Abstract
Malignant fibrous histiocytoma (MFH) is the most common sarcoma found in adults. We discuss a case of inflammatory MFH of dermal/epidermal origin presenting with a severe leukemoid reaction (LR). A 60 years old white male presented to hematology/oncology clinic complaining of mild shortness of breath on exertion. Past medical history was remarkable for removal of a left upper extremity necrotic mass 4.4 × 3 × 3 cm. Microscopy of the specimen showed clear surgical margin, and tumor cells restricted to the dermis without lymphovascular invasion. Immunohistochemestry was positive for CD 68 and CD 99. Chest x-ray was negative for metastatic disease. White blood cell count was 109.4 k/mm3 with 24 k/mm3 band neutrophils, and absolute neutrophil count of 69 k/mm3. CT scan of the thorax revealed numerous bilateral pulmonary nodules suspicious for metastasis. Based on these findings patient was diagnosed with metastatic cutaneous IMFH associated with a LR. Following review of medical literature, this appears to be the first reported case of inflammatory cutaneous MFH associated with LR. This histological variant is rare, and carries a poor prognosis. Thus, we would like to emphasize the need for investigating alternative therapies capable of improving the survival of these patients.
Highlights
Malignant fibrous histiocytoma (MFH) known as undifferentiated pleomorphic sarcoma (UPS) is the most common soft tissue tumor of adults
We discuss a case of an aggressive inflammatory MFH of dermal/epidermal origin located on the left upper extremity presenting with a severe leukemoid reaction
It can virtually affect any part of the body including solid organs such as the thymus, spermatic cord, and ovary
Summary
Malignant fibrous histiocytoma (MFH) known as undifferentiated pleomorphic sarcoma (UPS) is the most common soft tissue tumor of adults. It was first described by Kauffman and Stout [1]. MFH arises from a mesenchymal cell progenitor; it can develop as a novo lesion or secondary to radiation, surgery, osteonecrosis, Paget’s disease, fibrous dysplasia, among others. It can affect virtually any part of the body, but it is most frequently found in the extremities, and up to 50% of them are located in the lower limbs. We discuss a case of an aggressive inflammatory MFH of dermal/epidermal origin located on the left upper extremity presenting with a severe leukemoid reaction
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