Esophageal Gastrointestinal Stromal Tumors Research Articles

A Case of Esophageal Gastrointestinal Stromal Tumor Managed With Endoscopic Ultrasound Surveillance

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. They are classified based on their immunohistochemical studies with the expression of c-kit and typically present between 40-50-year-olds. GIST predominantly occur in the stomach (50%) and intestine (25%), followed by the colon and rectum (10%) and rarely in the esophagus (< 5%). Due to the rarity of esophageal GIST (ESOGIST), they are poorly understood and there is a paucity of research evaluating tumor characteristics and management. Our patient is an 83-year-old Hispanic male with a history of prior esophageal impactions due to known Schatzki's rings, presenting with postprandial vomiting. He denies any constitutional symptoms, dysphagia or odynophagia. His initial physical exam and labs are unremarkable. Endoscopy on admission shows a Schatzki's ring at the gastroesophageal junction and new 5mm nodule in the distal esophagus with three ulcers in the esophagus, stomach and duodenum respectively. Pathology of the nodule demonstrates squamous cell mucosa with smooth muscle bundles positive for desmin and negative for S100. Stomach biopsy is positive for H. pylori, which is treated with triple therapy. Endoscopic ultrasound (EUS) guided fine needle aspiration shows a spindle cell neoplasm that is C-kit, DOG-1, and SMA positive and desmin negative. Management of lesions < 2cm are controversial. Thus, given the tumor size and no high risk features on EUS, shared-decision-making was utilized to pursue management with EUS surveillance and planned resection if progressive. ESOGIST is uncommon and comprises less than 1% of all GIST. For lesions < 2cm with suspicious features on EUS, resection is indicated. Surgical resection is also directed for localized ESOGIST ≥2cm or which are symptomatic, with increased overall survival and disease-free survival with adjuvant Imatinib therapy. Enucleation is recommended for management of small tumors (2-5cm) with esophagectomy recommended for tumors >90mm. More research on the management of tumors between 60-90mm needs to be conducted. For larger tumors preoperative Imatinib therapy should be considered. This can improve resectability and is also the standard of therapy when surgery is contraindicated. This case is unique given the rarity of EOSGIST, and highlights the importance of further research on the management of these tumors to establish continued guidelines for care.

Current research status and progress of primary esophageal gastrointestinal stromal tumors in China

Primary esophageal gastrointestinal stromal tumors (GIST) is a rare independent disease with clinicopathological and molecular features different from other mesenchymal tumors. Authors searched and reviewed associated reports and summarized the morbidity trends, characteristics, diagnosis and treatment of esophageal GIST in China. Data show that the incidence and detection rate of esophageal GIST presents the rising trend. Imaging has some characteristics. Ultrasonography and CT are main methods and effective examinations to detect and prompt diagnosis of esophageal GIST. Pathology and immunohistochemistry are the evidence for a definite diagnosis. Risk classification is the important basis for selecting surgical methods and predicting prognosis. Surgery is the mainstay treatment. Very low and low risk patients with tumors less than 3 cm can choose endoscopic resection. For tumors of 3 to 5 cm, tumor resection must be considered. Thoracoscopy appears to be the first choice for surgery. Medium and high risk patients with tumors >5 cm should be treated with partial resection of esophagus. Surgery combined with targeted therapy and neoadjuvant therapy is the main treatment pattern and research direction.

Gastrointestinal stromal tumours of the oesophagus: a clinicopathological and molecular analysis of 27 cases.

Gastrointestinal stromal tumours (GISTs) may arise anywhere in the gastrointestinal tract, but are rare in the oesophagus. We describe the clinical, pathological and molecular characteristics of 27 primary oesophageal GISTs, the largest series to date. DNA was extracted and exons 9, 11, 13 and 17 of KIT, exons 12, 14 and 18 of PDGFRA and exon 15 of BRAF were amplified and sequenced. Oesophageal GISTs occurred in 14 men and 13 women aged between 22 and 80years (mean: 56years). All 27 cases were immunohistochemically positive for KIT, and 92 and 47% co-expressed CD34 or smooth muscle actin, respectively. Fifteen (71% of analysed cases) harboured KIT exon 11 mutations and one case each had a mutation in KIT exon 13 (K642E) or BRAF exon 15 (V600E). Long-term follow-up data (median, 96.5months) were obtained for 20 cases; two patients had metastases at presentation and seven had developed local recurrence and/or metastasis after surgery. A large tumour size (≥10cm), high mitotic rate (>5/5mm2 ), presence of a deletion mutation in KIT exon 11 involving codons 557-558 and a positive microscopic margin were associated with recurrence and metastasis. The KIT mutations identified in oesophageal GISTs are similar to those observed in gastric GISTs. Complete surgical resection with clear margins is recommended, if technically feasible, and genotyping can help to improve diagnosis and further patient management in oesophageal GIST.

Management of esophageal gastrointestinal stromal tumor: review of one hundred seven patients.

Gastrointestinal stromal tumors (GIST) are the most common type of gastrointestinal mesenchymal tumor, but are rarely found in the thoracic esophagus. There is no clear consensus about the optimal treatment of this rare disease. A systematic search of the literature was performed for localized esophageal GIST that was resected between 2000 and 2015, and individual patients were included from two major academic institutions. We obtained information on demographics, tumor size and location, mitotic rate, treatment method, and time to recurrence or death. We performed univariate and multivariate Cox regression analyses to evaluate the factors associated with recurrence or death. A total of 28 studies met our inclusion and exclusion criteria, and with two patients from two academic institutions, we had a total of 107 patients in the study. Due to lack of uniformity among studies, there were several missing data for different variables. The average patient age was 56 (n =98) with mostly males (60%, n =91). The average tumor size on the CT scan was 7.9±5.4 cm (n =91), located mostly in the distal esophagus (81%, n =74). A similar number of patients underwent enucleation (n =47) compared to esophagectomy (n =42). Approximately half of the patients had a mitotic rate of 0-4 mitosis per 50 high-powered field (48%, n =80). The median survival time was 73 months with a 5-year disease free survival of 57% (n =97). Univariate Cox regression analyses showed that a large tumor, undergoing esophagectomy, and a high mitotic rate were associated with poor survival or recurrence control. We found that patients with a lesion smaller than or equal to 5 cm on the CT scan had a better disease-free survival rate than those with a size greater than 5 cm (HR =12.41, p =0.014) and had a 5-year survival rate of 92% with 90% of those patients undergoing enucleation (n =29). Esophageal GIST is a very rare malignancy. The tumor size and mitotic rate of the tumor are associated with poor survival. However, patients with esophageal GIST measuring 5 cm or smaller may be safely treated with esophageal enucleation.

Giant oesophageal gastrointestinal stromal tumour presenting with dyspnoea and clubbed fingers

Gastrointestinal stromal tumours (GISTs) are mesenchymal neoplasms of the gastrointestinal tract originating from the interstitial cells of Cajal. Giant oesophageal GISTs are rare since the oesophagus is rarely the primary...

Patterns of care and clinical outcomes in primary oesophageal gastrointestinal stromal tumours (GIST): A retrospective study of the French Sarcoma Group (FSG)

BackgroundOesophageal GIST (ESOGIST) are very rare tumours requiring special consideration regarding diagnosis, surgical management, and perioperative treatment. MethodsA retrospective study was conducted across 9 centres in the French Sarcoma Group (FSG) to characterize all patients in the years 2000–2014. ResultsSeventeen patients (pts) with primary localized ESOGIST were identified, with median age 69 years (36–81) and 11 females. Eight tumours (T) occurred in the lower third of the oesophagus, five in the oesophageal gastric junction, two in the superior third, and two in the middle third. All pts underwent oesophagoscopy and/or endoscopic ultrasound (EUS) and CT scan. Fifteen had EUS guided biopsy. Nine pts received Imatinib (IM) as initial treatment resulting in six PR, three SD. Tumours were resected in nine pts (53%) (7 upfront, 2 after IM); via enucleation in four (44%) [median size 4 cm], oesophagectomy in five (56%) [median size 10 cm]. Resections were R0 in three pts (33%), R1 in six (66%). Eight pts (47%) had no tumour resection, and one patient was never treated. Six pts received adjuvant IM. With a median follow-up of 24 months (7–101), 11 pts are alive (64.7%), five died (29.4%), one was lost to follow-up. Two pts of 4 pts relapsed following enucleation. ConclusionsESOGIST can be reliably identified pre-operatively by EUS-guided biopsy. Surgery for ESOGIST is either enucleation or oesophagectomy depending on tumour size, location, and patient's individual surgical risk. Preoperative IM therapy could improve resectability and should be considered if surgery is contraindicated or would lead to negative impact on the functional status of the patient.

A Young Man with an Abnormal Chest X-ray

Gastrointestinal stromal tumors(GIST) are an uncommon neoplasms of the GI tract. We present a rare case of esophageal GIST in a previously healthy young male. A 30 year old male presented to the ED after waking up ith cough, eigastric pain, nausea and vomiting. A chest x- ray showed upper mediastinal widening. Contrast enhanced CT scan incshowed a posterior mediastinal mass. Transbronchial needle aspiration and biopsy of a subcarinal mass was performed which revealed necrotic tissuecand was not diagnostic. Repeat biopsy with right video assisted thoracic surgery was performed. Immunohistochemistry was positive for CD117. A repeat chest x-ray 3 months later revealed resolution of previously described mass. He remained well until 10 months after initial presentation when he presented with chest pain, back pain and dry cough. A chest CT revealed a recurrent 5 cm subcarinal density. Right thoracotomy and resection of the mass was performed. Pathology results showed GIST with high mitotic rate. Patient was started on Imatinib. Endoscopic ultrasound two months later showed a residual mass. Fine needle aspiration and biopsy was positive for GIST. He underwent esophagectomy. Histologic evaluation revealed high grade epithelioid GIST. Patient has been asymptomatic without recurrence since.Figure 1Figure 2Figure 3GISTs affect mainly older individuals. It occurs mainly in stomach and express KIT or cell surface marker cd117 in 85-90% of cases, which is a tyrosine kinase receptor. Initial patient evaluation is usually with CT scan. Radical surgery is the treatment of choice in primary resectable GISTs. Almost all are associated with a risk of recurrence with rate of 40-50%. Imatinib Mesylate, inhibitor of KIT Tyrosine Kinase is used as an adjuvant therapy to decrease the risk of post operative recurrence. High risk tumors have a risk of 66% recurrence, making it obvious that surgery alone is not sufficient to provide long term survival. It is also used as neoadjuvant in cases that complete surgical resection is impossible and also for reducing tumor size. Since patients with high risk GIST relapse within 2 years, the follow up should be adopted to tumor criteria. CTS in the technique of choice for follow up. Our patient is particularly unique because of the age of presentation and location of tumor. Immunohistochemistry for CD117 has a high value of specificity for GIST. Treatment is complete resection. Recurrence is mainly dependent on mitotic activity. Imatinib is a useful adjuvant.

Prognostic analysis of gastrointestinal stromal tumors complicated with gastrointestinal bleeding

To study the relationship between clinicopathological characteristics, prognosis and gastrointestinal bleeding in primary gastrointestinal stromal tumors (GIST). The clinicopathological and follow-up data of 200 patients with gastrointestinal stromal tumors treated in our hospital from April 2008 to December 2014 were retrospectively reviewed. The correlation of gastrointestinal bleeding with gastrointestinal stromal tumor clinicopathological characteristics and prognosis were analyzed. The 200 GIST patients were divided into two groups according to the bleeding in the digestive tract, including 57 gastrointestinal bleeding patients and 143 non-bleeding patients. The mean tumor diameter was 6.5 cm (range 1.8-22 cm) in the bleeding group and 2.5 cm (range 0.4-18 cm) in the non-bleeding group (P<0.05). Of the 57 bleeding patients, 31 located in the stomach, 25 in the small intestine, and one had colorectal bleeding. Fifty patients had mitotic index (MI) ≤ 5/50 HPF, other 6 patients ranged between 5 and 10/50 HPF and one patient had MI >10/50 HPF. Six GIST patients were complicated with tumor rapture. But in the non-bleeding group, 125 patients had gastric GIST, 8 in the small intestine, one colorectum, and 9 had esophageal or other GIST. 141 patients had MI ≤5/50 HPF, 1 patients ranged between 5 and 10/50 HPF and one patient had MI >10/50 HPF. Only 1 GIST patients was complicated with tumor rapture. The gastrointestinal bleeding was closely associated with tumor size, mitotic index, tumor location, risk classifications, tumor rapture and tumor recurrence (P<0.05 for all). The 3-year and 5-year survival rates of the 200 patients were 96.5% and 86.8%, respectively. 16 patients developed recurrence or metastasis, and 11 died of GIST. The 5-year survival rate of patients with gastrointestinal bleeding was 76.2%, significantly lower than that of patients without gastrointestinal bleeding (91.6%, P<0.05). GIST patients complicated with gastrointestinal bleeding have poor prognosis, and attention should be paid to stratifying patients for therapy.

Selective indications of surgery in esophageal gastrointestinal stromal tumors: A retrospective study of the French Sarcoma Group (FSG).

10534 Background: Esophageal GIST (ESOGIST) are rare tumors, accounting for less than 1% of all GISTs, requiring special consideration regarding evaluation, diagnosis, perioperative treatment and c...

Clinicopathological features of small gastrointestinal stromal tumors

To investigate the clinicopathological features of small gastrointestinal stromal tumors(GISTs) and to evaluate the efficacy of endoscopic therapy for GISTs. Clinicopathological and follow-up data of 418 patients with GISTs undergoing endoscopic therapy in the Zhongshan Hospital between January 2009 and July 2014 were analyzed retrospectively. All the cases were evaluated by the NIH risk classification and AIFP classification, and were grouped according to the tumor size and location. Nuclear atypia and mitotic count were used to evaluate the biological behavior of small GIST. Efficacy of endoscopic therapy was analyzed with follow-up data. Out of 418 patients, GISTs located in the esophagus was 14(3.3%), in the stomach 389(93.1%), in the duodenum 5(1.2%), in the rectum 10(2.4%). A total of 412(98.6%) patients were mainly spindle cells, and mitosis was not found in 320(76.5%) patients. In 389 small stomach GIST, 245(58.6%) were in fundic region. Cases were divided into four groups according to the size and the result revealed the bigger the size, the more the mitotic count. Nuclear atypia in the 1.5-1.9 cm group was significantly higher compared to other groups. Cases were divided into four groups according to the location and the result revealed the mitotic count was not associated to the location. While the nuclear atypia of stomach GIST was significantly higher than that of esophageal GIST and the nuclear atypia of rectum GIST was significantly higher than that of other positions. The median follow-up was 32(4-69) months. One case(gastric fundus GIST, >1.5 cm) presented local recurrence 23 months after operation and underwent endoscopic resection again. No recurrence or metastasis was found in other patients. Endoscopic resection technique is effective for small GISTs patients. The small GISTs with 0.4 cm diameter or less are often benign and should be followed up for long time. The small GISTs with 0.5 cm diameter or more possess the risk of malignancy, then surgical resection should be performed. Rectum small GISTs (except for 0.4 cm diameter or less) have worse biological behavior and should be removed.

Gastrointestinal stromal tumors (GISTs) at uncommon locations: A large population based analysis

Sparse information is available about GISTs in uncommon locations. Our large database analysis aims to determine the characteristics of GISTs in the esophagus, colon and rectum and compare to gastric GISTs. The Surveillance Epidemiology and End Results (SEER) database was queried from 1990 to 2009 using CS SCHEMA v0203. Characteristics of each location were compared to gastric GISTs. 4411 GIST (29 esophageal, 2658 stomach, 1463 small intestine, 126 colonic, and 135 rectal) from 1990 to 2009 were identified. Univariate and multivariate predictors of worse disease specific survival in both the entire cohort and surgical resection group include older age, male gender, tumor size > 5 cm, no surgical intervention and anatomical location. Although less likely to undergo surgical resection, esophageal GIST (all patients and resected) had a comparable survival to gastric GIST. A higher proportion of colonic GISTs presented with distant disease and had a worse disease specific survival when compared to rectal GISTs. Our results show a rising incidence in GISTs and highlight the characteristics of GISTs based on anatomical location. In addition, this is the first study to demonstrate that colonic GISTs behave differently when compared to rectal GISTs and warrants further prospective evaluation.

Esophageal gastrointestinal stromal tumor: is tumoral enucleation a viable therapeutic option?

The primary objective was to evaluate the feasibility of surgical enucleation of esophageal gastrointestinal stromal tumors (E-GISTs). Secondary objectives evaluated (i) the impact of tumor enucleation on oncological outcomes, (ii) the effect of pretherapeutic biopsy on the feasibility of E-GIST enucleation, and (iii) the impact of mucosal ulceration on outcome. E-GISTs are very rare tumors and esophageal resection has been the recommended approach. The feasibility and impact on outcomes of tumor enucleation are unknown. Through a large national multicenter retrospective study, 19 patients with E-GISTs were identified between 2001 and 2010. Patients who underwent either enucleation or esophagectomy were compared. Of over 19 patients identified with E-GISTs, curative treatment was surgical for 16 patients, with enucleation in 8 and esophagectomy in 8. In the enucleation group, median tumoral diameter was 40 mm (18-65 mm), without any mucosal ulceration, preoperative capsular ruptures, or incomplete resections. In the esophagectomy group, the median tumoral diameter was 85 mm (55-250 mm), with mucosal ulceration in 4 patients, preoperative capsular rupture in 1, and no incomplete resections. Severe postoperative complication rates were 50% and 25% in the esophagectomy and enucleation groups, respectively, with 2 postoperative deaths after esophagectomy. After a median follow-up of 6.4 years, 2 recurrences were observed after esophagectomy versus 0 after enucleation. Endoscopic biopsies did not expose patients to complications or local recurrence after enucleation. Endoscopic mucosal ulceration was associated with more aggressive tumors. E-GIST enucleation seems safe for tumors of less than 65 mm in diameter.

Diagnosis and surgical treatment of esophageal gastrointestinal stromal tumors.

To retrospectively evaluate our experience with the diagnosis and surgical resection of esophageal gastrointestinal stromal tumors (GISTs). Between January 2003 and August 2014, five esophageal GIST cases were admitted to our hospital. In this study, the hospital records, surgery outcomes, tumor recurrence and survival of these patients were retrospectively reviewed. The median age of the patients was 45.6 years (range: 12-62 years). Three patients presented with dysphagia, and one patient presented with chest discomfort. The remaining patient was asymptomatic. Four patients were diagnosed with esophageal GISTs by a preoperative endoscopic biopsy. Three patients underwent esophagectomy, and two patients underwent video-assisted thoracoscopic surgery. The mean operating time was 116 min (range: 95-148 min), and the mean blood loss was 176 mL (range: 30-300 mL). All tumors were completely resected. The mean length of postoperative hospital stay was 8.4 d (range: 6-12 d). All patients recovered and were discharged successfully. The median postoperative follow-up duration was 48 mo (range: 29-72 mo). One patient was diagnosed with recurrence, one patient was lost to follow-up, and three patients were asymptomatic and are currently being managed with close radiologic and clinical follow-up. Surgery is the standard, effective and successful treatment for esophageal GISTs. Long-term follow-up is required to monitor recurrence and metastasis.

Effective Downsizing of a Large Oesophageal Gastrointestinal Stromal Tumour with Neoadjuvant Imatinib Enabling an Uncomplicated and without Tumour Rupture Laparoscopic-Assisted Ivor-Lewis Oesophagectomy.

Neoadjuvant imatinib for gastrointestinal stromal tumours (GISTs) is increasingly used nowadays. As oesophagectomy is associated with high morbidity and mortality, a preoperative downsizing of an oesophageal GIST to limit the extent of resection would be ideal. Because these tumours are rare and neoadjuvant treatment with imatinib is recent, there is limited literature available regarding neoadjuvant administration of imatinib in patients with oesophageal GISTs. A 50-year-old woman presented with total dysphagia. An upper endoscopy and biopsy revealed a large submucosal KIT-positive GIST obstructing the mid oesophagus. CT confirmed a lesion measuring 99 mm × 50 mm × 104 mm. Because the size and location of the tumour increased the risk of intraoperative rupture, it was decided to administer preoperative imatinib. The patient had an excellent clinical and radiological response. Her dysphagia gradually resolved and the follow-up CT scans of the first 10 months showed a gradually reducing tumour size to 54 mm × 33 mm × 42 mm. The patient underwent an uneventful laparoscopic-assisted Ivor-Lewis oesophagectomy. Postoperatively, the patient continued with adjuvant imatinib. At the last follow-up, 1 year from operation and 38 months from the diagnosis, the patient is disease free.

Single-center experience of surgically resected gastrointestinal stromal tumors: A report of six cases, including a rare case involving the lower esophagus.

Gastrointestinal stromal tumors (GISTs) are rare, but remain the most common GI mesenchymal neoplasms. In the present study, six cases of GIST are reported, and one of these cases, a patient with esophageal GIST, is reported in-depth. Certain recent developments in the clinical therapy of GISTs are also discussed. The records of all surgically-resected GI stromal tumors treated at the Al-Hada Military Hospital between January 2007 and December 2012 were reviewed. There were six cases of surgically resected GISTs during this time period, three males and three females, with a mean age of 69.3±16.4 years. The stomach was involved in 66.7% of cases, the small intestine in 16.7% and the esophagus, which is an extremely rare site, in 16.7% of cases. The most common symptom at presentation was abdominal pain, followed by GI bleeding. The mean tumor size was 8.7±6.3 cm. Surgery was indicated by the presence of the aforementioned symptoms or a tumor size >5 cm. All tumors were completely resected with histologically negative margins. The diagnoses were established by immunohistochemistry. Four patients were classified as possessing a high-grade variant, and were administered with tyrosine kinase inhibitors (TKIs). Following a mean follow up of 31 months, no recurrence or mortality was detected. Complete surgical resection with tumor-free margins is the standard treatment for GISTs, and TKIs should be used as adjuvant therapy if the risk of progressive disease is high.

The Mesocolon

Colonic mobilization requires separation of mesocolon from underlying fascia. Despite the surgical importance of planes formed by these structures, no study has formally characterized their microscopic features. The aim of this study was to determine the histological and electron microscopic appearance of mesocolon, fascia, and retroperitoneum, prior to and after colonic mobilization. In 24 cadavers, samples were taken from right, transverse, descending, and sigmoid mesocolon. In 12 cadavers, specimens were stained with hematoxylin and eosin (3 sections) or Masson trichrome (3 sections). In the second 12 cadavers, lymphatic channels were identified by staining immunohistochemically for podoplanin. The ascending mesocolon was assessed with scanning electron microscopy. The above process was first conducted with the mesocolon in situ. The mesocolon was then surgically mobilized, and the process was repeated on remaining structures. The microscopic structure of mesocolon and associated fascia was consistent from ileocecal to mesorectal level. A surface mesothelium and underlying connective tissue were evident throughout. Fibrous septae separated adipocyte lobules. Where apposed to retroperitoneum, 2 mesothelial layers separated mesocolon and underlying retroperitoneum. A connective tissue layer occurred between these (ie, Toldt's fascia). Lymphatic channels were evident both in mesocolic connective tissue and Toldt's fascia. After surgical separation of mesocolon and fascia both remained contiguous, the fascia remained in situ and the retroperitoneum undisturbed. The findings demonstrate that the contiguous mesocolon and retroperitoneum are separated by mesothelial and connective tissue layers. These properties generate the surgical planes (ie, meso- and retrofascial planes) exploited in colonic and mesocolic mobilization.

Imaging and clinicopathologic features of esophageal gastrointestinal stromal tumors.

The purpose of this article is to describe the imaging and clinicopathologic characteristics of esophageal gastrointestinal stromal tumors (GISTs) and to emphasize the features that differentiate esophageal GISTs from esophageal leiomyomas. A pathology database search identified all surgically resected or biopsied esophageal GISTs, esophageal leiomyomas, and esophageal leiomyosarcomas from 1994 to 2012. Esophageal GISTs were included only if imaging studies (including CT, fluoroscopic, or (18)F-FDG PET/CT scans) and clinical data were available. Nineteen esophageal mesenchymal tumors were identified, including eight esophageal GISTs (42%), 10 esophageal leiomyomas (53%), and one esophageal leiomyosarcoma (5%). Four patients (50%) with esophageal GIST had symptoms, including dysphagia in three (38%), cough in one (13%), and chest pain in one (13%). One esophageal GIST appeared on barium study as a smooth submucosal mass. All esophageal GISTs appeared on CT as well-marginated predominantly distal lesions, isoattenuating to muscle, that moderately enhanced after IV contrast agent administration. Compared with esophageal leiomyomas, esophageal GISTs tended to be more distal, larger, and more heterogeneous and showed greater IV enhancement on CT. All esophageal GISTs showed marked avidity (mean maximum standardized uptake value, 16) on PET scans. All esophageal GISTs were positive for c-KIT (a cell-surface transmembrane tyrosine kinase also known as CD117) and CD34. On histopathology, six esophageal GISTs (75%) were of the spindle pattern and two (25%) were of a mixed spindle and epithelioid pattern. Five esophageal GISTs had exon 11 mutations (with imatinib sensitivity). Clinical outcome correlated with treatment strategy (resection plus adjuvant therapy or resection alone) rather than risk stratification. Esophageal GISTs are unusual but clinically important mesenchymal neoplasms. Although esophageal GISTs and esophageal leiomyomas had overlapping imaging features, esophageal GISTs tended to be more distal, larger, more heterogeneous, and more enhancing on CT and were markedly FDG avid on PET. Given their malignant potential, esophageal GISTs should be included in the differential diagnosis of intramural esophageal neoplasms.

An esophageal gastrointestinal stromal tumor in a patient with MEN1-related pancreatic gastrinoma: An unusual association and review of the literature

Both multiple endocrine neoplasia type 1 (MEN1)-related gastrinomas and gastrointestinal stromal tumors (GISTs) are rare neoplasms, and their association has been rarely reported. We describe an unusual association between a GIST and a MEN1-related gastrinoma. A 44-year-old man had undergone surgical removal of a pancreatic gastrinoma in 2004 and was then administered long-term somatostatin analogs, and diagnosed as having MEN1 syndrome. Following an uneventful follow-up, in April 2009, an upper gastrointestinal tract endoscopy showed esophageal narrowing, with evidence of a 2-cm solid mass on endoscopic ultrasonography. Histology revealed a tumor composed of elongated cells with plump cytoplasm arranged in a storiform pattern. The immunophenotype of the lesion was CD117 and Platelet Derived Growth Factor (PDGF) positive, whereas alpha-1 muscle actin and S-100 protein were negative. Due to morphological and immunohistochemical results, a final diagnosis of esophageal GIST was made. The association between GISTs and MEN1 could be casual, although a single case of the coexistence of a GIST and a MEN1-related gastrinoma has already been reported. A role of the MEN1 gene in the pathogenesis of GISTs could be hypothesized.

GIST des Ösophagus im linken tracheobronchialen Winkel: Resektion mittels rechtsthorakaler VATS

The vast majority of submucosal oesophageal tumours are leiomyomas. Gastrointestinal stromal tumours (GIST) account for only one percent of all gastrointestinal malignancies, whereby an oesophageal location represents a medical rarity. Although surgical resection is the gold standard, the optimal procedure remains a matter of debate. Conventional oesophageal resection is the most common therapeutic choice to ensure complete removal with an appropriate safety margin and avoid dissemination of neoplastic cells. Anyhow, occasional case reports about enucleation of oesophageal GIST have been published. This video paper for the first time describes the use of right-sided video-assisted thoracoscopic surgery (VATS) as a technique to enucleate an oesophageal GIST within the left tracheobronchial angle. A 68-year-old male patient was evaluated for recurrent haemoptysis and dysphagia and diagnosed with a 1.4 cm sized oesophageal tumour at the left tracheobronchial angle. CT scan showed a submucosal location without signs of invasive growth. After conducting endosonography we suspected a leiomyoma and performed minimally invasive enucleation with right-sided VATS. Employing independent lung ventilation the patient was put into a left lateral position. Four trocars were applied. After incision of the mediastinal pleura the tumour was localised with intraoperative gastroscopy and diaphanoscopy. By mobilisation of the oesophagus and transection of the azygous vein it was possible to bluntly dissect the mass from surrounding oesophageal muscular fibres and safely extract it with a specimen bag. After removal of the thoracic drainage on the first postoperative day the patient was discharged the day after. The further course was uneventful. Histological work-up showed a GIST pT1cN0M0 R0 with a Ki-67 index less than 1 % (UICC I). After 10 months of follow-up the patient is recurrence-free and shows no symptoms. Enucleation of a locally limited oesophageal GIST by right-sided VATS is a feasible and safe surgical procedure with dramatically reduced invasiveness compared to conventional open oesophageal resections.

Tumor rupture during surgery for gastrointestinal stromal tumors: Pay attention!

In a recently published letter to the editor, we debated the proposal by Coccolini et al to treat gastrointestinal stromal tumors (GISTs) of the esophagogastric junction with enucleation and, if indicated, adjuvant therapy. We highlighted that, because the prognostic impact of a T1 high-mitotic rate esophageal GIST is worse than that of a T1 high-mitotic rate gastric GIST, enucleation may not be adequate surgery for esophagogastric GISTs with a high mitotic rate. In rebuttal, Coccolini et al pointed out the possible bias in assessment of the mitotic rates due to the lack of standardized methods and underlined that the site and features of the tumor need to be carefully considered in evaluation of the risk-benefit balance. Here we confirm that, apart from the problematic issue of mitotic counting, enucleation should not be indicated for GISTs at any site to reduce the risk of tumor rupture, which has been recently considered to be an unfavorable prognostic factor, and to avoid microscopic residual tumor.