Orthopedic Surgery for Hemangioma Treatment & Management
- Author: Brian J Kistler, MD; Chief Editor: Harris Gellman, MD more...
Observation is appropriate for asymptomatic or mildly symptomatic hemangiomas of skeletal muscle and bone. If symptoms cannot be managed adequately by activity modification and nonnarcotic analgesics, further treatment may be considered. Embolization may be used to provide symptomatic relief of intramuscular hemangiomas.
When surgical excision is planned, embolization also may be used preoperatively to decrease intraoperative blood loss and postoperative recurrence. Excision of symptomatic intramuscular hemangiomas can provide permanent relief. However, because complete excision is required for long-lasting satisfactory results, this treatment option generally is restricted to hemangiomas contained within a single muscle belly. Even so, complete resection is not always possible; when incompletely resected, hemangiomas nearly always recur.
In addition, surgery can be associated with large-volume blood loss, even when preoperative embolization is employed.
Laser knife excision of hemangiomas is a technique developed to better control intraoperative bleeding. Preoperative ultrasound-guided hookwire localization may aid in defining the extent of a hemangioma during excision. Wang et al found it to be safe and effective for nonpalpable intramuscular hemangiomas and concluded that it may provide a better cosmetic result and improved functional recovery. Radiation has been used to treat soft-tissue hemangiomas in surgically inaccessible or potentially dangerous sites.
Local pedunculated synovial hemangiomas are removed surgically, often through an arthroscope. More diffuse lesions may be treated with intra-articular low-dose radiation therapy, open excision, or both when sufficiently symptomatic.
Hemangiomas of bone rarely require treatment. If symptoms are significant enough to warrant consideration of treatment, it is important to confirm the diagnosis, as more aggressive neoplasms (eg, metastatic renal cell carcinoma) may masquerade as hemangioma.
Radiation may be considered for symptomatic hemangiomas in surgically inaccessible sites, such as vertebral hemangiomas. However, some authors have found that selective arterial embolization is safer and more effective in the treatment of symptomatic vertebral lesions.
Bleacher et al proposed a new management algorithm for symptomatic and locally aggressive vertebral hemangiomas. Patient symptoms were divided into those with pain only and those with neurologic deficits. Patients with pain were treated with transarterial embolization or vertebroplasty after failing conservative management whereas patients who presented with neurologic deficits were treated with transarterial embolization, decompressive laminectomy, and vertebroplasty.
Symptomatic and locally aggressive vertebral hemangiomas have been reported in the pediatric population; however, due to the paucity of clinical data in this group of patients, no clinical guidelines have been established in the literature; therefore, the treatment should be individualized at this time.
Chemotherapy has been used in the treatment of extensive hemangiomatosis, particularly when the vascular proliferation is life- or limb-threatening.
No standard treatment is currently recognized for Gorham disease. Regression of lesions or stabilization of disease has been reported with steroids, radiation, surgery, bisphosphonates, zoledronic acid, and interferon alfa. Local resection with arthroplasty has been reported, with no evidence of recurrence in periarticular disease.
Kasabach-Merritt syndrome is treated with supportive measures, particularly transfusion of platelets. Steroids also have been used in the treatment of Kasabach-Merritt syndrome, with some success.
Good results of treatment with pentoxifylline were reported by de Prost et al. Pentoxifylline acts to restore blood flow and seems to possess antithrombotic activity as well. Pharmacologic management with interferon alfa-2a has also been attempted. Radiation therapy has had variable success. Surgical resection of the hemangioma often is difficult.
In a study evaluating the clinical characteristics, treatments, and outcomes of neonates with Kasabach-Merritt syndrome, Wand et al found concluded that steroid therapy had a low degree of efficacy and was associated with a high rate of relapse, whereas arterial embolization was effective. Accordingly, they recommended that a combination of steroid therapy and embolization therapy be considered as first-line treatment of neonatal Kasabach-Merritt syndrome and that vincristine be considered if that approach is ineffective.
Angiography is an important aspect of preoperative planning when the vascular supply of the lesion is in question and when preoperative embolization is considered. Embolization of high-flow lesions may be performed by interventional radiology to decrease intraoperative blood loss and to decrease the risk of postoperative recurrence. Low-flow lesions are not treatable by embolization. Instead, sclerosing agents may be used to decrease blood flow through low-flow hemangiomas.
Transarterial embolization can be a definitive treatment option or a preoperative intervention followed by surgical decompression for the treatment of symptomatic vertebral hemangiomas. Significant blood loss has been described as a complication of surgical treatment of locally aggressive vertebral hemangioma in patients with neurologic deficits.[18, 36]
Surgical excision attempts to achieve a marginal border unless the hemangioma is contained within a single muscle belly that can be excised completely to achieve a wide margin. The rate of local recurrence following wide excision has been reported as less than 10%, whereas local recurrence rates after marginal excision range from 25% to much higher. Meticulous hemostasis is essential in the prevention of postoperative hematoma.
Postoperatively, the surgical site is wrapped in a compressive dressing. The patient is required to maintain a minimal level of activity. Both of these measures are instituted to prevent the occurrence of postoperative hematoma.
Follow-up is required to assess symptomatic relief and to monitor for possible recurrence.
In both intramuscular and osseous hemangiomas, hemorrhage can occur spontaneously during biopsy or during surgical resection; such hemorrhages can be massive.[21, 36] Furthermore, hemangiomas typically recur following incomplete surgical excision. Some large hemangiomas may result in shunting of the blood to a degree significant enough to cause congestive heart failure.
Rarely, cord compression and neurologic deficits can result from vertebral hemangiomas. Approximately 30% of patients with Kasabach-Merritt syndrome die from hemorrhage or infection. Osteomalacia is an uncommon complication that has been associated with hemangioma.
Outcome and Prognosis
A significant number of intramuscular hemangiomas are associated with relatively mild symptoms, such as intermittent aching or discomfort with exercise. These may require no treatment and may have no significant sequelae. Unfortunately, those symptomatic enough to indicate treatment are those most likely to be incompletely excised, and thus, to recur. Recurrence rates following surgery range from 18-50%.
Outcomes for diffuse synovial hemangiomas are similar to those for intramuscular hemangiomas. Patients with localized synovial hemangiomas tend to have excellent results following surgical excision.
Hemangiomas of bone
Many osseous hemangiomas remain asymptomatic, require no treatment, and have no significant sequelae.
In a small series, patients with symptomatic or locally aggressive vertebral hemangiomas treated with interventional or surgical modalities had good results, with either complete resolution of symptoms or only mild symptoms reported by the patients. All patients returned to their prior levels of activity and reported minimal or no residual pain in daily activities.
Hemangiomatosis often becomes symptomatic during childhood, yet is nearly impossible to excise. Therefore, treatment with chemotherapy has been tried with variable success.
The rarity of Gorham disease precludes a clear assessment of its prognosis. Results of treatments have varied.
Future and Controversies
The true nature of hemangiomas remains controversial, with no clear consensus on an inciting cause, nor agreement as to whether hemangiomas are neoplasms, hamartomas, or malformations.
Future investigations likely will attempt to answer these questions, and may lead to innovations in therapy. Work is underway on the therapeutic use of angiogenic cytokines and angiogenesis inhibitors, including systemic administration of the antiangiogenic proteins AGM-1470 and angiostatin or of the matrix metalloproteinase inhibitor batimastat, and gene gun therapy with interleukin-12.
Some have argued that epithelioid hemangiomas are not a distinct clinicopathologic entity but rather a misdiagnosed hemangioendothelioma, which is a tumor with malignant potential, unlike hemangioma. Identification of WWTR1-CAMTA1 fusion as the genetic hallmark of epithelioid hemangioendothelioma regardless of anatomic location, provides an objective and powerful diagnostic tool that can be used to distinguish the two entities. The WWTR1-CAMTA1 fusion was used in a retrospective study with good results to reinforce prior data that epithelioid hemangiomas are benign lesions and not low-grade malignant soft-tissue tumors.
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