Plasmablastic lymphoma of visceral cranium, cervix and thorax in an HIV-negative woman

Abstract

Dear Editor, We would like to report a case of aggressive plasmablastic lymphoma (PBL) in an HIV-negative individual, characterised by a unique combination of unusual clinical and immunophenotypic features. A 67-year-old woman presented with a 20-day history of a painless mass lesion in the left supraclavicular fossa. She was also complaining of blurred vision and a continuous mild retrosternal pain. She denied fever, night sweats or weight loss. Her past medical history was negative for diseases or treatments associated with immunodeficiency. On physical examination, she had diplopia on upward gaze. A painless firm mass 3×4 cm in size was palpated in the left supraclavicular fossa. There was no peripheral lymphadenopathy, organomegaly or other remarkable findings. Laboratory investigations showed a normal full blood count and serum biochemical profile, including normal lactate dehydrogenase levels. No paraprotein was present on serum and urine electrophoresis. Enzyme-linked immunosorbent assay testing for HIV antibodies was negative. Computerised tomography (CT) imaging studies revealed a mass with soft tissue density occupying the left sphenoid sinus and infiltrating the ethmoid sinus and ipsilateral concha with associated areas of bone destruction. The mass was extending to the left orbit with thickening of the left inferior rectus muscle. There was also an evidence of tumour invasion of the left maxillary antrum and mandibular ramus. Furthermore, soft tissue masses compatible with multiple lymph node enlargement and block formation were present in the inferior frontolateral cervical region with extension to the anterior upper mediastinum. CT imaging of abdomen was negative for lymphadenopathy or organomegaly. A bone marrow biopsy was negative for neoplastic invasion. Biopsy of the cervical mass revealed a neoplasm causing complete effacement of nodal architecture and extending to the adjacent adipose tissue. The neoplastic tissue consisted of diffusely growing largeand medium-sized cells with polymorphic nuclei, frequent mitoses and moderate-to-large amount of eosinophilic or amphiphilic cytoplasm (Fig. 2a). Immunohistochemical investigation using markers of epithelial (Ker HMW, Ker LMW, Ker7, Ker20, EMA, Cam 5-2), lymphoid (CD3, CD20, CD45RO, CD45, CD30, CD21, CD79a, CD8, CD56, CD138, kappa and lambda chains, immunoglobulins), mesenchymal (S-100, vimentin, actin, desmin, CD31, CD34, GFAP (glial fibrillary acidic protein)), histiocytic (CD68), melanocytic (HMB-45) differentiation and various others (Bcl-2, CD99, neuron-specific enolase [NSE]) revealed only a strong expression of vimentin and a small percentage of cells with moderate positivity for NSE. Blind biopsies from the nasopharynx indicated only nonspecific immunologic stimulation of the lymphoid tissue. Ann Hematol (2007) 86:615–618 DOI 10.1007/s00277-007-0280-z