Diagnosis Of Sarcoma Research Articles

Synovial sarcoma: the misdiagnosed sarcoma.

Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. The management of synovial sarcomas is challenging as they are rare and have a poor prognosis. Early and correct diagnosis and treatment are critical for clinical outcomes. Misdiagnosis or delayed diagnosis can have devastating consequences for the patient. The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas. Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded. Surgery is the mainstay of treatment for synovial sarcomas. Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (>5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases. This article reviews synovial sarcomas from the perspectives of clinical and radiological presentation, histological and cytogenetic analysis, differential diagnosis, treatment, and prognosis.

Histiocytic Sarcoma

Histiocytic sarcoma is a rare and frequently missed diagnosis. With unusual and varied presentations, it typically indicates a rapid patient decline and poor outcomes. The diagnosis requires a high degree of clinical suspicion. In this case, we explore the progression of illness in a 56-year-old white male who initially presented with a 2-week history of soft-tissue nodules scattered throughout his head, neck, torso, and limbs, as well as shortness of breath and knee pain. After a thorough workup including computed tomography imaging of the neck, chest, and lower extremity, as well as a biopsy of a nodule with immunoperoxidase staining, a diagnosis of histiocytic sarcoma was established. Due to the severity of his malignancy, his hospital course was complicated by a deep vein thrombosis. Clinicians should be mindful of the risk of acute decompensation in such cases and can employ the various tenets of osteopathic theory to improve patient quality of life. Depending on the severity of illness, physicians may proceed to facilitate end-of-life measures with grace and dignity.

Machine Learning–Supported Diagnosis of Small Blue Round Cell Sarcomas Using Targeted RNA Sequencing

Small blue round cell sarcomas (SBRCSs) are a heterogeneous group of tumors with overlapping morphologic features but markedly varying prognosis. They are characterized by distinct chromosomal alterations, particularly rearrangements leading to gene fusions, whose detection currently represents the most reliable diagnostic marker. Ewing sarcomas (ESs) are the most common SBRCSs, defined by gene fusions involving EWSR1 and transcription factors of the ETS gene family, while the most frequent non-EWSR1-rearranged SBRCSs harbor a CIC rearrangement. Unfortunately, the identification of CIC::DUX4 translocation events, the most common CIC rearrangement, is challenging with current methods. Here, a machine-learning approach to support SBRCS diagnosis relying on gene expression profiles measured via targeted sequencing is presented. The analyses on a curated cohort of 69 soft tissue tumors (STT) showed markedly distinct expression patterns for SBRCS subgroups. A random forest (RF) classifier trained on ES and CIC-rearranged cases predicted probabilities of being CIC-rearranged > 0.9 for CIC-rearranged-like sarcomas and < 0.6 for other SBRCS. Testing on a retrospective cohort of 1335 routine diagnostic cases identified 15 candidate CIC-rearranged tumors with a probability > 0.75, all of which supported by expert histopathological reassessment. Furthermore, the multi-gene RF classifier appeared advantageous over using high ETV4 expression alone, previously proposed as surrogate to identify CIC rearrangement. Taken together, the expression-based classifier can offer valuable support for SBRCS pathological diagnosis.

Recent Advances in the Diagnosis, Pathogenesis, and Management of Myxoinflammatory Fibroblastic Sarcoma.

Myxoinflammatory fibroblastic sarcoma (MIFS) is an infiltrative, locally aggressive fibroblastic neoplasm of intermediate malignancy that typically arises in the distal extremities of middle-aged adults. It can histologically be confused with a number of benign and malignant conditions. Recently, high-grade examples of MIFS have been described. Immunohistochemistry plays a very limited role in the diagnosis of MIFS. Several genetic alterations have been identified in MIFS, including a t(1;10)(p22;q24) translocation with TGFBR3 and/or OGA rearrangements, BRAF rearrangement, and VGLL3 amplification. Although it appears that VGLL3 amplification is the most consistent alteration, the molecular pathogenesis of MIFS remains poorly understood. A wide resection is considered the standard treatment for MIFS. Radiotherapy may be a viable option in cases with inadequate surgical margins or cases where surgery is likely to cause significant functional impairment. The systemic treatment options for advanced or metastatic disease are very limited. This review provides an updated overview of the clinicoradiological features, pathogenesis, histopathology, and treatment of MIFS.

Primary intradural extraosseous Ewing's sarcoma of the L3 nerve root: illustrative case.

Ewing's sarcoma is an uncommon, aggressive malignancy that typically presents as an osseous lesion, most commonly in children and adolescents. Very rarely Ewing's sarcoma can present as an intradural extramedullary mass mimicking more common tumors. A 32-year-old female had a left L3 nerve root-associated lesion identified in the setting of recent-onset radiculopathy. Contrast-enhanced magnetic resonance imaging of the lumbar spine was favored to demonstrate a schwannoma or neurofibroma. Hemilaminectomy, facetectomy, and resection of the mass led to improved radiculopathy and a tissue diagnosis of Ewing's sarcoma. Immediate referral to medical oncology facilitated expeditious initiation of adjuvant chemotherapy and radiation. The differential diagnosis for newly identified nerve root-associated tumors should remain broad, including common benign pathologies and rare malignant entities. Tissue remains the gold standard for diagnosis, as preoperative imaging suggested a nerve sheath tumor. Malignant pathologies such as Ewing's sarcoma must be considered, especially in the setting of rapidly progressive symptoms or interval growth on serial imaging. Early diagnosis allows for the timely initiation of comprehensive oncological care. Long-term multidisciplinary follow-up is necessary for the surveillance of disease progression.

Use of fine-needle puncture under endosonographic control in the diagnosis of left atrium sarcoma – case report

Background. Over the past 30 years, with the development of endoscopic technologies and the advent endoscopic ultrasound-guided fine-needle aspiration (EuS-FnA) it has become possible to diagnose with preliminary verification of cardiac tumors, which in turn is an undoubted advantage in detecting cardiac pathology. A rare clinical case is presented: the detection of a tumor of the left atrium using high-tech endoscopic techniques, in particular a EuS-FnA. when studying the surgical material using immunohistochemistry and FISH hybridization, the final diagnosis sounded like intimal sarcoma. Diagnosis of intimal sarcoma is a complex clinical task, and difficulties in making a diagnosis delay the duration of treatment. Our experience demonstrates the modern role of endoscopy as a minimally invasive method of diagnosing intracardiac tumors.

Predicting prognosis and immune status in sarcomas by identifying necroptosis-related lncRNAs.

Sarcomas are a type of highly heterogeneous malignant tumors originating from mesenchymal tissues. Necroptosis is intricately connected to the oncogenesis and progression of tumors. The main goal of this research is to assess the prognostic value of necroptosis-related lncRNAs (NRlncRNAs) in sarcomas and to develop a risk model based on NRlncRNAs to evaluate prognostic and immune status of the sarcomas. We screened NRlncRNAs using the gene co-expression network, developed a prognostic risk model of sarcomas, and then verified the model. Following that, various bioinformatics analysis algorithms were employed to analyze the distinct characteristics of patients of the risk model. Furthermore, the function and regulatory mechanism of NRlncRNA SNHG6 in sarcomas were investigated through osteosarcoma cell experiments, such as qRT-PCR, Western blot, CCK-8, clone formation, and transwell assay. We successfully developed a NRlncRNAs-related prognostic risk model and screened 5 prognosis-related NRlncRNAs, with SNGH6 being the most significant for prognosis of patients. According to results, the significant differences exist in prognosis, clinical characteristics, and tumor immune status among patients of the risk model. The experiments of osteosarcoma cells demonstrated that NRlncRNA SNHG6 knockdown significantly attenuated the cells' proliferation, migration, and invasion. qRT-PCR and WB results showed that SNHG6 regulated AXL and AKT signaling. We have developed an innovative investigation on NRlncRNAs, which can serve as a reference for diagnosis, therapy, and prognosis of sarcomas. Additionally, we demonstrated that NRlncRNA SNHG6 regulated AXL and AKT signaling in osteosarcoma cells and the proliferation, migration, and invasion of tumor cells.

The association between ambient air pollution exposure and connective tissue sarcoma risk: a nested case-control study using a nationwide population-based database.

A nationwide population-based database was utilized in a nested case-control study to explore the association between ambient air pollution exposure and the likelihood of developing connective tissue sarcoma. The study examined 280 cases of connective tissue sarcoma diagnosed between 2000 and 2012. A random sample of 1120 control subjects was selected from a subpopulation of claim records without a connective tissue sarcoma diagnosis in a 1:4 ratio. The control subjects were selected based on similar characteristics as the connective tissue sarcoma patients, including gender, birth year, and the year of diagnosis of the case group with medical records. Risk factors for connective tissue sarcoma were collected for analysis. Our data on exposure to air pollutants was collected from Taiwan's Air Quality Monitoring Network, which has been gathering air quality data from a growing network of sampling stations (now 76) throughout the country since 1997. It was discovered that the risk of connective tissue sarcoma was significantly increased by the Charlson comorbidity index (CCI), elevated levels of specific air pollution indices (e.g., total hydrocarbons (THC), fine particulate matter (PM2.5), and O3_8 (the annual mean of the daily maximum 8-h average concentration of O3), the High Pollutant Standards Index (hPSI) (the percentage of days in a given year in Taiwan where the PSI exceeds 100), and an insurable monthly wage over US$1100. Further investigation is needed to explore the involvement of these air pollutants in the formation of connective tissue sarcoma.

The utility of MRI for the preoperative differential diagnosis of uterine sarcoma and leiomyoma: a single-center study.

Although uterine sarcoma is a rare disease, its prognosis is extremely poor;thus, it is important to differentiate it from uterine leiomyoma. In this retrospective study, we examined the association between preoperative MRI findings and postoperative pathology results in 170 patients with uterine tumors who underwent preoperative MRI examination at Fukushima Red Cross Hospital. In 4 cases of sarcoma / smooth muscle tumor of unknown malignant potential (STUMP), abnormal findings were found at a high frequency with T1-weighted imaging (T1WI) (75%), T2-weighted imaging (T2WI), diffusion-weighted imaging (DWI), and contrast enhancement (CE) (100%). In cases of ordinary leiomyoma, on the other hand, abnormal findings were less frequent. The rates of high DWI signal intensity for degenerated and cellular leiomyoma were 31% and 64%, respectively, and the CE-positive rates were 31% and 57%, respectively. Apparent Diffusion Coefficient (ADC) values appeared to be useful in differentiating degenerated leiomyoma from sarcoma. The relatively characteristic findings of uterine sarcoma on MRI images may overlap with those of degenerated leiomyoma and cellular leiomyoma, making it difficult to diagnose sarcoma on imaging alone. However, findings that distinguish sarcoma from ordinary, degenerated, and cellular leiomyoma cases are worthy of attention, to avoid overlooking sarcoma.

The automatic diagnosis artificial intelligence system for preoperative magnetic resonance imaging of uterine sarcoma.

Magnetic resonance imaging (MRI) is efficient for the diagnosis of preoperative uterine sarcoma; however, misdiagnoses may occur. In this study, we developed a new artificial intelligence (AI) system to overcome the limitations of requiring specialists to manually process datasets and a large amount of computer resources. The AI system comprises a tumor image filter, which extracts MRI slices containing tumors, and sarcoma evaluator, which diagnoses uterine sarcomas. We used 15 types of MRI patient sequences to train deep neural network (DNN) models used by tumor filter and sarcoma evaluator with 8 cross-validation sets. We implemented tumor filter and sarcoma evaluator using ensemble prediction technique with 9 DNN models. Ten tumor filters and sarcoma evaluator sets were developed to evaluate fluctuation accuracy. Finally, AutoDiag-AI was used to evaluate the new validation dataset, including 8 cases of sarcomas and 24 leiomyomas. Tumor image filter and sarcoma evaluator accuracies were 92.68% and 90.50%, respectively. AutoDiag-AI with the original dataset accuracy was 89.32%, with 90.47% sensitivity and 88.95% specificity, whereas AutoDiag-AI with the new validation dataset accuracy was 92.44%, with 92.25% sensitivity and 92.50% specificity. Our newly established AI system automatically extracts tumor sites from MRI images and diagnoses them as uterine sarcomas without human intervention. Its accuracy is comparable to that of a radiologist. With further validation, the system could be applied for diagnosis of other diseases. Further improvement of the system's accuracy may enable its clinical application in the future.

Long-term survival of a cat with a metastatic gastrointestinal stromal tumor

Case summary An 8-year-old spayed female mixed-breed cat presented with a 2-year history of recurrent vomiting and hyporexia. Physical examination revealed a palpable mass in the mesogastric abdominal region without discomfort upon touch. Abdominal ultrasonography revealed an intramural mass in the small intestine (duodenum) that caused a decrease in the segment lumen. A complete blood count revealed leukocytosis with marked neutrophilia, eosinophilia and mild hypoalbuminemia. Enterectomy was performed with 3 cm margins and end-to-end anastomosis at the duodenal site of the mass. Histology revealed neoplastic elongated spindle cells that were poorly confined, originating within the muscle layers and that had a mitotic index of 2/10 high-power field (hpf) (at 400×), human epidermal growth factor receptor-type 2 (HER-2) supporting the diagnosis of low-grade sarcoma. Immunohistochemical analysis was positive for KIT, confirming a gastrointestinal stromal tumor (GIST) with a Ki67 level of 15%. Furthermore, multikinase profile biomarkers revealed that the neoplastic cells expressed HER-2 (65%), epidermal growth factor receptor-1 (50%), vascular endothelial growth factor receptor 2 (35%), platelet-derived growth factor receptor beta (15%) and c-KIT (15%). Six months after the original surgery, CT revealed presumptive hepatic, splenic and peritoneal metastases. Toceranib phosphate was prescribed at a dose of 2.75 mg/kg and progressive disease was observed at 8 weeks of follow-up. Relevance and novel information To the best of our knowledge, this is the first case report to characterize the proliferation biomarker profile of a feline GIST in veterinary oncology. However, despite KIT expression in this tumor, the target drug did not inhibit tumor proliferation, providing new insights into this rare tumor in the feline species.

Undifferentiated Embryonal Sarcoma of Liver- A Rare Case Entity

Undifferentiated Embryonal Sarcoma of the Liver (UESL) is a rare hepatic malignant mesenchymal neoplasm in the paediatric population, typically affecting children between 6-10 years of age without sex predilection. It accounts for 9-15% of paediatric liver malignancies, with an incidence of 0.6-1.2 cases per one million patients. Here, the authors present a case of an 11-year-old male, who presented with right upper quadrant pain, loss of appetite for the past one month, and high-grade fever for the past three days. On examination, a firm mass in the right hypochondrium and epigastrium, about 4 cm below the right costal margin, was noted, moving with respiration. Blood work showed normal values and normal alpha-fetoprotein levels. An ultrasonogram revealed a cystic lesion, while a contrast-enhanced Computed Tomography (CT) scan showed a large hepatic space-occupying lesion with cystic and solid architecture, with the following differential diagnosis as, embryonal sarcoma and hepatoblastoma. The patient underwent surgery for a hepatic hydatid cyst, and the procedure performed was percutaneous aspiration, irrigation and respiration. Gross examination revealed multiple grey-brown and grey-white fragments with a variegated appearance. Microscopic analysis showed a malignant neoplasm composed of spindle cells, stellate cells, multinucleated giant cells in a myxoid stroma, with many atypical mitoses present. Periodic Acid-Schiff (PAS) positive eosinophilic hyaline globules were observed in the tumour cell cytoplasm, along with extensive areas of haemorrhage and necrosis. The final diagnosis of embryonal sarcoma was made after a panel of Immunohistochemistry (IHC) markers. The present case is presented here due to its rarity and diagnostic challenge, arising from the lack of a characteristic clinical presentation, serological markers and inconclusive radiological findings.

Soft tissue sarcoma diagnosis using machine and deep learning-survey

The collection of unique and diverse tumors known as soft-tissue sarcomas (STS) is hindered by a number of factors, such as delayed or inaccurate diagnosis, and a lack of clinical knowledge, and a restricted range of treatment alternatives. The tissues that surround, link, and support other body organs and structures are the target of a rare type of cancer known as soft tissue sarcomas. Muscle, fat, blood vessels, deep skin tissues, tendons, and ligaments are among the tissues that can be impacted by soft tissue sarcomas. Soft tissue sarcomas can arise in nearly every body component, including the arms, legs, and abdomen. The way that patients are treated medically is severely harmed by these diagnostic mistakes. Numerous machine learning models have been proposed by researchers to categorize cancers, but none of them have sufficiently addressed the issue of misdiagnosis. Furthermore, the majority of comparable research that has suggested models for the assessment of these malignancies do not take the heterogeneity and volume of the data into account. This research presents the comparison between machine and deep learning methods for the improved categorization of soft tissue sarcomas. This research further proposes on the early detection of STS. In the next stage of classification, an optimal Convolution Neural Network (CNN) is employed.

Orbital pleomorphic sarcoma: A case report

Aims/Purpose: To report a case involving an undifferentiated pleomorphic sarcoma localized in the orbit.Methods: Case report and literature review.Results: Undifferentiated pleomorphic sarcomas (UPS) are one of the least common types within all malignant sarcomas, with their incidence reaching 5–10% of the cases. Within this group of UPS, tumours involving the head and neck have an even lower incidence (approximately 3 to 5%). We report the case of an 86 year old male with a history of cardiovascular disease, presenting with a painless lesion in his right eye. The orbit CT reported a soft tissue lesion in contact with the right ocular globe, lacrimal gland and extraconal fat. At examination the patient registered best corrected visual acuity right eye (OD) no light perception right eye and left eye (OS) 20/50. At slit lamp examination OD is not evaluable due to corneal compromise. OS anterior segment and posterior pole with no alterations. Immunohistochemistry was negative for P40, desmine, CD34, SOX‐10 and cytokeratin cocktail. S100 had weak and patchy staining. These results led to a giant cell pleomorphic sarcoma diagnosis, histological grade 3. The patient underwent urgent orbital exenteration, with an adequate postsurgical evolution and oncology follow up.Conclusions: The diagnosis of an UPS is based on exclusion, due to their heterogeneous nature, with histological characteristics that overlap with other malignant mesenchymal neoplasms, mainly with metastatic sarcomatoid carcinoma and melanoma. These tumours are characterized by their rapid growth and potential for metastasis, especially in the lungs. Treatment includes surgical resection, sometimes combined with adjuvant chemotherapy. Strict periodic checkups are essential, since local recurrence is frequent (between 19 to 31%), with most cases occurring within the first 24 months.

An analysis of the effectiveness of differential diagnosis and surgical treatment of neck soft-tissue sarcomas in children

We conducted a retrospective sample study with prospective collection of follow-up data. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. In the time period from January 2013 to August 2020 (92 months), 126 patients with head and neck soft-tissue sarcomas (STS) received treatment at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. We included 25 patients who had undergone surgery for neck STS and divided them into 4 groups (rhabdomyosarcoma (RMS), non-RMS-like STS, RMS-like STS, IRS-IV STS – with distant metastasis at baseline). The median age at the time of correct diagnosis was 2.6 (0.5; 5.0). The median time from symptom onset to the verification of the correct pathomorphological diagnosis was 3.2 (1.6; 4.9) months. We discovered a significant number (13/25, 52%) of cases of biopsy that was performed improperly (excessive/non-diagnostic biopsy, fine-needle aspiration biopsy) at a general inpatient facility. The correct pathomorphological diagnosis was clinically and statistically much more often made at a reference center (20/25, 80%; p = 0.003). Moreover, more than half of pathomorphological diagnoses (8/13, 62%) made at a general inpatient facility were later changed at a reference center. Radical resection was achieved in 17/20 (85%) survivors. In 3/20 (15%) cases, a repeat surgery was not needed because of the patients' complete response to protocol-based treatment. Radiotherapy was carried out in 11/25 (44%) cases. Protocol-based treatment was completed in 19/25 (76%) patients, 18/25 (72%) patients achieved complete response, 2/25 (8%) patients were considered incurable, and 4/25 (16%) children died before the completion of therapy. Post-operative complications of varying severity were observed in 10/25 (40%) cases and were dependent on the degree of STS extension and the severity of the condition of the patients undergoing intensive protocol-based treatment. The median time of patient observation since diagnosis verification was 33.2 (15.6; 74.2) months. The five-year overall survival (OS) was 76.3% (95% confidence interval (CI) 51.8; 89.5), the five-year event-free survival without local disease progression – 73.9% (95% CI 41.8–90.1). Even though there weren't many patients with IRS-IV in our study (4/25, 16%), their exclusion from the analysis resulted in a higher 5-year OS rate: 88.2% (95% CI 60.2; 96.9). This study revealed significant problems in the differential diagnosis of neck STSs in children. In most patients receiving optimal protocol-based treatment, neck tumors can be radically removed at a specialized healthcare facility without mutilating surgery, which results in high 5-year OS and event-free survival rates in patients without distant metastasis. Our findings require further investigation in a larger sample of patients.

The diagnostic roles of fused &lt;sup&gt;18&lt;/sup&gt;F-fluorodeoxyglucose positron emission tomography/computed tomography and bone scintigraphy in children and young adults with bone sarcomas: a systematic review and meta-analysis

Osteosarcoma and Ewing sarcoma are the most common primary malignant bone diseases in children. An accurate diagnosis and staging of these tumors play a pivotal role in choosing the optimal treatment and predicting outcomes. In recent years, fused 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) has been increasingly used in the diagnosis of bone sarcomas. It is frequently applied in conjunction with, or as a replacement for bone scintigraphy (BS), in order to determine the extent of the disease. However, the questions on the diagnostic significance of these methods and the choice of the most effective approach to the management of children with bone sarcomas still remain unanswered. We conducted a systematic review and meta-analysis to compare the diagnostic roles of 18F-FDG PET/CT and BS in staging and restaging of bone sarcomas in children and young adults. The study was carried out in accordance with the Cochrane PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Two independent researchers looked for prospective and retrospective studies evaluating the sensitivity and specificity of 18F-FDG PET/CT and BS in staging and restaging of bone sarcomas in children and young adults, published over the last 15 years. The quality of the included studies was assessed using the QUADAS-2 tool. Summary Receiver Operating Characteristic curves were calculated using STATA 17 software packages and the RevMan 5.3 tool to evaluate the overall diagnostic value of PET/CT and BS. The certainty of evidence was evaluated using the GRADE system. This systematic review and meta-analysis included 8 studies (530 patients with bone sarcomas). These studies used 11 patient cohorts (osteosarcoma: 5 cohorts, 305 patients; Ewing sarcoma: 6 cohorts, 225 patients). We discovered that 18F-FDG PET/CT had high sensitivity in staging and restaging of bone sarcomas (94% (95% confidence interval (CI) 89–97)). On the other hand, BS demonstrated lower sensitivity (69% (95% CI 58–79), the mean difference being 25% (95% CI 18.89–31.00), p &lt; 0.001). At the same time, the specificity of 18F-FDG PET/CT and the specificity of BS were found to be comparable (96% (95% CI 83–99) and 92% (95% CI 82–97) respectively, p = 0.15). All the results were confirmed in a subgroup analysis of patients with osteosarcoma and Ewing sarcoma. The results of our systematic review and meta-analysis lead us to conclude that 18F-FDG PET/CT is a more sensitive method for staging and restaging of bone sarcomas in children and young adults, compared to BS. However, both methods have high specificity. Considering our findings, future clinical research in children with bone sarcomas should be aimed at further data collection to clarify the diagnostic roles of 18F-FDG PET/CT and BS both in children with osteosarcoma and in children with Ewing sarcoma, in order to identify clear indications and choose the best imaging method for detecting metastatic bone lesions, with the aim of developing an optimal diagnostic strategy.

Ovarian myeloid sarcoma with concurrent acute myeloid leukemia- A case report

Myeloid sarcoma is a rare extramedullary tumor of immature myeloid cells that can be composed of cells showing granulocytic or monocytic differentiation. It is frequently detected as masses in subcutaneous soft tissue, bone, skin, GIT, reproductive organs, CNS, heart, lungs, kidney and breast. We report a pediatric case of myeloid sarcoma presenting with bilateral ovarian masses.A 12 years old female presented with complaints of fever, lethargy, decreased appetite and easy fatiguability. Patient further developed swelling and pain in large joints along with slurring of speech and difficulty in swallowing. Examination revealed polyserositis along with hepatosplenomegaly. ANA IFA profile was positive. CECT abdomen showed heterogeneous lesions in bilateral adnexae along with a few enlarged matted lymph nodes in left para-aortic region (?Neoplastic/ ?Histiocytosis/ ?EM hematopoiesis). GBP was suggestive of leucoerythroblastic reaction. BM study with flowcytometry was suggestive of Acute myeloid leukemia with monocytic differentiation. Biopsy from both adnexae showed features consistent with poorly differentiated malignancy, favour haematolymphoid neoplasm. Immunophenotyping by IHC showed the cells to be positive for CD45 (dim), MPO &amp; CD117, thus establishing diagnosis of Myeloid sarcoma, both ovaries.Myeloid sarcoma is uncommon ovarian neoplasm and the clinical presentation may be with or without acute leukemia / MPN. Immunohistochemistry and ancillary studies are necessary to recognize this entity.

Ewing's sarcoma of central nervous system: a clinicopathological analysis of six cases

Objective: To investigate the clinicopathological characteristics, pathological diagnosis of Ewing's sarcoma of the central nervous system. Methods: Six cases of Ewing's sarcoma of the central nervous system diagnosed at the First Affiliated Hospital of Nanjing Medical University, Nanjing, China from 2015 to 2022 were collected. The clinical manifestations, histological morphology, immunophenotype and molecular genetics of these cases were analyzed. The related literature was reviewed. Results: There were four males and two females, with a male to female ratio of 2∶1. The onset age was 17-40 years, with a median age of 23 years. All 6 tumors were located in the spinal cord (2 cases of cervical vertebra, 1 case of thoracic vertebra, 2 cases of lumbar vertebra, and 1 case of sacral vertebra). The patients' clinical manifestations were mostly lumbago, weakness and numbness of lower limbs/limbs. In 1 case, the tumor recurred and metastasized to the suprasellar region and the third ventricle. Microscopically, the tumor showed diffuse infiltrative growth. In some cases, the tumor was closely related to the spinal meninges. The tumor cells were arranged in sheet, lobular, thin-rope, and nest-like patterns. Homer-Wright rosette was visible. The tumor cells were small to medium in size, and most of them had scant cytoplasm. A few cells had clear cytoplasm. Some areas were rhabdoid. The tumor cell nuclei showed focal mild pleomorphism. The chromatin was uniform and delicate while the nucleoli were not obvious. Mitosis was commonly seen. The tumor was separated by fibrous connective tissue and may be accompanied by mucinous degeneration. Immunohistochemistry showed that all tumors were positive for CD99, NKX2.2, Fli1, ERG. ATRX, H3K27me3, INI1 and BRG1 were all retained. Immunohistochemical stains for EMA, GFAP and Olig2 were negative. The Ki-67 proliferation index was 30%-70%. EWSR1 break-apart FISH test was positive. Conclusions: Ewing's sarcoma is rare in the central nervous system and needs to be distinguished from a variety of neoplasms with primitive undifferentiated small cell morphology. Immunohistochemistry and molecular genetics may be required for a proper diagnosis.

Clinical case of endometrial stromal sarcoma

Relevance. Endometrial stromal sarcomas are rare tumors that account for about 0.2% of all malignant tumors of the genital tract. It is believed that they are more common in premenopausal women. Endometrial stromal sarcomas are hormone-sensitive tumors. Because of the rarity of this tumor, there are a limited number of reports in the publications concerning the clinical control and the final result of such cases.Purpose. To study the main pathogenetic, clinical and morphological features of endometrial stromal sarcoma on the example of a clinical case.Materials and methods. The article presents a clinical case of malignant tumor of the uterus, histologically – endometrial stromal sarcoma.Results and conclusions. Diagnosis of endometrial stromal sarcoma is difficult; tumor verification requires mandatory histological examination. Difficulties in diagnosis largely determine the low early detection and poor prognosis in patients with endometrial sarcomas.

Pleomorphic Dermal Sarcoma: a case report

This case report brings a 79-year-old man, with a history of melanoma in situ on the extensor part of the left arm, who presented with an ulcerated nodule on the scalp, right parietal region, with rapid and progressive growth for 3 months. The lesion was clinically compatible with squamous cell carcinoma, Merkel carcinoma, amelanotic melanoma and pleomorphic dermal sarcoma. Exeresis was performed and the diagnosis of Pleomorphic Dermal Sarcoma (PDS) was confirmed by histological and immunohistochemical evaluation. Pleomorphic Dermal Sarcoma (PDS), formerly known as malignant fibrous histiocytoma or undifferentiated pleomorphic sarcoma of the skin, is a rare spindle cell mesenchymal tumor that may recur locally or metastasize. Our objective is to present this rare tumor to the reader, discussing the clinical, histological and immunohistochemical presentation, and to highlight the need for further studies on surgical management protocols and the development of guidelines for the standardization of management and safe follow-up of these patients.