Gastrointestinal stromal tumor natural history: Difference between revisions

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__NOTOC__
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{{CMG}}{{AE}}{{PSD}}
{{Gastrointestinal stromal tumor}}
{{Gastrointestinal stromal tumor}}
{{CMG}}{{AE}}{{Akshun}}


==Overview==
==Overview==
Most common site of involvement of GIST is stomach(70%).
If left untreated, patients with gastrointestinal stromal tumors (GIST) may progress to develop [[abdominal pain]], [[abdominal distension]] and [[perforation]]. A [[benign]] GIST may remain unchanged for years before its progression into [[malignancy]]. A GIST may [[rupture]] and lead to intra-[[abdominal]] or [[Gastrointestinal Bleeding|gastrointestinal bleeding]]. Ultimately, the GIST may [[metastasize]] and turn fatal. Common [[Complication (medicine)|complications]] of GIST include [[bowel obstruction]], [[bowel perforation]], and [[peritonitis]]. Depending on the extent of the [[tumor]] at the time of [[diagnosis]], the [[prognosis]] of GIST may vary. [[Prognosis]] of GIST depends upon size, location, spread and [[mitotic]] rate of the [[tumor]]. A benign GIST treated with surgical [[resection]] has much more better outcome as compared to [[Patient|patients]] with [[malignant]] GIST.
 
==Natural history==
==Natural history==
Common sites of involvement include:
*Around 75% patients of gastrointestinal stromal tumors (GIST) are [[asymptomatic]] and are diagnosed as an [[incidental finding]] on [[imaging studies]] done for other reasons.
*If left untreated, [[Patient|patients]] with GIST may progress to develop [[abdominal pain]], [[abdominal distension]] and [[perforation]].
*Depending upon the type of GIST ([[benign]] or [[malignant]]), a [[benign]] GIST may remain unchanged for years before its progression into [[malignancy]].<ref name="pmid24707244">{{cite journal |vauthors=Antonopoulos P, Leonardou P, Barbagiannis N, Alexiou K, Demonakou M, Economou N |title=Gastrointestinal and extragastrointestinal stromal tumors: report of two cases and review of the literature |journal=Case Rep Gastroenterol |volume=8 |issue=1 |pages=61–6 |year=2014 |pmid=24707244 |pmc=3975174 |doi=10.1159/000354724 |url=}}</ref>
*GIST are highly [[vascular]] and an untreated [[tumor]] may attain massive size leading to [[rupture]] and intra-[[Abdomen|abdominal]] or [[gastrointestinal bleeding]].
*A ruptured GIST may lead to coffee ground [[emesis]], black stools or even [[melena]].
*Ultimately, the GIST may [[Metastasis|metastasize]] and turn fatal.


*Stomach: 70%
==Complications==
*Small intestine: 20-25%
Common complications of GIST include:<ref name="pmid24530605">{{cite journal |vauthors=Sorour MA, Kassem MI, Ghazal Ael-H, El-Riwini MT, Abu Nasr A |title=Gastrointestinal stromal tumors (GIST) related emergencies |journal=Int J Surg |volume=12 |issue=4 |pages=269–80 |year=2014 |pmid=24530605 |doi=10.1016/j.ijsu.2014.02.004 |url=}}</ref>
*Anorectum: 7%
*[[Gastrointestinal bleeding]]
*Oesophagus
*[[Bowel obstruction]]
*[[Bowel perforation]]
*[[Peritonitis]]
*[[Volvulus]]
*[[Intussusception]]
*Surgical [[complications]] associated with [[resection]] include:
**[[Atelectasis]]
**[[Pneumonia]]
**[[Urinary tract infection]]
**[[Abscess]] formation
**[[Small bowel obstruction|Small-bowel obstruction]]
**[[Deep vein thrombosis|Deep venous thrombosis]]


GISTs occur not only anywhere along the gastrointestinal tract, but also in the mesentery, omentum and retroperitoneum, which is called  extra-gastrointestinal GISTs. Metastatic lesions may also be seen in cases of malignant extra-gastrointestinal GISTs
==Prognosis==
==Prognosis==
At the time of clinical presentation, the prognosis appears to be influenced by genetic events other than kinase mutations, although a particular kinase mutation may help to define the initial clinical course of a GIST. Based on retrospective studies from time periods that predated the clinical use of kinase inhibitors, current recommendations for assessing the risk of progression for a newly diagnosed primary GIST rely on three parameters:
*Depending on the extent of the [[tumor]] at the time of [[diagnosis]], the [[prognosis]] of GIST may vary.<ref>{{Cite web | title =Risk Assessment and Prognosis
 
  | url =http://www.cancer.gov/types/soft-tissue-sarcoma/hp/gist-treatment-pdq#section/_1 }}</ref>  
*Mitotic index (mitoses per 50 high-power fields)
*[[Prognosis]] of GIST depends upon size, location, spread and [[mitotic]] rate of the [[tumor]]  ([[Mitosis|mitoses]] per 50 high-power fields).<ref name="pmid12094371">{{cite journal |vauthors=Dematteo RP, Heinrich MC, El-Rifai WM, Demetri G |title=Clinical management of gastrointestinal stromal tumors: before and after STI-571 |journal=Hum. Pathol. |volume=33 |issue=5 |pages=466–77 |year=2002 |pmid=12094371 |doi= |url=}}</ref><ref name="pmid11206225">{{cite journal |vauthors=Crosby JA, Catton CN, Davis A, Couture J, O'Sullivan B, Kandel R, Swallow CJ |title=Malignant gastrointestinal stromal tumors of the small intestine: a review of 50 cases from a prospective database |journal=Ann. Surg. Oncol. |volume=8 |issue=1 |pages=50–9 |year=2001 |pmid=11206225 |doi= |url=}}</ref><ref name="pmid7834450">{{cite journal |vauthors=Conlon KC, Casper ES, Brennan MF |title=Primary gastrointestinal sarcomas: analysis of prognostic variables |journal=Ann. Surg. Oncol. |volume=2 |issue=1 |pages=26–31 |year=1995 |pmid=7834450 |doi= |url=}}</ref><ref name="pmid26223313">{{cite journal |vauthors=Güller U, Tarantino I, Cerny T, Schmied BM, Warschkow R |title=Population-based SEER trend analysis of overall and cancer-specific survival in 5138 patients with gastrointestinal stromal tumor |journal=BMC Cancer |volume=15 |issue= |pages=557 |year=2015 |pmid=26223313 |pmc=4518595 |doi=10.1186/s12885-015-1554-9 |url=}}</ref><ref name="pmid10377927">{{cite journal |vauthors=Carney JA |title=Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence |journal=Mayo Clin. Proc. |volume=74 |issue=6 |pages=543–52 |year=1999 |pmid=10377927 |doi=10.4065/74.6.543 |url=}}</ref>
*Tumor size
**[[Patient|Patients]] with [[Stomach|gastric]] GIST have been reported to have better outlook as compared to [[Patient|patients]] with extragastric GIST.
*Tumor location<ref>{{Cite web | title =Risk Assessment and Prognosis
**[[Patient|Patients]] with localized primary [[disease]] have a average survival period of 5 years.
  | url =http://www.cancer.gov/types/soft-tissue-sarcoma/hp/gist-treatment-pdq#section/_1 }}</ref>
**[[Patient|Patients]] with [[malignant]] [[Lesion|lesions]] and [[metastasis]] have an average survival period of 1-2 years.
**[[Patient|Patients]] with [[Mitosis|mitotic]] rate of >10 per 50 HPF have an [[average]] survival period of 1.5-2 years
**[[Patient|Patients]] with [[mitotic]] rate <10 per HPF have an average survival [[period]] of 8 years.


==Refrences==
==Refrences==
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Latest revision as of 19:32, 4 March 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]

Overview

If left untreated, patients with gastrointestinal stromal tumors (GIST) may progress to develop abdominal pain, abdominal distension and perforation. A benign GIST may remain unchanged for years before its progression into malignancy. A GIST may rupture and lead to intra-abdominal or gastrointestinal bleeding. Ultimately, the GIST may metastasize and turn fatal. Common complications of GIST include bowel obstruction, bowel perforation, and peritonitis. Depending on the extent of the tumor at the time of diagnosis, the prognosis of GIST may vary. Prognosis of GIST depends upon size, location, spread and mitotic rate of the tumor. A benign GIST treated with surgical resection has much more better outcome as compared to patients with malignant GIST.

Natural history

Complications

Common complications of GIST include:[2]

Prognosis

Refrences

  1. Antonopoulos P, Leonardou P, Barbagiannis N, Alexiou K, Demonakou M, Economou N (2014). "Gastrointestinal and extragastrointestinal stromal tumors: report of two cases and review of the literature". Case Rep Gastroenterol. 8 (1): 61–6. doi:10.1159/000354724. PMC 3975174. PMID 24707244.
  2. Sorour MA, Kassem MI, Ghazal A, El-Riwini MT, Abu Nasr A (2014). "Gastrointestinal stromal tumors (GIST) related emergencies". Int J Surg. 12 (4): 269–80. doi:10.1016/j.ijsu.2014.02.004. PMID 24530605. Vancouver style error: initials (help)
  3. "Risk Assessment and Prognosis".
  4. Dematteo RP, Heinrich MC, El-Rifai WM, Demetri G (2002). "Clinical management of gastrointestinal stromal tumors: before and after STI-571". Hum. Pathol. 33 (5): 466–77. PMID 12094371.
  5. Crosby JA, Catton CN, Davis A, Couture J, O'Sullivan B, Kandel R, Swallow CJ (2001). "Malignant gastrointestinal stromal tumors of the small intestine: a review of 50 cases from a prospective database". Ann. Surg. Oncol. 8 (1): 50–9. PMID 11206225.
  6. Conlon KC, Casper ES, Brennan MF (1995). "Primary gastrointestinal sarcomas: analysis of prognostic variables". Ann. Surg. Oncol. 2 (1): 26–31. PMID 7834450.
  7. Güller U, Tarantino I, Cerny T, Schmied BM, Warschkow R (2015). "Population-based SEER trend analysis of overall and cancer-specific survival in 5138 patients with gastrointestinal stromal tumor". BMC Cancer. 15: 557. doi:10.1186/s12885-015-1554-9. PMC 4518595. PMID 26223313.
  8. Carney JA (1999). "Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence". Mayo Clin. Proc. 74 (6): 543–52. doi:10.4065/74.6.543. PMID 10377927.


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