Small Round Research Articles

Bibliometric Analysis of Climate Change Articles on SCI Journal

This research used Bibliometric and spatial distribution to describe science research productivities of climate change articles on SCI during 2007-2018. Of 25278 articles on climate change field, total research publication and Article form is increasing from 2007 to 2018; gains 3325 articles of total scientific production; 2800 articles of Article form in 2018 year. Moreover, the Article form is the highest research production as well with 19917 articles (1st ranking). The USA has the highest publication in all the article types and total research productivity (23286 articles with 1st ranking) including 5369 independent articles (23.06%) and 17917 collaborative articles (76.94%). CLIMATIC CHANGE journal has the most research output with 1105 articles (4.37%) and 1st ranking. Vietnam is ranked 45th with 159 articles (0.63%) including 33 independent articles (50th ranking, 20.8%) and 126 collaborative articles (44th ranking, 79.2%). Further, research productivity is also revealed all the countries with different research productivity quantities on the world map as USA, Canada, Europe community, and some Asia countries has high publication. Particular, Independent publication is showed from small red round dot to big one, and cooperative publication is performed in different colors, in which USA has the most publication in dark blue and big red dot. Therefore, this paper revealed science growth, research publishing trend, and spatial distribution of countries on climate change articles, and it also provides knowledge as well as more understanding about climate change field.

S1998 Colonic Merkel Cell Carcinoma of Unknown Primary: Small Cell Carcinoma Outside the Usual

Introduction: Merkel cell carcinoma (MCC) is a rare neuroendocrine skin carcinoma with documented metastases to the liver, lungs, and gastrointestinal (GI) tract. Colonic metastases are rare but seen with recurrent or primary cutaneous lesions. Described is a patient who presented with bowel obstruction with a hepatic flexure MCC of unknown primary (MCCUP). Case Description/Methods: A 69-year-old female with a history of breast, lung, and anal cancer presented to the emergency department with abdominal pain and distension. Computed tomography of her abdomen demonstrated dilated loops of small bowel and a partial obstruction with transition point in the hepatic flexure. Colonoscopy demonstrated a 4 cm mass at the hepatic flexure obstructing the lumen. Biopsy revealed a high-grade neuroendocrine carcinoma. A right colectomy and histopathology confirmed small cell neuroendocrine carcinoma involving the colonic wall into the pericolic adipose tissue with nodal involvement. It was positive on cytokeratin CAM 5.2, synaptophysin, and Merkel cell polyomavirus (MCPvY) and negative on CDX-2 and TTF-1, excluding primary bowel or lung small cell carcinomas, respectively. Dermatologic evaluation did not reveal a primary cutaneous lesion. Genetic testing was unremarkable. Discussion: This is the first case of MCCUP presenting with colonic obstruction; prior described cases of MCC metastatic to the colon were recurrent disease or from primary skin lesions. Because MCC is rare, has non-specific symptoms, and may lack a primary lesion, MCCUP must be distinguished from other small round blue cell malignancies more commonly found in the GI tract. Thus, neuroendocrine, keratin and MCPyV ancillary staining are critical to distinguish MCC from B-cell lymphoma, small cell carcinoma of bowel or lung primary, and other metastatic dermal malignancies. MCPyV testing in the absence of prior diagnosis is uncommon but vital to the workup of neuroendocrine carcinomas when CDX-2 and TTF-1 are negative as MCPyV positive lymph nodes predict worse outcomes such as disease-free survival. In this patient immunosuppression and prior radiation increased the risk of MCC, and even suggest that this might be a colonic primary. Rarely, MCC and MCCUP are root causes of neuroendocrine carcinomas of the GI tract. In immunocompromised patients, biopsy and pathologic staining are necessary to distinguish insidious malignancies. MCPyV analysis is helpful for diagnosing MCCUP and informing its prognosis.

An unusual cause of oxygen failure in the anesthesia machine.

Dear Editor, A critical incident is being reported wherein a foreign body was detected in the oxygen pipeline inlet at the back of anesthesia workstation. The potentially catastrophic incident occurred during routine pre-use checkup of anesthesia workstation when there was no flow of oxygen on opening the flow control valve while pressure gauge on the pressure regulator of D-type oxygen cylinder was showing full pressure. The pressure gauge on the anesthesia machine was also showing no pressure. The oxygen cylinder and pressure regulator were changed but still there was no oxygen flow. It was then thought to change the connecting hose between cylinder and pipeline inlet at the machine end. As the NIST (non-interchangeable screw thread) connector was loosened from the machine end, an interesting finding was noticed, there was sudden movement of bobbin and oxygen flow started but soon the flow again ceased and the bobbin came back to the same position. Then again on opening the cylinder but keeping the connector at pipeline inlet slightly loosened led to the flow of oxygen and pressure gauge on the workstation also started showing pressure reading. Noticing this finding that loosening the NIST connector of the hose slightly from the pipeline inlet led to oxygen flow in the workstation led us to suspect a foreign body in the oxygen pipeline inlet of workstation. Subsequent careful search in the oxygen inlet led us to find a small black ring which was taken out with an 18 g needle as it was at the distal end of the inlet[Figure 1]. It was a small round rubber ring with a hole in the centre. Initially it was suspected it to be washer of the Bodock seal but closer inspection revealed it to be of different size.Figure 1: Foreign body in the oxygen inlet at the back of anesthesia machineNow the reason for absence of flow on tightening the connector was understood, probably because on tightening the rubber ring became flat and occluded the oxygen entry port of the inlet causing complete cessation of the oxygen flow. When the connector was slightly loosened, the central opening in the ring came back to its normal position thus allowing oxygen flow. The mechanism of entry of the foreign body at that unusual location could not be figured out since the same anesthesia machine has been in use for 4 years. The engineer of the manufacturer, Datex Ohmeda, was contacted and his viewpoint was that it could be a deliberate attempt by theatre personnel; however this could not be confirmed. The other possible explanation could be wear and tear of the ring that caused cessation of oxygen flow on being pressed tightly by metallic NIST connector[Figure 2].Figure 2: Worn out foreign body and NIST connectorThough there are many human errors and equipment malfunctions reported leading to oxygen supply failure[1], but such a presentation of a foreign body at the pipeline inlet has not been reported earlier. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Primary Bilateral Ovarian Lymphoma: A Rare Case Report

Primary ovarian lymphomas constitute 0.5% of all ovarian malignancies. We report one such case in a middle-aged woman who presented with a bilateral ovarian tumor. Bilateral oophorectomy with salpingectomy was sent for frozen section analysis that revealed a round cell tumor. Final histopathology showed a mitotically active small round blue cell tumor arranged in diffuse sheets and chords with an evident starry-sky pattern. Extensive immunohistochemistry (IHC) panel was performed. Histopathology along with IHC did not classify the tumor into diffuse-large-B cell-lymphoma or Burkitt lymphoma and a final diagnosis of high-grade B cell lymphoma (HGBL) was made. This case is presented for its rarity and to discuss the challenges on IHC to subclassify a lymphoma as HGBL. Although rare, the possibility of primary ovarian non-Hodgkin lymphoma should be kept in mind when dealing with bilateral ovarian tumors as a timely and accurate diagnosis can save unnecessary surgeries and improve outcome.

A Highly Discriminative Hybrid Feature Selection Algorithm for Cancer Diagnosis.

Cancer is a deadly disease that occurs due to rapid and uncontrolled cell growth. In this article, a machine learning (ML) algorithm is proposed to diagnose different cancer diseases from big data. The algorithm comprises a two-stage hybrid feature selection. In the first stage, an overall ranker is initiated to combine the results of three filter-based feature evaluation methods, namely, chi-squared, F-statistic, and mutual information (MI). The features are then ordered according to this combination. In the second stage, the modified wrapper-based sequential forward selection is utilized to discover the optimal feature subset, using ML models such as support vector machine (SVM), decision tree (DT), random forest (RF), and K-nearest neighbor (KNN) classifiers. To examine the proposed algorithm, many tests have been carried out on four cancerous microarray datasets, employing in the process 10-fold cross-validation and hyperparameter tuning. The performance of the algorithm is evaluated by calculating the diagnostic accuracy. The results indicate that for the leukemia dataset, both SVM and KNN models register the highest accuracy at 100% using only 5 features. For the ovarian cancer dataset, the SVM model achieves the highest accuracy at 100% using only 6 features. For the small round blue cell tumor (SRBCT) dataset, the SVM model also achieves the highest accuracy at 100% using only 8 features. For the lung cancer dataset, the SVM model also achieves the highest accuracy at 99.57% using 19 features. By comparing with other algorithms, the results obtained from the proposed algorithm are superior in terms of the number of selected features and diagnostic accuracy.

Effect of sex ratio and age parent to consumption feed, weight eggs and conversion ration on birds quail (Coturnix coturnix japonica)

Birds quail (Coturnix Coturnix japonica) is a type of bird lots of quail maintained in the community have morphology i.e. small body, round and tail short Setyawn, Sudjarwo, Widodo, and Prayogi (2011) stated that bird quail is one community poultry of the genus Coturnix which can be generated as producer eggs and meat. Destination from study this for knowing the effect of sex ratio and age parent to consumption feed, weight eggs, and conversion feed. Method study is test design Random Complete (RAL) pattern factorial with 2 factors, namely factor 1 is Sex Ratio and factor 2 is age parent with every 3 treatments and 3 replications. Research results show the sex ratio does not give influence real on consumption feed, the weight of eggs, and conversion feed. Average consumption feed of 675.63 (g/ head) to 683.61 (g/ head), average weight egg of 11.28 (g/ item) to 11.60 (g/ item), and the average conversion ration of 5.63 to 6.08. Influence to age parent gives influence that is not real to consumption feed, weight eggs, and conversion feed. Average consumption feed as big as 677.17 (g/ head) to 679.74 (g/ head), average weight egg of 11.28 (g/ item) to 11.55 (g/ item), and the average conversion ration of 5.71 (g/ head ) to 5.92 (g/ head ). Interaction balance male-female and age parent no give influence real to consumption feed, weight eggs, and conversion feed. The conclusion from the research is the influence of the balance of male-female and the age of the parent no give influence the consumption of feed, the weight of eggs, and conversion of feed.

Stemness Correlates Inversely with MHC Class I Expression in Pediatric Small Round Blue Cell Tumors.

Simple SummaryTumors occurring at a young age are distinct from tumors in older individuals, clinically and pathologically. As small round blue cell tumors (SRBCTs), they often show a resemblance to stem cells and immature precursor cells during embryonal development. Recently, immunotherapy has become an option for a subset of patients with limited success. We observed that in almost all the pediatric SRBCT types investigated (n = 1134) there was an inverse relationship, when comparing genes highly expressed in stem cells with genes encoding MHC class I molecules. MHC class I molecules are important in tumor cell recognition by cytotoxic T cells. We suspect that these tumors are derived from multipotent precursor cells that naturally show a low MHC class I expression and a lack of immune recognition necessary for prenatal proliferation and development.Recently, immunotherapeutic approaches have become a feasible option for a subset of pediatric cancer patients. Low MHC class I expression hampers the use of immunotherapies relying on antigen presentation. A well-established stemness score (mRNAsi) was determined using the bulk transcriptomes of 1134 pediatric small round blue cell tumors. Interestingly, MHC class I gene expression (HLA-A/-B/-C) was correlated negatively with mRNAsi throughout all diagnostic entities: neuroblastomas (NB) (n = 88, r = −0.41, p < 0.001), the Ewing’s sarcoma family of tumors (ESFT) (n = 117, r = −0.46, p < 0.001), rhabdomyosarcomas (RMS) (n = 158, r = −0.5, p < 0.001), Wilms tumors (WT) (n = 224, r = −0.39, p < 0.001), and central nervous system-primitive neuroectodermal tumors CNS-PNET (r = −0.49, p < 0.001), with the exception of medulloblastoma (MB) (n = 76, r = −0.24, p = 0.06). The negative correlation of MHC class I and mRNAsi was independent of clinical features in NB, RMS, and WT. In NB and WT, increased MHC class I was correlated negatively with tumor stage. RMS patients with a high expression of MHC class I and abundant CD8 T cells showed a prolonged overall survival (n = 148, p = 0.004). Possibly, low MHC class I expression and stemness in pediatric tumors are remnants of prenatal tumorigenesis from multipotent precursor cells. Further studies are needed to assess the usefulness of stemness and MHC class I as predictive markers.

Formation and evolution mechanisms of pores in Inconel 718 during selective laser melting: Meso-scale modeling and experimental investigations

The forming quality of selective laser melting (SLM) components largely depends on defects such as pore, which seriously deteriorates the mechanical properties. In this study, a meso-scale numerical model based on discrete element method (DEM) and computational fluid dynamics (CFD) has been proposed to simulate the major physical phenomenon during SLM-fabricated Inconel 718 (IN718) superalloy. The model is applicable to the calculation of transient temperature field, molten pool flow behavior and pore evolution (lack of fusion pore and keyhole-induced pore) considering the Marangoni effect and the recoil pressure, which is validated by experiments in terms of the dimension of the track. The numerical results indicated that the lack of fusion pore mainly occurred at inter-track with partially melted powder. It was observed that the morphology of lack of fusion pore gradually changed from small round to elongated irregular shape with decreasing energy density. The keyhole-induced pore appeared below the melt pool and came from the bubbles trapped in the keyhole under violent molten pool flow. Furthermore, bubble coalescence was found to be the main formation mechanism of large keyhole-induced pore. The simulated pore morphology and dimension were in good agreement with those found in bulk samples.

A rare case report of renal ewing sarcoma/primitive neuroectodermal tumor with ACTH production

BackgroundEwing sarcoma/primitive neuroectodermal tumor (PNET) of the renal is extremely rare. The common cause of ectopic ACTH syndrome is pulmonary neuroendocrine tumors, such as small cell carcinomas and carcinoid tumors. Here, we present an unusual case of ectopic ACTH syndrome and hypothyroidism caused by Ewing sarcoma/PNET of the right kidney.Case presentationA 19-year-old girl presented with a history of right lumbar pain and discomfort for 2 months, aggravated for 2 days. Abdominal contrast-enhanced computed tomography and computed tomography angiography showed an upper pole occupancy of the right kidney occupancy with subepithelial hemorrhage. Preoperative hormone levels including plasma total cortisol (PTC), adrenocorticotrophic hormone (ACTH) and thyroid hormone measurements were abnormal, indicating that the patient had Cushing syndrome and hypothyroidism. The patient underwent right radical nephrectomy. Histopathological analysis revealed a renal small round blue cell tumor (consistent with a primitive neuroectodermal tumor), with positive immunohistochemistry for CD99 and Ki67 (about 10%) and molecular pathology for EWSR1 gene fusions. PTC, ACTH and thyroid hormone returned to normal after surgery.ConclusionsWe report a rare ectopic ACTH syndrome and hypothyroidism due to renal Ewing sarcoma/PNET. The clinical manifestation of renal Ewing sarcoma/PNET is non-specific and the diagnosis relies on pathological morphology, immunohistochemistry and fusion gene detection. At present, surgery combined with radiotherapy and chemotherapy is used in the treatment, but the prognosis is still not optimistic.

Transmission Electron Microscopy: A Surgical Pathology Tool for Neuroblastoma.

Pediatric small round blue cell tumors (PSRBCT) are an intriguing and challenging collection of neoplasms. Light microscopy of small round blue cell tumors identifies small round cells. They harbor a generally hyperchromatic nucleus and relatively scanty basophilic cytoplasm. Pediatric small round blue cell tumors include several entities. Usually, they incorporate Wilms tumor, neuroblastoma, rhabdomyosarcoma, Ewing sarcoma, retinoblastoma, lymphoma, and small cell osteosarcoma, among others. Even using immunohistochemistry, the differential diagnosis of these neoplasms may be controversial at light microscopy. A faint staining or an ambiguous background can deter pathologists from making the proper diagnostic decision. In addition, molecular biology may provide an overwhelming amount of data challenging to distinguish them, and some translocations may be seen in more than one category. Thus, transmission electron microscopy (TEM) can be extremely valuable. Here we emphasize the modern protocol for TEM data of the neuroblastoma. Tumor cells with tangles of cytoplasmic processes containing neurosecretory granules can diagnose neuroblastoma.

Extraosseous Ewing Sarcoma in a Pelvic Region: A Case Report

Ewing sarcoma; neoadjuvant chemotherapy; radiation therapy.

Pituitary adenoma-Gonadotroph type in a 47 years old female patient: A case report

Pituitary adenomas are tumors of the anterior pituitary. Most pituitary tumors are slow-growing and benign. They are classified based on size or cell of origin. Pituitary adenoma can be classified as microadenoma, macroadenoma, and giant tumors based on size.Pituitary adenomas are mostly found incidentally on imaging modalities obtained for other reasons. Mutations in known Oncogenes have little role in the pathogenesis of most sporadic pituitary adenomas. Case presentation : A 47 years old female patient presented with complain of diminished vision , severe headache and dizziness since 1 months.MRI was advised which showed an ill-defined low signal intensity lesion of approximate 3.4x3.2x3 cm noted in clivus, bilateral spenoid sinus and sella region. Histologically, near-signature appearance is that of elongated cells surrounding blood vessels in a pseudorosette-like ependymoma-mimicking pattern. cells with small round nuclei and there is no significant mitotic activity. Conclusion : Pitutary adenoma-gonadotroph type is benign and can cause symptoms from mass effect or by effecting the level of the hormones. Differetial diagnosis includes ependymoma, Pitutary blastoma, Pitutary nodular hyperplasia, dermoid cyst, low grade astrocytoma, meningioma, rathke cleft cyst.

Basaloid Squamous Cell Carcinoma of the Uterine Cervix in a Northern Nigerian Hospital: A Case Report and Review of Literature

Basaloid squamous cell carcinoma occurs very rarely in the uterine cervix. Basaloid squamous cell carcinoma is a form of squamous cell carcinoma with a poor prognosis that primarily affects persons in their late sixties and early seventies. This paper describes a case of Basaloid squamous cell carcinoma in the fifth decade that had spread to the myometrium, ovaries, and fallopian tubes. The macroscopic findings of a total abdominal hysterectomy and bilateral salphingo-oophorectomy revealed an ulcerative fungating tumor distorting the whole cervix and macroscopic invasion of the posterior uterine wall. Histologic features revealed a Basaloid tumour disposed in nest exhibiting peripheral palisading and stromal retraction with most of the nest having comedo pattern of necrosis. The tumour cells are fairly small uniform round cells, with scanty cytoplasm having increased nuclei cytoplasmic ratio with vesicular to open nuclei. Immunohistochemical stains; Cytokeratins, EMA, p63 and Ki-67 showed cytokeratin positivity and EMA strong and diffuse positivity. Keywords: Basaloid Squamous cell Carcinoma, uterine cervix, p63, EMA, Ber-EP4

Mesenchymal chondrosarcoma of the head and neck with HEY1::NCOA2 fusion: A clinicopathologic and molecular study of 13 cases with emphasis on diagnostic pitfalls.

Mesenchymal chondrosarcoma (MCS) is a rare translocation-associated sarcoma, driven by a canonical HEY1::NCOA2 fusion. The tumors typically have a biphasic phenotype of primitive small blue round cells intermixed with hyaline cartilage. The head and neck (HN) region is a common site for MCS, accounting for 12-45% of all cases reported. We assembled a relatively large cohort of 13 molecularly confirmed HN MCS for a detailed clinicopathologic analysis. The underlying fusion events were determined using fluorescence in situ hybridization and/or targeted RNA sequencing. The median age of presentation was 19 years. Five MCSs (39%) had an intraosseous presentation (skull, maxilla, palate, and mandible), while the remaining eight cases occurred in the brain/meninges, orbit, and nasal cavity. Microscopically, HN MCSs were characterized by primitive round cells arranged in a distinctive nested architecture and a rich staghorn vasculature. A cartilaginous component of hyaline cartilage islands and/or single chondrocytes were present in 69% cases. A combined immunoprofile of CD99(+)/SATB2(+)/CD34(-)/STAT6(-) was typically noted. As this immunoprofile is non-specific, the referral diagnoses in cases lacking a cartilaginous component included Ewing sarcoma family and osteosarcoma. Among the seven patients with follow-up data, three developed distant metastasis and one died of disease. HN MCS may arise at intra- or extra-osseous sites. The HN MCS appears to have a more prolonged survival compared other MCS sites. Testing for HEY1::NCOA2 fusion is recommended in HN tumors with nested round cell morphology and staghorn vasculature that lack a distinctive cartilaginous component.

First Report of Leaf Spot Caused by Nigrospora hainanensis on Oxalis corymbosa in China.

Oxalis corymbosa DC. introduced into China as an ornamental plant in the mid-19th century is commonly known as an important medicinal and edible perennial herb (Zhou et al. 2021). The plant native to South America is also an invasive and widely distributed weed found in agricultural farms, gardens, and lawns, especially in sugarcane fields of Guangxi province, China. The coverage rate of O. corymbosa in sugarcane fields was normally more than 70%, sometimes up to 100%. In March of 2021, a leaf spot disease of O. corymbosa from sugarcane fields was encountered in Nanning city of Guangxi province, China. Early symptoms appeared as small yellowish round spots. The spots turned to be irregularly, usually exhibiting pale brown necrosis in the center with dark brown necrotic well-defined margins. Severely infected leaves turned to be blighted, then dead. To isolate the pathogen, diseased leave tissue fragments (4 mm × 4 mm) were soaked in 75% ethanol for 10 s followed by 2% sodium hypochlorite for 1 min, and rinsed by sterile water for three times. They were transferred to potato dextrose agar (PDA) medium cultured at 25 °C. Pure cultures were obtained by collected hypha tip from upcoming colonies. The colony features were similar to each other, floccose, white at first, becoming brown, dark brown or black on PDA after 7 days fully covered the 90 mm petri-dishes. Conidial determination were conducted on synthetic nutrient-poor agar medium (SNA) according to Wang et al. (2017). Conidia abundantly dispersed on SNA arising from conidiophores, which normally reduced to conidiogenous cells generated from hyphae. The conidiogenous cells were monoblastic, hyaline, globose or ampulliform, 6-8.5 (-12.5) × 5-7.5 (-9) μm in size (n=50). Conidia were solitary, smooth, black, sphaerical or ellipsoidal, (11-) 13-16.5 × (8-) 10-15.5 μm in size (n=100). Setae were not observed during the observation. The fungus was identified as Nigrospora sp. based on the morphology. One of the representative strains (FSC-3) was selected for genomic DNA extraction. The sequences of transcribed spacer region of rDNA (ITS), the partial translation elongation factor (TEF1), and the Beta-tubulin fragment (TUB) were respectively amplified using primer pairs ITS1/ITS4 (White et al. 1990), EF1-728F and EF2 (Carbone & Kohn 1999, Crous et al. 2013) and Bt2a and Bt2b (Glass & Donaldson 1995), deposited in the NCBI GenBank with accession numbers of OK083685 (541 bp), OK184809 (481 bp) and OK086377 (421 bp). BLASTn analysis showed that those ITS, TEF1 and TUB gene sequences shared 99%-100% identity with the type strain (CGMCC3.18129) of Nigrospora hainanensis (GenBank accession nos. NR153480, KY019415, KY019464, respectively). In addition, a maximum likelihood analysis using concatenated gene sequences of ITS, TEF1 and TUB was performed in RAxML v.7.2.8 (Stamatakis 2006) implementing the model of GTRCAT with 1,000 bootstrap replicates. The phylogenetic results indicated that the strain FSC-3 was N. hainanensis, which also confirmed after a morphological comparison with N. hainanensis (Wang et al. 2017). Pathogenicity was tested on living Oxalis corymbosa leaves (3 plants for each test) arising from cultivated roots grown for three weeks. It was conducted by dropping 5 μL conidial suspensions (105 conidia / mL) on the living leaves (two sites per leave) incubated in separate containers at 25 °C with 90-100% relative humidity after inoculation. Controls were treated with sterile distilled water. Pale brown small spots came up after 24 h, and then extended to brown larger spots. Symptoms after inoculation were similar to field ones, while the control plants remained healthy. The pathogenicity test was repeated twice with the similar results. Re-isolation of the pathogen from the inoculated leaves was determined based on morphology and sequence analysis to fulfill Koch's postulates. Nigrospora hainanensis had been found from diseased root and leaf tissues of sugarcane in Liuzhou city, Guangxi province (Raza et al. 2019). The results indicated that O. corymbosa was another host in sugarcane fields in Guangxi, China. To our knowledge, this is the first report of Nigrospora hainanensis causing leaf spot on Oxalis corymbosa in China.

Ocular rhinosporidiosis- A case report

We report the clinical manifestations of ocular rhinosporidiosis and its management.A 25 years old male belonging from rural background, presented to Ophthalmology OPD after being referred from ENT OPD with a painless, reddish-pink, fleshy mass on white portion of the right eye. He had a similar looking mass inside his right nostril. He gave history of some unknown particle entering his right eye and nostril while watering the paddy fields and lesion developed in those places after 4-5 days.On examination, he had a fleshy, red-coloured mass attached to the bulbar conjunctiva of his right eye, supero-nasal to cornea, about 2.5 mms from the limbus. It had an elevated surface with vessels growing over it which did not bleed on touch. There were small whitish round to oval spots on the surface of the lesion.The patient was started with tablet Dapsone (100mg BD) for 3months and he underwent excision of conjunctival lesion under local anaesthesia along with cauterization at its base. The excised mass was sent for histopathological examination. Additionally, he was given topical Moxifloxacin (0.5% w/v) eye drops for 2weeks and systemic antibiotics (Amoxy-Clav 625mg BD for 5days) as post surgical prophylaxis. ENT surgeons excised the mass present on the nasal mucosa.The patient was diagnosed clinically to have conjunctival rhinosporidiosis. This was confirmed by histopathological examination. The patient was followed up for18 months and was asymptomatic with no recurrence of the lesions in the eye or nose.

Abstract 4015: Primary sites affecting the prognosis of Ewing's sarcoma

Abstract Introduction Ewing’s sarcoma (ES) is a small round blue cell malignancy arising from the bone or soft tissue, and most commonly impacts adolescents and young adults. It is difficult to identify those who will likely relapse, however, ES has a very high recurrence rate. Given these realities, the objective of this study was to determine factors influencing the prognosis of ES patients. Methods Clinical and genomic data of 99 patients with ES, were obtained from the cBioPortal. Tumor mutational burden (TMB) and patient survival information were also obtained from the cBioPortal. Results In the data examined, ES had multiple primary sites of disease, including 16 femur, 10 hipbone, 10 rib, 10 tibia, 6 ischium pubis, 5 scapula, 4 fibula, 3 dorsal vertebra, 3 intra-abdominal, 3 lumbar vertebra, and 3 skull. A comparison of survival relationships between different primary sites of ES indicated that overall survival (OS) was significantly different between femur and dorsal vertebra (p = 0.023) and intra-abdominal and hipbone (p = 0.049). However, no significant difference was determined for TMB between the different primary sites. The median value of TMB was 0.23. Data were divided into two groups: high TMB (TMB-H,≥ 0.23 muts/Mb) and low TMB (TMB-L, &amp;lt; 0.23muts/Mb). Interestingly, the OS of patients with TMB-L was significantly higher than that of patients with TMB-H (p = 0.049; 59.45 versus 42.00 months). The presence of metastases at the time of diagnosis was associated with poor OS ( p = 0.023;) and was a clinical factor associated with outcomes having statistical significance. Conclusions Given the heterogeneity in outcomes for patients with ES, additional prognostic factors are needed to optimize clinical decision making. Prognosis was different depending on primary site of ES. Patients with ES in hipbone displayed a better prognosis compared to that in abdomen. Similarly, patients with ES in dorsal vertebra had a significantly higher OS than that in femur. As such, the primary site should perhaps be considered during ES treatment, although there was no significant difference between TMB and primary site. Our results indicate that high TMB is associated with inferior survival for ES and may provide insights for clinical practice. Citation Format: Jun-Qing Mao, Dan Zhao. Primary sites affecting the prognosis of Ewing's sarcoma [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 4015.

Mixed epithelial and stromal tumours of the kidney with malignant transformation: a clinicopathological study of four cases

Mixed epithelial and stromal tumour of the kidney is a complex benign neoplasm in which malignancy rarely arises. In this study, we report four mixed epithelial and stromal tumours in which sarcoma or carcinoma developed. In the first, a multifocal adenocarcinoma arose and areas of transition from benign to malignant epithelium were observed. Oestrogen and progesterone receptors were diffusely present in the nuclei of the spindle cell stroma of the benign component. The second was a sarcoma in which benign epithelial elements were intermixed. Outside the renal parenchyma, clusters of small benign glands surrounded by oestrogen receptor-positive benign stroma were present, supporting the diagnosis of mixed epithelial and stromal tumour. Fluorescence in situ hybridisation for SYT-SSX translocation and immunohistochemical results, specifically TLE1 -ativity, argued against primary renal synovial sarcoma. The patient died 24 months after surgery. The third tumour consisted of small blue round cells, positive for epithelial membrane antigen, BCL2, CD99, and FLI1. Throughout the tumour, the presence of benign appearing branching tubules in fibromuscular stroma, reactive for smooth muscle actin, desmin and progesterone receptor, supported the diagnosis of mixed epithelial and stromal tumour in which a small round blue cell sarcoma with EWSR1 rearrangement arose. In the fourth tumour, adenocarcinoma with papillary architecture arose in a typical mixed epithelial and stromal tumour. In summary, we present four cases of mixed epithelial and stromal tumour with malignant transformation, two showing carcinomatous and the other two with sarcomatous transformation. Identification of typical benign looking elements and the absence of SYT-SSX translocation are helpful in recognition of this entity.

Long-term control and safety of larotrectinib in a cohort of adult and pediatric patients with tropomyosin receptor kinase (TRK) fusion primary central nervous system (CNS) tumors.

2010 Background: Neurotrophic tyrosine receptor kinase ( NTRK) gene fusions are known oncogenic drivers in a variety of tumor types. Larotrectinib is a highly selective, CNS-active TRK inhibitor that demonstrated an objective response rate (ORR) of 30% and a 24-week disease control rate (DCR) of 73% across 33 evaluable adult and pediatric patients with TRK fusion primary CNS tumors, as of July 2020 (Doz et al, Neuro Oncol 2021). We report updated data on an expanded dataset of patients. Methods: Patients with TRK fusion primary CNS tumors in two clinical trials (NCT02637687, NCT02576431) were included. Larotrectinib was administered at 100 mg twice daily (BID) in adults and 100 mg/m2 (max 100 mg) BID in pediatric patients. Response was investigator-assessed. Results: As of July 2021, 38 adult and pediatric patients with TRK fusion primary CNS tumors were identified: high-grade glioma (HGG; n =23), low-grade glioma (LGG; n =9), and other (n =6; includes glioneuronal, neuroepithelial, diffuse leptomeningeal, neuroblastoma, recurrent small round blue cell, and not otherwise specified). Median age at enrollment was 10.8 years (range 1.3–79.0; 28 [74%] patients &lt; 18 years old). The gene fusions involved NTRK2 (n = 28), NTRK1 (n = 6), and NTRK3 (n = 4). Sixteen (42%) patients received one prior line of systemic therapy and 16 (42%) received ≥2 prior lines. The ORR for 37 evaluable patients was 30% (95% confidence interval [CI] 16–47): three complete responses, eight partial responses, 21 stable disease (16 patients ≥24 weeks), and five progressive disease. The 24-week DCR was 73% (95% CI 56–86) for all patients, 68% (95% CI 45–86) for patients with HGG, and 89% (95% CI 52–100) for patients with LGG. Twenty-five of 31 patients (81%) with measurable disease at baseline had tumor shrinkage. Median time to response was 1.9 months. Median duration of response (DoR) was not reached; median follow-up was 25.6 months. The 12-month DoR rate was 64%. Median progression-free survival (PFS) was 16.5 months (95% CI 6.7–not estimable); median follow-up was 27.4 months. Median overall survival (OS) was not reached; median follow-up was 26.7 months. The 24-month OS rate was 65%. Treatment duration ranged from 0.1+ to 38.7+ months. Twenty-two patients (58%) progressed on treatment and three continued treatment post-progression for ≥4 weeks. Treatment-related adverse events (TRAEs) were reported in 21 patients (55%); the majority of these patients (18/21 [86%]) reported Grade 1 or 2 TRAEs. No Grade 3 or higher treatment-related neurological adverse events were reported. There were no treatment discontinuations due to TRAEs. Conclusions: Larotrectinib achieved a high DCR, rapid and durable responses, and a manageable safety profile in patients with TRK fusion primary CNS tumors. These results support testing for NTRK gene fusions in patients with CNS tumors. Clinical trial information: NCT02637687, NCT02576431.

A multicenter evaluation of treatment patterns and outcomes for Adamantinoma-like Ewing sarcoma.

e23500 Background: Sarcomas are a rare and heterogenous group of malignancies comprised of &gt; 150 histologic subtypes. Adamantinoma-like Ewing sarcoma (ALES) is a rare, small round blue cell tumor that represents a variant of Ewing sarcoma (EWS). First described 20 years ago, ALES harbors a EWS subset defining EWS-related translocation; however, ALES has a complex epithelial differentiation component defining it as a distinct clinical entity. Most ALES cases arise in the head and neck, but it has also been reported in the extremities and thorax. Given the rarity of ALES, there is a paucity of literature to guide treatment and describe outcomes. Methods: We performed a retrospective review (January 1, 2010-January 1, 2022) at two sarcoma centers in the United States and identified 4 cases of ALES. Tumor and patient characteristics were described, and patient outcomes were assessed. Results: Four patients were identified. 3 (75%) were male. Median age at diagnosis was 37 years (range 23-57). Primary site of ALES was head and neck (50%) and thorax (50%). Median tumor size was 4 cm (2.7-5.5 cm). All patients underwent resection of their primary tumor, and two (50%)received adjuvant radiation therapy. All pts received/are receiving adjuvant chemotherapy. 75% of patients were treated with Vincristine, Doxorubicin, and Cyclophosphane (VDC) alternating with Ifosfamide &amp; Etoposide (IE) for 12-14 cycles. One patient received 4 cycles of adjuvant carboplatin and etoposide and radiation after an initial diagnosis of basaloid carcinoma but recurred 47 months later and diagnosed at ALES at that time. That patient underwent re-resection followed by adjuvant chemotherapy with Vincristine, Doxorubicin, and Ifosfamide (VAI). Median follow-up from initial diagnosis was 25 months (3-39 months). All patients are alive with no evidence of disease. Conclusions: Similar to treatment of EWS, a multidisciplinary strategy is paramount. VDC/IE is a reasonable treatment strategy. Further multicenter collaboration is essential to define clinical characterization of this rare sarcoma subtype and to guide treatment particularly in the relapsed or refractory setting.[Table: see text]