Machine learning to differentiate small round cell malignant tumors and non-small round cell malignant tumors of the nasal and paranasal sinuses using apparent diffusion coefficient values

Objective We used radiomics feature–based machine learning classifiers of apparent diffusion coefficient (ADC) maps to differentiate small round cell malignant tumors (SRCMTs) and non-SRCMTs of the nasal and paranasal sinuses. Materials A total of 267 features were extracted from each region of interest (ROI). Datasets were randomized into two sets, a training set (∼70%) and a test set (∼30%). We performed dimensional reductions using the Pearson correlation coefficient and feature selection analyses (analysis of variance [ANOVA], relief, recursive feature elimination [RFE]) and classifications using 10 machine learning classifiers. Results were evaluated with a leave-one-out cross-validation analysis. Results We compared the AUC for all the pipelines in the validation dataset using FeAture Explorer (FAE) software. The pipeline using RFE feature selection and Gaussian process classifier yielded the highest AUCs with ten features. When the “one-standard error” rule was used, FAE produced a simpler model with eight features, including Perc.01%, Perc.10%, Perc.90%, Perc.99%, S(1,0) SumAverg, S(5,5) AngScMom, S(5,5) Correlat, and WavEnLH_s-2. The AUCs of the training, validation, and test datasets achieved 0.995, 0.902, and 0.710, respectively. For ANOVA, the pipeline with the auto-encoder classifier yielded the highest AUC using only one feature, Perc.10% (training/validation/test datasets: 0.886/0.895/0.809, respectively). For the relief, the AUCs of the training, validation, and test datasets that used the LRLasso classifier using five features (Perc.01%, Perc.10%, S(4,4) Correlat, S(5,0) SumAverg, S(5,0) Contrast) were 0.892, 0.886, and 0.787, respectively. Compared with the RFE and relief, the results of all algorithms of ANOVA feature selection were more stable with the AUC values higher than 0.800. Conclusions We demonstrated the feasibility of combining artificial intelligence with the radiomics from ADC values in the differential diagnosis of SRCMTs and non-SRCMTs and the potential of this non-invasive approach for clinical applications. Key Points • The parameter with the best diagnostic performance in differentiating SRCMTs from non-SRCMTs was the Perc.10% ADC value. • Results of all the algorithms of ANOVA feature selection were more stable and the AUCs were higher than 0.800, as compared with RFE and relief. • The pipeline using RFE feature selection and Gaussian process classifier yielded the highest AUC.

Primary desmoplastic small round cell tumor of the submandibular gland: a case report and literature review

Background Desmoplastic small round cell tumor (DSRCT) is a sporadic, highly malignant tumor with a poor prognosis. The abdomen and pelvis have been reported as the primary localization sites. However, to the best of our knowledge, there are few reports on primary DSRCT in the submandibular gland. Case presentation We report a case of a 26-year-old Chinese man with a mass in the right submandibular gland. Imaging studies showed a hypoechoic mass in the right submandibular region. Intraoperative pathology revealed that the tumor tissue was composed of small round tumor cells and a dense desmoplastic stroma. On immunostaining, the tumor cells showed markers of epithelial, mesenchymal, myogenic, and neural differentiation. The EWSR1 gene rearrangement was detected by fluorescence in situ hybridization. Based on the overall morphological features and immunohistochemical findings, a final diagnosis of DSRCT was made. The patient was treated with comprehensive anti-tumor therapy mainly based on radiotherapy and chemotherapy. Conclusions DSRCT is an uncommon malignant neoplasm with rare submandibular gland involvement. In this report, we have described a case of DSRCT in the submandibular gland and reviewed the literature on DSRCT over the past 5 years. Considering the importance of differential diagnosis between DSRCT, especially with rare extra-peritoneal involvement, and small round blue cell tumors, a full recognition of the clinicopathological features will help to better diagnose this neoplasm.

A Case Report on Malignant Rhabdoid Round Cell Tumor in Pelvis

The malignant neoplasm is called as malignant rhabdoid tumor or renal tumor. The Malignant tumor had the highest rate of proliferation. Tiny, round, and generally undifferentiated cells make up malignant small round cell tumours. A round cell tumor is a group of malignant tumors composed of relatively small and undifferentiated cells with an increased nuclear - cytoplasmic ratio. Soft tissue malignant tumours of the abdomen and pelvis are a rare but serious kind of cancer. Examples of these tumours include Ewing's sarcoma, peripheral neuroectodermal tumour, rhabdomyosarcoma, synovial sarcoma, non-lymphoma, Hodgkin's retinoblastoma, neuroblastoma, and hepatoblastoma. Mast cell tumour, histiocytoma, lymphoma, plasmacytoma, and transmissible venereal tumours are some of the different types of round cell tumours. Melanomas are the cytologic "great impostor," as they might look on cytology as round cell tumours despite being classed as mesenchymal cancers. Rhabdoid tumours have long been thought to be extremely malignant and have a bad prognosis. Children with this form of tumour have a six to eleven-month median survival duration. They're also less common. In 5% of small round cell tumor patients, can be curable, and it is best achieved by combining systemic chemotherapy with thorough cytoreductive surgery. Here we report a 9-year-old female child who was diagnosed with a malignant rhabdoid round cell tumor in the pelvis. She has undergone excision of pelvic floor tumor andfurther managed with Chemotherapy.

Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor

Desmoplastic small round cell tumor (DRSCT) is a highly aggressive primitive sarcoma that primarily affects adolescent and young adult males. The 5-year survival rate is 15-30% and few curative treatment options exist. Although there is no standard treatment for DSRCT, patients are most often treated with a combination of aggressive chemotherapy, radiation, and surgery. Targeted therapy inhibitors of PDGFA and IGF-1R, which are almost uniformly overexpressed in DSRCT, have largely failed in clinical trials. As in cancer in general, interest in immunotherapy to treat DSRCT has increased in recent years. To that end, several types of immunotherapy are now being tested clinically, including monoclonal antibodies, radionuclide-conjugated antibodies, chimeric antigen receptor T cells, checkpoint inhibitors, and bispecific antibodies (BsAbs). These types of therapies may be particularly useful in DSRCT, which is frequently characterized by widespread intraperitoneal implants, which are difficult to completely remove surgically and are the frequent cause of relapse. Successful treatment with immunotherapy or radioimmunotherapy following debulking surgery could eradiate these micrometasteses and prevent relapse. Although there has been limited success to date for immunotherapy in pediatric solid tumors, the significant improvements in survival seen in the treatment of other pediatric solid tumors, such as metastatic neuroblastoma and its CNS spread, suggest a potential of immunotherapy and specifically compartmental immunotherapy in DSRCT.

A rare cause of bilateral pleural effusion – desmoplastic small round cell tumor

Desmoplastic small round cell tumor (DSRCT) is a rare, extremely aggressive and malignant tumor predominantly affects young adolescent males and typically presents as a large intra-abdominal mass. However, tumor arising from other body sites are also reported in the literature. Histology and immunohistochemistry play an important role in the diagnosis and differentiating this rare tumor from other round cell tumors. A multidisciplinary approach consisting of a combination of surgery, chemotherapy and radiation therapy is the treatment of choice as there is no standard therapy. We report a case of DSRCT of pleura presenting as bilateral pleural effusion in a young adolescent male who was treated with both surgery and chemotherapy. However patient succumbed to death after one year of diagnosis.

A CASE REPORT OF A YOUNG MAN WITH ABDOMINAL MASS: HUNT FOR DIAGNOSIS

Introduction: Extraskeletal Ewing sarcoma/PNET is a small round cell sarcoma showing gene fusions of EWSR1-FLI1. A 28-year-old male patient presented with right ank pain and low gr Case Report: ade fever since 15 days. On examination: a mass was palpable in the right hypochondrium. Provisional diagnosis of Liver abscess has been made. USG abdomen shows features cystic lesion in the liver with internal septation ?Liver abscess /Hydatid cyst. Intraoperatively, tumor was seen attached to upper pole of kidney. Since tumor was large, it was ruptured intraoperatively and debulking surgery has been done. Under microscopy, tumor was arranged in sheets with intervening stroma showing desmoplastic reaction. Perivascular pseudorosettes are seen. The diagnosis of malignant small round cell tumor has been given. On immunohistochemistry, tumor cells are positive for Vimentin, CD99, NKX2.2 , FLI1, Neurolaments, Synaptophysin with focal immunoreactivity for EMA, Pancytokeratin. Final diagnosis was EXTRASKELETAL EWING SARCOMA/PNET. Discussion: Extra-skeletal Ewing sarcoma/PNET is malignant soft tissue tumor seen in chest wall, thigh, paravertebal region etc. Retroperitoneum is a least common site. Most common presentation is swelling in the soft tissue with compressive symptoms. Histologically, it is composed of undifferentiated small round cells. Conclusion: Clinical examination and radiological ndings leads to ambiguous diagnosis in Ewing sarcoma/PNET. Hence proper histopathological study is essential for nal diagnosis.