SOFT TISSUE HEMANGIOMA

MAGDY M ABDEL-MOTA'AL M.D., Orthopaedic. Research Fellow.

ROBERT P STANTON M.D., Attending Pediatric Orthopaedic Surgeon

April 11, 1996

CLINICAL CASE PRESENTATION

ORTHOPAEDIC DEPARTMENT

THE ALFRED I. DUPONT INSTITUTE

WILMINGTON, DELAWARE

CASE HISTORY:

AM is a 6 year old female who presented to Outpatient Clinic with mass in her left foot.

PAST MIDICAL HISTORY

On 6-2-95: Excision biopsy of mass of the left foot. Intra-operative finding revealed a fatty vascular tumor at the planter aspect of the medial left foot which was infiltrative and adherent to the medial plantar artery the medial planter nerve, the flexor hallucis longus as well as the flexor tendon of the second, third, and fourth toes. The pathology report was a benign lesion.

On 11-7-95: Lower extremity arteriogram with transcatheter embolisation of left foot arteriovenous malformation.

PHYSICAL EXAMINATION:

There was firm tender mass within the planter arch area of the left foot. the mass was approximately 6.5 x l7 cm. It involved virtually the entire arch of the foot. there was a well healed scar that runs longitudinally over the medial arch portion of the foot.

MRI

Suggestive of a vascular hemangioma.

TREATMENT:

Mass excision. The abductor hallucis muscle was fibrosed. There was a vascular mass deep and lateral to the abductor hallucis muscle. The mass and the muscle were excised after careful dissection of the medial plantar nerve.

DISCUSSION: HEMANGIOMA

These benign vascular processes has been variously thought to represent hamartomatous malformations of normal vascular tissues or to represent benign neoplasm.

Age: They arise in childhood and adolescence, and although they persist indefinitely, they rarely first become apparent in later adult life.

Site: They are most common in the skin and subcutaneous tissues, appear often in the deep fascia and muscle and are exceptionally rare in bone.

Clinical presentation:

1. The superficial lesions presents as a painless mass that has a distinctive bluish tinge. They are soft and easily compressed.
2. The deep lesions present because of intermittent but persistent discomfort They seldom have any physical signs.

Histology:

1. The capillary form is composed of masses of capillaries, communicates freely with the systemic circulation and may be quite red in appearance.
2. Cavernous hemangiomas are composed of large, dilated, tortuous, thin walled endothelial cavities that when lying superficially, appear blue in color. They have little anastomosis with the systemic circulation. They intermittently increase and decrease in size, and have episodes of significant tenderness associated with episodes of clotting.

Staging Studies:

1. X-ray:
• Capillary hemangiomas seldom show even a discernible soft tissue mass.
• Cavernous hemangiomas are frequently visible because of areas of calcification within them. These calcification are phleboliths caused by clotting in the cavernous cavities.
2. Isotope scans:
• Capillary hemangiomas may show a modest increase consistent with the increased blood supply, but scans are seldom indicated for surgical planning.
3. Angiograghy:
• Angiograghy readily and accurately identifies the pattern and the extent of the hemangiomatous neovasculature.
4. CT scan:
• CT scan of capillary hemangiomas are unrewarding.
5. MRI:
• MRI defines the internal characteristics of hemangiomas and clearly distinguishes it from adjacent muscles.

SURGICAL STAGES:

1. In children and adolescents, the majority of hemangiomas are benign, active stage 2 lesion.
2. Occasionally they will permeate through all the tissue barriers in an aggressive stage 3 fashion.
3. Hemangiomas do not undergo malignant transformation.

TREATMENT:

1. Intracapsular excision is often followed by recurrence as the lesion rarely forms a pseudocapsule. It is most often diffusely infiltrative.
2. In theory extracapsular excision should provide a definitive procedure for stage 2 hemangioma, but it is impossible to dissect between the periphery of the lesion and the normal tissues without inadvertent transsectoin of occult extensions.
3. Wide excision does not always lead to complete cure, and is often injustified due to excessive morbidity.
4. Cryosurgery.
5. Injection with sclerosing agents.

DISCUSSION: MANAGEMENT OF SOFT TUMOR OF THE FOOT.

Special attention is required for tumor the foot because:

1. The foot is composed of a relative higher concentration of lymphatics and it also contains numerous tendons passing through synovial sheaths which lie adjacent to bone and neurovascular structures. Therefore the distribution of the tumors in the foot is differ from tumors arising elsewhere in the musculoskeletal system.
2. There is little muscular mass to permit adequate surgical margins of resection in cases of pedal tumors.
3. The fascial planes between the rays that leads to the periarticular soft tissues of the mid-foot have no barrier to proximal or distal extension and are extra-compartmental. Extension proximally into the leg from lesions of the foot is uncommon

CLINICAL PRESENTATION:

Lesions about the foot generally present early because:

1. The thin soft tissue covering: makes relatively small masses easily palpable.
2. Pain and discomfort is produced by mechanical disruption of the function of the tightly-bound gliding mechanisms

INCIDENCE:

Kirby (1989) analyzed the cases of 83 patients who had a soft tissue tumor in the foot. He found that 72 (87%) of the lesions were benign with ganglion cyst and planter fibromatosis being the most common. Eleven (13%) were malignant tumors, 5(45%) of which were malignant sarcomas.

Staging Studies:

1. X-ray:
• Soft tumors of the foot may present with one or more of the following: visible soft tissue mass calcification, or secondary osseous involvement.
2. CT scan:
• Sections 1.5 -2.0 mm in thickness should be taken through the area of concern to define the complex anatomy. The small size of the scan with its lack of resolution and abscence of significant fat planes in the soft tissue make CT scan of limited value about the foot.
3. MRI:
• MRI offers better details of soft tissue tumors and their relationship to surrounding structures.
4. Angiography:
• Angiography is rarely needed unless the surgeon is dealing with a vascular lesion.

BIOPSY:

Incisions for biopsy are influenced about the foot by the presumptive clinical diagnosis:

1. Lesions that appear benign are best approached with incisions that match the anatomical creases and that avoid the weight bearing surfaces.
2. Malignant lesions should be approached through longitudinal incisions, bearing in mind the approaches to be used in subsequent wide or radical local procedures

A marginal excision for diagnosis of a malignant lesion is much more likely to cause distal extension than is a carefully controlled incisional biopsy.

SURGICAL STAGES:

• Stage IA, Grade Low, Site Intracompartmental.
• Stage IB, Grade Low, Site Extracompartmental.
• Stage IIA, Grade High, Site Intracompartmental.
• Stage IIB, Grade High, Site Extracompartmental.
• Stage III, Grade Any, Site Any.

SURGICAL LOCATIONS:

• Intracompartmental Intraosseous, Extracompartmental Soft tissue extension
• Intracompartmental Intra-articular, Extracompartmental Soft tissue extension
• Intracompartmental Superficial, Extracompartmental Deep
• Intracompartmental Juxtacortical, Extracompartmental Intraosseous
• Intracompartmental Intrafascial compartments, Extracompartmental Extrafascial compartments

TREATMENT:

1. Stage I and 2 benign lesions are treated by marginal excision.
2. Stage 3 benign and stage I malignant soft tissue lesions need wide excision. In this setting it is rare that a lesion dose not involve the underlying bone with reactive tissues and frequently lesions extend through the large vascular perforations into the bone itself. Often an en block wide excision of skin, subcutaneous tissue, tendons, and parts of various bones and joints leads to more disability than a partial amputation that would achieve the same wide margin.
3. Stage II soft-tissue lesions in the foot require an amputation to achieve a radical margin.

REFERENCES:

1. Cohen EK, Kressel HY, Preosio T, MR imaging of soft tissue hemangioma: correlation with pathologic finding. AJR 1988; 150: 1079-1081.
2. Enneking WF; Musculoskeletal Tumor Surgery. Churchil, New York, Edinburgh, London, and Melbourne 1983.
3. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin. Orthop. 1980; 153: 106.
4. Keigley BA, Haggar AM, Gaba A, Ellis BI, Froelich JK, Wu KK. Primary tumors of the foot: MR image. Radiology 1989.
5. Kirby EJ, Shereff MJ, Lewis MM. Soft-tumor and tumor-lite lesion of the foot. An analysis of eighty-three cases. J Bone Joint Surg 1989; 71 (4):621-626.
6. Lane JM, Rosenthal HG. Pediatric foot tumors in; The Child's Foot and Ankle edited by J.C.Drennan, Raven Press, Ltd. New York 1992.
7. Seale KS, Lange TA, Manson D, Hackbarth DA. Soft tissue tumor of the foot and ankle. Foot Ankle 1988; 9(l): 19-27.