Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai‐Dorfman disease is a rare but well‐defined histiocytic proliferative disorder. There are only few reports or small series of cases on the fine‐needle aspiration (FNA) cytologic features of this entity. Our first case was a 14‐yr‐old girl who presented with low‐grade fever and neck swellings of 2 mo duration. FNA smears from the submandibular and cervical lymph nodes showed numerous histiocytes with evidence of lymphophagocytosis (emperipolesis) against a background of reactive lymphoid cells. The FNA cytodiagnosis was Rosai‐Dorfman disease. The patient was put on steroids, with improvement. The second patient was a 3½‐yr‐old girl who presented with fever and left cervical lymphadenopathy. The initial FNA smear from an outside laboratory was reported as reactive hyperplasia. A repeat FNA smear showed numerous histiocytes with evidence of emperipolesis, mild nuclear enlargement and multilobation/multinucleation in a few histiocytes, and reactive lymphoid cells. The cytodiagnosis was Rosai‐Dorfman disease. Review of the initial smear showed the Rosai‐Dorfman (RD) cells, but in smaller number. We conclude that FNA cytology is a useful tool in the diagnosis of SHML, but the diagnosis may be missed at initial stages when characteristic cytomorphologic features are not well‐developed. Diagn. Cytopathol. 2001;24:42–45. © 2001 Wiley‐Liss, Inc.

An 8 1/2-yr-old boy with proptosis was diagnosed to have low-grade leiomyosarcoma of the orbit following lateral orbitotomy and histology of an incompletely excised intraconal mass. He received chemotherapy but had recurrence of proptosis at the age of 12 yr. Ultrasonography revealed a solid mass and its fine-needle aspiration cytology features were consistent with recurrence of leiomyosarcoma. A year later, the cytodiagnosis was confirmed by histology of the excised tumor from a second lateral orbitotomy. Masson's trichrome stain confirmed the smooth muscle nature of the neoplasm and ultrastructural features were in favor of leiomyosarcoma. The patient received intermittent chemotherapy, and is clinically free from disease at the age of 17 yr.

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