Postoperative Surprises: A Carcinoid Tumor Within a Perforated Meckel's Diverticulum.

Meckel's diverticulum, a true diverticulum originating from the incomplete closure of the vitelline duct during embryologic development, rarely presents with carcinoid tumors. The coexistence of a Meckel's diverticulum and carcinoid tumor following laparoscopic sigmoid colectomy for diverticulitis is an uncommon phenomenon, with limited documented cases in the literature. We present a case of a 74-year-old male with a past medical history of hypertension and diverticulitis who underwent a laparoscopic sigmoid colectomy for dysplastic and cancerous changes of a polyp revealed during a screening colonoscopy. Initially, the patient's postoperative journey was uneventful with the resumption of regular bowel movements and favorable diet progression. However, he later presented to the emergency department for worsening abdominal pain and distension. Imaging prompted surgical intervention due to perforation and obstruction, resulting in the identification of a carcinoid tumor within a perforated Meckel's diverticulum. This case highlights the intricate challenges of postoperative complications, particularly the unexpected emergence of Meckel's diverticulum pathology following a colectomy. The atypical presentation, featuring a carcinoid tumor within a perforated Meckel's diverticulum, underscores the importance of evaluating abdominal symptoms postoperatively.

Middle Ear Carcinoid Tumour a Diagnostic Dilemma.

Carcinoid tumours are neuroendocrine tumours associated with gastrointestinal tract, lungs and rarely seen in middle ear. A 29 year old male presented with nonspecific symptoms of blocking sensation, reduced hearing and tinnitus in the right ear. Otoscopy showed congestion of right tympanic membrane with bulge in anterior quadrant with no blanching on siegalization. Audiometry showing conductive hearing loss. CT scan showing mildly enhancing mass in the right middle ear cavity. Complete excision was done and Histopathological studied showed features favouring adenoma and immunohistochemistry showed positive staining of Pan Cytokeratin, Synaptophysis and Chromogranin A, with Ki-67 01% suggestive of carcinoid tumour. Patient underwent PET Scaning post operatively to rule out carcinoid tumour elsewhere in the body. Patient is on follow-up every month to look for any recurrence.

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Progressing to Carcinoid Tumor: A Case of Chronic Cough.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), a rare disease previously overlooked, is gradually being recognized as an important precursor state to pulmonary neuroendocrine tumors. The very insidious onset of symptom presentation makes early diagnosis of DIPNECH almost impossible in clinical settings. In this report, we present a case of persistent and worsening cough for over five years with waxing and waning lung nodules of varying sizes which were eventually diagnosed as DIPNECH on biopsy. However, due to the location and the multiplicity of these nodules, surgical resection was not an option in this case. The diagnostic workup including imaging and biopsy, management options, and possible prognosis of DIPNECH are discussed in detail. This report highlights the growing recognition of DIPNECH as a clinical entity to be aware of during the formulation of a differential diagnosis for patients presenting with chronic unrelenting cough and associated lung nodules.

Management Strategies and Outcomes for Small Intestinal Neuroendocrine Tumours with Involvement of the Superior Mesenteric Vessels: A Systematic Review.

Small intestinal neuroendocrine tumours (SI-NETs) are the most common small intestinal tumours. A particularly challenging subset of these tumours is those that involve the superior mesenteric artery or vein for which the role and feasibility of surgery are often questioned. This systematic review aimed to identify and evaluate the management strategies used for these complex SI-NETs. The identified studies showed positive outcomes with surgery and multimodality therapy.

Prevalence, one-year-incidence and predictors of carcinoid heart disease.

BACKGROUND: Carcinoid heart disease (CHD) caused by neuroendocrine tumours (NET) is associated with an increased morbidity and mortality due to valvular dysfunction and right sided heart failure. The present study aimed to assess the prevalence and one-year-incidence of CHD in NET patients. Tumour characteristics, laboratory measurements, and echocardiographic findings were evaluated to identify predictors of CHD manifestation. METHODS: The study was an investigator-initiated, monocentric, prospective trial. Patients with NET without previously diagnosed CHD were included and underwent comprehensive gastroenterological and oncological diagnostics. Echocardiographic examinations were performed at baseline and after one year. RESULTS: Forty-seven NET patients were enrolled into the study, 64% of them showed clinical features of a carcinoid syndrome (CS). Three patients presented with CHD at baseline and three patients developed cardiac involvement during the follow-up period corresponding to a prevalence of 6% at baseline and an incidence of 6.8% within one year. Hydroxyindoleacetic acid (5-HIAA) was identified to predict the occurrence of CHD (OR, 1.004; 95% CI, 1.001-1.006 for increase of 5-HIAA), while chromogranin A (CgA), and Kiel antigen 67 (Ki 67%) had no predictive value. Six patients with CHD at twelve-month follow-up revealed a tendency for larger right heart diameters and increased values of myocardial performance index (MPEI) at baseline compared to NET patients. CONCLUSION: The prevalence at baseline and one-year-incidence of CHD was 6-7%. 5-HIAA was identified as the only marker which predict the development of CHD.

A Case of Gastric Glomus Tumor Misdiagnosed as Carcinoid Tumor.

Glomus tumor is a rare mesenchymal tumor commonly located in the periphery of glomus bodies, such as the subungual regions (e.g., fingernails and toenails). Other locations include the forearm, wrist, or trunk. Even rare is when these tumors are found in the submucosa. In the stomach, it is commonly found at the gastric antrum. Gastric glomus tumors (GGTs) are often found incidentally after a presumption of other gastric tumors is diagnosed, such as gastrointestinal stromal tumors (GISTs) or carcinoid tumors. The variable clinical presentation of GGT and the fact that histology is the only way to confirm the diagnosis is what makes GGT such an elusive tumor. Our case is a patient that presented with weight loss and reflux. Esophagogastroduodenoscopy (EGD) and colonoscopy were done, and the diagnosis of carcinoid tumor was presumed. Preliminary pathology was suggestive of a diagnosis of carcinoid tumor. The patient eventually had a subtotal gastrectomy, and a biopsy with immunohistochemical staining of the specimen was received, finally confirming the diagnosis of a GGT.

Skin manifestations of neuroendocrine neoplasms: review of the literature.

Neuroendocrine neoplasms (NENs) are a heterogeneous group of rare tumours derived from peptidergic neurons and specialized neuroendocrine cells capable of secreting various peptides or amines. These cells may be present in endocrine tissue or diffused in the tissues of the digestive or respiratory system. The article reviews the characteristic features of NENs, with particular emphasis on skin manifestations, such as necrolytic migratory erythema (NME), tongue inflammation, angular cheilitis, venous thrombosis and alopecia in glucagonoma; "flushing", "lion face", pellagra skin symptoms, "scleroderma-like features without Raynaud's phenomenon" in carcinoid tumours. The paper also presents the clinical picture of the neuroendocrine tumour of the skin - Merkel cell carcinoma. The aim of this study was to draw attention to the need for precise and comprehensive diagnosis of the patients, with particular emphasis on skin lesions as a revelator of neuroendocrine tumours. This management allows for the early implementation of appropriate treatment.

Incidental Meckel's Diverticulum With Neuroendocrine Tumor.

Meckel's diverticulum (MD), the most common congenital disease of the small bowel, commonly presents with symptoms of painless rectal bleeding and intestinal obstruction. The treatment of symptomatic MD involves resection of the lesion regardless of patient age; however, the excision of asymptomatic and incidentally identified MDs in adults remain controversial. On one hand, the complications arising from MDs decrease with age, leading to a lower benefit than risk ratio with prophylactic resection. On the other hand, malignancies, such as neuroendocrine tumors, may arise over time from untreated MDs. This can lead to poor prognostic complications, such as liver or lymph node metastases. In this case report, we describe an incidental Meckel's diverticulum discovered during an exploratory laparotomy for acute sigmoid diverticulitis in an adult male. Later biopsy findings discovered the lesion to contain a grade 1 neuroendocrine tumor. Based on our literature review findings, resection of the incidental Meckel's diverticulum was a reasonable approach given the low complication risks of the procedure and the possibility of malignant transformation and progression.

The Treatment of Psychotic Depression in a 64-Year-Old Patient With Carcinoid Syndrome.

Serotonin-producing tumours are a subset of neuroendocrine tumours which, when active, can lead to carcinoid syndrome caused by the secretion of vasoactive substances such as serotonin, histamine, and bradykinins. In addition to the common symptoms of flushing, diarrhoea, abdominal pain, and carcinoid heart disease, carcinoid syndrome is also known to cause psychiatric symptoms of depression as well as anxiety and, very rarely, psychotic symptoms. The treatment of the neuropsychiatric manifestations of the disease is also complicated by the concurrent use of somatostatin analogues, which also affect total circulating serotonin levels, a neurotransmitter implicated in the aforementioned psychiatric disorders. In this case report, we will discuss the management of psychotic depression in a 64-year-old female patient with a metastatic carcinoid tumour and our rationale for the use of vortioxetine in her treatment.

Neuroendocrine neoplasms of the ovary: an analysis of clinicopathological characteristics and prognosis with a focus on histological grading.

OBJECTIVE: Ovarian neuroendocrine neoplasm is a rare and highly heterogeneous neoplasm. This study is aimed to describe its demographic and clinicopathological features and identify its prognostic factors. METHODS: Clinical data of 399 patients diagnosed with ovarian neuroendocrine neoplasms between 2004 and 2016 in the Surveillance, Epidemiology, and End Results (SEER) database were analysed retrospectively. Survival curves were drawn using the Kaplan-Meier method, comparisons among different subgroups were evaluated using log-rank tests, and multivariate Cox regression analyses identified independent prognostic factors. RESULTS: The five-year survival rates of patients with different histological types (carcinoid tumour, neuroendocrine carcinoma and special type of carcinoid tumour) were 25.5%, 96.1% and 75.0%, respectively (P < 0.001). Multivariate Cox analysis revealed that in carcinoid tumours, advanced FIGO stage was the only predictor. Additionally, no significant difference was observed among stages II, III and IV using the log-rank test. In neuroendocrine carcinoma, an advanced FIGO stage and high-grade differentiation were risk factors, while chemotherapy was a protective factor. Among all ovarian neuroendocrine neoplasms with a known histological differentiation status, no significant difference was observed among different histological types; only high-grade differentiation was an independent risk factor, and chemotherapy was a protective factor. CONCLUSIONS: Patients with neuroendocrine carcinomas and carcinoid tumours of an advanced FIGO stage have a poor prognosis. Poor differentiation of neuroendocrine carcinomas indicates a short survival time, and adjuvant chemotherapy appears to be effective. Histological differentiation of ovarian neuroendocrine neoplasms is the most potent prognostic factor comparing to other known factors. Taken together, ovarian neuroendocrine neoplasms might be better classified as low- or high-grade ones rather than the currently used classification based on histological types in the future.

Advanced atypical lung carcinoid tumour successfully treated with carboplatin, etoposide and atezolizumab: A case report.

Atypical carcinoid tumours are relatively rare among lung cancers. Surgery is regarded as standard treatment for localized cases, but there is little established evidence on treatment strategies for advanced cases. Moreover, the efficacy of immune checkpoint inhibitors for advanced carcinoid tumours is unclear. Here, we report a case of a patient with an atypical carcinoid tumour in whom successful disease control was achieved with the use of combined cytotoxic chemotherapy and immunotherapy.

Highly aggressive neuroendocrine neoplasm of the ascending colon: a wolf in sheep's clothing.

Neuroendocrine neoplasms represent a broad group of uncommon tumours, comprising neuroendocrine tumours, mixed neuroendocrine non-endocrine neoplasms and neuroendocrine carcinomas. While most neuroendocrine neoplasms are well differentiated and exhibit indolent disease biology with excellent treatment response, neuroendocrine carcinomas represent a rare subtype with much more aggressive tumour behaviour, minimal response to adjuvant therapy and extremely poor prognosis. Herein, we report the case of a 47-year-old man who presented with a phlegmonous ascending colonic mass and associated calcified ileocolic lymphadenopathy. He underwent emergent right hemicolectomy, which diagnosed a T4aN2b neuroendocrine carcinoma. Within a week the patient displayed rapidly progressive locoregional nodal disease and he succumbed to disease within 5 weeks. We discuss the significance of calcified abdominal lymphadenopathy on computed tomography scans of the abdomen and review the literature surrounding this rare and highly fatal malignancy.

Intramedullary Spinal Cord Metastasis Mimicking Astrocytoma: A Rare Case Report.

Intramedullary spinal cord metastases (ISCMs) are infrequent lesions. Their incidence is estimated to range from 0.9 to 2.1%, found in autopsies of cancer patients. However, as the life expectancy of malignant tumour patients constantly increases, the reported incidences of ISCMs are consequently rising. This report presents a case of the misdiagnosis of an anaplastic astrocytoma type of tumour due to its similarities to small-cell neuroendocrine carcinoma. Therefore, we would like to underline the importance of further investigation that could assist and support the surgeon in the making of the differential diagnosis. We present the clinical case of a 73-year-old woman with a solitary intramedullary spinal cord metastasis as the initial manifestation of a carcinoid type of tumour. The patient was admitted to our department while presenting a rapid onset of paraparesis. Magnetic resonance imaging was performed, which showed an intramedullary mass at the C2-C6 vertebral level with a heterogeneous contrast enhancement. In light of these findings, the patient underwent surgery for a partial tumour resection. The lesion resulted in being a small-cell neuroendocrine type of carcinoma. This peculiar type of tumour presents similar radiological characteristics to the anaplastic astrocytoma type, which is why our diagnostical mismatch occurred. This is the report of a rare case of solitary intramedullary spinal cord metastasis, which is the result of an initial presentation of a lung small-cell neuroendocrine type of carcinoma. We conclude that ISCMs should be regularly considered as a part of the differential diagnosis of intramedullary lesions, especially in the case of a rapid onset and deterioration of neurological symptoms.

Clinicopathological analysis of primary carcinoid tumour of the ovary arising in mature cystic teratomas.

OBJECTIVE: To investigate the clinicopathological features, diagnosis and differential diagnosis of patients with primary ovarian carcinoid tumours arising in mature cystic teratomas. METHODS: This retrospective case series analysed the data from patients with primary ovarian carcinoid tumours arising in mature cystic teratomas. RESULTS: The study enrolled four patients. Histopathological analysis of the tumours identified the following subtypes: insular (n = 1), trabecular (n = 1) and strumal (n = 2). All four primary ovarian carcinoid tumours originated from a mature teratoma. The morphology of the primary ovarian carcinoids was similar to other neuroendocrine tumours. Strumal carcinoids were composed of different proportions of thyroid tissue intimately admixed with carcinoid tumour. Tumour tissue was arranged in insular and/or trabecular patterns. The nucleus of tumour cells displayed exquisite chromatin without obvious mitotic figures. Tumour tissues were positively stained for neuroendocrine markers chromogranin A, synaptophysin and CD56 to varying degrees. Strumal carcinoid tumours were cytokeratin 19 positive and thyroid transcription factor 1 negative. No recurrence or metastasis occurred during follow-up (12-71 months). CONCLUSION: Primary ovarian carcinoid tumours arising in mature cystic teratomas are rare. Diagnosis and differential diagnosis should be confirmed by clinical features, histopathological characteristics and specific immunophenotyping.

Tumor neuroendocrino, mucocele y adenoma tubulo-velloso: tres lesiones infrecuentes en el apéndice cecal / Neuroendocrine tumours, mucocele, and tubulovillous adenoma: three uncommon lesions in the cecal appendix

Introducción: los tumores del apéndice cecal constituyen un grupo heterogéneo de neoplasias con evolución y pronóstico variables; representan una pequeña parte de todas las neoplasias gastrointestinales y de las apendicetomías. Objetivo: realizar la comunicación de la presentación de tumores infrecuentes del apéndice cecal y revisar la literatura con énfasis la importancia del diagnóstico histopa-tológico. Presentación de caso: durante la necropsia del paciente HC: 199584 se realizó el diagnóstico histopatológico de un tumor neuroendocrino y mucocele apendicular y en la pieza quirúrgica del paciente HC: 223360 correspondiente a una apendicectomía se realizó el diagnóstico de adenoma tubulo-velloso, dadas que estas lesiones son poco frecuente se decide su comunicación y la revisión del tema en la literatura en idioma inglés y español a través de las bases de datos MEDLINE/PubMed, Elsevier, SciELO, EBSCO, Hinari y búsqueda avanzada en Google Scholar, encontrándose un total de 29 publicaciones entre 1990 a 2019. Conclusiones: el diagnóstico de tumores del apéndice cecal se realiza en forma incidental en el estudio anatomopatológico después de una apendicectomía por apendicitis aguda, es por ello que siempre se debe realizar el estudio histológico de la pieza quirúrgica, no solo para confirmar o negar el diagnostico que motivo la apendicetomía, sino para descartar la presencia de lesiones neoplásicas benignas o malignas.

Introduction: Tumors of the cecal appendix constitute a heterogeneous group of neoplasms with variable evolution and prognosis; they represent a small part of all gastrointestinal neoplasms and appendectomies. Objective: To communicate the presentation of infrequent tumours of the cecal appendix and to review the literature with emphasis on the importance of histopathological diagnosis. Case presentation: During the necropsy of patient HC: 199584, a histopathological diagnosis of carcinoid tumours and appendicular mucocele was made, and in the surgical specimen of patient HC: 223360 correspondings to an appendectomy, the diagnosis of tubu-lovillous adenoma were made, given that These lesions are rare, it is decided to communicate and review the subject in the literature in English and Spanish through the MEDLINE / PubMed, Elsevier, SciELO, EBSCO, Hinari and Advanced Search in Google Scholar data-bases, finding a total of 29 publications between 1990 to 2019. Conclusions: The diagnosis of tumours of the cecal appendix is made incidentally in the pathological study after an appendectomy for acute appendicitis, which is why the histological study of the surgical piece should always be carried out, not only to confirm or deny the diagnosis. What reason for the appendectomy but to rule out the presence of benign or malignant neoplastic lesions.

Long-term effect of everolimus in recurrent thymic neuroendocrine neoplasia.

PURPOSE: Neuroendocrine neoplasia (NEN) of the thymus is a very rare entity with a poor prognosis. None of the treatments was proofed by studies. Usually, therapy protocols for bronchopulmonary carcinoids are used. So far no data exist on the effect of mammalian target of rapamycin (mTOR) inhibitors. We describe our long-term experience with everolimus and give a thorough review of the therapeutic strategies used so far. PATIENTS AND METHODS: Four patients (mean age 46 years, range 37-55) with progressing thymic NEN (t-NEN) (two well-differentiated atypical carcinoids and two atypical carcinoids with large cell characteristics) were treated with everolimus 10 mg/day after the failure of at least one previous medical therapy. Everolimus was applied after a mean interval of 32.4 months (range 5-56) after the first diagnosis. The follow-up included clinical examination, imaging and chromogranin A testing in 3 or 6 monthly intervals. RESULTS: We observed stable disease for a mean of 20.8 months. Both patients with large cell characteristics t-NEN (Ki-67 of 20%) had rapid progress after 7 and 10 months and had more previous therapies (three and six) than the patients with well-differentiated t-NEN (Ki-67 5% and 10%, progress after 24 and 42 months, one and two previous therapies). No severe side effects occurred. In three of four patients, everolimus led to stable disease for the longest compared to the other nonsurgical therapies used. CONCLUSION: Comparing the sparse data available everolimus is a promising treatment for t-NEN at least in second-line therapy. A low Ki-67 index was associated with a better outcome.

Sarcoidosis and neuroendocrine tumours: case report and literature review.

Rare cases of co-existing sarcoidosis and carcinoid tumour have been previously reported in the literature. Both diseases may have vague and overlapping clinical presentations that can lead to delayed or missed diagnosis. To avoid this diagnostic pitfall, we discuss and compare the clinical presentations of all reported cases in the literature including our case. We also provide hypothesis to explain the relationship between the two diseases.

Neuroendocrine tumour of the ampulla of Vater: A rare neoplasm at an atypical site.

The periampullary neuroendocrine tumour is an infrequently occurring tumour. Its prevalence among gastrointestinal neuroendocrine neoplasms is less than 0.3%, and less than 2% out of periampullary tumours. These neoplasms have relatively poor prognosis. Jaundice and pain in the abdomen are the early and most commonly occurring symptoms with weight loss being a late event. The carcinoid syndrome presents infrequently in periampullary neuroendocrine tumour and happens only if hepatic metastasis occurs. In this scenario, histopathology plays a paramount role in the diagnosis. Specific immunohistochemical staining is used for diagnosis while the treatment options are local excision, endoscopic excision and pancreaticoduodenectomy. Here is a case report of a 42-year-old patient who presented with complaint of obstructive jaundice for one month. Periampullary carcinoid tumour was diagnosed on biopsy, and she underwent Pancreaticoduodenectomy as treatment. Literature shows that there is poor precision of preoperative and intraoperative lymph node metastatic involvement regardless of the size of the tumour. Hence, radical resection must be considered the standard approach.

Efficacy of Lutetium-Peptide Receptor Radionuclide Therapy in Inducing Prolonged Tumour Regression in Small-Bowel Neuroendocrine Tumours: A Case of Favourable Response to Retreatment after Initial Objective Response.

INTRODUCTION: The efficacy of 177Lu-Dotatate was shown in the NETTER-1 trial, an international, open-label, multicentre phase III clinical trial that evaluated the safety and efficacy of 177Lu-Dotatate in patients with well-differentiated, advanced midgut neuroendocrine tumours (NETs) with evidence of disease progression. Recently, retreatment with peptide receptor radionuclide therapy (PRRT) has been proposed as a valid therapeutic option in patients without other effective options who had responded to initial PRRT; however, data on this therapeutic option are still inadequate. CASE REPORT: In this report, we present the case of a patient who achieved a delayed complete radiological response after initial 177Lu-Dotatate treatment and who had a complete tumour response with PRRT retreatment 5 years later. CONCLUSIONS: This case report shows that, although rare, a complete, prolonged tumour response may occur in patients with advanced small-bowel NETs receiving PRRT. Retreatment with PRRT may be a valid option in cases of subsequent disease recurrence.

Desmoplastic mesenteric lesions do not respond radiographically to peptide receptor radionuclide therapy.

177 Lu-Dotatate treatment is indicated for progressive, well-differentiated, small bowel neuroendocrine tumours) NETs. These tumours often metastasise to mesenteric lymph nodes and produce a desmoplastic reaction, consisting of tumour cells interspersed with fibrotic tissue. We hypothesised that, in patients treated with 177 Lu-Dotatate, mesenteric tumours would remain stable even as liver tumour size changes were observed. We retrospectively reviewed the records of all patients treated with 177 Lu-Dotatate between April 2018 and December 2019. Among patients with desmoplastic mesenteric tumours and liver metastases, we evaluated changes in tumour size of mesenteric and liver lesions based on pre- and post-treatment anatomic scans. As a result of the infrequency of objective radiographic response, any reported changes in tumour size were considered significant. Twenty-one patients met the inclusion criteria: nine had evidence of shrinkage of liver lesion(s), one had mild progression of liver lesions, seven had stable hepatic disease and four had a mixed hepatic response. Two of the patients with hepatic tumour shrinkage met the criteria for a partial response via RECIST 1.1 (https://recist.eortc.org). Desmoplastic mesenteric lesions remained unchanged in size, regardless of the changes detected in liver lesions. In conclusion, 177 Lu-Dotatate does not impact desmoplastic mesenteric tumours which are typically associated with midgut NETs. Patients whose disease is predominantly confined to desmoplastic mesenteric lesions are unlikely to respond radiographically to peptide receptor radionuclide therapy. Moreover, the inclusion of desmoplastic mesenteric lesions as target lesions in RECIST measurements tends to increase rates of disease stability vs response or progression.