1,435 results on '"desmoid tumor"'
Search Results
202. Differentiation of intra-abdominal desmoid tumor from peritoneal seeding based on CT and/or 18F-FDG PET-CT in patients with history of cancer surgery.
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Suh, June, Kang, Hyo-Jin, and Kim, Se Hyung
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DESMOID tumors , *SOWING , *ONCOLOGIC surgery , *CHI-squared test , *FLUORODEOXYGLUCOSE F18 , *SOLAR plexus , *FISHER exact test , *PSOAS muscles - Abstract
Purpose: To investigate differential imaging features of intra-abdominal desmoid tumors and peritoneal seeding in patients with history of cancer surgery. Methods: Thirty-two patients who had a single pathologically proven intra-peritoneal lesion that developed after cancer surgery were enrolled between January 2000 and June 2019. There were 16 desmoid tumors and 16 peritoneal seeding lesions. Portal phase CT and/or 18F-FDG PET findings were analyzed by two radiologists in consensus for the following items: location, size, shape, margin, contour, homogeneity, necrosis, adjacent organ invasion, calcification, intra-lesional fat, peritoneal infiltration, mass effect, and degree of enhancement. Hounsfield units (HU) and maximum standardized uptake values (SUVmax) of the lesions were measured. Imaging findings were compared using the Chi square test, Fisher's exact test, and student t test. Results: Desmoid tumors frequently showed well-defined margins (9/16) and smooth contours (12/16), whereas peritoneal seeding had ill-defined margins (13/16) and lobulated contours (11/16) (P = 0.028 and 0.013, respectively). Intra-lesional fat was found more frequently in desmoid tumors (7/16) than peritoneal seeding (1/16) (P = 0.014). Desmoid tumors showed iso-attenuation (13/16) compared to psoas muscle in portal phase, while peritoneal seeding depicted high attenuation (12/16) (P = 0.002). Mean HU was significantly lower in desmoid tumors (64.3) than peritoneal seeding lesions (95.1) (P = 0.001). However, the mean SUVmax of desmoid tumors (4.1) did not significantly differ from peritoneal seeding lesions (5.2) (P = 0.519). Conclusion: Several CT features including iso-attenuation in portal phase and presence of intra-lesional fat can be helpful in differentiating desmoid tumors from peritoneal seeding in patients with history of intra-abdominal cancer surgery. [ABSTRACT FROM AUTHOR]
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- 2020
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203. Maksillada Görülen Agresif Fibrömatözis, Vaka Sünümü ve Literatür İncelemesi.
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Erdem, Murat Kaan, Evli, Cengiz, Erdem, Erdal, and Kolsuz, Mehmet Eray
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Copyright of Osmangazi Journal of Medicine / Osmangazi Tip Dergisi is the property of Eskisehir Osmangazi University and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2020
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204. Certain risk factors for patients with desmoid tumors warrant reconsideration of local therapy strategies.
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Bishop, Andrew J., Landry, Jace P., Roland, Christina L., Ratan, Ravin, Feig, Barry W., Moon, Bryan S., Zarzour, Maria A., Wang, Wei‐Lien, Lazar, Alexander J., Lewis, Valerae O., Torres, Keila E., Guadagnolo, B. Ashleigh, and Wang, Wei-Lien
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DESMOID tumors , *COMBINED modality therapy - Abstract
Background: The objective of this study was to evaluate treatment outcomes for patients with desmoid tumors (DTs) receiving local therapy with surgery alone, radiation therapy (RT) alone, or combined modality therapy (RT and surgery).Methods: This was a cross-sectional cohort study of 412 patients with nonmesenteric DTs who received local therapy at the authors' institution between 1965 and 2018.Results: The median follow-up time was 95 months (range, 1-509 months). Local recurrence occurred in 127 patients (31%) at a median time of 21 months (interquartile range, 12-38 months). The 5-year local control (LC) rate was 67%. Patient or tumor factors that were significantly associated with poorer 5-year LC in a multivariable analysis included an age ≤ 30 years (57% vs 75% for an age > 30 years; hazard ratio [HR], 1.73; P = .004), an extremity location (57% vs 71% for a nonextremity location; HR, 1.77; P = .004), and large tumors (59% for >10 cm [HR, 2.17; P = .004] and 65% for 5.1-10 cm [HR, 1.71; P = .02] vs 76% for ≤5 cm). Subset analyses of these high-risk patients revealed no local therapy strategy to be superior for young patients ≤ 30 years old (HR for surgery, 1.42; P = .33; HR for RT, 1.36; P = .38) or for large tumors > 10 cm (HR for surgery, 1.55; P = .46; HR for RT, 0.91; P = .91). However, for patients with extremity tumors, surgery alone was significantly associated with inferior LC (HR for surgery, 5.15; P < .001; HR for RT, 1.51; P = .38).Conclusions: Local therapy provides durable tumor control in the majority of patients with DTs. However, young patients, patients with an extremity location, and patients with large tumors are at increased risk of recurrence. When active treatment is indicated, systemic therapy should perhaps be considered as a first-line option in these high-risk subsets. Prospective multi-institutional studies evaluating this strategy are warranted. [ABSTRACT FROM AUTHOR]- Published
- 2020
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205. Intra‐abdominal desmoid tumor after laparoscopic low anterior resection for rectal cancer: A case report.
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Maemoto, Ryo, Miyakura, Yasuyuki, Tamaki, Sawako, Takahashi, Jun, Takayama, Noriya, Fukuda, Rintaro, Ishikawa, Hideki, Tsujinaka, Shingo, Lefor, Alan K, and Rikiyama, Toshiki
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DESMOID tumors , *ONCOLOGIC surgery , *RECTAL cancer , *SURGICAL excision , *LAPAROSCOPIC surgery , *ABDOMINAL wall - Abstract
Desmoid tumors are monoclonal fibroblastic proliferations arising from soft tissue classified as intra‐abdominal, extra‐abdominal and abdominal wall types. We present a patient with an intra‐abdominal desmoid tumor diagnosed 20 months after laparoscopic resection of rectal cancer. A 70‐year‐old woman with hematochezia was diagnosed with advanced rectal cancer. Preoperative chemoradiotherapy followed by laparoscopic low anterior resection was performed. During follow‐up, a nodular soft‐tissue density measuring 28 mm was detected in the presacral region. Metastasis from rectal cancer was diagnosed and four courses of chemotherapy were given, including capecitabine, oxaliplatin and bevacizumab. Computed tomography scan showed that the mass slightly decreased in size and surgical resection was performed. Histopathological examination revealed a proliferation of spindle‐shaped cells and collagenous stroma diagnosed as a desmoid tumor. This report highlights the possibility of a desmoid tumor in the differential diagnosis of an intra‐abdominal mass found during follow‐up after resection of colorectal cancer including following laparoscopic resection. [ABSTRACT FROM AUTHOR]
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- 2020
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206. Desmoid-type fibromatosis arising in the inguinal region in a young woman: a case report from a histopathological perspective.
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Bartoš, Vladimír
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GROIN , *DESMOID tumors , *YOUNG women , *STRIATED muscle , *MYOFIBROBLASTS - Abstract
Fibromatoses generally comprise a broad spectrum of myofibroblastic proliferation of similar histomorphology. Desmoid-type fibromatoses (desmoid tumors) are locally aggressive lesions which principally involve deep soft tissue structures. The author describes a 35-year-old female, who was found to have a nodular subcutaneous resistance arising in the upper portion of the right inguinal region. The tumor measured 40 x 25 x 15 mm and adhered to the aponeurosis of the abdominal internal oblique muscle. Histology revealed a dense mass of proliferating spindle-shaped myofibroblasts of uniform appearance. At the periphery, the lesion was poorly circumscribed and infiltrated an adjacent striated muscle. A diagnosis of desmoid-type fibromatosis was made. Desmoid tumor is a quite rare oncologic entity. Although it never metastasizes, it can lead to significant morbidity due to locally aggressive behaviour with a striking tendency to recur. Every patient once treated for deep fibromatosis requires long-term follow-up. [ABSTRACT FROM AUTHOR]
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- 2020
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207. Radiotherapy in the treatment of aggressive fibromatosis: experience from a single institution.
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Seidensaal, K., Harrabi, S. B., Weykamp, F., Herfarth, K., Welzel, T., Mechtersheimer, G., Lehner, B., Schneider, M., Fröhling, S., Egerer, G., Debus, J., and Uhl, M.
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DESMOID tumors , *ADENOMATOUS polyposis coli , *PROGRESSION-free survival , *RADIOTHERAPY , *ABDOMINAL wall - Abstract
Background: Desmoid-type fibromatosis is a rare, potentially locally aggressive disease. Herein we present our experience in the treatment with radiotherapy.Methods and Materials: In total 40 patients who received 44 treatments from 2009 to 2018 at the Heidelberg University Hospital with photons (N = 28) as well as protons (N = 15) and carbon ions (N = 1) were investigated. The median age at radiotherapy was 41 years [range 8-78]. Familial adenomatous polyposis (FAP) was confirmed for nine patients and 30 had a unifocal desmoid tumor. The localizations were abdominal wall, abdominopelvic cavity, thoracic wall, extremity, head and neck and trunk. The median prescribed dose was 54 Gy/ Gy (RBE) [range 39.6-66, IQR 50-60]. Eleven treatments were performed at the time of first diagnosis; 33 at the time of progression or recurrence. Post-operative radiotherapy was performed in 17 cases. The median planning target volume was 967 ml [84-4364 ml, IQR 447-1988]. Survival analysis was performed by the Kaplan-Meier Method.Results: The median follow-up time was 32 months [1-153]. At the end of the follow-up interval all patients but one were alive. The estimated local progression free survival of the treated lesion in 3 and 5 years was 76.4% and 63,8%, respectively. The progression-free survival in 3 and 5 years was 72.3 and 58.4% and the overall survival was 97.4 and 97.4%, respectively. In case of macroscopic tumor (N = 31) before radiotherapy a partial remission was observed in 12 cases (38.7%) and a complete remission in 4 cases (12.9%). Progression was observed in 13 (29.5%) cases, predominantly at the margin of the planning target volume (PTV, N = 5, 38,4%) followed by progression within the PTV (N = 4, 30.8%). In univariate analysis multifocal localization was associated with impaired progression-free survival (p = 0.013). One patient developed a grade V gastrointestinal bleeding, otherwise no acute toxicity >°III was observed. Late toxicity was depending on the localization of the desmoid tumor and was especially severe in patients with FAP and abdominopelvine desmoids including gastrointesinal fistula, perforation and abscess.Conclusion: Radiotherapy in the treatment of desmoids can lead to long term control. Treatment of patients with abdominopelvine desmoids should be avoided, as the risk of higher-grade complications is substantial. [ABSTRACT FROM AUTHOR]- Published
- 2020
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208. Tumor desmoide intraabdominal. Presentación de caso clínico y revisión de la literatura.
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Palacios-Fuenmayor, Luis J., Naranjo-Isaza, Ana M., Fuentes, Ossian, Palacio, María F., Martelo, Alfredo, Gómez, Luis R., and García, Héctor
- Abstract
Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2020
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209. Laparoscopic spleen-preserving distal pancreatectomy for a solid-cystic intraabdominal desmoid tumor at a gastro-pancreatic lesion: a case report.
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Sugimachi, Keishi, Iguchi, Tomohiro, Ohta, Mitsuhiko, Mano, Yohei, Hisano, Terumasa, Yokoyama, Ryohei, Taguchi, Kenichi, Ikebe, Masahiko, Morita, Masaru, and Toh, Yasushi
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DESMOID tumors ,ADENOMATOUS polyposis coli ,PANCREATECTOMY ,SURGICAL complications ,LAPAROSCOPIC surgery ,INTRA-abdominal hypertension ,PANCREATIC cysts ,PANCREATIC surgery ,SPLEEN surgery ,STOMACH surgery ,DIFFERENTIAL diagnosis ,IMMUNOHISTOCHEMISTRY ,LAPAROSCOPY ,PANCREATIC tumors ,RETROPERITONEUM ,CONNECTIVE tissue tumors - Abstract
Background: We report a case of an intraabdominal desmoid tumor that occurred at a gastro-pancreatic lesion with spontaneous cystic features, and present the successful laparoscopic resection of the tumor.Case Presentation: A 20-mm retroperitoneal cystic mass with a solid component was found adjacent to the stomach and pancreatic body in a 52-year-old woman with no history of familial adenomatous polyposis. Laparoscopic spleen-preserving distal pancreatectomy with wedge resection of the stomach was performed, and complete resection was achieved without intraoperative and postoperative complications. Histopathological examination by immunohistochemistry enabled diagnosis of a desmoid tumor that had originated from the stomach and invaded the pancreatic parenchyma with a spontaneous cystic change. We herein report an extremely rare case of an intraabdominal desmoid tumor with a spontaneous cystic change.Conclusion: Regardless of its rarity, desmoid tumor should be included in the preoperative differential diagnosis of a cystic intraabdominal mass, and laparoscopic function-preserving surgery may be an optimal treatment option. [ABSTRACT FROM AUTHOR]- Published
- 2020
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210. High prevalence of persistent emotional distress in desmoid tumor.
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Ingley, Katrina M., Klein, Roberta, Theobalds, Nicole, Burtenshaw, Sally, Abdul Razak, Albiruni R., Chen, Bo, Xu, Wei, Gladdy, Rebecca, Li, Madeline, and Gupta, Abha A.
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DESMOID tumors , *ABDOMINAL wall , *REGRESSION analysis , *SARCOMA , *PSYCHOLOGICAL distress , *EMOTIONAL experience - Abstract
Objective: Clinical experience suggests a high prevalence of emotional distress in patients with desmoid tumor (DT). We examine longitudinal Distress Assessment and Response Tool (DART) scores to estimate prevalence and persistence of distress, and compare cross-sectional data between DT and malignant sarcoma cohorts, to identify predictors of distress.Methods: Patients with DT completed DART at: T1-diagnosis, T2-during, T3-<6 months, and T4-≥6 months, post-treatment. DART includes patient-reported outcome measures of physical symptoms (ESAS-r), depression (PHQ-9), anxiety (GAD-7), and social difficulties (SDI-21). Descriptive prevalence and persistence of anxiety, depression, and wellbeing are reported, and mixed model regression analyses determine predictors of distress.Results: Between 2012 and 2018, a total of 152 DART screens from 94 patients with DT were completed (T1: n = 44, T2: n = 31, T3: n = 22, T4: n = 55). Patients had a mean age 40 years, 78% were female and DT locations were abdominal wall (48%), extremity (30%), and mesentery (22%). Moderate to severe ESAS-r scores (≥4) persisted at T4 for anxiety (20%), depression (13%), and poor wellbeing (31%). Compared to 402 patients with malignant sarcoma, patients with abdominal wall sited DT reported severe PHQ-9 and GAD-7 scores twice as frequently. Abdominal wall location, female sex, history of mood problems, and psychosocial concerns were significant predictors of anxiety, depression, and poor wellbeing in DT.Conclusions: Adults with DT experience persistently high emotional distress compared to patients with malignant sarcoma. Women with abdominal wall DT, prior mood, and current psychosocial concerns need early attention within multidisciplinary treatment settings to reduce persistent distress. [ABSTRACT FROM AUTHOR]- Published
- 2020
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211. Non Surgical Desmoid Tumor with Pulmonary and Scapular Localization: A Case Report.
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Benjelloun, Amine, Samri, Ikram, Janah, Hicham, Belassri, Salah, Benchanna, Rachid, Arsalane, Adil, and Zidane, Abdelfattah
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DESMOID tumors , *SCAPULARS , *HEMATOLOGY , *CARDIOPULMONARY system , *IMMUNOHISTOCHEMISTRY - Abstract
Background : Desmoid tumors are rare and represent 0.03% of all solid tumors. Their histology is benign but they are willingly aggressive with significant locoregional invasions and frequent recurrences. Case presentation : We report the case of a 52-year-old patient who presented with a scapular and pulmonary localization of this tumor, but stable over time. In view of potentially decaying surgery and stability of the lesions, simple monitoring was decided. The lesions are currently stable, two years after diagnosis. We discuss the clinical, radiological and therapeutic characteristics of this type of tumor, in the light of data from the literature. Conclusion : Desmoid tumors are rare, benign but agressive. Surgery is the main treatment but is often decaying. Simple monitoring can be scheduled in case of less agressive tumors. [ABSTRACT FROM AUTHOR]
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- 2020
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212. MESENTERIC DESMOID TUMOR AFTER ROUX-EN-Y GASTRIC BYPASS: A CASE REPORT.
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Navarini, Daniel, Albuquerque, Antônio Benincá, Rovani, André Luca Boeira, Silva, Victor Vasconcelos da, Ranzolin, Bernardo Antoniolli, Reffatti, Diego, Perin, Ramir Luan, and Madalosso, Carlos Augusto Scussel
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GASTRIC bypass , *BARIATRIC surgery , *DESMOID tumors , *COMPUTED tomography - Abstract
We report the case of a 37-year-old woman investigated for left flank pain 1 year after bariatric surgery (Roux-en-Y gastric bypass). Abdominal computed tomography (CT) revealed a solid intra-abdominal lesion measuring 9.3 x 9.4 x 10.4 cm, compressing adjacent structures with no signs of invasion. Ileocolectomy with partial mesenteric resection was performed. A histopathological and immunohistochemical analysis confirmed the diagnosis of mesenteric desmoid tumor. [ABSTRACT FROM AUTHOR]
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- 2020
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213. Radical palliative surgery: new limits to pursue
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Young-Spint, Mindy, Guner, Yigit S., Meyers, Frederick J., Schneider, Phillip, and Khatri, Vijay P.
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Medicine & Public Health ,Pediatric Surgery ,Surgery ,Pediatrics ,Desmoid tumor ,Palliative surgery ,Pediatric ,Palliation ,Radical surgery - Abstract
This case report describes the radical subtotal palliative resection of a massive recurrent desmoid tumor encompassing the abdomen, pelvis, and groin in a child who was 13 years old at the time of initial resection. Given the extensive distribution of the tumor en bloc resection, which is the standard treatment of desmoid tumors, would have meant performing a hemipelvectomy and repair of a large abdominal wall defect, likely with skin grafts and mesh. The patient’s personal goals however were to alleviate the pain and limited mobility that would allow her to re-attend high school and appear normal to her peers. Therefore, palliative surgery was pursued and currently the patient is 5 years out from her last surgery doing well. We believe that the option of surgical palliation in this case was warranted and should be an option for similar cases in the future.
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- 2009
214. Ruptured desmoid tumor imitating acute appendicitis - a rare reason for an emergency surgery.
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Asenov, Yavor, Genadiev, Stoyan, Timev, Alexander, Panaiotova, Jeni, Hadjiiska, Valeria, Veselin, Tihtchev, and Sedloev, Theophil
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DESMOID tumors ,APPENDICITIS ,SURGICAL emergencies ,ABDOMEN ,SARCOMA ,CANCER invasiveness - Abstract
Background: Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications.Case Presentation: We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis.Conclusions: Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation. [ABSTRACT FROM AUTHOR]- Published
- 2019
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215. Pediatric peripheral nerve tumors: clinical and surgical aspects.
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Guedes, Fernando, Brown, Rosana Siqueira, Torrão-Junior, Francisco José Lourenço, Barbosa, Daniel A. N., Ravanini, Guilherme de Andrade Gagheggi, and Amorim, Rogério Martin Pires
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PERIPHERAL nerve tumors , *SCHWANNOMAS , *EWING'S sarcoma , *BENIGN tumors , *DESMOID tumors , *NEUROFIBROMATOSIS 1 , *NEUROFIBROMA - Abstract
Purpose: Pediatric peripheral nerve tumors (PNTs) are rare. Most are related to neurofibromatosis type 1 (NF1) with the potential for malignancy. An ongoing debate occurs about the best approach to such patients. This study describes a cohort of pediatric patients with PNTs and discusses clinical characteristics and surgical treatment. Methods: We retrospectively reviewed the charts of seven pediatric patients with eight PNTs surgically treated from 2007 to 2018. Information concerning patient demographics, clinical presentation, PNTs characteristics, treatment choice, and outcome were recorded. Results: All children presented with intense pain and a palpable mass. Three of the eight tumors were associated with a neurological deficit. Among the four patients with NF1, two had a neurofibroma and two a malignant peripheral nerve sheath tumor (MPNST). Histologically, three of the lesions were a benign peripheral nerve sheath tumor (BPNST), three a MPNST, and one each a desmoid tumor and Ewing's sarcoma. Two of the eight tumors underwent partial tumor excision and six gross total excisions. Conclusions: Intense pain at rest, day, and/or night, preventing normal activities; a palpable, hard, immobile mass; an intense Tinel's sign related to the lump; clinical evidence of NF1; and high-speed growth of a tumor in the trajectory of the nerve or plexus should alert the clinician to the potential for malignancy. Preoperative biopsy is not indicated when clinical and imaging findings suggest a benign tumor. The surgical management of PNTs must be to achieve total resection, including wide margins with malignant tumors, though this is not always possible. [ABSTRACT FROM AUTHOR]
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- 2019
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216. Sino-orbital desmoid tumor in a pediatric patient – Case report with review of literature.
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Parulan, Mariel Angelou, Sundar, Gangadhara, Ong, Yew Kwang, Yeo, Tseng Tsai, Lee, Victor, and Kimpo, Miriam Santiago
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DESMOID tumors , *SOFT tissue tumors , *SKULL base , *LITERATURE reviews , *CELL tumors - Abstract
We report a case of a 2-year-old female who presented with bilateral progressive proptosis, visual loss, nasal obstruction, and breathing difficulty. Magnetic resonance imaging revealed a large sino-orbital mass that was extending to the orbital apex and skull base. An initial diagnosis of rhabdomyosarcoma was made elsewhere on the basis of the presence of round and spindle cell tumor. Subsequent biopsy with immunohistochemical staining was positive for nuclear staining with β-catenin, shifting the diagnosis to a myofibroblastic tumor, favoring desmoid-type fibromatosis. With image guidance, near complete excision of tumor was performed by a multidisciplinary team, while respecting danger zones such as the skull base and the optic nerve. Following a recurrence over 2 months, additional excision was performed with a 6-month treatment of methotrexate and vinblastine. Desmoid tumor is a rare form of soft tissue tumor uncommonly seen in the orbital area. Although benign, it is known to be recurrent and infiltrative. Few data are known and further information will aid in the management of these tumors. [ABSTRACT FROM AUTHOR]
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- 2019
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217. The Abdominoplasty Access for Desmoid Tumor Resection in the Rectus Abdominis Muscle
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Djedovic, Gabriel, Pierer, Gerhard, Rieger, Ulrich M., Di Giuseppe, Alberto, editor, and Shiffman, Melvin A., editor
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- 2016
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218. Desmoid Tumor
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Clark, Sue and Schwab, Manfred, editor
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- 2017
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219. The Notch signaling pathway in desmoid tumor: Recent advances and the therapeutic prospects.
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Zheng, Chuanxi, Huang, Jianghong, Xu, Gang, Li, Wei, Weng, Xin, and Zhang, Shiquan
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NOTCH genes , *DESMOID tumors , *NOTCH signaling pathway , *ADENOMATOUS polyposis coli , *SYMPTOMS , *CATENINS , *DRUG target - Abstract
Desmoid tumor (DT) is a rare fibroblastic soft-tissue neoplasm that is characterized by local aggressiveness but no metastatic potential. Although the prognosis is relatively favorable, the unpredictable disease course and infiltrative growth lead to significant impairments and morbidity. Aberrant activation of Wnt/β-catenin signaling has been well-established in the pathogenesis of sporadic DT and familial adenomatous polyposis (FAP) or Gardners syndrome-associated DT, suggesting therapy targeting this pathway is an appealing treatment strategy. However, agents against this pathway are currently in their preliminary stages and have not yet been implemented in clinical practice. Increasing studies demonstrate activation of the Notch pathway is closely associated with the development and progression of DT, which provides a potential alternative therapeutic target against DT. Early-stage clinical trials and preclinical models have indicated that inhibition of Notch pathway might be a promising treatment approach for DT. The Notch signaling activation is mainly dependent on the activity of the γ-secretase enzyme, which is responsible for cleaving the Notch intracellular domain and facilitating its nuclear translocation to promote gene transcription. Two γ-secretase inhibitors called nirogacestat and AL102 are currently under extensive investigation in the advanced stage of clinical development. The updated findings from the phase III randomized controlled trial (DeFi trial) demonstrated that nirogacestat exerts significant benefits in terms of disease control and symptom resolution in patients with progressive DT. Therefore, this review provides a comprehensive overview of the present understanding of Notch signaling in the pathogenesis of DT, with a particular emphasis on the prospective therapeutic application of γ-secretase inhibitors in the management of DT. • The standardized treatment option for patients with desmoid tumor (DT) remains a challenge. • The Notch pathway has been proved to play a critical role in the pathogenesis of DT. • The GSIs blocking the Notch pathway is a novel therapeutic strategy for DT. • Further studies are needed to identify the innovative therapeutic targets for DT. [ABSTRACT FROM AUTHOR]
- Published
- 2024
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220. AWAKE XIFOPUBIC LAPAROTOMY FOR DESMOID TUMOR DURING PREGNANCY – A CASE REPORT
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Silva, Mafalda
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Anestesia obstétrica ,Obstetrical anaesthesia ,Desmoid tumor ,Regional anaesthesia ,Tumor desmóide ,Anestesia regional - Abstract
Non obstetric surgery during pregnancy is rare with maternal malignancy being one of the most common causes. Sporadic pregnancy related desmoid tumours are even more uncommon and optimal management of this tumour is complex. Complete surgical excision is the most commonly accepted treatment. Surgery during pregnancy is challenging as it demands taking into consideration requirements for both the mother and the foetus. The choice of anaesthetic plan must take into account surgical and patient specificities, and the effects of anaesthesia on the foetus. We report the perioperative management of a pregnant woman in the second trimester of pregnancy who underwent complete excision of a desmoid tumour under regional anaesthesia. Intraoperative change of surgical plan posted an additional challenge. , A cirurgia não obstétrica durante a gravidez é rara, sendo uma das causas mais comuns a excisão de tumores maternos. Tumores desmóides esporádicos na gravidez, constituem uma causa ainda mais rara, sendo o tratamento ideal complexo. A excisão cirúrgica completa é atualmente o tratamento mais aceite. A cirurgia durante a gravidez é um desafio uma vez que requer ter em consideração ambos a mãe e o feto. A escolha de um plano anestésico requer ter em linha de conta especificidades cirúrgicas e da paciente, bem como os efeitos da própria anestesia no feto. Este caso clínico relata o manuseio perioperatório de uma grávida no segundo trimestre de gravidez que foi submetida a uma excisão completa de um tumor desmóide sobre anestesia loco regional. A alteração intraoperatória do plano cirúrgico constituiu um desafio adicional.
- Published
- 2023
221. Abdominal Wall Abnormality and Hernia
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Lee, Min Woo and Choi, Byung Ihn, editor
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- 2015
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222. Benign Diseases of the Mesentery and Omentum
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Kim, Jung Hoon and Choi, Byung Ihn, editor
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- 2015
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223. Tumors
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Hefti, Fritz, Jundt, Gernot, and Hefti, Fritz
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- 2015
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224. Soft Tissue Masses
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Roper, Glade E., Stein-Wexler, Rebecca, Stein-Wexler, Rebecca, editor, Wootton-Gorges, Sandra L., editor, and Ozonoff, M.B., editor
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- 2015
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225. Resection of Mesenteric Tumors
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Karakousis, Constantine P. and Karakousis, Constantine P.
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- 2015
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226. Hereditary Colorectal Cancer and Polyposis Syndromes
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Cho, Edward Eun, Gibbs, John F., Rodriguez-Bigas, Miguel, Rodriguez, Luz Maria, Chu, Quyen D., editor, Gibbs, John F., editor, and Zibari, Gazi B., editor
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- 2015
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227. A 50-year-old man with desmoid-type fibromatosis of upper extremity
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Vitorino Modesto dos Santos, Viviane Vieira Passini Soares, Mayza Lemes Duarte, Priscilla Souza de Faria, Uadson Silva Barreto, and Victor Manabu Yano
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Aggressive fibromatosis ,desmoid tumor ,surgery ,upper limb. ,Medicine - Abstract
This case study describes a 50-year-old man with a desmoid tumor of upper extremity, which had to be resected concomitant with the left arm amputation at middle third of the humerus. The course was initially stable, but evolved with aggressive infiltration of local soft tissues. During the watchful waiting time, three biopsies were done before establishing the diagnosis. Plain X-ray, magnetic resonance and positron emission tomography were the exams utilized. The authors emphasize diagnostic and management challenges involving this rare condition. Case reports can stimulate further studies to get additional knowledge about this rare entity.
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- 2017
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228. Radiologic images of an aggressive implant-associated fibromatosis of the breast and chest wall: case report and review of the literature
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Lourdes Alanis, MD, MPH, Robyn Roth, MD, Nati Lerman, MD, Julieta E. Barroeta, MD, and Pauline Germaine, DO
- Subjects
Breast fibromatosis ,Desmoid tumor ,Extra-abdominal tumor ,Chest wall ,Breast cancer ,Breast implants ,Mammography ,Magnetic resonance imaging ,Ultrasonography ,Pathology ,Medical physics. Medical radiology. Nuclear medicine ,R895-920 - Abstract
Fibromatosis of the breast is a rare benign disease compromising
- Published
- 2017
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229. Gradually shrinking intra-abdominal desmoid tumor derived from the stomach in a young boy: a case report
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Kazushi Miyata, Masahide Fukaya, and Masato Nagino
- Subjects
Desmoid tumor ,Stomach ,Young boy ,Surgery ,RD1-811 - Abstract
Abstract Background Intra-abdominal desmoid tumors, particularly those derived from the stomach, are rare. Such tumors are associated with a history of familial adenomatous polyposis (FAP), trauma, or surgical procedures in general. In addition, spontaneous shrinking of an intra-abdominal desmoid tumor is rarer. And desmoid tumors most commonly arise during the fourth decade of life. Case presentation A 17-year-old boy with lower abdominal pain was diagnosed with a gastrointestinal stromal tumor (GIST) or a hematoma at a local hospital. He had no history of FAP, trauma, or previous surgery. Abdominal computed tomography (CT) was performed for observational purposes three times over a 9-month period. The tumor gradually decreased in size over time; however, the tumor did not shrink sufficiently to be diagnosed as a hematoma. Because there was a high possibility of a GIST from the stomach, he underwent laparotomy. Operative findings revealed that the tumor was a hard mass firmly attached to both the greater curvature of the stomach and the inferior pole of the spleen. Pathologically, the tumor was diagnosed as a desmoid tumor derived from the stomach. Conclusion For a young boy without a history of FAP, trauma, or surgical procedures, it is difficult to define an intra-abdominal tumor near the stomach as a desmoid tumor. In such cases, surgical resection is recommended for a definitive diagnosis.
- Published
- 2017
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230. Activated Signaling Pathways and Targeted Therapies in Desmoid-Type Fibromatosis: A Literature Review
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Milea J. M. Timbergen, Ron Smits, Dirk J. Grünhagen, Cornelis Verhoef, Stefan Sleijfer, and Erik A. C. Wiemer
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desmoid-type fibromatosis ,signaling pathways ,desmoid tumor ,CTNNB1 mutation ,review ,ß-catenin ,Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,RC254-282 - Abstract
Desmoid-type fibromatosis (DTF) is a rare, soft tissue tumor of mesenchymal origin which is characterized by local infiltrative growth behavior. Besides “wait and see,” surgery and radiotherapy, several systemic treatments are available for symptomatic patients. Recently, targeted therapies are being explored in DTF. Unfortunately, effective treatment is still hampered by the limited knowledge of the molecular mechanisms that prompt DTF tumorigenesis. Many studies focus on Wnt/β-catenin signaling, since the vast majority of DTF tumors harbor a mutation in the CTNNB1 gene or the APC gene. The established role of the Wnt/β-catenin pathway in DTF forms an attractive therapeutic target, however, drugs targeting this pathway are still in an experimental stage and not yet available in the clinic. Only few studies address other signaling pathways which can drive uncontrolled growth in DTF such as: JAK/STAT, Notch, PI3 kinase/AKT, mTOR, Hedgehog, and the estrogen growth regulatory pathways. Evidence for involvement of these pathways in DTF tumorigenesis is limited and predominantly based on the expression levels of key pathway genes, or on observed clinical responses after targeted treatment. No clear driver role for these pathways in DTF has been identified, and a rationale for clinical studies is often lacking. In this review, we highlight common signaling pathways active in DTF and provide an up-to-date overview of their therapeutic potential.
- Published
- 2019
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231. Desmoid-type Fibromatosis of the Breast Mimicking Carcinoma
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Laura Wuyts and Aljosja De Schepper
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Mammary fibromatosis ,desmoid fibromatosis ,desmoid tumor ,extra-abdominal desmoid tumor ,aggressive fibromatosis ,Medical physics. Medical radiology. Nuclear medicine ,R895-920 - Published
- 2019
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232. Multifocal Desmoid-Type Fibromatosis: Case Series and Potential Relationship to Neuronal Spread.
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Skubitz KM, Marette S, Murugan P, Yueh B, and Clohisy DR
- Abstract
Multifocal desmoid-type fibromatosis (DTF) is very rare and usually regional. We report three cases that initially appeared to be multifocal, but subsequent detailed imaging revealed unsuspected tracking along nerves in two cases. This neural spread is reminiscent of neuromuscular choristoma (NMC), a rare developmental lesion in which mature skeletal muscle cells, or rarely smooth muscle cells, infiltrate and enlarge peripheral nerves. NMC is frequently associated with DTF. These two cases suggest that DTF spread along nerves and appeared as distinct multifocal lesions while actually being contiguous. The third case was felt to represent true multifocal tumor development, possibly due to tumor seeding at the time of chest surgery. The relationship of DTF to NMC is discussed., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Skubitz et al.)
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- 2024
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233. A 10-year-old male with chest wall Desmoid tumor - a rare tumor with unusual presentation.
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Abufara AA, Alsalah QA, Jabari AY, Hammouri AG, and Najajreh M
- Abstract
Desmoid fibromatosis (DF) is a connective tissue tumor that grows aggressively in musculoaponeurotic tissues. It has an annual incidence rate of 2-4/million and is commonly seen in individuals aged 15 to 60 years, with female predominance. While it can occur in any body part, it is commonly found in the extremities, abdominal wall, and abdominal mesentery. But it rarely develops in the chest wall. The cause of the tumor is unknown. However, trauma to the tumor site has been identified in 25% of known cases. The primary treatment is surgical resection. Local recurrence after surgical excision is common. Our study highlights the case of a 12-year-old male patient diagnosed with a desmoid tumor on the chest wall 2 years ago, at the age of 10 years, after mild trauma. The tumor was successfully managed with surgery without radiotherapy treatment and no recurrence was observed in the last 2 years., Competing Interests: None declared., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)
- Published
- 2024
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234. Desmoid Fibromatosis of the Anterior Abdominal Wall in Pregnancy: A Case Report and Review of the Literature.
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Zubor P, Henriksen CM, Økstad ME, Cerskuviene E, Visnovsky J, Kajo K, Valkov A, and Lind KO
- Abstract
A desmoid tumor (DT) is a rare benign neoplasm arising from muscle aponeurosis, associated mostly with trauma or pregnancy. DT has an infiltrative and locally aggressive growth pattern and usually does not metastasize. However, it has a high recurrence and complication rate. When it occurs in pregnancy, the pregnancy and delivery is taken as an individual case for optimal management by physicians and midwifes, who need to be cautious in finding the optimal delivery mode for the patient, which depends on the tumor size, location, behavior, and past history. The authors report a case of 29-year-old pregnant woman who previously underwent systemic oncological treatment for a large abdominal wall desmoid tumor and became pregnant afterwards. The history of DT presented a follow-up and delivery challenge. Observational management was chosen with an elective cesarean section at week 38 + 4 of pregnancy with uncomplicated postpartum follow-up. The authors detail the clinical management and chosen therapeutic approach; chemotherapy can be a choice in the treatment options for patients with DTs, although the majority of DTs are treated surgically with subsequent mesh plastic. Moreover, the authors provide a systematic review of the literature focused on the treatment management of DTs in pregnant women during pregnancy and the postpartum period, as pregnancy-associated desmoid tumors are a specific condition, where the optimal management is not well established, despite some guidelines for non-pregnant patients.
- Published
- 2024
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235. Trauma and sporadic desmoid tumor development: An approach toward real incidence and aspects of causality.
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Hohenberger P, Menge F, Hohenberger R, Kasper B, Marx A, Haller F, Baumgarten C, Wardelmann E, and Jakob J
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- Male, Female, Humans, Incidence, Mutation, Germ-Line Mutation, beta Catenin genetics, Fibromatosis, Aggressive epidemiology, Fibromatosis, Aggressive genetics, Fibromatosis, Aggressive pathology, Wounds, Nonpenetrating
- Abstract
Objectives: The development of desmoid tumors (DT) is associated with trauma, which is an aspect with medicolegal relevance. The objective of this study was to analyze the proportion and type of trauma (surgical, blunt/fracture, implants), its lag time, and mutations of the CTNNB1 gene in patients with sporadic DT., Methods: We analyzed a prospectively kept database of 381 females and 171 males, median age at disease onset 37.7 years (females) and 39.3 years (males) with a histologically confirmed DT. Patients with germline mutation of the APC gene were excluded. Details of the history particularly of traumatic injuries to the site of DT were provided by 501 patients., Results: In 164 patients (32.7%), a trauma anteceding DT could be verified with a median lag time of 22.9 months (SD, 7.7 months; range, 9-44 months). A prior surgical procedure was relevant in 98 patients, a blunt trauma in 35 patients, a punctuated trauma (injections, trocar) in 18 patients, and site of an implant in 10 patients. In 220 patients, no trauma was reported (43.9%), and 58 females (11.6%) had a postpregnancy DT in the rectus abdominis muscle. In 42 patients (8.4%), data were inconclusive. The distribution of mutations in the CTNNB1 gene (codon 41 vs. 45) was similar in patients with and without a history of trauma before DT development., Conclusions: A significant subgroup of patients suffers from a trauma-associated DT, predominantly at a prior surgical site including implants to breast or groin, accounting for 77.9% of the cases, whereas blunt trauma was responsible in 22.1%. We found no data to support that trauma-associated DT have different molecular features in the CTNNB1 gene., (© 2023 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.)
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- 2024
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236. MRI-based navigated cryosurgery of extra-abdominal desmoid tumors using skin fiducial markers: a case series of 15 cases.
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Segal O, Benady A, Pickholz E, Ovadia JE, Druckmann I, Dadia S, Rath E, Albagli A, and Efrima B
- Subjects
- Humans, Fiducial Markers, Magnetic Resonance Imaging, Retrospective Studies, Stereotaxic Techniques, Cryosurgery, Fibromatosis, Aggressive diagnostic imaging, Fibromatosis, Aggressive surgery, Surgery, Computer-Assisted methods
- Abstract
Background: Precision surgery is becoming increasingly important in the field of Orthopaedic Oncology. Image-guided percutaneous cryosurgery (CRA) has emerged as a valid treatment modality for extra-abdominal desmoid tumors (EDTs). To date, most CRA procedures use CT-based guidance which fails to properly characterize tumor segments. Computer-guided MRI navigation can address this issue however, the lack of a fixed landmark for registration remains a challenge. Successful CRA correlates directly with precision approaches facilitated by intraoperative imaging guidance. This is the first study that attempts to assess the feasibility and efficacy of a novel approach of using skin fiducial markers to overcome the challenge of a MRI-based navigation CRA for symptomatic or progressive EDTs., Methods: In this retrospective study conducted between 2018 and 2020, 11 patients at a single center with symptomatic or progressive EDTs were treated with CRA using intraoperative MRI navigation. Fifteen cryosurgery procedures were performed, each adhering to a personalized pre-operative plan. Total tumor size, viable and non-viable portions pre- and post-operation, and SF-36 questionnaire evaluating subjective health were recorded., Results: All CRAs demonstrated 100% adherence to the predetermined plan. Overall, tumor size decreased Median= -56.9% [-25.6, -72.4]) with a reduction in viable tissue, (Median= -80.4% [-53.3, -95.2]). Four patients required additional CRAs. Only one patient's tumor did not reduce in size. One patient suffered from local muscle necrosis. Pre-operation, the average physical and mental scores 41.6 [29.4, 43] and 26.3 [17.6, 40.9] respectively. Post-operation, the average physical and mental scores were 53.4[38, 59.7] and 38 [31.2, 52.7] respectively., Conclusion: These findings provide an early indication of the feasibility and efficacy of performing percutaneous cryosurgery using skin fiducial marker registration for MRI-computed navigation to treat EDTs safely. Larger cohorts and multicenter evaluations are needed to determine the efficacy of this technique., (© 2023. The Author(s).)
- Published
- 2023
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237. Diffusion weighted imaging changes in extra-abdominal desmoid tumor after cryotherapy.
- Author
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Cadour F, Tradi F, Bartoli A, Duffaud F, and Gaubert JY
- Subjects
- Male, Humans, Neoplasm Recurrence, Local diagnostic imaging, Neoplasm Recurrence, Local therapy, Diffusion Magnetic Resonance Imaging methods, Necrosis, Cryotherapy, Fibromatosis, Aggressive diagnostic imaging, Fibromatosis, Aggressive therapy
- Abstract
Desmoid tumors (DT) are rare benign tumors with a local invasion potential and recurrence. It is characterized on histology by an abnormal fibroblastic proliferation in a collagenous stroma, in variable proportions leading to heterogeneity of the lesion signal on magnetic resonance imaging (MRI). Current guidelines propose watchful waiting but in case of progression or symptoms, cryotherapy may be a therapeutic option in its extra-abdominal form. Tumor recurrence is mostly detected based on post-contrast magnetic resonance imaging (MRI). Although DWI sequence is the key-sequence for tumor detection in oncologic imaging, there are very few data in literature on diffusion weighted imaging (DWI) in DT generally and even fewer on DT after cryotherapy. DWI changes after cryotherapy may be confusing and suspicious of residual tumor or tumor recurrence when displaying low ADC values; thus knowledge of possible DWI patterns after cryotherapy of DT seem paramount. We found that the early changes of DT after cryotherapy are hyperintensity on DWI sequence with low ADC values (<1.00 × 10
-3 mm2 /s), without corresponding enhancement and a later decrease in signal of the treated lesion on DWI. The freezing-thawing cycles of cryotherapy turn DT into gelatinous necrosis with a slow resorption rate, as reported in the only few studies referring of changes of DWI signals after cryotherapy, which are on renal and prostate models. Hyperintensity on DWI with low ADC values may be seen in early MRI follow-up after cryotherapy of extra-abdominal DT, corresponding with tumor necrosis changes and should not be mistaken with recurrence.KEY MESSAGESMagnetic resonance imaging is the modality of choice for desmoid tumor (DT) follow-up, mainly based on contrast uptake which make data on diffusion weighted imaging (DWI) very rare.Cryotherapy is an accepted therapeutic option for DT that will lead to tumor necrosis.Hyperintensity on DWI with low apparent diffusion coefficient values is a possible expected early pattern on DWI after cryotherapy of DT.- Published
- 2023
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238. Genotype-phenotype correlation for extracolonic aggressive phenotypes in patients with familial adenomatous polyposis.
- Author
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Shimamoto Y, Takeuchi Y, Ishiguro S, Nakatsuka SI, Yunokizaki H, Ezoe Y, Nakajima T, Tanaka K, Ishihara R, Takayama T, Yoshida T, Sugano K, Mutoh M, and Ishikawa H
- Subjects
- Humans, Genes, APC, Genotype, Phenotype, Genetic Association Studies, Mutation, Fibromatosis, Aggressive genetics, Adenomatous Polyposis Coli genetics, Adenomatous Polyposis Coli pathology, Stomach Neoplasms genetics, Stomach Neoplasms pathology
- Abstract
Familial adenomatous polyposis (FAP) patients develop various life-threatening extracolonic comorbidities that appear individually or within a family. This diversity can be explained by the localization of the adenomatous polyposis coli (APC) variant, but few reports provide definitive findings about genotype-phenotype correlations. Therefore, we investigated FAP patients and the association between the severe phenotypes and APC variants. Of 247 FAP patients, 126 patients from 85 families identified to have APC germline variant sites were extracted. These sites were divided into six groups (Regions A to F), and the frequency of severe comorbidities was compared among the patient phenotypes. Of the 126 patients, the proportions of patients with desmoid tumor stage ≥III, number of FGPs ≥1000, multiple gastric neoplasms, gastric neoplasm with high-grade dysplasia, and Spigelman stage ≥III were 3%, 16%, 21%, 12%, and 41%, respectively, while the corresponding rates were 30%, 50%, 70%, 50%, and 80% in patients with Region E (codons 1398-1580) variants. These latter rates were significantly higher than those for patients with variants in other regions. Moreover, the proportion of patients with all three indicators (desmoid tumor stage ≥III, number of FGPs ≥1000, and Spigelman stage ≥III) was 20% for those with variants in Region E and 0% for those with variants in other regions. Variants in Region E indicate aggressive phenotypes, and more intensive management is required., (© 2023 The Authors. Cancer Science published by John Wiley & Sons Australia, Ltd on behalf of Japanese Cancer Association.)
- Published
- 2023
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239. Desmoid Fibromatosis With TP53 Mutation and Striking Nuclear Pleomorphism.
- Author
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Foster CR, Strauss M, Hornick JL, and Habeeb O
- Subjects
- Humans, Tumor Suppressor Protein p53 genetics, Mutation, Germ-Line Mutation, Immunohistochemistry, beta Catenin genetics, Fibromatosis, Aggressive diagnosis, Fibromatosis, Aggressive genetics
- Abstract
Desmoid fibromatosis is a myofibroblastic neoplasm of intermediate biologic potential, which has a strong predilection for local recurrence but does not metastasize. Arranged in long, sweeping fascicles with infiltrative borders, desmoid fibromatosis typically consists of uniform, bland myofibroblastic spindle cells that harbor mutation of CTNNB1 (or less often APC ) . In this report, we present a remarkable case of desmoid fibromatosis associated with striking nuclear pleomorphism. We hypothesize that this striking pleomorphism is due to a germline TP53 mutation, a finding first suspected due to strong and diffuse p53 staining by immunohistochemistry and subsequently confirmed by molecular testing. The combination of the pleomorphism and TP53 mutation in desmoid fibromatosis represents a novel finding unreported in the literature., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
- Published
- 2023
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240. INTRA-ABDOMINAL DESMOID TUMOR WITH AN UNUSUAL ORIGIN IN THE INTESTINAL WALL: CASE REPORT
- Author
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Tomas STICKAR, Juan Andrés Dárdano BERRIEL, Josep Lluis Molinero POLO, Yuhami Mitsahid Curbelo PEÑA, Julia Gardenyes MARTINEZ, Tonia Palau FIGUEROA, Jordi de Cozar DUCH, Manel Guixa GENER, Francesc Xavier Quer VALL, and Helena Valverdu CARTIE
- Subjects
Desmoid tumor ,Small bowel ,Surgery ,RD1-811 ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Published
- 2018
- Full Text
- View/download PDF
241. Myelopathy due to Intraspinal Desmoid Tumor
- Author
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Eltorai, Ibrahim M. and Eltorai, Ibrahim M.
- Published
- 2016
- Full Text
- View/download PDF
242. Inflammation, Infection, and Neoplasms in the Abdomen
- Author
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Cheon, Jung-Eun and Kim, In-One, editor
- Published
- 2014
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243. MRI in Muscle Tumors and Tumors of Fasciae and Tendon Sheaths
- Author
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Vanhoenacker, F. M., Adriaensen-van Roij, M. E. A. P. M., De Schepper, A. M., Reiser, Maximilian F, Series editor, Kauczor, Hans-Ulrich, Series editor, Hricak, Hedvig, Series editor, Knauth, Michael, Series editor, and Weber, Marc-André, editor
- Published
- 2014
- Full Text
- View/download PDF
244. Functional Imaging of Tumors of the Mesenterium and Retroperitoneum
- Author
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Toriihara, Akira, Tateishi, Ukihide, Luna, Antonio, editor, Vilanova, Joan C., editor, Hygino Da Cruz Jr., L. Celso, editor, and Rossi, Santiago E., editor
- Published
- 2014
- Full Text
- View/download PDF
245. Central Low-Grade Osteosarcoma
- Author
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Picci, Piero, Picci, Piero, editor, Manfrini, Marco, editor, Fabbri, Nicola, editor, Gambarotti, Marco, editor, and Vanel, Daniel, editor
- Published
- 2014
- Full Text
- View/download PDF
246. Desmoid Fibroma
- Author
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Campanacci, Laura, Picci, Piero, editor, Manfrini, Marco, editor, Fabbri, Nicola, editor, Gambarotti, Marco, editor, and Vanel, Daniel, editor
- Published
- 2014
- Full Text
- View/download PDF
247. Abdominal Wall Anatomy, Pathology, and Intervention
- Author
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Velasco, Jose M., Vaince, Faaiza, Hagopian, Ellen J., editor, and Machi, Junji, editor
- Published
- 2014
- Full Text
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248. Gardner Syndrome
- Author
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Golant, Alexandra, Zeichner, Joshua A., and Zeichner, Joshua, editor
- Published
- 2014
- Full Text
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249. Laryngeal reconstruction for recurrent desmoid tumor using three-dimensional modeling: A unique approach for a rare tumor.
- Author
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Tracy, Lauren F., Patel, Samip N., Buckmire, Robert A., and Shah, Rupali N.
- Abstract
Desmoid tumors are exceedingly rare within the larynx and cause significant morbidity due to their locally aggressive and infiltrative nature. Surgery is the mainstay of treatment with previous reports describing total and near-total laryngectomy for cure. We present a case of recurrent glottic desmoid tumor managed with hemilaryngectomy and reconstructed with temporoparietal free tissue, rib, and buccal grafts. Three-dimensional modeling was utilized to optimize aerodigestive function after laryngeal reconstruction. Laryngoscope, 129:1989-1992, 2019. [ABSTRACT FROM AUTHOR]
- Published
- 2019
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250. Breast desmoid tumor management in France: toward a new strategy.
- Author
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Duazo-Cassin, Ludwig, Le Guellec, Sophie, Lusque, Amélie, Chantalat, Elodie, Laé, Marick, Terrier, Philippe, Coindre, Jean-Michel, Boulet, Bérénice, Le Boulc'h, Morwenn, Gangloff, Dimitri, Meresse, Thomas, Chaput, Benoit, Al Ali, Amal, Rimareix, Françoise, Bonvalot, Sylvie, and Vaysse, Charlotte
- Abstract
Purpose: Desmoid tumors (DTs) are rare tumors that originate from myofibroblastic tissue. Recently, initial wait and see was recommended (ESMO guidelines Ann Oncol 2017) in the most frequent locations. This study investigates the outcome of breast desmoid tumor (BDT) according to the initial strategy. Method: Data from all consecutive patients treated from a BDT in four referral centers were collected. Only intra-mammary desmoid tumors were included. A pathological review and a molecular analysis (CTNNB1 gene mutation) were performed (National re-reading network of sarcomas-RRePS). Patients were grouped according to initial strategy: surgery group (SG) and active surveillance group (ASG). Results: A total of 63 patients (61 women, 2 men) met the inclusion criteria. Median age was 50 years (16–86). CTNNB1 mutation was found in 61% (n = 36). SG included 46 patients (73%) (41 partial mastectomies, 2 mastectomies, and 3 mastectomies associated to parietectomies). Surgical margins were positive in 15 patients (33.3%). Median follow-up of SG was 24.9 (0.5–209) months; and 4 patients (8.7%) developed recurrence. ASG included 17 patients (27%). Their median follow-up was 42.2 (0–214) months, and 15 patients (88.2%) did not require any additional treatment. Six patients (35%) had a spontaneous regression, 9 patients (52%) were stable, and 2 patients presented a significant progression that was treated by partial mastectomy. Conclusion: This study supports an initial nonsurgical approach to BDTs followed by surgery based on tumor growth in select cases, which is consistent with current ESMO recommendations. [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF
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