Dermatofibrosarcoma Infiltrating the Parotid Gland: Case Report and Literature Review

Case Report

Austin J Otolaryngol. 2015; 2(9): 1065.

Dermatofibrosarcoma Infiltrating the Parotid Gland: Case Report and Literature Review

De Liz AA*, Cardoso DB, Nery dos Passos Martins DA and Lunardi FA

Department of Otolaryngology and Head and Neck surgery, University of São Paulo, Brazil

*Corresponding author: Alcebiades Alves De Liz, Department of Otolaryngology and Head and Neck surgery, Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru–SP, Postal code: 17.012-900, Brazil

Received: October 30, 2015; Accepted: December 20, 2015; Published: December 24, 2015

Abstract

Introduction: Primary dermatofibrosarcoma protuberans (DFSP) of the salivary gland is extremely rare.

Case Presentation: A 37-year-old female complained of a 5-year, slowgrowing lump in the right parotid region without any other sign or symptom. At examination there was a firm, non-movable nodule on the right parotid gland. The ultrasound/Doppler showed a well-vascularized solid nodule with ill-defined borders on the inferior edge of the parotid. A total parotidectomy was performed. Sections revealed an ill-defined, non-encapsulated nodule measuring 2.5 x 2.0 x 1.3 cm. The histopathological diagnosis coupled with immunohistochemistry was consistent with DFSP.

Conclusion: We report a rare case of DFSP infiltrating the parotid gland. Whether it represents a primary lesion or a subcutaneous tumor infiltrating the parotid gland, this tumor presented clinically as a parotid lump and diffusely infiltrated this gland. Treatment modalities and prognosis are not well-defined due to the scarcity of cases described in the literature.

Introduction

Tumors of mesenchymal origin can occur in any part of the body but are rare in salivary glands [1]. Malignant mesenchymal tumors are seldom encountered in major salivary glands and soft tissue sarcomas account for only 0.3–1.5% of all salivary gland neoplasms [2]. Moreover, approximately 5% of all primary salivary gland tumors are of mesenchymal origin [3], but only 0.3–1.5% are malignant [4].

Dermatofibrosarcoma Protuberans (DFSP) was first described in 1924 by Darier and Ferrand as a ‘‘progressive and recurring dermatofibroma””, and is a low–grade malignant tumor that typically presents on the trunk and proximal extremities as a slow–growing, painless, firm, cutaneous plaque. However, unusual sites for DFSP, including the vulva and parotid gland, have been reported [5].

Primary DFSP of the salivary gland is extremely rare and to the best of our knowledge only 4 cases have been reported in the English literature [1].

Case Presentation

A 37-year-old female complained of a 5-year, slow-growing lump in the right parotid region without any other sign or symptom. Patient had no previous history of surgery or other relevant disease. At examination there was a firm, non-movable nodule on the right parotid gland with a slight skin retraction without inflammatory sings. The ultrasound/Doppler, bi-dimensional method, with a linear dynamic equipment (7.5 MHz) showed a well-vascularized solid nodule with ill-defined infiltrative borders at the inferior edge of the parotid measuring approximately 2.0 x 2,0 x 1,5 cm (Figure 1). No skin involvement was seen. A diagnosis of parotid tumor was performed and carcinoma was the main hypothesis. A total parotidectomy was performed.

Citation: De Liz AA, Cardoso DB, Nery dos Passos Martins DA and Lunardi FA. Dermatofibrosarcoma Infiltrating the Parotid Gland: Case Report and Literature Review. Austin J Otolaryngol. 2015; 2(9): 1065. ISSN :2473-0645