Esophageal Gastrointestinal Stromal Tumor Enucleation
Objective 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.
Background 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.
Methods 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.
Results 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.
Conclusions E-GIST enucleation seems safe for tumors of less than 65 mm in diameter.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor of the gastrointestinal tract and for the most part occur in the stomach (60%–70%) and small intestine (20%–30%). Esophageal gastrointestinal stromal tumors (E-GISTs) are extremely uncommon, accounting for 0.7% of all GISTs. As with most rare pathologies, many questions remain unanswered regarding their optimal management. Although pathological series of E-GISTs has been published, the reporting of surgical series has been limited to individual case reports and case series of small numbers. Not only are E-GISTs less common than abdominal GISTs, but the esophagus also differs anatomically, lacking both a confining serosal layer and a mesentery, meaning that segmental or wedge resections used for stomach and small intestinal GISTs are not feasible. Esophagectomy is an operation with a significant morbidity whose systematic use for small E-GISTs, with low malignant potential, seems questionable. The other surgical option is tumor enucleation, an approach that preserves the esophagus. Concerns exist that tumor enucleation could lend itself to incomplete tumor resection, with a higher risk of disrupting the tumor capsule and hence compromised oncological outcomes. The optimal surgical approach, therefore, remains to be defined and tailored to malignant risk.
Previously E-GISTs were confused with leiomyomas, leiomyoblastomas, and leiomyosarcomas; however, immunohistochemical staining for CD117 and CD34 has distinguished them from these other esophageal tumors. Preoperative biopsy or fine-needle aspiration cytology (FNAC) provides one possible means of differentiating E-GISTs from benign lesions and may guide decisions regarding the necessity and radicality of resection. Whether preoperative biopsies have an impact on the feasibility of surgical enucleation or oncological outcomes has not been established.
The aim of this study was consequently to evaluate (i) the feasibility and oncological outcomes of E-GIST enucleation, (ii) the impact of diagnostic tumor sampling on the feasibility of enucleation, and (iii) the impact of mucosal ulceration on outcome.
Abstract and Introduction
Abstract
Objective 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.
Background 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.
Methods 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.
Results 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.
Conclusions E-GIST enucleation seems safe for tumors of less than 65 mm in diameter.
Introduction
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor of the gastrointestinal tract and for the most part occur in the stomach (60%–70%) and small intestine (20%–30%). Esophageal gastrointestinal stromal tumors (E-GISTs) are extremely uncommon, accounting for 0.7% of all GISTs. As with most rare pathologies, many questions remain unanswered regarding their optimal management. Although pathological series of E-GISTs has been published, the reporting of surgical series has been limited to individual case reports and case series of small numbers. Not only are E-GISTs less common than abdominal GISTs, but the esophagus also differs anatomically, lacking both a confining serosal layer and a mesentery, meaning that segmental or wedge resections used for stomach and small intestinal GISTs are not feasible. Esophagectomy is an operation with a significant morbidity whose systematic use for small E-GISTs, with low malignant potential, seems questionable. The other surgical option is tumor enucleation, an approach that preserves the esophagus. Concerns exist that tumor enucleation could lend itself to incomplete tumor resection, with a higher risk of disrupting the tumor capsule and hence compromised oncological outcomes. The optimal surgical approach, therefore, remains to be defined and tailored to malignant risk.
Previously E-GISTs were confused with leiomyomas, leiomyoblastomas, and leiomyosarcomas; however, immunohistochemical staining for CD117 and CD34 has distinguished them from these other esophageal tumors. Preoperative biopsy or fine-needle aspiration cytology (FNAC) provides one possible means of differentiating E-GISTs from benign lesions and may guide decisions regarding the necessity and radicality of resection. Whether preoperative biopsies have an impact on the feasibility of surgical enucleation or oncological outcomes has not been established.
The aim of this study was consequently to evaluate (i) the feasibility and oncological outcomes of E-GIST enucleation, (ii) the impact of diagnostic tumor sampling on the feasibility of enucleation, and (iii) the impact of mucosal ulceration on outcome.
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