Roopali Mahajan
| Roopali Mahajan |
Roopali Mahajan1, Thomas P Sollecito1, Eric T Stoopler1, Takako I Tanaka1, Faizan Alawi2
1Oral Medicine, University of Pennsylvania School of Dental Medicine; 2Basic and Translational Sciences, University of Pennsylvania School of Dental Medicine
Introduction
Mycosis fungoides (MF) is a rare cutaneous T-cell lymphoma (CTCL). Involvement of the oral cavity is rare and associated with a poor prognosis. Tongue and palate are the most common sites of oral involvement. We present a well-documented case of oral T-cell Non-Hodgkin’s Lymphoma (NHL) presenting as extensive necrotic palatal ulcerations.
Methods
A 72-year-old male presented with a painful palatal ulceration of 3 weeks duration unresponsive to Amoxicillin/Clavunate potassium. The patient denied fever, trauma, bleeding or suppurative discharge, but endorsed dysphagia and pain. Past medical history included CTCL, hypertension, hyperlipidemia, diabetes mellitus, gastroesophageal reflux disease and arthritis. Family history was positive for a sister living with NHL. Social history revealed 50 pack/year cigarette use. Extraoral examination was unremarkable. Intraoral examination revealed necrotic, pseudomembranous ulceration with irregular, nonindurated borders involving hard and soft palate. Maxillofacial Computerized Tomography scan demonstrated an ovoid nodular lesion in the palate without extension into the maxillary sinus. Histopathological examination revealed a diffuse infiltrate of atypical lymphocytes with marked epidermotropism. Immunohistochemical stain revealed the atypical lymphocytes were CD3 positive confirming a T-cell lineage, with predominantly CD8 positive cells. Rare and scattered CD4 T-cells, CD30 positive cells and CD20 positive B cells were also identified. These findings were consistent with a T- cell lymphoma (TCL). Fluorodeoxyglucose (FDG) Positron Emission Tomography PET-CT showed intense FDG avidity in the hard palate highly suggestive of a malignant process without lymph node or visceral organ involvement. The patient was treated with Romidepsin and Brentuximab vedotin with significant improvement. Within 1-month, new ulcerations on upper edentulous alveolar ridge and lower gingiva, labial mucosa and vestibule. Repeat oral biopsy confirmed the diagnosis of TCL. Repeat PET-CT showed uptake in the soft palate/tonsillar region, brain, and spinal cord, suggesting widespread dissemination of T-cell lymphoma.
Conclusion
Patients with MF in oral cavity can often prove challenging to diagnose and manage. A careful history and examination supported by histologic and immunohistochemical analysis is crucial for accurate diagnosis and therapy. This case report is unique as the appearance of new oral lesions was the first sign of systemic progression of disease.