Spindle Cell Neoplasms Encountered in Dermatologic Surgery: A Review

Abstract

Cutaneous spindle cell tumors share the common feature of appearing as spindle-shaped cells on light microscopy. Their pathogenesis, presentation, and prognosis are highly variable, and numerous techniques for workup and treatment have been reported. We performed an analysis of the available scientific literature in order to codify the clinical, immunohistochemical, and biologic features of these tumors and to provide insight into the most effective practices for their management, with a focus on Mohs micrographic surgery (MMS). In this article, the clinical and histopathological characteristics of dermatofibrosarcoma protuberans, atypical fibroxanthoma, malignant fibrous histiocytoma, spindle cell squamous cell carcinoma, superficial leiomyosarcoma, desmoplastic melanoma, cutaneous angiosarcoma, and myofibrosarcoma are described, and methods for diagnosis, workup, treatment, and surveillance are evaluated. Cutaneous spindle cell neoplasms are diverse in origin, presentation, and behavior. Immunostaining assists in differentiating among the various types. Further workup is sometimes indicated to characterize local invasion or assess for metastatic disease. Surgery is typically the first-line treatment, and MMS is associated with low recurrence rates and a tissue-sparing advantage for many tumors. Adjuvant treatments, including radiation therapy, molecular-targeted therapy, and conventional chemotherapy, are sometimes indicated, and close clinical surveillance is required after treatment.

Dermatofibrosarcoma Protuberans Arising From a Keloid-like Benign Sclerotic Plaque

“…Monomorphic spindle shaped cells formed a lace-like pattern. Most but not all of DFSP specimens are strongly positive for CD34 [13,14,15]. Our patients lesion was also strongly positive for CD34.…”

“…When there is deeper involvement, tumor cells infitrate widely between collagen bundles of the deeper dermis and blend into the normal dermis that is described as a "storiform" pattern. Subcutis is extensively infiltrated and replaced in a typical lace-like pattern [3,13]. Usually there is low mitosis [2].…”

Recurrent cutaneous angiosarcoma of the ear masquerading as atypical fibroxanthoma

“…Immunohistochemical studies are necessary to distinguish between these tumors 8 . Stains for S-100 and cytokeratins are negative for angiosarcoma and distinguish this tumor from a spindled cell melanoma and squamous cell, respectively.…”

Electronic brachytherapy management of atypical fibroxanthoma: report of 8 lesions

“…Dermatologic surgery has traditionally been the first line of therapy for AFX, with a local post-operative recurrence rate of 5-25% [1, 2, 3, 4, 5, 6, 7]. Recurrences present rapidly, 7.3 to 24 months post-operatively [2, 8, 9].…”