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.