Cavernous Haemangioma of the Parotid Gland in Adults: A Review of the Literature & Case Report
Yousif I Eltohami1, Abeer H Alrofaey2 and Ahmed M Suleiman1
1University of Khartoum, Sudan
2 Al Neelain University, Sudan
Submission:November 15, 2018; Published: December 11, 2018
*Corresponding author: Yousif I Eltohami, Assistant professor of oral & maxillofacial surgery, Faculty of dentistry, University of Khartoum, Sudan.
How to cite this article: Yousif I E, Abeer H A, Ahmed M S. Cavernous Haemangioma of the Parotid Gland in Adults: A Review of the Literature & Case
Report. Adv Dent & Oral Health. 2018; 10(2): 555782. DOI: 10.19080/ADOH.2018.09.555782
Cavernous Hemangioma of the parotid gland is an exceptionally rare pathology of the salivary gland in adults, with only about 50 cases reported globally & a strikingly predominant female’s presentation, and an observed left sided preference. We thereby report an unusual case of a 34 years old Sudanese male with 5*5cm in diameter cavernous hemangioma affecting the right parotid salivary gland that started 6 years ago to alert surgeons to the possibility of such a lesion, which was diagnosed using MRA & treated with Superficial parotidectomy using Modified Blair incision with initial ligation of external carotid artery & facial nerve preservation , we describe the clinical course, explain the diagnostic approach, discuss the current treatment modalities and review the literature.
Hemangiomas are benign vascular tumors characterized by increased proliferation and turnover of endothelial cells. Traditionally, they were categorized as capillary, cavernous and mixed [1-5], but so far only cavernous hemangioma is reported in adults [3-6].
Out of 6% of salivary gland neoplasms arising in the head and neck, 80% arises from the parotid gland & among all parotid tumors 80% are benign . 65% of all hemangiomas are in the head and neck region, they can affect the skin, muscles & salivary glands [7,8] But, they principally affect the salivary glands with the parotid gland as the most common site 81-85%. They account for 0.4-0.6% of all parotid gland tumors, with 2% occurrence in adults vs. 50% in children first year of life [1,8,9] Adult hemangioms center itself as one of the rarest presentations; with only about 50 cases reported globally  and constitute a distinct entity of parotid pathology that requires specific diagnostic tools and management.
Achache et al.  argued that the use of confusing terminology as physician still use the generalized term ‘angioma’ to describe both tumors and vascular malformations, had added more struggle to the insufficient diagnostic techniques, inadequately adapted treatments and poorly oriented research on hemangioma. They recommended the use of the international classification resulting from the 10th Workshop of the International Society for the Study of Vascular Anomalies (ISSVA), held in Rome in 1996, which was based on clinical, evaluative, histological and hemodynamic elements .
It describes two large categories of vascular anomalies:
Proliferating vascular tumors (hemangiomas) and
Vascular malformations; ‘immature’ tumors, such as hemangiomas, which are observed in infants and, after a period of growth, eventually regress spontaneously; and ‘mature’ tumors, which do not regress, but continue to develop throughout life .
Recently, Hemangiomas are classified as benign vascular tumors, divided into infantile and congenital types, with further subdivision of congenital hemangiomas into No Involuting Congenital Hemangiomas (NICHs) and Rapidly Involuting Congenital Hemangiomas (RICHs) and partially involuting congenital hemangioma (PICHs) [4,13].
Due to rarity of this condition in adults, clinical behavior may be quite misleading and less reported as they are rarely biopsied with hematoma formation risk. We thereby report on a typical presentation of cavernous hemangioma of parotid gland in an adult black male with right parotid gland cavernous hemangioma with emphasis on the clinical diagnosis and treatment challenges, aspiring to add into consideration Cavernous hemangioma as part of the differential diagnosis of parotid masses in adults.
A 34 years old Sudanese male who is generally fit with no significant medical history & no family history of interest, presented to oral and maxillofacial surgery clinic complaining from painless firm unilateral facial right side parotid region mass presented six years ago and had since a slow progressive growth
pattern gradually reaching the present size (with no history of
sudden increase in size), patient denied any history of habits
(smoking, snuff dipping or alcohols drinking).
Clinical examinations showed a non-tender soft in texture,
freely mobile, firm with well-defined palpable margins, dome
shaped swelling of (5*5cm) diameter with in the right parotid
gland that raised the ear lobe the hollow below and behind the
ear lobule was obliterated. with intact non ulcerated, normal
overlying skin in color, texture & temperature (no discoloration,
no bruises nor pulsation), similar to the surrounding skin, without
apparent discharge, scarring or any dilated blood vessels, the
swelling did not show any change in size during meals, bending
or straining nor did the patient state any change in size associated
with weather changes. On palpation, it was neither hot nor had
thrills or bruits (non-pulsatile) (Figure 1).
On bending the head down, the size of the swelling increased
in size ‘turkey wattle’ sign. On applying pressure over both
external and internal jugular veins simultaneously the swelling in
the parotid region became more prominent ‘Reddi’s Sign’.
The facial nerve terminal branches were intact bilaterally and
symmetrical, patient shows no signs of numbness, facial paralysis
or any abnormal sensation.
Intra oral examination revealed normal parotid gland duct
(Stenson`s) opening, Normal mouth opening with poor oral hygiene,
there was no apparent soft palatal swelling or deviation of
the uvula excluding the deep parotid lobe and the parapharyngeal
space involvement. Neither Cervical lymphadenopathy nor other
Diagnostic approach; Fine needle aspiration cytology (FNA)
was taken by experienced pathologist but the results were
inconclusive, it revealed blood and epithelial cells. Magnetic
Resonance Angiography (MRI) was performed which reported
intense homogenous parotid mass with incorporation of terminal
branches of the External Carotid Artery (ECA) as the feeding
vessels (Figure 2).
Diagnosis; our clinical, radiographic & pathological impression
favored vascular malformation or tumor of the right parotid gland
surgery was carried out; initially ligation of External Carotid Artery
(ECA)was performed followed by superficial parotidectomy using
Modified Blair incision. The buccal branch of the facial nerve was
pushed by the tumor, but it was identified & preserved (Figure 3
Postoperatively, a no facial weakness was noticed, Patient
recovered well with regular follow up. The histopathological
examination of the excised tumor confirmed cavernous
haemangioma fully resected without neoplastic cells. At follow up;
patient is currently asymptomatic, with no recurrence.
To date, there is still not enough data regarding recent prevalence
of parotid glands Cavernous Hemangioma presentation in
adults. It considers extremely rare, with the 2005 WHO classification
of tumors stated about 50 cases reported globally [1,3,10],
mostly as individual case reports, Since then only few cases have
been described in the literature, & the cavernous type is the only
one described so far [4,6]. In general, vascular anomalies; tumors
and malformations remain one of the least well understood entities
encounter in clinical practice as there has been no animal
model for studying the underlying pathological mechanism and
developing therapeutic modules .
In contrary to the present case, the classical victim usually
was: Female [1,4,8,14] with 2:1 female to male ratio , it
usually fluctuates in size with pregnancy and menarche.
These phenomena suggest that the endothelial cells may be
quite responsive to circulating hormones . Where juvenile
hemangioma, displays predominately in males . With an
observed Left-sided preference has been noted [16,17], in both
parotid and submandibular gland cavernous hemangioma .
Haemangioma of major salivary gland especially parotid is quite
common in pediatric population. Ninety percent of it arise in the
first three decades of life and are the most common lesions of the
major salivary glands during infancy and early childhood [4,17].
Few published studies have provided quantitative evidence of
adult presentations of parotid cavernous hemangioma.
Nagao et al.  in a series of 20 cases of cavernous
heamangioma of parotid gland; where the average age was 26
years (range, 4 months to 50 years). Where in an analysis of all the
major salivary gland lesions recorded at the Armed Forces Institute
of Pathology (AFIP) found that 1.4% were benign mesenchymal
tumors. Of this group, 30% were hemangiomas. They were more
common in the parotid gland with 87.5% of all salivary gland
hemangiomas in comparison with 12% in submandibular gland
. A probable explanation of this big incidence difference
between parotid and submandibular is the lack of a well-defined
capsule and the presence of neurovascular structure in the
parotid gland which can be challenging in the surgical option of
treatment [17,18]. A ten years retrospective study, among 614
parotidectomy performed between 1998 and 2008 at Chirurgie
Cervico-Faciale institution, France; were 462 (75%) had a total of
10 cases (1.6%) of vascular malformations have been identified.
There was a marked female predominance (90%) as usual, there
was no side predominance .
They also stated that there were no bilateral cases , which
was also noted during the collection of this literature review. CC
Chuange et al.  stated that More than 90% of cases present
before the fourth decade of life. The average age of patients
presenting with hemangiomas is 10 years (range, 2 months to 74
years) , However, cavernous hemangiomas typically occur in
adolescents and adults, Adult salivary gland hemangiomas are
of the cavernous type, while infantile hemangiomas are usually
With this patient being an adult male in the fourth decade and
a right-sided presentation we consider it an atypical presentation
that showed be considered.
Histological presentation; The cavernous variant, as seen in
this case, is characterized by dilated, thin-walled tortuous vascular
spaces filled with blood and lined with flattened endothelial
cells which sometimes may be thickened by adventitial fibrosis,
calcificayion is also common [3,4,6,15,20].
The classic clinical presentation of a parotid hemangioma is
not very specific. It usually present as an asymptomatic slowly
progressive, poorly defined, soft, firm compressible, mobile,
painless swelling below and/or in front of the ear lobule  in
the parotid region that is rarely associated or not with skin lesions
characterized by red, red-bluish, or blue macules and/or papules,
as well as a vibration or pulsation when palpating the parotid
Although, it is very rare to observe bluish discoloration of the
skin with cavernous hemangioma, which is a characteristic feature
of benign hemangioendothelioma . The tumor expands slowly
but sometimes sudden increase in size can lead to compression of
vital structures especially at later stage . Invasion is commonly
by continuity with complete or partial replacement of parotid
tissue [4,22]. Symptoms; dyspnea or dysphagia, when present
were related to either the longstanding nature of the tumor or its
size . The main complaint related is usually aesthetic. It may
be associated with varying degrees of pain . Deformity and
severe pain depend on tumor size and the presence of intralesional
bleeding or acute thrombosis . Manual compression can be
used to relieve pain and local edema if present .
Facial palsy as part of the clinical presentation has not been
observed . They tend to be larger and less well circumscribed
than the capillary and juvenile hemangiomas . Although they
are typically slow flow lesions and may not be angiographically
evident . Cavernous hemangiomas in adults does not regress
[1,15], and they tend to have a chronic course and a slowly
progressive growth unlike pediatric one [1,25] The presence
of radiological radio-opacities phleboliths is very suggestive of
hemangioma or vascular malformation (cavernous hemangioma
in particular) [1,4,10,15,17,21].
Redid BR et al.  even stated that the presence of calcified
phleboliths on radiological imaging is a characteristic finding
of cavernous hemangioma of the salivary gland , although
cavernous hemangioma with multiple phleboliths are very rare
[1,4,17]. However, these only occur in 2-3% of cases and it should
be differentiated from sialoliths and other cause of head and neck
calcifications by a sialography [1,4,10,25].
The clinical examination to diagnose parotid hemangioma
is Turkey-Wattle Sign. If the hemangioma lies outside the gland,
there will not be any increase in the size [4,10,21,22,27]. Dempsey
et al.  described this specific clinical sign, was found in 50%
of their patients. Tumor volume increases with the Valsalva
maneuver or when the head is tilted forward, or the patient lies
flat . For many years this phenomenon was surprisingly
neglected by many examiners, but recently many had stated
its importance and encouraged adding it to the parotid gland
examination [10,21]. Petros Koltsidopoulos et al.  considered the phenomena an unusual pathognomonic manifestation in both
intramassetric (intramascular hemangimoa present less than
1% of all hemangiomas  with 13% in head and neck region
 & are considered very difficult to diagnose) & intraparotid
hemangioma. It can be examined with active teeth-clenching (on
contracting masseter muscle) or with dependent head positioning
(when the head is bent forward, or the patient lies flat) [21,28-
32] but is usually more prominent with the masseter muscle
contraction  (Figure 5).
The sign may be due to vascular engorgement within the
lesion, which impedes venous return from the head to the superior
vena cava. The name wattle comes from the red vascular structure
in the neck of the male turkey that can increase in size when filled
with blood .
Reddi BR et al.  described an observation that can be used
for diagnosis of parotid hemangioma, which we can address it
as Reddi’s Sign where hemangioma can be distented by blocking
its venous outflow. If it is located within the parotid gland, it will
stretch the parotid capsule. Thus, application of pressure over
parotid venous outflow (both external and internal jugular veins)
by placing the thumb of the opposite hand across them, careful to
avoid carotid bulb massage or compression while in front of the
patient with the neck muscles relaxed will cause the hemangioma
to bulge stretching the parotid capsule with distended outlines. It
makes the outline of the gland clinically apparent (Figure 6)
This observation is useful to differentiate a hemangioma
present in the parotid gland from one that is placed outside the
gland like an intramuscular hemangioma of the masseter muscle
&infratemporal fossa (where would not be any increase in size)
 or internal jugular phlebectasia .
Unfortunately, this assumption must be tested on several
patients if it were to be accepted as a valid clinical sign. Which
shows some difficulty Considering the rarity of the  & that
may add to the delay in discovering this sign .
If these signs are absent the diagnosis could be challenging,
particularly in an adult patient in whom this disease is usually
not taken into consideration in the differential diagnosis of parotid
mass. Therefore, recurrent mumps, tumors or cystic lesion of
glandular origin or hypertrophy of the masseter muscle are the
principal differential diagnosis. In adults, the pleomorphic adenoma
and the Warthin tumor are within the most common benign
tumors of salivary glands or sialolithiasis in case phileboths are
presence [1,4,10,14]. Hereby, History and physical examination
are elements supporting the diagnosis of probability [1,24,35].
The imaging techniques commonly used for investigation includes;
Ultrasonography (USG), Computed Tomography (CT) scan,
Magnetic resonance imaging (MRI) and Fine Needle Aspiration Cytology
(FNAC). Ultrasonography is the first investigation of choice
in salivary gland imaging [4,10]. On ultrasound, hemangiomas are
heterogeneous hypoechgen lesions in which calcified phieboliths
are identifiable .
MRI is regarded as the best imaging method for evaluation of
parotid hemangioma and preferred over CT scan for the evaluation
of soft tissue lesions in head and neck region [1,4,8,10,20,36]. It’s
the complementary examination of choice in their exploration, its
useful in demonstrating lesions of the parotid region thus making
it possible to suggest that the tumor is a hemangioma and for its
extension to be studied [1,3].
Hemangiomas generally appear as a lobulated lesion with a Ti
-weighted image, hyper-intense on T2 signal with homogeneous
enhancement and empty signal areas within the lesions [1,3,37,38].
It also helps determine the surgical approach for the tumor and to
reveal its relationship with adjacent structures. [1,38].
Fine Needle Aspiration Cytology FNAC is useful in the preoperative
diagnosis of tumors of the head and neck, so it’s considered
the final procedure for a definitive histologic diagnosis, but
it is regarded unnecessary preoperatively in case of hemangioma
because of risk of generating a hematoma and when MRI is highly suggestive of the diagnosis [1,4,8,10,38]. Color Doppler Flow meter
is also suggested to support diagnosis [5,21,26].
Therefore, a typical clinical presentation and characteristic
radiologic findings are enough for the diagnosis [1,38]. The
histological findings reveal Large thin walled tortuous and dilated
blood vessel, lined by flattened endothelial cells is a typical sign
of cavernous hemangioma This striking histological feature
assuming a periductal arrangement [4,11, 20,39-41].
Treatment of vasoformative tumors can be divided into three
a. Conservative management with clinical monitoring,
b. Medically aggressive treatment or
c. Surgically invasive removal
Never the less, The therapeutic options for cavernous
haemangiomas in adults are limited , Their treatment is exclusively
surgical [1,3,19,42] opposite of infantile haemangiomas that can
be treated medically with a variety of options as endovascular
sclerotherapy ,intralesional or systemic corticosteroids,
vincristine and propanolol, intra-lesional alcohol injection,
interferon , cryotherapy, laser surgery, vascular ligation, laser
photocoagulation & compression therapy followed cavernous
hemangioma do not regress unlike most infantile hemangioma
which resolve spontaneously ,there is an inclination to avoid
conservative treatment and go for surgical options ,still there is
few exceptional adult cases where conservative medical treatment
with clinical monitoring is a better option as in patients unfit for
surgery [1,3,14,42,43]. Sclerosing agents such as ethanol, ethanol
amine oleate, sotradecol, tetradecyl sodium sulphate, ethanol and
bleomycin hydrochloride can be injected percutaneously under
radiological guidance [23,44-46].
Surgical resection and sclerotherapy are associated with a risk
of facial nerve injury, which is increased in the case of very large
lesion situated adjacent to the facial nerve, which can be difficult
to precisely assess preoperatively. The patient must always be
informed about the risk of this complication .
Super Selective pre-surgical embolization (a procedure
performed by interventional radiologist that allows the
improvement performance of subsequent surgery interventions
, & that are constantly evolving into standalone therapeutic
modalities) is suggested 24-72 hours before surgery to decrease
the risk of extensive intraoperative bleeding & reduce tumor
vasculature [1,3,4,10,28,47], especially in cases where the
hemangioma is extensive or the supplying vessel is a major one,
that’s why Before surgery, the vascular supply of the tumor a
thorough imaging study by angio MRI (MRA) aimed at detecting
the vascular supply (main & collateral) should be analyzed in order
to avoid intraoperative bleeding complications [3,24,48,49]. For
small lesions, surgical excision poses little difficulty, in extensive
lesions the facial nerve may be difficult to identify and should
be monitored intraoperatively . In general, more aggressive
treatment is particularly considered when parotid hemangiomas
are: large, deforming, ulcerated or involve neighboring structures,
with functional consequences  (Figure 1).
Currently, the established documented clinical practice in the
Adult cavernous intraparotid hemangioma in adults is surgery:
partial or total parotidectomy according to level of involvement,
using modified blair incision or modified face lift incision which
give better aesthetic results, taking into consideration a presurgical
super selective embolization 24-72 hours before surgery,
with or without intraoperative supplying vessel ligation & facial
nerve preservation [1,4,10,11,19,45].
Moreover, Xie et al.  introduced a minimally invasive
endoscopic approach via a small preauricular incision performed
on 5 patients with benign APG tumors and no postoperative
complications and recurrences were found.
SS Intra-arterial Embolization can be done with: Temporary
materials; Gelfoam, collagen, Iodine oil, or Perminant materials;
butyl cyanoacrylate (NBCA), Ethiodol (iodine & poppyseed),
acrylic glue (Glubran), stainless steel, silky threads [4,24,51].
Several syndromes may be associated with cavernous hemangiomas,
Kasabach-Merritt syndrome (large cavernous hemangioma
associated with thrombocytopenic purpura, intravascular
coagulation and platelet sequestration in the tumor) is the most
Hemangiomas may arise from the gland proper or by invasion
of subcutaneous blood vessels into the gland structure. Since
Scarcella et al.  which is one of the oldest reports of cavernous
hemangioma of the parotid gland in adults, the etiology of the
disease was a point of great controversy . It’s considered
benign proliferations of vessels closely resembling normal vessels,
Marx et al.  argued that similarity to normal vessels is so great
that it is unclear whether they represent vessel malformations,
true neoplasms, or hamartomatous overgrowths [4,17,48].
But, with the advancement in genetic study, the etiology of
hemangioma is still elusive. Controversies surround all explanation
from gene mutation to developmental factors for etiopathogenesis
of hemangioma. Few evidences demonstrate that dendritic cells
play an important role in the formation of hemangioma through
some cytokine, such as vascular endothelial growth factor while
some experts concluded that hemangioma originates from embryo
of angioblasts [4,36].
Recently, the expression of cyclooxygenase 2 (COX2) protein
on endothelial cells of several vascular spaces of cavernous
hemangioma have been found but has little evidence with the
relationship of vascular tumors. However, there has been reported
that high doses of celecoxib have inhibited the cell proliferation
of angiosarcomas cell lines. So, it could be considered as a new
therapeutic line research for tumors of vascular origin [1,3,4,8,53].
It is currently believed that hemangiomas are benign and
congenital neoplasms, which are usually congenital or subclinical
at birth undetected for long periods of time until sudden growth
years later induces pain or cosmetic deformity (e.g., hereditary
hemorrhagic telangiectasia and several facial hemangiomas)
Cavernous hemangioma of the parotid gland in adults is
a rare benign pathology. Nevertheless, it should be included
within the differential diagnosis of salivary gland swellings.
Definitive diagnosis although difficult, it can be accomplished
through clinical and radiographic findings. Through enlightening
clinicians to the clinical presentations and examination methods
to diagnose a parotid hemangioma, more cases will be recognized.
We hope to encourage a group of leading pioneers to experiment
with this information and document their findings, which may
widen our data regarding the prevalence and etiology of the
disease. The current unified management is surgery: partial or
total parotidectomy according to level of involvement, using
Modified Blair incision or Modified Face Lift incision, taking
into consideration a pre-surgical super selective embolization if
possible, with or without intraoperative supplying vessel ligation
& facial nerve preservation is proven to be successful caring no