Rare Tumor Research Articles

Characterization and Management of Epithelioid Hemangioma on the Lip Mucosa: A Case Report

Epithelioid Hemangioma (EH) is a rare benign vascular tumor characterized by well-formed blood vessels and epithelioid endothelial cells. Known as Angiolymphoid Hyperplasia with Eosinophilia (ALHE), the World Health Organization does not recommend the use of this term since 2020. Despite its common occurrence in subcutaneous soft tissues of head and neck, cases involving oral mucosa and other extra cutaneous areas are infrequent. This article presents a case of EH in an unusual location and discusses the differential diagnosis with other entities, including Kimura´s Disease (KD). We report the clinical case of a 26 year old man with no significant medical history who presented with a painless increase in volume on the right upper lip, subsequently diagnosed as EH through biopsy and histopathology. The lesion exhibited typical clinical features and a complex histological pattern including epithelioid endothelial cells and an inflammatory infiltrate rich in eosinophils. Diagnosis of EH is challenging due to morphological variability and the need to differentiate it from similar conditions such as KD. Differential diagnosis is crucial, especially in atypical locations. This case shows the importance of histopathology and complementary examinations in diagnosis and the need to consider KD in the differential diagnosis due to its therapeutic and prognostic implications. Therefore, it is concluded that EH, although benign, requires a rigorous diagnostic approach to differentiate it from similar diseases and manage it appropriately. Continuous control is essential due to the potential for recurrence. This case shows the importance of careful differential diagnosis, especially in atypical locations, to optimize management and improve patient outcomes.

Abstract C069: Unraveling the complexity of malignant peritoneal mesothelioma: A comprehensive case study of diagnosis, clinical features, and therapeutic challenges in a 75-year-old male

Abstract Introduction: Malignant Peritoneal Mesothelioma (MPM) is a rare and aggressive intra-abdominal neoplasm originating from mesothelial cells. Although asbestos exposure is a known risk factor, MPM's molecular pathogenesis differs from its pleural counterpart. Case Presentation: A 75-year-old male, with a past medical history of recently diagnosed mesothelioma, presented to the emergency department for a two-week progression of abdominal distension, early satiety, and dyspnea aggravated by activity. One year prior, he initiated medical evaluation for fluid retention and abdominal distension. A recent omental biopsy confirmed the diagnosis of mesothelioma, prompting the initiation of chemotherapy every three weeks. Despite paracentesis performed monthly, the patient experienced worsening shortness of breath and early satiety post-chemotherapy. Discussion: Malignant Peritoneal Mesothelioma poses a diagnostic challenge due to the absence of specific signs or symptoms. Common manifestations include abdominal distension, pain, early satiety, weight loss, dyspnea, chest pain, and fatigue. Rare symptoms encompass new-onset hernia, fever of unknown origin, night sweats, or incidental findings during physical examinations or laparoscopy. Delays in presentation and diagnosis contribute to the advanced stage at diagnosis, with most cases having already spread throughout the abdominal cavity. Three distinct presentations of MPM include the Dry Painful Type, characterized by abdominal pain with minimal ascites; the Wet Type, presenting with abdominal distension and ascites; and the Mixed Type, combining abdominal pain and distension. Paraneoplastic phenomena, such as fever, thrombocytosis, malignancy-related thrombosis, hypoglycemia, hypoalbuminemia, paraneoplastic hepatopathy, and rarely, Coombs-positive Hemolytic anemia, further complicate the clinical picture. Surgical gross and microscopic descriptions of liver biopsies indicated chronic hepatitis with mild portal inflammation, grade 1, and no fibrosis, stage 0. Notably, the omental biopsy confirmed Malignant Mesothelioma of the epithelioid type. Immunohistochemical stains supported the diagnosis, revealing positivity for CK AE1/AE3, calretinin, WT1, CK7 (focally), and GATA3 (focally), while testing negative for BAP1, CDX2, chromogranin, CK20, hepatocyte antigen, PAX8, PSA, synaptophysin, and TTF1. Due to the rarity of MPM, no standardized treatment protocols exist. Cytoreductive surgery (CRS) and hyperthermic intraoperative peritoneal perfusion with chemotherapy (HIPEC) are recommended for selected patients without extraperitoneal disease spread, possessing a good performance status and potential for complete surgical cytoreduction. Systemic chemotherapy, particularly pemetrexed-based regimens, is advocated for patients ineligible for CRS/HIPEC. Conclusion: MPM's aggressive nature poses diagnostic and therapeutic challenges. The scarcity of standardized guidelines emphasizes the importance of ongoing research to enhance understanding, improve diagnostics, and develop targeted therapies Citation Format: Aliya M Khan, Mehmet Hepgur, Aryama Sharma. Unraveling the complexity of malignant peritoneal mesothelioma: A comprehensive case study of diagnosis, clinical features, and therapeutic challenges in a 75-year-old male [abstract]. In: Proceedings of the 17th AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2024 Sep 21-24; Los Angeles, CA. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2024;33(9 Suppl):Abstract nr C069.

Disseminated pleomorphic rhabdomyosarcoma in a horse

An 8-y-old National Show Horse mare was presented for evaluation of pneumonia and laminitis. Harsh bronchovesicular sounds were auscultated throughout both lung fields, and the mare had signs of moderately painful laminitis. Thoracic ultrasonography revealed lung consolidation throughout the dorsal aspect of both lungs, and radiography revealed an extensive diffuse-to-patchy bronchointerstitial lung pattern. The mare’s clinical condition rapidly deteriorated, and euthanasia was elected. On postmortem examination, the lungs, omentum, spleen, liver, adrenal glands, kidneys, and femur contained 0.5–2.5-cm, firm, tan nodules. Histologically, the lungs, spleen, liver, kidneys, adrenal glands, omentum, left eye, and femur were infiltrated by bundles and nests of pleomorphic polygonal-to-spindloid cells intermixed with frequent multinucleate cells. Lymphatic vessels in the affected tissues were frequently distended with tumor emboli. Neoplastic cells were diffusely positive for vimentin, desmin, sarcomeric actin, myoblastic differentiation protein 1, and myogenin, supportive of the diagnosis of rhabdomyosarcoma (RMS), which is a rare neoplasm in horses. Cross-striations were not evident with H&E or phosphotungstic acid–hematoxylin stains. Markedly pleomorphic neoplastic cells, multinucleate cells, and lack of cross-striations suggested the subclassification of pleomorphic RMS.

Management and follow-up strategies for patients with head and neck paraganglioma

Abstract Objective Head-neck paragangliomas (HNPGLs) are rare tumors with approximately half arising due to germline pathogenic variants (PVs) in succinate dehydrogenase genes (SDHx). Patients with HNPGL have heterogenous propensity to recur and metastasize. Thus, we aim to assess prevalence and predictors of recurrent (RD) and/or metastatic disease in patients with and without SDHx-related HNPGLs. Design and methods This cross-sectional study used retrospective data of 214 patients enrolled in six referral centers. Data included sex, age, primary tumor treatment, location, and size, biochemical phenotype, germline PVs, presence of RD (locoregional or new tumor), and/or metastasis. Results Patients with and without SDHx-related HNPGLs showed 74% and 40% prevalence of RD, respectively. Patients without SDHx-related HNPGLs presented with recurrent tumors only in head-neck regions. The only independent predictor for RD in the entire cohort was presence of SDHx PVs. Metastatic prevalence reached 9-13%. For patients with SDHx-related HNPGLs, large tumor size (>2.3cm, OR:50.0, CI:2.6-977.6), young age at initial diagnosis (<42years, OR:27.3, CI:1.8-407.2), and presence of SDHB PV (OR:15.6; CI:1.5-164.8) were independent predictors of metastasis. For patients without SDHx-related HNPGLs, only carotid-body location was an independent predictor of metastasis (OR:18.9, CI:2.0-182.5). Conclusions Patients without SDHx-related HNPGLs require long-term follow-up due to high prevalence of RD with imaging largely restricted to head-neck regions. As carotid-body HNPGLs have the highest metastatic risk among sporadic tumors, radical treatment with frequent follow-up is suggested until population-based data are available. Importantly, patients with SDHx-related HNPGLs might benefit from early radical treatment when tumors are still small to reduce metastatic risk.

Site-discordant expression of myeloid cell nuclear differentiation antigen in blastic plasmacytoid dendritic cell neoplasm

Abstract Objectives Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic neoplasm that can show clinical, morphologic, and immunophenotypic overlap with acute myeloid leukemia. Myeloid cell nuclear differentiation antigen (MNDA) is a nuclear protein expressed by myelomonocytic cells previously reported to be reliably absent in BPDCN and proposed as a useful adjunct for the distinction of BPDCN and acute myeloid leukemia. We encountered a case of BPDCN that showed strong nuclear expression of MNDA in bone marrow and breast samples and weak to absent expression in skin samples, prompting us to reevaluate the expression of MNDA in BPDCN. Methods We collected all available BPDCN cases from the Stanford University archives collected in the past 10 years and subjected them to MNDA immunohistochemistry. In select cases, molecular profiling by next-generation sequencing was performed. Results We found 4 cases (of 8 total examined [50%]) with convincing site-discordant MNDA expression. This expression was seen in 3 of 6 (50%) bone marrow samples, 1 of 2 (50%) breast soft tissue samples, and 3 of 14 (up to 21%) skin samples and was not obviously predicted by age, sex, history of myeloid neoplasm, or treatment history. In 2 cases, MNDA was strongly expressed in 2 distinct sites (breast/bone marrow, skin/bone marrow) and negative in subsequent samples. Conclusions Our findings suggest that MNDA expression in BPDCN is anatomic site dependent and transient, with noncutaneous infiltrates showing more frequent expression than cutaneous infiltrates. These results caution against the use of MNDA to exclude BPDCN when considering the differential diagnosis of a blastic extramedullary infiltrate.

Liposarcoma del cordón espermático

Introduction: liposarcoma of the spermatic cord (LSC) is a rare condition and accounts for about 3 to 7% of all paratesticular sarcomas. A case is presented in an 80-year-old patient who consulted for a left inguinoscrotal painless swelling, that had been present for several years. Physical examination revealed a soft left hemiscrotal tumor that appeared not to be adherent to the testicle. Ultrasonography showed a solid, heterogeneous hyperschoic paratesticular mass with central calcifications and peripheral hypervascularity. During surgery, a diffuse lipomatous tumor was found in the spermatic cord on the superior pole of the left testicle, separated from it. Anatomopathological examination revealed a well-differentiated liposarcoma of the spermatic cord. Discussion: this neoplasm features a slow-growing palpable paratesticular mass. This site of occurrence is rare and represents 7 to 10% of all scrotal tumors. Imaging differentiation between LSC and benign lesions is sometimes challenging. Surgical treatment should include radical orchiectomy wide local excision, and spermatic cord and inguinal ring surrounding soft tissue resection. Conclusion: spermatic cord liposarcoma is a rare and unusual mesenchymal tumor and should be considered among the differential diagnoses of scrotal tumors.

The Significance of the Morphological Appearance of Peritoneal Lesions on Imaging in Patients With Peritoneal Malignancies-A Report From Phase 1 of the PRECINCT Study.

This is a report from Phase 1 of the prospective, observational, PRECINCT (Pattern of peritoneal dissemination and REsponse to systemic Chemotherapy IN Common and uncommon peritoneal Tumours) study, in which we studied the incidence of disease at pathological evaluation in different morphological appearances of peritoneal malignancies (PM) on imaging. Radiological findings were captured in a specific format that included a description of the morphological appearance of PM and a correlation performed with pathological findings. In 630 patients enroled at seven centres (September 2022-December 2023), 24 morphological terms were used. Among prespecified terms (N = 8 used in 6350 [92.2%] regions), scalloping was pathologically positive in 93.5%, confluent disease in 78.8%, tumour nodules in 69.6%, thickening in 66.1%, infiltration in 56.3%. Among unspecified appearances (N = 16) for 540 (7.8%) regions, 'enhancement' was positive in 41.5%, micronodules in 65.3% and nodularity in 60.2%. Hierarchal clustering placed gastric cancer and rare tumours together and colorectal cancer, ovarian cancer and peritoneal mesothelioma in one cluster. The incidence of disease at pathological evaluation for most morphological appearances was high (> 50%). Morphological description should be provided in routine radiology reports. A set of standardized terms with their description should be developed by a consensus among experienced radiologists.

Case Report: Adrenal schwannoma associated with ganglioneuroma

BackgroundAn adrenal collision tumor (ACT) denotes the presence of distinct tumors with diverse behavioral, genetic, and histological features independently co-existing within the adrenal tissue without intermingling, and occurrences of such cases are infrequent. The concurrent occurrence of adrenal schwannoma and adrenal ganglioneuroma is exceedingly rare, and the diagnosis of these ACTs has been notably challenging due to their atypical clinical manifestations and imaging characteristics.Case summaryA 37-year-old man presented to the hospital 3 weeks after a computed tomography (CT) examination that revealed a left adrenal mass. Physical examination findings were unremarkable. Both CT and magnetic resonance imaging scans indicated the presence of a left adrenal mass. Plasma cortisol, adrenocorticotropic hormone, and renin–angiotensin–aldosterone system tests yielded normal results. Preoperative imaging confirmed the diagnosis of left adrenal pheochromocytoma. After thorough surgical preparation, a laparoscopic partial left adrenalectomy was performed. Subsequent postoperative pathological analysis identified adrenal schwannoma in conjunction with adrenal ganglioneuroma. The patient recovered well and was discharged on postoperative day 4. A routine urology clinic visit was included in his postoperative care plan. During follow-up assessments, CT scans of the left adrenal gland revealed no abnormalities.ConclusionAdrenal schwannoma combined with ganglioneuroma represents an exceptionally rare collision tumor characterized by the absence of typical clinical or imaging features, leading to potential misdiagnosis. Adrenal incidentalomas present as multifaceted conditions, and this case serves to heighten awareness of their intricate nature. Due to the challenges in preoperative differentiation of various adrenal mass types, postoperative pathological analysis is imperative for guiding the subsequent treatment course for the patient.

Chondroblastoma of the femoral head-surgical management, outcomes and clinical perspectives: a case report

Chondroblastoma is a rare benign tumor predominantly affecting the epiphyses of long bones, often presenting in the second decade of life. It is known for its potential to recur and cause joint complications despite its benign histology. This report details the case of a 12-year-old boy diagnosed with chondroblastoma of the femoral head, who underwent surgical treatment involving curettage and the use of synthetic hydroxyapatite (HA) bone substitutes. The patient presented with groin pain and limping, with imaging studies revealing an osteolytic lesion in the femoral head. A core needle biopsy confirmed the diagnosis. To manage the lesion, an anterolateral approach was used to perform extensive curettage, and the defect was filled with HA blocks. Postoperatively, the patient was managed with gradual weight-bearing and regular follow-ups. At five years, the patient exhibited normal hip function with no evidence of tumor recurrence, collapse of the femoral head, or osteoarthritis. This case demonstrates that the use of synthetic HA as a bone substitute in the treatment of chondroblastoma can effectively preserve joint integrity and function, providing a viable alternative to traditional grafting methods. The approach was successful in maintaining articular congruence and preventing complications, highlighting the potential benefits of synthetic materials in managing subchondral bone lesions.

Atrial Myxoma – A Rare Cause of Heart Failure

Abstract Atrial myxomas are rare cardiac tumors that usually present with diverse, unrelated symptoms. Conversely, heart failure (HF) is a common cardiac disorder that is mostly caused by uncontrolled systemic hypertension. It is a progressive cardiac disease that is managed with guideline directed medical therapy (GDMT). We present the case of a farmer who presented with typical symptoms of HF. Echocardiography however revealed an unusual cause of his symptoms—a huge mass in his left atrium (LA) prolapsing through the mitral valve, causing functional mitral stenosis. The mass is suspected to be an atrial myxoma. Surgical excision of this mass is proposed as the cure, which will obviate the need for continuous use of GDMT. This case report and literature review bring to fore the mechanisms by which atrial myxoma can cause HF and the other varied clinical presentations of atrial myxomas.

Intraventricular ganglioneuroblastoma: an uncommon location for a rare tumour in a young adult with review of literature

Intraventricular ganglioneuroblastoma: an uncommon location for a rare tumour in a young adult with review of literature

Adult Uterine Wilm’s Tumor Miss-interpreted as Carcinosarcoma: A Case Report

Introduction: Wilm’s tumor (WT) is a common renal malignancy in children. Adult extrarenal WT involving the uterus is extremely rare. Herein, we reported a case of uterine WT in an old woman who was primarily diagnosed with carcinosarcoma. Case Presentation: A 63-year-old woman presented with abdominal pain and radiologic evidence of pelvic mass originating from the uterus, which was suggestive of sarcoma. Her Serum levels of Cancer Antigen 125 (CA125), Human Epididymis protein 4 (HE4), Cancer Antigen 19-9 (CA19-9), and Alpha Fetoprotein (AFP) were found to be elevated. She underwent total hysterectomy, bilateral salpingo-oophrectomy, and omentectomy. Pathologic examination of the specimens revealed a large uterine mass with serosal surface and omental invasion. The initial diagnosis was carcinosarcoma based on the mixed epithelial and mesenchymal components observed on microscopic examination. Triphasic components of papillary, primitive tubules, and glomeruloid structures, along with mesenchymal and blastemal elements were identified upon second opinion. An immunohistochemistry (IHC) study revealed positive reactions for WT1, CKAE1 /AE3, EMA, Glypican-3, and CD56 in all components of the tumor cells. P53 expression was normal. GATA3, ER, PR, and CD10 were negative. The final diagnosis was changed to WT. Conclusions: Uterine WT is an extremely rare malignancy, which could be associated with elevated serum AFP. Diagnosis and treatment of these rare tumors pose challenges for both pathologists and clinicians.

The many faces of myxopapillary ependynomas.

Myxopapillary ependymomas (MPE), classified as grade 2 tumors by the WHO, are rare spinal neoplasms. Despite their slow growth and generally benign nature, MPE have a high recurrence rate and potential for cerebrospinal fluid dissemination. This study aims to identify the MRI characteristics and pathological patterns of MPE and investigate potential correlations between the MRI characteristics and specific histopathological patterns. We assessed 13 patients (7 men; mean age, 45.1 years) with pathologically proven MPE. MRI images were reviewed for tumor location, size, T1 and T2 signal characteristics, contrast enhancement, hemosiderin cap presence, vertebral scalloping, drop metastasis, and prominent intradural flow voids. Four histopathological patterns (microcystic, solid, hemorrhagic, and high hyalin content) were defined and segmented, with surface areas measured and percentages calculated relative to the total tissue surface. Most tumors were in the lumbar region (84.61%), with MRI revealing typical features such as T2 hyperintensity (100%) and contrast enhancement (92.3%). A rare non-enhancing MPE was noted. Large tumors exhibited a microcystic pathology pattern, with two cases with this pattern showing drop metastasis on MRI. Smaller tumors typically presented a solid pathology pattern with homogenous MRI signals. This study underscores the diverse MRI presentations of MPE and suggests a potential link between microcystic patterns in pathology and large MPE with drop metastasis.ABBREVIATIONS: MPE= myxopapillary ependymoma.

Primary Ewing Sarcoma in the Sphenoidal Sinus: A Case Report.

Ewing's sarcoma (EWS) is a rare malignant bone tumor that primarily affects children and young adults. While it typically affects long bones, it can occur in the head and neck region, including the paranasal sinuses in rare cases. We present a challenging case of a 45-year-old female diagnosed with an EWS of the sphenoidal and the right nasal fossa. A subtotal removal of the mass was performed on the patient followed by combined adjuvant radiotherapy and chemotherapy. Due to its rarity, the diagnosis and management of EWS in the paranasal sinuses are challenging.

Mixed adenoneuroendocrine carcinoma: case report

Mixed adenoneuroendocrine carcinoma is a rare tumor of the gastrointestinal tract with double differentiation into adenomatous and neuroendocrine carcinoma, each component with at least 30%. A 60-year-old female with acute abdominal pain. Surgical treatment was decided, finding a tumor at the level of the cecum and ascending colon, a right hemicolectomy and ileostomy were performed. Mixed adenoneuroendocrine carcinoma can appear in various organs. They are highly malignant tumors, with a high risk of metastasis. These tumors do not present symptoms or specific radiological or laboratory findings; diagnosis depends on postoperative histopathological and immunohistochemical studies.

Concurrent Esophageal Leiomyoma and Helicobacter pylori Infection

Esophageal leiomyomas are rare benign tumors arising from smooth muscle cells in the esophagus. Despite their rarity, they present unique diagnostic and management challenges due to varied symptomatology and anatomical locations. Here, we present a case of a 40-year-old male with unresolved dysphagia and epigastric pain referred for esophagogastroduodenoscopy (EGD). EGD revealed a medium-sized submucosal mass at the gastroesophageal junction, partially obstructing the lumen. Concurrently, severe chronic active gastritis with Helicobacter pylori (H. pylori) infection was confirmed upon gastric biopsy. Subsequent evaluation included endoscopic ultrasound (EUS), which identified a likely benign esophageal leiomyoma. Although a surgical referral was recommended, the patient was deferred. This case highlights the importance of EGD in diagnosing esophageal leiomyomas and uncovering incidental findings that can significantly influence patient management. The rare presentation of this leiomyoma at the gastroesophageal junction, combined with the co-occurrence of severe H. pylori-induced gastritis and chronic GERD, introduces new considerations for understanding the pathophysiology of esophageal tumors. The rare combination of these factors highlights the need for further research into their interactions, providing novel insights that could influence future diagnostic strategies and therapeutic approaches for similar cases.

Abstract B032: Evaluating T cell-inflammation in pancreatic neuroendocrine tumors

Abstract Background: Pancreatic neuroendocrine tumors (pNETs) are rare tumors arising from neuroendocrine cells in the pancreas. Their clinical behaviour and prognosis are variable and durable responses are often difficult to achieve with conventional therapies. Immunotherapy approaches may be efficacious in a subgroup of pNETs. In a recent study that did not include pNETs, we showed that a four-chemokine signature (c-Score: CCL4, CCL5, CXCL9, CXCL10) predicts T cell-inflammation and response to immune checkpoint inhibitors across cancers. We have hypothesized that the c-Score will also identify pNET subgroups with T cell-inflammation. Methods: The c-Score and hallmarks of the cancer-immunity cycle are being evaluated in pNETs as a cohort study (n=100 cases) of the Marathon of Hope Cancer Centres Network whole genome and transcriptome tumor sequencing (WGTS) project. The cohort study will compare pNET profiles with those of other pancreatic and peri-ampullary tumors. Results: As of June 1, 2024, primary tumor data from 23 cases were available for analysis, including 16 pNETs, 2 pancreatic ductal adenocarcinomas (PDAC), 2 pancreatic adenosquamous carcinoma (PASC), 2 distal cholangiocarcinomas (dCCA), and 1 ampullary carcinoma (AC). Dimension reduction by principle component analysis (PCA) revealed clustering based on histological subtype. Interestingly, PCA using immune genes also demonstrate subgrouping that overlapped with the histological subtypes, suggesting that immunity of pancreatic/peri-ampullary neoplasms may be directed by histological types rather than the pancreas gland itself. These cancers showed a broad distribution of c-Score expression, with pNETs having the lowest c-Score. Tumors with high expression of the c-Score, including 2 pNETs, had increased expression of cancer-immunity cycle gene sets. DUX4, FAM186A, and IGFN1 were amongst the most common genes mutated in all tumors. Conclusions: Early data suggest that pNETs generally harbor low c-Score values and hallmarks of cancer-immunity, with the existence of a subgroup of pNETs displaying T cell-inflammation. Citation Format: Joan Miguel Romero, James Vafiadis, Tess Aalto, Ruomeng Fang, Yifan Wang, George Zogopoulos. Evaluating T cell-inflammation in pancreatic neuroendocrine tumors [abstract]. In: Proceedings of the AACR Special Conference in Cancer Research: Advances in Pancreatic Cancer Research; 2024 Sep 15-18; Boston, MA. Philadelphia (PA): AACR; Cancer Res 2024;84(17 Suppl_2):Abstract nr B032.

Navigating the Diagnosis and Treatment of Extra-Adrenal Pheochromocytoma Presenting with Chronic Abdominal Pain: A Clinical Conundrum

Background: Extra-adrenal pheochromocytomas, or paragangliomas, are rare neuroendocrine tumors that present diagnostic challenges due to their nonspecific symptoms and radiologic features.Objective: To report a case of extra-adrenal pheochromocytoma presenting as chronic abdominal pain and hepatic masses, emphasizing the diagnostic and therapeutic challenges.Methods: A 36-year-old male with a history of diabetes and hypertension presented with weight loss and abdominal pain. Initial evaluation included comprehensive physical examination, laboratory tests, ultrasonography, and CT imaging of the abdomen and chest. Partial hepatectomy was performed, and the diagnosis was confirmed through histopathological and immunohistochemical analysis.Results: Imaging identified three mixed echogenic hepatic masses, with the largest measuring 5.6 x 5.4 cm. Enlarged para-aortic lymph nodes were observed, the largest being 4.0 x 3.5 cm. CT chest revealed a mass on the chest wall (7.0 x 3.0 cm) with metastatic lung nodules. Immunohistochemistry confirmed paraganglioma with positivity for chromogranin A and neuron-specific enolase.Conclusion: This case underscores the importance of considering paragangliomas in differential diagnoses of atypical abdominal masses and highlights the critical role of histopathological confirmation in guiding management.

Locally Advanced Porocarcinoma in a Vietnamese Patient: A Literature Review and Case of a Rare Tumor Diagnosed Using Global Health Collaboration

Locally Advanced Porocarcinoma in a Vietnamese Patient: A Literature Review and Case of a Rare Tumor Diagnosed Using Global Health Collaboration

Incidence, survival, and prognostic nomogram of patients with small intestinal neuroendocrine tumors: A SEER population-based study.

Small intestinal neuroendocrine tumors (SI-NETs) are a group of rare and significantly heterogeneous tumors with limited research currently available. This study aimed to investigate the incidence, survival, and prognostic factors of SI-NETs. We selected data from the surveillance, epidemiology, and end results (SEER) database between 2000 and 2019 and evaluated the incidence trend of SI-NETs during this period. We utilized the Kaplan-Meier method to examine the association between clinical variables and survival rates. Based on the multivariable Cox regression analysis results, we developed a nomogram to predict the 1-, 2-, and 3-year cancer-specific survival (CSS) of SI-NETs patients. We evaluated the consistency, accuracy, and clinical utility of the nomogram by drawing calibration curves, receiver operating characteristic (ROC) curves, and decision curve analysis (DCA) curves. The incidence of SI-NETs showed an upward trend in recent years. Age, grade, T stage, M stage, and primary tumor surgery were independent risk factors for CSS in SI-NETs patients. The nomogram model based on these risk factors showed high accuracy and clinical benefit. SI-NETs are rare tumors with an increasing incidence rate. The nomogram model is expected to be an effective tool for personalized prognosis prediction in SI-NETs patients, which may benefit clinical decision-making.