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SIGNS AND SYMPTOMS

The presenting signs and symptoms of various scalp and skull lesions are quite similar, regardless of the primary pathology. The most common presenting complaint is a visible or palpable mass. Congenital lesions may be noticed at birth by the examining physician or the parents. Birth-related swelling of the scalp may obscure a congenital lesion or cephalohematoma that will later become obvious. Newborns and infants with high-flow vascular malformations may have signs of high-output heart failure including failure to thrive or cardiac murmurs.

Some lesions are painful or tender to palpation. In preverbal children, pain may be expressed as irritability or failure to thrive. Careful neurological examination is important, but newborns and infants may harbor surprisingly large intracranial masses or cerebral anomalies and demonstrate neurological function appropriate for the chronological age. Head circumference is one of the most valuable measurements in the assessment of young children.

Abnormal tufts of hair, discoloration of the scalp, and palpable skull defects all may present for neurosurgical evaluation at various ages. In older children, it is important to determine whether the lesion has changed in size or character recently before consultation. Lesions previously thought to be confined to the scalp may be referred after attempted excision by a general pediatric surgeon or dermatologist showed extension through the calvarium.
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DIAGNOSTIC STUDIES

Most children referred to neurosurgeons for the evaluation of scalp or skull masses undergo imaging with computed tomography (CT) or magnetic resonance (MR) imaging. Indeed, neuroimaging is recommended for all but the most obviously extracranial masses. One third of patients evaluated with solitary nontraumatic "lumps" on the head have some degree of intracranial extension. Occasionally a predominantly intracranial process such as a subdural empyema or even a brain tumor may present with scalp swelling, especially in infants.

Midline or pedunculated scalp masses should be evaluated with MR imaging to assess the degree of intracranial extension and any relation to the dural sinuses. Some bony lesions of the vertex and many scalp lesions are not well visualized on routine axial CT. Vertex skull lesions are obscured on axial scans tangential to the lesion and direct coronal or thin section cuts with three-dimension reconstruction may be necessary. With scalp lesions, the abnormal area should be marked and called to the attention of the radiologist before scanning. MR imaging is preferable to CT for evaluating soft tissue masses and CT is superior for bony masses. The two imaging modalities frequently are complementary.
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SURGERY

In children with a scalp or skull tumor, surgery is indicated for decompression of neural structures, curative or palliative resection, correction of disfiguring deformities, relief of pain, and biopsy of an unknown lesion. It is not the goal of this chapter to describe the techniques that may be applied, but rather, to provide an overall framework with which to approach the surgical management of these patients. All respective procedures should be planned in anticipation of a reconstructive procedure. It is the authors' belief that the psychosocial effects of a cranial defect or a cosmetically disfiguring scar on the developing child as well as others' attitudes toward the child should never be minimized.

In many cases, the defect left by resection of a skull tumor may be repaired primarily with a split thickness bone graft taken from adjacent cranium. Accordingly, the scalp incision should be designed to allow maximum vasculanty as well as access to potential donor craniotomy sites. Despite its length, the standard coronal scalp incision is frequently the best choice. In situations in which poor wound or graft healing may be anticipated (e.g., when radiation or chemotherapy is indicated) it its advisable to delay the reconstruction until healing conditions are optimized, which may help avoid the loss of both donor and recipient grafts.

The need for intradural exploration is usually apparent from preoperative imaging studies; most scalp or skull tumors do not demonstrate intradural extension. However, congenital lesions frequently have a definite connection to the underlying brain that must be appreciated. Failure to completely excise a dermal tract may allow growth of an intracranial dermoid cyst or result in delayed infection. In addition, adhesion of the brain to overlying dura has been reported to cause neurological symptoms analogous to spinal cord "tethering".4 Thus, serious consideration should be given to intradural exploration when transdural extension is apparent.
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Actual neoplasms of the scalp are quite rare in children, but congenital lesions of the scalp are rather common.

Two such lesions require special attention due to the known potential for malignant degeneration. Nevus sebaceous is a congenital lesion primarily of sebaceous glands of the scalp that may transform into basal cell carcinoma. Giant congenital compound melanocytic nevi may occur anywhere in the skin including the scalp. These nevi may give rise to malignant melanomas. Early prophylactic resection of these lesions is recommended to decrease the risk of future malignancy.
In practice, superficial lesions confirmed to the dermis are usually treated by dermatologists and plastic surgeons; it is not until subgaleal or cranial involvement is apparent that neurosurgical consultation is obtained. The following conditions, although not necessarily neoplastic, are frequently encountered by pediatric neurosurgeons. Back to top

Scalp Nodules

Subcutaneous nodules of the scalp are common in children. The majority do not possess any intracranial extension or any relation to the neural structures.7 However, lesions of the scalp that on palpation are found to be fixed to the skull, or those overlying the midline may require further neurosurgical evaluation.
Scalp nodules result from numerous processes. In children especially, enlarged lymph nodes may present as scalp nodules, particularly in the postauricular region. There is frequently a history of upper respiratory infection and the lymphadenopathv is self-limited. It is also not uncommon in children to notice very firm nodules fixed to the skull after trauma, with or without skull fracture. These periosteal reactions should also resolve spontaneously.

Multiple painless subcutaneous nodules of the scalp may result from an inflammatory process of unknown origin known generically as "necrobiosis." Necrobiotic granulomas of the scalp are described under man titles including rheumatoid nodules, benign rheumatoid nodules, pseudo-rheumatoid nodules, subcutaneous granuloma annulare, and subcutaneous palisadinggranulomas of the scalp. Histologically, necrosis and degeneration of dermal collagen is the predominant feature, surrounded by histiocvtes, epithelioid cells, and multinucleated cells.

In contrast to true rheumatoid nodules associated with rheumatoid arthritis, these granulomas involve the scalp and pretibial subcutaneous tissue and are not found near the joints. Progression to clinical rheumatoid arthritis does not usually occur. The lesions have a predilection for the occipital and frontal scalp and usually resolve spontaneously. In general, biopsy is not indicated in typically appearing lesions.

Neurofibromas of the scalp may accompany neurofibromatosis type 1 (NFl) and to a lesser extent, neurofibromatosis type 2 (NF2). With an incidence of 1 in 3,000 live births, NFl is a commonly observed condition in pediatric neurosurgical practice. In a patient with NFl or NF2, the discovery of painless, subcutaneous nodules of the scalp does not usually present a diagnostic problem. Occasionally, however, a nodule may be found in the absence of known neurofibromatosis. Characteristic cutaneous markings such café-au-lait spots and auxiliary freckling should be sought although they may not vet be present in young children. Biopsy of a lesion that shows neurofibroma, although not usually essential, may prompt consideration of the diagnosis of neurofibromatosis. Resection of neurofibromas of the scalp may be indicated when lesions are painful (e.g., in proximity to the occipital nerve) or disfiguring. Consideration of biopsy or resection should be made in neurofibromas that have rapidly enlarged because malignant degeneration is possible.

A variety of benign proliferative disorders of mesenchymal tissue occur in infancy and may result in masses involving the scalp. skull, and occasionally, the dura. These lesions are referred to by various terms and have excellent long term prognoses despite initial periods of rapid growth. Infantile myofibromatosis is characterized by diffuse or nodular proliferations of cells resembling fibroblasts and smooth muscle cells. Although considered the most common benign fibrous tumor of infancy, involvement of the scalp and skull is relatively rare. Some lesions appear to arise from the dura and result in significant intracranial mass effect although the brain and arachnoid are not invaded. In these cases, dural resection is recommended because recurrence may occur from involved dural.

Cranial fasciitis of childhood is a rare, benign lesion of unknown etiology that appears histologically similar to the more common nodular fasciitis and infantile myofibromatosis. It occurs predominantly in infancy and may present as a rapidly growing subcutaneous mass, often with intracranial or intraorbital extension. Operation is generally indicated for histological diagnosis and relief of mass effect. Despite an appearance alarmingly suggestive of malignancy, these lesions usually do not recur after removal. Back to top

Scalp Defects

Failure of closure of the cranial neural tube results in a spectrum of anomalies ranging from massive encephaloceles to small skull defects through which the cranial meninges herniate without underlying cerebral abnormality. Small midline lesions, usually in the occipital region, are frequently referred to as atretic cephaloceles and are commonlv evaluated in pediatric neurosurgical practice as scalp masses. The lesions are soft, compressible, and may increase in size with Valsalva maneuvers. The overlying skin is frequently thin and discolored. Neural elements may be present within the cavity of the lesion with variable or no connection to the underlying brain. There are frequently associated structural anomalies of the brain seen on MR imaging.

When involving the scalp, aplasia cutis congenita is a full-thickness skin defect usually occurring in the midline vertex. In 20% of cases there is a defect of the underlying skull. The dura may also be missing and in severe lesions the child may be born with a large area of exposed brain. Initial efforts are aimed at the prevention of infection with timely coverage of the defect. Staged procedures may be necessary with tissue expansion and skull reconstruction. Back to top

Vascular Lesions of the Scalp 

Vascular lesions of the scalp are common in children, occurring in up to 75% of all newborns. The most common type appears as pink-to-red macular lesions over the forehead, face, or nuchal regions, and usually fades completely over the first year or two of life. Some cutaneous vascular lesions actually progress with age. So-called port wine stains represent dermal regions containing abnormal blood vessels, usually capillaries and venules within the superficial vascular plexus. Port wine stains may become increasingly conspicuous with progressive deformity of the soft tissues and occasionally, the underlying bone. This process appears to result from progressive ectasia of the involved vessels rather than vessel proliferation. A deficiency of normal sympathetic innervation of the involved vessels may be causative.

From a neurosurgical point of view, port wine stains of the scalp and face may be markers for underlying cerebral involvement. Five percent of patients with port wine stains have Sturge-Weber syndrome, with abnormal pial vasculature of the ipsilateral cerebral cortex, seizures, and varying degrees of cognitive impairment. Treatment of the cutaneous lesions is usually by argon or tunable dye laser irradiation and is dependent on cosmetic and psychological concerns in young children.
Cutaneous hemangiomas and cavernous malformations occur predominantly in the head and neck in newborns. They typically progress during the first year of life with spontaneous, often remarkable, involution thereafter. As with port wine stains, there is rarely isolated involvement of the scalp and therapeutic decisions are based on cosmetic considerations. Back to top

Arteriovenous Malformations of the Scalp

Fistulous arteriovenous communications may occur in the scalp and appear as either a pulsatile, compressible mass or with high output heart failure in infants. These scalp arteriovenous malformations (AVMs) are frequently referred to as cirsoid {Gr. kirsos varix} aneurysms of the scalp. Most scalp AVMs, especially in children, appear to be congenital, although up to one third may be acquired posttraumatically. In infants, these lesions may enlarge, consuming an increasing proportion of the cardiac output and creating marked venous dilatation throughout the scalp and face.

Plain x-rays of the skull and noncontrast CT of the brain usually show no abnormalities. Contrast CT may show enhancement of the subgaleal scalp, but angiography is necessary to delineate the vascular anatomy clearly. Arterial feeding and venous draining vessels are predominantly extracranial, although a small contribution may be demonstrated from "parasitized" intracranial vessels with compression of the extracranial arterial supply.

Direct surgical treatment of AVMs of the scalp may be exasperating, with significant blood loss intraoperatively and an unexpectedly high recurrence rate postoperatively. Modern endovascular techniques of either direct puncture or transarterial embolization with a variety of thrombogenic agents can significantly reduce blood flow or even obliterate the malformation. Surgery is often required to resect residual fistulae or to remove the mass of embolic material. Apart from blood loss, the most significant surgical complication is scalp necrosis. Care must be taken not to carry the epigaleal dissection into the hair follicles of the scalp. The scalp incision should be planned based on the anatomy of the AVM as well as on the possible need for scalp flap rotation. Back to top

Sinus Pericranii

In contrast to scalp AVMs, the vascular lesions referred to as sinus pericranii are relatively slow-flow anastamoses between extracranial veins and intracranial veins, usually involving the superior sagittal sinus. These lesions present as painless swelling of the scalp, usually in the midline or para-midline. The lesion is compressible, deflates with head elevation, and refills with Valsalva maneuver or with the head in a dependent position. Like scalp AVMs, most sinus pericranii appear to be congenital, with a minority acquired after trauma. Sinus pericranii present predominantly in childhood but may come to medical attention during adulthood, especially when acquired post traumatically.
Most sinus pericranii can be diagnosed clinically. Skull x-rays show only the skull defect, which may be quite small. Contrast CT and conventional angiography frequently fail to adequately illustrate the lesions. Direct injection of contrast into the blood filled subgaleal mass may be required if radiographic confirmation is desired. Sinus pericranii is usually adequately treated by direct surgical obliteration without preoperative embolization. The decision to operate is based on symptoms, cosmetic concerns, and fear of bleeding from the lesion, the perceived risk of which is usually greater than the actuality.
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LESIONS OF THE SKULL

Epidermoid and Dermoid Cysts

Dermoid and epidermoid cysts are the most common lesion of the scalp and calvarium encountered by pediatric neurosurgeons; they account for 15 to 60% of masses in this region. Dermoid cysts and dermal tracts are more likely to involve the scalp and skull in young children; epidermoids tend to occur intracranially in older children and young adults. These congenital lesions are thought to result from varying degrees of failure of dysfunction of neuroectoderm from cutaneous ectoderm during closure of the neural tube (Figure 24-2).

Dermoid cysts contain elements of full-thickness skin, including epithelium, hair structures, and sebaceous glands. There may or may not be a visible sinus or pit overlying the lesion. The lesions are usually first observed by the parents in the newborn period. Occasionally, a small cyst may enlarge or become infected, prompting evaluation. Growth occurs as epithelial cells desquamate and break down, producing an accumulation of keratin and cholesterol. The most common location in children is at the anterior fontanel, where there is rarely dural penetration, and many experienced surgeons forego neuroimaging except in unusual situations. Dermoid cysts also occur at the pterion, usually without dural penetration. However, dermoid cysts of the occipital midline, usually have some degree of intracranial involvement and MR imaging is recommended. Even when neuroimages fail to show intracranial penetration, exploration frequently shows a slender tract extending through the cranial bone and attaching to the dura. In these cases, the tract may simply be coagulated and divided. Extensive intradural exploration is usually not indicated because of the relationship to midline dural sinuses. Back to top

Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is an abnormal proliferation of the Langerhans cell histiocytic subtype. The inciting stimulus is as yet unidentified, although detective immunoregulanon may play a role. A neoplastic origin is also currently debated. Depending upon the extent of involvement, LCH has been variously known as eosinophiic granuloma, Hand-Schüller-Christian disease, or Letterer-Siwe syndrome. In the aggregate, these forms of LCH are also known as histiocytosis X.

LCH usually appears before puberty, typically in children 4 to 12 years of age. The most frequent site of involvement is the skull, although other bones and virtually any organ, including the brain, may be affected. Presentation is typically with a painful scalp mass, which may have a history of recent growth. Skull radiographs show a radiolucent, "punched-out" lesion without sclerotic margins. CT of the skull defines extension intracranially (usually minimal) and into the diploic space.

Although unifocal involvement of the skull is common, it is important to rule out involvement at other sites. Multiple bone involvement occurs in 28% of children and 20% of adults. Radiographic skeletal survey and radionuclide bone scan appear to be complementary for the evaluation of polvostotic disease. Bone scan better detects lesions in the ribs and vertebrae and skeletal X-rays arc more accurate for the skull and long bones. Cerebral involvement is often heralded by diabetes insipidus, and hypothalamic lesions may be seen on MR imaging.

Treatment of unifocal LCH of the skull usually consists of complete, full-thickness excision of the lesion; there is rarely underlying dural penetration. Reconstruction of the defect with autologous split-thickness cranial bone from adjacent skull is usually easily performed at the time of primary resection. Biopsy and curettage have been recommended but without clean bone margins, healing of the bone graft may be impaired. Complete resection is associated with a low recurrence rate and adjuvant treatment is not usually indicated. Disseminated or multisystem involvement, patient age younger than 2 years at diagnosis, and hepatosplenomegaly or thrombocytopenia are poor prognostic factors. Recurrent or progressive disease may be treated with low-dose radiation or chemotherapy. Optimal chemotherapeutic regimens for high-risk patients have yet to be defined.

Figure 24-I. (A) Atretic cephalocele. This newborn was found to have a soft skin covered lesion in the occipital midline region. Hydrocephalus was also present. (B, C) MR scans show the lesion to be mostly fluid-filled. A small extension of abnormal neural tissue is also present. The abnormal configuration of the tectum and cerebellum suggest a firm fruste of meningoencephalocele.

Figure 24-2. (A, B) Sagittal and coronal TI MR scans show appearance of a relatively large dermoid cyst of the anterior
fontanel. The underlying brain and dura are uninvolved.

In keeping with the unknown etiology of LCH, the natural history of the disease is also not clearly understood; likewise, the influence of current therapeutic modalities is not well defined. Neither surgery, radiation, nor chemotherapy have been shown conclusively to affect outcome, and lesions have been observed to resolve spontaneously. The reported occurrence of new bone lesions in children is 22% and the local recurrence rate after treatment (all modalities) is 6%. Recurrence is usually seen within 2 years of diagnosis although in adults this may occur many years later. It thus seems reasonable to follow children with LCH of the skull for at least 5 years with X-rays, bone scans, or both. Fortunately, recurrent or new bone lesions without extraskeletal involvement are not associated with a worsened prognosis. Back to top

Aneurysmai Bone Cysts

Aneurysmal bone cysts (ABC) are tumorlike expansions of the diploic space that distort and attenuate the overlying cortical bone. The cystic lesions themselves are filled with blood and lined by connective tissue with giant cells and trabecular bone. About 80% of these lesions come to medical attention before the end of the second decade of life. Only 2% of ABC arise in the bones of the skull. Of these, about two thirds are confined to the maxilla or mandible. Presentation in the skull usually involves a painful expanding mass. Cranial neuropathies may be present if the lesion is in proximity to the cranial nerve foramina.

Most experience with ABC has been obtained from lesions in the long bones. Although the etiology of ABC is not known, in the long bones nearly one third of the lesions are considered "secondary" to other bone lesions such as giant cell tumors and osteoblastomas. Recently, a similar association has been observed in ABC of the skull. Thus, although the CT appearance of these lesions is characteristic, a diligent search must be made for any accompanying lesion.

Aneurysmal bone cysts have a distinctive radiographic appearance, with expansion but not destruction of the cortical bone and fluid/fluid levels within the cysts themselves. Although they may expand intracranially, they do not penetrate dura. Aneurysmal bone cysts are highly vascular lesions and direct operation in children is frequently associated with significant blood loss. Preoperative angiography is thus indicated, with preparation for embolizalion at the time.

Treatment is usually surgical, after embolization. Complete resection is the goal after primary reconstruction. Curettage of residual abnormal bone along margins that are not safely resectable is acceptable. In the long bones, curettage alone is associated with a recurrence rate of up to 60%. For this reason, and considering the association of ABC with other bone lesions, close postoperative CT surveillance is recommended. Residual or recurrent lesions usually respond to radiation therapy, with its inherent risks. Cryosurgery reduces recurrences rates after primary surgery and offers an alternative to radiation therapy when treating recurrent disease. However, application in the cranium has not yet been reported. In cases in which the risk of operative resection is considered too great, direct injection of acrylic sclerosing agents has been reported to be effective. Back to top

Osteomas are seen mostly in young adults and rarely in children. They arise from mature cortical bone and have a distinctive CT appearance of a hyperdense, expansile lesion without bony destruction. MR imaging is variable in these tumors and thus of limited use. If the lesion is asymptomatic without cosmetic significance, observation is acceptable. It is not known whether simply drilling these lesions to achieve a normal contour is associated with a significant rate of recurrence. We prefer complete resection and repair with a split-thickness cranial bone graft, for which the recurrence rate is low.

By contrast, osteoblastoma occurs more frequently in children. Despite the ominous suffix, osteoblastoma in children is usually considered benign. It accounts for about 1% of benign bone tumors, and 10 to 20% of all osteoblastomas occur in the skull. Histologically, it is composed of immature osteoid material laced with osteoblasts. The appearance is similar to osteoid osteoma but demonstrates more variable calcification within the lesion. Because of the variable calcification pattern, radio-graphs and CT may fail to render the correct diagnosis. Although histologically benign, osteoblastomas may enlarge and are frequently painful. Complete full-thickness resection is recommended. In older patients, osteoblastomas may have a more malignant appearance with features of low-grade osteosarcoma.

Osteoid osteomas may occur in any bone but are quite rare in the skull. The average age at presentation is 19 years and the lesions are painful in the majority of eases. The pain classically is dull and throbbing, more severe at night, and relieved by aspirin. The recurrence rate after complete resection is low. Back to top

Fibrous dysplasia is a benign disease of unknown etiology in which normal bone is replaced by fibrous connective tissue. The abnormal tissue is composed of fibroblasts and collagen interspersed with immature woven bone. The process begins within the medullary cavity of the affected bone and expands, thinning and distorting the cortical bone. Involvement may be confined to a single bone (monostotic) or include multiple, usually contiguous bones (polyostotic). As the area of dysplasia grows, it appears to be able to cross suture lines into adjacent bones. This type of polyostotic disease is particularly prominent in the craniofacial skeleton and frequently attains the attention of craniofacial surgeons and neurosurgeons.

Polyostotic fibrous dysplasia may occur with precocious puberty or other hypersecretory endocrinopathies, most frequently involving growth hormone, thyroid hormone, or cortisol. This association is part of the McCune-Albright syndrome, which may also include "café au lait" hyperpigmented skin lesions. Fibrous dysplasia and McCune-Albright syndrome together may represent a spectrum of disease resulting from abnormal expression of a subtype of common regulatory proteins called G proteins.
Fibrous dysplasia accounts for about 2.5% of all bone tumors. It appears most commonly around puberty and has equal distribution between the sexes. Although disease activity is greatest before skeletal maturation, presentation or progression may occur well into adulthood. Involvement of the skull occurs in up to 27%, and 50% of patients with monostotic and polyostotic fibrous dvsplasia, respectively.

The initial presentation usually involves a history of painless, progressive deformity of the skull. When the bones of the orbit are involved there may be displacement of the globe with proptosis and diplopia. The changes often occur so gradually that diplopia is unnoticed by the patient. With involvement of the bones of the skull base, the cranial nerve foramina may become narrowed. The most common cranial neuropathy results from narrowing of the optic canal. Decreased visual acuity usually occurs gradually although acute loss of vision has been reported.60'62 Temporal bone involvement may result in tinnitus, decreased audition, facial palsy, and temporomandibular joint restriction.

Fibrous dysplasia is best imaged with thin-section CT in the coronal and axial planes. The diagnosis is usually apparent by the CT findings of expansion of the involved bone with a "ground glass" appearance. MR imaging may be useful to evaluate intracranial extension or the relation of cranial nerves to the lesion. A grading system penetrate the dura and expand along the subdural space. In general we make no attempt at the time of primary resection to reconstruct the calvarium as healing and immunocompetence will be inhibited by the adjuvant therapy. The initial scalp incision should however take into account the possible need for cranioplasty in the future.

Figure 24-3. (A, B) This 14-year-old boy presented with an enlarging mass in the vertex of the skull. Sagittal and coronal MR
scans show a heterogeneously enhancing lesion with significant intracranial extension and distortion of the underlying brain. Complete evaluation showed pulmonary nodules and the presumed primary tumor in the pelvis. 

(C) Angiography shows the tumor to be highly vascular and supplied by extracranial branches of the external carotid artery. The patient underwent embolization of the tumor before surgery. 

(D) Initial appearance of the tumor at surgery. A gross total resection was performed. The tumor was Ewing's sarcoma.

 
Figure 24-4. (A, B) The patient shown received chemotherapy and radiation. After 18 months, there was no evidence of disease and the patient had a 5-cm cranial defect. 

(C, D) Split-thickness autologous cranioplasty was performed through the original incision. Two semicircular grafts were used to minimize potential defects in case of infection or tumor recurrence. At I year after the cranioplasty, the patient remains disease free.

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NEUROBLASTOMA

Leukemias and Lymphomas

Many of the various leukemias and lymphomas involve the bones of the cranium by virtue of bone marrow invasion. Again, systemic therapy based upon histological diagnosis and institutional protocols generally obviates the need for surgical treatment.

Neuroblastoma is the most common extracranial solid tumor in children. By virtue of the number of patients involved, it is therefore the most common tumor to metastasize to the skull in this age group. Skull metastases may present as a swelling of the skull and x-rays and CT demonstrate a lytic lesion with a characteristic "hair-on-end" appearance. Cranial MR imaging is usually performed as part of the metastatic workup, although intracranial metastases are rare. These metastases are rarely resected because the tumor is usually responsive to current chemotherapy regimens, depending on the clinical stage.28 Operation may be indicated for biopsy of suspected recurrent disease or resection of apparently solitary disease after systemic therapy.

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PEARLS FROM THE AUTHORS' EXPERIENCE

• Most scalp and skull lesions in children are benign.

• Most malignant neoplasms of the skull are metastatic.

• Complete resection of fibrous dysplasia is not always necessary.

• Complete resection of aneurysmal bone cysts is curative.

• Resection of scalp and skull lesions should be performed in anticipation of immediate or delayed reconstruction.

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EDITOR'S COMMENTARY

The authors have presented the wide spectrum of scalp and skull masses that occur in childhood. Neurosurgeons need to be aware of the potential for intracranial extension of some lesions and to know when CT or MR imaging is medicated. They need to be aware of the potentially high vascularity of certain lesions such as aneurysmal bone cysts and to consider preoperative angiography and embolization. In one tumor type after another, the authors point out that complete resection followed by skull reconstruction is the optimal treatment Those who manage children with these lesions must be capable of providing such treatment

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