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Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad
This case talks about a 80-year-old man with a scalp tumor.
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1. Question
This is an 80 year old male with a scalp tumor. The neoplastic cells were negative for S100, HMB45, cytokeratins Cam5.2, AE1/3, and 34BE12, as well as CD34.
Diagnoses:
Correct
Answer: D
Histologic Description: This is a pleomorphic spindle cell neoplasm that involves the dermis but also extends into the subcutaneous tissue and skeletal muscle. In some sections, perineural and vascular invasion were identified. The lesion is arising in chronically sun damaged skin; however, there is no evidence of in situ or invasive melanoma or squamous cell carcinoma. These are the typical features of pleomorphic dermal sarcoma.
Differential Diagnosis: Atypical fibroxanthoma (AFX) is on the spectrum of pleomorphic dermal sarcoma; however, by definition it does not involve subcutaneous tissues, does not show lymphovascular invasion or perineural invasion, and does not show necrosis. AFX generally has a benign outcome, reflecting its extremely superficial nature. Squamous cell carcinomas should demonstrate in situ component or labeling for cytokeratins, while melanoma would demonstrate either in situ or invasive component, and should demonstrate some labeling for S100 or HMB45. Unlike AFX, pleomorphic dermal sarcoma has significant potential for metastatic spread. Patients who do develop metastases have poor outcome.
Incorrect
Answer: D
Histologic Description: This is a pleomorphic spindle cell neoplasm that involves the dermis but also extends into the subcutaneous tissue and skeletal muscle. In some sections, perineural and vascular invasion were identified. The lesion is arising in chronically sun damaged skin; however, there is no evidence of in situ or invasive melanoma or squamous cell carcinoma. These are the typical features of pleomorphic dermal sarcoma.
Differential Diagnosis: Atypical fibroxanthoma (AFX) is on the spectrum of pleomorphic dermal sarcoma; however, by definition it does not involve subcutaneous tissues, does not show lymphovascular invasion or perineural invasion, and does not show necrosis. AFX generally has a benign outcome, reflecting its extremely superficial nature. Squamous cell carcinomas should demonstrate in situ component or labeling for cytokeratins, while melanoma would demonstrate either in situ or invasive component, and should demonstrate some labeling for S100 or HMB45. Unlike AFX, pleomorphic dermal sarcoma has significant potential for metastatic spread. Patients who do develop metastases have poor outcome.
