A rare case of inflammatory myofibroblastic tumor of the vulva in a newborn

Lenh, Bui-Van; Duc, Nguyen Minh; My, Thieu-Thi Tra; Van, Nguyen Dinh; Huong, Bui-Thi My; Hoang, Van Trung; Bang, Mai Tan Lien; Thong, Pham Minh

doi:10.1016/j.radcr.2021.03.020

Cited by 2 publications

(2 citation statements)

References 11 publications

(16 reference statements)

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“…The diagnosis was confirmed by ALK immunoexpression though molecular testing was not performed. Another case of vulvar IMT was reported in a newborn, but the morphology does not appear classic for IMT in the published figure, there was no myoid immunoexpression, ALK staining was not performed, and FISH was negative for ALK rearrangement (19). In the current case, the pathologic features were classic for IMT as described in the uterus (2,6,11).…”

Section: Discussionmentioning

confidence: 54%

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

Bowman

Medeiros

Fadare

et al. 2022

International Journal of Gynecological Pathology

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Gynecologic tract origin of inflammatory myofibroblastic tumor (IMT), a receptor tyrosine kinase fusion driven tumor with malignant potential, is uncommon and mostly involves the uterine corpus where misclassification as a smooth muscle tumor may occur due to overlapping morphologic features. With rare exception, uterine IMT involves ALK rearrangements and exhibits ALK immunoexpression. Molecularly confirmed vulvovaginal IMT has not been reported, but several low-grade mesenchymal tumors in this region exhibit myxoid stroma and/or inflammatory infiltrates that may resemble IMT. The aims of this study were to define the diagnostic specificity of ALK immunoexpression for IMT among a broad spectrum (107 cases) of vulvovaginal mesenchymal tumors in the differential diagnosis of IMT and to report the clinicopathologic features of vulvovaginal IMT identified in our archives or via retrospective ALK staining of otherwise classified vulvovaginal tumors. Review of archives from 5 different centers revealed a single case of vulvar IMT in a 62-yr-old woman. The 2.5 cm well-circumscribed tumor exhibited the typical microscopic features of IMT, namely a loose fascicular distribution of bland spindle cells within a myxoid stroma, accompanied by an infiltrate of plasma cells, lymphocytes, and eosinophils. The tumor cells exhibited expression for smooth muscle actin, desmin, h-caldesmon, and ALK. Break-apart fluorescence in situ hybridization confirmed the presence of ALK rearrangement. The patient did not receive any treatment and is alive without disease 32 mo later. No evidence of ALK expression was detected in any of the other 107 vulvovaginal tumors, which included 14 aggressive angiomyxomas, 2 superficial angiomyxomas, 12 angiomyofibroblastomas, 8 cellular angiofibromas, 15 smooth muscle neoplasms, 10 peripheral nerve sheath tumors, 20 fibroepithelial polyps, and a variety of other low grade mesenchymal tumors. Although vulvovaginal ALK-rearranged IMT is exceedingly rare, the behavior remains to be fully understood. ALK immunohistochemistry, which appears specific for IMT in this anatomic site, is advised in the evaluation of vulvovaginal mesenchymal tumors exhibiting myxoid stroma and/or an inflammatory infiltrate.

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Section: Discussionmentioning

confidence: 54%

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

Bowman

Medeiros

Fadare

et al. 2022

International Journal of Gynecological Pathology

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“…Our case was negative for ALK1, which is the key marker for IMT 13–15. Also, IMT has not been reported on the vulva outside of a neonatal case 16…”

Section: Differential Diagnosismentioning

confidence: 48%

Vulval superficial myofibroblastoma with lymphocytic and eosinophilic infiltrate

Tan,

Morgan,

Scurry

2023

BMJ Case Rep

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We present the case of a vulval superficial myofibroblastoma with a lymphocytic and eosinophilic rim in a woman in her late 20s. The tumour presented in pregnancy as a cystic lesion with pain and increasing size. While the histopathology of superficial myofibroblastomas has been well defined in the literature, to our knowledge, there has been no documentation of the presence of an inflammatory infiltrate of lymphocytes and eosinophils surrounding and within the tumour. This may potentially act as a diagnostic or prognostic reference.

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A rare case of inflammatory myofibroblastic tumor of the vulva in a newborn

Cited by 2 publications

References 11 publications

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms

Vulval superficial myofibroblastoma with lymphocytic and eosinophilic infiltrate

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