Small Round Cell Research Articles

Establishment of predictive nomogram and web-based survival risk calculator for desmoplastic small round cell tumor: A propensity score-adjusted, population-based study.

Desmoplastic small round cell tumor (DSRCT) is a rare undifferentiated malignant soft tissue tumor with a poor prognosis and a lack of consensus on treatment. This study's objective was to build a nomogram based on clinicopathologic factors and an online survival risk calculator to predict patient prognosis and support therapeutic decision-making. A retrospective cohort analysis of the Surveillance, Epidemiology and End Results (SEER) database was performed for patients diagnosed with DSRCT between 2000 and 2019. The least absolute shrinkage and selection operator (LASSO) Cox regression analysis was applied to identify the individual variables related to overall survival (OS) and cancer-specific survival (CSS), as well as to construct online survival risk calculators and nomogram survival models. The nomogram was employed to categorize patients into different risk groups, and the Kaplan-Meier method was utilized to determine the survival rate of each risk category. Propensity score matching (PSM) was used to assess survival with different therapeutic approaches. A total of 374 patients were included, and the median OS and CSS were 25 (interquartile range 21.9-28.1) months and 27 (interquartile range 23.6-30.3) months, respectively. The nomogram models demonstrated high predictive accuracy. PSM found that patients with triple-therapy had better CSS and OS than those who received surgery plus chemotherapy (median survival times: 49 vs 34 months and 49 vs 35 months, respectively). The nomogram successfully predicted the DSRCT patients survival rate. This approach could assist doctors in evaluating prognoses, identifying high-risk populations, and implementing personalized therapy.

Update of pediatric bone tumors-other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone.

There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours. We discuss other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone. We have detailed osteogenic tumors and osteoclastic giant cell-rich tumors, as well as notochordal tumors, chondrogenic tumors, and vascular tumors of the bone in separate manuscripts.

A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl)

BackgroundUndifferentiated small round cell sarcomas (URCSs) represent a diagnostic challenge, and their optimal treatment is unknown. We aimed to define the clinical characteristics, treatment, and outcome of URCS patients. MethodsURCS patients treated from 1983 to 2019 at 21 worldwide sarcoma reference centres were retrospectively identified. Based on molecular assessment, cases were classified as follows: (1) CIC-rearranged round cell sarcomas, (2) BCOR::CCNB3-rearranged round cell sarcomas, (3) unclassified URCSs. Treatment, prognostic factors and outcome were reviewed. ResultsIn total, 148 patients were identified [88/148 (60%) CIC-rearranged sarcoma (median age 32 years, range 7–78), 33/148 (22%) BCOR::CCNB3-rearranged (median age 17 years, range 5–91), and 27/148 (18%) unclassified URCSs (median age 37 years, range 4–70)]. One hundred-one (68.2%) cases presented with localised disease; 47 (31.8%) had metastases at diagnosis. Male prevalence, younger age, bone primary site, and a low rate of synchronous metastases were observed in BCOR::CCNB3-rearranged cases. Local treatment was surgery in 67/148 (45%) patients, and surgery + radiotherapy in 52/148 (35%). Chemotherapy was given to 122/148 (82%) patients. At a 42.7-month median follow-up, the 3-year overall survival (OS) was 92.2% (95% CI 71.5–98.0) in BCOR::CCNB3 patients, 39.6% (95% CI 27.7–51.3) in CIC-rearranged sarcomas, and 78.7% in unclassified URCSs (95% CI 56.1–90.6; p < 0.0001). ConclusionsThis study is the largest conducted in URCS and confirms major differences in outcomes between URCS subtypes. A full molecular assessment should be undertaken when a diagnosis of URCS is suspected. Prospective studies are needed to better define the optimal treatment strategy in each URCS subtype.

Incarcerated metastasis as the first manifestation of a rare sarcoma.

Desmoplastic small round cell tumor belongs to a group of soft tissue sarcomas. It is a rare disease, since its discovery in 1989, only hundreds of cases have been described in the literature. Due to the rare incidence of the tumor, this disease remains unknown in common medical practice. It occurs most often in men at young age. Its prognosis is serious and the average survival of patients ranges from 1.5 to 2.5 years. Treatment options include surgical resection, chemo-, radio- and targeted therapy. Our work presents a case report of a 40 year-old patient sufferred from this sarcoma. The first manifestation of the disease was an incarcerated epigastric hernia with omentum and sarcoma metastasis. A resection of the incarcerated part of the omentum was performed with a biopsy from another intra-abdominal lesion. Biopsy specimens were sent for histopathological evaluation. For the generalization of the disease, further surgery was not indicated and systemic palliative chemotherapy in the VDC-IE regimen was chosen. At the time of submitting the manuscript, the patient has survived 6 months since the surgical procedure.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignant tumors in children: Initial experience in a single institution.

Peritoneal malignant tumors in children are rare but commonly associated with disease progression and poor outcome. The successful treatment experience of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in adult peritoneal carcinoma has been applied to pediatric peritoneal malignancy in recent years. However, patients with desmoplastic small round cell tumor (DSRCT) accounted for the majority of patients treated with CRS and HIPEC in previous studies. The role of CRS and HIPEC remains controversial due to the rarity of the disease and the limited sample size of studies. Additionally, the cases using CRS and HIPEC except DSRCT were mainly small case reports with unclear outcomes. We present our experience in the treatment of pediatric peritoneal malignancies using CRS and HIPEC, with more emphasis on the safety, feasibility, and short-term outcome. A retrospective query from December 2019 to February 2022 identified 19 children with peritoneal malignancies who underwent CRS and HIPEC in our institution. Clinical characteristics, therapies, and outcomes were summarized and analyzed. The median age of the patients was 6.4 years (range, 0.7-13.9 years). The histologic types included rhabdomyosarcoma (7), Wilms tumor (2), clear cell sarcoma of the kidney (2), undifferentiated sarcoma (2), immature teratoma (1), peritoneal serous carcinoma (1), malignant rhabdoid of the kidney (1), malignant germ cell tumor (1), neuroblastoma (1), and epithelioid inflammatory myofibroblast sarcoma (1). Seven patients underwent initial operation, and 12 patients received reoperation for tumor recurrence. The median peritoneal carcinomatosis index was 5 (range, 2-21). There were no perioperative deaths or life-threatening complications of CRS and HIPEC. Two patients had grade 3 complications of wound infection and wound dehiscence. With a median follow-up time of 14 months (range, 1.5-31 months), 14 patients were alive, and 5 died of tumor recurrence. Of the 14 patients who were alive, 2 relapsed after CRS and HIPEC and then received radiotherapy and molecular-targeted therapy or chemotherapy. CRS and HIPEC are safe and feasible in children, without increasing serious complications in the peri- and postoperative periods. The complication is acceptable. The short-term outcome shows possible effectiveness in pediatric peritoneal malignant tumors. The long-term effectiveness needs to be verified by additional cases and long-term follow-ups.

Regulation of EWSR1-FLI1 Function by Post-Transcriptional and Post-Translational Modifications.

Ewing sarcoma is the second most common bone tumor in childhood and adolescence. Currently, first-line therapy includes multidrug chemotherapy with surgery and/or radiation. Although most patients initially respond to chemotherapy, recurrent tumors become treatment refractory. Pathologically, Ewing sarcoma consists of small round basophilic cells with prominent nuclei marked by expression of surface protein CD99. Genetically, Ewing sarcoma is driven by a fusion oncoprotein that results from one of a small number of chromosomal translocations composed of a FET gene and a gene encoding an ETS family transcription factor, with ~85% of tumors expressing the EWSR1::FLI1 fusion. EWSR1::FLI1 regulates transcription, splicing, genome instability and other cellular functions. Although a tumor-specific target, EWSR1::FLI1-targeted therapy has yet to be developed, largely due to insufficient understanding of EWSR1::FLI1 upstream and downstream signaling, and the challenges in targeting transcription factors with small molecules. In this review, we summarize the contemporary molecular understanding of Ewing sarcoma, and the post-transcriptional and post-translational regulatory mechanisms that control EWSR1::FLI1 function.

Epidemiological Study of Hepatopancreatobiliary Sarcoma in Iran, 2010 - 2014

Background: Hepatopancreatobiliary (HPB) malignancies are a relatively rare but important type of cancer with usually poor prognosis. Prompt diagnosis and treatment are crucial to improving the patient’s outcome. For this issue, acknowledgment of epidemiological characteristics is helpful. Objectives: In this study, the epidemiological characteristics of cases with HPB sarcoma among Iranian patients were assessed. Methods: In this epidemiological study, 133 consecutive cases with HPB sarcoma in Iran from 2010 to 2014 were evaluated, including gallbladder, pancreas, hepatic, pancreatic body and head, and other biliary tumors. Results: Nine gallbladder sarcoma cases were reported between 2010 and 2014, accounting for 6.7% of all HPB sarcoma cases, and leiomyosarcoma was the most common. A total of 16 patients of pancreatic origin were reported during this period, with the highest rate of 6 cases in 2011; in this sarcoma category, malignant type spindle cell tumor was the most common type accounting for 12.5% of all cases. Ninety-five cases of liver sarcomas were reported generally. Non-specified and desmoplastic small round cell tumors were the most common types. Five cases of pancreatic head sarcomas were reported during 2010 - 2013. There was no predominant tumor type. Five cases of pancreatic head sarcoma were reported during 2010 - 2013. There was no dominant tumor type. About the other biliary tract sites, six patients were reported. Gastrointestinal stromal sarcoma was the most common type. Conclusions: Hepatopancreatobiliary sarcoma in the Iranian population generally has diverse types, and there are different patterns according to the age and sex distribution in patients according to the initial location of the tumor and also their pathological subtypes.

Ewing’s sarcoma of the right kidney with multiple metastasis on PET/CT

BackgroundEwing’s sarcoma is categorized as small round cell aggressive tumor which commonly occurs in bone and soft tissue. Primary renal Ewing’s sarcoma is an extremely rare entity and accounts for less than 1% of renal masses. It is characterized by a very aggressive course, with very few reported cases in the literature. Primary renal Ewing’s sarcoma is indistinguishable from renal cell carcinoma on anatomical imaging and therefore need histopathological correlation for confirmation. The positron emission tomography (PET) fused with computed tomography (CT) helps in the evaluation of disease, for staging and metastatic workup.Case presentationWe present a case of 48-year-old lady who presented to urology department with difficulty in passing urine for 5 months associated with pain in the right flank region. She had an episode of haematuria 5 months back. Contrast enhanced CT showed a large lobulated heterogeneously enhancing right retroperitoneal mass in renal fossa. Histopathological examination and immunohistochemistry were suggestive of Ewing’s sarcoma. The PET/CT revealed FDG avid right renal mass with lymph nodal and skeletal metastasis.ConclusionsEwing’s sarcoma being aggressive tumour usually present with bone, liver and lung metastasis along with lymph nodal metastasis at time of diagnosis. The 18 F FDG PET/CT helps in diagnosis, staging, appropriate treatment planning, restaging, response evaluation and prognostication of Ewing’s sarcoma.

Ganglioneuronal Differentiation in Ewings Sarcoma of the Chest Wall (Askin Tumour): An Uncommon Post-Chemotherapy Change

Ewing sarcoma is classified under undifferentiated small round cell sarcomas of bone and soft tissue. Such tumours arising in the chest wall were previously called Askin’s tumour, however this terminology is not preferred at present. These tumours classically have a small round cell morphology and fusion of FET family of genes (usually EWSR1) with a member of the ETS family of transcription factors. Rare cases of Ewing sarcoma are found to have extensive neural differentiation with ganglion cells and neuropil at diagnosis as well as following chemotherapy. We report a 12 year old boy who presented with a large mass in the right hemi-thorax, which was diagnosed as Ewing sarcoma with EWSR1 translocation by FISH. However, following four cycles of chemotherapy (Vincristine, Doxorubicin, Cyclophosphamide alternating with ifosphamide and etoposide), the resection specimen showed predominantly neural differentiation mimicking a ganglioneuroma with only small areas of small round cell component. EWSR1 translocation was also demonstrated in the resection specimen. After a period of nine months, the child presented with recurrence at the same site and was treated with Irinotecan, cyclophosphamide with topotecan and temozolamide giving excellent clinical response. In this paper, we emphasize the rare occurrence of extensive neural differentiation in Ewing sarcoma and the need to revisit the upfront chemotherapy protocols for patients who exhibit this rare phenomenon

Immunohistochemical Analysis of Paediatric Small Round Blue Cell Tumours at the Tertiary Care Centre, Ludhiana, India

Immunohistochemical Analysis of Paediatric Small Round Blue Cell Tumours at the Tertiary Care Centre, Ludhiana, India

Expression of Esophageal Cancer-Related Gene 4: A Candidate Tumor Suppressor Gene Regulated by Methylation Level in Laryngeal Malignant Tumor

To clarify the expression level and methylation level of esophageal cancer related gene 4 in laryngeal malignant tumor and tumor adjacent tissues, and to explore its potential correlation with the clinicopathological characteristics of patients with laryngeal cancer is the objective of the study. The clinical samples were collected from 20 patients with primary laryngeal cancer in Xiangtan Central Hospital from December 2017 to December 2018. The level of expression and methylation of esophageal cancer related gene 4 in the samples were detected by the next-generation sequencing technology. In 15 cases, the messenger ribonucleic acid expression of esophageal cancer related gene 4 of tumor was significantly lower than that in adjacent tissues (p<0.05). In 18 cases, the methylation level of esophageal cancer related gene 4 in tissue of laryngeal squamous carcinoma was 46.7 %, which was significantly higher than that in adjacent tissues (26.2 %, p<0.01). Combined with clinical data analysis, it was found that, in laryngeal high and middle differentiated squamous cell carcinoma, the expression level of esophageal cancer related gene 4 was low and the methylation level was significantly increased. However, the expression of esophageal cancer related gene 4 in small round cell carcinoma was high, while the methylation level was significantly reduced. Smoking and drinking habits are the related factors of laryngeal high and middle differentiated squamous cell carcinoma. These results indicated significant difference of the esophageal cancer related gene 4 expression and methylation level between laryngeal malignant tumor and tumor adjacent tissues. Furthermore, the correlation between esophageal cancer related gene 4 expression and methylation level in laryngeal malignant cancer was found. Esophageal cancer related gene 4 could be regarded as a potential therapeutic target gene for laryngeal malignant cancer.

The sun beside the pancreatic neck: The lymph node gastrinoma (with video).

The sun beside the pancreatic neck: The lymph node gastrinoma (with video).

Correlation NKX2.2 IHC and EWSR1 break-apart FISH in the diagnosis of Ewing sarcoma: Can combined NKX2.2 and CD99 immunoexpression obviate or minimize the need of FISH testing? First assessment study from Indian tertiary cancer care center.

Ewing sarcoma (ES) are malignant small round cell tumors (MSRCT) characterized by rearrangements of EWSR1 gene. Although gold standard for diagnosis is detection of specific fusion genes by molecular testing, these ancillary tests are costly and only available in limited number of settings. There is a persuasive evidence for reliability of NKX2.2 immunohistochemistry (IHC) as a surrogate marker for EWSR1 gene rearrangement in ES. The aim of this study is to correlate the NKX2.2 immuno-expression with genetically confirmed ES cases and also to assess the reliability and accuracy of NKX2.2 along with combined positivity of NXX2.2 and CD99 in diagnosing ES and differentiating it from other relevant histological mimics. The present study is a retrospective study conducted over a period of 6-year duration in a tertiary cancer care center. We evaluated NKX2.2 immunoexpression in 35 genetically confirmed cases of ES and also in pertaining differential entities (n = 58) of ES including rhabdomyosarcoma (n = 20), lymphoblastic lymphoma (n = 14), Wilms tumor (n = 10), poorly differentiated synovial sarcoma (n = 4), small-cell osteosarcoma (n = 4), neuroblastoma (n = 5), and mesenchymal chondrosarcoma (n = 1). CD99 was performed in the category of MSRCTs showing NKX2.2 positivity to evaluate combined specificity for the diagnosis of ES. Of the 35 genetically confirmed cases of ES, 29 cases (83%) showed NKX2.2-positive expression (83% sensitivity). Compared to ES, NKX2.2 was positive in only 05% cases (3/58 cases) of non-ES MSRCT. Only two of five cases of neuroblastomas and one case of mesenchymal chondrosarcoma showed NKX2.2 positivity. CD99 positivity was seen in 100% of ES and in the single case of mesenchymal chondrosarcoma. All five cases (100%) of neuroblastoma were negative for CD99. The presented study, which is the first from an Indian oncology center, showed NKX2.2 IHC is quite reliable in diagnosis of ES in the right clinicopathological context. With remarkable sensitivity and specificity of NKX2.2 IHC for diagnosis of ES, we propose that combined positivity of CD99 and NKX2.2 IHC can obviate or minimize the need of EWSR1 gene rearrangement molecular testing for diagnosis of ES.

Primary Cutaneous Diffuse Large B Cell Lymphoma, Leg Type: A Rare Neoplasm Masquerading as Squamous Cell Carcinoma

Primary Cutaneous Diffuse Large B Cell Lymphoma, Leg Type (PCDLBCL, LT) comprises 4% of all cutaneous lymphomas, and it presents as an aggressive form of lymphoma. Rarely do pathologist/cytologists find specimens of PCDLBCL, LT on Fine Needle Aspiration Cytology (FNAC). The mimickers on FNAC are metastatic small cell carcinoma, Merkel Cell Carcinoma (MCC), Malignant Melanoma (MM), Cutaneous Alveolar Rhabdomyosarcoma and Primary Cutaneous Ewing's Sarcoma (PCES). A 75-year-old female patient presented in the Department of Surgery with multiple nodules on the leg for three months. On cytology small round cell tumour with possibility of non hodgkin lymphoma was given and biopsy was advised. The confirmation of PCDLBCL, LT on cytology is not recommended however, it is emphasised that it can be picked up on FNAC and distinguished from its mimickers. Because of the aggressive nature and poor prognosis with the frequent relapses and tendency for extracutaneous spread, it is considered to be a distinct type of cutaneous lymphoma which needs to be diagnosed and treated as early as possible.

Transthoracic Fine-Needle Aspiration Cytology of Pulmonary Spindle and Mesenchymal Neoplasms: A Pandora’s Box

Introduction: Pulmonary spindle cell and mesenchymal lesions are paradox for pathologists due to their rarity, overlapping morphology, and differentials ranging from benign to malignant lesions, and correct diagnosis is essential due to major treatment implications. This study highlights the role of fine-needle aspiration cytology, clot core biopsy, and immunohistochemistry in diagnosis of spindle cell lesions in lung, thus playing a key role in patient management. Methods: It is a retrospective study of lung FNA with predominantly spindle and mesenchymal cells from 2015–2020 which were classified cytomorphologically into spindle, epithelioid, small round cell, and biphasic, and IHC panels are applied accordingly. FNA from mediastinum and chest wall was excluded. Results: 60 cases of lung FNA with spindle and mesenchymal cells were identified and included 6 benign and 54 malignancies which included 24 primary pulmonary malignancies and 30 metastases. Most common primary malignancy was sarcomatoid carcinoma, and most common metastasis was malignant peripheral nerve sheath tumour. FNA was paucicellular in 7 cases and was reported as benign in 7 cases and malignant in 46 cases. There were two false-negative cases. One case of pulmonary blastoma was reported as inflammatory pseudotumour on cytology, and other case of chondrosarcoma was reported as chondroid tumour. Sensitivity and specificity of FNA in distinguishing benign lesions and malignancies were 93.8% and 100%, respectively. Conclusion: FNA along with clot core biopsy/cell block and IHC plays a pivotal role in the subsequent pathway taken for diagnostic or therapeutic management of these patients without the need for second sampling or trucut biopsies in a low resource setting.

Cytoreductive surgery and hyperthermic chemoperfusion of the pleural cavity in a child with desmoplastic small round cell tumor. The first Russian experience

Desmoplastic small round cell tumor is a rare and aggressive malignant tumor. The abdominal cavity is the most frequent site of this tumor, but rarely it affects the pleural cavity. This article presents a clinical case of a 10-year-old male with a desmoplastic small round cell tumor of the pleural cavity. Here we describe the first experience of hyperthermic intrapleural chemoperfusion in a pediatric patient in Russia. The patients' parents gave their consent to the use of their children's data, including photographs, for research purposes and in publications.

Patterns of care and outcome of CIC-rearranged sarcoma patients: A nationwide study of the French sarcoma group.

CIC-rearranged sarcomas (CIC-RS) represent the most frequent subset of "Ewing-like" undifferentiated small round cell sarcomas. These tumors tend to be more aggressive than Ewing sarcomas. Moreover, treatment strategy can differ according to teams. The primary aim of this retrospective study was to describe the characteristics, treatments, and outcome for patients with CIC-RS included in the French NETSARC+ database. Pediatric and adult patients from 13 French centers with a diagnosis of CIC-RS were registered from October 2008 to March 2021. Patients and tumors characteristics were collected from the national network NETSARC+ database (http://netsarc.sarcomabcb.org). CIC-RS diagnosis was pathologically and molecularly confirmed with a central review by expert pathologists. Two groups of patients were studied: those treated as classical Ewing sarcomas (cohort EwS) and those treated as high-grade soft tissue sarcomas (cohort STS) according to ESMO and/or EpSSG guidelines. Survival was calculated using the Kaplan-Meier method and the log-rank test was used to compare survival. Among 79 patients, the male/female sex ratio was 0.7 and the median age at diagnosis was 27 years (range 2-87). With a median follow-up of 37 months, 39 patients died of the disease. Median overall survival from diagnosis was 18 months, with no significant difference between both cohorts (p=0.9). Nevertheless, when focusing on patients with metastatic disease at diagnosis (N=21), all patients from cohort STS died of disease while some patients from cohort EwS were still alive and in complete remission. FSG experience confirms the aggressive clinical course of CDS patients regardless of chemotherapy regimen.

Ewing’s sarcoma of the mandible- A case report

Ewing’s sarcoma is a rare aggressive variant of small round cell tumors and is an uncommon malignancy that occurs usually in childhood. It constitutes 10–15% of all primary malignant tumors and represents the second most common malignant bone tumor occurring in children and young adults after osteosarcoma - with an estimated incidence of 2.93 cases/million inhabitants under 20 years of age/year. This paper presents a case of 10-year-old male child who reported with a rapidly progressing swelling in the right mandibular posterior ramus region. The patient was diagnosed with Ewing’s sarcoma on the basis of history, clinical, radiological and confirmed with histopathological &amp; immunohistochemical examination. Since the exact diagnosis is hard to achieve before biopsy, the condition poses a difficult diagnostic dilemma for the clinician.

Geometrical analysis identified morphological features of hydrogel-induced cancer stem cells in synovial sarcoma model cells

Cancer stem cells (CSCs) has been a key target to cure cancer patients completely. Although many CSC markers have been identified, they are frequently cancer type-specific and those expressions are occasionally variable, which becomes an obstacle to elucidate the characteristics of the CSCs. Here we scrutinized the relationship between stemness elevation and geometrical features of single cells. The PAMPS hydrogel was utilized to create the CSCs from mouse myoblast C2C12 and its synovial sarcoma model cells. qRT-PCR analysis confirmed the significant increase in expression levels of Sox2, Nanog, and Oct3/4 on the PAMPS gel, which was higher in the synovial sarcoma model cells. Of note, the morphological heterogeneity was appeared on the PAMPS gel, mainly including flat spreading, elongated spindle, and small round cells, and the Sox2 expression was highest in the small round cells. To examine the role of morphological differences in the elevation of stemness, over 6,400 cells were segmented along with the Sox2 intensity, and 12 geometrical features were extracted at single cell level. A nonlinear mapping of the geometrical features by using uniform manifold approximation and projection (UMAP) clearly revealed the existence of relationship between morphological differences and the stemness elevation, especially for C2C12 and its synovial sarcoma model on the PAMPS gel in which the small round cells possess relatively high Sox2 expression on the PAMPS gel, which supports the strong relationship between morphological changes and the stemness elevation. Taken together, these geometrical features can be useful for morphological profiling of CSCs to classify and distinguish them for understanding of their role in disease progression and drug discovery.

Clinicopathologic Features of Neuroblastoma-like Schwannoma: A Case Report of Unusual Morphologic Variant

Neuroblastoma-like schwannoma is known as a rare unusual variant of schwannoma with difficulties of differential diagnosis with neuroblastoma, Ewing sarcoma/peripheral neuroectodermal tumor and other cutaneous small round cell tumors. Herein, we describe a neuroblastoma-like schwannoma that was presented as a painless lesion on the dorsal side of the left hand in a 39-year-old woman. Composed collagen fibers in the central core of rosettes and diffuse expression of S100 protein in the tumor cells found in the biopsy specimens confirmed the Schwann cell origin of the tumor.