Alveolar rhabdomyosarcoma mimicking Burkitt-like lymphoma


Dear Editor, Rhabdomyosarcoma is a rare malignancy in adults, of which two major histological subtypes have been described, alveolar and embryonal variants. Few cases of alveolar rhabdomyosarcoma masquerading hematological malignancies, either acute leukemia [1] or non-Hodgkin lymphoma [2–4] have been reported so far. We report here a case of a young adult with alveolar rhabdomyosarcoma that mimicked Burkitt-like lymphoma. An 18-year-old man with no remarkable medical history came complaining of pelvic pain. The only finding was multiple retroperitoneal centimetric lymphadenopathy in CT scan, so the patient was discharged. Three months later, he was referred to our department with the same pain and a dramatic spread of the lymphadenopathy to the ischiorectal region causi ng r i gh t u r e t e r a l obs t r uc t i on and subsequen t ureterohydronephrosis. His complete blood count showed bicytopenia: hemoglobin level was 10 g/dl, platelet count was 81×10/l, normal leukocyte and neutrophil counts, and a mild leucoerythroblastic picture on the blood film. His lactate dehydrogenase level was elevated (2162 IU/l, normal <250 IU/ l). Chest CT scan revealed spine involvement (T11–T12), and PET scan showed numerous enlarged lymph nodes extending from the mediastinum to the iliac fossa, and generalized bone marrow hypermetabolism. The bone marrow aspirate displayed infiltration of monomorphic, large-sized cells with regular nucleus outline, intermediate chromatin, prominent nuclei, and abundant amount of basophilic cytoplasm with multiple vacuoles (Fig. 1a, b). Altogether, clinical and morphological data were consistent with Burkitt-like lymphoma. Yet, a closer observation of the marrow smears revealed the presence of a few clusters of cells (Fig. 1c), in addition to exceptional paranuclear blue inclusions indenting the nuclei (Fig. 1d, arrow). The examination of the lymph node biopsy yielded the diagnosis of alveolar rhabdomyosarcoma with myogenin (Fig. 1e) and desmin (Fig. 1f) positivity and weak positivity of CD56 (Fig. 1g). Fluorescent in situ hybridization confirmed FOXO1 rearrangement [5, 6]. We report here an atypical presentation of alveolar rhabdomyosarcoma mimicking Burkitt-like lymphoma. Paranuclear blue inclusions or bodies are known to be evocative of small-cell carcinoma [7, 8] but can also be seen in other malignancies, such as rhabdomyosarcoma or even non-Hodgkin lymphoma [7]. Additionally, it has been demonstrated that paranuclear blue inclusions can be a valuable tool to accurately diagnose and differentiate metastatic small-cell carcinoma from non-Hodgkin lymphoma [9]. In our case, the presence of paranuclear blue inclusions as well as cohesive cells suggested a non-hematopoietic origin of the malignancy and helped orient our attention towards the exact diagnosis. * Orianne Wagner-Ballon

DOI: 10.1007/s00277-016-2646-6

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@article{Benayoun2016AlveolarRM, title={Alveolar rhabdomyosarcoma mimicking Burkitt-like lymphoma}, author={Emmanuel Benayoun and Bouchra Badaoui and Jehan M Dupuis and Nathalie Auger and Julien Moroch and Orianne Wagner-Ballon}, journal={Annals of Hematology}, year={2016}, volume={95}, pages={1017-1018} }