Painful Cutaneous Syncytial Myoepithelioma: From Nonspecific Symptoms to Histopathologic Diagnosis

Guillén-Climent, Santiago; Vázquez, B.; Pinazo, María Isabel; Monteagudo, Carlos

doi:10.1016/j.adengl.2020.01.011

Cited by 2 publications

(3 citation statements)

References 7 publications

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“…Histologically, cutaneous myoepithelioma presents as well-circumscribed dermal lesions, occasionally extending into the superficial subcutis with no connection to the upper epidermis. There are two main architectural patterns: a well-circumscribed solid proliferation of a syncytial arrangement of spindle-shaped or epithelioid cells with abundant eosinophilic syncytial cytoplasm and minimal stroma 11 and a reticular architecture with epithelioid or spindle-shaped cells with mild pleomorphism embedded in myxoid or hyalinized stroma. 12 Mitotic activity within these tumors may serve as an indicator of a more aggressive behavior.…”

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

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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.

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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

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“…To the best of our knowledge, less than 80 cases have been previously documented in the literature, making this tumor a very rare entity [ 2 , 3 , 4 , 5 , 6 , 7 ]. CSM from a clinical perspective does not display any particular feature; therefore, histological examination is essential for establishing a diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Histologically, DF manifests as a dermal nodule composed of epithelioid binucleated cells within a fibrovascular stroma; but, unlike CSM, this lesion lacks syncytial architecture [ 5 , 15 ]. ES typically exhibits a combination of epithelioid and spindle cells with cellular atypia and infiltrative growth, often accompanied by satellite nodules [ 6 ]. In the early stage, JXG presents as an exophytic lesion commonly found in children that is characterized by the presence of mononuclear or multinuclear lipidized cells.…”

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

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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.

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Painful Cutaneous Syncytial Myoepithelioma: From Nonspecific Symptoms to Histopathologic Diagnosis

Cited by 2 publications

References 7 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

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