Hemangiopericytoma of meningo-fronto-naso-orbito-maxillary complex

Background

Described by Stout and Murray in 1942, hemangiopericytoma (HPC) is considered to be a tumour of pericytes of Zimmermann, which are baroreceptors on luminal wall of vascular channels. It is more commonly reported in trunk and lower extremities.1 Sinonasal cavity accounts for less than 5% of all reported cases in head and neck region.2 It usually occurs in fourth to fifth decade of life.3

HPC of sinonasal cavity is best excised by transoral and transfacial approach, therefore we present a case of HPC arising from inferior part of frontal lobe, extending up to the left maxillary sinus, involving the left eye globe and ipsilateral paranasal sinuses, undertaken for a bilateral maxillary artery embolisation followed by a wide local excision.

Case presentation

A 40-year-old woman presented with a large swelling on her face compressing her left eye, leading to dull, continuous pain since 3 years. The patient primarily reported altered facial appearance, inability to smell, burning headache with dull and continuous pain on her face, since 3 years. Headache was reported to cease after an episode of epistaxis 1 year ago.

Initially the swelling was inconspicuous and she underwent local excision two times. At the time of current presentation, the patient had single well-defined oval swelling of approximately 8×6 cm on her face extending superior-inferiorly from 1 cm above the glabellar region to left ala of nose, and mediolaterally from middle third of right eye to lateral canthal region of left eye was present, left eye was displaced inferiolaterally, with hypertelorism (figure 1).

Swelling appeared soft to firm in consistency, with no local rise in temperature, covered by normal-appearing skin with taught texture at central region and multiple venous channels visible, no bruit on auscultation. She had anosmia since 2 years, no clinical lymphadenopathy and normal deglutition. Mouth opening was adequate with normal appearing oral cavity. Vision in left eye was intact, with decreased acuity and restricted movements of eyeball in lateral and medial gazes. Menstruation ceased since 5 years.

Investigations

CT revealed swelling starting from the base of frontal lobe, extending bilaterally into the frontal sinuses and involving ethmoid, left sphenoid partially and left maxillary sinus. The tissue and muscle planes were compressed and intact. There was substantial thinning of the inner table of frontal bone, compression of the extraocular muscles, gross erosion of nasal bone along with medial wall of the maxillary sinus. Medial wall of left orbit was completely eroded by the mass, posteriorly extending to the vomer bone (figure 2).

Ultrasonography of the swelling revealed multiple cavitations within the swelling; and on reducing its frequency, there was feeble response of the vasculature.

Fine needle aspiration cytology was done to yield blood tinged extirpate. CT guided true cut biopsy reported it to be HPC. A supracarotid angiography (figure 3) revealed neoangiogenesis from bilateral maxillary arteries. Transfemoral supracarotid reversible angioembolisation was done by procuring stents bilaterally in maxillary arteries.

Differential diagnosis

Provisional diagnosis of low flow arteriovenous malformation was made, in view of the previous histopathological (H/P) report of haemangioma, esthesioneuroblastoma was considered as first differential diagnosis.

Treatment

Bilateral eyebrow incision with Webber Ferguson’s approach (figure 4) was considered to remove the redundant skin of the face and simultaneously excise the tumour in toto. Tumour was released from the base of maxilla, by LeFort I level osteotomy on left side. Dissection was done on left eye globe by orbital fat as plane of dissection, and posteriorly vomer bone was kept as the end of dissection, superior dissection started with dura mater as the plane of dissection (figure 5). We always use customised orbital floor soft tissue retractor to retract the soft orbital tissues4 at the time of orbital surgeries. A 24×13 cm tumour was excised in toto (figure 6), temporalis fascia graft was used to cover base of skull. When the bones excised to expose the tumour of the facial skeleton, we recommended bone mapping/sketching to preserve the bones for future use in reconstruction.5

To maintain facial architecture, medial nasal cartilage was tugged with 2-0 vicryl suture with frontal bone, and temporalis muscle with Bachet’s flap was used to develop sling base for left globe of the eye. Redundant skin was excised and extraoral incision closure was done with the help of 5-0 Prolene suture (figure 7). H/P evaluation reported it to be HPC. A modified Weber Ferguson incision with Borle’s extension6 was kept as a part of treatment plan if reconstruction was planned with extended temporalis myofascial flap.7 The patient was given routine injectable antibiotics (covering both aerobic and anaerobic) and anti-inflammatory and analgesics. Hydration of the patient was maintained via intravenous fluid therapy and with the help of Ryle’s tube.

Outcome and follow-up

The patient’s postoperative healing was uneventful, suture removal was done on the 7th postoperative day. She was discharged on 10th postoperative day after shifting to oral antibiotics for 2 more weeks. The patient is on regular follow-up and is completely disease free after 1-year follow-up.