HEMANGIOPERICYTOMA

DR SUHAS K R
 Introduction

3-5% of all soft tissue sarcomas

1% of all vascular tumors.

The head and neck incidence is 15-30%

Usually seen in the orbit, nasal cavity, oral cavity, jaw, parotid
gland, parapharyngeal space, masticator space and jugular
foramen

5th to 6th decade of life

History

 Head and neck

The first reported case of HPC in head and neck was of nasal
HPC, another was in the tongue base in 1949 by Stout and
Murray

Since then, only approximately 300 cases of HPCs have been

mentioned in the literature
 Origin

Vascular tumors arising from Zimmermans pericytes

These cells are arranged alongside capillary vessels and have

smooth muscle characteristics

Responsible for vessel caliber regulation owing to their contractile

capability, modulating both flux and permeability
 Etiology

No etiological factors identified

a past history of trauma,

prolonged steroid use and

hypertension
 Clinical features

Slow-growing painless mass

No outward sign of the vascular nature of the tumour is usually

evident

Pain occurs in locally invasive large lesions or when it is

confined in unyielding spaces such as the paranasal sinuses

However, pain is characteristic of bone or pulmonary

metastases.

 Differential diagnosis of
hemangiopericytoma

juvenile hemangioma,

glomus tumor, angiosarcoma,

leiomyoma, leiomyosarcoma, schwannoma, mesothelioma,

liposarcoma, benign and malignant histiocytoma,

synovial sarcoma, chondrosarcoma, neuroblastoma

 Investigation

FNAC Cant confirm the diagnosis

INCISIONAL BIOPSY - when there is suspicion of malignancy

Risk of bleeding

Avoided in para spinal, closed spaces.

 Radiology

The tumour usually appears as a
solid mass, hypodense on CT and
isointense on T1-weighted images
on MRI.
On T2- weighted images, the
tumour shows equal or lower
signal intensity
Hypervascularity, cystic low
attenuation zones, septations and
speckled calcifications
 HPC like neoplasms

Non-HPC neoplasms that occasionally display HPC-like features (e.g.
synovial sarcoma)

True HPCs - clear evidence of myoid/pericytic differentiation -

glomangiopericytoma/myopericytoma, infantile myofibromatosis, and
a subset of sinonasal HPCs.

Solitary fibrous tumor (SFT) lesional group, which includes fibrous-

to-cellular SFTs, and related lesions - giant cell angiofibromas and
lipomatous HPCs
 Pathology

HPC is a well-circumscribed, brown, spongiform lesion,
surrounded by a pseudo-capsule

Small satellite nodules separate from the main tumor mass

They can be lobulated or nodular, firmly attached to muscle or

fascia, and soft, firm or friable
 Pathology
Small closely packed cells with ill defined cytoplasm and darkly
stained nuclei
Slit like or sinusoidal vascular spaces are interspersed between cells

Vimentin is the only marker expressed consistently

 Prognostic factors

Factors to differentiate benign from malignant

> four mitosis per field,

HPE
high cellularity,

pleomorphic tumour cells and

foci of haemorrhage and necrosis

size greater than 6.5 cm

 Tumor behaviour

Metastatic disease - in up to 50% of patients with the haematogenous
route.

It mostly metastasizes to lungs, bones and liver whereas lymph node

involvement is rare.

In head and neck - 40% recur locally and 10% have distant metastases

Biological behaviour of these neoplasms is rather peculiar, as benign-

looking, non-mitotic HPCs have been reported to metastasize
 Oral cavity

The most common site of origin - palate, followed by the
mandible and the lower lip

Most of the oral hemangiopericytomas - invasive

Local recurrence or distant mets - 25 %

Reported cases

 Management

Surgery - wide local excision

Angiography and pre-operative embolization may be

performed in large tumours with significant vascularity, in order
to decrease the size and vascularity of the tumour
 Adjuvant therapy

In general, haemangiopericytomas are considered to be radioresistant
and radiation therapy is not suggested as the primary treatment.

Reserved only for inoperable metastases or treatment of post-operative

surgical fields in case of close or positive margins

Someya et al 2001 - post op RT showed significantly improved local

control - 4 cases only
 Adjuvant chemo

Infantile type has high response to chemo (Del Rosario and Saleh
1997)
Vincristine, cyclophosphamide, doxorubicin, methotrexate have all
been tried with limited success

CT in inoperable and metastatic patients

No role in R0 resections
 Childhood hemangiopericytoma

Median age at diagnosis 13.5 years or less than one year

Better clinical behavior than the adult type

Chemoresponsiveness and spontaneous regression have been reported

in children younger than 1 year

Aggressive multimodality therapy

 Prognosis

Usually worse in adult type

Enzinger and smith (1976 )reported 10 yr survival rate of 70 %

Espat et al 2002 - 93 % and 86 % two and five year survival rate

Even though there is high local recurrence and distant metastasis rate
HPC has low disease associated mortality
 Take home message

Relatively uncommon vascular tumours more so in head and neck

HPC is "defined" - by reactivity for vimentin, with or without CD34

and CD57 and lacks other immunodeterminants of epithelial, neural,
and myogenous differentiation.

Of all the vascular tumors the diagnosis of hemangiopericytoma is one

of the most controversial
 Take home message

Surgical excision still remains the treatment of choice.

Role of adjuvant therapy is largely undefined

Clinical behaviour is difficult to predict

Patients need to be followed up closely for an extended period



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