Rosai-Dorfman disease involving the entire esophagus.

We present a case of Rosai-Dorfman disease (RDD) occurred in a 6-year-old male child, characterized by extensive involvement of the esophagus. Eight months ago, the child presented with persistent fever and a diffuse dark red rash. MRI revealed a mass occupying the left nasal cavity and septal sinus and biopsy pathology confirmed the diagnosis of RDD. Following dexamethasone and prednisone treatment, the child experienced dysphagia. 18F-FDG PET/CT revealed multiple lesions with increased metabolism in the left nasal sinus, lymph nodes, widespread skin lesions, and the entire esophagus. Subsequent biopsies of lymph nodes, abdominal skin, and esophageal lesions was consistent with RDD involvement (Fig. 1F-1G). The child is presently undergoing six cycles of VCR+Ara-c+Dex chemotherapy and the treatment is going well.

Computed tomography features and clinicopathological characteristics of gastric glomus tumor.

BACKGROUND: Gastric glomus tumor (GGT) is a rare neoplasm that is difficult to distinguish from other gastric submucosal tumors due to a lack of diagnostic experience. The goal of this study was to better understand GGT by looking at its clinicopathological features, computed tomography (CT) features, and differential diagnosis. METHODS: The clinical data and CT findings of 21 pathologically confirmed GGT patients were examined. The clinical characteristics and CT findings of benign GGT were compared to gastric stromal tumors (GST) (n = 30) and heterotopic pancreas (n = 30). RESULTS: The 21 cases included six males and fifteen females ranging in age from 42 to 64 years. The lesions were found in the gastric body in four cases and the antrum in seventeen. GGT was diagnosed as benign in 20 cases and malignant in one. In benign cases, the glomus cells were small, uniform, showed perivascular hemangiopericytoma­like or solid nest­like structures. Obvious mitotic figures were observed in the malignant case. SMA staining was positive in the tumor cells. A quasi-round or round solid mass protruded into the gastric cavity in 20 benign cases, with a clear and smooth edge. The long to short diameter ratio was 1.01 ± 0.15. All of the benign cases had obvious enhancement, with homogeneous enhancement in ten cases and heterogeneous enhancement in ten cases, as well as central filling enhancement in 12 cases. The ratio of CT value of lesion to abdominal aorta in arterial phase and venous phase were (0.41 ± 0.11) and (0.81 ± 0.20), which were significantly higher than GST and heterotopic pancreas. The irregular mass broke through the gastric wall and invaded liver with poorly defined boundary and internal necrosis, heterogeneous persistent moderate enhancement with thickening blood supply arteries was seen in one malignant case with a long diameter of 150 mm and a thick diameter of 30 mm. CONCLUSIONS: CT enhancement usually shows persistent obvious enhancement, especially in arterial phase, which provides important value for the diagnosis. CT findings can help in the differential diagnosis of GGT and other submucosal tumors.

Intravascular papillary endothelial hyperplasia of the spleen in a child: a case report.

BACKGROUND: Intravascular papillary endothelial hyperplasia (IPEH) is a vascular tumor characterized by the proliferation of endothelial cells with papillary formation. It is a rare benign condition affecting the head and neck. Currently, no cases of IPEH of the spleen have been reported. Here, we report a case of IPEH of the spleen in a child and discuss its clinical manifestations, imaging features, and surgical treatment. CASE PRESENTATION: A 5-year-old female presented with a 4-month-old tumor in the left upper abdomen, abdominal pain, and constipation. She underwent radiography, barium enema, US, and MRI. A solid space-occupying mass was found in the left abdominal cavity on preoperative imaging, and it was diagnosed as angiosarcoma. The lesion was surgically resected. Histopathological analysis was consistent with IPEH. CONCLUSION: Clinicians should consider the possibility of IPEH in patients presenting with tumors in the spleen, which is curable by surgical resection. Malignant vascular tumors must be excluded in the differential diagnosis of IPEH to prevent misdiagnosis and inappropriate overtreatment.

Can visceral fat parameters based on computed tomography be used to predict occult peritoneal metastasis in gastric cancer?

BACKGROUND: The preoperative prediction of peritoneal metastasis (PM) in gastric cancer would prevent unnecessary surgery and promptly indicate an appropriate treatment plan. AIM: To explore the predictive value of visceral fat (VF) parameters obtained from preoperative computed tomography (CT) images for occult PM and to develop an individualized model for predicting occult PM in patients with gastric carcinoma (GC). METHODS: A total of 128 confirmed GC cases (84 male and 44 female patients) that underwent CT scans were analyzed and categorized into PM-positive (n = 43) and PM-negative (n = 85) groups. The clinical characteristics and VF parameters of two regions of interest (ROIs) were collected. Univariate and stratified analyses based on VF volume were performed to screen for predictive characteristics for occult PM. Prediction models with and without VF parameters were established by multivariable logistic regression analysis. RESULTS: The mean attenuations of VFROI 1 and VFROI 2 varied significantly between the PM-positive and PM-negative groups (P = 0.044 and 0.001, respectively). The areas under the receiver operating characteristic curves (AUCs) of VFROI 1 and VFROI 2 were 0.599 and 0.657, respectively. The mean attenuation of VFROI 2 was included in the final prediction combined model, but not an independent risk factor of PM (P = 0.068). No significant difference was observed between the models with and without mean attenuation of VF (AUC: 0.749 vs 0.730, P = 0.339). CONCLUSION: The mean attenuation of VF is a potential auxiliary parameter for predicting occult PM in patients with GC.

Pleomorphic adenoma of the left lacrimal gland recurred and transformed into myoepithelial carcinoma after multiple operations: A case report.

BACKGROUND: Myoepithelial carcinoma (MC) is a clinically rare malignancy, there is controversy regarding its etiology and its biological behavior is not fully elucidated. Extensive surgical resection is the main treatment method. We describe a case of pleomorphic adenoma (PA) with multiple postoperative recurrences after malignant transformation, and the history of the disease in this patient was more than 20 years. Complete resection during the first surgery of PA and long-term postoperative follow-up is necessary. CASE SUMMARY: A 34-year-old male with PA and a history of 5 postoperative recurrences over 21 years, each surgically removed, presented 15 d ago with headache, nasal congestion, protrusion of the right eyeball and loss of vision in the right eye, with progressively worsening symptoms. The patient underwent surgery, and MC was confirmed by pathology examination. A small PA component was locally visible under light microscope. The patient had a recurrence of the tumor 2 mo after surgery and underwent surgical resection. CONCLUSION: During the first operation for PA, care should be taken not to rupture the envelope to prevent tumor cell implantation, and when complete resection is not possible due to the anatomical site, postoperative radiotherapy is necessary to control the lesion and prevent infiltration and malignant transformation of the tumor to MC. Computed tomography and magnetic resonance imaging is important for establishing diagnosis and developing a treatment plan.

Postoperative multiple metastasis of clear cell sarcoma-like tumor of the gastrointestinal tract in adolescent: A case report.

BACKGROUND: Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) is a rare malignant gastrointestinal mesenchymal soft tissue tumor. Its genetic feature is EWSR1 gene rearrangement. Histologically, it is often accompanied by a varying number of CD68-positive osteoclast-like giant cells. CCSLGT mostly occurs in the small intestinal wall of young people and children. In terms of clinical manifestations, there is no significant difference between it and other gastrointestinal tumors, and the diagnosis depends on immunohistochemistry and gene detection. CASE SUMMARY: A 16-year-old man developed dizziness and fatigue 2 mo ago, and 10 d ago showed progressive exacerbation of paroxysmal epigastric pain and stopped flatulence and defecation. Computed tomography showed a soft tissue mass in the distal ileum. After complete resection of the lesion, it was diagnosed by combined immunohistochemical and genetic examination as CCSLGT. After surgery, the patient gradually developed lymph node, liver, lung, bone, left thigh, pleura and adrenal metastasis. The survival time was 4 years and 8 mo. CONCLUSION: Whole abdominal computed tomography enhancement is recommended for patients with gastrointestinal symptoms. There is no effective treatment for CCSLGT with multiple metastases via the lymphatic system and bloodstream after surgical resection.

Primary clear cell sarcoma of soft tissue in the posterior cervical spine invading the medulla oblongata: A case report.

BACKGROUND: Clear cell sarcoma (CCS) is a rare and highly malignant soft tissue tumor, usually occurring in the deep soft tissues of the distal tendons and aponeurosis of the extremities, especially the feet and knees. CCS originating in the head and neck is extremely rare. The clinical manifestations of CCS in the head and neck are not typical, and the imaging manifestations have certain characteristics, but the diagnosis still depends on pathological examination and genetic testing. CASE SUMMARY: A 33-year-old male patient had paroxysmal headache for more than 4 years, accompanied by nausea and vomiting, which could be relieved after rest. Computed tomography angiography showed a left paraspinal soft tissue mass. Contrast-enhanced imaging showed obvious uneven enhancement with adjacent bone lytic destruction. Magnetic resonance imaging examination showed isosignal on T1-weighted images, slightly high signal on T2-weighted images (T2WI), high signal on Tirm fat suppression sequence, significantly high signal on diffusion weighted imaging, and obvious and uneven enhancement. The lesion invaded the anterior medulla oblongata through the left atlantoaxial foramen and compressed the cervical spinal cord on T2WI. Primary CCS of soft tissue was diagnosed by pathology and genetic examination. CONCLUSION: CCS should be considered in the differential diagnosis of soft tissue tumors of the head and neck, and their diagnosis depends on pathological examination and genetic testing.

Multimodality Imaging Evaluation of Primary Right Atrial Paraganglioma: A Case Report and Literature Review.

Background: Cardiac paraganglioma (CPGL) accounts for 1-3% of cardiac tumors and is usually benign. In total, 35-50% of CPGL lesions secrete catecholamines, causing hypertension, excessive sweating, palpitations, headache, and other symptoms. Preoperative imaging evaluation is important to determine the location of the cardiac mass, its blood supply vessels, and the relationship with surrounding structures. Multimodal imaging techniques combine with morphological and functional information to provide powerful methods for preoperative diagnosis and lesion localization. Furthermore, they can assist to reduce the incidence of intraoperative and postoperative complications and improve patient prognosis. Case Report: A 67-year-old woman suffered from paroxysmal palpitations with a heart rate of 110 beats per minute 1 month ago. Urine catecholamine and methoxyepinephrine levels were significantly increased. The patient had a 5-year history of hypertension with a maximum blood pressure of 160/100 mmHg. Computed tomography (CT) examination found a soft tissue mass in the right atrium with heterogeneous and significant enhancement, whose blood supply was from the left ileal branch artery. The patient then underwent cardiac magnetic resonance (CMR). The lesion showed inhomogeneous iso signals on the T1-weighted image (T1WI), slightly high signals on the T2 fat-suppression image, inhomogeneous high signals on the diffusion-weighted imaging (DWI), and apparent diffusion coefficient (ADC) images. The mass exhibited heterogeneous and significant enhancement on the first perfusion and delayed scans after intravenous contrast injection. However, abnormal signals were surprisingly found in the patient's right lung, and the possibility of metastatic lesions could not be excluded. The patient underwent F-18 fluorodeoxyglucose-positron emission tomography/computed tomography (18F-FDG PET/CT) to rule out metastatic lesions. A fluorodeoxyglucose (FDG)-avid soft tissue mass was shown in the right atrium, with the maximum standardized uptake value (SUVmax) at about 15.2, as well as a pathological intake of brown fat throughout the body. Combined with clinical symptoms, CPGL was considered without significant sign of metastasis in 18F-FDG PET/CT. Finally, the patient underwent surgical resection and the post-operative pathology confirmed a CPGL. Conclusion: The combination of 18F-FDG PET/CT with the CMR containing different image acquisition sequences provides a powerful aid for preoperative non-invasive diagnosis, localization, and staging of CPGL, which helps to reduce intraoperative and postoperative complications and improve patient prognosis.

Extranodal nasal NK/T cell lymphoma with bronchial mucoepidermoid carcinoma and lung adenocarcinoma: a case report.

Extranodal nasal NK/Tcell lymphoma (ENKTCL) is a relatively rare type of non-Hodgkin's lymphoma. It is highly malignant, highly invasive, and easy to relapse. Most patients have a poor prognosis. We report a 48-year-old woman who presented with irritant dry cough that had persisted for 6 m. CT showed a mass in the right nasal cavity, with uneven density similar to soft tissue, with slight uneven enhancement. The mass and the upper, middle, and lower turbinates were not clearly demarcated, involving multiple adjacent sinus cavities, and the local bone showed osteolytic destruction; MRI showed isosignal on T1WI and slightly hypersignal on T2WI and DWI. In addition, there was a mass of soft tissue density at the bronchial opening in the right middle lobe, showing uneven and obvious enhancement; a cavity was seen in the nodule of the right lower lobe, and the adjacent pleura was stretched, showing moderate enhancement. The nasal mass was diagnosed as extranodal NK/T cell lymphoma, the right middle lobe mass was diagnosed as mucoepidermoid carcinoma, and the right lower lobe mass was diagnosed as lung adenocarcinoma. ENKTCL rarely invades the lungs. If a patient has a lung occupying lesion similar to it, biopsy confirmation should be considered to avoid misdiagnosis as a chest metastasis that affects the treatment effect.

Use of radiomics containing an effective peritumoral area to predict early recurrence of solitary hepatocellular carcinoma ≤5 cm in diameter.

Background: Hepatocellular carcinoma (HCC) is the sixth leading type of cancer worldwide. We aimed to develop a preoperative predictive model of the risk of early tumor recurrence after HCC treatment based on radiomic features of the peritumoral region and evaluate the performance of this model against postoperative pathology. Method: Our model was developed using a retrospective analysis of imaging and clinicopathological data of 175 patients with an isolated HCC ≤5 cm in diameter; 117 patients were used for model training and 58 for model validation. The peritumoral area was delineated layer-by-layer for the arterial and portal vein phase on preoperative dynamic enhanced computed tomography images. The volume area of interest was expanded by 5 and 10 mm and the radiomic features of these areas extracted. Lasso was used to select the most stable features. Results: The radiomic features of the 5-mm area were sufficient for prediction of early tumor recurrence, with an area under the curve (AUC) value of 0.706 for the validation set and 0.837 for the training set using combined images. The AUC of the model using clinicopathological information alone was 0.753 compared with 0.786 for the preoperative radiomics model (P >0.05). Conclusions: Radiomic features of a 5-mm peritumoral region may provide a non-invasive biomarker for the preoperative prediction of the risk of early tumor recurrence for patients with a solitary HCC ≤5 cm in diameter. A fusion model that combines the radiomic features of the peritumoral region and postoperative pathology could contribute to individualized treatment of HCC.