PMC4848873_01
CT of neck demonstrating bilateral diffuse lymphadenopathy with the largest lymph node measuring 2.4 cm (arrow)
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Fig1
Computed tomography (CT) of neck, chest, abdomen, and pelvis demonstrated bilateral diffuse lymphadenopathy in the neck with the largest lymph node measuring 2.4 cm (Fig. 1), but no adenopathy in the hilum, mediastinum, axilla, abdomen, retroperitoneum, pelvis, or inguinal region. Pathologic findings are demonstrated in Fig. 2 and Table 1.
Biopsy of right incisural ulcer and left palatal perimolar ulcer. Hematoxylin and eosin (H&E) stain demonstrating scattered large atypical lymphocytes with occasional Hodgkin Reed-Sternberg (HRS) morphology in a background of mixed inflammatory infiltrate. These large atypical cells are positive for CD30
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Fig2
Pathologic findings are demonstrated in Fig. 2 and Table 1.
CT of neck demonstrating prominent maxillary gingival and palatal swelling and ulcerations with significant loss of maxillary teeth on the left side
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Fig3
Repeat CT (Fig. 3) demonstrated irregular enhancing soft tissue deep to the left nasolabial region and anterior premaxilla, encroaching on the left nasal vestibule.
a Left sided facial swelling causing lip droop and associated drooling. b , c Left palatal EBVMCU with associated maxillary teeth loss. d EBVMCU along right upper inner gum line extending from incisors to molars. e Hypertrophy of right upper gum line
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Fig4
On clinical exam, there was obvious left facial swelling along the left medial cheek and premaxillary region extending into the lip (Fig. 4a). An ulcerative indurated mass was visible eroding the left maxilla and palate (Fig. 4b, c). A second ulcer was apparent on the right upper inner gum line extending from the incisors to molars (Fig. 4d) with gum hypertrophy (Fig. 4e) and induration of the right buccal mucosa extending about halfway back.
Hematoxylin and eosin (H&E) stain of left palatal ulcer demonstrating a heterogeneous admixture of large atypical lymphocytes with occasional Hodgkin Reed-Sternberg (HRS) morphology, small lymphocytes, and histiocytes. HRS cells express CD30, PAX5, and EBER1 consistent with EBV-infected B-lymphocytes
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Fig5
Large atypical lymphocytes were uniformly positive for CD30, PAX5, MUM1, EBER, EBV LMP (Fig. 5), and negative for CD3, CD10, and CD20 (in the setting of recent rituximab exposure).
Axial and sagittal views of radiotherapy plan
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Fig6
Our patient received external beam radiation therapy using a three-dimensional conformal plan and a three-field approach (opposed lateral and anteroposterior fields; Fig. 6).
Post-radiation treatment response with resolution of the ulcers along right inner gingiva and left palate. Facial swelling has also resolved
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Fig7
She experienced a very brisk and complete clinical response with resolution of all oral ulcers and gum hypertrophy (Fig. 7) durable to 6 months.