed as recurrent erythema. In this case, necrolytic migratory erythema was the main clinical manifestation, and he received surgical resection after admission although with liver metastasis. Postoperative pathological results showed that the heterogeneity of proliferative index in primary (Ki-67: 5~10%) and metastatic (Ki-67: 25~30%) tumors were obviously observed. One month postoperatively, abdominal CT and MRI showed multiple liver metastasis (type III) again. Interestingly, the skin rash was obviously improved after treatment with somatostatin combined with chemotherapy (octreotide, temozolomide and capecitabine). Subsequently, the patient received transarterial embolization (TAE). Up to now, no progression was noted for liver metastasis. Due to its rarity, clinical diagnosis is challenging; thus, further understanding of the disease by clinicians is helpful for early diagnosis and treatment, so as to improve the prognosis of patients.
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Affiliation(s)
- Chaoyong Shen
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, People's Republic of China
| | - Jiaoxue He
- Department of Oncology, Wenjiang District People's Hospital, Chengdu, Sichuan, People's Republic of China
| | - Xiaoyan Le
- Department of Oncology, Wenjiang District People's Hospital, Chengdu, Sichuan, People's Republic of China
| | - Linmao Zheng
- Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, People's Republic of China
| | - Dan Cao
- Department of Abdominal Oncology, Cancer Center of West China Hospital, Sichuan University, Chengdu 610041, Sichuan, People's Republic of China
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