Hamartoma Treatment & Management

Updated: Oct 23, 2017
  • Author: Rohit Seth, MD, PhD, MRCS(Edin); Chief Editor: Harris Gellman, MD  more...
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Treatment

Approach Considerations

Because most hamartomas are found incidentally, their treatment depends on the patient’s signs and symptoms at the time of presentation. Pain is a common finding and can be treated conservatively with analgesics. If symptoms are not controlled, further investigation is usually required and surgical management is considered.

Most patients may not require any intervention. However, for those who do require intervention, procedures may include biopsy, curettage, and fracture fixation. If bony involvement is extensive, amputation of digits or limbs may result.

Regardless of the lesion or mass present, clinicians must adhere to certain basic principles. Whenever possible, benign tumors should be treated conservatively. Surgical intervention may be indicated in the following situations [52] ):

  • If the diagnosis is in any doubt and if biopsy is required to distinguish between benign and malignant lesions
  • If pathologic fractures occur due to bone weakness
  • If the patient becomes symptomatic, with bony masses compressing the surrounding structures or interfering with muscle and joint movement so that function is impaired or that prominent bony protuberances cause cosmetic deformities
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Surgical Therapy

For patients undergoing surgery, the preoperative workup and management are similar for most hamartomatous lesions (see Workup). Specific intraoperative details are beyond the scope of this article; however, indications for surgical treatment of various hamartomas are briefly summarized here.

Specific postoperative details are also beyond the scope of this article. Follow-up care can range from no further intervention and simple reassurance to 6-month or annual visits, depending on the type of hamartoma and on the risk of malignant change.

Bone-forming tumors

Bone island

A bone island is important as a differential diagnosis. If this lesion enlarges, biopsy may be necessary. If the lesion is becoming symptomatic or if the patient has a history of malignancy, biopsy is indicated.

When radiographs demonstrate findings characteristic of a bone island, no follow-up is required, because the lesions are benign and are not associated with morbidity or mortality. Follow-up or biopsy may be indicated if a lesion is especially large, if it grows rapidly, or if it is found in a patient with symptoms or a history of malignancy that could produce osteoblastic metastases. [53]

Osteopoikilosis

The lesions of osteopoikilosis are benign and asymptomatic and do not alter morbidity or mortality. Some lesions may increase or decrease in size and number, or they may even disappear completely. [53]  Despite osteopoikilosis being a benign disease, patients with osteopoikilosis should be monitored. Numerous case reports have demonstrated pathologies associated with osteopoikilosis, including spinal canal stenosis, hip fracture, and malignant degeneration. [54, 55, 56, 57]

Melorheostosis

The course of melorheostosis is insidious, with a slow, chronic progression of symptoms and periodic exacerbations. As melorheostosis becomes progressive, pain management is often the toughest challenge. Successful pain control can be achieved with the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and bisphosphonates. However, such medications do not alter the course of bone lesion formation. [58, 59, 60, 61]  Most cases are benign. In severe cases, lengthening of tendons or correction of deformities may be warranted, and amputation may be indicated. [62]

Osteopathiastriata

In and of itself, osteopathiastriata is benign and asymptomatic, and patients have a normal life expectancy. Although the combination of osteopathiastriata, macrocephaly, and cranial sclerosis is rare, it can cause disfigurement and disability secondary to pressure on the cranial nerves. Sequelae may include facial nerve palsy, deafness, and reduction of the visual field resulting from narrowing of the optic foramina.

With osteopoikilosis, melorheostosis, and osteopathiastriata, the relatively constant size and symmetrical distribution of the lesions make them obvious. If the disease is associated with subcutaneous nodules, they may be excised for cosmetic reasons. Surgery is indicated if patients become symptomatic. Evidence in the literature describes osteosarcoma formation. [63]  When any of these conditions is associated with Buschke-Ollendorff syndrome, surgery may be required for cosmetic reasons.

Cartilage-forming tumors

Osteochondroma and multiple osteochondromas

Symptomatic patients with osteochondroma or multiple osteochondromas require surgical treatment. The specific presentation determines the urgency with which surgery must be performed. Patients with vascular ischemia, spinal cord compression, or rapidly progressive malignant degeneration require surgery on an urgent basis.

The prognosis and long-term survival of patients with osteochondroma or multiple osteochondromas vary greatly. In general, the prognosis is favorable because most osteochondromas cease to grow when skeletal maturity is achieved. When lesions continue to grow in adulthood, surgery is usually curative. One study showed that surgery resolved preoperative symptoms associated with osteochondromas in 93.4% of cases. [64]  Failure to remove the entire cartilaginous cap or its overlying periosteum is the basis for most recurrences.

If a high-grade cancer grows, the outcome is somewhat uncertain. In rare cases, osteochondromas have spontaneously resolved during childhood or puberty. Continuous follow-up care is essential for children with multiple osteochondromas. Patients and their families should be informed about the hereditary nature of the condition.

Symptomatic patients with epiphyseal osteochondroma also require surgical treatment. Early excision is recommended to avoid intra-articular development, which would limit joint motion. [36]  Individuals with an epiphyseal osteochondroma have a normal life expectancy. No malignant changes have been reported.

Enchondromatosis

Enchondromatosis can result in severe bone deformities, such as bowing and shortening. The risk of malignant change is high and is an indication for further intervention.

The prognosis of patients with enchondromatosis is difficult to assess. Most often, enchondromas are of no consequence, and patients are asymptomatic. The lesions are not life threatening and do not alter the person’s life expectancy. The two main factors that affect mortality and morbidity are painful malignant transformation and pathologic fracture, respectively. The prognosis may be better for patients with the dispersed forms of the disease than for those with localized forms, which may induce limb shortening or deformity.

Fiber-forming tumors

Nonossifying fibroma

Most nonossifying fibromas regress, and no surgical intervention is required. Surgery is indicated for symptomatic lesions, as when patients present after sustaining pathologic fractures. Symptomatic lesions should first be treated conservatively. Conservative care consists of activity limitation and immobilization, in addition to yearly or biyearly radiography. If symptoms persist with conservative treatment, wide surgical curettage with autogenous bone graft is indicated. [65]

Nonossifying fibroma does not increase morbidity or mortality. For large, nonsymptomatic lesions, the only management indicated is yearly or biyearly radiographic examination until the lesion ceases growth and appears stable.

Special attention should be given to lesions larger than 33 mm and to those involving more than 50% of the diameter of the bone. [66]  Attention is especially needed for lesions found in young children because of the risk of further growth.

Arata et al reviewed pathologic fractures in nonossifying fibromas over 49 years at the Mayo clinic. [66]  Treatment consisted of cast immobilization, along with biopsy at a later date, simple curettage, curettage and autogenous bone-grafting, or segmental resection of fibular lesions.

Fibrous dysplasia

In patients with fibrous dysplasia, surgery is indicated as prophylaxis or treatment of pathologic fractures. With monostotic lesions, segmental resection can be performed.

Fibrous dysplasias progress in childhood but stabilize when skeletal maturity is achieved. Sarcomatous change occurs in 1.5% of patients. [67]

Benign non-matrix-forming tumors

Hemangioma of bone

With hemangiomas of bone, intervention is indicated when patients become symptomatic. The symptoms determine the treatment, which can include irradiation, laminectomy, embolization, spinal reconstruction after tumor resection, or a combination of these procedures.

Most lesions are asymptomatic, and clinically significant symptoms develop in only 1-2% of patients. Because of the rarity of this lesion, no information about prognosis is available. [68]

Skeletal hemangiomatosis and lymphangiomatosis

In patients with skeletal hemangiomatosis or lymphangiomatosis, the lesions can regress, and surgical intervention may not be required. When treatment is needed, however, it usually involves curettage, grafting, and irradiation. Surgery may be needed to treat pathologic fractures or deformity.

Because skeletal hemangiomatosis and lymphangiomatosis are rare, each patient must be evaluated and treated on an individual basis, with his or her own prognosis. The prognosis improves for individuals without visceral involvement.

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