Xiaoping Yi

Rhabdomyosarcoma in adrenal region of a child with hypertension and fever: a case report and literature review.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children but rarely involves the adrenal. We describe a case of alveolar rhabdomyosarcoma (ARMS) of the right adrenal in a 5-year-old boy with a characteristic history of hypertension and recurrent fever. On surveillance imaging, a right adrenal mass was incidentally detected, and a right adrenalectomy was subsequently performed. After the surgery, the fever disappeared simultaneously, and the blood pressure gradually returned to normal level. This is the first reported case in children.

Preoperative prediction of microvascular invasion of hepatocellular carcinoma with IVIM diffusion-weighted MR imaging and Gd-EOB-DTPA-enhanced MR imaging

Microvascular invasion (MVI) is regarded as one of the independent risk factors for recurrence and poor prognosis of hepatocellular carcinoma (HCC). The presence of MVI in HCCs was evaluated on the basis of pathological reports of surgical specimens and was defined as tumor within a vascular space lined by endothelium that was visible only on microscopy. The aim of the study was to investigate the usefulness of intravoxel incoherent motion (IVIM) diffusion weighted (DW) magnetic resonance (MR) imaging in predicting MVI of HCC. Preoperative IVIM DW imaging and Gd-EOB-DTPA-enhanced MRI (DCE-MRI) of 51 patients were analyzed. Standard apparent diffusion coefficient (ADC), D (the true diffusion coefficient), D* (the pseudodiffusion coefficient) and f (the perfusion fraction), relative enhancement (RE) and radiological features were evaluated and analyzed. Univariate analysis revealed that HCCs with MVI had a higher portion of an irregular tumor shape than HCCs without MVI (p = 0.009), the Standard ADC, D value were significantly lower in HCCs with MVI (p = 0.022, p = 0.007, respectively). Multivariate analysis revealed that an irregular shape (p = 0.012) and D value ≤ 1.16×10-3mm2/sec (p = 0.048) were independent predictors for MVI. Combining the two factors of an irregular shape and D value, a sensitivity of 94.4% and specificity of 63.6% for predicting MVI was obtained. In conclusion, we found that an irregular shape and D value ≤ 1.16×10-3mm2/sec may suggest the presence of MVI in HCCs.

Long-term follow-up and novel splice donor mutation in MEN1 in a Chinese family

Heterozygous germline mutation of the MEN1 tumor suppressor gene is responsible for multiple endocrine neoplasia type 1. Parathyroid and thoracic neuroendocrine tumor specimens and DNA from two Han Chinese MEN1 family patients were analyzed using whole exome and Sanger sequencing. The proband (II-3) was sequentially diagnosed with pituitary adenoma, pancreatic tumor, adrenal cortical tumor, abdominal lipoma, and parathyroid adenoma during the 6-year follow-up. The son of the proband (III-6) was also diagnosed with a thoracic neuroendocrine tumor and a parathyroid adenoma during this period. Splice alterations were studied by RT-PCR and sequencing. The mutation impact was evaluated using bioinformatics. Sequence analysis revealed a novel splice donor mutation, MEN1 IVS9 + 1G > C, that changed the splicing mode of MEN1 to halt translation before two nuclear localization signals in the menin protein. Novel somatic mutations, MEN1 c.1402_1405delGAGG and c.286 C > T, were identified in the parathyroid adenoma of II-3 and thoracic neuroendocrine tumor of III-6, respectively, indicating a two-hit etiology of MEN1 syndrome. Our study revealed the clinical and genetic basis of MEN1 in this Han Chinese family and provides insight into MEN1 mechanisms, diagnosis, and management.

Catheter-Based Computed Tomography Angiography in Anterolateral Thigh Perforator Mapping of Chinese Patients

Background During reconstructive surgery, anterolateral thigh (ALT) flap harvest is challenging due to variation and uncertainty in perforator distribution. We performed a pilot study to identify the predictive value of catheter‐based computed tomography angiography (C‐CTA) and traditional CTA (T‐CTA) in ALT perforator mapping for patients whose ALT perforators were difficult to identify. Methods Thirty‐four consecutive T‐CTA/C‐CTA‐mapped ALT flaps were evaluated for extremity reconstruction. The perforator location, origin, and course were compared between T‐CTA/C‐CTA imaging and intraoperative findings. The mapping efficiency of T‐CTA and C‐CTA was compared thoroughly. Results Among the 34 ALT thigh flaps, 117 (36) of the 130 perforators identified intraoperatively were visible on C‐CTA (T‐CTA) in a subgroup of Chinese limb trauma patients with limited activity. C‐CTA showed a satisfactory efficiency in perforator mapping, which was much better than the efficiency of T‐CTA. C‐CTA also showed a much better sensitivity (90.00 vs. 27.69%), specificity (94.74 vs. 66.67%), and accuracy (91.07 vs. 36.69%), and a much lower false‐positive (1.68 vs. 26.53%), and false‐negative rate (10.00 vs. 72.31%). Moreover, C‐CTA could accurately predict the origin and septocutaneous or intramuscular course in all identified perforators. All flaps were elevated successfully and survived. Conclusion C‐CTA outperforms T‐CTA in the preoperative perforator mapping of ALT flaps in a subgroup of Chinese limb trauma patients. C‐CTA should be the method of choice for perforator mapping in patients whose ALT flaps are intended for extremity reconstruction.

Pancreatic primitive neuroectodermal tumor: Focus on radiological features and differential diagnosis – A case report and literature review

Primitive neuroectodermal tumor (PNET) is an exceedingly rare type of malignant tumor. The diagnosis of pancreatic PNET is usually challenging for radiologists and surgeons, especially when an accurate preoperative diagnosis is needed. Herein, we report a case of a 36-year-old patient with a mass diagnosed as PNET in the head of the pancreas and present a literature review. Compared to previous literature reports, there were some imaging features observed by computed tomography (CT) in our case that might be helpful for a relatively accurate preoperative diagnosis. PNET should be considered preoperatively for soft-tissue neoplasms of the pancreas when the diagnosis of more common pancreatic tumors is not favored by signs in CT, especially in pediatric and adolescent populations. This case is the 20th case of pancreatic PNET reported in literature.

Adrenal incidentaloma: machine learning-based quantitative texture analysis of unenhanced CT can effectively differentiate sPHEO from lipid-poor adrenal adenoma

Objective: To evaluate the feasibility and accuracy of machine learning based texture analysis of unenhanced CT images in differentiating subclinical pheochromocytoma (sPHEO) from lipid-poor adenoma (LPA) in adrenal incidentaloma (AI). Methods: Seventy-nine patients with 80 LPA and 29 patients with 30 sPHEO were included in the study. Texture parameters were derived using imaging software (MaZda). Thirty texture features were selected and LPA was performed for the features selected. The number of positive features was used to predict results. Logistic multiple regression analysis was performed on the 30 texture features, and a predictive equation was created based on the coefficients obtained. Results: LPA yielded a misclassification rate of 19.39% in differentiating sPHEO from LPA. Our predictive model had an accuracy rate of 94.4% (102/108), with a sensitivity of 86.2% (25/29) and a specificity of 97.5% (77/79) for differentiation. When the number of positive features was greater than 8, the accuracy of prediction was 85.2% (92/108), with a sensitivity of 96.6% (28/29) and a specificity of 81% (64/79). Conclusions: Machine learning-based quantitative texture analysis of unenhanced CT may be a reliable quantitative method in differentiating sPHEO from LPA when AI is present.

Cortical Surface Area Rather Than Cortical Thickness Potentially Differentiates Radiation Encephalopathy at Early Stage in Patients With Nasopharyngeal Carcinoma

Radiation encephalopathy (RE) is one of the most severe complications in nasopharyngeal carcinoma (NPC) patients after radiotherapy (RT). However, the morphological alteration of early RE is insufficiently investigated. We aimed to investigate the cortical thickness and surface area alterations in NPC patients with or without RE in the follow-up. A total of 168 NPC patients each underwent a single scan and analysis at various times either Pre-RT (n = 56) or Post-RT (n = 112). We further divided the Post-RT NPC patients into three groups based on the time of the analysis following RT (Post-RTwithin 6 months and Post-RT7-12 months) or whether RE signs were detected in the analysis (Post-RTRE proved in follow-up). We confined the vertex-wise analyses of the cortical thickness and surface area to the bilateral temporal lobes. Interestingly, we revealed a gradual increase in the cortical surface area of the temporal lobe with increasing time after RT within the Post-RTRE proved in follow-up group, consistent with the between-group findings, which showed a significant increase in cortical surface area in the Post-RTRE proved in follow-up group relative to the Pre-RT group and the Post-RTwithin 6 months group. By contrast, such a trend was not observed in the cortical thickness findings. We concluded that the cortical surface area, rather than cortical thickness, may serve as a potential biomarker for early diagnosis of RE.

Gliosarcoma: a clinical and radiological analysis of 48 cases

ObjectivesTo retrospectively review the radiological and clinicopathological features of gliosarcoma (GSM) and differentiate it from glioblastoma multiforme (GBM).MethodsThe clinicopathological data and imaging findings (including VASARI analysis) of 48 surgically and pathologically confirmed GSM patients (group 1) were reviewed in detail, and were compared with that of other glioblastoma (GBM) cases in our hospital (group 2).ResultsThere were 28 men and 20 women GSM patients with a median age of 52.5 years (range, 24-80 years) in this study. Haemorrhage (n = 21), a salt-and-pepper sign on T2-weighted images (n = 36), unevenly thickened wall (n = 36) even appearing as a paliform pattern (n = 32), an intra-tumoural large feeding artery (n = 32) and an eccentric cystic portion (ECP) (n = 19) were more commonly observed in the GSM group than in GBM patients. Based on our experience, GSM can be divided into four subtypes according to magnetic resonance imaging (MRI) features. When compared to GBM (group 2), there were more patients designated with type III lesions (having very unevenly thickened walls) and IV (solid) lesions among the GSM cases (group 1). On univariate prognostic analysis, adjuvant therapy (radiotherapy, chemotherapy, and radiochemotherapy) and existence of an eccentric cyst region were prognostic factors. However, Coxs regression model showed only adjuvant therapy as a prognostic factor for GSM.ConclusionsWhen compared to GBM, certain imaging features are more likely to occur in GSM, which may help raise the possibility of this disease. All GSM patients are recommended to receive adjuvant therapy to achieve a better prognosis with radiotherapy, chemotherapy or radiochemotherapy all as options.Key Points• Diagnosis of gliosarcoma can be suggested preoperatively by imaging.• Gliosarcoma can be divided into four subtypes based on MRI.• Paliform pattern and ECP tend to present in gliosarcoma more than GBM.• The cystic subtype of gliosarcoma may predict a more dismal prognosis.• All gliosarcoma patients should receive adjuvant therapy to achieve better prognosis.

Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases

Objectives To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions. Methods Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed. Results Of eighteen PNET patients, fifteen were male and three were female, with a median age of 36 years (range, 2–65 years). The onset of symptoms was most often nonspecific and insidious. The mean tumor diameter was 7.2 cm (range, 3.0–12.1 cm), with necrosis in fifteen cases, cystic changes in eight, partition structure in five, calcification in five, hemorrhage in two, and mural nodules in one. Contrast enhanced computed tomography showed multiple tiny feeding arteries within the masses in six cases, resulting in a crab-like appearance, and mild ring enhancement pattern in five cases. Eleven cases showed surrounding invasion and metastasis. Of the eighteen PNET cases, nine cases showed smooth, well-defined margins, and nine cases had irregular, ill-defined margins. A median survival was 10.0±1.6 months. However, chemotherapy had efficacy on patients even those with advanced disease. Conclusions Primary intra-abdominal and retroperitoneal PNETs are rare, and imaging features documented here may help the diagnosis of this severe disease. Notably, two signs present in retroperitoneal PNET tumors, including a mild ring enhancement pattern and a crab-like appearance of the tiny feeding arteries, may have the potential to help us improve the ability to make a relatively reliable diagnosis.