関: desmoid

WordNet

having or showing determination and energetic pursuit of your ends; "an aggressive businessman"; "an aggressive basketball player"; "he was aggressive and imperious; positive in his convictions"; "aggressive drivers"
characteristic of an enemy or one eager to fight; "aggressive acts against another country"; "a belligerent tone" (同)belligerent
tending to spread quickly; "an aggressive tumor" (同)fast-growing, strong-growing

PrepTutorEJDIC

侵略的な,攻撃的な / 積極的な,反対を恐れない

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/05/22 17:08:59」(JST)

wiki en

Desmoid fibromatosis, H&E stain. Banal fibroblasts infiltrate the adjacent tissue in fascicles. Mitoses may be infrequent.

Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors. Desmoid tumors can arise in virtually any part of the body, and are tumors that arise from cells called fibroblasts, which are found throughout the body and provide structural support, protection to the vital organs, and play a critical role in wound healing. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. They can be either relatively slow-growing or malignant. However, aggressive fibromatosis is locally aggressive. When they are aggressive they can cause life-threatening problems or even death when they compress vital organs such as intestines, kidney, lungs, blood vessels, nerves etc. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.^[1]

Histologically they resemble very low-grade fibrosarcomas,^[2] but they are very locally aggressive and tend to recur even after complete resection. There is a tendency for recurrence in the setting of prior surgery; in one study, two-thirds of patients with desmoid tumors had a history of prior abdominal surgery.^[3]

Risk factors for desmoid disease amongst FAP patients include female sex, a 3' APC mutation, a positive family history and a history of previous abdominal surgery.^[4]

Classification

Desmoid tumors may be classified as extra-abdominal, abdominal wall, or intra-abdominal (the last is more common in patients with FAP). It is thought that the lesions may develop in relation to estrogen levels or trauma/operations.

A 3' APC mutation is the most significant risk factor for intra-abdominal desmoid development amongst FAP patients.^[5] FAP patients presenting with an abdominal wall desmoid pre-operatively are at an increased risk of developing an intra-abdominal desmoid post-operatively.^[6]

Desmoid tumours of the breast are rare. Although benign, they can mimic breast cancer on physical examination, mammography and breast ultrasound and can also be locally invasive. Even though they occur sporadically, they can also be seen as a part of Gardner's syndrome. A high index of suspicion and a thorough triple examination protocol is necessary to detect rare lesions like a desmoid tumour which can masquerade as breast carcinoma. Desmoid tumour of the breast may present a difﬁculty in the diagnosis especially where imaging studies are not conclusive and suggest a more ominous diagnosis.^[7]

Treatment

Treatment may consist of watching and waiting, complete surgical removal, radiation therapy, antiestrogens (ex. Tamoxifen), NSAIDs, chemotherapy or microwave ablation.

Patients with desmoid tumors should be evaluated by a multi-disciplinary team of surgeons, medical oncologists, radiation oncologists, geneticists and nurses. There is no cure for desmoid tumors and when possible patients are encouraged to enlist in clinical trials.^[8]

A biopsy is always indicated as the deﬁnitive method to determine nature of the tumour. Management of these lesions is complex, the main problem being the high rates of recurrence in FAP associated disease. Conversely, for intra-abdominal fibromatosis without evidence of FAP, although extensive surgery may still be required for local symptoms, the risk of recurrence appears to be lower.^[9] Wide surgical resection with clear margins is the most widely practiced technique with radiation, chemotherapy, or hormonal therapy being used to reduce the risk of recurrence.^[7]

Current experimental studies are being done with Gleevec (Imatinib) and Nexavar (sorafenib) for treatment of desmoid tumors, and show promising success rates.

References

^ Nieuwenhuis MH, De Vos Tot Nederveen Cappel W, Botma A, et al. (February 2008). "Desmoid tumors in a Dutch cohort of patients with familial adenomatous polyposis". Clin. Gastroenterol. Hepatol. 6 (2): 215–9. doi:10.1016/j.cgh.2007.11.011. PMID 18237870.
^ "desmoid" at Dorland's Medical Dictionary
^ Lynch HT, Fitzgibbons R (December 1996). "Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature review". Am. J. Gastroenterol. 91 (12): 2598–601. PMID 8946994.
^ Sinha A, Clark SK (2010). "Risk factors predicting desmoid occurrence in patients with familial adenomatous polyposis: a meta-analysis". Colorectal Dis. 13 (11): no. doi:10.1111/j.1463-1318.2010.02345.x. PMID 20528895.
^ Sinha A, Clark SK (June 2010). "Risk factors predicting intra-abdominal desmoids in familial adenomatous polyposis: a single centre experience". Tech Coloproctol. 14 (2): 141–6. doi:10.1007/s10151-010-0573-4. PMID 20352275.
^ Sinha A, Clark SK (2010). "Surgical prophylaxis in familial adenomatous polyposis: do pre-existing desmoids outside the abdominal cavity matter?". Fam Cancer 9 (3): 407–11. doi:10.1007/s10689-010-9342-9. PMID 20428953.
^ ^a ^b Rammohan A, Wood JJ (2012). "Desmoid tumour of the breast as a manifestation of Gardner's syndrome". Int J Surg Case Rep. 3 (5): 139–142. doi:10.1016/j.ijscr.2012.01.00. PMC 3312056. PMID 22370045.
^ http://www.dtrf.org/index.php/about-br-desmoid-tumors/about-desmoid-tumors.html
^ Wilkinson MJ, Fitzgerald JE, Thomas JM, Hayes AJ, Strauss DC (2012). "Surgical resection for non-familial adenomatous polyposis-related intra-abdominal fibromatosis". BJS. 99 (5): 706–13. doi:10.1002/bjs.8703. PMID 22359346.

External links

Aggressive (Desmoid) Fibromatosis: A Problem for the Sarcoma Team by Randall Burt, MD and David Viskochil, MD, PhD
Desmoid Tumor Research Foundation
[1]

UpToDate Contents

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1. デスモイド腫瘍：疫学、危険因子、分子学的病因、臨床症状、診断、および局所療法 desmoid tumors epidemiology risk factors molecular pathogenesis clinical presentation diagnosis and local therapy
2. デスモイド腫瘍：全身療法 desmoid tumors systemic therapy
3. ガードナー症候群 gardner syndrome
4. 軟部組織肉腫の臨床所見、病理組織学的検査、診断的評価、および病期分類 clinical presentation histopathology diagnostic evaluation and staging of soft tissue sarcoma
5. 末梢神経腫瘍 peripheral nerve tumors

English Journal

Fibromatosis arising in association with neuromuscular hamartoma of the mandible.

Taher LY, Saleem M, Velagapudi S, Dababo A.SourceDepartment of Pathology, Taibah University, Al-Madina, Saudi Arabia, leena.taher@gmail.com.
Head and neck pathology.Head Neck Pathol.2013 Sep;7(3):280-4. doi: 10.1007/s12105-012-0418-8. Epub 2013 Jan 12.
Aggressive fibromatosis is a benign but locally-aggressive tumor, which most often affects the muscles of the shoulder, the pelvic girdle, and the thigh. It usually affects adolescents and young adults. Desmoplastic fibroma, considered the bone counterpart of soft tissue fibromatosis, is a rare tumo
PMID 23315379

A 13 kg intra-abdominal mass: a case of mesenteric fibromatosis.

Menegazzo M, Tonello M, Bardini R.SourceUOC Chirurgia Generale, Azienda Ospedaliera Padova, Via Giustiniani 2, 35128, Padua, Italy.
Updates in surgery.Updates Surg.2013 Sep;65(3):237-40. doi: 10.1007/s13304-012-0143-6. Epub 2012 Mar 13.
Mesenteric fibromatosis is a benign fibrous tumor, characterized by proliferations of fibroblasts and myofibroblasts, locally aggressive but non-metastasizing. It can occur rarely in association with familial adenomatous polyposis or sporadically (related with previous trauma, abdominal surgery or p
PMID 22410827

Local recurrence after surgery for primary extra-abdominal desmoid-type fibromatosis.

van Broekhoven DL, Verhoef C, Elias SG, Witkamp AJ, van Gorp JM, van Geel BA, Wijrdeman HK, van Dalen T.SourceDepartment of Surgery, Diakonessenhuis Utrecht, Utrecht, The Netherlands.
The British journal of surgery.Br J Surg.2013 Aug;100(9):1214-9. doi: 10.1002/bjs.9194. Epub 2013 Jun 27.
BACKGROUND: Desmoid-type fibromatosis is a locally aggressive soft tissue tumour with a biological behaviour that varies between relatively indolent and progressive growth. Although there is a trend towards conservative treatment, surgery remains the standard treatment for extra-abdominal desmoid tu
PMID 23804156

Japanese Journal

胸壁デスモイド腫瘍局所再発に対して放射線治療が有効であった1例

羽隅透,斎藤泰紀,阿部二郎,星史彦,川村昌輝,田中遼太
日本呼吸器外科学会雑誌 = The journal of the Japanese Association for Chest Surgery 25(6), 672-677, 2011-09-15
症例は33歳，女性．左肩痛を主訴に来院．画像上，左胸郭入口部胸壁に径45×25mm大の腫瘤性病変を認めた．辺縁明瞭で周囲への明らかな浸潤像を認めないことから良性腫瘍と判断し，第4肋間後側方開胸にて切除を行った．術後病理検査にてデスモイド腫瘍の診断となり，また切除断端は陽性であった．術後1年5ヵ月目に腫瘍の断端部再発と左上肢痛の増悪を認めた．放射線治療を選択し60Gyの照射を行った．腫瘍縮小は軽度で …
NAID 10029458766

PS-072 再発を来し再手術でも断端陽性のaggressive fibromatosisの1例 : 人工膜使用と術後再発防止に関する考察(治療に難渋した良性疾患1,ポスターシンポジウム,病気の子供達に笑顔小児外科に夢そして革新を,第47回日本小児外科学会学術集会)

大竹紗弥香,西明,畑中政博,山本英輝,鈴木則夫,若井公子,平戸純子,黒岩実
日本小児外科学会雑誌 46(3), 526, 2010-05-20
NAID 110008015908

Related Pictures

★リンクテーブル★

リンク元	「侵襲性線維腫症」「進行性線維腫症」
関連記事	「aggressive」

「侵襲性線維腫症」

Aggressive fibromatosis
	MR scan of pelvis - desmoid tumor
Classification and external resources
ICD-10	D48.1
DiseasesDB	29794
eMedicine	article/1060887
MeSH	D018222
	[edit on Wikidata]