Spindle Cell Hemangioma of Lower Lip: The Fourth Case Report with Review of Literature

Abstract

Background

Spindle cell hemangioma previously known as spindle cell hemangioendothelioma is a benign vascular tumour with rare presentation in head and neck. Presentation in lip is even rarer with three cases reported previously.

Method

This report describes a case of spindle cell hemangioma presented as an asymptomatic growth on lower lip of a 32-year-old male. Clinicopathological characterization of this case along with previously reported 15 cases of spindle cell hemangioma of head and neck were conducted.

Result

Microscopic evaluation shows a well-circumscribed vascular neoplasm of spindled and epithelioid endothelial cells. Large ectatic thin-walled vascular spaces were seen engorged with RBCs. The neoplasm was CD31 positive. Slight predilection for female gender and young age were observed. Minimal possibility of recurrence was also observed.

Conclusion

Spindle cell hemangioma needs to be considered in the differential diagnosis of vascular tumours of head and neck to avoid misdiagnosis of aggressive vascular neoplasms.

Introduction

Hemangioendothelioma has encompassed multiple vascular proliferations including both benign and malignant neoplasms. With the inception of the terminology ‘hemangioendothelioma’, Mallory in 1908 [1] included all proliferations that arose from endothelial cells. Stout and Kauffman [2] described hemangioendothelioma as a small group of vascular lesions having low metastatic potential and high tendency for local persistence. Weiss and Enzinger [3] proposed that the term hemangioendothelioma should be restricted to those vascular neoplasms showing a borderline biological behaviour, intermediate between benign hemangiomas and highly malignant angiosarcomas.

Spindle cell hemangioendothelioma is one of the several variants of hemangioendothelioma. Weiss and Enzinger [3] described this entity in 1986 as a vascular lesion with intermediate malignancy. However, there exists controversy regarding the hamartomatous, reactive or neoplastic nature of this lesion. Fletcher et al. [4] proposed the reactive nature of the lesion associated with local abnormality of blood flow which may be congenital or acquired. Imayama et al. [5] used ultrasound analysis to suggest the benign nature of the lesion. Perkins and Weiss [6] through a large case series stressed upon the fact that spindle cell hemangioma is a vascular lesion having least metastatic potential but marginal recurrence potential. These evidences have relabelled the lesion as ‘spindle cell hemangioma’ (SCH) which is described as a benign neoplasm or a vascular malformation in which secondary episodes of thrombosis may develop. The usual location of SCH is acral regions. Reports of presentation in the head and neck area are scarce. SCH in lip has been reported in three occasions making this case report as the fourth in English literature. This report, along with presenting the rare case, would also thoroughly review 15 cases of SCH of head and neck regions which are reported till date.

Case Report

A 32-year-old male presented with an asymptomatic firm erythematous and lobulated growth on lower lip (Fig. 1). The patient did not have any other significant systemic diseases. With the clinical differential diagnosis of benign salivary gland lesions and benign mesenchymal tumour, it was excised. Size of the excisional specimen was 2.5 cm × 1.5 cm × 1 cm. Microscopic findings (Fig. 2) of the processed tissue revealed a well-circumscribed lesion. Neoplastic mass showed a lobular growth pattern. There were numerous ectatic thin-walled vessels lined by plump spindle-shaped endothelial cells. Large venous spaces were seen filled with erythrocytes. Clusters of epithelioid cells were also seen. Few atypical endothelial cells and mitotic figures were also observed. Immunohistochemical marker for vascular markers, CD31, was positive in endothelial cells lining the miniature vessels and cavernous spaces as well as spindle cells (Fig. 3). A diagnosis of spindle cell hemangioma was made. No evidence of recurrence was seen after 8 months of follow-up.

Fig. 1.

Asymptomatic lobulated growth on lower lip

Fig. 2.

(Haematoxylin and eosin stain, 10X) well-circumscribed lesion showing a lobular growth pattern of numerous ectatic thin-walled vessels lined by plump spindle-shaped endothelial cells and clusters of epithelioid cells

Fig. 3.

Immunohistochemical marker CD31 was positive in endothelial cells lining the miniature vessels and cavernous spaces as well as spindle cells

Discussion

The rare occurrence of SCH in head and neck region may be attributed to lack of familiarity with the histopathologic features of SCH and overlapping features with hemangiomas. The present case would be the 16^th case among all head and neck SCH reported till date in English literature. Among SCH presented on lip, this case would be the fourth one. Considering the paucity of this lesion, we have presented the case in detail as well as critically reviewed the previously presented 15 cases (Table 1).

Table 1.

Cases of SCH head and neck reported till date

Sl. no	Author/year	Site	Age/gender	Immunohistochemistry	Size of lesion (in cm)	Follow-up
1.	Scott and Rosei 1988 [7]	Ear	70/M	NR	NR	NR
2.	Tosios 1995 [8]	Mandibular vestibule	12/F	HAM56 and vimentin positive in lining endothelial cells Factor Vlll positive in endothelial cells and round vacuolated cells Vimentin positive in epithelioid cells and majority of spindle cells PCNA positive in less than 3% of the tumour cells	1	LF
3.	Baron 2002 [9]	Lateral nasal side wall	17 months/M	NR	1 × 1	24 FOR
4.	Ide 2004 [10]	Palate	55/M	CD31 positivity	1.2	NR
5.	Lade 2005 [11]	Posterior pharyngeal wall	25/M	NR	6	48 FOR
6.	Sheehan 2007	Buccal mucosa	44/M	CD31 and CD34 positive.	1.2	NR
7.	Tosios 2008 [12]	Upper lip	29/F	CD34 and factor-VIII positive in endothelial cells and epithelioid cells Smooth-muscle actin positive in endothelial cells and spindle cells Few CD68 positive cells were seen in the solid areas	1	36 FOR
8.	Minagawa 2011 [13]	Temporal muscle	67/F	NR	4	24
9.	Cai et al. 2013 [14]	Lower Lip	34/F	Vimentin and α-SMA positive and CD31, CD34 and D2-40S-100 protein, keratin (AE1/AE3) and CK19 were negative in spindle cell component	2	NR
10.	Higashino 2014 [15]	Occipital region	37/F	NR	2.5	NR
11.	Gbolahan 2015 [16]	Orbit	9/F	CD34 positive	8 × 6	21 FOR
12	Sapariya 2015 [17]	Thyroid	30/F	Thyroid follicles with thyroglobulin positivity Endothelial cells with strong positivity for CD 34 and factor-VIII Spindle cells positive for vimentin and negative for thyroglobulin, CD 34 and factor-VIII	4.3 × 2.8	NR
13.	Chhava 2015 [18]	Floor of mouth	33/M	CD-31, CD-34 positive in spindle cells and endothelial cells	1 × 1.5	6 FOR
14.	French 2016 [19]	Tongue	52/F	CD31 positive in spindle cells and endothelial cells	2	24 FOR
15.	Murakami 2018 [20]	Upper lip	41/F	CD34 and SMA positive in most endothelial cells and occasional spindle cells	3	24FOR
16.	Present case 2020	Lower lip	32/M	CD 31 positive in endothelial cells and occasional spindle cells.	2.5	8FOR

NR not reported, FOR Free of recurrence, LF Lost to follow-up

A critical research on pathophysiology of SCH concluded that this may be regarded as a benign vascular neoplasm or malformations similar to angiomatosis in which alterations in blood flow possibly explain the consequential features [5, 21]. Site of abnormal vasculature [4] and injury [5] may predispose initiation of SCH. Areas of diminished blood flow result in vascular collapse with formation of cellular zones, and areas of vascular engorgement with stasis promote thrombosis and organization. Evidencing least metastatic potential, Perkins et al. [6] has suggested that this should be labelled as spindle cell hemangioma for solitary lesions and spindle cell hemangiomatosis for multifocal lesions.

Clinical differential diagnosis in all cases including this was benign salivary gland tumours and other mesenchymal tumours. Vascular lesions were not suspected in any of the cases. The reason may be submucosal location of the lesion [22]. Largest reported size among 15 cases was that of an SCH orbit, 8 cm, and nine out of sixteen cases of SCH occurred in female. However, three cases [12, 14, 23] reported in lip were in female with size ranging from 1 to 3 cm, while this case was in a male with a lesion size of 2.5 cm. Although SCH has a propensity for patients in the second decade or less than that, it may occur at any age [4, 6]. However, out of 15 reported cases eleven patients were in fourth decade and above.

Microscopic evaluation of spindle cell hemangioma describes a well-circumscribed lesion with nodular proliferations of spindled endothelial cells. Thin walled dilated veins supported by endothelial cells are the constant finding. Occasional phlebolith and/or thrombi are also seen which was not observed in our case. In addition to the spindle cells, another group of cells with round to ovoid morphology having vacuolated cytoplasm arranged in solid aggregates are also seen. The histological differential diagnosis centres on several vascular neoplasms. The presence of epithelioid cells raises the possibility of epitheloid hemangioendothelioma which is another vascular neoplasm of intermediate malignancy and a rare occurrence in head and neck [23, 24]. However, lack of infiltrative growth pattern and myxohyaline matrix differentiates spindle cell hemangioma from epithelioid hemangioendothelioma. Kaposi’s sarcoma and cavernous hemangioma are other possible diagnosis for such microscopic findings. Although spindled areas may be mistaken for Kaposi’s sarcoma, lack of features like infiltrative growth, nuclear atypia, higher numbers of mitotic figure and epitheloid endothelial cells excludes Kaposi’s sarcoma. Immunohistochemistry for human herpes virus may help diagnosing certain cases of Kaposi’s sarcoma. However, in the settings of acquired immunodeficiency syndrome and few non-immunocompromised patients, Kaposi’s sarcoma has to be considered as a differential diagnosis. Angiosarcoma can occasionally have spindled morphology. However, lack of infiltrative growth pattern, nuclear atypia and high numbers of mitotic figures again are the exclusion criteria. Lobular capillary hemangioma, a variant of pyogenic granuloma may also need careful exclusion from SCH. Presence of dense inflammatory cells and absence of cavernous spaces may qualify the lesion as pyogenic granuloma [25].

Most cases of cutaneous hemangiomas are sporadic in nature except few which are associated with Mafucci’s syndrome [26], Klippel–Trenaunay syndrome [4], lymphedema [4], early onset of varicose veins and superficial cutaneous lymphatic malformations [4]. Except Cai’s [14] case of SCH on lip, no syndromic association was observed. Recurrence rate is high in cutaneous spindle cell hemangiomas. In fact, Perkins et al. [6] reported 58% recurrence in a series of 78 cases which is attributed to incomplete excision or contiguous spread along or multifocal involvement of a vessel. Reviewing the cases demonstrated no recurrence in eight cases having followed up. Therefore, recurrence rate of head and neck SCH may be far less compared to cutaneous SCH. However, this evidence based upon handful of cases should not defend patients with head and neck SCH from being followed up.

In order to prevent the misinterpretation of a more aggressive vascular tumour, spindle cell hemangioma should be considered as a differential diagnosis among soft tissue neoplasm of head and neck. The clinical and histopathologic features described above may help clinician and pathologist, respectively, to diagnose SCH.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest.