A Comparative Study of Serum C-reactive Protein Level Between Pre-eclampsia and Normal Pregnancy in Tertiary Level Hospital

Authors: Jannat S , Islam A , Yesmin S , Shanta FI , Jabin AM , Sanjida S and Trishna D

DOI: 10.23880/cprj-16000217

Volume 9, Issue 1

Abstract

Perivascular epithelioid cell tumors (PEComas) represent a rare family of mesenchymal neoplasms with distinctive morphological and immunophenotypic features. Gastrointestinal PEComas constitute an uncommon subset with variable clinical behavior, ranging from benign localized lesions to malignant and metastatic neoplasms. Accurate diagnosis requires integration of histologic features, immunohistochemical analysis demonstrating myomelanocytic differentiation, and exclusion of mimics such as gastrointestinal stromal tumors, metastatic melanoma, and epithelioid smooth muscle tumors. Central to the pathogenesis of PEComas is dysregulation of the mTOR signaling pathway. TSC1 and TSC2 alterations underlie mTOR activation in a significant proportion of conventional PEComas, whereas a subset of cases harbor TFE3 gene rearrangements that define a biologically and clinically distinct subgroup. These molecular features not only clarify pathogenetic mechanisms but also support targeted therapeutic strategies, most notably mTOR inhibitors, which have demonstrated meaningful clinical activity in advanced or unresectable disease. Risk stratification remains challenging, as existing criteria—particularly those proposed by Folpe and colleagues—were developed from heterogeneous cohorts and incompletely predict behavior in gastrointestinal sites. Emerging evidence suggests the incorporation of additional features such as Ki-67 index, necrosis, mitotic activity, and molecular alterations may improve prognostic accuracy. Comprehensive surgical excision remains the primary treatment for localized tumors, while systemic therapy, particularly mTOR pathway inhibition, is reserved for recurrent, unresectable, or metastatic disease. Continued multidisciplinary collaboration, expanded molecular profiling, and development of prospective registries are essential to refine risk prediction, therapeutic selection, and long-term management of gastrointestinal PEComas.

Keywords: PEComa; Gastrointestinal tract; mTOR pathway; TFE3 rearrangement; Molecular diagnostics; Targeted therapy

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