Incidentally Found Rectal Carcinoid Tumor in a 46-Year-Old Female: The Potential for Complications and the Importance of Screening Guidelines.

Carcinoid tumors are rare neuroendocrine tumors that can be found in the gastrointestinal tract as well as other areas throughout the body. The neurosecretory nature of these tumors can have implications for other chronic diseases that patients may have, such as diabetes. Certain treatments that may be implemented for patients who have carcinoid tumors, such as somatostatin analogs and Everolimus, can also alter blood glucose control. This highlights the importance of diagnosing and treating carcinoid tumors as early as possible to avoid complications associated with metastasis and more intense treatment. With more advanced diseases, clinicians should consider the possible effects of carcinoid tumors and their treatments on other chronic conditions as they manage the patient. For gastrointestinal carcinoid tumors, colonoscopy screening guidelines are incredibly important to counsel patients on, as resection can yield a complete cure for carcinoid tumors when they are found at an early stage. Here, we describe the case of an incidentally diagnosed rectal carcinoid tumor in a 46-year-old female patient with a history of type 2 diabetes mellitus and hypertension.

Testicular neuroendocrine tumor in a 32-year-old man: A case report.

Key Clinical Message: A 32-year-old male with painful scrotal swelling who underwent radical orchiectomy and was diagnosed with a testicular neuroendocrine tumor. Determining whether testicular neuroendocrine tumor is primary or metastasis from another origin is crucial. Abstract: Testicular neuroendocrine tumors (TNET) are one of the rarest human neoplasms, with about 132 identified cases until 2015. Testicular neuroendocrine tumors are frequently manifest with painless scrotal swelling or mass. In this study, we present a 32-year-old male with a chief complaint of painful progressive swelling of the right testicle without any history of trauma. All laboratory tests were within the normal range. Ultrasound revealed two hyper-vascular masses in the right testicle. Computed tomography was performed, and patients had no evidence of metastases. The patient underwent right radical orchiectomy, and a histopathological examination diagnosed the specimen with a well-differentiated testicular neuroendocrine tumor. Because of the rarity of TNET, there are many controversial issues in the treatment, especially in cases with metastatic TNET. Determining whether testicular neuroendocrine tumor is primary or metastasis from another origin is crucial. Further studies are required to achieve optimum treatment for TNET.

Incidental Finding of Well-Differentiated Duodenal Neuroendocrine Tumor on Diagnostic Upper Endoscopy.

Neuroendocrine tumors (NETs) are rare and slow-growing. They are often found incidentally, and patients typically present with vague symptoms. This is a case report detailing an 83-year-old female who presents with signs and symptoms consistent with esophageal stricture and was incidentally found to have a duodenal NET. Treatment typically involves surgical removal and carries a good prognosis. With complete surgical resection of localized tumors, the chance of progression or recurrence is low.

Liver transplant for primary biliary tract neuroendocrine tumor in a nine-year-old girl.

BACKGROUND: Neuroendocrine tumors (NETs) are rare epithelial neoplasms that arise most commonly from the gastrointestinal tract. In pediatrics, the most common site of origin is in the appendix, with the liver being the most common site of metastasis. Neuroendocrine tumors arising from the biliary tract are extremely rare. METHODS: We describe a case of a nine-year-old girl who presented with obstructive cholestasis and was found to have multiple liver masses identified on biopsy as well-differentiated neuroendocrine tumor with an unknown primary tumor site. RESULT: The patient underwent extensive investigation to identify a primary tumor site, including endoscopy, endoscopic ultrasound, and capsule endoscopy. The patient ultimately underwent definitive management with liver transplant, and on explant was discovered to have multiple well-differentiated neuroendocrine tumors, WHO Grade 1, with extensive infiltration into the submucosa of bile duct, consistent with primary biliary tract neuroendocrine tumor. CONCLUSION: Identifying the site of the primary tumor in NETs found within the liver can be challenging. To determine if an extrahepatic primary tumor exists, workup should include endoscopy, EUS, and capsule endoscopy. Children with well-differentiated hepatic NETs, with no identifiable primary tumor, and an unresectable tumor, are considered favorable candidates for liver transplantation.

CT-based radiomics for differentiating peripherally located pulmonary sclerosing pneumocytoma from carcinoid.

BACKGROUND: Pulmonary sclerosing pneumocytoma (PSP) and pulmonary carcinoid (PC) are difficult to distinguish based on conventional imaging examinations. In recent years, radiomics has been used to discriminate benign from malignant pulmonary lesions. However, the value of radiomics based on computed tomography (CT) images to differentiate PSP from PC has not been well explored. PURPOSE: We aimed to investigate the feasibility of radiomics in the differentiation between PSP and PC. METHODS: Fifty-three PSP and fifty-five PC were retrospectively enrolled and then were randomly divided into the training and test sets. Univariate and multivariable logistic analyses were carried to select clinical predictor related to differential diagnosis of PSP and PC. A total of 1316 radiomics features were extracted from the unenhanced CT (UECT) and contrast-enhanced CT (CECT) images, respectively. The minimum redundancy maximum relevance and the least absolute shrinkage and selection operator were used to select the most significant radiomics features to construct radiomics models. The clinical predictor and radiomics features were integrated to develop combined models. Two senior radiologists independently categorized each patient into PSP or PC group based on traditional CT method. The performances of clinical, radiomics, and combined models in differentiating PSP from PC were investigated by the receiver operating characteristic (ROC) curve. The diagnostic performance was also compared between the combined models and radiologists. RESULTS: In regard to differentiating PSP from PC, the area under the curves (AUCs) of the clinical, radiomics, and combined models were 0.87, 0.96, and 0.99 in the training set UECT, and were 0.87, 0.97, and 0.98 in the training set CECT, respectively. The AUCs of the clinical, radiomics, and combined models were 0.84, 0.92, and 0.97 in the test set UECT, and were 0.84, 0.93, and 0.98 in the test set CECT, respectively. In regard to the differentiation between PSP and PC, the combined model was comparable to the radiomics model, but outperformed the clinical model and the two radiologists, whether in the test set UECT or CECT. CONCLUSIONS: Radiomics approaches show promise in distinguishing between PSP and PC. Moreover, the integration of clinical predictor (gender) has the potential to enhance the diagnostic performance even further.

Diagnostic and therapeutic challenges of a rare large ovarian strumal carcinoid in pregnancy, about a case report.

INTRODUCTION: Ovarian strumal carcinoid is a rare type of germ cell tumor. It usually affects perimenopausal and postmenopausal women. Very few cases of stromal carcinoid have been reported in the literature in women of childbearing age, particularly during pregnancy. The clinical presentation of the tumor, and in particular its non-specific clinical and radiological appearance and rarity, explain the difficulties in diagnosis and management. PRESENTATION OF CASE: Herein, we describe a rare case of a 36-year-old patient who was followed-up in our outpatient clinic for organic cyst of the ovary. The ultrasound revealed a multilocular regular cystic mass with a modestly thickened wall and fine septations. The MRI indicated a right ovarian cyst with solid tissue. The levels of tumor markers were normal. The patient was lost to follow-up and did not return until six months later. She was admitted in our Department with acute ovarian torsion and underwent emergency surgery at 17 weeks' gestation. A laparoscopic cystectomy of the right ovary was provisionally performed. Pathology revealed an ovarian strumal carcinoid tumor. DISCUSSION: Patients with ovarian stromal carcinoid have an excellent prognosis. Ovarian strumal carcinoid 's primary therapy method is operation. The majority of original ovarian carcinoid tumors progress slowly, and practically all thyroid carcinoid tumors are clinical stage I with a positive prognosis. CONCLUSION: In the absence of standardized treatment, the association of carcinoid strumal tumor with pregnancy, underlines the need for early diagnosis and appropriate multidisciplinary management, taking into account both the maternal and fetal prognosis.

Making progress against rare cancers: A case study on neuroendocrine tumors.

In April 2023, the National Cancer Institute offered a roadmap for cancer research to achieve Cancer Moonshot goals. To reach these goals requires making progress for all cancers, not just those that are most common. Achieving progress against rare cancers, as well as common cancers, requires involvement of large clinical research networks. In 2020, the Patient-Centered Outcomes Research Institute (PCORI) launched an initiative on Conducting Rare Disease Research using PCORnet, the National Patient-Centered Clinical Research Network. The purpose of this commentary is to introduce the broader community of cancer researchers to the PCORnet NET-PRO study (comparing the effects of different treatment approaches for neuroendocrine tumors on patient-reported outcomes) thereby demonstrating how researchers can use the PCORnet infrastructure to conduct large-scale patient-centered studies of rare cancers.

Resistant gastroenteropancreatic neuroendocrine tumors: a definition and guideline to medical and surgical management.

Gastroenteropancreatic neuroendocrine tumors (NETs), also historically known as carcinoids, are tumors derived of hormone-secreting enteroendocrine cells. Carcinoids may be found in the esophagus, stomach, small intestine, appendix, colon, rectum, or pancreas. The biologic behavior of carcinoids differs based on their location, with gastric and appendiceal NETs among the least aggressive and small intestinal and pancreatic NETs among the most aggressive. Ultimately, however, biologic behavior is most heavily influenced by tumor grade. The incidence of NETs has increased by 6.4 times over the past 40 years. Surgery remains the mainstay for management of most carcinoids. Medical management, however, is a useful adjunct and/or definitive therapy in patients with symptomatic functional carcinoids, in patients with unresectable or incompletely resected carcinoids, in some cases of recurrent carcinoid, and in postoperative patients to prevent recurrence. Functional tumors with persistent symptoms or progressive metastatic carcinoids despite therapy are called "resistant" tumors. In patients with unresectable disease and/or carcinoid syndrome, an array of medical therapies is available, mainly including somatostatin analogues, molecular-targeted therapy, and peptide receptor radionuclide therapy. Active research is ongoing to identify additional targeted therapies for patients with resistant carcinoids.

A Case Report of Neuroendocrine Tumor in Presacral Region: How Can It Be Managed? Laparoscopy versus Laparotomy.

Presacral or retrorectal tumors are rare, usually asymptomatic, and diagnosed accidentally during physical examination or imaging. Symptomatic tumors may present with perianal pain, bowel dysfunction, and urinary symptoms due to the mass compression or invasion of the surrounding tissues and organs. Surgical resection is the first choice for treating presacral tumors. Clinicians should choose surgical procedures based on the location and size of the tumors. We presented a 43-year-old woman who suffered from pelvic pain and primary infertility from two years ago. A large mass between the posterior vaginal wall and the rectum was found on recto-vaginal examination. Magnetic resonance imaging (MRI) revealed a large 120×115 mm benign multiloculated cystic mass. Eventually, the mass was removed through laparoscopic surgery. The pathology report indicated a carcinoid tumor (grade I) with no lymphovascular invasion. Thus, presacral tumors are resectable through laparoscopy with lower complications than open surgery.

Incidental Carcinoid Tumor of the Appendix After Appendectomy During Pregnancy.

Neuroendocrine tumors comprise a range of neoplasms with varying spectra of origin, biological activity, clinical features, and histological appearance. In this case report, we present a pregnant 33-year-old female who was brought to the emergency department (ED) complaining of acute right iliac fossa pain accompanied by diarrhea and vomiting. Initial management showed no improvement. Lab results, clinical history, and physical exam were suggestive of appendicitis, so an exploratory minimally invasive laparoscopic exam was performed. The histopathological analysis of the excised appendix confirmed the diagnosis of acute appendicitis and periappendicitis. Incidentally, a 0.6 cm neuroendocrine tumor (carcinoid tumor) was identified on the wall of the appendiceal tip. The tumor extended at multiple points into the subserosal fat, and the serous surface and the resection margin were negative for the tumor. After seven days of the initial procedure, the patient presented with abdominal pain and a fever. An abdominal ultrasound was performed, revealing the presence of free fluid. A second exploratory laparoscopy revealed adhesions between the fallopian tubes and cecum, as well as a collection of purulent fluid. The management consisted of adhesiolysis, cavity lavage, and drainage, along with antibiotic therapy, pain management, and close monitoring of the mother's and fetus's status. The patient had a successful recovery and was discharged home a week after surgery. She gave birth to a full-term, healthy baby and remains free of tumor relapse. This case highlights the importance of obtaining histopathological interpretation of any extracted tissue during surgery. Guidelines regarding the management of carcinoids during pregnancy are not available, and when considering surgical intervention, an open or laparoscopic approach must be carefully evaluated.

Lobar Mucus Plugging Reflecting Central Bronchial Tumoral Obstruction: A Case Report.

Early diagnosis of bronchopulmonary carcinoid tumors is crucial as the surgical excision is the main treatment and determines the prognosis. We present the case of a 66-year-old heavy-smoker man who had started to complain about a cough a few months ago. We diagnosed him with an endobronchial mass on a chest computed tomography scan and lobar bronchoceles resulting from mucus plugging distal to the tumor obstruction. These findings were retrospectively visible on the previous chest radiograph that had initially been interpreted as non-contributary.

Incidence and Management of Appendiceal Neuroendocrine Tumors in Pediatric Population: A Bicentric Experience with 6285 Appendectomies.

BACKGROUND: Neuroendocrine tumors (NETs) are rare tumors that arise from neuroendocrine cells and are the most common tumors of the appendix. NETs of the appendix usually cause no symptoms and often go unnoticed until they cause acute appendicitis or are discovered during an accidental appendectomy. As the trend towards the conservative treatment of acute appendicitis increases in the pediatric population, the question arises as to whether the majority of NETs go undetected and are only discovered at an advanced stage. The purpose of the proposed study is to review the incidence and outcomes of treatment for NETs of the appendix in children and include the data presented in the data pool for further review. METHODS: From 1 January 2009 to 1 November 2023, a total of 6285 appendectomies were performed in two large pediatric centers in Croatia. After a retrospective review of the case records and histopathologic findings, a total of 31 children (0.49%) were diagnosed with NET of the appendix and included in the further analysis. The primary outcome of this study was the incidence and treatment outcome of pediatric patients diagnosed with NET of the appendix. Secondary outcomes included the patients' demographic, clinical, and laboratory data and the histopathologic characteristics of tumor species. RESULTS: The overall incidence of NETs of the appendix was stable over the study years, with minor fluctuations. The median age of patients was 14 (interquartile range-IQR: 12, 16) years, with a female predominance (64.5%). The majority of patients (96.8%) presented with acute abdominal pain and underwent appendectomy because acute appendicitis was suspected. Acute appendicitis was confirmed by histopathology in 18 (58%) cases. NETs of the appendix were not detected preoperatively in any of the patients. Among patients with confirmed acute appendicitis, most (n = 14; 77.8%) were found to have non-perforated acute appendicitis. In most children, the tumor was located at the tip of the appendix (n = 18; 58.1%), and the majority of tumors had a diameter of less than 1 cm (n = 21, 67.7%). The mitotic count (n = 25, 80.6%) and Ki-67 proliferation index (n = 23, 74.2%) were low in most patients, so most tumors were classified as NET G1 (n = 25, 80.6%), while NET G2 and NET G3 were found in four (12.9%) and two (6.5%) patients, respectively. All children were treated with appendectomy only. The median follow-up time was 54 (IQR: 24, 95) months. CONCLUSIONS: The incidence of appendiceal NET among pediatric patients is very low. NET occurs most frequently in adolescents, with a female predominance. Most tumors are less than 1 cm in diameter, located at the tip, and associated with non-perforated appendicitis. Appendectomy is the treatment of choice, and major surgery was not necessary in our cohort.

Iridociliary Body Metastasis of Atypical Carcinoid: Case Management with Intravitreal Anti-Vascular Endothelial Growth Factor Injections.

We report a rare case involving a 52-year-old female diagnosed with an atypical bronchial carcinoid tumor with metastases to the mediastinum, hilar lymph nodes, breast, and pancreas. In additional, the patient had metastases to the iris and ciliary body, resulting in progressive vision loss in her left eye. Treatment was successful by intravitreal injections of anti-vascular endothelial growth factor.

Cracking the Code of a Rare Colonic Mesentery Gastrointestinal Neuroendocrine Tumor Disguised as Chronic Gastroenteritis: The Critical Art of Timely Detection.

Neuroendocrine tumors (NETs) typically present in the setting of metastasis from other solid organs and are considered late manifestations of the disease. Therefore, primary tumors are extremely rare. NETs of the colonic mesentery occur more than 70% of the time in the appendix, small intestine, and rectum. Here, we describe the case of a patient who presented with multiple episodes of diarrhea and abdominal pain, which was waxing and waning in occurrence, with CT findings of a rare primary NET.

Spindle cell thymoma and its histological mimickers.

Spindle cell thymomas are the most common spindle cell neoplasms of the anterior mediastinum. These tumors belong to the group of thymic epithelial neoplasms and are known for their wide histomorphologic spectrum. This histological heterogeneity is the reason why unequivocal diagnosis can be challenging, especially when dealing with small biopsy material. Conversely, less conventional patterns of the tumor may also pose significant diagnostic problems in resected material and the differential diagnosis often includes other spindle cell neoplasms that are known to arise in the mediastinal cavity. These can be of variable origin and may share overlapping pathological features with spindle cell thymoma. Since spindle cell thymomas are tumors that primarily affect the adult population and predominantly arise from the thymic gland in the anterior mediastinum, this review will focus on the differential diagnosis with other spindle cell neoplasms that share similar demographic characteristics and, for the most part, originate from the anterior mediastinal compartment. These include other epithelial spindle cell tumors of thymic origin (sarcomatoid thymic carcinoma and spindle cell carcinoid tumor), mesenchymal neoplasms [solitary fibrous tumor (SFT), synovial sarcoma, and dedifferentiated liposarcoma] and various other tumors with spindle cell morphology, that may occasionally involve the anterior mediastinum. The clinical, pathological, immunohistochemical and molecular hallmarks of these lesions will be discussed and useful tips for the differential diagnosis with spindle cell thymoma will be provided.

A Case of Familial Appendiceal Neuroendocrine Tumor.

The risk of developing appendiceal neuroendocrine tumor (aNET) may be attributed to multiple factors. A familial clustering is found in less than 1% of the cases. We report the case of a 25-year-old woman who initially presented with a clinical presentation of acute appendicitis and was subsequently diagnosed with aNET by histopathological examination after an emergency appendectomy. While revealing the result to the patient, she was found to have a positive family history of appendiceal carcinoid tumor. Although rare and only found in 1% of the cases, aNET found in family history should raise the suspicion of neuroendocrine tumors in other family members.

Middle Ear Neuroendocrine Tumor Mimicking As Chronic Otitis Media.

Neuroendocrine neoplasms (NEN) of the head and neck are a rare and diverse group of tumors. Here, we report a case of a 40-year-old man presenting with symptoms resembling chronic left otitis media, including left ear otorrhea, otalgia, and reduced hearing. Otoscopic examination revealed a whitish mass located behind the tympanic membrane. The patient underwent examination under anesthesia and left cortical mastoidectomy, and a histopathological examination of the middle ear biopsy indicated the presence of an epithelial tumor with neuroendocrine differentiation, suggestive of middle ear adenoma. A staging CT scan performed three months after the mastoidectomy showed a hypodensity in the middle ear cavity, with no significant bony erosion, which could potentially indicate a residual or recurrent tumor. Consequently, a radical mastoidectomy was performed. The histopathological examination confirmed the presence of middle ear adenoma with neuroendocrine differentiation.

Carcinoid Tumor Presenting as Hemoptysis and Elevated Diaphragm.

A bronchial carcinoid tumor is a rare pulmonary neuroendocrine tumor. This report describes a case where a patient experienced multiple episodes of hemoptysis and dyspnea on exertion over the course of five months. An initial chest X-ray showed an elevated right hemidiaphragm with atelectasis, and a follow-up chest computed tomography (CT) scan was ordered to further assess this finding. The CT revealed a tumor occluding 90% of the right main stem bronchus lumen. A bronchoscopy with biopsy was then performed, confirming the diagnosis of a pulmonary carcinoid tumor. The patient underwent surgical resection of the tumor, a right upper lobe sleeve lobectomy, and a mediastinal lymph node dissection, resulting in full eradication of the tumor. This case highlights the need for physicians to maintain a broad differential when evaluating a patient with hemoptysis and an elevated diaphragm.

DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare, but increasingly recognized entity that primarily affects middle-aged and elderly women. It is characterized by abnormal proliferation of pulmonary neuroendocrine cells (PNECs) and is considered a preinvasive lesion for carcinoid tumorlets/tumors. Sometimes, DIPNECH is accompanied by constrictive bronchiolitis which usually manifests as chronic cough and/or dyspnea, along with airflow limitation on spirometry. The telltale imaging sign of DIPNECH is the presence of multiple noncalcified pulmonary nodules and mosaic attenuation on CT. However, these clinico-radiologic features of DIPNECH are characteristic but nonspecific; thus, histopathologic confirmation is usually necessary. DIPNECH has an indolent course and only rarely leads to respiratory failure or death; progression to overt neuroendocrine tumor (carcinoid) of the lung occurs in a minority of patients. Of available therapies, somatostatin analogs and mechanistic target of rapamycin inhibitors are the most promising. In this review, we provide an update regarding the diagnosis and management of DIPNECH and describe critical gaps in our understanding of this entity, including the central terms 'diffuse' and 'idiopathic.' We also summarize the inconsistencies in definitions employed by recent studies and discuss the pitfalls of the DIPNECH definitions proposed by the World Health Organization in 2021. In this context, we propose an objective and reproducible radio-pathologic case definition intended for implementation in the research realm and seeks to enhance homogeneity across cohorts. Furthermore, we discuss aspects of PNECs biology which suggest that PNEC hyperplasia may contribute to the pathogenesis of phenotypes of lung disease aside from constrictive bronchiolitis and carcinoid tumorlets/tumors. Finally, we steer attention to some of the most pressing and impactful research questions awaiting to be unraveled.

Improving segmentation and detection of lesions in CT scans using intensity distribution supervision.

We propose a method to incorporate the intensity information of a target lesion on CT scans in training segmentation and detection networks. We first build an intensity-based lesion probability (ILP) function from an intensity histogram of the target lesion. It is used to compute the probability of being the lesion for each voxel based on its intensity. Finally, the computed ILP map of each input CT scan is provided as additional supervision for network training, which aims to inform the network about possible lesion locations in terms of intensity values at no additional labeling cost. The method was applied to improve the segmentation of three different lesion types, namely, small bowel carcinoid tumor, kidney tumor, and lung nodule. The effectiveness of the proposed method on a detection task was also investigated. We observed improvements of 41.3% → 47.8%, 74.2% → 76.0%, and 26.4% → 32.7% in segmenting small bowel carcinoid tumor, kidney tumor, and lung nodule, respectively, in terms of per case Dice scores. An improvement of 64.6% → 75.5% was achieved in detecting kidney tumors in terms of average precision. The results of different usages of the ILP map and the effect of varied amount of training data are also presented.