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.
Highlights
Sarcomas are a family of rare mesenchymal neoplasms consisting of over 100 pathologically and genetically heterogeneous tumors accounting for approximately 1% of all malignancies in adults
When transperitoneal core needle biopsy is performed without complications, the procedure does not have an impact on prognosis.[28]
The peritoneum is without relevance to R status; a gastrointestinal stromal tumors (GISTs) disrupted in terms of peritoneal penetration otherwise resected with negative margins is still considered an R0 resection
Summary
Tumor fragility, or transmural tumor necrosis may result in spontaneous perforation with intraperitoneal tumor cell spillage (and spillage of intraluminal contents), a surgical emergency. This may be relatively rare but can happen to deeply ulcerated GISTs with thin tumor walls. 4. Microscopic Adjacent Organ Infiltration: This finding corresponds to T4b in the TNM classification of gastrointestinal carcinomas. Microscopic Adjacent Organ Infiltration: This finding corresponds to T4b in the TNM classification of gastrointestinal carcinomas We acknowledge that this may be a somewhat controversial category. It is not rupture, but represents longstanding peritoneal exposure of a biologically aggressive tumor, and, even if resected en bloc with clear margins, the prognosis is poor.[11,13–15,27] In the Oslo series, all patients with this particular presentation relapsed despite R0 resections.[13,14] Of note, fibrous or inflammatory adhesions without microscopic infiltration showed better prognostic outcomes and should not be considered rupture
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