Multiple primary sporadic gastrointestinal stromal tumors in the adult: an underestimated entity.
نویسندگان
چکیده
PURPOSE Gastrointestinal stromal tumors (GIST) are commonly regarded as solitary tumors. The occurrence of multiple lesions is considered an extraordinary event restricted to pediatric GISTs and rare hereditary conditions. Beyond these well-defined situations, the presentation of multiple synchronous lesions is commonly viewed as the result of the metastatic spreading of a single primary GIST. Based on this axiom, patients with multifocal disease are classified as advanced stage and treated as such. Whether, indeed, the detection of several lesions in sporadic adult GIST patients may be suggestive of phenomena of tumor multiplicity still needs to be clarified. EXPERIMENTAL DESIGN From a multicentric series of 442 consecutive cases, 26 of which with advanced disease, we selected 5 patients who presented up to three distinct GIST nodules. Five additional cases with similar characteristics were also contributed by two other institutions. The clonal relationship between the synchronous lesions was assessed by comparing KIT/PDGFRA mutation and microsatellite pattern. RESULTS An independent origin of the synchronous lesions was established in 6 of 10 cases. Notably, in one patient, one lesion arose in the peritoneum, which is ordinarily regarded as a site of metastasis. CONCLUSIONS Our data indicate that a significant fraction of GIST patients with multifocal presentation are actually affected by multiple primary tumors, suggesting that mesenchymal GIST precursor cells of these individuals are somehow primed to transformation. Thus, in the presence of multifocal GIST manifestations, an accurate characterization of the different tumor sites should be undertaken for a proper patient staging and therapy planning.
منابع مشابه
The study of 13 cases of Gastrointestinal stromal tumors in Kerman
13 cases of gastrointestinal stromal tumors (GIST) diagnosed in Kerman university hospitals between 1370-1376 are reviewed.of these cases,6 were male and 7 were female.patients, age ranged between 19 to 70 years with a median of 47.8 years. in 8 cases tumor developed in stomach and in 5 cases it originated from small intestine.all available H & E stained slides were reviewed and PAS and masson...
متن کاملFrequency of KIT Mutation in Gastrointestinal Stromal Tumors According to Histologic and Immunohistochemical Findings, the First Report from Iran
Background: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. They are believed to originate from the interstitial cells of Cajal. Most of these tumors contain activating mutations in the KIT receptor tyrosine kinase. This is the first study in Iran to evaluate GISTs at the molecular level.Methods: In the present study, during 5 years (...
متن کاملPrimary Stromal Sarcoma of Breast: A Rare Entity
Primary soft tissue sarcomas of the breast constitute less than 5% of all soft tissue sarcomas and less than 1% of malignant breast cancers. The rarity of this tumor limits most studies to small retrospective case reviews and case reports. Primary breast sarcomas are locally aggressive tumors as evidenced by the high rate of local recurrence when excisional surgery is performed. A contemporary ...
متن کاملUnique case of sporadic multiple gastro intestinal stromal tumour
INTRODUCTION Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract and they derived from transformed neoplastic precursors of Cajal's interstitial cell (ICC). PRESENTATION OF THE CASE We are presenting a sporadic and exemplary case of 42 multiple GISTs in a young female patient. Our patient showed anemia for the gastric GIST bleeding and ...
متن کاملAdult Patient with Synchronous Gastrointestinal Stromal Tumor and Xp11 Translocation-Associated Renal Cell Carcinoma: A Unique Case Presentation with Discussion and Review of Literature
Gastrointestinal stromal tumor (GIST) is the most common primary mesenchymal tumor of the gastrointestinal tract. This entity comprises a wide spectrum of tumors that vary from benign to overtly malignant, with the majority of these tumors harboring oncogenic mutations of the KIT receptor tyrosine kinase that can aid in diagnosis as well as in targeted therapy. Although the majority of GISTs ar...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Clinical cancer research : an official journal of the American Association for Cancer Research
دوره 14 18 شماره
صفحات -
تاریخ انتشار 2008