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  1. Mohd Kamil A, Draman CR, Seman MR, Kalavathy R, Mubarak MY
    Saudi J Kidney Dis Transpl, 2016 Jul-Aug;27(4):787-90.
    PMID: 27424699 DOI: 10.4103/1319-2442.185259
    Malignancy is a significant long-term complication of successful renal transplantation. Not only the rate is higher but also cases are highly aggressive. We report a case of metastatic, small cell, neuroendocrine tumor in a post-renal transplant patient with progressive left inguinal nodes and right lumbar swellings. He had a remarkably elevated serum lactate dehydrogenase levels with multiple metastatic masses in the left inguinal, left iliac fossa, and right lower abdominal wall on abdominal computed tomography scan. Excisional biopsy of a left inguinal node revealed extensive infiltration with malignant cells in it. Immunohistochemistry staining was positive for synaptophysin, prostatic specific antigen, and CD56. While the primary small cell carcinoma of genitourinary organs is rare, the case illustrates the highly aggressive nature of the disease in a kidney transplant recipient.
    Matched MeSH terms: Neuroendocrine Tumors*
  2. Kamarudin Z, Jamaluddin N, Latar NHM, Pauzi SHM, Muhammad R
    J Cancer Res Ther, 2023 Oct 01;19(7):2104-2107.
    PMID: 38376332 DOI: 10.4103/jcrt.jcrt_1506_21
    High-grade neuroendocrine carcinoma (NEC) of the tonsil is rare and has a poor prognosis. The usual presentation is a neck mass with locoregional cervical lymphadenopathy. An axillary lymphadenopathy as a primary presentation of NEC of the tonsils is uncommon and challenging to treat. Tonsil neuroendocrine tumors display aggressive behaviors associated with early recurrence and metastasis after surgical resection. Managing this condition is demanding compared to NECs of gastrointestinal origin since, to date, the management of head-and-neck neuroendocrine tumors is still not well established. We present a 49-year-old female with a rare case of NEC of the tonsil presenting primarily with axillary lymph nodes metastasis. The patient's axillary lymph node was biopsied and revealed a Grade III neuroendocrine tumor. A positron emission tomography (PET) scan was done in searching of a primary lesion and showed a highly metabolic mass of the left tonsil as well as a left axillary lymph node suggestive of metastasis. The patient has been managed with a multimodality approach, with a combination of chemotherapy regimen and surgical resection of the axillary lymph node. Subsequent PET scan evaluation showed a complete response of the primary tumor with residual left axillary lymph node metastasis. NECs of tonsil presented with axillary lymph nodes metastasis is rare and has a poor prognostic outcome. It poses a dilemma with regard to management, as surgical resection of the metastasis is not promising given the possibility of early recurrence.
    Matched MeSH terms: Neuroendocrine Tumors*
  3. Dahiya D, Raman K, Singh H
    Pol Przegl Chir, 2011 Nov;83(11):627-9.
    PMID: 22246096 DOI: 10.2478/v10035-011-0099-0
    The report presents a rare case of carcinoid tumor in a 17 year female who presented with epigastric pain of one week duration. She was diagnosed to have type I choledochal cyst on abdominal ultrasound and MRI. She underwent total excision of choledochal cyst with roux-en-Y hepaticojejunostomy. Histopathological examination revealed a neuroendocrine tumor within choledochal cyst which was immunoreactive for Chromogranin A. Patient is well at 6 months of follow up. These tumors are characteristically slow-growing, therefore awareness of its presence preoperatively can facilitate optimal management by performing surgical resection with negative margins which offers the best chance of long-term survival.
    Matched MeSH terms: Neuroendocrine Tumors/diagnosis*; Neuroendocrine Tumors/pathology; Neuroendocrine Tumors/surgery*
  4. Arumugam M, Jamil A, Amiseno RA, Rosli N, Abdul Shukor N
    Malays J Pathol, 2020 Aug;42(2):277-281.
    PMID: 32860382
    INTRODUCTION: Merkel cell carcinoma (MCC) is a rare and aggressive malignancy of the skin, with poor clinical outcomes. Typical conditions include a rapidly growing, solitary dome-shaped, violaceous nodule. Several root causes have been identified - sun exposure, age, lighter skin, immunocompromised state, and polyomavirus infection. Wide local excision is the best treatment. The tumour is radiotherapy-responsive. However, the success rate of the treatment with chemotherapy is rather limited. Immunotherapy has shown promising results. Early detection is important to prevent morbidity and mortality.

    CASE REPORT: In this literature work, we reported on a particular case of MCC, as exhibited by an 84-year-old Chinese woman, and discussed the clinical features and management of MCC.

    DISCUSSION: We highlighted that MCC cases have a link to the polyomavirus 5. Patients who were identified with the Polyomavirus 5, and underwent immunotherapy, were seen to depict much better prognosis.

    Matched MeSH terms: Neuroendocrine Tumors/etiology; Neuroendocrine Tumors/pathology; Neuroendocrine Tumors/surgery
  5. Malays J Pathol, 2018 Apr;40(1):79-82.
    PMID: 29704389
    No abstract available.
    Matched MeSH terms: Neuroendocrine Tumors/classification*; Neuroendocrine Tumors/pathology*
  6. Ignee A, Jenssen C, Arcidiacono PG, Hocke M, Möller K, Saftoiu A, et al.
    Endoscopy, 2018 11;50(11):1071-1079.
    PMID: 29689572 DOI: 10.1055/a-0588-4941
    BACKGROUND: The prevalence of malignancy in patients with small solid pancreatic lesions is low; however, early diagnosis is crucial for successful treatment of these cases. Therefore, a method to reliably distinguish between benign and malignant small solid pancreatic lesions would be highly desirable. We investigated the role of endoscopic ultrasound (EUS) elastography in this setting.

    METHODS: Patients with solid pancreatic lesions ≤ 15 mm in size and a definite diagnosis were included. Lesion stiffness relative to the surrounding pancreatic parenchyma, as qualitatively assessed and documented at the time of EUS elastography, was retrospectively compared with the final diagnosis obtained by fine-needle aspiration/biopsy or surgical resection.

    RESULTS: 218 patients were analyzed. The average size of the lesions was 11 ± 3 mm; 23 % were ductal adenocarcinoma, 52 % neuroendocrine tumors, 8 % metastases, and 17 % other entities; 66 % of the lesions were benign. On elastography, 50 % of lesions were stiffer than the surrounding pancreatic parenchyma (stiff lesions) and 50 % were less stiff or of similar stiffness (soft lesions). High stiffness of the lesion had a sensitivity of 84 % (95 % confidence interval 73 % - 91 %), specificity of 67 % (58 % - 74 %), positive predictive value (PPV) of 56 % (50 % - 62 %), and negative predictive value (NPV) of 89 % (83 % - 93 %) for the diagnosis of malignancy. For the diagnosis of pancreatic ductal adenocarcinoma, the sensitivity, specificity, PPV, and NPV were 96 % (87 % - 100 %), 64 % (56 % - 71 %), 45 % (40 % - 50 %), and 98 % (93 % - 100 %), respectively.

    CONCLUSIONS: In patients with small solid pancreatic lesions, EUS elastography can rule out malignancy with a high level of certainty if the lesion appears soft. A stiff lesion can be either benign or malignant.

    Matched MeSH terms: Neuroendocrine Tumors/drug therapy*; Neuroendocrine Tumors/pathology
  7. Kaewput C, Suppiah S, Vinjamuri S
    World J Nucl Med, 2018 Jan-Mar;17(1):34-40.
    PMID: 29398963 DOI: 10.4103/wjnm.WJNM_16_17
    The aim of our study was to correlate tumor uptake of 68Ga-DOTA-NOC positron emission tomography/computed tomography (PET/CT) with the pathological grade of neuroendocrine tumors (NETs). 68Ga-DOTA-NOC PET/CT examinations in 41 patients with histopathologically proven NETs were included in the study. Maximum standardized uptake value (SUVmax) and averaged SUV SUVmean of "main tumor lesions" were calculated for quantitative analyses after background subtraction. Uptake on main tumor lesions was compared and correlated with the tumor histological grade based on Ki-67 index and pathological differentiation. Classification was performed into three grades according to Ki-67 levels; low grade: Ki-67 <2, intermediate grade: Ki-67 3-20, and high grade: Ki-67 >20. Pathological differentiation was graded into well- and poorly differentiated groups. The values were compared and evaluated for correlation and agreement between the two parameters was performed. Our study revealed negatively fair agreement between SUVmax of tumor and Ki-67 index (r = -0.241) and negatively poor agreement between SUVmean of tumor and Ki-67 index (r = -0.094). SUVmax of low-grade, intermediate-grade, and high-grade Ki-67 index is 26.18 ± 14.56, 30.71 ± 24.44, and 6.60 ± 4.59, respectively. Meanwhile, SUVmean of low-grade, intermediate-grade, and high-grade Ki-67 is 8.92 ± 7.15, 9.09 ± 5.18, and 3.00 ± 1.38, respectively. As expected, there was statistically significant decreased SUVmax and SUVmean in high-grade tumors (poorly differentiated NETs) as compared with low- and intermediate-grade tumors (well-differentiated NETs). SUV of 68Ga-DOTA-NOC PET/CT is not correlated with histological grade of NETs. However, there was statistically significant decreased tumor uptake of 68Ga-DOTA-NOC in poorly differentiated NETs as compared with the well-differentiated group. As a result of this pilot study, we confirm that the lower tumor uptake of 68Ga-DOTA-NOC may be associated with aggressive behavior and may, therefore, result in poor prognosis.
    Matched MeSH terms: Neuroendocrine Tumors
  8. Kavitha Nagandla, Sharifah Sulaiha, Sivalingam Nalliah, Norfadzilah Mohd Yusof
    MyJurnal
    Neuroendocrine carcinoma of the female
    reproductive tract are a heterogeneous group of rare
    neoplasms posing both diagnostic and therapeutic
    challenges. The recent classification by WHO
    includes neuroendocrine carcinomas (NECs) and
    neuroendocrine tumours (NETs). NECs are the poorly
    differentiated small cell carcinoma (SCNEC) and
    large cell neuroendocrine carcinoma (LCNEC), while
    well-differentiated NETs include typical carcinoids
    (TC) and atypical carcinoids (AC). Majority of
    these tumours have an aggressive clinical course and
    published data is supportive of multi-modal therapeutic
    strategies. Etoposide/platinum based chemotherapy is
    commonly advocated. Histopathological categorisation
    and diagnosis are paramount to guide therapy.
    Well-differentiated carcinoid and atypical
    carcinoid tumours should be managed similar to
    gastroenteropancreatic neuroendocrine tumours.
    This review discusses the current classification, clinicpathologic
    characteristics and advances in the diagnostic
    evaluation and the treatment options of neuroendocrine
    carcinoma of the cervix.
    Matched MeSH terms: Neuroendocrine Tumors
  9. Azizan, N., Hayati, F., Madatang, A., Abu Seman, F.
    MyJurnal
    Endometrial stromal sarcoma (ESS) is a rare malignant tumour of the endometrium, accounts for less than 1% of all
    uterine malignancies. Routinely, it is diagnosed morphologically, supported by immunomarkers of CD10 and
    vimentin. CD56 is used widely in neuroendocrine tumour. In our current practice, CD56 is not used to support the
    diagnosis of ESS. We present a case of a postmenopausal lady with advanced ESS who had expression of CD56
    upon immunohistochemical study
    Matched MeSH terms: Neuroendocrine Tumors
  10. Mohd Kamil MK, Ngiu CS, Md Isa N, Yaacob Y Y, Deborah Chew CH, Wong ZQ, et al.
    Med J Malaysia, 2018 02;73(1):60-62.
    PMID: 29531208 MyJurnal
    Neuroendocrine neoplasm is an epithelial neoplasm with predominant neuroendocrine differentiation that can arise from many organs in the body. We reported a rare case of gastric neuroendocrine carcinoma which accounts for less than 1% of all gastric tumours that is associated with poor prognosis. The recognition of this rare tumour in early stage is challenging and high suspicious into it might bring to early detection and so forth might improve the prognostication.
    Matched MeSH terms: Neuroendocrine Tumors
  11. Koh KS, Telisinghe PU, Bickle I, Abdullah MS, Chong CF, Chong VH
    Asian Pac J Cancer Prev, 2015;16(8):3279-83.
    PMID: 25921132
    BACKGROUND: Colorectal cancer (CRC) is the most common gastrointestinal cancer and the incidence is increasing. CRC is more common with increasing age, but a proportion occurs in young adults, termed young CRC. This study assessed the incidence and the demographic of young CRC in Brunei Darussalam.

    MATERIALS AND METHODS: All histologically proven CRC between 1986 and 2014 registered with the Department of Pathology cancer registry were reviewed and data extracted for analyses. Young CRC was defined as cancer in patients aged less than 45 years. The various population groups were categorized into locals (Malays, Chinese and Indigenous) and expatriates.

    RESULTS: Over the study period, there were 1,126 histologically proven CRC (mean age 59.1 ± 14.7 years, Male 58.0%, Locals 91.8% and 8.2% expatriates). Young CRC accounted for 15.1% with the proportion declining over the years, from 29% (1986-1990) to 13.2% (2011-2014). The proportion of young CRC was highest among the indigenous (30.8%), followed by the expatriates (29.3%), Malays (14.3%) and lowest among the Chinese (10.8%). The mean age of young CRC was 35.9 ± 6.2; lowest among the indigenous (33.5 ± 6.7), expatriate (34.9 ± 6.0) groupd and the Malays (35.6 ± 6.5) compared to the Chinese (38.6 ± 4.6), a similar trend being observed in the non-young CRC groups. There were no difference between the genders and tumor locations (rectum or colon) between the young and the non-young CRC cases. Female young CRC was significantly younger than male (p<0.05) without any significant variation between the various population groups (p>0.05).

    CONCLUSIONS: Our study showed that the young CRC accounted for 15.1% of all CRC with declining trend observed over recent years. Young CRC was more common among indigenous, expatriates and Malays and least common among the Chinese. There were no differences in the gender and tumor locations.
    Matched MeSH terms: Neuroendocrine Tumors/ethnology; Neuroendocrine Tumors/epidemiology*; Neuroendocrine Tumors/pathology
  12. Kang WH, Wahab NA, Kamaruddin NA
    J ASEAN Fed Endocr Soc, 2020;35(1):102-104.
    PMID: 33442175 DOI: 10.15605/jafes.035.01.16
    Goblet cell carcinoid (GCC) is a rare neoplasm of the vermiform appendix and can be mistaken as a typical neuroendocrine tumour (TNET). The natural history of this disease is more aggressive compared to TNETs and requires a more aggressive approach. We report a case of a 37-year-old male who was initially diagnosed with TNET, but subsequently revised as Tang's A GCC. He underwent appendectomy and right hemicolectomy. Aside from a persistently elevated carcinoembyrogenic antigen (CEA) result, his 18F-fluorodeoxyglucose (FDG) PET/CT and a 68-Gallium DOTATATE PET/CT scan showed no FDG or DOTATATE avid lesions.
    Matched MeSH terms: Neuroendocrine Tumors
  13. Chan KH, Lee CH, Sharif SZ, Hayati F, Sallapan S
    Ann Med Surg (Lond), 2020 Dec;60:438-441.
    PMID: 33251002 DOI: 10.1016/j.amsu.2020.11.035
    Background: Metastatic neuroendocrine tumours (NETs) to the breast are very rare entities.

    Case presentation: A 26-year-old lady presented with anterior neck swelling with symptoms of superior vena cava syndrome for 6 months. Imaging study revealed a mediastinal mass which was preceded with core biopsy which was consistent with high-grade small cell NETs. Despite second-line adjuvant chemotherapy and radiotherapy, her disease became advanced which was confirmed via restaging scan. There were bilateral breast lesions discovered during the scan which was deemed to be metastatic NETs histologically. Despite prompt initiation of treatment, she succumbed 1 year after the radiotherapy due to disease progression.

    Conclusion: High suspicion of an index is needed for diagnosis when patients with known primary NETs present with suspicious breast lesions. Triple assessment is mandatory, however histopathology assessment and immunohistochemistry staining are the mainstay of diagnosis.

    Matched MeSH terms: Neuroendocrine Tumors
  14. Wong L, Kanthasamy SV, Durairaj G, Thangaratnam RR
    Int J Surg Case Rep, 2020;77:48-52.
    PMID: 33137672 DOI: 10.1016/j.ijscr.2020.10.091
    INTRODUCTION: Intussusception usually occurs in the paediatric population. When it occurs in the adult population, it is normally caused by a malignant intraluminal pathology.

    PRESENTATION OF CASE: A 72-year-old female presented to us with right-sided abdominal pain for 3 weeks, associated with vomiting and diarrhoea. She had an appendectomy done 30 years ago and a recent myocardial infarction. Abdominal examination revealed a previous appendectomy scar and tenderness over the right lumbar region. Computed tomography showed ileocaecal intussusception. Right hemicolectomy with a double barrel stoma was performed as she was unstable intraoperatively. Histopathological examination of the tumour showed a well-differentiated neuroendocrine tumour. Subsequent PET scan showed no systemic disease and a reversal of the stoma was done. She remained disease free for a year.

    DISCUSSION: Our patient had undergone a right hemicolectomy despite the high risk of mortality, as there is a high chance of malignancy. Double barrel stoma was done, as she was unstable intraoperatively. Fortunately, she recovered well and had her stoma reversed without any further recurrence of her disease.

    CONCLUSION: Adult patients who present with intussusception should be managed with resection, as there is a high possibility of a malignancy. Early resection should be planned to prevent further spread of the tumour.

    Matched MeSH terms: Neuroendocrine Tumors
  15. Tan TH, Boey CY, Lee BN
    Nucl Med Mol Imaging, 2018 Apr;52(2):119-124.
    PMID: 29662560 DOI: 10.1007/s13139-017-0496-3
    Purpose: The National Cancer Institute is the only referral centre in Malaysia that provides 68Ga-DOTA-peptide imaging. The purpose of this study is to determine the impact of 68Ga-DOTA-peptide PET/CT on the management of gastrointestinal neuroendocrine tumours (GI-NET).

    Materials and Methods: A cross-sectional study was performed to review the impact of 68Ga-DOTA-peptide (68Ga-DOTATATE or 68Ga-DOTATOC) PET/CT on patients with biopsy-proven GI-NET between January 2011 and December 2015. Suspected NET was excluded. Demographic data, tumoral characteristics, change of disease stage, pre-PET intended management and post-PET management were evaluated.

    Results: Over a 5-year period, 82 studies of 68Ga-DOTA-peptide PET/CT were performed on 44 GI-NET patients. The most common primary site was the rectum (50.0%) followed by the small bowel, stomach and colon. Using WHO 2010 grading, 40.9% of patients had low-grade (G1) tumour, 22.7% intermediate (G2) and 4.5% high (G3). Of ten patients scheduled for pre-operative staging, 68Ga-DOTA-peptide PET/CT only led to therapeutic change in three patients. Furthermore, false-negative results of 68Ga-DOTA-peptide PET/CT were reported in one patient after surgical confirmation. However, therapeutic changes were seen in 20/36 patients (55.6%) scheduled for post-surgical restaging or assessment of somatostatin analogue (SSA) eligibility. When 68Ga-DOTA-peptide PET/CT was used for monitoring disease progress during systemic treatment (sandostatin, chemotherapy, everolimus and PRRT) in metastatic disease, impact on management modification was seen in 19/36 patients (52.8%), of which 84.2% had inter-modality change (switch to everolimus, chemotherapy or PRRT) and 15.8% had intra-modality change (increased SSA dosage).

    Conclusions: 68Ga-DOTA-peptide PET/CT has a significant impact on management decisions in GI-NET patients as it can provide additional information on occult metastasis/equivocal lesions and supply the clinician an opportunity to select patients for targeted therapy.

    Matched MeSH terms: Neuroendocrine Tumors
  16. Gunavathy M, Rohana AG, Norlela S, Nor Azmi K
    Med J Malaysia, 2014 Jun;69(3):133-7.
    PMID: 25326355 MyJurnal
    Gastroenteropancreatic neuroendocrine tumours (GEP- nETs) are rare neoplasms with a complex spectrum of presentation. The study cohort (n=64) included the diagnoses of carcinoid, (n=26, 41%), insulinoma, (n=25, 39%), undetermined (n=10, 16%), VIPoma, glucagonoma and multiple endocrine neoplasia (MEn-1) (n= 3). Almost half of the patients (n=31) had distant metastasis at diagnosis, the commonest being carcinoid tumours. Presenting symptoms were due to either hormonal expressions or mass effects. diagnoses in all patients were made based on positive immunohistochemical staining for chromogranin and synaptophysin. Less than half (n=30) had either serum chromogranin A, urinary 5-hydroxyindole acetic acid (5-hIAA), serum insulin or C-peptide levels performed. Commonest diagnostic imaging modalities were computed tomography (CT) scan (94%) and abdominal ultrasound (15%). Curative or palliative surgery was performed in 58 patients. Systemic therapy included long acting somatostatin analogues (n=14), chemotherapy (n=7) and interferon-α2b (n=1). nine patients died, all of who had metastatic disease at diagnosis. All patients with insulinoma (n=25) were assessed by endocrinologists whilst carcinoid tumours were mainly managed by surgeons (n=16/26). Involvements of oncologists and gastroenterologists were minimal. This study showed that patients with GEP-nETs in Malaysia commonly presented late in the disease with presence of distant metastases. Less than half had adequate hormonal and biochemical examinations performed for diagnostic as well as prognostic purposes, and only a third received systemic therapy. Lack of institutionalbased database, clinical expertise and multi-disciplinary involvement contributed to the inadequate surveillance and management of the disease.
    Matched MeSH terms: Neuroendocrine Tumors
  17. Nur Hidayah Bahrom, Anis Safura Ramli, Nor Suraya Samsudin, Norliana Dalila Mohamad Ali, Nor Salmah Bakar
    MyJurnal
    This is a case of a 62-year-old Indian man who was diagnosed with a rare type of lung
    neuroendocrine tumour (NET) of atypical carcinoid (AC) subtype which comprises only 0.1%–
    0.2% of pulmonary neoplasms. He initially presented to a private hospital in May 2018 with a
    6-month history of chronic productive cough and haemoptysis. Chest X-Ray (CXR), CT scan,
    bronchoscopy, biopsy and broncho-alveolar lavage were conducted. At this stage, imaging and
    histopathological investigations were negative for malignancy. Diagnosis of bronchiectasis was
    made and he was treated with antibiotic and tranexamic acid. Due to financial difficulties, his
    care was transferred to a university respiratory clinic in June 2018. His condition was monitored
    with CXR at every visit and treatment with tranexamic acid was continued for 6 months.
    However, due to persistent haemoptysis, he presented to the university primary care clinic in
    Dec 2018. Investigations were repeated in January 2019 where his CXR showed increased
    opacity of the left retrocardiac region and CT scan revealed a left lower lobe endobronchial
    mass causing collapse with mediastinal lymphadenopathy suggestive of malignancy.
    Bronchoscopy, biopsy and histopathology confirmed the presence of NET. Although the Ki-67
    index was low, the mitotic count, presence of necrosis and evidence of liver metastases
    favoured the diagnosis of AC. A positron emission tomography Ga-68 DONATOC scan showed
    evidence of somatostatin receptor avid known primary malignancy in the lungs with suspicions
    of liver metastasis. He was subsequently referred to the oncology team and chemotherapy was
    initiated. This case highlights the challenge in diagnosis and management of patients with AC.
    Physicians ought to be vigilant and have a high index of suspicion in patients who present with
    persistent symptoms on multiple visits. Early diagnosis of NET would prevent metastasis and
    provide better prognosis. Continuous follow-up shared care between primary care and
    secondary care physicians is also essential to provide ongoing psychosocial support for
    patients with NET, especially those with metastatic disease
    Matched MeSH terms: Neuroendocrine Tumors
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