Angiomatosis of the paranasal sinuses

Abstract

Angiomatosis is a rare benign lesion of the head and neck that can be mistaken for either a vascular malformation or malignant disease as a result of its infiltrative nature. The recurrence rate of angiomatosis requiring surgery is reported to be >90%, and as such the otolaryngologist treating this condition should endeavor to remove all disease during the first operation while maintaining a high level of suspicion for recurrence during postoperative surveillance. This case represents the first report of angiomatosis involving the nose and/or paranasal sinuses, and extends the differential diagnosis of sinonasal tumors, of which the otolaryngologist must be aware. A description of the surgical care and postoperative surveillance recommendations is detailed in this first reported case of angiomatosis of the paranasal sinuses.

Rare presentation of angiomatosis in the paranasal sinuses mimicking juvenile nasopharyngeal angiofibroma in a 16 year old male

“…However, angiomatosis displays a slight female predilection, but also typically presents in the first 2 decades of life [1] . The incidence of JNA is much higher than that of angiomatosis worldwide [ 6 , 14 ]. Given that our patient is a 16 year old male with a mass centered in the sphenoid and pterygopalatine fossa, the preoperative diagnosis of JNA was most likely.…”

“…However, angiomatosis has rarely ever been reported in the head and neck; a handful of case reports have mentioned oropharyngeal involvement specifically, describing lesions of the cheek/lip, masseter, mandible/malar region [ 1 , 7 ]. Only one other case report in the literature has described angiomatosis affecting the paranasal sinuses [6] .…”

“…This condition is commonly seen in the lower extremities, with additional cases reported in other sites such as the heart, forearm, abdominal wall, genitalia, and retroperitoneum [1] , [2] , [3] , [4] , [5] . Cases in the head and neck region are rare however, with singular case reports demonstrating involvement of the paranasal sinuses, cheek/lip, malar region, masseter, and mandible [ 1 , 6 , 7 ].…”

“…due to its aggressive growth and infiltrative nature [1] . Because of the latter, achieving complete surgical resection with negative margins has proven to be challenging with recurrence rates of greater than 90% [6] . Additionally, it is reported that around 40% of patients experience more than one recurrence every 5 years [7] .…”

“…Unlike JNA, angiomatosis is exceedingly rare in the head and neck and most reported cases have been limited to the oropharyngeal cavity [7] . Only one other case of angiomatosis in the paranasal sinuses has been reported in the literature [6] . In this case, we report a 16 year old male with sinonasal and skull base angiomatosis which mimicked the clinical and radiographic presentation of JNA.…”