Clinical, pathologic, and molecular analyses of superficial low‐grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy

Low‐grade fibromyxoid sarcoma (LGFMS) is a histopathologically deceptive soft tissue neoplasm with bland cytology, which is typically encountered in deep soft tissue of adults. We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to dia...

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Veröffentlicht in:Journal of cutaneous pathology 2022-07, Vol.49 (7), p.638-644
Hauptverfasser: Modi, Mitul B., Pei, Susan, Hedberg, Matthew L., Fischer, Andrew S., Guerrero, Jessenia, Carreon, Chrystalle Katte, Cooper, Kumarasen, Seykora, John T.
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container_end_page 644
container_issue 7
container_start_page 638
container_title Journal of cutaneous pathology
container_volume 49
creator Modi, Mitul B.
Pei, Susan
Hedberg, Matthew L.
Fischer, Andrew S.
Guerrero, Jessenia
Carreon, Chrystalle Katte
Cooper, Kumarasen
Seykora, John T.
description Low‐grade fibromyxoid sarcoma (LGFMS) is a histopathologically deceptive soft tissue neoplasm with bland cytology, which is typically encountered in deep soft tissue of adults. We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to diagnose on histopathologic and clinical findings alone. LGFMS commonly mimics benign neoplasms such as cellular neurothekeoma, fibromatosis, neurofibroma, and perineurioma. Malignancies included in the differential diagnosis are soft tissue neoplasms such as dermatofibrosarcoma protuberans and myxofibrosarcoma. A high degree of reported variation in pattern and cellularity among LGFMS further complicates the diagnosis. Careful examination and appropriate immunohistochemistry panels including MUC4 are essential for narrowing the differential diagnosis. Molecular studies for possible FUS translocation can confirm the diagnosis of LGFMS. Sufficient sampling and workup of these lesions are critical, especially in younger patients. Young age and superficial presentation can easily sway dermatopathologists/dermatologists toward an incorrect diagnosis of benignancy.
doi_str_mv 10.1111/cup.14215
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We report two cases of superficial LGFMS in young patients (16 and 21 years old, respectively), which were difficult to diagnose on histopathologic and clinical findings alone. LGFMS commonly mimics benign neoplasms such as cellular neurothekeoma, fibromatosis, neurofibroma, and perineurioma. Malignancies included in the differential diagnosis are soft tissue neoplasms such as dermatofibrosarcoma protuberans and myxofibrosarcoma. A high degree of reported variation in pattern and cellularity among LGFMS further complicates the diagnosis. Careful examination and appropriate immunohistochemistry panels including MUC4 are essential for narrowing the differential diagnosis. Molecular studies for possible FUS translocation can confirm the diagnosis of LGFMS. Sufficient sampling and workup of these lesions are critical, especially in younger patients. 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source MEDLINE; Wiley Online Library Journals Frontfile Complete
subjects Adolescent
Adult
Cytology
Dermatofibrosarcoma protuberans
Differential diagnosis
Fibroma - diagnosis
Fibroma - pathology
Fibrosarcoma - diagnosis
Fibrosarcoma - pathology
FUS translocation
Humans
Immunohistochemistry
MUC4
Neoplasia
Nerve Sheath Neoplasms
Patients
pediatric
Sarcoma
soft tissue neoplasm
Soft Tissue Neoplasms - pathology
superficial low‐grade fibromyxoid sarcoma
Young Adult
title Clinical, pathologic, and molecular analyses of superficial low‐grade fibromyxoid sarcoma in two young patients: A rare and deceptive mimic of benignancy
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