Spindle cell hemangioma (SCH) is a unique benign vascular lesion. We present a case of SCH in the upper lip of a 41-year-old woman. A submucosal nodular mass 30 × 20 mm in size was observed in the left upper lip. The mass developed 5 years earlier and enlarged after repeated ethanol injections. The mass was elastic firm, mobile, bluish in color, and well demarcated in magnetic resonance imaging. Under the clinical diagnosis of hemangioma, surgical excision was performed under local anesthesia. Microscopically, the lesion was composed of irregular cavernous spaces and multiple solid cellular areas. Cavernous spaces were filled with a mix of erythrocytes and organizing thrombi. The solid areas showed proliferation of spindle-shaped cells arranged haphazardly or in short interlacing fascicles. Immunohistochemically, most cells strongly reacted with vimentin. CD31, CD34, factor VIII, smooth muscle actin, and Wilms tumor-1 reacted with endothelial cells lining the cavernous spaces. The cells within solid areas consisted of mixed cell population with variable reaction for the markers except for factor VIII. From these findings, the diagnosis of SCH was made. Two years after surgery, no recurrence was noted. A review of SCH in the head and neck region is made.
Spindle cell hemangioma (SCH) is a unique vascular lesion, which almost exclusively affects the dermis and subcutaneous tissues of the distal extremities. Perkins and Weiss named a solitary single tumor SCH and multifocal lesions crowded within the same region “spindle cell hemangiomatosis” [
We present an additional case in the mucosa of the upper lip of a 47-year-old woman and review of the literature on SCH of the head and neck region.
A 41-year-old woman presented with a mass on the left upper lip and difficulty in pronunciation. The mass developed after she bit the upper lip 5 years earlier. The volume of mass was not reduced; however, the patient complained of pain. One year after the development, she visited an otolaryngologist. The mass was diagnosed as mucocele and aspirated. However, only blood was aspirated from this lesion, and the lesion’s size was not reduced. Two years after aspiration, the size of mass increased, and she visited a plastic surgeon. The lesion was diagnosed as hemangioma by magnetic resonance imaging (MRI). Ethanol was injected into the lesion twice. Although the lesion was slightly reduced at the first injection, it did not change at the second. She consulted a dental clinic two years after the second injection and then was referred to our department.
Oral examination revealed a circumscribed submucosal single nodule, approximately 30 × 20 mm in size in the left upper lip. The overlying mucosa was smooth, with bluish discoloration. On palpation, the nodule was elastic firm and mobile (Figure
Clinical finding before surgery.
MRI findings. (a) T1-weighted image. (b) T2-weighted image.
Excised tumor: lateral view.
Clinical finding 6 months after surgery.
The size of excised the lesion was 30 × 20 mm. The specimen had reddish brown surfaces covered by thin-walled capsule including the mucosa of partial lower lip and inflow blood vessels which were well demarcated. Microscopically, the lesion was a well-circumscribed mass surrounded by fibrous connective tissue and showed a variety of cellularity imparting a lobular architecture in low power (Figures
(a) Well-defined submucosal mass with cavernous spaces, solid areas, and number of thrombi. (hematoxylin and eosin stain, original magnification ×5). (b) Irregular cavernous spaces lined by flat endothelial cells. (hematoxylin and eosin stain, original magnification ×100). (c) The spindle-shaped cells in solid areas. (hematoxylin and eosin stain, original magnification ×100).
Immunohistochemically, most endothelial cells lining the cavernous spaces, spindle cells within solid areas, and epithelioid cells within both areas strongly reacted with vimentin (Figures
(a) Cavernous area (vimentin, original magnification ×100). Most endothelial cells around blood vessels in cavernous area strongly reacted. (b) Solid area (vimentin, original magnification ×200). Spindle cells and epithelioid in solid area are strongly positive.
(a) Cavernous area (CD34, original magnification ×100). Most endothelial cells around vessels in cavernous space are positive. (b) Solid area (CD34, original magnification ×200). Spindle cells in solid area are focally positive.
(a) Cavernous area (SMA, original magnification ×100). Most endothelial cells around vessels are positive. (b) Solid area (SMA, original magnification ×200). Spindle cells in solid area are focally positive.
(a) Cavernous area (WT-1, original magnification ×100). Endothelial cells are strongly positive in cavernous area. (b) Solid area (WT-1, original magnification ×200). Spindle cells in solid area are focally positive.
SCH has been diagnosed as various entities including mucocele, hemangioma, pyogenic granuloma, synovial sarcoma, and enchondroma (Table
Main clinical features of 13 cases of spindle cell hemangioma of the head and neck.
Author | Age | Gender | Duration (months) | Site | Maximum size (cm) | Clinical diagnosis | Follow-up (months) |
---|---|---|---|---|---|---|---|
Tosios et al. [ |
29 | Female | 12 | Upper lip | 1 | Mucocele | 36FOD |
Scott and Rosai [ |
70 | Male | RP | Ear | NA | Cavernous hemangioma | NA |
Tosios et al. [ |
12 | Female | NA | Mandibular vestibule | 1 | Hemangioma or pyogenic granuloma | LFU |
Baron et al. [ |
1.5 | Male | 13 | Lateral nasal side wall | 1 | Hemangioma | 24 recurrence |
Ide et al. [ |
55 | Male | 3 | Palate | 1.2 | Pyogenic granuloma | 12FOD |
Lade et al. [ |
25 | Male | 6 | Posterior pharyngeal wall | 6 | Synovial sarcoma | 48FOD |
Sheehan et al. [ |
44 | Male | NA | Buccal mucosa | 1 | Vascular tumor | 13FOD |
Chavva et al. [ |
33 | Male | 8 | Oral floor | 1 | Minor salivary gland tumor | 6FOD |
Cai et al. [ |
34 | Female | 24 | Lower lip | 2 | Enchondromas (Maffucci syndrome) | NA |
Minagawa et al. [ |
67 | Female | 4 | Temporal muscle | 4 | Vascular tumor | 24FOD |
French et al. [ |
52 | Female | 6 | Tongue | 2 | Polypoid haemangiomatous lesion | 24FOD |
Gbolahan et al. [ |
9 | Female | 12 | Orbit | 8 | Tumor in the inferolateral orbit | 21FOD |
Present case | 41 | Female | 60 | Upper lip | 3 | Hemangioma | 24FOD |
RP: recent presentation; NA: not available; FOD: free of disease; LFU: lost to follow-up.
Approximately 10% of cases are associated with other developmental anomalies or syndromes, including early-onset varicose veins, lymphedema, Klippel-Trenaunay-Weber syndrome, Maffucci syndrome, epithelioid hemangioendothelioma, and superficial cutaneous lymphatic malformations [
SCH is relatively uncommon. Only 12 cases have been reported in the soft tissues of the head and neck region (Table
Histologically, SCH shows variable cellularity imparting a lobular architecture on low power. Large, ectatic, vascular spaces lined by flattened endothelial cells are frequently present. The cavernous spaces may contain calcified thrombi, referred to as phleboliths. Between the dilated vascular spaces, there is proliferation of spindle cells composed of endothelial cells, pericytes, and fibroblasts. The endothelial cells often have focal epithelioid features and show focal cytoplasmic vacuolization. Mitotic activity and atypia are low [
SCHs resemble the features of Kaposi’s sarcoma, such as male predominance and occasional multifocal growth. But Kaposi’s sarcoma rarely contains cavernous vessels with thrombi and phleboliths and lacks epithelioid cells, and their spindle cells react for the endothelial marker CD34. On the other hand, SCH does not present the hyaline globules seen in Kaposi’s sarcoma or express human herpes virus 8 latent nuclear antigen-1 [
Immunohistochemically, the cells lining the cavernous space and vacuolated epithelioid cells are positive for vimentin, CD31, CD34, and factor-VIII-related antigen, supporting the endothelial nature [
Immunohistochemical analysis of the SCH cases in head and neck in the literature.
Author | CD34 | CD31 | VIMENTIN | SMA | Factor VIII | CD68 | S100 | EMA | AE1/AE3 | D2-40 | WT-1 | |||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
C | S | C | S | C | S | C | S | C | S | C | S | C | S | C | S | C | S | C | S | C | S | |
Tosios et al. [ |
+ | + | + | + | + | + | + | + | ||||||||||||||
Scott and Rosai [ |
+ | + | + | + | ||||||||||||||||||
Tosios et al. [ |
+ | + | ||||||||||||||||||||
Ide et al. [ |
+ | − | + | − | + | + | + | − | ||||||||||||||
Sheehan et al. [ |
+ | + | + | + | ||||||||||||||||||
Chavva et al. [ |
+ | + | + | + | ||||||||||||||||||
Cai et al. [ |
+ | − | + | − | + | + | + | + | − | − | − | − | + | − | ||||||||
French et al. [ |
+ | + | ||||||||||||||||||||
Gbolahan et al. [ |
+ | + | ||||||||||||||||||||
Present case | + | + | + | + | + | + | + | + | + | − | − | − | − | − | − | − | − | − | − | − | + | + |
C: cavernous area; S: solid area; (+): immunopositivity; (−): immunonegativity.
Surgical excision is the standard treatment for SCH, with excellent prognosis. Although more than 50% of patients may develop new lesions in the same anatomic region several years after initial excision [
SCH is considered an unusual vascular lesion in the oral cavity. Awareness of clinical, histologic, and histochemical features is important for differential diagnosis of the lesion from more aggressive vascular tumors to avoid unnecessary treatment. Although the common size of SCH is less than 20 mm, the size of it is guessed to be increased to more than 30 mm by frequent stimulation. In the diagnosis of SCH in head and neck, it is considered CD 31, CD 34, vimentin, and SMA are beneficial. We suppose the surgical removal of SCH is appropriate treatment with good clinical outcome.
The authors declare that they have no conflicts of interest.
The authors thank Dr. Shuhei Yamashita, the president of Hattori memorial hospital, for perioperative management of the patient and Dr. Motokatsu Tsuyuki, the vice president of Hattori memorial hospital for histological examination.