Oral and Maxillofacial Surgery Cases 6 (2020) 100149

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Oral and Maxillofacial Surgery Cases

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Spindle cell haemangioma in head and neck: Report of an

uncommon vascular lesion and review of treatment modalities
till present
Degal Saikrishna a, K.P. Mahesh b, *, Namitha M. Hiriyanna a
a
Oral and Maxillofacial Surgery, JSS Academy of Higher Education and Research, JSS Dental College, Mysuru, 570015, India
b
Oral Medicine and Radiology, JSS Academy of Higher Education and Research, JSS Dental College, Mysuru, 570015, India

A R T I C L E I N F O

Keywords:
Haemangioma
Spindle cell
Sarcoma
Vascular Utumor

Abstract

Spindle cell haemangioma is an uncommon vascular lesion, often affecting the dermis and subcutaneous tissues of distal ex­
tremities. As few as 15 cases in the head and neck region have been reported in literature till present. It is often diagnosed as various
other entities including mucocele, haemangioma, pyogenic granuloma, synovial sarcoma, and enchondroma. The histopathologic
fields of SCH resemble angiosarcoma and Kaposi’s sarcoma bringing difficulties in diagnosis and clinical management. Here we report
a case of a large spindle cell haemangioma occurring in the buccal mucosal fold in a 10-year old boy, that was surgically resected in
toto with no recurrence till present.

1. Introduction

Oral hemangiomas are common, benign neoplasms that are most frequently seen in infants and children. They represent congenital
vascular tumours composed of hyperplastic vascular endothelial cells that have the capacity for excessive proliferation but normally
undergo regression and eventual involution. Spindle cell haemangioma is an unusual variant of this pathology, that is probably a
vascular malformation or a benign process superimposed upon a malformation and hence inadequately understood[1]. It has been
reported as few as fifteen times in the head and neck region, with most lesions presenting as submucosal nodules although,
intra-muscular and intra-orbital presentations have been reported. The histopathologic fields of SCH resemble angiosarcoma and
Kaposi’s sarcoma bringing in difficulties in diagnosis and clinical management.

* Corresponding author. Correspondig author.

E-mail addresses: degalasaikrishna@gmail.com (D. Saikrishna), medmahesh1973@gmail.com (K.P. Mahesh), namithamh@hotmail.com
(N.M. Hiriyanna).

https://doi.org/10.1016/j.omsc.2020.100149
Received 30 November 2019; Received in revised form 4 March 2020; Accepted 26 March 2020
Available online 3 April 2020
2214-5419/© 2020 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
D. Saikrishna et al. Oral and Maxillofacial Surgery Cases 6 (2020) 100149

2. Case report

A ten-year boy, presented with a complaint of swelling over the left face that had been noticed by parents two weeks prior and
persisted hence. The swelling had slowly increased in the two weeks and there was no associated history of pain in the region. The
parents did not give any history of trauma and claimed no significant medical history.
Extraoral examination revealed a diffuse swelling lateral to nasolabial fold on the left side of the face. On inspection the swelling
extended anteriorly from the left lateral wall of the nose and extended posteriorly till maxillary third molar area. Superiorly it extended
to 2–3mm below the infra orbital margin. Overlying skin appeared stretched and no secondary changes were seen(Fig. 1). On palpation
swelling was soft to firm and non-tender, all extensions were confirmed. And a submucosal nodule approximately 25 � 15 mm was
noted beneath the maxillary buccal vestibular fold. There was no local rise in temperature.
Intraoral examination revealed that teeth 63, 64 and 65 had deep caries but was asymptomatic. On inspection, the submucosal
nodule was not obviously seen, however a bluish hue of the overlying mucosa was noted over the left buccal mucosa. On palpation the
nodule size and consistency was reaffirmed. No other secondary changes were observed.
A provisional diagnosis of dental abscess was made based on the history of duration of the complaint with differential diagnoses of
haemangioma, mucocele and lipoma.
Intra oral periapical radiograph taken of 63, 64 and 65 showed caries approximating pulp and erupting 24 and 25 with no
remarkable periapical changes. Fine needle aspiration was done after which the submucosal nodule significantly reduced in size. The
fine needle aspiration cytology revealed degenerated erythrocytes, macrophages and haemorrhage with no evidence of malignancy.
The nodule had returned to its initial size on presentation within 24 hours. At this juncture further investigations were carried out. An
ultrasound of the left cheek revealed a well-defined cyst extending posterior to masseter muscle, approximately 3 � 2cm, with internal
echoes suggestive of haemorrhage. Based on the ultrasound report a contrast enhanced computed tomography was advised to evaluate
the region of interest, which revealed a well-defined iso-dense lesion measuring 2.4 � 2.1 � 2.9cm (APxCRxTT) in the left buccal
region with fat stranding in adjacent soft tissue, extending into infratemporal fossa abutting masseter and buccinator. Post-contrast

Fig. 1. Clinical appearance at presentation.

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study, the lesion showed no obvious enhancement and no erosion of maxillary alveolus and lateral antral wall. Transverse facial and
buccal branches of the facial artery were seen in the vicinity of the lesion. Under the clinical diagnosis of haemangioma, the lesion was
planned for excision under general anaesthesia.
Intraoperatively, the lesion was approached through a buccal vestibular incision. Careful blunt dissection was done in the sub­
mucosal plane to separate tumour from the surrounding tissues (Fig. 2). There we no identifiable inflow feeder vessels and excision was
done in toto with a part of the buccal mucosa. The wound was primarily closed. The postoperative course was uneventful. The patient
was regularly recalled to evaluate for recurrence of the lesion and has remained recurrence-free 15 months after surgery.
On gross examination, the soft tissue specimen measured 50 � 15mm. It had a reddish-blue surface covered by a thin-walled
capsule including a tiny area of the oral mucosa (Fig. 2). On microscopic examination, a loose fibrous connective tissue containing
numerous thin-walled vessels lined by endothelial cells, some containing thrombi, were seen. Admixed cellular fronds of spindle-
shaped cells having a fairly regular oval or elongated nuclei were present along with epithelioid cells that showed cytoplasmic vac­
uolations (Fig. 3A and B). Bundles of smooth muscle were noted around the blood vessels and in the spindled cell areas in a few areas.
On immunohistochemistry, CD31 was strongly positive to mark vascular distribution in the lesion (Fig. 3C). The lesion was hence
diagnosed as SCH.

3. Literature review

In 1986, Weiss & Enzinger first described “spindle cell haemangioendothelioma”, a vascular tumour comprising histological
features of both cavernous haemangioma and Kaposi’s sarcoma, developing as superficial circumscribed red-brown masses over distal
extremities. Histologically, they noted cavernous blood spaces containing phleboliths, separated by spindled fibroblastic cells and
suggested the term spindle cell haemangioendothelioma (SCHE) for this vascular tumour of low-grade malignancy[2].
The exact pathogenesis and biologic behaviour of SCH remain unclear. Fletcher et al. observed histological evidence of malformed
vasculature at the affected site and a local progression over many years with no true recurrence[3]. Imayama et al. observed in their
patient, nodules that showed initial rapid painful development followed by long periods of quiescence[4]. They hypothesized cyclic
recanalization of microvascular segments following thrombosis and described SCHE as a reactive vascular proliferation rather than an
angiosarcoma. Perkin and Weiss explained areas of diminished flow resulting in vascular collapse and formation of “cellular zones”
and areas of vascular engorgement promoting thrombosis. They suggested a contiguous spread along a vessel that explained re­
currences which they had observed and noted no evidence of metastatic potential. Hence solitary lesions were designated as spindle
cell haemangioma and multifocal lesions as spindle cell haemangiomatosis[5]. SCH may occur in syndromic multiple nodular form and
has been associated with Maffucci syndrome, Ollier disease, Millroy disease, Klippel-Trenaunay syndrome, von Willenbrand disease
and acute myelomonocytic leukaemia[6]. Solitary form although appearing sporadically may arise after repeated traumatic injury
such as repeated injections[7] and repeated surgical insults. The tumour occurs at all ages with equal sex prevalence[5]. The nodules
develop rapidly and painfully, with firm initial consistency and become soft and asymptomatic as they enter their quiescent period.
Microscopically, SCH has a biphasic appearance and cells that lack nuclear atypia. There are irregular cavernous spaces lined by a
single layer of endothelial cells and intervening fronds of spindled cells composed of endothelial cells and fibroblasts in varying
proportions and nests of polygonal epithelioid cells with intracytoplasmic vacuolization. Histologically, spindle cells seen in SCH and
Kaposi sarcoma can pose a diagnostic challenge. In Kaposi sarcoma, there is a more infiltrative growth pattern and the vacuolated
epithelioid cells are not seen. Hyaline globules are often found in the spindled cells. Nuclear immunoreactivity is present for HHV8
uniformly in all Kaposi sarcoma and not SCH. Angiosarcoma may also have a spindled morphology, but it shows more infiltrative

Fig. 2. Surgical site (Left) and Specimen (Right).

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D. Saikrishna et al. Oral and Maxillofacial Surgery Cases 6 (2020) 100149

Fig. 3. A) & B) Histopathology C) CD31 immunohistochemistry.

growth and nuclear atypia and hence can be differentiated from SCH. Immunohistochemically, in SCH, cells lining cavernous vessels
are positive for CD31, CD34, vimentin and factor VII related antigen, supporting the endothelial origin. Spindle cells react negatively
for endothelial markers and positively for vimentin. IHC has been considered a final modality for establishing SCH diagnosis[8].
For cutaneous SCH therapeutic approaches similar to those used for angiomatous lesions including selective embolization, systemic
steroids, cryotherapy, laser therapy, radiation therapy, cytotoxic drugs and biologic modifiers like interferons, interleukins have been
applied[9]. In the head and neck region, 15 cases of SCH have been reported. These cases have a spectrum from 1.5 years to 70 years
old with equal sex prevalence. It is often diagnosed as various other entities including minor salivary gland tumour, mucocele, hae­
mangioma, arteriovenous malformation, pyogenic granuloma, synovial sarcoma, and enchondroma.
Singh et al. under the tentative diagnosis of a vascular malformation treated their patient with 2 sessions of 3% Sodium Tetradecyl
Sulphate intralesional injections. Non resolution at follow up of 12 weeks prompted them to change the treatment course and sur­
gically excise the lesion under general anaesthesia[8]. Murakami et al. surgically excised the lesion on the lower lip of their patient
after failed ethanol intralesional injections [7]. Scott et al. performed an incisional biopsy for a suspected cavernous haemangioma on
the ear which was later diagnosed as SCH[10]. Minagawa et al. performed an incisional biopsy of a mass over temporal region, with
provisional diagnosis of a highly vascular tumour. They further performed surgical excision[11]. The rest of head and neck reported
cases were surgically excised in the initial treatment.
Follow up periods were not available in five cases, and ranged between 6 months and 4 years in the rest. Of the 14 cases that
underwent surgical resection, recurrences were absent in 11, follow up details were not available in two cases and one case was lost to
follow up. Only one case of recurrence was reported with surgical excision after 2 years, that was re-explored with frozen sections to
confirm negative margins. The present case is reported in a ten-year-old in the maxillary buccal vestibular area that was excised in toto
(Table 1).

4. Discussion

Haemangioma is the foremost vasoformative tumour of the oral/pharyngeal region, representing at least one-third of all hem­
angiomas in humans. It presents as a benign neoplasm composed of excess of vascular channels often self-involuting and managed
conservatively and rarely debulked through a surgical procedure. It is usually present at birth as infantile haemangioma in its su­
perficial, deep, mixed, local and multifocal forms and in young adults or older individuals as true benign neoplasms of capillary or
cavernous nature or as an abnormal proliferation after trauma. Mucosal hemangiomas are typically soft, moderately well-
circumscribed, painless masses with a red or blue colouration. Adult-onset hemangiomas tend to slowly enlarge over a period of

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Table 1
Treatment Modalities in head and neck SCH.
Sl. Author Year Location Age(in Provisional diagnosis Initial treatment Final treatment Follow Recurrence
No years)/ up
sex period

1 Singh et al. 2018 Left Masseter 30/ Vascular malformation 2 sessions of Surgical excision 24 ABSENT
[8] muscle Male Intralesional 3% months
Sodium Tetradecyl
Sulphate injection
2 Murakami 2018 Upper lip 41/ Haemangioma 2 sessions of Surgical excision 24 ABSENT
et al.[7] Female intralesional months
ethanol injections
3 French et al. 2016 Dorsum of 52/ NA Surgical excision – 24 ABSENT
[12] tongue Female months
4 Gbolahan 2015 Orbit 9/ NA Surgical excision – 21 ABSENT
et al.[13] Female months
5 Chavva 2015 Floor of 33/ Minor salivary gland Surgical excision – 6 ABSENT
et al.[14] mouth Male tumor months
6 Higashano 2014 Right Occiput 37/ Arteriovenous Marginal excision – NA NA
et al.[15] Female malformation
7 Minagawa 2011 Left temporal 67/ Highly vascularized Incisional Biopsy Surgical excision NA NA
et al.[11] muscle Female soft tissue tumor with with Temporal
malignant potential muscle resection
through zygomatic
arch osteotomy
8 Tosios et al. 2008 Upper lip 29/ Mucocele Surgical excision – 3 years ABSENT
[6] Female
9 Shehaan 2007 Buccal 44/ Benign mesenchymal Surgical excision – 13 ABSENT
et al.[16] mucosa Male tumours and salivary months
gland tumours
10 Ide et al. 2004 Palate 55/ Pleomorphic adenoma Surgical excision – 1 year ABSENT
[17] Male
11 Tosios et al. 1995 Mandibular 12/ Haemangioma/ Surgical excision – Lost to
[18] buccal fold Female Pyogenic Granuloma follow
up
12 Scott and 1988 Ear 70/ Cavernous Incisional biopsy – NA NA
Rosai[10] Male haemangioma/
Dermatofibroma/
Kaposi’s sarcoma
13 Baron et al. 2002 Lateral nasal 1.5/ NA Surgical excision Surgical excision 2 years PRESENT
[19] side wall Male with frozen section
14 Lade et al. 2005 Posterior 25/ NA Surgical excision – 4 years ABSENT
[20] pharyngeal Male following elective
wall tracheotomy
15 Cai et al. 2013 Lower lip 34/ Maffucci syndrome Surgical excision – NA NA
[21] Female
Present case Buccal 10/ Dental abscess Surgical excision – 18 ABSENT
Mucosa Male months

NA- Not Available, - Not performed.

months or years while congenital lesions tend to keep pace with body growth. Superficial lesions blanch under pressure and deeper
lesions are dome-shaped and seldom blanch. They are seldom encapsulated. Typically a central feeder vessel is present with excessive
veins and capillaries in a focal area of connective tissue.
The spindle cell variant of haemangioma has atypical features. Rarely do these lesions develop in the head and neck including the
oral cavity. In the periphery of many lesions, there are thick-walled, irregular blood vessels resembling a localized arteriovenous shunt.
In the present reported case, there was an absence of adhesion to a major vessel on contrast-enhanced computed tomography as well as
intraoperatively, thus contributing evidence to the pathogenesis of SCH as a low flow reactive vascular proliferation.
Although cutaneous SCH lesions appear to be associated with recurrences, local excisions in oral SCH provides satisfactory results.
Surgical excision appears to be the standard treatment for SCH.

5. Conclusion

SCH has to be considered in the differential diagnosis of oral cavity vascular tumours to avoid misdiagnosis as a more aggressive
tumour.

Acknowledgements

The authors acknowledge the skilful assistance of Dr. Vidya G Doddawad.

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