Anaplastic variant of diffuse large B-cell lymphoma associated with cutaneous fistulas; an unusual presentation.

We present a 66-year-old female patient who developed right cervical and submandibular swelling. She had visited an Ear-Nose-Throat surgeon who performed a right cervical lymph node excision. The patient had multiple enlarged lymph nodes in the right cervical and submandibular areas. There were also 3 discharging fistulas: the first was high in the right posterior cervical area; the second, low in the right anterior cervical area; and the third was in the right submental area just left of the midline. There were red-purple, slightly painful, indurated areas surrounding the discharging tracts, which produced serohemorrhagic mucus (Figure 1). A swab of the discharge showed no bacterial growth. The histopathological examination of the lymph node biopsy revealed a necrotic and hemorrhagic lymphoid infiltrate penetrating from nasopharyngeal tissue into the subcutaneous soft tissue and skin. The neoplastic lymphoid cells were large, round, oval with pleomorphic nuclei and prominent nucleoli. They were growing in a cohesive and sinusoidal pattern. Immunohistochemically, the neoplastic cells were strongly positive for CD20 and mostly for CD30. Some of them showed intranuclear bcl6 positivity. ALK, CD3, CD5, CD138, CD10, Tdt and bcl2 were negative. The final diagnosis was diffuse large B cell lymphoma (DLBCL), anaplastic variant. Neck, chest and abdominopelvic CT were normal. The patient had Ann Arbor stage IIB disease. She was administered 6 courses of R-CHOP with complete response of the enlarghed lymph nodes and B-symptoms while the fistulas over the skin disappeared. The presentation of anaplastic variant of DLBCL with discharging cutaneous fistulas has not been reported before. Nevertheless, tracheomediastinal [1], carotid-cavernous [2], and gastropleural fistulas [3] secondary to DLBCL have been reported. After elimination of other diagnoses, like tuberculosis, actinomycosis, oncologic malignancies, this rare presentation of anaplastic variant of DLBCL should be kept in mind in the differential diagnosis of cutaneous fistulas in the neck region. References