Dermatology: Practical and Conceptual 44 Observation | Dermatol Pract Concept 2017;7(3):9 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Introduction Malignant fibrous histiocytoma (MFH), currently classified as undifferentiated pleomorphic sarcoma, is the most frequent soft tissue sarcoma in adulthood, but it is not as common as a primary skin tumor [1]. The age of presentation ranges between 50 and 70 years, two-thirds occur among men, and the Caucasian population is more commonly affected. MFH affects mostly the thighs and trunk. Infrequently, it presents in the head and neck in adults [2,3]. The lesions are usually diagnosed in advanced stages, and despite currently proposed therapies such as radiotherapy or chemotherapy, the patient’s prognosis is usually poor with a tendency to local recurrence and systemic metastasis [1]. Early recognition is crucial to improve clinical outcome. Dermoscopy has shown to be use- ful in the assessment of both melanoma and non-melanoma skin tumors [4]. Case Report A 75-year-old male with history of multiple basal cell carci- nomas and actinic keratosis presented for a biannual routine skin examination. In the left cheek a hypopigmented lesion was detected. It was discretely erythematous, with poorly defined limit, and increased consistency on palpation (Figure 1). The lesion was not present at the time of his previous visit and the patient was not aware of the lesion and did not know the evolution. No previous history of radiotherapy Dermoscopic findings in an early malignant fibrous histiocytoma on the face Gabriel Salerni1,2, Carlos Alonso1,2, German Sanchez-Granel2, Mario Gorosito1,3 1 Dermatology Department, Hospital Provincial del Centenario de Rosario, Argentina 2 Diagnóstico Medico Oroño, Rosario, Argentina 3 Dermatopathology Department, Hospital Provincial del Centenario de Rosario-Universidad Nacional de Rosario, Argentina Key words: dermoscopy, skin cancer Citation: Salerni G, Alonso C, Sanchez-Granel G, Gorosito M. Dermoscopic findings in an early malignant fibrous histiocytoma on the face. Dermatol Pract Concept 2017;7(3):9. DOI: https://doi.org/10.5826/dpc.0703a09 Received: February 14, 2017; Accepted: April 11, 2017; Published: July 31, 2017 Copyright: ©2017 Salerni et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Gabriel Salerni, MD, v Oroño 1515, Rosario, Argentina. Tel. +54.341.5222222. Email: gabrielsalerni@hotmail.com Malignant fibrous histiocytoma (MFH), currently classified as undifferentiated pleomorphic sarcoma, is the most frequent soft tissue sarcoma in adulthood, but it is not as common as a primary skin tumor. MFH affects mostly the thighs and trunk, head and neck is an infrequent presentation in adults. MFH is often diagnosed in advanced stages, with a tendency to local recurrence and systemic metastasis. Since tumor thickness and size are identified as major prognostic factors, early recognition becomes crucial to improve prognosis. We present a case of a cutaneous malignant fibrous histiocytoma located on the face in which dermoscopy was useful in clinical management and definition. ABSTRACT Observation | Dermatol Pract Concept 2017;7(3):9 45 irregular cells, some giant, with large and hyperchromatic nuclei and high mitotic index, which adopted a storiform arrangement, intermixed with thick collagen bundles (Figure 3A and B). In the immunohistochemical study, the cytokera- tins and the HMB45 were negative, whereas the CD68 was marked strongly positive (Figure 3C, D and E). Discussion Malignant fibrohistiocytic tumors include various soft tissue sarcomas that show a spectrum of peculiar clinical-patho- or other procedure was reported. There were no regional lymphadenopathies. Dermoscopy showed a shiny white-red structureless area with crystalline structures such as short white streaks and rosettes, the latter term used to designate four closely aggregated white, small dots in correspondence to a follicular opening arranged in a rhombus [5]. Focal superficial linear telangiectasias were also noted. No specific criteria for mela- nocytic lesion were observed (Figure 2). Excisional biopsy was performed. Histopathology reported acanthosis, hyperkeratosis and marked cellular vacuolization. The dermis was totally invaded by a neofor- mation formed by a large number of intensely pleomorphic, Figure 1. Clinical image. Hypopigmented lesion with discrete ery- thema and poorly defined limits. [Copyright: ©2017 Salerni et al.] Figure 2. Dermoscopy image. A white-red structureless area with crystalline structures (short white streaks and rosettes) was observed. Focal superficial linear telangiectasias were also noted. [Copyright: ©2017 Salerni et al.] Figure 3. Non-ulcerated, mitotically active dermal fibrohistiocytic proliferation with infiltrative pattern; CD 68 positive, cytokeratin and HMB45 negative. [Copyright: ©2017 Salerni et al.] 46 Observation | Dermatol Pract Concept 2017;7(3):9 The dermoscopic findings were not conclusive: the pres- ence of erythema and a shiny white-red structureless area along with short white streaks and rosettes primarily sug- gested a squamous tumor and less likely a basal cell carci- noma. The findings were not specific for melanocytic lesion or non-melanocytic lesion, so according to the two-step algorithm, the diagnosis of melanoma cannot be ruled out and biopsy is mandatory. Tumor size and depth have been identified as the main prognostic factors [8]. Therefore, establishing an accurate and early diagnosis is of crucial in improving the tumor prognosis. To our knowledge, this is the first description of the dermoscopic aspect of MFH. Dermoscopy was helpful in defining clinical management allowing for the early recogni- tion of this tumor in an unusual presentation. MFH should be included in the differential diagnosis when assessing non- pigmented lesions with dermoscopy. References 1. Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: an analy- sis of 200 cases. Cancer. 1978;41:2250-2266. 2. Gibbs J, Huang P, Lee R, McGrath B, et al. Malignant fibrous his- tiocytoma: an institutional review. Cancer Invest. 2001;19:23-27. 3. Sureda N, Bosch M, Valente E, Kurpis M, Ruiz A. Malignant Fibrohistiocytoma: cephalic location as infrequent presentation. Arch Argent Dermatol. 2008;58:55-59. 4. Zalaudek I, Kreusch J, Giacomel J, Ferrara G, Catricalà C, Argen- ziano G. How to diagnose nonpigmented skin tumors: a review of vascular structures seen with dermoscopy: part II. Nonmelanocytic skin tumors. J Am Acad Dermatol. 2010;63(3):377-386. 5. Cuellar F, Vilalta A, Puig S, Palou J, Salerni G, Malvehy J. New dermoscopic pattern in actinic keratosis and related conditions. Arch Dermatol. 2009;145(6):732. 6. O’Brien JE, Stout AP. Malignant Fibrous Xanthomas. Cancer. 1964; 17: 1445-55. 7. Weiss SW, Goldblum JR. Malignant fibrohistiocytic tumors. In: Gibson LE, ed. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St. Louis: CV Mosby; 2001:535–569. 8. Pezzi CM, Rawlings MS, Esgro JJ, Pollock RE, Romsdahl MM. Prognostic factors in 227 patients with malignant fibrous histio- cytoma. Cancer. 1992;69:2098-2103. logical findings with distinctive biological behavior. These tumors are grouped into three subtypes: atypical fibroxan- thoma, dermatofibrosarcoma protuberans and malignant fibrohistiocytoma. Malignant fibrous histiocytoma (MFH) is a high-grade sarcoma that was first described by O’Brien and Stout in 1964 [6] and is currently classified as undif- ferentiated pleomorphic sarcoma. The term MFH is used in the medical literature to describe a high-grade pleomorphic sarcoma that affects adults. It is the sarcoma most frequently present in this age group [1]. MFH originates from pluripotent mesenchymal cells with the ability to differentiate into histiocytes, fibroblasts and myofibroblasts of the muscle or muscle fascia. The etiology of these tumors is unknown, although, as in all sarcomas, previous radiotherapy in the area may induce its occurrence. There are five histological subtypes: storiform-pleomorphic, myxoid, giant cell, inflammatory and angiomatoid. The pleomorphic storiform pattern is the most common, found in two-thirds of cases. In this variety, a large pleomorphic cell population with high nuclear atypia and mitosis is observed together with cells with fusiform morphology that adopt a “spoke wheel” or storiform pattern [7]. MFH usually affects the proximal limbs, although it has been described in the cephalic location. It can present as a cutaneous lesion in the form of a primary tumor or as metas- tasis from MFH at other sites. The pathogenesis is undefined, although MFH has been associated with physical, chemical and viral factors. Approximately two-thirds of the tumors are located within the skeletal muscle, with fewer than 10% confined to the subcutis [7]. It usually presents as a slow-growing tumor- ous lesion, though there is no clinically characteristic presen- tation that allows for differentiation from other sarcomas. The definitive diagnosis of MFH relies on histologi- cal studies, while immunohistochemistry helps establish the differential diagnosis with other entities with similar morphological patterns, such as pleomorphic varieties of rhabdomyosarcoma, leiomyosarcoma, liposarcoma, der- matofibrosarcoma protuberans, melanoma and atypical fibroxanthoma.