Case presentation:
A 34-year-old man was admitted to the Maxillofacial Surgery department, for evaluation of facial asymmetry due to a painful large lesion over the right cheek which was first noticed by the patient 12 months ago.
The lesion started as a small painful lesion in the right buccal mucosa which progressed gradually and extended to the nasal cavity.
Some local anti-inflammatory and antibiotic treatments which were previously prescribed by general practitioners did not bring the expected therapeutic effect.
The patient denied any B symptoms (fever, weight loss, and night sweats).
His past medical history was unremarkable except opium ingestion a few years ago which through epidemiologic data on that time the oral opiums in Iran were mostly contaminated with lead.
The local examination revealed a firm subcutaneous mass on the right side of the face which was non-mobile and tender on palpation.
Intraoral examination showed an ulceroproliferative lesion in the right buccal mucosa which had an ill-defined, indurated margin and was tender on palpation and also covered with whitish pseudomembrane.
On neck examination, few cervical lymph nodes also were palpable on both sides of the neck.
His blood investigations showed white blood cell (WBC) count 3700/µl (neutrophils 87.0% ,lymph 10.0 %); hemoglobin (Hgb) 14.5 g/dl; platelet count (Plt) 1.96 × 104/µl, (ESR) 47  mm/h , lactate dehydrogenase (LDH) 420 IU/l (normal range 150–500 U/L)
The patient underwent computed tomography (CT) scan of the oral cavity and neck which revealed
Destructive and expansive soft tissue density with central necrosis in right maxillary sinus extending to the nasal cavity and right ethmoid sinus with the destruction of the lateral wall of maxillary sinus and extension to cheek and buccal area, secretions were also seen in the right sphenoid sinus.
No intraorbital invasion was seen, bilateral mildly enlarged cervical lymph nodes were seen. (figure1)
Due to characteristic features of the imaging and clinical history of the patient’s deep cheek (buccal mucosa), a biopsy was performed, and histopathologic and IHC findings were in favor of a rare diagnosis of Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). (figure 2 )
Immunohistochemical results were as following: LCA+, CD3+, CD7+, CD5 -, CD20 -,CD30- and Ki67 was around 70–80%. (figure 2)
A whole-body bone and CT scan were performed for staging, and imaging findings did not reveal mediastinal or retroperitoneal lymphadenopathy or visceral involvement.
Bone marrow biopsy and cerebrospinal fluid cytology were negative for malignant cells.
Using the Ann Arbor staging, the patient was staged at the I EA level of the disease.
The patient received CHOEP (cyclophosphamide, adriamycin, vincristine, etoposide,
prednisone) following radiotherapy.