Soft Tissue Tumors In Children Research Articles

Role of diffusion-weighted MRI in evaluation of pediatric musculoskeletal soft tissue masses

BackgroundIn pediatric patients, soft tissue masses encompass a wide heterogeneous group of benign and malignant lesions. MRI is a powerful diagnostic tool in the workup of soft tissue tumors in children, and it helps in characterization of lesion and evaluation of the extent of the lesion. However, conventional MRI techniques are not specific in differentiating benign from malignant lesions. So to improve characterization of tumors, DWI was added to MRI techniques as it increases sensitivity and specificity by detecting the micro-diffusion changes of water into intra- and extracellular spaces. The aim of this work was to highlight the diagnostic value of DWI in detection and characterization of different musculoskeletal soft tissue masses in pediatrics.ResultsThere was a statistically significant difference regarding the mean ADC value of benign and malignant masses (P value = 0.001*). The mean ADC value for all benign masses (n = 41) was 1.495 ± 0.55 SD × 10–3 mm2/s, while the mean ADC value for all malignant masses (n = 21) was 0.449 ± 0.27 SD × 10–3 mm2/s. The cutoff ADC value between benign and malignant masses was 0.88 × 10–3 mm2/s. This cutoff ADC value has sensitivity of 100.0%, specificity of 92.3%, PPV of 66.7%, NPV of 100.0% and diagnostic accuracy of 93.3%.ConclusionIn pediatric patients, DWI is an innovative valuable noninvasive imaging technique for characterization of musculoskeletal soft tissue masses and discrimination between benign and malignant masses.

Synovial Sarcoma in Children, Adolescents, and Young Adults: A Report From the Children's Oncology Group ARST0332 Study.

Synovial sarcoma (SS) is the second most common malignant soft tissue tumor in children. ARST0332 evaluated a risk-based treatment strategy for young patients with soft tissue sarcoma designed to limit therapy for low-risk (LR) disease and to test neoadjuvant chemoradiotherapy for unresected higher-risk disease. Newly diagnosed patients with SS age < 30 years were assigned to four treatment arms based on disease features: A (surgery only), B (55.8 Gy radiotherapy [RT]), C (ifosfamide and doxorubicin [ID] chemotherapy plus 55.8 Gy RT), and D (neoadjuvant ID and 45 Gy RT, then surgery and RT boost based on margins followed by adjuvant ID). Patients treated in Arms A and B were considered LR, arms C and D without metastases as intermediate-risk (IR), and those with metastases as high-risk (HR). Of the 146 patients with SS enrolled, 138 were eligible and evaluable: LR (46), IR (71), and HR (21). Tumors were 80% extremity, 70% > 5 cm, 70% high-grade, 62% invasive, 95% deep, and 15% metastatic. Treatment was on arm A (29.7%), B (3.6%), C (16.7%), and D (50%). There were no toxic deaths and four unexpected grade 4 adverse events. By risk group, at a median follow-up of 6.8 years, estimated 5-year event-free survival was LR 82%, IR 70%, and HR 8%, and overall survival was LR 98%, IR 89%, and HR 13%. After accounting for the features that defined risk category, none of the other patient or disease characteristics (age, sex, tumor site, tumor invasiveness, and depth) improved the risk stratification model. The risk-based treatment strategy used in ARST0332 produced favorable outcomes in patients with nonmetastatic SS relative to historical controls despite using RT less frequently and at lower doses. The outcome for metastatic SS remains unsatisfactory and new therapies are urgently needed.

Progression and Differentiation of Alveolar Rhabdomyosarcoma Is Regulated by PAX7 Transcription Factor-Significance of Tumor Subclones.

Rhabdomyosarcoma (RMS), is the most frequent soft tissue tumor in children that originates from disturbances in differentiation process. Mechanisms leading to the development of RMS are still poorly understood. Therefore, by analysis of two RMS RH30 cell line subclones, one subclone PAX7 negative, while the second one PAX7 positive, and comparison with other RMS cell lines we aimed at identifying new mechanisms crucial for RMS progression. RH30 subclones were characterized by the same STR profile, but different morphology, rate of proliferation, migration activity and chemotactic abilities in vitro, as well as differences in tumor morphology and growth in vivo. Our analysis indicated a different level of expression of adhesion molecules (e.g., from VLA and ICAM families), myogenic microRNAs, such as miR-206 and transcription factors, such as MYOD, MYOG, SIX1, and ID. Silencing of PAX7 transcription factor with siRNA confirmed the crucial role of PAX7 transcription factor in proliferation, differentiation and migration of RMS cells. To conclude, our results suggest that tumor cell lines with the same STR profile can produce subclones that differ in many features and indicate crucial roles of PAX7 and ID proteins in the development of RMS.

Abstract 3048: Surfaceome profiling identifies the immune checkpoint molecule B7-H3 as a key player of immune-evasion in rhabdomyosarcoma

Abstract Introduction: Pediatric rhabdomyosarcoma (RMS) is the most common soft tissue tumor in children, with nearly 20% of children presenting with locally aggressive and/or metastatic disease. Despite aggressive chemotherapy and radiotherapy, the 5-year overall survival rate is still less than 40%. In addition, resistance to chemotherapy remains the major cause of cancer-related mortality. Therefore, there is an unmet need to develop more effective and tolerable therapeutic strategies for the treatment of RMS. Our overall objective is to leverage transcriptomic and proteomic profiling methods to identify RMS-specific surface proteins targetable with immune-based therapies. Methods: To identify therapeutic targets, we performed cell-surface capture and mass spectrometry on fusion-positive (FP), fusion-negative (FN) RMS and normal skeletal muscle. Transcriptomic datasets of RMS tumor specimens and normal tissue were employed to identify high-confidence RMS-specific cell-surface proteins. Expression of immune checkpoint molecules was confirmed by flow cytometry, western blot and PCR. Paraffin-embedded tissue sections were used to determine protein expression and prognostic value in RMS patients. We developed an in vitro T-cell killing assay to assess the functional role of immune checkpoint molecules on the antitumor immune response. Results: Surfaceome profiling of RMS cells revealed unique protein signatures specific for each tumor type and normal tissue. We have identified 5061 cell-surface proteins in RMS and 31.6% of proteins were correctly located at the plasma membrane. By integrating proteomic and transcriptomic data, we have identified 30 and 38 proteins significantly overexpressed in fusion-negative and fusion-positive RMS respectively compared to normal muscle. We have identified the immune checkpoint molecule B7-H3 as cell-surface protein overexpressed in both RMS subtypes. Strong expression of B7-H3 was also observed in patient's tumor specimens. Genetic deletion of B7-H3 gene in RMS cells was associated with higher antitumor immune response in tumor cells co-cultured with CD8+-T cells. No difference was observed in tumor cell proliferation and radioresistance. Conclusion: Through integrated transcriptomic and proteomic profiling, we have discovered B7-H3 as a key regulator of tumor immune-evasion in RMS. B7-H3 is a suitable immunotherapy target for the treatment of RMS. Further studies are warranted to elucidate underlying mechanisms of B7-H3 function in RMS. Citation Format: Roxane R. Lavoie, Yohan Kim, Mohamed E. Ahmed, Doris Phelps, Emily Baer, Cristine Charlesworth, Benjamin Madden, Liguo Wang, Haidong Dong, Peter Houghton, John Cheville, Patricio Gargollo, Candace Granberg, Fabrice Lucien. Surfaceome profiling identifies the immune checkpoint molecule B7-H3 as a key player of immune-evasion in rhabdomyosarcoma [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2021; 2021 Apr 10-15 and May 17-21. Philadelphia (PA): AACR; Cancer Res 2021;81(13_Suppl):Abstract nr 3048.

Bone and soft tissue tumours in children : Proposal for arational diagnostic approach

Bone and soft tissue tumours are often incidental findings in children. Because they are usually benign tumours, nonspecialised radiologists generally have little experience in the diagnosis and differentiation from malignant tumours. Various imaging techniques are used in the diagnosis of skeletal tumours. Imaging techniques used to evaluate bone and soft tissue tumours include sonography, computed tomography (CT) and magnetic resonance imaging (MRI). An algorithm to determine malignancy of bone and soft tissue tumours in children is proposed. By using the presented algorithms, further diagnostic procedures such as biopsies can be avoided in the majority of children with bone and soft tissue tumours. Aggressive bone lesions and unclear soft tissue tumours are guided to biopsy to confirm diagnosis. The algorithms presented are based on the proposals of European professional societies and have been adapted by the authors for use in children and adolescents. In the clarification of soft tissue tumours, sonography is the first diagnostic tool; depending on the sonographic findings, MRI is the technique for further clarification. Biopsy confirmation of the diagnosis in unclear cases or in cases of probable malignancy should be carried out in apaediatric oncology centre.

COMPUTER TOMOGRAPHY AND X-RAY DIAGNOSTICS OF PERIPHERAL PRIMITIVE NEUROEECTODERMAL TUMORS (PNET) IN CHILDREN

Relevance: Peripheral primitive neuroectodermal tumor (PNET) belongs to the group of malignant tumors that develop from migrating embryonic neural crest cells. PNET includes a number of nosological forms: Askin's tumor, esthesioneuroblastoma, the very peripheral primitive neuroectodermal tumor, Ewing's sarcoma . PNET accounts for 3–9% of all soft tissue tumors in children and 19% of all soft tissue sarcomas. In European countries and the United States, the frequency of their occurrence is 3.4 cases per year per 1 million children under 15 years of age, in Kazakhstan - 0.6-1.2 cases per 1 million child population. Rapid tumor growth, malignancy and early metastasis to other organs and systems predetermines its special role in oncology. Objective of the study: to improve the quality and availability of early diagnosis of sarcomas in children in medical institutions of the general medical network. Results of the study: 35 cases of peripheral PNET in children were analyzed. The age of the patients is from 1.5 to 17 years. The average age is 9.3 years. There were 1.3 times more boys among patients than girls. Extra-skeletal localization was found in one patient (3.6%). Radiographically, it is often almost impossible to make a differential diagnosis between the manifestations of Ewing's sarcoma and primary chronic or "healed" (antibiotic) forms of acute hematogenous osteomyelitis in the initial phase of the process, before the formation of the extraosseous soft tissue component. The process of bone damage is more often localized in its diaphysis and subsequently spreads to the metaphyses of the bone.

A Giant Mesentric Lipoblastoma in a Two-Year Girl: A Case Report

Back ground: Abdominal lipoblastomas which are benign but uncommon soft tissue tumors in children rarely arise from the mesentery.However, benign, these may attain in large size and cause compression symptoms. Complete resection and close post-operative monitoring are necessary for avoidance of recurrence. Histopathology with immunohistochemistry in conjunction with the morphology is the gold standard for diagnosis. Herein such a rare a case of mesenteric lipoblastoma with huge abdominal distention is reported.&#x0D; Case presentation: A 2-year-old girl presented with an enormous abdominal swelling occupying almost all quadrants of the abdomen with repeated dull abdominal pain. The swelling was a non-tender and tensely cystic. USG and CT Scan could not distinguish the proper origin or location of the mass but demonstrated as a large encapsulated and lobulated fatty mass measuring about (16.4x10.0x18.6cm) involving right hypochondriac, epigastric, bilateral ilio-lumber, umbilical &amp; hypogastric regions, pushing the bowel loops upward &amp; laterally with mild mesenteric thickening of perilesional mesentery and separated from the liver &amp; both kidneys and other intraabdominal organs. After exploratory laparotomy there revealed a well capsuled large (about 15x11x14cm)soft yellow mass displacing the entire intra-abdominal contents, connected by a single vascular pedicle with the mesentery and loosely enclosing the mesentery as a whole. Histology and immunohistochemistry of completely resected mass conûrmed the presence of mature adipocytes and immuno-morphology was compatible with Lipoblastoma.&#x0D; Conclusion: This case represents one of the largest mesenteric lipoblastomas in accordance to the patient’s age that histologically, mostly composed of mature adipocytes with few lipoblasts.&#x0D; J Shaheed Suhrawardy Med Coll, December 2020, Vol.12(1); 54-57

Pediatric Nasopharyngeal Rhabdomyosarcoma: About a Case and Review of Literature

Introduction: Rhabdomyosarcoma (RMS) is the most common soft tissue tumor in children, with the head and neck location accounting for up to 40% of cases. The embryonal and alveolar histologic variants are more commonly seen in pediatric patients. The treatment is multimodal and the prognosis of this clinical entity is always gloomy. We report the case of nasopharyngeal rhabdomyosarcoma in a 7-year-old boy child with a good response to early post-therapy. Case Presentation: The patient was diagnosed with nasopharyngeal RMS revealed by a congested nose, ptosis and bilateral blindness, and who received induction chemotherapy followed by concurrent radiotherapy followed by adjuvant chemotherapy. The evolution is marked by a good clinical course but persistence of bilateral blindness. Conclusion: The RMS nasopharyngeal often presents with nonspecific symptoms. Multimodal therapy should be performed including surgery, chemotherapy and radiotherapy.

Childhood soft tissue tumors, one center experience

Aim: Studies on childhood soft tissue tumors have generally been conducted with malignant tumors. There are few studies in the literature comprising all soft tissue tumors. The current study aimed to examine the incidence of all benign and malignant soft tissue tumors during childhood in East Mediterranean region.Materials and Methods: This study retrospectively analyzed the data gathered from patients diagnosed with soft tissue tumors according to the WHO 2020 classification. It focused on those aged 0–18 years that underwent excisional biopsy at Adana City Training and Research Hospital (Adana, Turkey) between 2014 and 2020.Results: Of the total patients, 47.5% were female and 52.5% male. Of these, 87.2% of the cases were benign, 9.2% malignant, and 3.6% intermediate. Hemangioma (55%) was the most common benign soft tissue tumor, and the most common location was the head–neck. Other benign soft tissue tumors were neurofibroma (8.8%), lipoma (6.1%), schwannoma (5.1%), and neuroma (4.4%). Rhabdomyosarcoma (53.2%) was the most common malignant soft tissue tumor. The embryonal subtype was the most common (36.6%). Other malignant soft tissue tumors were extra-osseous PNET/Ewing’s sarcoma (16.6%), synovial sarcoma (13.3%), and leiomyosarcoma (3.3%).Conclusion: Similar to the literature, in this study, the most common benign soft tissue tumor in children was hemangioma, and the most common malignant soft tissue tumor was rhabdomyosarcoma. It is hoped that this study will contribute to the literature as it is the first study to cover all childhood soft tissue tumors at national and regional scale.

MYBPC2 and MYL1 as Significant Gene Markers for Rhabdomyosarcoma.

Background:Rhabdomyosarcoma is the most common soft tissue tumor in children. Rhabdomyosarcoma commonly results in pain and bleeding caused by tumor compression and is prone to early metastasis and recurrence, which can seriously affect the therapeutic outcomes and long-term prognosis. Up to 37.7% of rhabdomyosarcomas may metastasize. Therefore, the molecular mechanisms underlying rhabdomyosarcoma must be explored to identify an effective target for its early diagnosis and specific treatment.Methods:A dataset of 18 rhabdomyosarcoma tissue samples and 6 healthy skeletal muscle samples was downloaded. Differentially expressed genes between rhabdomyosarcoma and healthy tissue samples were identified by GEO2R. Kyoto Encyclopedia of Genes and Genomes and gene ontology pathway enrichment analyses were performed. A protein–protein interaction network was constructed, and hub genes were identified. Expression and survival analyses of hub genes were performed. Additionally, 30 patients with rhabdomyosarcoma were recruited, and overall survival information and samples were collected. Reverse transcription quantitative real-time polymerase chain reaction assays were performed to verify the expression of MYBPC2 and MYL1 in rhabdomyosarcoma tumor tissues. The Kaplan–Meier method was used to explore overall survival based on our clinical data.Results:In total, 164 genes were up-regulated and 394 were down-regulated in rhabdomyosarcoma tumor tissues. Gene ontology analysis revealed that variations were predominantly enriched in the cell cycle, muscle contraction, muscle system processes, cytoskeleton, nucleotide binding, and cytoskeletal protein binding. The protein–protein interaction network revealed 3274 edges, and 441 nodes were constructed. Ten hub genes were identified; of these, MYBPC2 and MYL1 were significantly up-regulated in rhabdomyosarcoma. Compared with the healthy group, patients with rhabdomyosarcoma exhibiting high expression of MYBPC2 and MYL1 exhibited significantly worse overall survival.Conclusions:We found differentially expressed genes between rhabdomyosarcoma and healthy tissue samples. MYBPC2 and MYL1 may be involved in the pathogenesis of rhabdomyosarcoma and therefore deserve further exploration.

Abstract B28: A cross-species enhancer activity analysis approach to identify the Ewing sarcoma cell of origin

Abstract Ewing sarcoma is a malignant bone and soft tissue tumor in children and adolescents. In 85% of all cases the formation of Ewing sarcoma is caused by a chromosomal translocation, leading to the expression of the oncogene EWS-FLI1. This aberrant transcription factor is the main driver of the disease and leads to massive transcriptional dysregulation. Although the genetic mechanism driving Ewing sarcoma is well understood, an animal model adequately mimicking the disease is still lacking. One reason why modeling attempts have remained difficult is the elusive cell of origin in Ewing sarcoma. Several cell types including neural crest progenitor cells and mesenchymal stem cells have been proposed, but none of them has yet been confirmed as cell of origin of Ewing sarcoma. Towards identifying this cell of origin, we are currently following a novel cross-species approach by exploring the use of enhancers, which are specifically active in Ewing sarcoma cells, but were not created de novo by EWS-FLI1. Injection of reporter constructs into zebrafish embryos allows for visualization of cell types with activity of the selected enhancers and provides us with cell-of-origin candidate cell types. To investigate which of these candidate cells are permissive for development of Ewing-like tumors, we will also express EWS-FLI1 in these cells. If successful, this approach will not only reveal the Ewing sarcoma cell of origin but also result in a zebrafish Ewing sarcoma model, which will help to understand tumor initiation and progression and, furthermore, will be a valuable tool to develop novel therapeutic strategies. Citation Format: Sarah Grissenberger, Anna Poetsch, Monika Heinzl, Luisa Morelli, Nathan Sheffield, Niko Popitsch, Heinrich Kovar, Martin Distel. A cross-species enhancer activity analysis approach to identify the Ewing sarcoma cell of origin [abstract]. In: Proceedings of the AACR Special Conference on the Advances in Pediatric Cancer Research; 2019 Sep 17-20; Montreal, QC, Canada. Philadelphia (PA): AACR; Cancer Res 2020;80(14 Suppl):Abstract nr B28.

SNAIL Promotes Metastatic Behavior of Rhabdomyosarcoma by Increasing EZRIN and AKT Expression and Regulating MicroRNA Networks.

Rhabdomyosarcoma (RMS) is a predominant soft tissue tumor in children and adolescents. For high-grade RMS with metastatic involvement, the 3-year overall survival rate is only 25 to 30%. Thus, understanding the regulatory mechanisms involved in promoting the metastasis of RMS is important. Here, we demonstrate for the first time that the SNAIL transcription factor regulates the metastatic behavior of RMS both in vitro and in vivo. SNAIL upregulates the protein expression of EZRIN and AKT, known to promote metastatic behavior, by direct interaction with their promoters. Our data suggest that SNAIL promotes RMS cell motility, invasion and chemotaxis towards the prometastatic factors: HGF and SDF-1 by regulating RHO, AKT and GSK3β activity. In addition, miRNA transcriptome analysis revealed that SNAIL-miRNA axis regulates processes associated with actin cytoskeleton reorganization. Our data show a novel role of SNAIL in regulating RMS cell metastasis that may also be important in other mesenchymal tumor types and clearly suggests SNAIL as a promising new target for future RMS therapies.

Advances in clinicopathology and molecular biology of soft tissue tumors in children

软组织肿瘤包括良性、中间性和恶性。根据形态学可分为梭形细胞、上皮样细胞、圆形细胞、多形性细胞等类型。近年来，因分子生物学技术的发展而使软组织肿瘤的分类及生物行为学的划分出现了一些变化。该文主要对发生于儿童的梭形细胞和圆形细胞软组织肿瘤临床病理学研究进展进行综述。.

Embryonal Rhabdomyosarcoma of the Testis

Rhabdomyosarcoma (RMS), arising from mesenchymal cells, is the most common soft tissue tumour in children and accounts for up to half of all sarcomas. We present a case of a 15 year old male presented with gradually increasing right sided scrotal swelling for last one year. The patient was later diagnosed as right testicular malignancy with metastasis in abdominal lymph nodes and lungs. Inguinal orchidectomy was performed and final histopathological diagnosis was embryonal rhabdomyosarcoma of right testis.&#x0D; Journal of Surgical Sciences (2019) Vol. 23(2): 98-99

СОВЕРШЕНСТВОВАНИЕ ПОДХОДОВ К ЛЕЧЕНИЮ ДЕТЕЙ С ПОВЕРХНОСТНЫМИ МЛАДЕНЧЕСКИМИ ГЕМАНГИОМАМИ ГОЛОВЫ И ШЕИ С ПРИМЕНЕНИЕМ ВЫСОКОЭНЕРГЕТИЧЕСКИХ ЛАЗЕРОВ

Background. Hemangiomas of the skin and mucous membranes, which belong to the group of vascular abnormalities, make up about 50% among other soft tissue tumors in children. Laser coagulation is one of the treatment methods that can provide both a radical removal of superficial hemangiomas and leaving an imperceptible trace.&#x0D; The goal is to increase the effectiveness of treatment of children with superficial infant hemangiomas of the head and neck by substantiating the differentiated selection of a high-energy laser.&#x0D; Methodology. A total of 512 children were included in the study. In accordance with the tasks of the study, the total body of material was divided into four parts: evaluation of the long-term results of the treatment of infant hemangiomas; a comparative study of the effectiveness of two laser systems in the treatment of infantile hemangiomas with varying degrees of elevation over the surrounding skin; assessment of the aesthetic efficiency of using laser technologies against the background of the modernization of the cooling method in the treatment of infantile hemangiomas of the face and neck in children; the study of the social effectiveness of the protocol for choosing a method of treatment of infantile hemangiomas of the head and neck in children.&#x0D; Results. The obtained statistically significant difference in changes on all scales between the two groups mathematically confirmed that the modernized approach makes it possible to obtain higher social effectiveness of the treatment, apparently due to the greater aesthetics of the remaining scars after treatment.&#x0D; Conclusions. A pronounced difference was found in the response of hemangiomas to various types of lasers, the combination of laser-coagulation with constant water cooling has a greater aesthetic effectiveness than the use of periodic ice applications, the social effectiveness of the approach using the protocol for choosing treatment tactics is higher than with standard approaches.

Chemotherapy in Pediatric-Type Soft-Tissue Sarcomas

Soft-tissue sarcoma (STS) is rare and represents approximately 7% of cancers in children and in adolescents less than 20 years of age. Rhabdomyosarcoma (RMS) is most prevalent in children less than 10 years of age and peaks again during adolescence (16–19 years of age). Multi-agent chemotherapy constitutes the mainstay of treatment for RMS. In other non-rhabdomyosarcoma soft-tissue tumours, such as synovial sarcoma, evidence for routine use of chemo-therapy is less robust, and alternative treatment options, including targeted agents and immunotherapy, are being explored. In this review, we focus on chemotherapy for pediatric-type RMS and discuss the advances and challenges in systemic treatment for select non-rhabdomyosarcoma soft-tissue tumours in children and adolescents. We support an increasingly cooperative approach for treating pediatric and adult STS.

Systemic therapy in pediatric-type soft-tissue sarcoma.

Soft-tissue sarcoma (sts) is rare and represents approximately 7% of cancers in children and in adolescents less than 20 years of age. Rhabdomyosarcoma (rms) is most prevalent in children less than 10 years of age and peaks again during adolescence (16-19 years of age). Multi-agent chemotherapy constitutes the mainstay of treatment for rms. In other non-rhabdomyosarcoma soft-tissue tumours, such as synovial sarcoma, evidence for routine use of chemotherapy is less robust, and alternative treatment options, including targeted agents and immunotherapy, are being explored. In this review, we focus on chemotherapy for pediatric-type rms and discuss the advances and challenges in systemic treatment for select non-rhabdomyosarcoma soft-tissue tumours in children and adolescents. We support an increasingly cooperative approach for treating pediatric and adult sts.

Berberine and palmatine inhibit the growth of human rhabdomyosarcoma cells.

A natural isoquinoline alkaloid, berberine, has been known to exhibit anti-tumor activity in various cancer cells via inducing cell cycle arrest. However, it has not been investigated whether berberine and its analogs inhibit the growth of rhabdomyosarcoma (RMS), which is the most frequent soft tissue tumor in children. The present study examined the anti-tumor effects of berberine and palmatine on expansions of three human embryonal RMS cell lines; ERMS1, KYM1, and RD. Intracellular incorporation of berberine was relatively higher than that of palmatine in every RMS cell line. Berberine significantly inhibited the cell cycle of all RMS cells at G1 phase. On the other hand, palmatine only suppressed the growth of RD cells. Both of berberine and palmatine strongly inhibited the growth of tumorsphere of RD cells in three-dimensional culture. These results indicate that berberine derivatives have the potential of anti-tumor drugs for RMS therapy.Abbreviations: ARMS: alveolar rhabdomyosarcoma; ERMS: embryonal rhabdomyosarcoma; RMS: rhabdomyosarcoma.

Proliférations fibroblastiques et myofibroblastiques cutanées de l’enfant

The diagnosis of cutaneous fibroblastic and myofibroblastic proliferations in children remains difficult because the clinical presentation is poorly specific. They represent 12 % of soft tissue tumours in children, extending from benign or reactive entities to lesions with uncertain potential or malignant tumours. The final diagnosis requires the association of clinical data (presentation, age of the patient, location and number of lesions, evolution) and hictological data. Molecular biology is becoming helpful in the classification of such lesions, the final objective remaining the best therapeutic management with the future use of targeted therapies.

Cervical Infantile Fibrosarcoma: a rare cause of paediatric parapharyngeal neck mass

ABSTRACTSoft tissue tumors are not uncommon in childhood and comprise entities that range from common to very rare malignancies. Infantile fibrosarcoma (IFS) is a rare pediatric malignancy mainly seen in the first two years of life. The data about the incidence of infantile fibrosarcoma occurring in the neck in the Indian subcontinent is scarce. To the best of our knowledge, only one case of infant cervical IFS has been reported previously in the Indian subcontinent. We present another case of an eight-year-old male patient with a rapidly growing mass on the left side of the neck. He was successfully treated with a combined modality of surgery and chemotherapy with a good outcome. Among the soft tissue tumors of childhood, IFS is a rare entity. It has a good prognosis and lesser chance of distant metastasis as compared to adult fibrosarcoma. Though surgical excision is the mainstay of treatment, chemotherapy also has a significant role in the treatment of primary tumor and metastasis. We discuss the stated case to bring to the notice this uncommon cause, which can be considered as a differential diagnosis of upper cervical swellings. A better understanding of this entity would help in early diagnosis and aggressive treatment, reducing the overall morbidity and mortality.