Gastrointestinal stromal tumour (GIST) is a recent recognised tumour entity. In the past, those tumours were classified as leiomyomas, leiomyosarcomas and leiomyoblastomas, but it is now evident that GIST is a separate tumour entity and is the most common sarcoma of the gastrointestinal tract especially with advances in immunohistochemical staining techniques and improvements in microscopic structural imaging. We present a case of GIST of unusual location and presentation pattern, with an overview over current GISTs’ diagnosis and management strategies. The precise incidence and tumour behaviour of rare extragastrointestinal stromal tumour (EGIST) remain to be clarified. Further research is needed in large series with long duration of follow-up and modified risk stratification assessment tailored for EGISTs.
Summary
Phyllodes tumors (PT) are fibroepithelial neoplasms of the breast showing a peculiar leaf-like appearance. They account for 0.3 to 1% of all primary breast tumors and 2.5% of all fibroepithelial breast tumors. PT are classified into benign, borderline and malignant based upon their stromal morphology with a distribution of 60%, 20%, and 20%, respectively. Malignant PT of the breast constitute an uncommon challenging group of fibroepithelial neoplasms. They have a relatively high tendency to recur, although distant metastasis is uncommon, and nearly exclusive to malignant PT. Adequate surgical resection remains the standard approach to achieve maximal local control. Giant malignant PT are rare and a pose a diagnostic dilemma for pathologists, especially when comprised of sarcomatous elements. This review highlights the morphological features of PT detected in cytology and histology specimens and discusses diagnostic pitfalls and differential diagnosis.
HighlightsFibromatosis is an infiltrating histologically low grade spindle cell proliferation composed of fibroblastic cells with a variable amount of collagen.The treatment of choice for breast fibromatosis; is primary surgical excision with clean margins.It is characterized by being locally aggressive but not metastasizing.Fibromatosis has a high rate of recurrence after surgical excision.Management of recurrent breast fibromatosis remains controversial because of the low incidence and, in consequence, the limited data.
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