Desmoid tumor overview: Difference between revisions
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If left untreated, desmoid tumor produces few symptoms early in the course. Common complications of desmoid tumor include progression to agressive fibromatosis, involvement of intra abdominal viscera, and metastasis. The prognosis varies with the associated disease of desmoid tumor. Desmoid tumor associated with FAP has the least favorable prognosis. | If left untreated, desmoid tumor produces few symptoms early in the course. Common complications of desmoid tumor include progression to agressive fibromatosis, involvement of intra abdominal viscera, and metastasis. The prognosis varies with the associated disease of desmoid tumor. Desmoid tumor associated with FAP has the least favorable prognosis. | ||
==History and Symptoms== | ==Diagnosis== | ||
===History and Symptoms=== | |||
Symptoms of desmoid tumor include uncharacteristic pain, [[abdominal mass]], [[nausea]], and [[vomiting]]. | Symptoms of desmoid tumor include uncharacteristic pain, [[abdominal mass]], [[nausea]], and [[vomiting]]. | ||
==Physical Examination== | ===Physical Examination=== | ||
Common physical examination findings of desmoid tumor include abdominal tenderness, pallor, and elevated temperature.<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | Common physical examination findings of desmoid tumor include abdominal tenderness, pallor, and elevated temperature.<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | ||
==CT== | ===CT=== | ||
CT scan may be diagnostic for desmoid tumor. On CT scan, desmoid tumor is characterized by well circumscribed mass, homogeneously or focally hyperattenuating, and tumor that will demonstrate enhancement following administration of intravenous contrast.<ref name="radio"> Desmoid tumor. Radiopedia(2015) http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015</ref><ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | CT scan may be diagnostic for desmoid tumor. On CT scan, desmoid tumor is characterized by well circumscribed mass, homogeneously or focally hyperattenuating, and tumor that will demonstrate enhancement following administration of intravenous contrast.<ref name="radio"> Desmoid tumor. Radiopedia(2015) http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015</ref><ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | ||
==MRI== | ===MRI=== | ||
Abdominal MRI may be diagnostic of desmoid tumor. On [[MRI]], desmoid tumor is characterized by dense cellularity and loss of signal following fat saturation.<ref name="radio"> Desmoid tumor. Radiopedia(2015) http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015</ref> | Abdominal MRI may be diagnostic of desmoid tumor. On [[MRI]], desmoid tumor is characterized by dense cellularity and loss of signal following fat saturation.<ref name="radio"> Desmoid tumor. Radiopedia(2015) http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015</ref> | ||
==Other Imaging Findings== | ===Other Imaging Findings=== | ||
Other imaging findings of desmoid tumor include ultrasonography, which demonstrates tumor size and location. On ultrasonography, desmoid tumors appear as well-defined lesions with variable echogenicity. The lateral borders may appear ill defined or irregular.<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | Other imaging findings of desmoid tumor include ultrasonography, which demonstrates tumor size and location. On ultrasonography, desmoid tumors appear as well-defined lesions with variable echogenicity. The lateral borders may appear ill defined or irregular.<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | ||
==Medical Therapy== | ==Treatment== | ||
===Medical Therapy=== | |||
Medical therapy for desmoid tumor includes [[antiestrogen]]s (ex. [[tamoxifen]]), [[non-steroidal anti-inflammatory drug|NSAIDs]], [[chemotherapy]] or microwave ablation. | Medical therapy for desmoid tumor includes [[antiestrogen]]s (ex. [[tamoxifen]]), [[non-steroidal anti-inflammatory drug|NSAIDs]], [[chemotherapy]] or microwave ablation. | ||
==Surgery== | ===Surgery=== | ||
Surgical resection is not recommended among patients with advanced or metastatic [[malignancy]].<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | Surgical resection is not recommended among patients with advanced or metastatic [[malignancy]].<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | ||
Revision as of 19:54, 26 January 2016
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Desmoid tumor Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2]
Overview
Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors. Desmoid tumors are tumors that arise from cells called fibroblasts. Fibroblasts are found throughout our body and their main function is to provide structural support and protection to the vital organs such as lung, liver, blood vessels, heart, kidneys, skin, intestines etc. and they also play a critical role in wound healing. When fibroblast cells undergo mutations they can become cancerous and become desmoid tumors (also known as "aggressive fibromatosis"). Desmoid tumors can arise in virtually any part of the body. These tumors often occur in women in their 30's, but can occur in anyone at any age. Desmoid tumors can be slow growing or extremely aggressive. These are slow-growing musculoaponeurotic tumors without any metastatic potential. 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]
Classification
Desmoid tumor may be classified according to etiology into 5 subtypes.[2]
Pathophysiology
Desmoid tumor may occur as part of Gardner syndrome. A minority of desmoid tumors are associated with Turcot syndrome, Familial adenomatous polyposis, and estrogen therapy.[3]
Causes
There are no established direct causes for desmoid tumor.
Differentiating Desmoid tumor from other Diseases
Desmoid tumor must be differentiated from acute hematoma, lymphoma, and fibrosarcoma.[2]
Epidemiology and Demographics
Desmoid tumor occurs in less than 0.03% of all tumors. The incidence of desmoid tumor is approximately 30 per 100,000 individuals worldwide. Females are more commonly affected with desmoid tumor than males. Desmoid tumor commonly affects individuals between 20 to 40 years of age.[4][2]
Risk Factors
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.[5]
Natural History, Complications and Prognosis
If left untreated, desmoid tumor produces few symptoms early in the course. Common complications of desmoid tumor include progression to agressive fibromatosis, involvement of intra abdominal viscera, and metastasis. The prognosis varies with the associated disease of desmoid tumor. Desmoid tumor associated with FAP has the least favorable prognosis.
Diagnosis
History and Symptoms
Symptoms of desmoid tumor include uncharacteristic pain, abdominal mass, nausea, and vomiting.
Physical Examination
Common physical examination findings of desmoid tumor include abdominal tenderness, pallor, and elevated temperature.[2]
CT
CT scan may be diagnostic for desmoid tumor. On CT scan, desmoid tumor is characterized by well circumscribed mass, homogeneously or focally hyperattenuating, and tumor that will demonstrate enhancement following administration of intravenous contrast.[3][2]
MRI
Abdominal MRI may be diagnostic of desmoid tumor. On MRI, desmoid tumor is characterized by dense cellularity and loss of signal following fat saturation.[3]
Other Imaging Findings
Other imaging findings of desmoid tumor include ultrasonography, which demonstrates tumor size and location. On ultrasonography, desmoid tumors appear as well-defined lesions with variable echogenicity. The lateral borders may appear ill defined or irregular.[2]
Treatment
Medical Therapy
Medical therapy for desmoid tumor includes antiestrogens (ex. tamoxifen), NSAIDs, chemotherapy or microwave ablation.
Surgery
Surgical resection is not recommended among patients with advanced or metastatic malignancy.[2]
Reference
- ↑ 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.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Economou, Athanasios; Pitta, Xanthi; Andreadis, Efstathios; Papapavlou, Leonidas; Chrissidis, Thomas (2011). "Desmoid tumor of the abdominal wall: a case report". Journal of Medical Case Reports. 5 (1): 326. doi:10.1186/1752-1947-5-326. ISSN 1752-1947.
- ↑ 3.0 3.1 3.2 Desmoid tumor. Dr Tim Luijkx and Radswiki et al. Radiopedia 2015 http://radiopaedia.org/articles/aggressive-fibromatosis. Accessed on January 20, 2015
- ↑ Desmoid tumor. Dr Tim Luijkx and Radswiki et al.Radiopaedia 2015.http://radiopaedia.org/articles/desmoid-tumour. Accessed on January 21, 2016
- ↑ 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.