Extraordinarily Large Malignant Extra Gastro Intestinal Stromal Tumor Presenting as an Abdominal Mass

2019 ◽  
Vol 2 (2) ◽  
pp. 84-87
Author(s):  
P Paudyal ◽  
TN Subba ◽  
A Pradhan ◽  
S Dhakal ◽  
S Karki ◽  
...  

Background: Gastrointestinal stromal tumors (GIST) are rare of all gastrointestinal neoplasms with histology and immunohistochemistry similar to gastrointestinal stromal tumors. They occur outside the gastrointestinal tract rarely and are, hence, called the extra gastrointestinal stromal tumors (EGIST). Herein, we report a rare case of malignant extra intestinal gastrointestinal tumor. Case report: A 39 yr old female presented to the surgical OPD with a lump in the abdomen since 4 months. We received a lobulated tumor which measured 40x25x20 cm3 and showed capsule. Cut surface was predominantly solid, grey white with areas of hemorrhage and necrosis along with cystic areas. Histopathology and immunohisto chemistry revealed it to be a malignant EGIST of mesentery because of no continuity of the tumor with the small intestine microscopically. Conclusion: EGIST is a rare no epithelial tumor of mesentery. Omentum histopathological examination is a standard technique to diagnose the tumor and to differentiate it immunohistochemically from other tumors having similar morphology. Correct diagnosis plays valuable role in the treatment.

2015 ◽  
Vol 68 (11-12) ◽  
pp. 413-417 ◽  
Author(s):  
Vlado Janevski ◽  
Redzep Selmani ◽  
Vesna Janevska ◽  
Liljana Spasevska ◽  
Julija Zhivadinovik

Introduction. Gastrointestinal stromal tumors are the most common mesenchymal tumors of the digestive tract. Leiomyosarcomas of the gastrointestinal tract are rare mesenchymal neoplasms which grossly and histologically resemble gastrointestinal stromal tumors. They may be differentiated from gastrointestinal stromal tumors by using immunohistochemistry and they are typically positive for ? smooth muscle actin and desmin and negative for c-kit, CD34 and DOG1.1. They often express calponin and h-caldesmon. Case Report. We present a case of a 59-year-old male with anemia, weight loss, intermittent abdominal pain and right abdominal mass. Colonoscopy revealed an exophytic ulcerated neoplastic mass in the ascending colon and abdominal computed tomography scan showed an ill-defined heterogeneous tumor mass which surrounded almost the whole ascending colon. The patient underwent right hemicolectomy and partial resection of ileum. Histopathological examination revealed a leiomyosarcoma composed of atypical spindle cells positive for ? smooth muscle actin, desmin and vimentin, and negative for c-kit, CD34, S100 and neuron specific enolase. The patient is alive 8 months after the operation, undergoing chemotherapy. Conclusion. We have concluded that the multimodal approach comprising chemotherapy and complete surgical resection controls the leiomyosarcomas.


2021 ◽  
Vol 11 (8) ◽  
pp. 694
Author(s):  
Gina Gheorghe ◽  
Nicolae Bacalbasa ◽  
Gabriela Ceobanu ◽  
Madalina Ilie ◽  
Valentin Enache ◽  
...  

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. They are potentially malignant, and have an unpredictable evolution. The origin of these tumors is in the interstitial cells of Cajal, which are cells that are interposed between the intramural neurons and the smooth muscle cells of the digestive tract. GISTs are characterized by mutations in the gene c-Kit, but also other mutations, such as those of the platelet-derived growth factor receptor alpha. The most common locations of these tumors are the stomach and small intestine, although they can occur at any level of the digestive tract and occasionally in the omentum, mesentery and peritoneum. Most cases of GISTs are sporadic, and about 5% of cases are part of family genetic syndromes. The correct diagnosis of GIST is determined by histopathological examination and immunohistochemistry. According to histopathology, there are three main types of GISTs: spindle cell type, epithelioid type and mixed type. The therapeutic management of GIST includes surgery, endoscopic treatment and chemotherapy. The prognosis of patients with GIST varies depending on a number of factors, such as risk category, GIST stage, treatment applied and recurrence after treatment.


2015 ◽  
Vol 68 (11-12) ◽  
pp. 418-420 ◽  
Author(s):  
Nada Dimkovic ◽  
Danijela Bojic ◽  
Petar Svorcan ◽  
Aleksandar Jankovic ◽  
Petar Djuric ◽  
...  

Introduction. Gastrointestinal stromal tumors are the most common mesenchymal tumors of the digestive tract. Leiomyosarcomas of the gastrointestinal tract are rare mesenchymal neoplasms which grossly and histologically resemble gastrointestinal stromal tumors. They may be differentiated from gastrointestinal stromal tumors by using immunohistochemistry and they are typically positive for ? smooth muscle actin and desmin and negative for c-kit, CD34 and DOG1.1. They often express calponin and h-caldesmon. Case Report. We present a case of a 59-year-old male with anemia, weight loss, intermittent abdominal pain and right abdominal mass. Colonoscopy revealed an exophytic ulcerated neoplastic mass in the ascending colon and abdominal computed tomography scan showed an ill-defined heterogeneous tumor mass which surrounded almost the whole ascending colon. The patient underwent right hemicolectomy and partial resection of ileum. Histopathological examination revealed a leiomyosarcoma composed of atypical spindle cells positive for ? smooth muscle actin, desmin and vimentin, and negative for c-kit, CD34, S100 and neuron specific enolase. The patient is alive 8 months after the operation, undergoing chemotherapy. Conclusion. We have concluded that the multimodal approach comprising chemotherapy and complete surgical resection controls the leiomyosarcomas.


2021 ◽  
Vol 22 (2) ◽  
pp. 493
Author(s):  
Christos Vallilas ◽  
Panagiotis Sarantis ◽  
Anastasios Kyriazoglou ◽  
Evangelos Koustas ◽  
Stamatios Theocharis ◽  
...  

Gastrointestinal stromal tumors (GISTs) are the most common types of malignant mesenchymal tumors in the gastrointestinal tract, with an estimated incidence of 1.5/100.000 per year and 1–2% of gastrointestinal neoplasms. About 75–80% of patients have mutations in the KIT gene in exons 9, 11, 13, 14, 17, and 5–10% of patients have mutations in the platelet-derived growth factor receptor a (PDGFRA) gene in exons 12, 14, 18. Moreover, 10–15% of patients have no mutations and are classified as wild type GIST. The treatment for metastatic or unresectable GISTs includes imatinib, sunitinib, and regorafenib. So far, GIST therapies have raised great expectations and offered patients a better quality of life, but increased pharmacological resistance to tyrosine kinase inhibitors is often observed. New treatment options have emerged, with ripretinib, avapritinib, and cabozantinib getting approvals for these tumors. Nowadays, immune checkpoint inhibitors form a new landscape in cancer therapeutics and have already shown remarkable responses in various tumors. Studies in melanoma, non-small-cell lung cancer, and renal cell carcinoma are very encouraging as these inhibitors have increased survival rates. The purpose of this review is to present alternative approaches for the treatment of the GIST patients, such as combinations of immunotherapy and novel inhibitors with traditional therapies (tyrosine kinase inhibitors).


Diagnostics ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. 636
Author(s):  
Masato Tanaka ◽  
Sumeet Sonawane ◽  
Koji Uotani ◽  
Yoshihiro Fujiwara ◽  
Kittipong Sessumpun ◽  
...  

Background: Percutaneous biopsy under computed tomography (CT) guidance is a standard technique to obtain a definitive diagnosis when spinal tumors, metastases or infections are suspected. However, specimens obtained using a needle are sometimes inadequate for correct diagnosis. This report describes a unique biopsy technique which is C-arm free O-arm navigated using microforceps. This has not been previously described as a biopsy procedure. Case description: A 74-year-old man with T1 vertebra pathology was referred to our hospital with muscle weakness of the right hand, clumsiness and cervicothoracic pain. CT-guided biopsy was performed, but histopathological diagnosis could not be obtained due to insufficient tissue. The patient then underwent biopsy under O-arm navigation, so we could obtain sufficient tissue and small cell carcinoma was diagnosed on histopathological examination. A patient later received chemotherapy and radiation. Conclusions: C-arm free O-arm navigated biopsy is an effective technique for obtaining sufficient material from spine pathologies. Tissue from an exact pathological site can be obtained with 3-D images. This new O-arm navigation biopsy may provide an alternative to repeat CT-guided or open biopsy.


2012 ◽  
Vol 6 ◽  
pp. CMO.S9180 ◽  
Author(s):  
Claudio Casella ◽  
Vincenzo Villanacci ◽  
Filippo D'adda ◽  
Manuela Codazzi ◽  
Bruno Salerni

Background Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. More rarely neoplasms with histology and immunohistochemistry similar to GISTs may occur outside the gastrointestinal tract (omentum, mesentery and retroperitoneum) and are so-called Extra-gastrointestinal Stromal Tumors (EGISTs). EGISTs arising in the retroperitoneum are extremely rare: to date, only 58 cases have been reported in the literature. Case Report We herein report a case of a primary EGIST of the retroperitoneum surgically treated. The pre-operative radiological evaluation showed a retroperitoneal mass, placed in left paravertebral region. Results Morphological and immunohistochemical features led to a diagnosis of extra-gastrointestinal stromal tumor (intermediate-low risk form). Conclusions As a result of the rarity of reports of primary EGISTs of retroperitoneum we need to analyze the data of reported cases in order to gain a better understanding about the pathogenesis, prognosis and optimal treatment of this disease.


2016 ◽  
Vol 63 (2) ◽  
pp. 115-117
Author(s):  
Beatrice Lintoiu ◽  
◽  
Irina Balescu ◽  
Nicolae Bacalbasa ◽  
◽  
...  

Schwannomas are rare tumors originating from the Schwann cells, that form the neural sheath. These tumors occur most frequently in the head, neck, arms and limbs. Primary schwannomas of the colon and rectum are extremely rare. They are usually benign. Pre-operative biopsy examinations may be difficult and immunohistochemistry is necessary for the correct diagnosis. In contrast to gastrointestinal stromal tumors, schwannomas are negative for CD117 and positive for S100 protein and vimentin. In extremely rare cases, they can present with malignant degeneration if not surgically removed. Therefore, the gold standard treatment for schwannomas is surgical resection.


2010 ◽  
Vol 76 (11) ◽  
pp. 1244-1250 ◽  
Author(s):  
Jaime Ruiz-Tovar ◽  
María Diez-Tabernilla ◽  
Gada Housari ◽  
Enrique Martinez-Molina ◽  
Alfonso Sanjuanbenito

The aim of this study is to analyze the clinical outcome of gastrointestinal stromal tumors (GISTs) and to determine new prognostic factors. We perform a retrospective study of all the patients diagnosed with GIST in any location and operated on between 2000 and 2008 at our institution. We analyzed 35 patients, 16 males (45.7%) and 19 females (54.3%), with a mean age of 64 ± 13.8 years. The tumors were located in the stomach in 22 patients (62.9%), in the small bowel in 10 (28.6%), and the retroperitoneum in three (8.6%). Referring to gastric GIST, endoscopy revealed an ulceration in the mucosa in five cases, suggesting an epithelial neoplasm. In all these cases, pathology of the biopsy specimen was nonconclusive. Survival rate at 1 and 5 years was 94.3 and 88.6 per cent, respectively. Disease-free survival at 1 and 2 years was 91.4 and 88.6 per cent, respectively. Analyzing prognostic factors, a lower disease-free survival was observed among patients with constitutional syndrome at diagnosis ( P = 0.000), small bowel GIST ( P = 0.037), and tumors not expressing actin ( P = 0.015). A lower global survival was observed among men ( P = 0,036), patients with an abdominal mass ( P = 0.033) or with constitutional syndrome ( P = 0.007) at diagnosis and tumors at a retroperitoneal location ( P = 0.0002). Gastric GIST may be confused with epithelial neoplasms, modifying the surgery. In our patients, masculine gender, constitutional syndrome and abdominal mass at diagnosis, small bowel and retroperitoneal location, and actin negative tumors are bad prognostic factors.


2021 ◽  
Vol 8 (4) ◽  
pp. 1325
Author(s):  
Samir Paruthy ◽  
Shivani B. Paruthy

Gastrointestinal stromal tumors (GIST) are soft tissue mesenchymal tumor originating from intestinal cells of Cajal which are pace makers of the intestine. GIST commonly arises from stomach and intestine although they seem to appear occasionally in mesentery, omentum and esophagus, but origin from retro-peritoneum is very infrequent.  We herein report case of a huge retroperitoneal GIST measuring 16x13x9 cm size, in a 52-year-old lady occupying whole abdomen arising as pelvic mass. GIST was initially mistaken with associated large intramural fibroid uterus and a large pedunculated sub-serosa broad ligament fibroid. Patient was taken for abdominal open hysterectomy with complete tumor excision after all adhesions from adjoining intestine was separated. Histopathological examination of the mass confirmed it was retroperitoneal GIST with positive immune-histochemistry. Patient responded well to Imatinib therapy, and for last 4 years there has been no residual disease or recurrence as evidenced with regular follow up. 


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