Hemangiopericytoma Of The  Larynx In Childhood : A Rare Clinical Entity

Dr. Ashok B Galande; Dr. Hemant K Shukla

Volume : VI, Issue : IV, April - 2017

Hemangiopericytoma Of The Larynx In Childhood : A Rare Clinical Entity

Dr. Ashok B Galande, Dr. Hemant K Shukla

Abstract :

Hemangiopericytoma are rare vascular neoplasms of head and neck. Laryngeal involvement is even extremely rare, with only 12 previously reported cases in the literature. Ours is the first case in the literature and in India to be present in childhood. We present with an unusual case of a 6 years old boy with tracheostomy done at GMCH for the stridor and diagnosed as case of supraglottic mass with glottic extension. Biopsy and Immunohistochemistry was done which was Vimentin, CD34, CD31 positive s/o malignant hemangiopericytoma. After full clinic-radiological evaluation and discussion with multidisciplinary team, in a view of locally advanced lesion preoperative EBRT was given, but disease progressed after EBRT. Hence total laryngectomy with permanent tracheostomy was done with HPE report consistent with a malignant haemangiopericytoma. Patient is currently 9 months post opt follow-up doing well with no recurrence. Otolaryngologists and surgical oncologist need to be aware of this rare tumor that can be treated successfully with surgical resection. Close and long term follow up is needed.