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Desmoid tumor

Caterina Rebecchini, Antoine Nobile, Simonetta Piana, Rossella Sarro, Bettina Bisig, Sykiotis P Gerasimos, Chiara Saglietti, Maurice Matter, Laura Marino, Massimo Bongiovanni
Various histological variants of papillary thyroid carcinoma have been reported, some with clinical implications, some with peculiar, sometimes misleading morphologies. One of these rare and poorly characterized variants is papillary thyroid carcinoma with nodular fasciitis-like stroma, of which fewer than 30 cases have been documented, mostly as isolated reports. It is a dual tumor comprising a malignant epithelial proliferation that harbors typical features of conventional papillary thyroid carcinoma, admixed with a prominent mesenchymal proliferation resembling nodular fasciitis or fibromatosis...
October 7, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
M Creuzé, P Afchain, A Munck, J Viala, A Bonnard, V Bertrand
Desmoid tumors (DT) are rare and nonmetastasizing fibroblastic neoplasms, characterized by local invasiveness. They occur sporadically or arise in the context of familial adenomatous polyposis (FAP; 5-10% of cases). Most cases develop sporadically in young adults, but some cases also occur in children. We report the case of an adolescent girl with FAP and DT, and we discuss the therapeutic strategies. An adolescent girl with FAP underwent surgery at the age of 14 years with total proctocolectomy. She had a neo-mutation in the APC gene at codon 1068, which is not usually associated with DT...
September 27, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
M S Gentile, A Jacobson, H Wang, S Goldberg, E Choy, J T Mullen, F J Hornicek, Y L E Chen, T F DeLaney
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Chiara E Mussi, Piergiuseppe Colombo, Chiara Lo Russo, Anaid Kasangian, Ferdinando Cananzi, Andrea Marrari, Emanuela Morenghi, Rita De Sanctis, Vittorio Quagliuolo
PURPOSE: Before the wait-and-see policy became the standard approach in abdominal wall desmoid tumors, surgery was performed on a systematic basis. Surgery remains indicated for progressing tumors but its extent is debatable. The abdominal wall is a common site of origin of sporadic desmoids, usually associated with a favorable prognosis. We analyzed the results of surgery at this specific site. METHODS: Data from 33 patients affected by sporadic desmoid tumors of the abdominal wall (31 primary, 2 recurrent) consecutively treated at our cancer center between January 2000 and September 2013 were retrospectively studied...
September 3, 2016: Tumori
Luyuan Li, John N Jensen, Sara Szabo, Peter VanTuinen, Sean M Lew
Desmoid tumors, also known as aggressive fibromatosis, are locally infiltrating musculoaponeurotic neoplasms arising in connective tissues. Desmoid tumors may be associated with familial adenomatous polyposis (FAP), a genetic disorder that presents with hundreds to thousands of precancerous colorectal polyps. The authors report the case of an 18-month-old boy who underwent resection of a right temporal desmoid tumor (initially diagnosed as cranial fasciitis) and developed a bilateral frontoparietal calvarial desmoid tumor 2 years later...
September 16, 2016: Journal of Neurosurgery. Pediatrics
Takuya Nagata, Yusuke Demizu, Tomoyuki Okumura, Shinichi Sekine, Naoki Hashimoto, Nobukazu Fuwa, Tomoaki Okimoto, Yutaka Shimada
BACKGROUND: Desmoid tumors, which are associated with familial adenomatous polyposis (FAP), tend to occur frequently in the abdominal wall and mesentery. Currently, there are no recognized treatments other than surgery, and frequent surgeries result in gastrointestinal obstructions and functional gastrointestinal disorders. CASE PRESENTATION: After surgery that was performed on a 39-year-old patient with FAP, we performed a second tumor excision which was the procedure used for frequently occurring mesenteric desmoid tumors...
2016: World Journal of Surgical Oncology
Jiwon Sarah Park, Yves-Paul Nakache, Jeremy Katz, Robert D Boutin, Robert J Steffner, Arta M Monjazeb, Robert J Canter
BACKGROUND: Surgical resection of desmoid tumors has traditionally been the mainstay of therapy, but this is a potentially morbid approach with high rates of recurrence. Given increasing reports of active surveillance in this disease, we sought to evaluate our experience with conservative management hypothesizing this would be an effective strategy. MATERIALS AND METHODS: Using a prospectively maintained database of sarcoma patients from 2008 to 2015, we identified 47 patients with a diagnosis of desmoid tumor from all anatomic sites...
September 2016: Journal of Surgical Research
S A Begum, T S Chowdhury, T Mahmud, S Chowdhury, T A Chowdhury, S J Urmi, S Khatun, A Nessa, N Fatema
A 25 years married women having one child delivered vaginaly presented in the department of Obs & Gynae, Bangabandhu Sheikh Mujib Medical University, Bangladesh on April 2013 with pelvic pain and discomfort. No history of previous pelvic trauma was present. Patient examination showed a isolated mass in the right lower abdomen, right adnexa, extending to the pelvic wall upto lower end of ureter. Tumor markers were within normal limit. Intravenous pyelogram (IVP) showed mild right hydroureter and hydronephrosis with obstruction at the lower end of ureter...
July 2016: Mymensingh Medical Journal: MMJ
Deepa Bhat, Vanessa Wear, Elliot Weisenberg, Rosalinda Alvarado
Desmoid-type fibromatosis of the breast (also referred to as desmoid tumor or aggressive fibromatosis) is exceedingly rare. Although it does not metastasize, desmoid-type fibromatosisis is frequently locally aggressive. Recurrence is common (up to 35%), even after presumed total excision of the primary tumor [1]. The breast is an unusual location for the development of this tumor, with relatively few cases reported in the literature. We report a case of desmoid-type fibromatosis in the breast in a 31-year-old female who presented with a four-month history of a palpable left breast mass...
August 1, 2016: Breast Disease
Phanchaporn Wongmaneerung, Areewan Somwangprasert, Kirati Watcharachan, Chagkrit Ditsatham
BACKGROUND: Desmoid tumor of the breast is very rare and locally aggressive but has no distant metastasis. Bilateral lesions are extremely rare, found in only 4% of patients. Two cases of bilateral desmoid tumor of the breast are reported. The clinical presentation, diagnosis, imaging, treatment, and follow-up outcomes of recurrence as well as a brief literature review are provided. CASE REPORTS: Case 1 is a 31-year-old woman who presented with nipple retraction...
2016: International Medical Case Reports Journal
Jalal Vahedian Ardakani, Ali Zare Mehrjardi, Massoud Baghai Wadji, Amir Saraee
Desmoid tumors are locally aggressive and non-metastatic neoplasms with a high rate of recurrence. Desmoid tumors of the pancreas are, however, very rare, and only a few cases have been reported in the literature. This paper reports an anecdotal case of a diffuse pancreatic desmoid tumor with the involvement of the pancreatic head, body, and-partially-tail. The patient underwent the Whipple procedure and subtotal pancreatectomy. Histopathological assessment showed that the tissues were partly positive for smooth muscle actin, but not for S100 or PanCK...
August 2016: Indian Journal of Surgery
Danique L M van Broekhoven, Dirk J Grünhagenl, Thijs van Dalen, Frits van Coevorden, Han J Bonenkamp, Lukas B Been, Marc H A Bemelmans, Sander D S Dijkstra, Chiara Colombo, Alessandro Gronchi, Cornelis Verhoef
BACKGROUND: The efficacy of the classical treatment modalities surgery and radiotherapy in the treatment of aggressive fibromatosis is presently disputed and there is a shift towards a more conservative approach. The aim of the present study is to objectify tumor growth in patients with extra-abdominal or abdominal wall aggressive fibromatosis, while adhering to a "watchful waiting" policy. Other objectives are to investigate quality of life and to identify factors associated with tumor growth, in particular the relation with the presence of a CTNNB1-gene mutation in the tumor...
2016: BMC Cancer
Mohamed Fawzy, Mohamed Sedky, Hossam ElZomor, Magdy El Sherbiny, Emad Salama, Ahmed Mahdy
BACKGROUND: Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence. AIM OF THE STUDY: The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients. PATIENTS AND METHODS: The study reviewed 66 NRSTS patients who presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, between January 2008 and December 2013...
August 19, 2016: Journal of Pediatric Hematology/oncology
Valerie P Grignol, Raphael Pollock, John Harrison Howard
Desmoid tumors are rare, comprising 3% of soft tissue tumors. Surgical resection has been the standard of care; however, this has begun to evolve into a movement of watchful waiting as observational studies have shown long-term stability of many tumors without treatment and even spontaneous regression in 5% to 10% of cases. When surgical therapy is used, wide local excision with microscopically negative margins is the goal of resection but should not be at the expense of organ or limb function. Recurrence rates after surgical resection are approximately 20%; a variety of multimodal therapies are useful in controlling disease...
October 2016: Surgical Clinics of North America
Jennifer Hammer, Daniel Léonard, François Chateau, Nora Abbes Orabi, Olga Ciccarelli, Radu Bachmann, Christophe Remue, Benoît Lengelé, Alex Kartheuser
INTRODUCTION: Desmoid tumors are rare proliferative and invasive benign lesions. They can be sporadic, but in most instances, desmoid tumors develop in the context of Gardner's syndrome with principal localization in the abdominal cavity and abdominal wall. CASE-REPORT: We report the case of a 24-year-old female presenting Gardner's syndrome with a symptomatic abdominal wall desmoid tumor. Lack of response to medical treatment led to surgical management consisting in a complete resection and parietal reconstruction with a biologic mesh...
August 18, 2016: Acta Chirurgica Belgica
Haitham H Khalil, Marco N Malahias, Balapathiran Balasubramanian, Madava G Djearaman, Babu Naidu, Melvin F Grainger, Maninder Kalkat
BACKGROUND: Management of complex thoracic defects post tumor extipiration is challenging because of the nature of pathology, the radical approach, and the insertion of prosthetic material required for biomechanical stability. Wound complications pose a significant problem that can have detrimental effect on patient outcome. The authors outline an institutional experience of a multidisciplinary thoracic oncoplastic approach to improve outcomes. METHODS: Prospectively collected data from 71 consecutive patients treated with chest wall resection and reconstruction were analyzed (2009-2015)...
July 2016: Plastic and Reconstructive Surgery. Global Open
Akihiko Matsumine, Masaya Tsujii, Tomoki Nakamura, Kunihiro Asanuma, Takao Matsubara, Takuya Kakimoto, Yuki Yada, Akinori Takada, Noriko Ii, Yoshihito Nomoto, Akihiro Sudo
BACKGROUND: When a soft tissue sarcoma (STS) is located at the distal part of an extremity and involves the tendon, a wide excision usually causes severe functional disability. We therefore developed a minimally invasive surgical technique using intraoperative electron-beam radiotherapy (IOERT) to reduce the incidence of post-operative functional disability in patients with peri-/intra-tendinous STS. We assessed the clinical outcomes of the novel minimally invasive surgery. METHODS: The study population included five patients who received treatment for distal extremity STSs...
2016: World Journal of Surgical Oncology
Pooja J Sheth, Spencer Del Moral, Breelyn A Wilky, Jonathan C Trent, Jonathan Cohen, Andrew E Rosenberg, H Thomas Temple, Ty K Subhawong
OBJECTIVE: Imaging criteria for measuring the response of desmoid fibromatosis to systemic therapy are not well established. We evaluated a series of patients with desmoids who underwent systemic therapy to document magnetic resonance imaging (MRI) features associated with a positive clinical response. MATERIALS AND METHODS: This Institutional Review Board-approved retrospective study included 23 patients (mean age 40.5) with 29 extra-abdominal tumors. Therapeutic regimens included cytotoxic chemotherapy (n = 19), targeted therapy (n = 3), and nonsteroid anti-inflammatory drugs (NSAIDS; n = 1)...
October 2016: Skeletal Radiology
Austin D Williams, Kimberly Heightchew, Veeraiah Siripirapu
INTRODUCTION: Intra-abdominal desmoid tumors (DTs) are a rare and anatomically diverse group of locally-aggressive, benign neoplasms. They are often difficult to diagnose, even in patients who possess risk factors for the disease. Even after a diagnosis has been reached, the optimal therapy is often not well-defined. PRESENTATION OF CASE: The case discussed of a 33-year old male with a giant intra-abdominal desmoid is an example of both the diagnostic and therapeutic dilemmas that arise when confronted with a patient with a DT...
2016: International Journal of Surgery Case Reports
(no author information available yet)
INTRODUCTION: Retroperitoneal soft tissue sarcomas (RPS) are rare tumors. Surgery is the mainstay of curative therapy, but local recurrence is common. No recommendations concerning the best management of recurring disease have been developed so far. Although every effort should be made to optimize the initial approach, recommendations to treat recurring RPS will be helpful to maximize disease control at recurrence. METHODS: An RPS transatlantic working group was established in 2013...
October 2016: Annals of Surgical Oncology
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