Aggressive fibromatosis of the abdominal wall, limbs and limb girdles

Phillips, Stephen Richard; A’Hern, R.; Thomas, Joseph

doi:10.1002/bjs.4792

Cited by 77 publications

(60 citation statements)

References 37 publications

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“…[19][20][21][22][23][24][25] Studies in adult AF have reported functionsparing surgery as their primary objective and suggested that, to reduce the risk of major sequelae, marginal surgery may be acceptable. [26][27][28][29] Our series revealed a high local recurrence rate after incomplete surgery; however, disease control was no better in Group II than in Group III. In principle, our findings suggest that primary surgery should only be attempted if it can be complete (ie, if it can achieve histologically negative margins), and nonmutilating excisions are naturally feasible; otherwise, neoadjuvant systemic treatment should be recommended.…”

Section: Discussionmentioning

confidence: 99%

“…Several observations from the adult literature that desmoid tumors can remain stable for a long time, with or without primary treatment, prompt the suggestion that a ''waitand-see'' approach (clinical/radiologic monitoring alone) may be suitable for patients who have nonevolving disease, and therapies should be given only in the event of tumor growth. 23,[27][28][29] Systemic treatment, and particularly low-dose chemotherapy, may play a significant role in the treatment of locally advanced disease; however, the role for adjuvant chemotherapy remains unclear. Although others have reported some benefits, 17 no useful information emerged from our series on its role for patients in Group II.…”

Section: Discussionmentioning

confidence: 99%

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Aggressive fibromatosis in children and adolescents

Meazza¹,

Bisogno

Gronchi³

et al. 2009

Cancer

132

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BACKGROUND:Aggressive fibromatosis (AF) is a rare tumor of intermediate malignancy that has a strong potential for local invasiveness and recurrence. To date, there are no general recommendations for the clinical management of pediatric AF.METHODS:The authors retrospectively analyzed 94 patients aged ≤21 years, including 23 patients who underwent complete surgery (Group I), 42 patients who underwent incomplete surgery with microscopic residual tumor (Group II), and 29 patients who underwent either biopsy or macroscopically incomplete surgery (Group III).RESULTS:The 5‐year event‐free survival (EFS) and overall survival rates were 44% and 99%, respectively. Local recurrences developed in 22% of patients in Group I, in 76% of patients in Group II, and in 76% of patients in Group III. Two of 7 patients with abdominal disease died of tumor progression, whereas none of the patients with extra‐abdominal AF died of their disease. Systemic treatment was given to 15 patients as first‐line treatment and to 34 patients at time the time they developed recurrent disease: The response rate was 47% in the former patients and 50% in the latter patients. Objective responses were observed in 11 of 19 patients who received combined methotrexate plus vinblastine/vinorelbine, in 7 of 15 patients who received alkylating‐agent chemotherapy, and in 4 of 11 patients who received other therapies (tamoxifen, sulindac, interferon alfa).CONCLUSIONS:The current analysis suggested that the clinical course of AF in children may resemble that of AF in adults. Local recurrences did not affect the chance of responding to systemic therapy or the survival rate. The completeness of initial resection was the main factor that influenced EFS, whereas disease control after marginal resection was much the same as that achieved after intralesional surgery/biopsy. Good responses to systemic treatments, and particularly to low‐dose chemotherapy, were observed as reported previously in adults. Cancer 2010. © 2010 American Cancer Society.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Aggressive fibromatosis in children and adolescents

Meazza¹,

Bisogno

Gronchi³

et al. 2009

Cancer

132

View full text Add to dashboard Cite

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“…9,10 Although unable to metastasize, these highly infiltrative and locally destructive lesions have a significant tendency to recur, with recurrence rates ranging from 19 to 38% in multiple studies. 2,[11][12][13][14][15][16][17][18][19][20][21][22][23] Currently there are no reliable methods to identify those tumors with a higher risk for recurrence; the molecular determinants of desmoid behavior remain primarily unknown. 23 Surgical resection remains the mainstay of desmoid therapy; however, these tumors frequently require multiple and at times debilitating surgical interventions for their control, resulting in significant treatment-related morbidity such as amputation or loss of significant portions of foregut.…”

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confidence: 99%

Specific Mutations in the β-Catenin Gene (CTNNB1) Correlate with Local Recurrence in Sporadic Desmoid Tumors

Lazar

Tuvin

Hajibashi

et al. 2008

The American Journal of Pathology

414

375

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Desmoid fibromatosis is a rare, nonmetastatic neoplasm marked by local invasiveness and relentless recurrence. Molecular determinants of desmoid recurrence remain obscure. ␤-Catenin deregulation has been commonly identified in sporadic desmoids although the incidence of CTNNB1 (the gene encoding ␤-catenin) mutations is uncertain. Consequently, we evaluated the prevalence of CTNNB1 mutations in a large cohort of sporadic desmoids and examined whether mutation type was relevant to desmoid outcome. Desmoid specimens (195 tumors from 160 patients, 1985 to 2005) and control dermal scars were assembled into a clinical data-linked tissue microarray. CTNNB1 genotyping was performed on a 138-sporadic desmoid subset. Immunohistochemical scoring was performed per standard criteria and data were analyzed using Kaplan-Meier and other indicated methods. CTNNB1 mutations were observed in 117 of 138 (85%) of desmoids. Three discrete mutations in two codons of CTNNB1 exon 3 were identified: 41A (59%) , 45F (33%) , and 45P (8% , excluded from further analysis because of rarity). Five-year recurrence-free survival was significantly poorer in 45F-mutated desmoids (23% , P < 0.0001) versus either 41A (57%) or nonmutated tumors (65%). Nuclear ␤-catenin expression was observed in 98% of specimens and intensity was inversely correlated with incidence of desmoid recurrence (P < 0.01). In conclusion, CTNNB1 mutations are highly common in desmoid tumors. Furthermore, patients harboring CTNNB1 (45F) mutations are at particular risk for recurrence and therefore may especially benefit from adjuvant therapeutic approaches. (Am J Pathol

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“…S. Phillips и соавт. [43] привели результаты лече-ния 23 пациентов по аналогичной методике. Некоторые авторы [44][45][46][47] отмечают удовлетворительные функцио-нальные результаты при использовании сетчатых проте-зов из политетрафторэтилена (ePTFE) и целлюлярного дермального матрикса.…”

Section: Discussionunclassified

Results of surgical treatment in patients with abdominal desmoid fibromas

Kostrygin¹,

Ryabov²,

Khomyakov³

et al. 2018

Onkol. Z. im. P.A. Gercena

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Десмоидные фибромы (ДФ) (син. -агрессивный фи-броматоз) -мезенхимальные опухоли, развивающиеся из дифференцированных фибробластов и избыточного ко-личества коллагеновых волокон [1].ДФ встречаются относительно редко, составляя 0,03% всех новообразований и 3% всех опухолей мягких тканей [1]. Частота составляет 2-4 человека на 1 млн населения. В США ежегодно регистрируется 900 новых случаев этого заболевания [2]. ДФ чаще встречаются у женщин, чем у мужчин. На долю пациентов мужского пола приходится не более 3,5% всех случаев заболеваний, женщин -96,5%. В основном ДФ развиваются в молодой популяции, чаще в возрасте 25-35 лет [1]. Цель исследования -улучшить онкологические, функциональные и эстетические результаты лечения пациентов с десмо-идными фибромами (ДФ) абдоминальной локализации за счет совершенствования хирургических методик. Материал и методы. Представлен опыт хирургического лечения 61 пациента с применением разработанной в МНИОИ им. П.А. Гер-цена оригинальной методики. При этом операции в объеме R0 были выполнены у 92% из 49 пациентов с первичными опухолями. У 58% R0-резекция выполнена в группе из 12 пациентов, которым первичная операция выполнена в другом ЛПУ, а в МНИОИ им. П.А. Герцена был диагностирован рецидив заболевания. Результаты. При длительности наблюдения от 1 до 92 мес (медиана 38,8 мес) рецидивов после R0-резекций не зарегистрировано. Все пациенты в настоящее время живы, продолжают наблюдение. У всех больных достигнут хороший косметический и функциональный результат и обе-спечено хорошее качество жизни. Заключение. Хирургическое вмешательство является основным методом радикального лечения ДФ абдоминальной локализации и обеспечивает стойкое выздоровление у большинства пациентов. Субтотальное удаление пораженной прямой мышцы живота в едином блоке с апоневротическим футляром (R0) из срединного доступа позволяет достигнуть низкой частоты местных рецидивов. При локализации ДФ в косых и поперечных мышцах живота необходимо выполнять широкое иссечение пораженных мышц в пределах здоровых тканей с соблюдением принципов футлярности. Objective -to improve the oncological, functional and aesthetic results of treatment in patients with abdominal desmoid fibromas (DFs), by improving surgical techniques. Subject and methods. The paper presents experience with surgical treatment in 61 patients, by applying the original procedure developed at the P.A. Herzen Moscow Oncology Research Institute (MORI). In this case, 92% of the 49 patients with primary tumors received R0 resection. The latter was made in 58% in a group of 12 patients in whom the primary operation had been performed in another healthcare facility; and a disease recurrence was diagnosed at the P.A. Herzen MORI. Results. No recurrences after R0-resections were recorded at a follow-up of 1 to 92 months (median, 38.8 months) in this study. All the patients are currently alive and continue to be followed up. All the patients achieved good cosmetic and functional results and had a good quality of life. Conclusion. Surgery is the main option for radical t...

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Aggressive fibromatosis of the abdominal wall, limbs and limb girdles

Cited by 77 publications

References 37 publications

Aggressive fibromatosis in children and adolescents

Aggressive fibromatosis in children and adolescents

Specific Mutations in the β-Catenin Gene (CTNNB1) Correlate with Local Recurrence in Sporadic Desmoid Tumors

Results of surgical treatment in patients with abdominal desmoid fibromas

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