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Infantile fibrosarcoma

Dean Pavlick, Alexa B Schrock, Denise Malicki, Philip J Stephens, Dennis J Kuo, Hyunah Ahn, Brian Turpin, Kamran Badizegan, 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
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
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
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
Amy C Kite, Lora M Rotstein, Jennifer L Rhodes
No abstract text is available yet for this article.
2016: Eplasty
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
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
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
Sungeeta Agrawal, Lisa Swartz Topor
No abstract text is available yet for this article.
May 2, 2016: Rhode Island Medical Journal
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
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
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
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
Hidetaka Yamamoto, Akihiko Yoshida, Kenichi Taguchi, Kenichi Kohashi, Yui Hatanaka, Atsushi Yamashita, Daisuke Mori, Yoshinao Oda
AIMS: The aim of this study was to elucidate the pathological features of inflammatory myofibroblastic tumour (IMT) with gene rearrangement other than ALK. METHODS AND RESULTS: We investigated anaplastic lymphoma kinase (ALK), ROS1, ETV6, NTRK3 and RET in 36 cases of IMT by using immunohistochemical (IHC) staining, fluorescence in-situ hybridization, and reverse transcription polymerase chain reaction (RT-PCR). IHC staining showed ALK and ROS1 to be positive in 22 of 36 (61...
July 2016: Histopathology
Monica Brenca, Sabrina Rossi, Maurizio Polano, Daniela Gasparotto, Lucia Zanatta, Dominga Racanelli, Laura Valori, Stefano Lamon, Angelo Paolo Dei Tos, Roberta Maestro
Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. The vast majority of GISTs are driven by oncogenic activation of KIT, PDGFRA or, less commonly, BRAF. Loss of succinate dehydrogenase complex activity has been identified in subsets of KIT/PDGFRA/BRAF-mutation negative tumours, yet a significant fraction of GISTs are devoid of any of such alterations. To address the pathobiology of these 'quadruple-negative' GISTs, we sought to explore the possible involvement of fusion genes...
March 2016: Journal of Pathology
Bita Geramizadeh, Bijan Khademi, Mehran Karimi, Golsa Shekarkhar
Infantile fibrosarcoma of head and neck is rare and the presence of this tumor in ethmoid sinus is even more uncommon. To the best of our knowledge, <5 cases have been reported in the last 20 years in the English literature, so far, only one of which has been infantile type in a 15 months old girl. In this case report, we will explain our experience with a rare case of infantile fibrosarcoma originating from ethmoid sinus in a 5-year-old boy who presented with dyspnea and epistaxis. After biopsy, it was diagnosed as fibrosarcoma of sinus origin...
May 2015: Journal of Oral and Maxillofacial Pathology: JOMFP
Ali Nael, William W Wu, Lisa Shane, Nagesh Rao, Theodore Zwerdling
Neonatal intestinal masses with spindle cell morphology have broad differential diagnoses and require a multidisciplinary approach to make the final diagnosis. Spindle cell masses with heterotopic cartilage in the gastrointestinal tract are very rare, and, to our knowledge, have not previously been reported in the neonate. Here we present a case of intestinal primitive spindle cell neoplasm with extensive heterotopic cartilage that manifested initially as acute abdomen in a 6-day-old term infant. Plain radiography demonstrated pneumoperitoneum, prompting diagnostic laparotomy that identified a perforated mass involving the midileum...
July 2016: Pediatric and Developmental Pathology
Hikmet Zeytun, Mehmet Hanifi Okur, Erol Basuguy, Serkan Arslan, Bahattin Aydogdu, Gul Turkcu, Mehmet Serif Arslan
Infantile fibrosarcoma is a very rare soft tissue tumor that originates most commonly in the body and extremities. We present a neonate with an infantile fibrosarcoma that originated in the ileocecal region and was detected incidentally without symptoms. This is the first case of fibrosarcoma reported in the ileocecal region.
January 2016: Pediatric Surgery International
Amirataollah Hiradfar, Tala Pourlak, Davoud Badebarin
INTRODUCTION: Pulmonary fibrosarcoma has been an extremely rare tumor in children. Wide surgical resection of infantile fibrosarcoma would be the treatment of choice. CASE PRESENTATION: Post-operative chemotherapy has shown the benefit in the cases of residual disease after initial surgery and metastatic disease in the literature. We have presented the case of a 70-days old male child with primary infantile fibrosarcoma of the left lung and distant metastasis of skull...
May 2015: Iranian Journal of Cancer Prevention
Ryu Yanagisawa, Masahiko Noguchi, Kenya Fujita, Kazuo Sakashita, Kenji Sano, Yoshifumi Ogiso, Yoshihiko Katsuyama, Yoshiaki Kondo, Kazutoshi Komori, Ikuko Fujihara, Rei Kitamura, Takehiko Hiroma, Tomohiko Nakamura
Clinical and radiological diagnosis of infantile fibrosarcoma (IFS) is challenging because of its similarity to vascular origin tumors. Treatment involves complete resection. Although chemotherapy may allow more conservative resection, treatment guidelines are not strictly defined. One IFS patient with an unresectable tumor had disease progression during chemotherapy. A primary tumor sample showed high VEGFR-1/2/3 and PDGFR-α/β expression. After pazopanib therapy, most tumor showed necrosis within 29 days and could be removed completely, with no relapse in 8 months post-resection...
February 2016: Pediatric Blood & Cancer
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