Claire L. Haycox

Immunohistochemical characterization of dermatofibrosarcoma protuberans with practical applications for diagnosis and treatment

Dermatofibrosarcoma protuberans (DFSP) is a rare, clinically challenging, soft tissue tumor. The main histologic differential of DFSP is usually a dermatofibroma. In 1990, the first report appeared demonstrating that cells of DFSP express the human progenitor antigen CD34 on their surface. Since then, there have been increasing reports of the usefulness of immunohistochemical staining with CD34 to differentiate DFSP from dermatofibroma and other soft tissue tumors. This literature is reviewed with special emphasis on the insights studies have provided into the histogenesis of DFSP. The literature demonstrating the practical applications of CD34 staining in the diagnosis and treatment of DFSP is also discussed.

Dermatofibrosarcoma protuberans (DFSP) : Growth characteristics based on tumor modeling and a review of cases treated with Mohs micrographic surgery

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare cutaneous tumor that is clinically challenging since it is locally highly invasive and aggressive, although it rarely metastasizes. Traditionally, wide and deep local surgical excision has been regarded as the treatment of choice for DFSP. However, even 3-cm-wide local excision margins have resulted in a local recurrence rate of 11%. In recent years, reports of DFSPs successfully treated with Mohs micrographic surgery have been appearing in the literature. The DFSP literature is reviewed here, including worldwide experience reported to date in the use of Mohs surgery to excise DFSPs. Using this margin control technique, the recurrence rate is shown to be 2.4%, much lower than the best previously reported recurrence rate of 11% when wide local excision was used. Three-dimensional reconstructions of DFSPs, based on Mohs micrographic surgical technique, are also presented. These illustrations provide new insight into the growth characteristics of these tumors and why Mohs micrographic surgery is emerging as the treatment of choice for DFSP.

Subcutaneous T-cell lymphoma treated with systemic chemotherapy, autologous stem cell support, and limb amputation

Subcutaneous T-cell lymphoma is an unusual variant of peripheral T-cell lymphoma in which the malignant infiltrate preferentially involves the subcutis. The disease is often initially misdiagnosed as a benign inflammatory panniculitis or a granulomatous disease. We describe subcutaneous T-cell lymphoma in a 39-year-old man who was treated with systemic chemotherapy, autologous stem cell support, and amputation of the limb primarily involved with the lymphomatous infiltrate. This is the first report of amputation being included in the treatment regimen of subcutaneous T-cell lymphoma. Because preferential involvement of the extremities often occurs in patients with subcutaneous T-cell lymphoma, surgical debulking of refractory disease by partial or complete limb amputation may be a useful therapeutic adjunct.

Primary Disseminated Varicella Presenting as an Acute Abdomen

We report a patient admitted with acute abdominal pain initially thought to be due to pancreatitis of unclear etiology. Later during his hospitalization he was diagnosed with primary varicella infection. The association between varicella and systemic multiorgan disease needs to be recognized in both immunocompetent and immunocompromised patients. A prompt diagnosis prevents delay in the treatment of varicella, as well as in monitoring for and preventing complications of disseminated infection.