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Atypical thymic carcinoid is an extremely rare tumor with a poor prognosis. In addition to its known association with multiple endocrine neoplasia type 1, its hallmark characteristics include local invasion and early distant metastasis. In this report, we share our experience treating atypical thymic carcinoid in a patient with Zollinger-Ellison syndrome.

Abstract Background Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant inherited condition affecting multiple endocrine organs, resulting in significant morbidity and decreased life expectancy. Early tumor identification allows for timely patient management, reduces morbidity, and improves disease outcomes. Patients with MEN1 typically present with primary hyperparathyroidism caused by multiple parathyroid tumors, however, thymic and bronchial carcinoid tumors are also less common manifestations. MEN1-related neuroendocrine tumors often show hematogenous metastasis, with the liver being the most common metastatic site. Skeletal metastases from neuroendocrine tumors are extremely rare. As few as 50 case reports were identified in a recently published literature review on skeletal metastases from carcinoid tumors. To our knowledge, studies related to MEN1 have not been previously conducted. Case presentation We present a case of MEN1-related atypical ovarian carcinoid presenting as the first disease manifestation in a 30-year old woman. After two years, another atypical carcinoid was incidentally diagnosed in the contralateral ovary during a caesarean section. Syndromic MEN1 was not diagnosed clinically despite her young age and bilateral involvement. The patient remained disease-free for two years without further adjuvant treatment prior to clinic presentation with complaints of chest discomfort and body pain. Radiologic and pathologic investigations identified multifocal simultaneous neuroendocrine tumors involving the parathyroid, thymus, pancreas, and adrenal glands, in addition to multiple other metastatic sites. The findings ultimately resulted in the patient being diagnosed with MEN1. Conclusions This extremely rare case emphasizes that ovarian carcinoids, especially when bilateral, could be the initial manifestation of MEN1. The significance of this differential diagnosis was highlighted by the subsequent detection of widespread skeletal metastasis resulting from the carcinoid tumors. A low threshold of suspicion, systemic diagnostic work-up, and regular follow-up are of utmost importance to timely diagnosis of MEN1.

We describe a case of a primary carcinoid tumor of the testis in a 35-year-old man as an incidental finding. Testicular ultrasound showed a 1.1 cm hypoechoic/isoechoic mass with some calcification in the left testicle. The pathology examination of the radical orchiectomy demonstrated a pure carcinoid tumor, with the adjacent coarse calcification. Fluorescence in situ hybridization showed 35 % of the tumor cells had one additional chromosome 12p11.21 signal. This case adds to the rare reports in the literature of a primary pure carcinoid tumor of the testis, and provides additional insight into the radiological and pathological correlation of this disease.

Abstract Carcinoid tumors can be a cause for right heart valve disease, also known as Hedinger syndrome or carcinoid heart disease. Proper understanding of the pathophysiology is of the uttermost importance for adequate treatment of these patients, especially during heart surgery.

Introduction: Well-differentiated neuroendocrine pulmonary tumours (NETp) are morphologically classified as typical carcinoid (TC) and atypical carcinoid (AC). There are limited data on systemic treatment for metastatic disease. Objective: Our study evaluated the median progression-free survival of patients with metastatic tumours, comparing TC and AC status for different treatments. Methods: Retrospective series of patients with metastatic NETp treated from 2002 to 2019 in a large cancer centre were analysed. Our primary endpoint was progression-free survival according to morphological classification (TC vs. AC). All patients received at least one treatment modality (e.g., somatostatin analogue [SSA], chemotherapy [ChP], and everolimus [Eve]). Variables were analysed using the chi-square test, median progression-free survival (mPFS) rates (months), with comparisons evaluated by the log-rank test. Results: Twenty-seven patients were included: 44% with TC and 56% with AC. TC patients were on average 58-years-old, 83.3% were female, and 33.3% received more than one treatment. AC patients were on average 61-years-old, 66.7% were female, and 20% received more than one treatment. All patients were treated more frequently with SSA (TC: 75% vs. AC: 80%, p=0.756). Cisplatin and etoposide were the most frequent ChP regimen (TC: 75% vs. AC: 30%, p=0.248). Patients with TC and AC treated with SSA had higher mPFS in months (TC mPFS SSA: 14.5, Eve: 2.50, ChP: 4.0, SSA + Eve: 4.50; AC mPFS SSA: 7.50, Eve: 4.50, ChP: 7.50, SSA + Eve: 7.00). Conclusion: Although the statistical analyses did not show a significant difference between treatment, numerically, more patients with TC or AC experienced tumor control with SSAs, where the mPFS pairs showed a possible tendency to differentiate themselves from the other regimes (Eve and ChP).

Abstract: Pulmonary mucoepidermoid carcinoma (PMEC) is a rare pulmonary neoplasm. Although 18 F-FDG PET/CT has been shown to present with increased metabolic activity in PMEC, literature does not report increased somatostatin receptor expression in these tumors. We present the case of a 15-year-old boy where PMEC mimicked a typical carcinoid of the lung on DOTANOC PET/CT by showing significant uptake on 68 Ga-DOTANOC., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Primary renal carcinoid tumors are exceedingly rare. We report a 37-year-old woman with primary infertility, who was found to have a primary renal carcinoid tumor. She became pregnant and gave birth to a baby after removal of the tumor. This is the first case in the English literature of primary renal carcinoid tumor related with primary infertility.

This case presents a patient with ectopic secretion of adrenocorticotrophic hormone from a carcinoid tumor resulting in severe hypertension, posterior reversible encephalopathy syndrome (PRES), and refractory status epilepticus. Secondary hypertension may be difficult to identify in critically ill patients. Severely uncontrolled hypertension presenting with PRES caused by Cushing syndrome is rare but should be considered. Management requires the correct diagnosis and specific treatment. A missed or delayed diagnosis can lead to serious consequences and disability. [ABSTRACT FROM AUTHOR]

The purpose of this publication is to report a child with choroidal metastasis from a primary lung carcinoid tumor treated with 125-iodine plaque brachytherapy while achieving complete tumor regression. A 15-year-old girl with a history of stage IV lung carcinoid tumor developed painless progressive visual loss in her right eye. Ophthalmological exam documented a unilateral solitary large choroidal mass with complete exudative retinal detachment. Planned palliative treatment with plaque brachytherapy was performed utilizing a total dose of 45 Gy to the tumor apex. No tumor regression was noted after 6 months of follow-up. Ten months after primary therapy, re-treatment with a total dose of 90 Gy to the tumor apex was performed due to tumor progression. Complete regression was achieved after secondary treatment without evidence of recurrence after 18 months of follow-up. In conclusion, high-dose plaque brachytherapy may induce complete tumor regression in select adolescent patients with choroidal metastasis from lung carcinoid tumor. [ABSTRACT FROM AUTHOR]

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Small bowel-origin carcinoid tumor is indolent but may metastasize relentlessly to various sites, including the liver. Over the past 9 years, we have treated a 69-year-old woman who has undergone 5 percutaneous liver ablations, 5 hepatic intra-arterial chemoembolizations, an ovarian cryoablation, and a trans-ventral hernia mesenteric cryoablation. These interventions are all related to her inoperable carcinoid malignancy. After the patient presented with swelling of the abdomen and both lower extremities, computed tomography (CT) angiography was performed, revealing a circumferential hepatic metastatic mass encasing the intrahepatic inferior vena cava (IVC) and extensive third spacing of fluids specific to the IVC distribution below the diaphragm. A venogram of the intrahepatic IVC revealed extrinsic compression causing 95% narrowing of the vessel. A balloon was advanced to the level of the lesion and inflated, increasing the caliber of the vessel. Subsequently, 2 covered aortic stent graft cuffs were deployed in an overlapping fashion within the lumen of the IVC, traversing the area of narrowing. Next, an open-cell aortic dissection stent was placed across both overlapping aortic stents from the renal veins to the hepatic veins. Following this, three 17-gauge cryoablation probes were inserted into the segment 1 intrahepatic lesions encasing the newly stented IVC via an anterior percutaneous approach. Two 10-min freeze cycles were performed with intraoperative CT imaging, demonstrating circumferential coverage of the lesions. Posttreatment venogram revealed patent stent grafts within the intrahepatic IVC, and restoration of vessel patency. No immediate postoperative complications were noted. The patient's abdominal and lower extremity swelling resolved completely within 1 week after procedure. Two-month follow-up CT demonstrated markedly decreased size of the metastatic lesions and no adverse effects. Six- and 9-month PET-CT scans demonstrated maintained patency of the IVC stent. This palliative procedure allowed the patient to maintain good performance status and alleviated her symptoms of IVC syndrome. The radial force generated by the multiple aortic stents will ostensibly maintain the patency of the intrahepatic IVC. Cryoablation of the encasing metastatic lesion was performed with markedly decreased size of the tumor on the 2-month follow-up. [ABSTRACT FROM AUTHOR]

Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors with variable clinical presentation and prognosis. Surgery, when feasible, is the most effective and often curative treatment. However, NENs are frequently locally advanced or already metastatic at diagnosis. Consequently, additional local or systemic therapeutic approaches are required. Immunotherapy, based on chimeric antigen receptor T cells (CAR-T), is showing impressive results in several cancer treatments. The aim of this narrative review is to analyze the available data about the use of CAR-T in NENs, including studies in both preclinical and clinical settings. We performed an extensive search for relevant data sources, comprising full-published articles, abstracts from international meetings, and worldwide registered clinical trials. Preclinical studies performed on both cell lines and animal models indicate a significant therapeutic effect of CAR-T cells in NENs. Ongoing and future clinical trials will clarify the possible role of these drugs in patients with highly aggressive NENs.

Intra thoracic tumors can lead to a variety of para-neoplastic manifestations which is often overlooked. Awareness about the para-neoplastic manifestations and the possible intra thoracic causes for the same will lead to early diagnosis and appropriate management. In this self assessment quiz we have discussed regarding the para-neoplastic manifestations in Carcinoid tumor.

Background: Contrary to the increasing incidence of gastroenteropancreatic neuroendocrine tumors (GEP-NETs), GEP-NETs of the accessory papilla of the duodenum are extremely rare. Furthermore, there have been no recommendations regarding the treatment strategy for GEP-NETs of the accessory papilla of the duodenum. We present a case of GEP-NET of the accessory papilla of the duodenum successfully treated with robotic pancreatoduodenectomy. Case presentation: A case of a 70-year-old complaining of no symptoms was diagnosed with GEP-NET of the accessory papilla of the duodenum. A 8-mm tumor was located at the submucosal layer with a biopsy demonstrating a neuroendocrine tumor grade 1. The patient underwent robotic pancreatoduodenectomy as curative resection for the tumor. The total operative time was 406 min with an estimated blood loss of 150 mL. The histological examination revealed a well-differentiated neuroendocrine tumor with low Ki-67 index (< 1%). In the posterior areas of the pancreas, the lymph node metastases were detected. The patient was followed up for 6 months with no recurrence postoperatively. Conclusions: Considering the potential risks of the lymph node metastases, the standard treatment strategy for GEP-NETs of the accessory papilla of the duodenum should be radical resection with pancreatoduodenectomy. Minimally invasive approach can be the alternative to the conventional open surgery. [ABSTRACT FROM AUTHOR]

• Lung carcinoid tumors associated with DIPNECH is a rare condition. • The rate of atypical carcinoid tumors subtype is significantly higher in patients with DIPNECH compared to others. • Mediastinal lymph-nodes (pN2+) invasion is more frequently associated to resected carcinoid tumors in DIPNECH patients. Diffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) is a rare disease often associated with carcinoid tumors. We aimed at evaluating the impact of DIPNECH on characteristics and prognosis of patients who underwent radical treatment of pulmonary carcinoid tumors. We reviewed all patients operated on for curative-intent resection of carcinoid tumor in our department from 2001 to 2020. Cases exhibiting both pathological and radiological features of DIPNECH, as assessed by respective thoracic expert physicians, were analyzed separately. 172 cases of resected carcinoid tumors were identified, including 25 (14.5 %) harboring pathological criteria of DIPNECH and radiologic features like mosaic attenuation (92.0 %), multiple nodules < 5 mm (76.0 %), and mucoid impactions (32 %). In DIPNECH patients, major pulmonary resections were usually performed (92.0 %) and resected tumors were mostly classified as pT1 (92 %). Mean Ki67 staining was 3.7 ± 5.2 %. The early postoperative period was mostly uneventful (96.0 %) and 5-year survival was 92.9 ± 6.9 %. Compared to non-DIPNECH cases, we found that patients were older (mean 65.6 ± 9.3 versus 54.1 ± 17.9, p = 0.002), more frequently female (84.0 % versus 56.5 %, p = 0.009), and exhibiting diabetes mellitus (45.8 % versus 18.5 %, p < 0.001) or hypertension (45.8 % versus 24.1 %, p = 0.039). The rate of atypical carcinoid tumors was significantly higher in DIPNECH patients (40.0 % versus 19.9 %, p = 0.027), as well as rate of mediastinal lymph-nodes involvement (pN2+) (36.0 % versus 4.1 %, p < 0.001). At multivariate analysis, only DIPNECH pattern and atypical histology were independent factors of pN2 invasion which was the only predictor of poorer prognosis on Log-Rank test. Carcinoid tumors with proven DIPNECH are associated with negative pathological features and may deserve a dedicated perioperative management. [ABSTRACT FROM AUTHOR]

Introduction: Octreotide acetate subcutaneous injection is indicated to treat acromegaly and the symptoms of carcinoid tumors and vasoactive intestinal peptide tumors (VIPomas). This formative human factors study assessed the octreotide acetate pen injector and accompanying instructions for use (IFU) with self-trained participants.Methods: The study enrolled patients with diagnoses of acromegaly, carcinoid tumors, or VIPomas and healthcare practitioners (HCPs) who treat patients with these diagnoses. The IFU provided a stepwise process with illustrations to train participants on using the pen injector. Participants familiarized themselves with the pen injector and the IFU before administering 2 unaided injections into skin-like pads; administering the full dose into the pad was considered a successful injection. The investigators evaluated each injection by performance measures-specific tasks necessary to safely and correctly administer the medication-and subjective measures, which included participant comments, feedback from questions, and suggestions for improvements.Results: The study enrolled 11 participants-8 patients and 3 HCPs. Participants had a success rate of 100% for both injections. Errors included 1 participant priming the pen with the incorrect dose and 2 participants not holding the injector button for 10 s after the injection. Neither error led to a failed injection. To improve the IFU, participants suggested changing the order of wording on the priming step, clarifying illustrations of the plunger, and stronger indications to hold the injector button.Conclusion: The octreotide pen injector and IFU were usable by self-trained participants. Participant errors and suggestions provided a foundation for recommendations to improve the IFU. [ABSTRACT FROM AUTHOR]

Carcinoid tumors (CTs) develop from neuroendocrine cells and are usually located in the gastrointestinal tract or bronchopulmonary system. Cutaneous involment of CT is rare and is often of pulmonary origin. Information about CT skin metastasis is based on case reports. It usually occurs as a multiple nodules after diagnosis of the primary tumour. Treatment options for skin lesions include surgical excision, CO2 laser, opioid analgesics, somatostatin and electrocauterization. We present a case of atypical CT skin metastasis. [ABSTRACT FROM AUTHOR]

This study analyzed the Surveillance, Epidemiology, and End Results (SEER) database concerning the characteristics of primary renal neuroendocrine neoplasms and their relationships with overall survival. This is the largest cohort described to date, with 132 cases. The results indicate that age, sex, and tumor size and stage are associated with poor prognosis. Purpose: Primary renal neuroendocrine neoplasms (NENs) are exceedingly rare. We used the Surveillance, Epidemiology, and End Results (SEER) data to summarize clinicopathologic characteristics, treatment outcomes, and prognostic factors of primary renal NENs. Methods: Data were identified from the SEER database. Clinicopathologic characteristics were compared by the Pearson chi-square or correction test, in which continuous variables were analyzed by t test. Kaplan-Meier analyses and log-rank tests were used to compare the differences in overall survival (OS). Univariable and multivariable regression model analyses of OS were conducted using the Cox proportional hazard model. Also, we used directed acyclic graphs to guide the multivariable regression model and to try to determine the impact of each of surgery, chemotherapy, and radiotherapy on OS. Results: A total of 132 patients were enrolled. There were significant differences in age, grade, tumor size, SEER stage, surgery, and chemotherapy between patients with carcinoid tumors and those with neuroendocrine carcinomas. Patients with disease with carcinoid tumors, younger age, smaller tumor size, and lower SEER exhibited better survival outcomes. Univariable and multivariable regression models analyses indicated that age, sex, tumor size, and SEER stage were independent prognostic factors for OS. Directed acyclic graphs guided the respective inclusion of variables in the multivariable regression model to assess the causal effect of surgery, chemotherapy, and radiotherapy on OS. The results showed that surgery, chemotherapy, and radiotherapy did not improve OS. Conclusion: Primary renal NENs are exceedingly rare and exhibit different biological behavior. Older age, male sex, larger tumor size, and tumors not confined to the renal parenchyma may indicate poor prognosis. Resection of all visible disease remains the reference-standard treatment of choice. Longer-term studies with a larger patient cohort are needed to determine systemic therapeutic options. [ABSTRACT FROM AUTHOR]

Copyright of Acta Médica Peruana is the property of Colegio Medico del Peru and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. 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. 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. 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. • Strumal carcinoid tumor of the ovary is a rare and unique teratoma of the ovary, which consists of thyroid tissue mixed with neuroendocrine neoplasm. • These tumors frequently have no symptoms or are accompanied by vague signs like the diffuse abdomen ache in this instance. • The presence of ovarian stromal tumor cannot be predicted preoperatively based solely on clinical symptoms, ultrasound data, radiographic and macroscopic examination makes distinguishing ovarian stromal tumor from ovarian cancer challenging. • Patients with ovarian stromal carcinoid have an excellent prognosis. [ABSTRACT FROM AUTHOR]