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Infantile fibrosarcoma

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https://www.readbyqxmd.com/read/28525423/congenital-infantile-fibrosarcoma-associated-with-a-lipofibromatosis-like-component-one-train-may-be-hiding-another
#1
Romain Swiadkiewicz, Louise Galmiche, Kahina Belhous, Olivia Boccara, Sylvie Fraitag, Florence Pedeutour, Bérangère Dadone, Jacques Buis, Arnaud Picard, Daniel Orbach, Natacha Kadlub
Congenital infantile fibrosarcoma (CIFS) is a soft tissue sarcoma of infants mainly involving lower extremities and usually developing during the first year of life. At another end of the spectrum of pediatric fibroblastic lesions, lipofibromatosis is a rare benign infiltrative soft tissue tumor that affects children. The authors report in this study a particular presentation with a CIFS surrounded by lipofibromatosis-like areas. The presence of a surrounding benign tumor confused and delayed CIFS diagnosis...
June 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28510278/tfg-met-fusion-in-an-infantile-spindle-cell-sarcoma-with-neural-features
#2
Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu
An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression...
May 16, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28509377/h3-1-k36m-mutation-in-a-congenital-onset-soft-tissue-neoplasm
#3
Kristin D Kernohan, David Grynspan, Raveena Ramphal, Eric Bareke, You Chang Wang, Elizabeth Nizalik, Jiannis Ragoussis, Nada Jabado, Kym M Boycott, Jacek Majewski, Sarah L Sawyer
We describe a patient who presented with a congenital soft tissue lesion initially diagnosed as infantile fibromatosis at 15 days of age. Unusually, the mass demonstrated malignant progression leading to death at 20 months of age. Biological progression to malignancy is not known to occur in fibromatosis, and fibrosarcoma is not known to progress from a benign lesion. Whole-exome sequencing of the tumor identified a driver mutation in histone H3.1 at lysine (K)36. Our findings support the link between oncohistones and infantile soft tissue tumors and provide additional evidence for the oncogenic effects of p...
May 16, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28478739/translocation-renal-cell-carcinoma-in-a-child-previously-treated-for-infantile-fibrosarcoma
#4
Jing Ma, Ci Pan, Minzhi Yin
We report a child who developed a TFE3/Xp11.2 translocation renal cell carcinoma (RCC) when he was 3 years and 3 months old, after previous treatment for infantile fibrosarcoma (IFS). When he was 3 months old, a left axilla mass has been detected, which was tan and solid, was 1.5 cm in greatest dimension, and composed of sheets of spindle cells that was positive for vimentin and fibronectin. Fluorescence in situ hybridization showed positive result in ETV6 gene rearrangements. The final diagnosis was IFS...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28371408/two-cases-of-humoral-hypercalcemia-of-malignancy-complicating-infantile-fibrosarcoma
#5
Ryan Hirschfeld, Jennifer J G Welch, Douglas J Harrison, Robin Kremsdorf, Anjulika Chawla
We report two infants with infantile fibrosarcoma (IFS) complicated by severe hypercalcemia. Assessment demonstrated suppressed parathyroid hormone and 1,25-dihydroxyvitamin D levels with elevated circulating levels of parathyroid hormone related protein, indicating the diagnosis of humoral hypercalcemia of malignancy (HHM). HHM is a paraneoplastic syndrome rarely associated with pediatric malignancies. Hypercalcemia manifested clinically with neurologic symptoms and soft tissue calcium deposition and required aggressive management with intravenous fluids, diuretics, and supplemental electrolytes...
March 29, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28317309/infantile-fibrosarcoma-treated-with-postoperative-vincristine-and-dactinomycin
#6
Hiroki Yoshihara, Yuri Yoshimoto, Yosuke Hosoya, Daisuke Hasegawa, Takafumi Kawano, Akiko Sakoda, Hajime Okita, Atsushi Manabe
Infantile fibrosarcoma is a non-rhabdomyosarcoma soft-tissue sarcoma that occurs in infancy and which has a relatively good prognosis. A vincristine and dactinomycin (VA) regimen has been shown to be effective, although the duration of chemotherapy has not been well defined. We describe the case of a 4-month-old boy with a mass at the left dorsum of the foot who was diagnosed with infantile fibrosarcoma after resection of the tumor, the margin of which was macroscopically positive. VA treatment was carried out with careful monitoring of response and adverse effects...
March 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28256570/recurrent-bcor-internal-tandem-duplication-and-bcor-or-bcl6-expression-distinguish-primitive-myxoid-mesenchymal-tumor-of-infancy-from-congenital-infantile-fibrosarcoma
#7
Teresa Santiago, Michael R Clay, Sariah J Allen, Brent A Orr
Primitive myxoid mesenchymal tumor of infancy is a rare sarcoma that preferentially affects infants. It can be locally aggressive and rarely metastasizes, but the long-term outcome of children with this tumor is mostly unknown. Histologically, it is characterized by primitive cells with abundant myxoid stroma. Internal tandem duplication of B-cell CLL/lymphoma 6 (BCL6)-interacting co-repressor (BCOR) exon 15 has recently been described in clear cell sarcoma of kidney, central nervous system high-grade neuroepithelial tumor with BCOR alteration, and primitive myxoid mesenchymal tumor of infancy...
June 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28097808/identification-of-ntrk-fusions-in-pediatric-mesenchymal-tumors
#8
Dean Pavlick, Alexa B Schrock, Denise Malicki, Philip J Stephens, Dennis J Kuo, Hyunah Ahn, Brian Turpin, Justin M Allen, Mark Rosenzweig, Kamran Badizadegan, Jeffrey S Ross, Vincent A Miller, Victor Wong, Siraj M Ali
BACKGROUND: NTRK fusions are known oncogenic drivers and have recently been effectively targeted by investigational agents in adults. We sought to assess the frequency of NTRK fusions in a large series of pediatric and adolescent patients with advanced cancers. PROCEDURE: Genomic profiles from 2,031 advanced cancers from patients less than 21 years old who were assayed with comprehensive genomic profiling were reviewed to identify NTRK fusions. RESULTS: Total of nine cases (0...
January 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28018804/extracalvarial-composite-infantile-myofibromatosis-case-report-and-literature-review
#9
Alexander Ivanov, Tibor Valyi-Nagy, Dimitrios Nikas
Infantile soft tissue tumors of the head are very rare and the majority of them are myofibromas. The authors present the case of a 1-day-old boy with a scalp tumor with several distinct histopathological features including myofibroma, hemangiopericytoma, and fibrosarcoma consistent with the diagnosis of composite infantile myofibromatosis. Genetic testing was negative for trisomy 17, translocation (12; 15), FUS, and ETV6 translocations. Despite the ominous histopathological features, the clinical course was benign...
December 2016: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/27910123/congenital-infantile-fibrosarcoma-mimicking-a-cutaneous-vascular-lesion-a-case-report-and-review-of-the-literature
#10
Tyler Enos, Gregory A Hosler, Naseem Uddin, Adnan Mir
Congenital infantile fibrosarcoma (CIFS) is a rare neoplasm of infancy that occurs most frequently in the extremities, and when presenting in the skin, may sometimes resemble infantile hemangiomas or other vascular lesions. Clinically, these tumors differ from hemangiomas in the time of onset, morphology, and growth pattern and must be evaluated histologically for definitive diagnosis. We describe an infant with a neoplasm involving the distal left forearm initially presumed to be a vascular lesion after evaluation by two separate ultrasound studies...
February 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/27698854/misdiagnosed-infantile-rhabdomyofibrosarcoma-a-case-report
#11
Tao Pan, Ken Chen, Run-Song Jiang, Zheng-Yan Zhao
Infantile rhabdomyofibrosarcoma is a rare form of soft-tissue tumor often associated with difficulties in diagnosis. The disease is positioned intermediately between rhabdomyosarcoma and infantile fibrosarcoma in terms of clinical presentation, immunohistochemistry, behavior, morphology and ultrastructural features. Reports of rhabdomyofibrosarcoma cases are limited in the literature. The present case describes a 26-month-old female who presented with a slowly progressive, soft-tissue mass in the right chest wall...
October 2016: Oncology Letters
https://www.readbyqxmd.com/read/27602180/a-case-report-of-an-invasive-infantile-fibrosarcoma-of-the-forearm
#12
Amy C Kite, Lora M Rotstein, Jennifer L Rhodes
No abstract text is available yet for this article.
2016: Eplasty
https://www.readbyqxmd.com/read/27399243/pediatric-fibroblastic-and-myofibroblastic-tumors-a-pictorial-review
#13
REVIEW
Kiran M Sargar, Elizabeth F Sheybani, Archana Shenoy, John Aranake-Chrisinger, Geetika Khanna
Pediatric fibroblastic and myofibroblastic tumors are a relatively common group of soft-tissue proliferations that are associated with a wide spectrum of clinical behavior. These tumors have been divided into the following categories on the basis of their biologic behavior: benign (eg, myositis ossificans, myofibroma, fibromatosis colli), intermediate-locally aggressive (eg, lipofibromatosis, desmoid fibroma), intermediate-rarely metastasizing (eg, inflammatory myofibroblastic tumors, infantile fibrosarcoma, low-grade myofibroblastic sarcoma), and malignant (eg, fibromyxoid sarcoma, adult fibrosarcoma)...
July 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/27398324/infantile-fibrosarcoma-of-tongue-a-rare-tumor
#14
Fazal I Wahid, Bakht Zada, Gul Rafique
Infantile fibrosarcoma is very rare tumor and seldom occurs in oral cavity. Overall good prognosis is reported in more than 80% cases. We present a 5-month old female patient with swelling of the tongue for four months. This was excised completely. Histopathology and immunohistochemistry revealed it as infantile fibrosarcoma of grade II.
July 2016: APSP Journal of Case Reports
https://www.readbyqxmd.com/read/27385891/unusual-case-of-infantile-fibrosarcoma-evaluated-on-f-18-fluorodeoxyglucose-positron-emission-tomography-computed-tomography
#15
Akshay Bedmutha, Natasha Singh, Divya Shivdasani, Nitin Gupta
Infantile fibrosarcoma (IFS) is a rare soft-tissue sarcoma originating from extremities and occasionally from axial soft tissue. The prognosis is good with favorable long-term survival. It is rarely metastasizing tumor, the chances being lesser with IFS originating from extremities. Use of neoadjuvant chemotherapy (NACT) as a treatment regime further reduces the chances of local relapse and distant metastasis. The organs commonly affected in metastatic IFS are lungs and lymph nodes. We report an unusual case of an IFS originating from extremity, which received NACT, yet presented with an early metastatic disease involving soft tissues and sparing lungs and lymph nodes, as demonstrated on fluorodeoxyglucose positron emission tomography-computed tomography...
July 2016: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/27128515/hypercalcemia-of-malignancy-in-a-newborn-with-infantile-fibrosarcoma
#16
Sungeeta Agrawal, Lisa Swartz Topor
No abstract text is available yet for this article.
May 2, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27093299/infantile-fibrosarcoma-with-ntrk3-etv6-fusion-successfully-treated-with-the-tropomyosin-related-kinase-inhibitor-loxo-101
#17
Ramamoorthy Nagasubramanian, Julie Wei, Paul Gordon, Jeff C Rastatter, Michael C Cox, Alberto Pappo
Infantile fibrosarcoma (IFS) is a rare pediatric cancer typically presenting in the first 2 years of life. Surgical resection is usually curative and chemotherapy is active against gross residual disease. However, when recurrences occur, therapeutic options are limited. We report a case of refractory IFS with constitutive activation of the tropomyosin-related kinase (TRK) signaling pathway from an ETS variant gene 6-neurotrophin 3 receptor gene (ETV6-NTRK3) gene fusion. The patient enrolled in a pediatric Phase 1 trial of LOXO-101, an experimental, highly selective inhibitor of TRK...
August 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27065699/chest-wall-infantile-fibrosarcomas-a-rare-presentation
#18
Anand Pandey, Shiv Narain Kureel, Ravi Pandharinath Bappavad
Infantile fibrosarcoma is rare and represents less than 1 % of all childhood cancers. Commonly, it arises in the limbs followed by the trunk and head and neck. We present a rare case of infantile fibrosarcoma of trunk at two different sites in a newborn with brief review of the relevant literature.
March 2016: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/27020209/congenital-mesoblastic-nephroma-a-study-of-19-cases-using-immunohistochemistry-and-etv6-ntrk3-fusion-gene-rearrangement
#19
Dina El Demellawy, Caitlin A Cundiff, Ahmed Nasr, John A Ozolek, Nancy Elawabdeh, Shelley A Caltharp, Pourya Masoudian, Katrina J Sullivan, Joseph de Nanassy, Bahig M Shehata
Mesoblastic nephroma (MN) is the most common renal tumour in the first 3 months of life and accounts for 3-5% of all paediatric renal neoplasms. To further understand the morphological variants of MN, we identified 19 cases of MN (five classic, eight cellular and six mixed) and examined each case for markers known to be important in urogenital embryological development (PAX8, WT1 and RCC), stem cell associated markers (Oct 4, CD34 and c-kit), muscle/myofibroblastic markers (muscle specific actin, calponin and h-caldesmon), aberrant transcription factors, cell cycle regulation and other oncogenic proteins (p16, cyclin D1 and beta-catenin)...
January 2016: Pathology
https://www.readbyqxmd.com/read/26849118/conservative-strategy-in-infantile-fibrosarcoma-is-possible-the-european-paediatric-soft-tissue-sarcoma-study-group%C3%A2-experience
#20
Daniel Orbach, Bernadette Brennan, Angela De Paoli, Soledad Gallego, Peter Mudry, Nadine Francotte, Max van Noesel, Anna Kelsey, Rita Alaggio, Dominique Ranchère, Gian Luca De Salvo, Michela Casanova, Christophe Bergeron, Johannes H M Merks, Meriel Jenney, Michael C G Stevens, Gianni Bisogno, Andrea Ferrari
BACKGROUND: Infantile fibrosarcoma (IFS) is a very rare disease occurring in young infants characterised by a high local aggressiveness but overall with a favourable survival. To try to reduce the total burden of therapy, the European pediatric Soft tissue sarcoma Study Group has developed conservative therapeutic recommendations according to initial resectability. MATERIAL AND METHODS: Between 2005 and 2012, children with localised IFS were prospectively registered...
April 2016: European Journal of Cancer
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