Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. DFSP frequently exhibits extensive local infiltration. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence. Although DFSP has a reported high rate of recurrence, metastasis is more uncommon. Fibrosarcomatous DFSP is an aggressive variant with an increased risk for local recurrence and metastasis. If achieving negative margins or resection is not feasible, radiation therapy or systemic treatment are options that may be considered by a multidisciplinary team. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline recommended treatment options available for DFSP.
NCCN Guidelines® Insights: Merkel Cell Carcinoma, Version 1.2024
The NCCN Guidelines for Merkel Cell Carcinoma (MCC) provide recommendations for diagnostic workup, clinical stage, and treatment options for patients. The panel meets annually to discuss updates to the guidelines based on comments from expert review from panel members, institutional review, as well as submissions from within NCCN and external organizations. These NCCN Guidelines Insights focus on the introduction of a new page for locally advanced disease in the setting of clinical node negative status, entitled “Clinical N0 Disease, Locally Advanced MCC.” This new algorithm page addresses locally advanced disease, and the panel clarifies the meaning behind the term “nonsurgical” by further defining locally advanced disease. In addition, the guideline includes the management of in-transit disease and updates to the systemic therapy options.
Basal Cell Skin Cancer, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology
Basal cell carcinoma (BCC) is the most common form of skin cancer in the United States. Due to the high frequency, BCC occurrences are not typically recorded, and annual rates of incidence can only be estimated. Current estimated rates are 2 million Americans affected annually, and this continues to rise. Exposure to radiation, from either sunlight or previous medical therapy, is a key player in BCC development. BCC is not as aggressive as other skin cancers because it is less likely to metastasize. However, surgery and radiation are prevalent treatment options, therefore disfigurement and limitation of function are significant considerations. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline an updated risk stratification and treatment options available for BCC.
NCCN Guidelines® Insights: Squamous Cell Skin Cancer, Version 1.2022
The NCCN Guidelines for Squamous Cell Skin Cancer provide recommendations for diagnostic workup, clinical stage, and treatment options for patients with cutaneous squamous cell carcinoma. The NCCN panel meets annually to discuss updates to the guidelines based on comments from panel members and the Institutional Review, as well as submissions from within NCCN and external organizations. These NCCN Guidelines Insights focus on the introduction of a new surgical recommendation terminology (peripheral and deep en face margin assessment), as well as recent updates on topical prophylaxis, immunotherapy for regional and metastatic disease, and radiation therapy.
Endoscopic spectrum and practical classification of small bowel gastrointestinal stromal tumors (GISTs) detected during double-balloon enteroscopy
Abstract Background and study aims Information about the endoscopic characterization of small bowel gastrointestinal tumors (GISTs) is limited. The aim of this case study was to describe the endoscopic spectrum of small bowel GISTs and to present a practical classification. Patients and methods Observational, retrospective, consecutive case series of patients with small bowel GIST. Results A total of 10 small bowel GISTs were found in patients (6 male, 4 female, mean age 52 years, range 28 to 68).). All patients presented with obscure gastrointestinal bleeding (overt, n = 8, occult, n = 2). Most GISTs were present in the proximal or middle small bowel (n = 7). The endoscopic tumor characteristics could be categorized as follows: submucosal round (n = 4), submucosal sessile (n = 2), and invasive/penetrating) (n = 4). The mucosa overlying the tumor was normal (n = 4), grooved (n = 3) or frankly ulcerated (n = 3). Tumor size ranged from 8 mm to 50 mm. Biopsy was negative in all patients with normal mucosa but showed tumor in all patients with ulcerations. Regardless of biopsy results, all patients were sent for surgery. Nine resections were carried out. One patient refused surgery. There were no complications of endoscopy in this cohort. Conclusion Our series shows that GISTs have a wider spectrum of endoscopic characteristics than previously described. The round type with normal overlying mucosa was equally prevalent as the grooved or ulcerated variant. Endoscopists should be aware of this wide spectrum of presentation of small bowel GIST.
Cutaneous Melanoma, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology
The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Cutaneous melanoma have been significantly revised over the past few years in response to emerging data on immune checkpoint inhibitor therapies and BRAF-targeted therapy. This article summarizes the data and rationale supporting extensive changes to the recommendations for systemic therapy as adjuvant treatment of resected disease and as treatment of unresectable or distant metastatic disease.