Atypical fibroxanthoma of the scalp with recurrent and multiple regional cutaneous metastases

Nergard, Jennifer; Glener, Julie; Reimer, Danielle; Greenwald, Jeffrey

doi:10.1016/j.jdcr.2016.09.016

Cited by 8 publications

(2 citation statements)

References 10 publications

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“…In spite of virtually identical histological and immunohistochemical findings, AFX and UPS differ clinically. AFX in the skin is characterized by a benign clinical course and extremely rare metastasis, although such cases were reported, 16 while UPS has a more aggressive clinical course and a higher frequency of metastasis. 5 Those tumors also show difference in their genetic alteration that might contribute to their different biological behavior.…”

Section: Discussionmentioning

confidence: 99%

Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

et al. 2021

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Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. The differential diagnosis, histological pitfalls of this poorly characterized mesenchymal lesions, and the challenges associated with treatment are discussed.

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Section: Discussionmentioning

confidence: 99%

Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

et al. 2021

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“…While S100, HMB45, MART-1, cytokeratins, CD34, CD31, desmin and h-caldesmon are absent in AFX/PDS. CD10 (or the common acute lymphoblastic leukaemia antigen, CALLA) is considered a useful marker for AFX (positive in 95-100% of cases), but also 1/3 of malignant melanomas, about half cases of squamous cell carcinomas and half cases of leiomyosarcomas are positive 3 , 6 , 21 , 22 . CD99 is a glycoprotein, indicative of myofibroblastic differentiation with a positive rate in AFX between 35% and 73% 19 , 20 .…”

Section: Discussionmentioning

confidence: 99%

Atypical fibroxanthoma/pleomorphic dermal sarcoma of the scalp with aberrant expression of HMB-45: a pitfall in dermatopathology

et al. 2020

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Summary Atypical fibroxanthoma (AFX) has been considered as the non-infiltrating precursor lesion of pleomorphic dermal sarcoma (PDS), which shows an aggressive clinical behavior, because of its extensive invasion of the deeper skin layers. Although these two tumors may represent two stages of the same disease, it can be difficult to differentiate between them, because of their similar clinical and histological features 1 . Furthermore, they must be distinguished from a spindled variant of squamous carcinoma, melanoma and leiomyosarcoma 2 . AFX/PDS still remains a diagnosis of exclusion, that needs to combine immunohistochemical markers for a definitive diagnosis. Usually AFX/PDS shows positivity for CD10, CD99, CD68, vimentin and lysozyme, while S100, HMB45, MART-1, cytokeratins, CD34, CD31, desmin and h-caldesmon are absent. We report a case of 89-year-old male, with a history of squamous cell carcinoma removed from the right ear, presented to our department with a recently growing, ulcerated and bleeding 2 cm nodule on the scalp. After surgery the tumor recurred with infiltration to the cranial theca. The final histological diagnosis was “pleomorphic dermal sarcoma” (PDS), which showed an unexpected positivity for HMB45. We present, to the best of our knowledge, the first case of AFX/PDS with an aberrant diffuse expression of HMB45 and an aggressive biological behavior, that leads us to a difficult exclusion diagnosis.

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Systematic Review and Case Series of Pleomorphic Dermal Sarcoma: an Uncommon Cutaneous Pathology Predominantly in the Head and Neck

Shinn

Elder

et al. 2022

SN Compr. Clin. Med.

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Atypical fibroxanthoma of the scalp with recurrent and multiple regional cutaneous metastases

Cited by 8 publications

References 10 publications

Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

Atypical fibroxanthoma/pleomorphic dermal sarcoma of the scalp with aberrant expression of HMB-45: a pitfall in dermatopathology

Systematic Review and Case Series of Pleomorphic Dermal Sarcoma: an Uncommon Cutaneous Pathology Predominantly in the Head and Neck

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