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Tanaka Y, Toyokawa T, Yoshii M, Miki Y, Tamura T, Lee S, Maeda K. Giant Intra-Abdominal Desmoid Tumor in a Young Man: A Case Report and Literature Review. Surg Case Rep 2025; 11:24-0019. [PMID: 40124320 PMCID: PMC11926331 DOI: 10.70352/scrj.cr.24-0019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2024] [Accepted: 02/05/2025] [Indexed: 03/25/2025] Open
Abstract
INTRODUCTION Desmoid tumors are rare soft-tissue tumors with a high recurrence rate; however, histologically, these tumors are benign. We describe a case in which a giant desmoid tumor was resected in a young man without any apparent causative factors. CASE PRESENTATION A 21-year-old man was referred to our hospital for treatment after presenting to a nearby hospital with right inguinal pain. Abdominal magnetic resonance imaging showed an intra-abdominal mass measuring 34 × 15 × 8 cm with partial signal hyperintensity on T2-weighted imaging and hypointensity on T1-weighted imaging, extending from the left abdominal cavity to the pelvic region. Although no definitive diagnosis was obtained preoperatively, surgery was performed under suspicion of gastrointestinal stromal tumor or other significant disease. A mass was identified firmly adherent to the transverse colon, gastric wall, and diaphragm, and these organs were partially resected. The excised specimen measured 38 × 21 × 8 cm and weighed 6400 g. Macroscopically, the tumor showed a smooth surface and homogeneous interior. Pathological examination revealed atypical cells with spindle-shaped nuclei and collagen fiber hyperplasia in the stroma, and immunostaining was negative for c-kit, CD34, desmin, S-100, and positive for β-catenin, leading to a confirmed diagnosis of desmoid tumor. Fifteen months after surgery, a local recurrence with a diameter of 3.0 cm was identified, and the patient remains under careful follow-up. CONCLUSIONS Intra-abdominal desmoid tumors larger than 30 cm are extremely rare. When encountering a young patient with a large intra-abdominal tumor, the possibility of desmoid tumor should be considered.
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Affiliation(s)
- Yusuke Tanaka
- Department of Gastroenterological Surgery, Tsukazaki Hospital, Himeji, Hyogo, Japan
- Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan
| | - Takahiro Toyokawa
- Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan
| | - Mami Yoshii
- Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan
| | - Yuichiro Miki
- Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan
| | - Tatsuro Tamura
- Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan
| | - Shigeru Lee
- Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan
| | - Kiyoshi Maeda
- Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan
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Nishida T, Gotouda N, Takahashi T, Cao H. Clinical importance of tumor rupture in gastrointestinal stromal tumor. J Dig Dis 2024; 25:542-549. [PMID: 37210619 DOI: 10.1111/1751-2980.13190] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2023] [Revised: 05/16/2023] [Accepted: 05/18/2023] [Indexed: 05/22/2023]
Abstract
Risk factors of gastrointestinal stromal tumors (GISTs) include tumor size, location, mitosis, and tumor rupture. Although the first three are commonly recognized as independent prognostic factors, tumor rupture is not a consistent finding. Indeed, tumor rupture may be subjectively diagnosed and is rarely observed. Moreover, the criteria used for diagnosis differ among oncologists, which may result in inconsistent outcomes. Based on these conditions, a universal definition of tumor rupture was proposed in 2019 and consists of six scenarios: tumor fracture, blood-stained ascites, gastrointestinal perforation at the tumor site, histologically proven invasion, piecemeal resection, and open incisional biopsy. Although the definition is considered appropriate for selection of GISTs with worse prognostic outcomes, each scenario lacks a high level of evidence and there is yet no consensus for some, including histological invasion and incisional biopsy. It may be, however, important to have common criteria for clinical decision-making, which may facilitate reliability, external validity, and comparability of clinical studies in rare GISTs. After the definition, several retrospective reports indicated that even with adjuvant therapy, tumor rupture was associated with high recurrence rates and poor prognostic outcomes. The prognosis of patients with ruptured GISTs is improved by 5-year adjuvant therapy compared with 3-year therapy. Nevertheless, the universal definition requires further evidence, and prospective clinical studies based on the definition are warranted.
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Affiliation(s)
- Toshirou Nishida
- Department of Surgery, Japan Community Health-care Organization Osaka Hospital, Osaka, Japan
- Department of Surgery, National Cancer Center Hospital, Tokyo, Japan
- Laboratory of Nuclear Transport Dynamics, National Institute of Biomedical Innovation, Health and Nutrition, Ibaraki, Osaka, Japan
| | - Naoto Gotouda
- Department of Hepatobiliary and Pancreatic Surgery, National Cancer Center Hospital East, Kashiwa, Japan
| | - Tsuyoshi Takahashi
- Department of Gastroenterological Surgery, Osaka University, Suita, Osaka, Japan
| | - Hui Cao
- Department of Gastrointestinal Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
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Patel K, Hutapea P. Study of Tissue Damage Induced by Insertion of Composite-Coated Needle. Med Eng Phys 2024; 123:104094. [PMID: 38365334 DOI: 10.1016/j.medengphy.2023.104094] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2023] [Revised: 11/15/2023] [Accepted: 12/21/2023] [Indexed: 02/18/2024]
Abstract
Medical interventions have significantly progressed in developing minimally invasive techniques like percutaneous procedures. These procedures include biopsy and internal radiation therapy, where a needle or needle-like medical device is inserted through the skin to access a target inside the body. Ensuring accurate needle insertion and minimizing tissue-damage or cracks are critical in these procedures. This research aims to examine the coated needle effect on the force required to insert the needle (i.e., insertion force) and on tissue-damage during needle insertion into the bovine kidney. Reducing the needle insertion force, which is influenced by needle surface friction, generally results in a reduction in tissue-damage. Surgical needles were coated with a composite material, combining Polytetrafluoroethylene, Polydopamine, and Activated Carbon. Force measurement during needle insertion and a histological study to determine tissue-damage were conducted to evaluate the effectiveness of the coating. The insertion force was reduced by 49 % in the case of the coated needles. Furthermore, a histological analysis comparing tissue-damage resulting from coated and uncoated needles revealed an average 39 % reduction in tissue-damage with the use of coated needles. The results of this study demonstrate the potential of coated needles to enhance needle insertion and safety during percutaneous procedures.
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Affiliation(s)
- Kavi Patel
- Department of Mechanical Engineering, Temple University, Philadelphia, PA 19122, United States of America
| | - Parsaoran Hutapea
- Department of Mechanical Engineering, Temple University, Philadelphia, PA 19122, United States of America.
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Smadja J, El Zein S, Pierron G, Watson S, Laas E, Ramtohul T, Tzanis D, Servois V, Bonvalot S. Percutaneous Uterine Needle Biopsy with Microscopic and Array-CGH Analyses for Preoperative Sarcoma Diagnosis in Patients with Suspicious Myometrial Tumors on MRI: A Prospective Pilot Study (SARCGYN). Ann Surg Oncol 2023; 30:943-953. [PMID: 36287348 DOI: 10.1245/s10434-022-12697-5] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2022] [Accepted: 10/04/2022] [Indexed: 01/10/2023]
Abstract
BACKGROUND Unlike for soft tissue sarcomas, percutaneous biopsy is not validated for uterine myometrial tumors, leading to leiomyosarcoma inadvertent morcellation and overtreatment in childbearing patients. This study aimed to evaluate preoperative percutaneous uterine needle biopsy (PUB) with microscopic examination (M-PUB) and array-comparative genomic hybridization (MCGH-PUB). METHODS This was a prospective single-center cohort study including all consecutive patients who were candidates for hysterectomy because of suspected uterine leiomyosarcoma on magnetic resonance imaging (MRI) who received PUB. Microscopic and array-CGH analyses with genomic index (GI) counts were performed to guide the therapeutic strategy. Smooth-muscle tumors with suspect features with a GI above 15 were deemed malignant, as were tumors without microscopic malignant features with a complex genomic profile (GI above 30 or malignant profile). Preoperative diagnoses based on M-PUB and MCGH-PUB were compared with the postsurgical pathological specimen or follow-up. RESULTS From November 2016 to February 2022, 34 patients were included. Based on the surgical specimen (N = 23) or follow-up (N = 11), final diagnoses were 11 sarcomas and 23 non-sarcomas. The median follow-up was 12 months (IQR 6-37). The diagnostic accuracies of M-PUB and MCGH-PUB were 94% and 100%, respectively. The sensitivity, specificity, and negative predictive value of MCGH-PUB were 100%, 100%, and 100%, respectively. A high GI was significantly associated with malignancy (P < 0.001). Genomic analyses allowed malignancy upgrades for four tumors. There were no complications and no dissemination along the biopsy track. CONCLUSION MCGH-PUB is safe and accurate for preoperatively diagnosing uterine sarcomas and should be used routinely after suspicious MRI to tailor surgery.
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Affiliation(s)
- Jeremy Smadja
- Department of Radiology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Sophie El Zein
- Department of Pathology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Gaelle Pierron
- Laboratory of Somatic Genetics, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Sarah Watson
- Department of Medical Oncology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Enora Laas
- Department of Surgical Oncology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Toulsie Ramtohul
- Department of Radiology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Dimitri Tzanis
- Department of Surgical Oncology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Vincent Servois
- Department of Radiology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | - Sylvie Bonvalot
- Department of Surgical Oncology, Institut Curie, Université Paris Sciences et Lettres, Paris, France.
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Colapkulu-Akgul N, Gunel H, Beyazadam D, Ozsoy MS, Alimoglu O. Gastrointestinal Stromal Tumors: Recurrence and Survival Analysis of 49 Patients. Middle East J Dig Dis 2023; 15:19-25. [PMID: 37547161 PMCID: PMC10404080 DOI: 10.34172/mejdd.2023.315] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/27/2022] [Accepted: 09/20/2022] [Indexed: 08/08/2023] Open
Abstract
Background: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor originating from the gastrointestinal tract and have a broad spectrum of clinicopathological features affecting disease management regarding the treatment modalities. Methods: A retrospective study of 49 patients who underwent surgery for gastrointestinal tumors between 2008 and 2016 was conducted. Clinical, pathological, and immunohistochemical features of patients with and without recurrence were statistically analyzed. Results: Twenty-nine (59.1%) patients had gastric; 16 (32.6%) had small intestinal; 3 (6.1%) had mesenteric; and 1 (2.2%) had rectal GISTs. Microscopic tumor necrosis and tumor ulceration were also significant for disease recurrence (P = 0.005, P = 0.049). High-risk patients according to Miettinen's risk classification were more likely to develop a recurrence (P < 0.001). Additionally, high-grade tumors were also a risk factor for recurrence (P < 0.001). Ki-67 levels were available in 40 patients and the mean Ki-67 level was 16.8 in patients with recurrence, which was a significant risk factor in regression analysis (HR: 1.24, 95%, CI: 1.08-1-43). Five-year disease-free survival rates of non-gastric and gastric GISTs were 62.3% and 90%, respectively (P = 0.044). Conclusion: Larger tumors and higher mitotic rates are more likely to develop recurrence. High Ki-67 levels were also associated with recurrence.
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Affiliation(s)
| | - Humeyra Gunel
- Department of Pathology, Istanbul Medeniyet University, Goztepe Prof. Dr. Suleyman Yalcin City Hospital, Istanbul, Turkey
| | - Damla Beyazadam
- Department of General Surgery, Istanbul Medeniyet University, Goztepe Prof. Dr. Suleyman Yalcin City Hospital, Istanbul, Turkey
| | - Mehmet S Ozsoy
- Department of General Surgery, Istanbul Medeniyet University, Goztepe Prof. Dr. Suleyman Yalcin City Hospital, Istanbul, Turkey
| | - Orhan Alimoglu
- Department of General Surgery, Istanbul Medeniyet University, Goztepe Prof. Dr. Suleyman Yalcin City Hospital, Istanbul, Turkey
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Jakob J, Salameh R, Wichmann D, Charalambous N, Zygmunt AC, Kreisel I, Heinz J, Ghadimi M, Ronellenfitsch U. Needle tract seeding and abdominal recurrence following pre-treatment biopsy of gastrointestinal stromal tumors (GIST): results of a systematic review. BMC Surg 2022; 22:202. [PMID: 35597932 PMCID: PMC9124402 DOI: 10.1186/s12893-022-01648-2] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2022] [Accepted: 05/12/2022] [Indexed: 11/25/2022] Open
Abstract
BACKGROUND Gastrointestinal stromal tumors (GIST) are rare abdominal tumors. Pretreatment biopsies may be used to diagnose a GIST and enable tailored treatment. Some experts are skeptical about biopsies because they fear tumor cell seeding. The objective of this study was to determine if pretreatment biopsy is associated with increased tumor recurrence. METHODS We performed a systematic literature search and included studies assessing the oncological outcome of GIST patients who underwent a pre-treatment core needle biopsy or fine needle aspiration. We assessed methodological quality with the Newcastle-Ottawa-Scale for non-randomized studies. This review was registered in the PROSPERO database (CRD42021170290). RESULTS Three non-randomized studies and eight case reports comprising 350 patients were eligible for inclusion. No prospective study designed to answer the review question was found. One case of needle tract seeding after percutaneous core needle biopsy of GIST was reported. None of the studies reported an increased rate of abdominal recurrence in patients with pretreatment biopsy. CONCLUSIONS The existing evidence does not indicate a relevant risk of needle tract seeding or abdominal recurrence after pre-treatment biopsy of GIST. Biopsy can safely be done to differentiate GIST from other tumors and to select the most appropriate treatment.
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Affiliation(s)
- Jens Jakob
- Department of Surgery, Sarcoma Unit, University Medical Center Mannheim, Th.-Kutzer-Ufer 1-3, 68163, Mannheim, Germany.
| | - Rashad Salameh
- Department of Visceral, Thoracic, Vascular and Transplant Surgery, University Hospital Dresden, Dresden, Germany
| | - David Wichmann
- Department of General, Visceral and Pediatric Surgery, University Medical Center, Goettingen, Germany
| | - Nicos Charalambous
- Department of Visceral, Thoracic, Vascular and Transplant Surgery, University Hospital Dresden, Dresden, Germany
| | - Anne-Christine Zygmunt
- Department of General, Visceral and Pediatric Surgery, University Medical Center, Goettingen, Germany
| | - Inga Kreisel
- Department of General, Visceral and Pediatric Surgery, University Medical Center, Goettingen, Germany
| | - Judith Heinz
- Department of Medical Statistics, University Medical Center Goettingen, Goettingen, Germany
| | - Michael Ghadimi
- Department of General, Visceral and Pediatric Surgery, University Medical Center, Goettingen, Germany
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Sugiyama Y, Sasaki M, Kouyama M, Tazaki T, Takahashi S, Nakamitsu A. Current treatment strategies and future perspectives for gastrointestinal stromal tumors. World J Gastrointest Pathophysiol 2022; 13:15-33. [PMID: 35116177 PMCID: PMC8788163 DOI: 10.4291/wjgp.v13.i1.15] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/08/2021] [Revised: 05/23/2021] [Accepted: 11/15/2021] [Indexed: 02/06/2023] Open
Abstract
Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that originate from the gastrointestinal tract, mostly from the stomach. GISTs are derived from the myenteric interstitial cells of Cajal and are caused by several mutations in the c-kit and platelet-derived growth factor receptor genes. Clinically, GISTs are detected by endoscopic and imaging findings and are diagnosed by immunostaining. Surgery is the first line of treatment, and if the tumor is relatively small, minimally invasive surgery such as laparoscopy is performed. In recent years, neoadjuvant therapy has been administered to patients with GISTs that are suspected of having a large size or infiltration to other organs. Postoperative adjuvant imatinib is the standard therapy for high-risk GISTs. It is important to assess the risk of recurrence after GIST resection. However, the effect of tyrosine kinase inhibitor use will vary by the mutation of c-kit genes and the site of mutation. Furthermore, information regarding gene mutation is indispensable when considering the treatment policy for recurrent GISTs. This article reviews the clinicopathological characteristics of GISTs along with the minimally invasive and multidisciplinary treatment options available for these tumors. The future perspectives for diagnostic and treatment approaches for these tumors have also been discussed.
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Affiliation(s)
- Yoichi Sugiyama
- Department of Gastrointestinal Surgery, JA Hiroshima General Hospital, Hatsukaichi 738-8503, Hiroshima, Japan
| | - Masaru Sasaki
- Department of Gastrointestinal Surgery, JA Hiroshima General Hospital, Hatsukaichi 738-8503, Hiroshima, Japan
| | - Mohei Kouyama
- Department of Gastrointestinal Surgery, JA Hiroshima General Hospital, Hatsukaichi 738-8503, Hiroshima, Japan
| | - Tatsuya Tazaki
- Department of Gastrointestinal Surgery, JA Hiroshima General Hospital, Hatsukaichi 738-8503, Hiroshima, Japan
| | - Shinya Takahashi
- Department of Surgery, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima 734-8551, Japan
| | - Atsushi Nakamitsu
- Department of Gastrointestinal Surgery, JA Hiroshima General Hospital, Hatsukaichi 738-8503, Hiroshima, Japan
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van Houdt WJ, IJzerman NS, Schrijver AM, Huis In 't Veld E, Thway K, Jones RL, Fotiadis N, Hayes AJ, Bruining A, Zavrakidis I, van Coevorden F, Steeghs N, Mathijssen RHJ, Strauss DC, Smith MJF. Oncological Outcome After Diagnostic Biopsies in Gastrointestinal Stromal Tumors: A Retrospective Cohort Study. Ann Surg 2021; 274:e1093-e1098. [PMID: 31850986 DOI: 10.1097/sla.0000000000003744] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
OBJECTIVE To analyze whether the route of preoperative biopsy influences oncological outcome in GIST patients. SUMMARY OF BACKGROUND DATA Preoperative biopsies are widely used for diagnosing GIST. Little is known about the risk of tumor seeding after different routes of biopsy. METHODS Patients who underwent resection of a primary GIST between 1996 and 2014 were identified from 2 databases from 2 tertiary referral centers. Survival data were obtained using the Kaplan-Meier method. Possible confounders were identified using Cox regression analysis. The primary endpoint was local recurrence free survival (RFS) and the secondary endpoint was DSS. RESULTS A total of 228 patients were included, with a median age of 62 years (range 17-86) and a median follow-up time of 53 months (range 1-204). From these patients, 42 patients did not have a biopsy (18%), 70 underwent a transcutaneous biopsy (31%), and 116 a transluminal biopsy (51%). A total of 42 patients (19.0%) had a local and/or distant recurrence. From the 70 patients with a transcutaneous biopsy, only 1 patient developed a needle tract recurrence (1.4%). Local RFS and DSS were both significantly shorter in the transcutaneous biopsy group on univariate analysis compared to the other groups; however, in multivariate analysis the route of biopsy did not influence local RFS (P = 0.128) or DSS (P = 0.096). CONCLUSIONS Transluminal or transcutaneous biopsies for diagnosing GIST do not significantly alter the risk of local recurrent disease or DSS in multivariate Cox regressions. The risk of needle tract seeding after transcutaneous biopsy was low.
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Affiliation(s)
- Winan J van Houdt
- Sarcoma Unit, Department of Surgical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands
| | - Nikki S IJzerman
- Sarcoma Unit, Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands
- Department of Medical Oncology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, the Netherlands
| | - Anne Marjolein Schrijver
- Sarcoma Unit, Department of Surgical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands
| | - Eva Huis In 't Veld
- Sarcoma Unit, Department of Surgical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands
| | - Khin Thway
- Sarcoma Unit, Department of Pathology, Royal Marsden Hospital, London, UK
| | - Robin L Jones
- Sarcoma Unit, Department of Medical Oncology, Royal Marsden Hospital and Institute of Cancer Research, London, UK
| | - Nicos Fotiadis
- Sarcoma Unit, Department of Radiology, Royal Marsden Hospital, London, UK
| | - Andrew J Hayes
- Sarcoma Unit, Department of Surgical Oncology, Royal Marsden Hospital and Institute of Cancer Research, London, UK
| | - Annemarie Bruining
- Sarcoma Unit, Department of Radiology, Netherlands Cancer Institute, Amsterdam, the Netherlands
| | - Ioannis Zavrakidis
- Netherlands Cancer Institute - Antoni van Leeuwenhoek, Department of Epidemiology and Biostatistics, Amsterdam, the Netherlands
| | - Frits van Coevorden
- Sarcoma Unit, Department of Surgical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands
| | - Neeltje Steeghs
- Sarcoma Unit, Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands
| | - Ron H J Mathijssen
- Department of Medical Oncology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, the Netherlands
| | - Dirk C Strauss
- Sarcoma Unit, Department of Surgical Oncology, Royal Marsden Hospital and Institute of Cancer Research, London, UK
| | - Myles J F Smith
- Sarcoma Unit, Department of Surgical Oncology, Royal Marsden Hospital and Institute of Cancer Research, London, UK
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Schmidt T, Ghadimi M, Fuchs HF, Bruns CJ. [Surgical and interdisciplinary treatment of gastrointestinal stromal tumors]. Chirurg 2021; 93:27-33. [PMID: 34709443 DOI: 10.1007/s00104-021-01527-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/28/2021] [Indexed: 11/30/2022]
Abstract
Gastrointestinal stromal tumors (GISTs) are the most frequent potentially malignant mesenchymal tumors of the gastrointestinal tract. The treatment of GISTs has been revolutionized since imatinib and other tyrosine kinase inhibitors were introduced for the treatment of GISTs, which inhibit the tyrosine kinases c‑KIT and platelet-derived growth factor receptor (PDGFR) alpha. Even after the introduction of this targeted treatment GISTs can only be cured by surgical resection. With interdisciplinary multimodal treatment the prognosis of metastasized GIST can now be further improved by surgical resection of the primary tumor and the metastases, potentially leading to a cure. Neoadjuvant therapy can reduce the extent of surgical resection and hereby enable organ preservation and reduce surgical morbidity. To evaluate molecular and clinical predictors and to offer an optimal therapeutic plan, patients with GISTs and certainly patients with advanced GISTs should be evaluated by interdisciplinary sarcoma boards.
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Affiliation(s)
- Thomas Schmidt
- Klinik für Allgemein‑, Viszeral‑, Tumor und Transplantationschirurgie, Universitätsklinikum Köln, Kerpener Str. 62, 50937, Köln, Deutschland.
| | - Markus Ghadimi
- Klinik für Allgemein‑, Viszeral‑, Tumor und Transplantationschirurgie, Universitätsklinikum Köln, Kerpener Str. 62, 50937, Köln, Deutschland
| | - Hans F Fuchs
- Klinik für Allgemein‑, Viszeral‑, Tumor und Transplantationschirurgie, Universitätsklinikum Köln, Kerpener Str. 62, 50937, Köln, Deutschland
| | - Christiane J Bruns
- Klinik für Allgemein‑, Viszeral‑, Tumor und Transplantationschirurgie, Universitätsklinikum Köln, Kerpener Str. 62, 50937, Köln, Deutschland
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Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors. Cancers (Basel) 2021; 13:cancers13133158. [PMID: 34202544 PMCID: PMC8268322 DOI: 10.3390/cancers13133158] [Citation(s) in RCA: 35] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2021] [Revised: 06/18/2021] [Accepted: 06/22/2021] [Indexed: 12/31/2022] Open
Abstract
Simple Summary Gastrointestinal stromal tumors (GIST) are potentially malignant tumors and require evidence-based surgical and/or medical treatment. Laparoscopy has similar safety and prognostic outcomes to those of laparotomy and is currently a standard procedure for localized GISTs. However, surgery for gastric GISTs less than 2 cm may be re-evaluated due to the indolent nature of the GIST and other competing risks among GIST patients. A work-up with endoscopy and endoscopic ultrasonography as well as endoscopic or percutaneous biopsy is important for the preoperative diagnosis of GISTs. Medical treatment with tyrosine kinase inhibitors is the mainstay for recurrent/metastatic GISTs. The activity of an individual drug is well correlated with gene alterations, and, in the era of precision medicine, cancer genome profiling should be considered before medical treatment. Abstract Gastrointestinal stromal tumors (GISTs) are the most frequent malignant mesenchymal tumors in the gastrointestinal tract. The clinical incidence of GISTs is estimated 10/million/year; however, the true incidence is complicated by frequent findings of tiny GISTs, of which the natural history is unknown. The initial work-up with endoscopy and endoscopic ultrasonography plays important roles in the differential diagnosis of GISTs. Surgery is the only modality for the permanent cure of localized GISTs. In terms of safety and prognostic outcomes, laparoscopy is similar to laparotomy for GIST treatment, including tumors larger than 5 cm. GIST progression is driven by mutations in KIT or PDGFRA or by other rare gene alterations, all of which are mutually exclusive. Tyrosine kinase inhibitors (TKIs) are the standard therapy for metastatic/recurrent GISTs. Molecular alterations are the most reliable biomarkers for TKIs and for other drugs, such as NTRK inhibitors. The pathological and genetic diagnosis prior to treatment has been challenging; however, a newly developed endoscopic device may be useful for diagnosis. In the era of precision medicine, cancer genome profiling by targeted gene panel analysis may enable potential targeted therapy even for GISTs without KIT or PDGFRA mutations.
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Abstract
Gastrointestinal stromal tumours (GIST) have an incidence of ~1.2 per 105 individuals per year in most countries. Around 80% of GIST have varying molecular changes, predominantly mutually exclusive activating KIT or PDGFRA mutations, but other, rare subtypes also exist. Localized GIST are curable, and surgery is their standard treatment. Risk factors for relapse are tumour size, mitotic index, non-gastric site and tumour rupture. Patients with GIST with KIT or PDGFRA mutations sensitive to the tyrosine kinase inhibitor (TKI) imatinib that are at high risk of relapse have improved survival with adjuvant imatinib treatment. In advanced disease, median overall survival has improved from 18 months to >70 months since the introduction of TKIs. The role of surgery in the advanced setting remains unclear. Resistance to TKIs arise mainly from subclonal selection of cells with resistance mutations in KIT or PDGFRA when they are the primary drivers. Advanced resistant GIST respond to second-line sunitinib and third-line regorafenib, as well as to the new broad-spectrum TKI ripretinib. Rare molecular forms of GIST with alterations involving NF1, SDH genes, BRAF or NTRK genes generally show primary resistance to standard TKIs, but some respond to specific inhibitors of the activated genes. Despite major advances, many questions in both advanced and localized disease remain unanswered.
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Affiliation(s)
- Jean-Yves Blay
- Department of Medicine, Centre Leon Berard, UNICANCER & University Lyon I, Lyon, France.
| | - Yoon-Koo Kang
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Toshiroo Nishida
- Surgery Department, National Cancer Center Hospital, Chuo-ku, Tokyo, Japan
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Hedenström P, Andersson C, Sjövall H, Enlund F, Nilsson O, Nilsson B, Sadik R. Pretreatment Tumor DNA Sequencing of KIT and PDGFRA in Endosonography-Guided Biopsies Optimizes the Preoperative Management of Gastrointestinal Stromal Tumors. Mol Diagn Ther 2021; 24:201-214. [PMID: 32124386 PMCID: PMC7113213 DOI: 10.1007/s40291-020-00451-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
BACKGROUND Neoadjuvant tyrosine kinase inhibitor (TKI) therapy increases the chance of organ-preserving, radical resection in selected patients with gastrointestinal stromal tumors (GISTs). We aimed to evaluate systematic, immediate DNA sequencing of KIT and PDGFRA in pretreatment GIST tissue to guide neoadjuvant TKI therapy and optimize preoperative tumor response. METHODS All patients who were candidates for neoadjuvant therapy of a suspected GIST [the study cohort (SC)] were prospectively included from January 2014 to March 2018. Patients were subjected to pretreatment endosonography-guided fine-needle biopsy (EUS-FNB) or transabdominal ultrasound-guided needle biopsy (TUS-NB), followed by immediate tumor DNA sequencing (< 2 weeks). A historic (2006-2013) reference cohort (RC) underwent work-up without sequencing before neoadjuvant imatinib (n = 42). The rate of optimal neoadjuvant therapy (TherapyOPTIMAL) was calculated, and the induced tumor size reduction (Tumor RegressionMAX, %) was evaluated by computed tomography (CT) scan. RESULTS The success rate of pretreatment tumor DNA sequencing in the SC (n = 81) was 77/81 (95%) [EUS-FNB 71/74 (96%); TUS-NB 6/7 (86%)], with mutations localized in KIT (n = 58), PDGFRA (n = 18), or neither gene, wild type (n = 5). In patients with a final indication for neoadjuvant therapy, the TherapyOPTIMAL was higher in the SC compared with the RC [61/63 (97%) versus 33/42 (79%), p = 0.006], leading to a significantly higher Tumor RegressionMAX in patients treated with TKI (27% vs. 19%, p = 0.015). CONCLUSIONS Pretreatment endosonography-guided biopsy sampling followed by immediate tumor DNA sequencing of KIT and PDGFRA is highly accurate and valuable in guiding neoadjuvant TKI therapy in GIST. This approach minimizes maltreatment with inappropriate regimens and leads to improved tumor size reduction before surgery.
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Affiliation(s)
- Per Hedenström
- Division of Medical Gastroenterology, Department of Internal Medicine, Sahlgrenska University Hospital, Blå Stråket 3, 413 35, Gothenburg, Sweden. .,Department of Internal Medicine and Clinical Nutrition, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
| | - Carola Andersson
- Department of Clinical Pathology and Genetics, Sahlgrenska University Hospital, Gothenburg, Sweden
| | - Henrik Sjövall
- Division of Medical Gastroenterology, Department of Internal Medicine, Sahlgrenska University Hospital, Blå Stråket 3, 413 35, Gothenburg, Sweden.,Department of Internal Medicine and Clinical Nutrition, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
| | - Fredrik Enlund
- Department of Clinical Pathology and Genetics, Sahlgrenska University Hospital, Gothenburg, Sweden
| | - Ola Nilsson
- Department of Clinical Pathology and Genetics, Sahlgrenska University Hospital, Gothenburg, Sweden
| | - Bengt Nilsson
- Department of Surgery, Sahlgrenska University Hospital, Gothenburg, Sweden
| | - Riadh Sadik
- Division of Medical Gastroenterology, Department of Internal Medicine, Sahlgrenska University Hospital, Blå Stråket 3, 413 35, Gothenburg, Sweden
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13
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Xu SJ, Lin GS, Ling HJ, Guo RJ, Chen J, Liao YM, Lin T, Zhou YJ. Nomogram to Predict Preoperative Occult Peritoneal Metastasis of Gastrointestinal Stromal Tumors (GIST) Based on Imaging and Inflammatory Indexes. Cancer Manag Res 2020; 12:11713-11721. [PMID: 33239911 PMCID: PMC7681585 DOI: 10.2147/cmar.s275422] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2020] [Accepted: 10/06/2020] [Indexed: 12/27/2022] Open
Abstract
Background Preoperative imaging examination is the primary method for diagnosing metastatic gastrointestinal stromal tumor (GIST), but it is associated with a high rate of missed diagnosis. Therefore, it is important to establish an accurate model for predicting occult peritoneal metastasis (PM) of GIST. Patients and Methods GIST patients seen between April 2002 and December 2018 were selected from an institutional database. Using multivariate logistic regression analyses, we created a nomogram to predict occult PM of GIST and validated it with an independent cohort from the same center. The concordance index (C-index), decision curve analysis (DCA) and a clinical impact curve (CIC) were used to evaluate its predictive ability. Results A total of 522 eligible GIST patients were enrolled in this study and divided into training (n=350) and validation cohorts (n=172). Factors associated with occult PM were included in the model: tumor size (odds ratio [OR] 1.194 95% confidence interval [CI], 1.034-1.378; p=0.016), primary location (OR 7.365 95% CI, 2.192-24.746; p=0.001), tumor capsule (OR 4.282 95% CI, 1.209-15.166; p=0.024), Alb (OR 0.813 95% CI, 0.693-0.954; p=0.011) and FIB (OR 2.322 95% CI, 1.410-3.823; p=0.001). The C-index was 0.951 (95% CI, 0.917-0.985) in the training cohort and 0.946 (95% CI, 0.900-0.992) in the validation cohort. In the training cohort, the prediction model had a sensitivity of 82.8%, a specificity of 93.8%, a positive predictive value of 54.7%, and a negative predictive value of 98.4%; the validation cohort values were 94.7%, 85.0%, 43.9% and 99.2%, respectively. DCA and CIC results showed that the nomogram had clinical value in predicting occult PM in GIST patients. Conclusion Imaging and inflammatory indexes are significantly associated with microscopic metastases of GIST. A nomogram including these factors would have an excellent ability to predict occult PM.
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Affiliation(s)
- Shao-Jun Xu
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
| | - Guo-Sheng Lin
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
| | - Hong-Jian Ling
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
| | - Ren-Jie Guo
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
| | - Jie Chen
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
| | - Yi-Ming Liao
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
| | - Tao Lin
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
| | - Yong-Jian Zhou
- Department of Gastric Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Department of General Surgery, Fujian Medical University Union Hospital, Fuzhou, People's Republic of China.,Key Laboratory of Ministry of Education of Gastrointestinal Cancer, Fujian Medical University, Fuzhou, Fujian Province, People's Republic of China
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14
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Risk Factors of Gastrointestinal Stromal Tumor Recurrence. ANADOLU KLINIĞI TIP BILIMLERI DERGISI 2020. [DOI: 10.21673/anadoluklin.755659] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
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15
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The challenge of treating elderly patients with advanced bone and soft tissue sarcomas. Crit Rev Oncol Hematol 2020; 155:103108. [DOI: 10.1016/j.critrevonc.2020.103108] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2020] [Revised: 07/24/2020] [Accepted: 09/07/2020] [Indexed: 01/13/2023] Open
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16
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Askari A, Brittain R, Hilmi M, Hajuthman W, Al-Bahrani A. Unusual presentations, management and outcomes of gastric stromal tumors: a single-center case series. Ann Gastroenterol 2020; 34:26-32. [PMID: 33414618 PMCID: PMC7774666 DOI: 10.20524/aog.2020.0540] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/06/2020] [Accepted: 07/09/2020] [Indexed: 01/11/2023] Open
Abstract
Background Gastrointestinal stromal tumors (GISTs) are uncommon mesenchymal tumors of the gastrointestinal tract. This study explores the safety of laparoscopy and the long-term oncological outcome in gastroesophageal GIST treatment. Methods A prospectively maintained single-institution database was examined. The variables collected were patient demographics and comorbidities, surgical access (laparoscopic/open), type of surgery, length of stay, and complications. Results A total of 69 patients underwent GIST resection between January 2011 and June 2018, of whom 56.5% were male; the median age was 68 years (interquartile range 60-76). The majority of patients (78.3%, n=54/69) had a laparoscopic resection. Median length of stay was 6 days in the laparoscopic group and 9 days in the open group (P=0.003). Most patients had wedge excision (n=57/69, 82.6%), while 12 patients (17.4%) required a gastrectomy (one a Merendino type). All patients had an R0 resection and 1 patient (1.4%) had a recurrence, despite having a low-risk grade original tumor with negative margins. Patients in the open group had a significantly higher proportion of patients with a high-risk tumor (50%) compared to the laparoscopic group (3.7%, P=0.001). The mean survival was 92.7 months (95% confidence interval 86.3-99.2). Survival was better in the laparoscopic group (100.4 months) compared with the open group (55.1 months, P<0.001). Conclusion Laparoscopic gastric GIST resection is an oncologically safe alternative to open surgery and is associated with a shorter hospital stay with no difference in complication rates or recurrence rates.
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Affiliation(s)
- Alan Askari
- Department of General Surgery, West Hertfordshire Hospitals NHS Trust, Vicarage Road, Watford (Alan Askari, Rory Brittain, Wasim Hajuthman, Ahmed Al-Bahrani)
| | - Rory Brittain
- Department of General Surgery, West Hertfordshire Hospitals NHS Trust, Vicarage Road, Watford (Alan Askari, Rory Brittain, Wasim Hajuthman, Ahmed Al-Bahrani)
| | - Marwa Hilmi
- General Practitioner, Lodge Surgery, St Albans (Marwa Hilmi), United Kingdom
| | - Wasim Hajuthman
- Department of General Surgery, West Hertfordshire Hospitals NHS Trust, Vicarage Road, Watford (Alan Askari, Rory Brittain, Wasim Hajuthman, Ahmed Al-Bahrani)
| | - Ahmed Al-Bahrani
- Department of General Surgery, West Hertfordshire Hospitals NHS Trust, Vicarage Road, Watford (Alan Askari, Rory Brittain, Wasim Hajuthman, Ahmed Al-Bahrani)
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17
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Tao K, Zeng X, Liu W, Wang S, Gao J, Shuai X, Zhang P. Primary Gastrointestinal Stromal Tumor Mimicking as Gynecologic Mass: Characteristics, Management, and Prognosis. J Surg Res 2020; 246:584-590. [PMID: 31653414 DOI: 10.1016/j.jss.2019.09.043] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2019] [Revised: 08/12/2019] [Accepted: 09/19/2019] [Indexed: 01/04/2023]
Abstract
BACKGROUND Primary gastrointestinal stromal tumor (GIST)-mimicking gynecologic masses are easily misdiagnosed. This study aimed to investigate the clinicopathological features, management, and prognosis of primary GIST mimicking as gynecologic mass. METHODS Clinicopathological and survival data of GIST mimicking as gynecologic mass admitted to our center from January 2005 to December 2017 were retrospectively analyzed. RESULTS Thirty-eight patients were included. The most common primary tumor site was the jejunoileum (n = 33, 86.9%), and 33 patients (86.9%) were classified as high recurrence risk. The short-term outcomes of operative incision length (P = 0.004), time to gas passage (P = 0.002), and hospital stay (P = 0.012) with laparoscopy-assisted resection were significantly shorter than those with open resection, showing no significant differences of long-term outcomes. With a median follow-up of 56 mo for 31 patients (81.6%), 18 (58.1%) received adjuvant therapy with imatinib. The 5-year disease-free survival and disease-specific survival of pelvic high-risk GIST was 37.0% and 48.3%, respectively; both were significantly lower than those of the other female high-risk group (both P < 0.05). CONCLUSIONS GIST mimicking as gynecologic mass is not uncommon, most originate from the jejunoileum and have a high risk of recurrence. Laparoscopy-assisted resection may be preferable for more favorable short-term outcomes. Compared with the other female high-risk GIST, pelvic high-risk GIST has a significantly worse prognosis; a longer duration of adjuvant therapy with imatinib than recommended may be beneficial.
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Affiliation(s)
- Kaixiong Tao
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Xiangyu Zeng
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Weizhen Liu
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Shaohai Wang
- Department of Obstetrics and Gynecology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Jinbo Gao
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Xiaoming Shuai
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Peng Zhang
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
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18
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Landi B, Blay JY, Bonvalot S, Brasseur M, Coindre JM, Emile JF, Hautefeuille V, Honore C, Lartigau E, Mantion G, Pracht M, Le Cesne A, Ducreux M, Bouche O. Gastrointestinal stromal tumours (GISTs): French Intergroup Clinical Practice Guidelines for diagnosis, treatments and follow-up (SNFGE, FFCD, GERCOR, UNICANCER, SFCD, SFED, SFRO). Dig Liver Dis 2019; 51:1223-1231. [PMID: 31387778 DOI: 10.1016/j.dld.2019.07.006] [Citation(s) in RCA: 54] [Impact Index Per Article: 9.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/04/2019] [Revised: 07/08/2019] [Accepted: 07/10/2019] [Indexed: 12/11/2022]
Abstract
BACKGROUND This document is a summary of the French Intergroup guidelines regarding the management of gastrointestinal stromal tumours (GISTs) updated in December 2018. DESIGN This collaborative work summarizes clinical practice recommendations (guidelines) on the management of GISTs. It is based on recent literature review, ESMO recommendations and expert opinions. RESULTS The diagnosis of GIST is based on histological examination and immunohistochemistry with markers KIT and DOG-1. Each case must be discussed within a multidisciplinary team. Complete surgical resection tumour, avoiding peroperative perforation, is the potentially curative treatment of localized GISTs. The estimation of the recurrence risk is essential, or adjuvant treatment,and follow-up adaptation. Genotyping (KIT and PDGFRA) of all but very low-risk GISTs is recommended. The nature of mutation has a prognostic value and predictive influence on drug efficacy. Imatinib, a tyrosine-kinase inhibitor, is the standard adjuvant treatment after R0 resection of a GIST with a high risk of recurrence, and the first line therapy for advanced GISTs. Suninitib and regorafenib are respectively the second- and third-line standard treatments for advanced GISTs. CONCLUSION Guidelines for management of GISTs are continuously evolving and need to be regularly updated. This constant progress is made possible through clinical and translational research.
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Affiliation(s)
- Bruno Landi
- Departments of Hepatogastroenterology and Digestive Oncology, European Georges Pompidou Hospital, Paris, France.
| | - Jean-Yves Blay
- Departments of Medical Oncology, Léon Bérard Center, Lyon, France
| | | | - Mathilde Brasseur
- Departments of Hepatogastroenterology and Digestive Oncology, CHU Robert Debré Hospital, Reims, France
| | | | - Jean François Emile
- Departments of Pathology, Hôpital Ambroise-Paré, Boulogne-Billancourt, France
| | - Vincent Hautefeuille
- Departments of Hepatogastroenterology and Digestive Oncology, CHU Amiens Picardie, Amiens, France
| | - Charles Honore
- Departments of Surgery, Gustave Roussy Cancer Campus, Villejuif, France
| | - Eric Lartigau
- Departments of Radiotherapy, Oscar Lambret Center, Lille, France
| | | | - Marc Pracht
- Departments of Medical Oncology, Eugène Marquis Center, Rennes, France
| | - Axel Le Cesne
- Departments of Medical Oncology, Gustave Roussy Cancer Campus, Villejuif, France
| | - Michel Ducreux
- Departments of Medical Oncology, Gustave Roussy Cancer Campus, Villejuif, France
| | - Olivier Bouche
- Departments of Hepatogastroenterology and Digestive Oncology, CHU Robert Debré Hospital, Reims, France
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19
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Nishida T, Hølmebakk T, Raut CP, Rutkowski P. Defining Tumor Rupture in Gastrointestinal Stromal Tumor. Ann Surg Oncol 2019; 26:1669-1675. [PMID: 30868512 PMCID: PMC6510879 DOI: 10.1245/s10434-019-07297-9] [Citation(s) in RCA: 57] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/24/2018] [Indexed: 12/25/2022]
Abstract
Tumor rupture is an important risk factor predictive of recurrence after macroscopically complete resection of gastrointestinal stromal tumors (GISTs), and an indication for defined interval or even lifelong adjuvant therapy with imatinib according to guidelines. However, there is no consensus or universally accepted definition of the term ‘tumor rupture’, and, consequently, its incidence varies greatly across reported series. Without predefined criteria, the clinical significance of rupture has also been difficult to assess on multivariate analysis of retrospective data. We reviewed the relevant literature and international guidelines, and, based on the Oslo criteria, proposed the following six definitions for ‘tumor rupture’: (1) tumor fracture or spillage; (2) blood-stained ascites; (3) gastrointestinal perforation at the tumor site; (4) microscopic infiltration of an adjacent organ; (5) intralesional dissection or piecemeal resection; or (6) incisional biopsy. Not all minor defects of tumor integrity should not be classified as rupture, i.e. mucosal defects or spillage contained within the gastrointestinal lumen, microscopic tumor penetration of the peritoneum or iatrogenic damage only to the peritoneal lining, uncomplicated transperitoneal needle biopsy, and R1 resection. This broad definition identifies GIST patients at particularly high risk of recurrence in population-based cohorts; however, its applicability in other sarcomas has not been investigated. As the proposed definition of tumor rupture in GIST has limited evidence based on the small number of patients with rupture in each retrospective study, we recommend validating the proposed definition of tumor rupture in GIST in prospective studies and considering it in clinical practice.
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Affiliation(s)
- Toshirou Nishida
- Department of Surgery, National Cancer Center Hospital, Chuoku, Tokyo, Japan.
| | - Toto Hølmebakk
- Department of Abdominal and Pediatric Surgery, Oslo University Hospital, The Norwegian Radium Hospital, Oslo, Norway
| | - Chandrajit P Raut
- Division of Surgical Oncology, Department of Surgery, Brigham and Women's Hospital, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA
| | - Piotr Rutkowski
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Institute - Oncology Center, Warsaw, Poland
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20
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Burke E, Saeed M, Anant P, Sami B, Salama M, Ahmed I. Large intra-abdominal desmoid tumour posing diagnostic and therapeutic challenges, a case report. J Surg Case Rep 2018; 2018:rjy304. [PMID: 30443319 PMCID: PMC6232280 DOI: 10.1093/jscr/rjy304] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2018] [Revised: 10/08/2018] [Accepted: 10/26/2018] [Indexed: 11/29/2022] Open
Abstract
We present the case of a 46-year-old gentleman originally from China who presented to the acute surgical assessment unit complaining of upper abdominal discomfort, dyspepsia and early satiety ongoing for the previous 6 months. On exam he had a palpable mass in the left upper quadrant. He underwent an esophagogastroduodenoscopy which was normal and later received a CT abdomen which identified a well-circumscribed soft tissue mass in the mesenteric fat and lying adjacent to the transverse colon with no obvious cleavage plane between them. Colonoscopy was then performed which was normal. After discussion at MDT he was taken for laparotomy. At laparotomy the mass was found to be adherent to major vessels, small bowel and large bowel necessitating an extended right hemicolectomy and small bowel resection. The mass itself could not be completely excised. Histology from the resected specimen confirmed desmoid tumour.
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Affiliation(s)
- Eoghan Burke
- Department of Surgery, Our Lady Of Lourdes Hospital, Drogheda, Ireland
| | - Munir Saeed
- Department of Surgery, Our Lady Of Lourdes Hospital, Drogheda, Ireland
| | - Paul Anant
- Department of Surgery, Our Lady Of Lourdes Hospital, Drogheda, Ireland
| | - Babur Sami
- Department of Surgery, Our Lady Of Lourdes Hospital, Drogheda, Ireland
| | - Mohamed Salama
- Department of Surgery, Our Lady Of Lourdes Hospital, Drogheda, Ireland
| | - Ibrahim Ahmed
- Department of Surgery, Our Lady Of Lourdes Hospital, Drogheda, Ireland
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Jakob J, Hohenberger P. Neoadjuvant Therapy to Downstage the Extent of Resection of Gastrointestinal Stromal Tumors. Visc Med 2018; 34:359-365. [PMID: 30498703 PMCID: PMC6257203 DOI: 10.1159/000493405] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023] Open
Abstract
INTRODUCTION Gastrointestinal stromal tumors (GIST) are rare malignant tumors in terms of incidence, and they are not linked to specific symptoms. Often, primary tumors, particularly of the stomach, rectum, or rectovaginal space, are quite large when detected, and multivisceral resection seems to be the treatment of choice as the mainstay of therapy is complete tumor removal. If a gain-of-function mutation in the KIT gene is present, drug therapy with receptor tyrosine kinase inhibitors (RTKIs) might significantly downstage primary GIST tumors. METHODS A review of the literature was performed to identify the current evidence for preoperative treatment of GIST regarding toxicity, efficacy, and oncological outcome, including mutational data from our own database. RESULTS Four phase II as well as several cohort studies showed acceptable toxicity and no increased perioperative morbidity of preoperative imatinib. Progressive disease during preoperative treatment was a rare event, and partial response was achieved in 40-80% of all patients. For methodological reasons, the trials cannot prove an oncological long-term superiority of preoperative treatment. CONCLUSION Preoperative therapy with imatinib is safe and recommended for patients with locally advanced GIST. Neoadjuvant imatinib therapy may enable less invasive and organ-sparing surgery, avoid tumor rupture during extensive resectional procedures, and improve the quality of perioperative RTKI treatment.
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Affiliation(s)
- Jens Jakob
- Division of Surgical Oncology and Thoracic Surgery, Department of Surgery, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany
- Department of General, Visceral and Pediatric Surgery, University Medical Center, Göttingen, Germany
| | - Peter Hohenberger
- Division of Surgical Oncology and Thoracic Surgery, Department of Surgery, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany
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22
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Tu L, Hohenberger P, Allgayer H, Cao H. Standard Approach to Gastrointestinal Stromal Tumors - Differences between China and Europe. Visc Med 2018; 34:353-358. [PMID: 30498702 PMCID: PMC6257205 DOI: 10.1159/000494347] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022] Open
Abstract
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. With the considerable research and application of molecular-targeted therapy for GISTs in the last two decades, GISTs have become a model of multidisciplinary oncological treatment. Although Western clinical guidelines are available for GISTs, such as those by the European Society of Medical Oncology (ESMO), the clinical situations in China are different from those in European countries. There are distinct differences between the clinical practice, diagnostic methods, surgical approach, and availability of new targeted agents in China and those in Europe. This review summarizes the Chinese GIST consensus guidelines compared to the European ones, which may provide an optimal approach to the diagnosis and management of GIST patients.
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Affiliation(s)
- Lin Tu
- Department of Gastrointestinal Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
| | - Peter Hohenberger
- Division of Surgical Oncology, Mannheim University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany
| | - Heike Allgayer
- Department of Experimental Surgery - Cancer Metastasis, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany
| | - Hui Cao
- Department of Gastrointestinal Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
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Sanchez-Hidalgo JM, Duran-Martinez M, Molero-Payan R, Rufian-Peña S, Arjona-Sanchez A, Casado-Adam A, Cosano-Alvarez A, Briceño-Delgado J. Gastrointestinal stromal tumors: A multidisciplinary challenge. World J Gastroenterol 2018; 24:1925-1941. [PMID: 29760538 PMCID: PMC5949708 DOI: 10.3748/wjg.v24.i18.1925] [Citation(s) in RCA: 47] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/27/2018] [Revised: 04/27/2018] [Accepted: 05/06/2018] [Indexed: 02/06/2023] Open
Abstract
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors located in the alimentary tract. Its usual manifestation is gastrointestinal bleeding. However, small asymptomatic lesions are frequently detected as incidental finding. Characteristically, most GISTs (> 95%) are positive for the KIT protein (CD117) by IHC staining and approximately 80%-90% of GISTs carry a mutation in the c-KIT or PDGFRA genes. Mutational analysis should be performed when planning adjuvant and neoadjuvant therapy, due to its possible resistance to conventional treatment. The arise of tyrosine kinase inhibitor has supposed a revolution in GISTs treatment being useful as adjuvant, neoadjuvant or recurrence disease treatment. That is why a multidisciplinary approach to this disease is required. The correct characterization of the tumor at diagnosis (the diagnosis of recurrences and the evaluation of the response to treatment with tyrosine kinase inhibitors) is fundamental for facing these tumors and requires specialized Endoscopist, Radiologists and Nuclear Medicine Physician. Surgery is the only potentially curative treatment for suspected resectable GIST. In the case of high risk GISTs, surgery plus adjuvant Imatinib-Mesylate for 3 years is the standard treatment. Neoadjuvant imatinib-mesylate should be considered to shrink the tumor in case of locally advanced primary or recurrence disease, unresectable or potentially resectable metastasic tumors, and potentially resectable disease in complex anatomic locations to decrease the related morbidity. In the case of Metastatic GIST under Neoadjuvant treatment, when there are complete response, stable disease or limited disease progression, complete cytoreductive surgery could be a therapeutic option if feasible.
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Affiliation(s)
- Juan Manuel Sanchez-Hidalgo
- Department of General and Digestive Surgery, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
| | - Manuel Duran-Martinez
- Department of General and Digestive Surgery, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
| | - Rafael Molero-Payan
- Department of Intern Medicine, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
- Lipids and Atherosclerosis Research Unit, IMIBIC/Hospital Universitario Reina Sofía/Universidad de Córdoba, Cordoba 14004, Spain
| | - Sebastian Rufian-Peña
- Department of General and Digestive Surgery, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
| | - Alvaro Arjona-Sanchez
- Department of General and Digestive Surgery, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
| | - Angela Casado-Adam
- Department of General and Digestive Surgery, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
| | - Antonio Cosano-Alvarez
- Department of General and Digestive Surgery, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
| | - Javier Briceño-Delgado
- Department of General and Digestive Surgery, Reina Sofia University Hospital, Avda. Menéndez Pidal s/n, Cordoba 14004, Spain
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24
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Nishida T. Asian consensus guidelines for gastrointestinal stromal tumor: what is the same and what is different from global guidelines. Transl Gastroenterol Hepatol 2018; 3:11. [PMID: 29552662 DOI: 10.21037/tgh.2018.01.07] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2018] [Accepted: 01/12/2018] [Indexed: 12/12/2022] Open
Abstract
There are some disparities between the clinical practice and profiles of cancer in Asia and those in Europe & North America. In Asia, surgical oncologists still have a major role in the multidisciplinary therapy of gastrointestinal stromal tumor (GIST), whereas medical oncologists hold this status in the West. Although the incidence of clinical GIST is considered similar between the two areas, small gastric GISTs are more frequently treated by surgery in East Asia compared with Europe & North America. The diagnosis and treatment of small submucosal tumors (SMTs), including GIST, is important in Asian clinical practice guidelines for GIST. Most items of Asian and Western GIST guidelines are very similar. There are slight differences between the two guidelines in the degree of recommendation, which may come from disparities of clinical practice and available medicines. Importantly, most clinical evidence in the GIST guidelines has been established by clinical trials conducted in Western countries, and the number of clinical trials is still limited in Asia, suggesting that Asian GIST patients may have limited access to investigational drugs after standard therapy. Finally, both Asian and Western GIST guidelines are well-harmonized in some parts, and their contents may reflect the medical circumstances of each region.
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Affiliation(s)
- Toshirou Nishida
- Department of Surgery, National Cancer Center Hospital, Chuoku, Tokyo 104-0045, Japan
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25
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Abstract
GI stromal tumors (GISTs) are neoplasms with a varying malignancy potential ranging from virtually indolent tumors to rapidly progressing cancers. GISTs occur throughout the intestinal tract, and most harbor an activating mutation in either KIT or platelet-derived growth factor A ( PDGFRA). Diagnosis is made using immunohistochemistry, but molecular testing with mutation analysis is paramount for selection of appropriate therapy. Most small GISTs are cured with surgery. Tyrosine kinase inhibitor (TKI) therapy has led to substantial improvements in survival, both for patients with localized GIST and those with advanced disease. Adjuvant therapy with imatinib benefits patients with a high risk of recurrence, with studies suggesting most benefit with at least 3 years of therapy. Neoadjuvant imatinib therapy should be considered for patients requiring extensive surgery, aiming at shrinking the tumor to allow organ preservation and less extensive surgery. The following three TKIs have been approved for the management of advanced disease: imatinib, sunitinib, and regorafenib; imatinib is usually the best tolerated of the three and the standard first-line treatment. TKIs benefit the majority of patients with advanced GIST but have no or limited efficacy in patients with the PDGFRA D842V mutation or patients with GIST lacking KIT and PDGFRA mutations. Surgery, the mainstay of primary tumor management, also plays a role in the advanced disease setting for selected patients, as do some other approaches such as palliative radiation therapy. Research continues to identify novel therapies, in particular effective agents to treat TKI-refractory disease.
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Affiliation(s)
- Margaret von Mehren
- Margaret von Mehren, Fox Chase Cancer Center, Philadelphia, PA; and Heikki Joensuu, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Heikki Joensuu
- Margaret von Mehren, Fox Chase Cancer Center, Philadelphia, PA; and Heikki Joensuu, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
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26
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Van Houdt W, Schrijver A, Cohen-Hallaleh R, Memos N, Fotiadis N, Smith M, Hayes A, Van Coevorden F, Strauss D. Needle tract seeding following core biopsies in retroperitoneal sarcoma. Eur J Surg Oncol 2017; 43:1740-1745. [DOI: 10.1016/j.ejso.2017.06.009] [Citation(s) in RCA: 46] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/05/2017] [Revised: 05/24/2017] [Accepted: 06/08/2017] [Indexed: 01/21/2023] Open
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27
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Berger‐Richardson D, Swallow CJ. Needle tract seeding after percutaneous biopsy of sarcoma: Risk/benefit considerations. Cancer 2016; 123:560-567. [DOI: 10.1002/cncr.30370] [Citation(s) in RCA: 85] [Impact Index Per Article: 9.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2016] [Accepted: 08/08/2016] [Indexed: 12/17/2022]
Affiliation(s)
- David Berger‐Richardson
- Division of General Surgery, Department of SurgeryUniversity of TorontoToronto Ontario Canada
- Institute of Medical ScienceUniversity of TorontoToronto Ontario Canada
- Lunenfeld‐Tanenbaum Research Institute, Mount Sinai HospitalToronto Ontario Canada
| | - Carol J. Swallow
- Division of General Surgery, Department of SurgeryUniversity of TorontoToronto Ontario Canada
- Institute of Medical ScienceUniversity of TorontoToronto Ontario Canada
- Lunenfeld‐Tanenbaum Research Institute, Mount Sinai HospitalToronto Ontario Canada
- Department of Surgical OncologyPrincess Margaret Cancer CentreToronto Ontario Canada
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