Practical Approach to the Diagnosis of the Vulvo-Vaginal Stromal Tumors: An Overview

Abstract

Background: The category of the “stromal tumors of the lower female genital tract” encompasses a wide spectrum of lesions with variable heterogeneity, which can be nosologically classified on the basis of their morphologic and immunohistochemical profiles as deep (aggressive) angiomyxoma (DAM), cellular angiofibroma (CAF), angiomyofibroblastoma (AMFB) or myofibroblastoma (MFB). Despite the differential diagnosis between these entities being usually straightforward, their increasingly recognized unusual morphological variants, along with the overlapping morphological and immunohistochemical features among these tumours, may raise serious differential diagnostic problems. Methods and Results: The data presented in the present paper have been retrieved from the entire published literature on the PubMed website about DAM, CAF, AFMB and MFB from 1984 to 2021. The selected articles are mainly represented by small-series, and, more rarely, single-case reports with unusual clinicopathologic features. The present review focuses on the diagnostic clues of the stromal tumours of the lower female genital tract to achieve a correct classification. The main clinicopathologic features of each single entity, emphasizing their differential diagnostic clues, are discussed and summarized in tables. Representative illustrations, including the unusual morphological variants, of each single tumour are also provided. Conclusion: Awareness by pathologists of the wide morphological and immunohistochemical spectrum exhibited by these tumours is crucial to achieve correct diagnoses and to avoid confusion with reactive conditions or other benign or malignant entities.

Aggressive Angiomyxoma of the Lower Female Genital Tract in Pregnancy: A Review of the MITO Rare Tumors Group

“…There is usually a prominent vascular component, characterized by thick-walled vessel [ 34 ]. Neoplastic cells exhibit a poorly defined, scant cytoplasm and round, hyperchromatic nuclei; mitoses are absent or rare [ 35 ]. By immunohistochemistry, neoplastic spindle cells stain positively for vimentin, desmin, and smooth muscle actin (SMA) [ 35 ].…”

“…Neoplastic cells exhibit a poorly defined, scant cytoplasm and round, hyperchromatic nuclei; mitoses are absent or rare [ 35 ]. By immunohistochemistry, neoplastic spindle cells stain positively for vimentin, desmin, and smooth muscle actin (SMA) [ 35 ]. Variable expression of CD34 is detected; S100 and cytokeratin markers are usually negative [ 9 ].…”

Rare Vulvar Angiomyofibroblastoma: Case Series and Literature Review

“…This recurrence is not invasive and easily cured by a second operation. 15 Only a few malignant cases were reported. A study of 50 cases demonstrated that all patients undergo complete resection of the tumor and are followed up from 3 months to 25 years after surgery, and only one case develops sarcoma 2 years after complete resection.…”

A tale of two vulvar angiomyxomas: Two cases and review of literature

“…Furthermore, Superficial and Aggressive angiomyxomas should be distinguished from other myxoid and vascular tumors, such as angiomyofibroblastoma, cellular angiofibroma, myxoid liposarcoma, myxofibrosarcoma, myxoid desmoid fibromatosis, and myxoid dermatofibrosarcoma protuberans (DFSP), among others. Although these tumors entities may present similarly, they can be diagnosed based on characteristic clinicopathologic, immunohistochemical, and molecular findings ( Angelico et al, 2022 , Baheti et al, 2015 , Ganeshan et al, 2019 , Mentzel et al, 2007 ). Similarly to Aggressive angiomyxoma, angiomyofibroblastoma and cellular angiofibroma also have a predilection to the lower female genital tract.…”