Cutaneous syncytial myoepithelioma with folliculocentric growth and <i>EWSR1</i> gene rearrangement: A case report

Nagayasu, Mayumi Akaki; Tanaka, Hiroyuki; Nogami, Keiji; Kukita, Yoji; Motoi, Toru; Manabe, Toshiaki; Kataoka, Hiroaki

doi:10.1111/cup.14419

Cited by 2 publications

(2 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…CSM constitutes a relatively rare diagnostic entity, with roughly 80 documented cases in the medical literature7–10…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Syncytial Myoepithelioma: An Uncommon and Distinct Variant of Cutaneous Epithelioid Neoplasm

Shaker,

Phelps,

Niedt

et al. 2024

The American Journal of Dermatopathology

View full text Add to dashboard Cite

Background: Cutaneous syncytial myoepithelioma (CSM) is an uncommon and distinct variant of cutaneous myoepithelioma. We aim to present a case of CSM to enhance the recognition of this unique variant, encompassing its clinical characteristics, histopathological features, immunohistochemical staining, and therapeutic approaches. Case presentation: A 10-year-old girl presented with a dome-shaped nodule located on the skin of her left medial distal arm. Microscopic examination of the skin biopsy revealed a well-defined dermal nodular lesion, surrounded by an epidermal collarette. Tumor cells were composed of epithelioid to spindle-shaped cells with round-to-oval nuclei, small nucleoli, and abundant eosinophilic cytoplasm with a syncytial-like growth pattern. A moderate degree of nuclear pleomorphism was noted. Mitotic activity was not prominent. Immunohistochemical staining revealed positive staining for epithelial membrane antigen, GLUT1, collagen IV, and S100. Smooth muscle actin, CD10, and CD68 showed patchy positivity. CD31, CD34, p63, SOX10, anaplastic lymphoma kinase (ALK), glial fibrillary acidic protein, pankeratin (AE1/AE3/PCK26), Melan-A, and CD1a were negative. Fluorescence in situ hybridization targeting TFE3 and ALK genes was negative. The differential diagnosis included ALK-negative epithelioid cell histiocytoma, epithelioid perineurioma, and CSM. Based on the above findings, a diagnosis of CSM was rendered. Discussion: CSM is a benign cutaneous neoplasm composed of sheets of histiocytoid or short spindle cells with pale eosinophilic cytoplasm with a syncytial-like growth pattern. Clinically, CSM often presents as a painless, slow-growing nodule or plaque in a broad anatomical distribution with a preference for the distal extremities.. CSM is characteristically positive for epithelial membrane antigen (EMA) and S100 protein and negative for keratins. In challenging cases, molecular testing for EWSR1 gene rearrangement and EWSR1-PBX3 gene fusion aid in confirming the diagnosis. Conclusions: The histologic features of CSM present a unique set of challenges posing a diagnostic dilemma, as they can bear resemblance to a range of benign and malignant cutaneous neoplasms including ALK-negative epithelioid cell histiocytoma, epithelioid perineurioma, malignant or nevoid melanoma, and epithelioid sarcoma. An accurate diagnosis is crucial for guiding proper clinical management considering that this entity typically demonstrates an excellent prognosis following a complete surgical excision.

show abstract

“…CSM constitutes a relatively rare diagnostic entity, with roughly 80 documented cases in the medical literature7–10…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Syncytial Myoepithelioma: An Uncommon and Distinct Variant of Cutaneous Epithelioid Neoplasm

Shaker,

Phelps,

Niedt

et al. 2024

The American Journal of Dermatopathology

View full text Add to dashboard Cite

show abstract

“…Immunohistochemically, CSM is characterized by the co-expression of smooth muscle and epithelial markers. From the literature, it can be seen that it is frequently positive for EMA (100%), S-100 protein (87%), and AE1/AE3 (82%), and to a lesser degree for SMA (69%), p63 (55%), and GFAP (37%) [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Syncytial Myoepithelioma with Positive CD34 Immunohistochemical Staining: An Unusual Tumor and a Challenging Diagnosis

Ariasi,

Romanò,

Ghini

et al. 2023

Dermatopathology

View full text Add to dashboard Cite

Cutaneous syncytial myoepithelioma (CSM) is a rare type of cutaneous neoplasm that typically presents as a solitary and well-circumscribed nodule on the skin. It predominantly occurs on the upper and lower extremities of adult patients. Immunohistochemically, CSM is characterized by the co-expression of smooth muscle and epithelial markers. Fluorescence in situ hybridization (FISH) targeting the EWSR1 gene rearrangement is an important diagnostic tool for CSM. In our case report, we found the focal positivity for CD34, which has never been previously observed; this was mostly confined to a central area of the neoplasm.

show abstract

Cutaneous syncytial myoepithelioma with folliculocentric growth and EWSR1 gene rearrangement: A case report

Cited by 2 publications

References 14 publications

Cutaneous Syncytial Myoepithelioma: An Uncommon and Distinct Variant of Cutaneous Epithelioid Neoplasm

Cutaneous Syncytial Myoepithelioma: An Uncommon and Distinct Variant of Cutaneous Epithelioid Neoplasm

Cutaneous Syncytial Myoepithelioma with Positive CD34 Immunohistochemical Staining: An Unusual Tumor and a Challenging Diagnosis

Contact Info

Product

Resources

About