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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/08/20 03:57:54」(JST)

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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, which are benign, slow-growing tumors without any metastatic potential. However, aggressive fibromatosis is locally aggressive. Despite their benign nature, they can damage nearby structures causing organ dysfunction. 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 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 gender, 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 and NSAIDs, or chemotherapy.

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. Wide surgical resection with clear margins is the most widely practiced technique with radiation, chemotherapy, or hormonal therapy being used to reduce recurrence rates.^[7]

Current experimental studies are being done with Gleevec (Imatinib) 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. http://linkinghub.elsevier.com/retrieve/pii/S1542-3565(07)01107-X.
^ "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. http://www.sciencedirect.com/science/article/pii/S2210261212000120.

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. 隆起性皮膚線維肉腫：治療 dermatofibrosarcoma protuberans treatment
5. 隆起性皮膚線維肉腫：疫学、病因、臨床症状、診断、および病期分類 dermatofibrosarcoma protuberans epidemiology pathogenesis clinical presentation diagnosis and staging

English Journal

Treatment and follow-up strategies in desmoid tumours: a practice guideline.

Ghert M1, Yao X2, Corbett T3, Gupta AA4, Kandel RA5, Verma S6, Werier J7.
Current oncology (Toronto, Ont.).Curr Oncol.2014 Aug;21(4):e642-9. doi: 10.3747/co.21.2112.
OBJECTIVES: We set out to determine the optimal treatment options-surgery, radiation therapy (rt), systemic therapy, or any combinations thereof-for patients with desmoid tumours once the decision to undergo active treatment has been made (that is, monitoring and observation have been determined to
PMID 25089635

A systematic review of active treatment options in patients with desmoid tumours.

Yao X1, Corbett T2, Gupta AA3, Kandel RA4, Verma S5, Werier J6, Ghert M7.
Current oncology (Toronto, Ont.).Curr Oncol.2014 Aug;21(4):e613-29. doi: 10.3747/co.21.1995.
INTRODUCTION: We conducted a systematic review to determine the optimal treatment options in patients with desmoid tumours who have declined observational management.METHODS: A search was conducted of the medline and embase databases (1990 to September 2012), the Cochrane Library, and relevant guide
PMID 25089111

Advances in therapy for pediatric sarcomas.

Weiss A1, Gill J, Goldberg J, Lagmay J, Spraker-Perlman H, Venkatramani R, Reed D.
Current oncology reports.Curr Oncol Rep.2014 Aug;16(8):395. doi: 10.1007/s11912-014-0395-z.
Pediatric sarcomas are relatively rare malignancies individually. As a group they are typically approached with combination chemotherapies in addition to local control. Fortunately, these malignancies have been approached through careful clinical trial collaboration to define risk groups and appropr
PMID 24894064

Japanese Journal

後腹膜腔に発生したデスモイド腫瘍の1例 (特集泌尿器最新症例集2012)

原田詩乃,宮田景子,安森弘太郎他
臨床放射線 57(7), 913-917, 2012-07-00
NAID 40019381005

デスモイド腫瘍の治療 (シンポジウム難治性良性腫瘍の治療)

五嶋孝博
臨床整形外科 47(6), 521-525, 2012-06-00
NAID 40019343001

Related Pictures

★リンクテーブル★

リンク元	「デスモイド」「類線維腫」「類腱腫」「fibroid tumor」「aggressive fibromatosis」

「デスモイド」

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