Учебники / Head_and_Neck_Cancer_Imaging

and/or mastoid air cells should call such a diagnosis into question.

A high riding jugular bulb or a jugular bulb diverticulum might be mistaken for a mass within the jugular foramen. Close evaluation of all images in the different planes and on the various sequences typically reveals inconsistency of the flow pattern with the classic flow void seen in at least one plane or on one sequence in such cases.

Mottled fatty replacement of the bone marrow may mimic a diffuse infiltrative process of the skull base that might be impossible to distinguish from lymphoma, leukemia, multiple myeloma or diffuse metastatic disease. Correlation to clinical symptoms, age, gender and history of an underlying malignancy might help to develop a risk profile that does or does not warrant further evaluation with bone scan, bone marrow biopsy or cross-sectional imaging follow-up.

13.5 Pathology

The skull base is composed of different tissue components and houses a variety of structures that can serve as the origin of skull base masses. Therefore, the spectrum of possible skull base abnormalities is very broad. Skull base lesions overall are rare, with metastatic disease and lymphoma accounting for most of the lesions. Primary malignancies of the skull base are even more scarce but can be often mistaken for benign lesions, and vice versa, creating a diagnostic dilemma. In addition, some of the benign entities show an aggressive growth pattern that might require more aggressive treatment. Therefore, the discussion of pathology of the skull base will also include benign entities that can be mistaken for or that need to be treated more like a malignant lesion.

Skull base lesions can be divided into two broad categories: diffuse or localized abnormalities. Diffuse involvement of the skull base may manifest as diffuse replacement of the bone marrow or as multi-focal lesions. Localized lesions in turn can be subdivided into region-specific and region-non-specific entities. This approach in the evaluation of skull base abnormalities is an important starting point. The exact location of the lesion together with its imaging characteristics, patient’s demographics and clinical presentation

might then further narrow the differential diagnostic considerations.

13.5.1

Malignant Lesions Causing Di use or Multi-focal Skull Base Involvement

Multiple myeloma, lymphoma, leukemia and metastatic disease are the main malignant entities able to cause diffuse replacement of the bone marrow or multi-focal involvement of the skull base (Figs. 13.8 and 13.9). Often, the skull and the visualized portions of the cervical spine are also affected (Fig. 13.8a). Although any primary cancer can metastasize to the skull base, prostate, lung and breast cancer outnumber the other malignancies (Laigle-Donadey et al. 2005; Layer et al. 1999).

13.5.2

Mimics of Malignant Lesions Causing Di use or Multi-focal Skull Base Involvement

Diffuse replacement of the fatty by red bone marrow due to chronic anemia may mimic diffuse infiltration of the skull base by an underlying malignant entity. MR imaging findings are often not helpful as both

Fig. 13.9. Axial contrast enhanced T1-weighted image through the upper orbit level shows replacement of the fatty bone marrow with associated bony expansion of the greater sphenoid wings bilaterally (black arrows), clivus (black arrowhead), petrous apexes (white arrows) and in the occipital skull (white arrowheads) related to multiple myeloma

entities result in decreased attenuation of the bone marrow on T1and fast spin-echo T2-weighted images and show enhancement following intravenous contrast administration (Laigle-Dondey et al. 2005; Yildrim et al. 2005; Loevner et al. 2002). CT may or may not provide additional clues as not all patients with multiple myeloma, lymphoma, leukemia or metastatic disease show bony destruction within the diploic space or early erosions of the inner and or outer table. Therefore, clinical history and laboratory results might provide the most helpful information in some patients. Bone marrow expansion can also occur and mimic diffuse malignant involvement of the skull base and skull. It is usually seen with severe anemia such as hemolytic anemia or thalassemia (Yildrim et al. 2005; Tunaci et al. 1999). MR might deliver ambiguous results but CT usually solves the dilemma as the bone thickening caused by the bone marrow expansion is in disproportion to the preserved diploic trabecula and the intactness of the inner and outer tables of the skull (Fig. 13.10). Sites of extrapoetic bone marrow, such as within the paranasal sinuses, might occasionally also be seen, supporting the benign nature of the bone marrow expansion (Tunaci et al. 1999; Joseph et al. 2004).

Gorham, or vanishing bone disease, is an extremely rare bone disorder that can occur at any age and affects both genders equally. This disease is char-

acterized by osteolysis of unknown etiology that is associated with angiomatous proliferation within the bone or adjacent soft tissues (Gorham et al. 1954). It can manifest as single or multiple lytic lesions involving any bone, but the calvarium and the mandible are most commonly affected (Fig. 13.11). Since it is such a rare entity, metastatic disease or multiple myeloma will be by far the most common cause of multiple lytic bony lesions.

13.5.3

Non-region Specific, Localized Malignant Skull Base Lesions

All malignancies that can cause diffuse or multi-focal involvement of the skull base (metastatic disease, lymphoma, leukemia and plasmocytoma) as well as different sarcomas can present as localized lesions anywhere in the skull base. These might be difficult to distinguish from each other without further clinical information. In general, metastatic disease is more aggressive in appearance and causes frank bone destruction even in early stages, while localized lymphoma and leukemia (often referred to as chloroma) show more permeative growth pattern with preservation of the diploic trabecula and of the cortical bony margins even with large tumors (Figs. 13.12 and 13.13). In addition, it has been reported that highly cellular tumors with small extracellular compartments, such as lymphoma, leukemia and Ewing’ sarcoma, often demonstrate re-

Fig. 13.10. Axial CT displayed in bone windows demonstrates marked expansion of the greater sphenoid wings (arrows) that is also more cystic in appearance within the body of the sphenoid bone on the right (arrowhead). Thalassemia is the underlying cause of bony expansion in this patient

Fig. 13.12. Axial CT image displayed in bone window shows destruction of the sphenoid body (arrowheads) and clivus (arrow) caused by an aggressive skull base metastasis

stricted diffusion on the diffusion weighted images (DWI), with increased signal intensity on the DWI and reduced signal intensity on the apparent diffusion coefficient (ADC) maps (Guo et al. 2002; Mascalchi et al. 2005). In contrast, metastatic disease typically does not show restricted diffusion.Therefore,DWI might be a useful tool in narrowing the differential diagnostic possibilities in patients with inconclusive conventional MR and clinical findings.

Ewing’s sarcoma is a rare and highly malignant neoplasm of the bone arising from the primitive neuroectoderm (Rueda-Franco and Lopez-Correla

1995; Kozlowski et al. 1991). It most commonly in-

volves the long bones, pelvis, ribs and only very rarely the skull base and vault. It typically occurs in children between 5 and 13 years of age. Neurological deficits are the leading clinical symptoms caused by the mass effect from the tumor. Aggressive chemotherapy is the primary and usually successful treatment choice for Ewing’s sarcoma, while surgery is reserved for cranial nerve decompression and radiation therapy for tumors unresponsive to chemotherapy (Carlotti et al. 1999). On imaging, Ewing’s sarcoma presents as an osteolytic lesion causing erosions of the inner and/or outer table in association with an extracranial soft tissue mass of variable size and a significant epidural component causing local mass effect upon the adjacent brain parenchyma. The epidural component of the tumor rarely invades the dura or the brain parenchyma. On CT, the tumor itself is usually isoto hyperdense and shows heterogeneous enhancement following intravenous contrast administration. MR is superior in delineation of the epidural component to adjacent dura and brain parenchyma. In addition, the restricted diffusion seen on the DWI part of the study might provide useful hints to the nature of the mass (Guo et al. 2002; Mascalchi et al. 2005). In recent years, spectral karyotyping has been introduced as an additional diagnostic tool as it has been shown that Ewing’s sarcomas have a characteristic 11;22 chromosomal translocation and/or EWS/FLI1 fusion transcript (Carlotti et al. 1999).

Osteosarcoma is the second most common primary bone tumor after multiple myeloma of the skeleton. It typically arises from the metaphysis of the long bones, especially of the femur and proximal tibia. It only very rarely involves the skull and skull base.

Secondary osteosarcomas are also rare and usually occur in the setting of previous radiation therapy or arising from benign lesions with malignant potential such as Paget’s disease, multiple osteochondromatosis and chronic osteomyelitis (Salvati et al. 1993; Shinoda et al. 1993). The imaging characteristics depend on the type of the osteosarcoma.While parosteal osteosarcoma is limited to the outer table, the other types of osteosarcomas show inner and outer table involvement with associated involvement of the subdural space and brain parenchyma in approximately 40% of cases (Garland 1945). The degree of intracranial involvement is inversely related to patient’s prognosis (Whitehead et al. 1998). Small fluid–fluid levels may be seen in cases of telangiectatic osteosarcoma. Radical surgical excision is the treatment of choice which is usually not feasible in the skull base. Therefore, patients with skull base osteosarcomas are

carrying a worse prognosis than patients with osteosarcomas of other parts of the skeleton.

13.5.4

Mimics of Non-region Specific, Localized Malignant Skull Base Lesions

Fibrous dysplasia of the skull base is relatively common and is a developmental anomaly of the bone forming mesenchyma in which the transformation of woven to lamellar bone is disturbed. This causes an arbitrary arrangement of the bony trabeculae that are embedded in an overgrowth of a well-vascular- ized fibrous stroma. It represents a benign lesion, with extremely low potential for malignant transformation that usually occurs secondary to radiation therapy (Utz et al. 1989). Fibrous dysplasia is often

mistaken for a skull base tumor as it can assume any appearance on MR imaging (Fig. 13.14) (Chong and Fan 1998; Utz et al. 1989). It may show low attenuation on all sequences reflecting the high degree of mineralization but can also demonstrate any other combination of signal intensities on the various sequences without or with mild to extensive enhancement reflecting the different degrees of fibrous and cystic components. In contrast, plain radiographs and CT show characteristic imaging features with bone marrow expansion, “ground glass” appearance and preservation of the adjacent cortex (Fig. 13.14b). Only rarely, fibrous dysplasia presents as a lytic lesion. It is usually unilateral and monostotic in nature. It can occur anywhere in the skull but prefers the anterior and central skull base. It spares the membranous labyrinth, facial nerve canal and the internal auditory canal. Clinically, it is silent in the majority of cases and is usually incidentally found on imaging. The symptomatic patients seek medical attention because of cosmetically undesirable skull and/or facial deformities, or neurological symptoms caused by narrowing of the skull base neural foramina and subsequent cranial nerve compression that may benefit from decompressive surgery.

Langerhans cell histiocytosis is a group of idiopathic neoplasms that are characterized by proliferation of mature eosinophilic and specialized bone marrow-derived Langerhans cells. Three major

types have been described: infantile form (LettererSiwe disease), Hand-Schüller-Christian disease and eosinophilic granuloma (Howarth et al. 1999). Eosinophilic granuloma represents the localized form of Langerhans cell histocytosis and represents the variety most commonly seen by radiologists. It typically occurs in children between 5 and 15 years of age. The lesions are usually asymptomatic but may manifest with bone pain, palpable soft tissue mass or symptoms related to mass effect such as proptosis or cranial nerve neuropathies (Miller et al. 1978). Usually eosinophilic granuloma presents as unifocal bony lesions, typically involving the frontal and temporal bones (DiNardo and Wetmore 1989). On imaging, eosinophilic granuloma presents as a radiolucent, round to oval punch-out bony defect without sclerosis of its margins, with an enhancing soft tissue mass within the bony defect. Hand-Schüller- Christian disease is a chronic, systemic and multi-fo- cal variant of Langerhans cell histiocytosis with peak onset between 2 and 10 years of age. These patients typically present with diabetes insipidus, exophthalmos, chronic otorrhea and hearing loss related to multi-focal and/or bilateral skull and skull base involvement. Other organs (lymph nodes in 50%, liver in 20%, spleen in 30%) and skin might also be involved (Howarth et al. 1999). Letterer-Siwe disease is a diffuse, systemic and the most severe form of Langerhans cell histocytosis. It presents in children

less than 2 years of age with skin eruption, anemia and hepatosplenomegaly. It is universally fatal within a few years. While solitary bone lesions are treated locally with curettage or excision, systemic chemotherapy and or bone marrow transplantation is indicated in the systemic forms of the disease (Howarth et al. 1999).

Dermoid tumors are uncommon lesions and represent inclusion cysts rather than true neoplasms. They derive from misplaced ectodermal elements during the process of neural tube closure. The tumors are usually well-defined and multi-lobulated in appearance. They have an outer connective tissue capsule that is lined with stratified squamous epithelium and contains hair follicles, different glands, desquamated epithelial keratin and most importantly some lipid material. This composition influences the heterogeneous appearance of these tumors on imaging. Nevertheless, these tumors typically contain various amounts of lipid material that is visible on imaging as very low density on CT images and high signal intensity on T1-weighted images, facilitating the correct diagnosis (Smirniotopoulos and Chiechi

1995; Gormley et al. 1994). Contrast enhancement is uncommon. Dermoid tumors involving the central nervous system typically present as a midline intracranial mass. Skull, skull base and orbit represent the most common extracranial location in the head and neck region (Cummings et al. 2004). Complete surgical excision is the standard treatment. Resectability, however, depends upon the location of the tumor, e.g. encroachment upon vasculature and nerves.

13.5.5

Malignant Central Skull Base Lesions

Invasive and malignant pituitary gland tumors are rare and represent a continuum of disease, as pituitary carcinomas usually occur in the setting of pre-existing invasive pituitary gland tumors (Ragel and Couldwell 2004).Both show locally aggressive growth pattern with tendency for early skull base invasion in contrast to the slowly growing benign pituitary gland tumors that like to decompress into the suprasellar cistern (Fig. 13.15). Distinct immunohistochemical markers (interleukin 6 and heat shock protein 27) that have been found to be elevated in invasive types but not in the benign variants of pituitary gland tumors are felt to be responsible for this biological aggressiveness of the invasive tumor variants (Gandour-Edwards et al. 1995). Often these tumors are large at presentation or fast growing on serial follow-up. Otherwise, they show similar imaging

characteristics to their benign variants. Their large size and local aggressive growth pattern sometimes make them difficult to distinguish from other malignant central skull base lesions (Figs. 13.15 and 13.16). The majority of invasive variants of pituitary gland tumors are endocrinologically active with 42% secreting adrenocorticotrophic hormone (ACTH) and 33% prolactin. Presence of metastatic disease distinguishes the invasive pituitary gland tumor from the carcinoma form (Ragel and Couldwell 2004). Metastatic disease primarily occurs to the central nervous system (cerebrum, cerebellum, spinal cord, leptomeninges and subarachnoid space) and search for it needs to be conducted in suspected invasive pituitary gland tumor as metastatic lesions might be already present on the initial imaging study. Systemic metastases most commonly involve the lymph nodes, bone, liver and ovaries. Overall, pituitary carcinomas have a very poor prognosis with a reported 1-year survival rate of 34%. For the patient it is critical that the radiologist recognizes the invasive nature of a pituitary gland tumor, as an aggressive treatment plan is critical prior to development of metastatic disease.

Fig. 13.15. Contrast enhanced, sagittal multiplanar CT reformation displayed in soft tissue window illustrates a large central skull base mass with gross invasion of the sphenoid sinus (s). In addition, the clivus (c) is almost completely involved by this mass that also bulges posteriorly into the prepontine cistern (white arrowheads) where it flattens the pons (p). The sella (black arrowhead) is also involved without significant suprasellar component. This represented an invasive pituitary gland adenoma. Please notice the discrepancy between the suprasellar and the intraclival component that is more characteristic for aggressive pituitary gland tumor variants

Neuroblastoma is an embryonal malignancy arising from neuroblasts of the sympathetic nervous system (Brodeur and Castleberry 2001). They can occur as primary or secondary neuroblastomas of the skull base. The primary neuroblastomas arise from the olfactory bulb and are therefore discussed in Chap. 11. Secondary neuroblastomas represent metastatic disease to the skull base with a tendency for orbital wall involvement including the portions formed by the greater sphenoid wings (Fig. 13.17). Little has been reported in regard to imaging findings of metastatic neuroblastoma to the skull base; however, since neuroblastoma is a small cell tumor with high cellularity and little extracellular space it is not surprising that it shows restricted diffusion on DWI (Fig. 13.17c). Patients with orbital involvement may present with proptosis, periorbital ecchymosis, diplopia or vision loss. Otherwise, the symptoms are related to the primary lesions, “blueberry muffin” resembling skin disease or a paraneoplastic systemic syndrome with myoclonic jerking and random eye movements (=opsoclonus) (Brodeur and Castleberry 2001). The treatment and prognosis of neuroblastoma depends upon tumor stage, age of the patient, histological and molecular features.

In the central skull base region, schwannomas may arise from cranial nerve III through VI. Benign trigeminal nerve (cranial nerve V) schwannomas are the most common cranial nerve tumors with a reported incidence of up to 0.4% of all intracranial

tumors. Malignant schwannomas are even more rare. All but trigeminal nerve malignant schwannomas have been linked to neurofibromatosis type 1 (Stone et al. 2001). Malignant schwannomas of the trigeminal nerve are rather sporadic in occurrence with no known risk factors. The clinical presentation of the benign and malignant variants of central skull base schwannomas is similar and related to dysfunction of the cranial nerve primarily involved and subsequent mass effect related dysfunction of the remaining cranial nerves within the cavernous sinuses. There are no characteristic distinguishing imaging features between benign and malignant types of schwannomas. The more aggressive nature of the schwannoma may be suggested when rapid tumor growth or extensive nerve involvement is seen. In addition, presence of early erosions of the skull base foramina that are in disproportion to the size of the schwannoma might provide important clues (Stone et al. 2001). Due to the rarity of malignant schwannomas little is known in regard to most optimal treatment. Complete surgical resection is certainly the main goal but usually not feasible. In addition, it has been reported that malignant schwannomas have 50% local recurrence rate despite negative surgical margins and adjuvant radiotherapy. Therefore, the overall prognosis is poor with a reported 5-year survival between 38% and 65% (Stone et al. 2001).

Primary extradural meningioma of the skull base is rare with its malignant variant being extraordinarily

uncommon (Van Tassel et al. 1991). Both entities will be discussed in this section together as their imaging findings overlap and the benign form may mimic malignant central skull base lesions. Primary extradural meningiomas are thought to arise from ectopic meningocytes or arachnoid cap cells that are trapped in a fracture line or suture during trauma or molding of the head at birth, respectively (Turner and Laird 1966; Azar-Kian et al. 1974). Most commonly extradural meningiomas are therefore intra-osseous in nature and often involve the greater sphenoid wing which is in part related to the high number of sutures in this location (Fig. 13.18). In contrast to the intradural variant, there is no gender preference and the extradural type shows a bimodal peak with manifestation at older age as well as in the second decade of life (Lang et al. 2000). More than half of these lesions are hyperostotic on plain films and CT studies potentially mimicking prostatic cancer metastasis or fibrous dysplasia (Fig. 13.18a). Only approximately 35% present as osteolytic and 6% as mixed lesions (Crawford et al.1996; Tokgoz et al.2005).Spiculated appearing peri-

osteal reaction might be present (Fig. 13.18a). Often a soft tissue component of variable size is seen which might be difficult to distinguish from adjacent musculature and/or dura on CT images (Fig. 13.18b,c). MR is superior in this regard due to its increased soft tissue detail. As on CT, extradural meningiomas may show variable internal signal characteristics and different degrees of contrast enhancement primarily reflecting the degree of hyperostosis. Interestingly, it has been shown that the extradural type of meningiomas has a higher incidence for malignant degeneration than the intradural variant with a reported risk of 11% and 2%, respectively (Tokgoz et al. 2005). It has been described that meningiomas presenting with osteolysis and an extracranial soft-tissue component are more likely aggressive and, therefore, more often malignant in nature (Fig. 13.18). In addition, it has been shown that malignant meningiomas have the tendency to show restricted diffusion on DWI (Filippi et al. 2001). Complete surgical resection is the treatment of first choice which is not always possible in cases of skull base meningiomas.

13.5.6

Malignant Lesion at the Junction of Central to Posterior Skull Base

Chordomas represent rare, locally invasive and slow growing tumors. Histologically, they resemble benign tumors; however, the majority of authors consider these low grade malignancies due to their aggressive local growth pattern and their low risk for metastatic disease (Erdem et al. 2003). They originate from embryonic remnants of primitive notochord at the spheno-occipital synchondrosis. The skull base is the second most common location. The majority of chondromas occur at the distal end of the notochord in the sacral region. Different histological subtypes have been reported such as chondroid and myxoid variants. Chordomas may present at any age but show a predilection for the 4th decade and for males. The onset of symptoms is insidious, reflecting the slow growth pattern of these tumors. However, at some point these

patients become symptomatic and most commonly present with headaches and diplopia secondary to cranial nerve VI palsy. This is not unexpected as the cranial nerve VI courses through the medial portion of the petrous apex within the Dorello’s canal, in close proximity to the spheno-occipital fissure. The vast majority of chordomas are midline in position with relatively symmetric bilateral tumor growth. Only less than 15% of chordomas have been reported to originate from the petrous apex simulating a chondrosarcoma. On CT, chordomas classically present as large, relatively well-circumscribed, lytic bone lesions arising from the clivus (Fig. 13.19a). Often intratumoral calcifications are seen representing small bony sequestra left behind from bone destruction or true calcifications in case of the chondroid variant. The associated soft tissue mass is usually slightly hyperdense in appearance demonstrating moderate to marked enhancement following intravenous contrast administration (Fig. 13.19b). Although it is easy to de-