Skip to main content
Download PDF

Extra-gastrointestinal stromal tumor arising in the lesser omentum with a platelet-derived growth factor receptor alpha (PDGFRA) mutation: a case report and literature review

World Journal of Surgical Oncology volume 18, Article number: 183 (2020) Cite this article

Abstract

Background

Gastrointestinal stromal tumors (GIST) arising from sites other than the gastrointestinal (GI) tract, termed extra-gastrointestinal stromal tumors (EGIST), are rare. Among EGIST, those with platelet-derived growth factor receptor alpha (PDGFRA) mutations are even rarer, with only a few cases reported. About 80% of GIST has KIT mutations, and 10% of GIST have PDGFRA mutations, which commonly affect the TK2 domain (exon 18). Among the exon 18 mutations, the D842V substitution is limited to gastric GIST. In EGIST, the degree of KIT and PDGFRA mutations varies on where the location of the tumor is, and it is suggested that omental EGIST is similar to gastric GIST. Adjuvant imatinib therapy is recommended for high-risk GIST; however, it is known that imatinib is less effective against GIST with a PDGFRA D842V mutation.

Case presentation

A 75-year-old man was referred to our hospital with an extrinsic tumor of the lesser curvature of the gastric body. Intraoperative findings showed a tumor located outside of the lesser omentum with no connection between the tumor and the gastric wall. The tumor was subsequently resected. Pathological examination indicated a GIST arising in the lesser omentum measuring 70 mm in its longer dimension. Because the tumor had a PDGFRA mutation (D842V substitution), imatinib was suspected to lack efficacy to the tumor. Thus, although the tumor was considered clinically to have a high risk of recurrence, adjuvant imatinib therapy was not indicated. The patient has been free of recurrence for 29 months since the surgery.

Conclusion

We described a case of EGIST with a PDGFRA mutation arising in the lesser omentum. And we reviewed 57 cases of omental EGIST and showed that the clinicopathological characteristics and mutation status in omental EGIST were very similar to gastric GIST. In particular, PDGFAR D842V mutation rate in omental EGIST seemed as high as that in gastric GIST. These results suggested that omental EGIST is strongly related to gastric GIST, so the behavior of omental EGIST might be akin to gastric GIST. However, further studies are required to determine the prognosis and the necessity of adjuvant therapy for EGIST with a PDGFRA mutation.

Background

A gastrointestinal stromal tumor (GIST) is a relatively rare mesenchymal tumor of the gastrointestinal (GI) tract and is defined as a spindle cell or epithelioid neoplasm expressing a c-kit (CD117) immunophenotype, which is the tyrosine kinase (TK) component [1]. GIST usually has KIT mutations; however, a few have been reported with mutations in platelet-derived growth factor receptor alpha (PDGFRA) [2]. As mentioned above, GIST usually arises from the GI tract; however, entity GIST termed extra-gastrointestinal stromal tumors (EGIST) may arise in sites other than the GI tract. EGIST represent less than 5% of all stromal tumors [3]. Similar to GIST arising in the GI tract, EGIST usually show KIT mutations; however, EGIST with PDGFRA mutations appear to be rare. We report a case of EGIST with a PDGFRA mutation arising in the lesser omentum.

Case presentation

A 75-year-old man was referred to our hospital due to an abdominal mass detected by abdominal ultrasonography performed during a health check. He had histories of diabetes mellitus, hypertension, hyperlipidemia, and gallstones. Esophagogastroduodenoscopy (EGD) revealed a gently sloping protruding lesion with smooth surface mucosa, located anterior to the lesser curvature of the gastric body (Fig. 1a). Endoscopic ultrasonography (EUS) showed a submucosal hypoechoic tumor with a diameter of 79 × 58 mm that seemed to be connected to the smooth muscle layer of the stomach (Fig. 1b). Due to the risk of dissemination, fine-needle aspiration biopsy was not performed. Enhanced computed tomography (CT) revealed a solid tumor, 70-mm in diameter, which also seemed to be connected to the lesser curvature side of the gastric body (Fig. 1c). Based on the results of EGD, EUS, and CT, we diagnosed this tumor as an extramural growth-type gastric submucosal tumor highly suspicious of GIST and planned surgical resection. Resection of the tumor through laparotomy was performed. The tumor was located at lesser omentum, measured 70 mm in its longer dimension, and was connected to the lesser omentum. The tumor was very close to the stomach; however, no connection was present between the tumor and gastric wall, and it could be resected without rupturing the pseudocapsule and did not contain any gastric component. The tumor had a smooth surface; the cut surface was solid with extensive hemorrhage and degeneration (Fig. 2). Histological examination showed the proliferation of epithelioid to spindle-shaped tumor cells with oval, rather uniform nuclei (Fig. 3a). The mitotic count was less than 1 per 50 high-power fields (HPFs). The tumor cells were immunohistochemically positive for KIT, CD34, and discovered on GIST-1 (DOG-1) but negative for S-100 and alpha-SMA (Fig. 3b–d). The Ki-67 labeling index was 3.3%. We finally diagnosed the tumor as an EGIST originating from the lesser omentum.

Fig. 1
figure 1

Findings of preoperative examination. Esophagogastroduodenoscopy (EGD) revealed a gently sloping protruding lesion with smooth surface mucosa, located anterior to the lesser curvature of the gastric body. Ultrasonic endoscopy (EUS) showed an extra-stomach hypoechoic tumor with a diameter of 79 × 58 mm that seemed to be connected to the smooth muscle layer of the stomach. Enhanced computed tomography (CT) revealed a solid tumor, 70-mm in diameter, which also seemed to be connected to the lesser curvature side of the gastric body

Full size image
Fig. 2
figure 2

Macroscopic tumor findings. The tumor was very close to the stomach; however, the pseudocapsule of the tumor was fully kept without rupture and the resected specimen did not contain any gastric component. The tumor had a smooth surface and consisted of a solid component with extensive hemorrhage and degeneration

Full size image
Fig. 3
figure 3

Histological and immunohistochemical tumor findings. Hematoxylin and eosin (HE) staining showing spindle-shaped tumor cells (a). Positive immunohistochemistry for KIT (b), CD34 (c), and DOG-1 (d)

Full size image

According to Joensuu’s classification, the size was 70 mm (over 50 mm) and the location of the tumor was “nongastric,” we considered this tumor high-risk [4]. Then, to determine the indication of adjuvant therapy, we performed a genetic examination of the tumor, which revealed no KIT mutation but instead revealed a PDGFRA D842V substitution. Based on a previous report that imatinib was less effective against GIST with a PDGFRA D842V mutation [2], adjuvant imatinib therapy was not indicated for the present patient. Instead, the patient has been carefully followed up by CT examination every 6 months and remains free of recurrence for 29 months after surgery.

Discussion

GISTs are specific mesenchymal tumors that are considered to originate from the interstitial cells of Cajal (ICCs), the pacemaker of the peristaltic movement of the GI tract [5]. Hence, they usually occur throughout the length of the GI tract, from the esophagus to the anus. However, there are GISTs arising in sites other than the GI tract, such as the omentum, mesentery, and retroperitoneum. Those GISTs are called EGIST [3].

GIST is a relatively rare tumor, which occurs in 6.8–14.5 cases per million people per year [6]. The common primary sites for GIST are the stomach (60–70%), jejunum (25–30%), colorectum (5–15%), and duodenum (5%) [7, 8]. EGISTs are estimated less than 10% of all GISTs, and they occur in the mesentery (7.7–29.4%), retroperitoneum (25.5–25.6%), and omentum (7.7–15.7%) [3, 9, 10]. Hence, it is suggested that this case, EGIST in the omentum, seems to be extremely rare.

Some studies have reported that there are similar epidemiological and histological findings between GIST and EGIST [9, 11]. We searched all articles on EGIST in the PubMed database, published up to December 2019 using the following keywords: extra-gastrointestinal stromal tumor, EGIST, gastrointestinal stromal tumor, GIST, omental, and omentum. We also examined the reference sections of published articles. We excluded articles with obvious irrelevance and finally acquired and reviewed 57 cases of omental EGIST from 25 series of small case series and case reports. The characteristics of these cases were summarized and compared with whole GIST in Table 1 [6, 10, 12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35]. The age at onset, gender ratio, and histological form of omental EGIST were very similar to those of GIST.

Table 1 Clinicopathological findings of patients with omental extra-gastrointestinal stromal tumors (n = 57) [6, 10, 12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35]
Full size table

KIT and/or PDGFRA mutations are thought to be the main drivers of GIST [2]. About 80% of GISTs have KIT mutations, of which the most common are in-frame deletions or insertions or missense mutations within the juxtamembrane domain (exon 11). And 10% of GIST have PDGFRA mutations, which commonly affect the TK2 domain (exon 18) [2, 36,37,38]. However, some reports showed that the proportion of these mutations depends on the location of GIST [7, 39]. In particular, among the exon 18 mutations, the D842V substitution is limited to GIST of the stomach [2, 36,37,38]. Also, in EGIST, the degree of KIT and PDGFRA mutations varies on where the location of the tumor is [3]. Namely, it is suggested that omental EGIST is similar to gastric GIST, mesenteric is similar to intestinal, and retroperitoneal can take either gastric or intestinal characteristics [11]. Within 57 omental EGIST cases we reviewed this time, 25 cases described about gene mutation; gene mutations of these cases were summarized and compared with GIST of other organs in Table 2 [7, 10, 14, 17, 19, 21, 24,25,26, 28, 39]. Mutation status in omental EGIST was very similar to gastric GIST. In particular, PDGFRA D842V mutation rate of omental EGIST seemed comparable with gastric GIST. These results suggested that omental EGIST is strongly related to gastric GIST, so the behavior of omental EGIST might be akin to gastric GIST.

Table 2 Mutation subtypes according to the primary location of GIST [7, 10, 14, 17, 19, 21, 24,25,26, 28, 39]
Full size table

These results may provide some suggestions into the question of where omental EGIST originates. There are some hypotheses about the origin of EGIST: (1) they are derived from extraintestinal undifferentiated mesenchymal cells capable of differentiating to ICCs [14], (2) they originate from mesenchymal undifferentiated stem cells [13], and (3) they lose the extramural contact with the intestinal wall in GISTs [40]. However, their origin remains unclear due to their rarity. Further accumulation of EGIST cases and related studies is needed.

The standard treatment for GIST without metastasis is surgical complete resection. As for EGIST also, complete resection must be the only way to cure at present. As discussed above, omental EGIST has a similar clinicopathological and genetic background to gastric GIST. However, the prognosis of EGIST, including omental EGIST, after resection is thought to be less favorable than that of GIST [3, 9, 11]. -The reason is suggested that EGIST is often found in a very large state at the onset [9,10,11]. In fact, as shown in Table 1, the median tumor diameter at the onset of omental EGIST was 15 cm, extremely large. Because EGIST is not exposed to the GI tract, it is unlikely to show symptoms such as bleeding into the digestive tract or stenosis, and then the tumor detection tends to be delayed. And the larger the tumor grows, the worse some parameters predicting the prognosis, such as mitotic activity, cellularity, nuclear atypia, and necrosis, become, so it is convincing that the prognosis of EGIST, which is often found in larger states, is worse than that of GIST [27, 35].

The risk factors for recurrence of GIST are tumor size in the largest dimension > 5 cm and mitotic count > 5 per 50 HPF [2]. For GIST belonging to these high-risk groups, postoperative adjuvant imatinib therapy is known to improve prognosis [41, 42]. However, the response of GISTs to imatinib treatment depends on the mutation status. While most GISTs are sensitive to imatinib, those with PDGFRA D842V substitutions (exon 18) are resistant to imatinib [43, 44]. There is a report that the recurrence-free survival after curative resection in patients with PDGFRA mutations was more favorable than in patients with KIT mutations [37]. Owing to imatinib resistance and favorable prognosis, patients with GIST bearing PDGFRA D842V substitutions are not generally considered to be candidates for adjuvant imatinib therapy [44, 45]. As for EGIST, prognostic data is scarce and reported mutations are even more limited; hence, there is no consensus regarding the recommendations for adjuvant imatinib for this tumor. Prognosis of 5 cases who bare PDGFRA D842V substitutions in our review was 4–120 months (median 38 months) without recurrence and mortality. The prognosis seemed to be not so bad; however, the number of the cases was too small. Further studies are required to identify the risk factors of EGIST recurrence. At present, it is at best to deduce the results of GIST and apply them to EGIST.

In this case, we observed a mitotic count of < 1 per 50 HPF and Ki-67 labeling index of 3.3%. The estimated risk of tumor recurrence from these factors seemed to be relatively low [2, 46]. However, we emphasized the fact that the tumor was relatively large in 70 mm diameter (> 50 mm) and the tumor was EGIST (located outside the GI tract); we regarded this case as high-risk. As mentioned above, omental EGIST has a certain number of PDGFRA mutations; we previously performed a genomic examination to determine the indication of adjuvant therapy. Then, PDGFRA D842V substitutions were shown; this tumor was expected to be resistant to imatinib. Thus, the patient was not considered to be a candidate for adjuvant imatinib and was instead followed up frequently. Some TK inhibitors, such as avapritinib and crenolanib, had shown certain potent activities for GIST with PDGFRA mutations [47]. Early development of these drugs is expected.

Conclusion

We described an extremely rare case of EGIST arising in the lesser omentum with a PDGFRA mutation. Our review showed that there were many clinicopathological and genetic similarities between omental EGIST and gastric GIST. However, the prognosis of omental EGIST was rather poorer than that of gastric GIST; much about EGIST, such as its origin and the effects of imatinib, still remains unknown. Further research is required.

Availability of data and materials

All data generated during this study are included in this published article.

Abbreviations

GIST:

Gastrointestinal stromal tumor

GI:

Gastrointestinal

PDGFRA:

Platelet-derived growth factor receptor alpha

TK:

Tyrosine kinase

EGIST:

Extra-gastrointestinal stromal tumor

EGD:

Esophagogastroduodenoscopy

EUS:

Endoscopic ultrasonography

CT:

Computed tomography

HE:

Hematoxylin-eosin

HPF:

High-power fields

DOG-1:

Discovered on GIST-1

ICC:

Interstitial cells of Cajal

References

  1. Miettinen M, Lasota J. Gastrointestinal stromal tumors--definition, clinical histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001;438:1–12.

    CAS  PubMed  Google Scholar 

  2. Rubin BP, Heinrich MC, Corless CL. Gastrointestinal stromal tumour. Lancet. 2007;369:1731–41.

    CAS  PubMed  Google Scholar 

  3. Yi JH, Park BB, Kang JH, Hwang IG, Shin DB, Sym SJ, Ahn HK, Lee SI, Lim DH, Park KW, et al. Retrospective analysis of extra-gastrointestinal stromal tumors. World J Gastroenterol. 2015;21:1845–50.

    PubMed  PubMed Central  Google Scholar 

  4. Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol. 2008;39:1411–09.

    PubMed  Google Scholar 

  5. Wang X, Mori I, Tang W, Utsunomiya H, Nakamura M, Nakamura Y, Zhou G, Kakudo K. Gastrointestinal stromal tumors: are they of Cajal cell origin? Exp Mol Pathol. 2002;72:172–7.

    CAS  PubMed  Google Scholar 

  6. Akahoshi K, Oya M, Koga T, Shiratsuchi Y. Current clinical management of gastrointestinal stromal tumor. World J Gastroenterol. 2018;24:2806–17.

    PubMed  PubMed Central  Google Scholar 

  7. Barnett CM, Corless CL, Heinrich MC. Gastrointestinal stromal tumors: molecular markers and genetic subtypes. Hematol Oncol Clin North Am. 2013;27:871–88.

    PubMed  Google Scholar 

  8. Theiss L, Contreras CM. Gastrointestinal stromal tumors of the stomach and esophagus. Surg Clin North Am. 2019;99:543–53.

    PubMed  Google Scholar 

  9. Apostolou KG, Schizas D, Vavouraki E, Michalinos A, Tsilimigras DI, Garmpis N, Damaskos C, Papalampros A, Liakakos T. Clinicopathological and molecular factors, risk factors, treatment outcomes and risk of recurrence in mesenteric and retroperitoneal extragastrointestinal stromal tumors. Anticancer Res. 2018;38:1903–9.

    PubMed  Google Scholar 

  10. Yamamoto H, Oda Y, Kawaguchi K, Nakamura N, Takahira T, Tamiya S, Saito T, Oshiro Y, Ohta M, Yao T. Tsuneyoshi M: c-kit and PDGFRA mutations in extragastrointestinal stromal tumor (gastrointestinal stromal tumor of the soft tissue). Am J Surg Pathol. 2004;28:479–88.

    PubMed  Google Scholar 

  11. Agaimy A, Wünsch PH. Gastrointestinal stromal tumours: a regular origin in the muscularis propria, but an extremely diverse gross presentation. A Review of 200 Cases to Critically Re-Evaluate the Concept of So-Called Extra-Gastrointestinal Stromal Tumours. Langenbecks Arch Surg. 2006;391:322–9.

    PubMed  Google Scholar 

  12. Takahashi T, Kuwao S, Yanagihara M, Kakita A. A primary solitary tumor of the lesser omentum with immunohistochemical features of gastrointestinal stromal tumors. Am J Gastroenterol. 1998;93:2269–73.

    CAS  PubMed  Google Scholar 

  13. Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr NJ, Emory TS, Sobin LH. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol. 1999;23:1109–18.

    CAS  PubMed  Google Scholar 

  14. Sakurai S, Hishima T, Takazawa Y, Sano T, Nakajima T, Saito K, Morinaga S, Fukayama M. Gastrointestinal stromal tumors and KIT-positive mesenchymal cells in the omentum. Pathol Int. 2001;51:524–31.

    CAS  PubMed  Google Scholar 

  15. Fukuda H, Suwa T, Kimura F, Sugiura T, Shinoda T, Kaneko K. Gastrointestinal stromal tumor of the lesser omentum: report of a case. Surg Today. 2001;31:715–8.

    CAS  PubMed  Google Scholar 

  16. Suzuki K, Kaneko G, Kubota K, Horigome N, Hikita H, Senga O, Miyakawa M, Shimojo H, Uehara T, Itoh N. Malignant tumor, of the gastrointestinal stromal tumor type, in the greater omentum. J Gastroenterol. 2003;38:985–8.

    PubMed  Google Scholar 

  17. Sakurai S, Hasegawa T, Sakuma Y, Takazawa Y, Motegi A, Nakajima T, Saito K, Fukayama M, Shimoda T. Myxoid epithelioid gastrointestinal stromal tumor (GIST) with mast cell infiltrations: a subtype of GIST with mutations of platelet-derived growth factor receptor alpha gene. Hum Pathol. 2004;35:1223–30.

    CAS  PubMed  Google Scholar 

  18. Caricato M, Ausania F, Valeri S, Rabitti C, Tonini G, Coppola R. An omental mass: any hypothesis? Colorectal Dis. 2005;7:417–8.

    CAS  PubMed  Google Scholar 

  19. Todoroki T, Sano T, Sakurai S, Segawa A, Saitoh T, Fujikawa K, Yamada S, Hirahara N, Tsushima Y, Motojima R, Motojima T. Primary omental gastrointestinal stromal tumor (GIST). World J Surg Oncol. 2007;5:66.

    PubMed  PubMed Central  Google Scholar 

  20. Gun BD, Gun MO, Karamanoglu Z. Primary stromal tumor of the omentum: report of a case. Surg Today. 2006;36:994–6.

    PubMed  Google Scholar 

  21. Kim JH, Boo YJ, Jung CW, Park SS, Kim SJ, Mok YJ, Kim SD, Chae YS, Kim CS. Multiple malignant extragastrointestinal stromal tumors of the greater omentum and results of immunohistochemistry and mutation analysis: a case report. World J Gastroenterol. 2007;13:3392–5.

    CAS  PubMed  PubMed Central  Google Scholar 

  22. Franzini C, Alessandri L, Piscioli I, Donato S, Faraci R, Morelli L, Del Nonno F, Licci S. Extra-gastrointestinal stromal tumor of the greater omentum: report of a case and review of the literature. World J Surg Oncol. 2008;6:25.

    PubMed  PubMed Central  Google Scholar 

  23. Castillo-Sang M, Mancho S, Tsang AW, Gociman B, Almaroof B, Ahmed MY. A malignant omental extra-gastrointestinal stromal tumor on a young man: a case report and review of the literature. World J Surg Oncol. 2008;6:5012.

    Google Scholar 

  24. Terada T. Primary multiple extragastrointestinal stromal tumors of the omentum with different mutations of c-kit gene. World J Gastroenterol. 2008;14:7256–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  25. Dedemadi G, Georgoulis G, Kontopanos D, Anagnostou E, Morphopoulos G, Emile JF, Christopoulos C. Extragastrointestinal stromal tumors of the omentum: review apropos of a case with a novel gain-of-function KIT mutation. J Gastrointest Cancer. 2009;40:73–8.

    PubMed  Google Scholar 

  26. Zheng S, Huang KE, Tao DY, Pan YL. Gene mutations and prognostic factors analysis in extragastrointestinal stromal tumor of a Chinese three-center study. J Gastrointest Surg. 2011;15:675–81.

    CAS  PubMed  Google Scholar 

  27. Mouaqit O, Jahid A, Ifrine L. Omar El Malki H, Mohsine R, Mahassini N, Belkouchi A: Primary omental gastrointestinal stromal tumors. Clin Res Hepatol Gastroenterol. 2011;35:590–3.

    CAS  PubMed  Google Scholar 

  28. Kim KH, Nelson SD, Kim DH, Choi KU, Kim SJ, Min KW, Jang KS, Paik SS, Oh YH, Chae SW, et al. Diagnostic relevance of overexpressions of PKC-theta and DOG-1 and KIT/PDGFRA gene mutations in extragastrointestinal stromal tumors: a Korean six-centers study of 28 cases. Anticancer Res. 2012;32:923–37.

    CAS  PubMed  Google Scholar 

  29. Ogawa H, Gotoh K, Yamada T, Takahashi H, Ohigashi H, Nagata S, Tomita Y, Yano M, Ishikawa O. A case of KIT-negative extra-gastrointestinal stromal tumor of the lesser omentum. Case Rep Gastroenterol. 2012;6:375–80.

    PubMed  PubMed Central  Google Scholar 

  30. Fagkrezos D, Touloumis Z, Giannila M, Penlidis C, Papaparaskeva K, Triantopoulou C. Extra-gastrointestinal stromal tumor of the omentum: a rare case report and review of the literature. Rare Tumors. 2012;4:e44.

    PubMed  PubMed Central  Google Scholar 

  31. Behammane H, Aggouri Y, Oussaid M, Laalim SA, Toughrai I. Ibn majdoub K. Mazaz K: Stromal tumor of the lesser omentum : a case report. Pan Afr Med J. 2014;17:236.

    PubMed  Google Scholar 

  32. Skandalos IK, Hotzoglou NF, Matsi K, Pitta XA, Kamas AI. Giant extra gastrointestinal stromal tumor of lesser omentum obscuring the diagnosis of a choloperitoneum. Int J Surg Case Rep. 2013;4:818–21.

    PubMed  PubMed Central  Google Scholar 

  33. Trombatore C, Palmucci S, Angelico G, Vasquez E, Petrillo G, Puleo S, Di Cataldo A. Extragastrointestinal stromal tumor of lesser omentum: a challenging radiological and histological diagnosis. Clin Imaging. 2015;39:1123–7.

    PubMed  Google Scholar 

  34. Dimofte MG, Porumb V, Ferariu D, Bar NC, Lunca S. EGIST of the greater omentum - case study and review of literature. Rom J Morphol Embryol. 2016;57:253–8.

    PubMed  Google Scholar 

  35. Patnayak R, Jena A, Parthasarathy S, Prasad PD, Reddy MK, Chowhan AK, Rukamangadha N, Phaneendra BV. Primary extragastrointestinal stromal tumors: a clinicopathological and immunohistochemical study - a tertiary care center experience. Indian J Cancer. 2013;50:41–5.

    CAS  PubMed  Google Scholar 

  36. Heinrich MC, Corless CL, Demetri GD, Blanke CD, von Mehren M, Joensuu H, McGreevey LS, Chen CJ, Van den Abbeele AD, Druker BJ, et al. Kinase mutations and imatinib response in patients with metastatic gastrointestinal stromal tumor. J Clin Oncol. 2003;21:4342–9.

    CAS  PubMed  Google Scholar 

  37. Joensuu H, Rutkowski P, Nishida T, Steigen SE, Brabec P, Plank L, Nilsson B, Braconi C, Bordoni A, Magnusson MK, et al. KIT and PDGFRA mutations and the risk of GI stromal tumor recurrence. J Clin Oncol. 2015;33:634–42.

    CAS  PubMed  Google Scholar 

  38. Debiec-Rychter M, Dumez H, Judson I, Wasag B, Verweij J, Brown M, Dimitrijevic S, Sciot R, Stul M, Vranck H, et al. Use of c-KIT/PDGFRA mutational analysis to predict the clinical response to imatinib in patients with advanced gastrointestinal stromal tumours entered on phase I and II studies of the EORTC Soft Tissue and Bone Sarcoma Group. Eur J Cancer. 2004;40:689–95.

    CAS  PubMed  Google Scholar 

  39. Wardelmann E, Hohenberger P, Reichardt P, Merkelbach-Bruse S, Schildhaus H-U, Büttner R: Gastrointestinal stromal tumors of the stomach. Updates and differences compared to other locations. Pathologe 2010, 31:195-198.

  40. Goh BK, Chow PK, Kesavan SM, Yap WM, Chung YF, Wong WK. A single-institution experience with eight CD117-positive primary extragastrointestinal stromal tumors: critical appraisal and a comparison with their gastrointestinal counterparts. J Gastrointest Surg. 2009;13:1094–8.

    PubMed  Google Scholar 

  41. Dematteo RP, Ballman KV, Antonescu CR, Maki RG, Pisters PW, Demetri GD, Blackstein ME, Blanke CD, von Mehren M, Brennan MF, et al. American College of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant GIST Study Team: Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: a randomised, double-blind, placebo-controlled trial. Lancet. 2009;373:1097–104.

    CAS  PubMed  PubMed Central  Google Scholar 

  42. Joensuu H, Eriksson M, Sundby Hall K, Hartmann JT, Pink D, Schütte J, Ramadori G, Hohenberger P, Duyster J, Al-Batran SE, et al. One vs three years of adjuvant imatinib for operable gastrointestinal stromal tumor: a randomized trial. JAMA. 2012;307:1265–72.

    CAS  PubMed  Google Scholar 

  43. Lasota J, Miettinen M. Clinical significance of oncogenic KIT and PDGFRA mutations in gastrointestinal stromal tumours. Histopathology. 2008;53:245–66.

    CAS  PubMed  Google Scholar 

  44. Cassier PA, Fumagalli E, Rutkowski P, Schoffski P, Van Glabbeke M, Debiec-Rychter M, Emile JF, Duffaud F, Martin-Broto J, Landi B, et al. Outcome of patients with platelet-derived growth factor receptor alpha-mutated gastrointestinal stromal tumors in the tyrosine kinase inhibitor era. Clin Cancer Res. 2012;18:4458–64.

    CAS  PubMed  Google Scholar 

  45. Corless CL, Schroeder A, Griffith D, Town A, McGreevey L, Harrell P, Shiraga S, Bainbridge T, Morich J, Heinrich MC. PDGFRA mutations in gastrointestinal stromal tumors: frequency, spectrum and in vitro sensitivity to imatinib. J Clin Oncol. 2005;23:5357–64.

    CAS  PubMed  Google Scholar 

  46. Yu Z. Wenqing Hu, Ping Chen, Masanobu Abe, Lei Shi, Si-yuan Tan, Yong Li, Liang Zong: Ki67 is a biological marker of malignant risk of gastrointestinal stromal tumors: a systematic review and meta-analysis. Medicine (Baltimore). 2017;96:e7911.

    Google Scholar 

  47. Martin-Broto J, Moura DS. New drugs in gastrointestinal stromal tumors. Curr Opin Oncol. 2020;32:314–20.

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

Not applicable

Funding

Not applicable

Author information

Authors and Affiliations

  1. Gastric Surgery Division, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan

    Kohei Kanamori, Yukinori Yamagata, Keiichi Date, Takeyuki Wada, Tsutomu Hayashi, Sho Otsuki, Takaki Yoshikawa, Hitoshi Katai & Toshiro Nishida

  2. Head and Neck Medical Oncology Division and Gastrointestinal Medical Oncology Division, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan

    Yoshitaka Honma

  3. Division of Pathology and Clinical Laboratories, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan

    Shigeki Sekine

Authors
  1. Kohei Kanamori
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yukinori Yamagata
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Yoshitaka Honma
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Keiichi Date
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Takeyuki Wada
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Tsutomu Hayashi
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Sho Otsuki
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Shigeki Sekine
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Takaki Yoshikawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Hitoshi Katai
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Toshiro Nishida
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

KK, YY, KD, TW, TH, SO, TY, and HK were involved in the determination of treatment strategy and patient management. KK and YY decided manuscript conception, and TY and TN advised the process of determination of manuscript conception. KK and SS drafted the pathological part of the manuscript. YY and TH drafted the medical treatment part of the manuscript. YY drafted the manuscript and revised it critically for important intellectual content. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Yukinori Yamagata.

Ethics declarations

Ethics approval and consent to participate

This study was performed in accordance with the principles of the Declaration of Helsinki. The requirement for ethical approval was waived by the National Cancer Center Institutional Review Board.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and accompanying images.

Competing interests

The authors declare that they have no competing interests.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kanamori, K., Yamagata, Y., Honma, Y. et al. Extra-gastrointestinal stromal tumor arising in the lesser omentum with a platelet-derived growth factor receptor alpha (PDGFRA) mutation: a case report and literature review. World J Surg Onc 18, 183 (2020). https://doi.org/10.1186/s12957-020-01961-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/s12957-020-01961-1

Keywords

World Journal of Surgical Oncology

ISSN: 1477-7819

Contact us