A rare case of angiosarcoma of the maxillary sinus is reported. Review of the literature concerning angiosarcoma of the head and neck revealed 14 previously published cases involving the nose, nasal sinuses, and nasopharynx. Angiosarcoma of the skin and soft tissues of the head and neck differs in behavior from that affecting the nose, nasal sinuses, and nasopharynx. The case reported presents the protracted clinical course of this malignant tumor.

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Sinus histiocytosis with massive lymphadenopathy (SHML) was found in two siblings, both of whom had retropharyngeal involvement. In one of the children the retropharyngeal swelling appeared to produce obstructive symptoms, requiring excision of the involved lymph nodes. These cases appear to constitute not only the first proven familial occurrence of SHML but also the only reported instances of retropharyngeal involvement. SHML is an uncommon disease of unknown etiology that must be distinguished from other diseases associated with marked lymphadenopathy, particularly as its course is generally benign and not altered by any known form of medical treatment.