Spindle cell haemangioma in head and neck: Report of an uncommon vascular lesion and review of treatment modalities till present


  • SCH is a rare vascular tumour, even rarer in head and neck region.

  • SCH is reactive vascular proliferation rather than an angiosarcoma.

  • Unique pathophysiology influences SCH’s surgical management.

  • Local excision has provided best results for SCH.


Spindle cell haemangioma is an uncommon vascular lesion, often affecting the dermis and subcutaneous tissues of distal extremities. 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.


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[ ]. 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.

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.

Fig. 1
Clinical appearance at presentation.

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 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 submucosal 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.

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

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 vacuolations ( Fig. 3 A 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. 3 C). The lesion was hence diagnosed as SCH.

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

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[ ].

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[ ]. Imayama et al. observed in their patient, nodules that showed initial rapid painful development followed by long periods of quiescence[ ]. 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 recurrences 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[ ]. 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[ ]. Solitary form although appearing sporadically may arise after repeated traumatic injury such as repeated injections[ ] and repeated surgical insults. The tumour occurs at all ages with equal sex prevalence[ ]. 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 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[ ].

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[ ]. 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, haemangioma, 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 surgically excise the lesion under general anaesthesia[ ]. Murakami et al. surgically excised the lesion on the lower lip of their patient after failed ethanol intralesional injections [ ]. Scott et al. performed an incisional biopsy for a suspected cavernous haemangioma on the ear which was later diagnosed as SCH[ ]. 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[ ]. 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 ).

Aug 8, 2020 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Spindle cell haemangioma in head and neck: Report of an uncommon vascular lesion and review of treatment modalities till present

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