CASE 16442 Published on 12.09.2019

Spindle cell haemangioma of the foot

Section

Musculoskeletal system

Case Type

Clinical Cases

Authors

Mats Van den broecke 1, David Creytens 2, Gwen Sys 3, Koenraad Verstraete 4

1 Universiteit Gent, BELGIUM

2 Professor, Ghent university hospital, department of pathology

3 Professor, Ghent university hospital, department of orthopedic surgery

4 Professor, Ghent university hospital, department of radiology

Patient

35 years, male

Categories
Area of Interest Musculoskeletal soft tissue ; Imaging Technique MR
Clinical History

We report a case of a 35-year-old male patient who experienced a painless mass at the dorsal aspect of the second web space of      the left forefoot for two years. The patient mentioned only a little discomfort when wearing narrow cycling shoes. Clinical investigation revealed an unspecific soft tissue mass at the dorsal aspect of the proximal phalanx of the second and third toe. There was no numbness and Tinel’s sign was negative.

Imaging Findings

Magnetic resonance imaging revealed a lobulated mass at the dorsum of the forefoot between the proximal phalanges of the second and third toe. [Fig. 1] The lesion contained tangles of tortuous blood vessels, no phleboliths were found. The mass appeared slightly hyperintense to muscle on T1 images and hyperintense on T2 images. Dynamic contrast-enhanced imaging showed strong enhancement during the first pass of contrast medium and a continuous further but slower enhancement in the second phase. [Fig. 2] Based on these imaging findings a vascular tumuor (haemangioma) was suspected and biopsy was performed.

Histological assessment revealed a relatively well-circumscribed vascular lesion composed of dilated thin-walled cavernous type vascular channels, lined by a thin layer of endothelium without cytonuclear atypia or multilayering. A little phlebolith was found in these vessels. More central in the lesion solid spindle cell areas with a slit-like growth pattern were observed.

Discussion

Spindle cell haemangioma (SCH), also known as spindle cell haemangioendothelioma, is an uncommon vascular tumour that was first described in 1986 by Weiss and Enzinger. SCH was initially described as a type of haemangioendothelioma of intermediate or low-grade malignancy because the tumour was thought to have limited malignant potential and to have frequent local recurrence. [1] Since then various investigations have been conducted, including immunohistochemical studies, revealing that these lesions are benign. [2]

As in our case, this tumour is commonly detected in the superficial soft tissue of the distal parts of the extremities. The head, neck, chest and abdomen have also been reported as primary sites of origin in a minority of cases. They most often have an indolent course, but can become painful and disfiguring. It usually occurs in young adults and both sexes are equally affected. [1,2] There is usually no associated discoloration of the overlying skin making initial clinical suspicion of a vascular lesion difficult. [3]

SCH can present as a solitary lesion or can be multifocal. Multifocal SCH's have been associated with Maffucci syndrome, Ollier disease, lymphedema, Klippel Trenaunay syndrome and early onset varicose veins. [2]

On magnetic resonance imaging haemangiomas are typically well-defined, lobulated and heterogeneous with no features of local invasion. On T1 the overall signal is often intermediate to slightly higher than muscle and high-signal intensity tends to dominate on T2-weighted images. The lesion shows marked enhancement after contrast medium administration.

The differential diagnosis of SCH includes other types of (cavernous and capillary) haemangiomas, Kaposi's sarcoma, kaposiform haemangioendothelioma and intravascular papillary endothelial hyperplasia. The combination of SCH's cavernous spaces with organised thrombi, plump endothelial cells, multitude of spindle cells and minimal mitotic activity can histologically differentiate it from the other diagnoses. [2] There are also differences in the immunohistochemical staining patterns, including human herpesvirus 8 (HHV-8) positivity in Kaposi’s sarcoma, which is negative in SCH. [4]

Preoperative planning should include an MRI to guide biopsy and to assess involvement of deeper structures. [2]

The standard treatment is wide surgical excision. Limited excisions almost certainly reoccur, but there is no consensus on the incidence of reoccurrence. When deciding how to treat each individual case, the slow growth of the tumour and the morbidity that the surgery may cause must be taken into consideration. [2] In our case local surgical excision was performed and no recurrence was reported until 9 months after surgery.

Written informed patient consent for publication has been obtained.

Differential Diagnosis List
Spindle cell haemangioma
Final Diagnosis
Spindle cell haemangioma
Case information
URL: https://www.eurorad.org/case/16442
ISSN: 1563-4086