RESUMEN
INTRODUCTION: We describe the first case of ALHE in the orbit with extension to the maxillary sinus and the importance of a multidisciplinary approach to achieve removal of the lesion. CASE STUDY: A 72-year-old man presented with epiphora of the left eye and several episodes of recurrent acute dacryocystitis. Magnetic resonance imaging revealed a solid homogeneous mass located in the inferomedial region of the left orbit. In addition, it was associated with destruction of the adjacent ethmoidal wall and upper wall of the left maxillary sinus. Incisional biopsy of the orbital mass was compatible with ALHE. RESULTS: It was decided to perform surgery using an orbital floor approach, left medial wall via subconjunctival and caruncular approach together with an endoscopic nasal approach (ESS), achieving complete removal of the orbital mass and cleaning of the maxillary sinus. After one year of treatment, no tumor recurrence was evident through endoscopy and imaging tests and the patient is asymptomatic. CONCLUSIONS: ALHE is a very rare benign vascular tumor that presents subcutaneous nodules in the head and neck region. We do not know of any case of ALHE in the paranasal sinuses described in the literature, either in isolation or together with orbital or cutaneous ALHE. In conclusion, ALHE disease should be considered as a diagnosis when faced with an orbital mass with extension to the paranasal sinuses, and a complete excision through a combined endonasal and orbital approach prevents recurrence in most cases.
RESUMEN
El hemangioma fusocelular es un tumor vascular benigno poco frecuente. Se ha relacionado con el síndrome de Maffucci, el síndrome de Klippel-Trenaunay y otras malformaciones venosas. Habitualmente son nódulos dérmicos de color violáceo en las extremidades distales de niños y adultos jóvenes, sin predilección por ningún sexo. Se presenta el caso de una paciente de 22 años que padecía un nódulo subcutáneo, doloroso a la presión, en la pierna derecha. A la exploración física se observó una tumoración redondeada violácea de 1,5 cm. Se sospechó la presencia de un hemangioma. Se realizó una escisión simple y se envió al departamento de anatomía patológica. Se describió la muestra como nódulo intraluminal constituido por una proliferación de células de aspecto fusiforme con moderada atipia y aisladas figuras de mitosis y se confirmó el diagnóstico de hemangioma fusocelular de localización intravascular. El diagnóstico del hemangioma fusocelular es anatomopatológico y como tratamiento suele bastar la escisión simple, aunque frecuentemente recidivan. Dado que el hemangioma fusocelular es una entidad poco conocida y puede ser confundida con otras lesiones vasculares, se considera interesante la comunicación del presente caso. El conocimiento de este tipo de lesión puede aumentar los casos notificados.
Spindle cell hemangioma is a rare benign vascular tumor. It has been linked to Maffucci syndrome, Klippel-Trenaunay syndrome, and other venous malformations. They are usually purplish dermal nodules on the distal extremities of children and young adults, with no sex predilection. A case of a 22-years-old patient who suffered from a subcutaneous nodule, painful on pressure, in the right leg is presented. On physical examination, a 1.5 cm violaceous round tumor was observed. The presence of a hemangioma was suspected. A simple excision was performed and sent to the pathology department. The sample was described as an intraluminal nodule made up of a proliferation of spindle-shaped cells with moderate atypia and isolated mitotic figures, and the diagnosis of intravascular spindle cell hemangioma was confirmed. The diagnosis of spindle cell hemangioma is anatomopathological and simple excision is usually sufficient as treatment, although they frequently recur. Since spindle cell hemangioma is a little-known entity and can be confused with other vascular lesions, the communication of this case is considered interesting. Knowledge of this type of injury can increase reported cases.
