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Extra-Axial Neoplasms, Cysts and Tumor-Like Lesions 1.2 Anne G

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Extra-Axial Neoplasms, Cysts and Tumor-Like Lesions 1.2 Anne G Chapter Extra-Axial Neoplasms, Cysts and Tumor-Like Lesions 1.2 Anne G. Osborn Contents Supratentorial Extra-axial Tumors Introduction . 27 in Adults Supratentorial Extra-axial Tumors in Adults . 27 Sella and Parasellar Region . 28 Adult supratentorial extra-axial neoplasms occur in sev- Intrasellar Masses . 28 eral general locations (Fig. 1): (1) The sella and parasel- Suprasellar Masses . 28 lar region; (2) skull and meninges; (3) CSF spaces (sub- Parasellar Masses . 29 arachnoid cisterns and ventricles); and (4) the pineal re- Skull and Meninges . 29 gion. In this chapter we focus on lesions that involve the Meningioma . 29 Metastases . 29 sellar region and the skull and its linings. Supratentorial Extra-axial Tumors in Children . 29 Sella and Parasellar Region . 29 Intrasellar Masses . 29 Suprasellar Masses . 30 Skull and Meninges . 30 Infratentorial Extra-axial Tumors in Adults . 30 Cerebellopontine Angle . 31 Temporal Bone Tumors . 31 Cisternal Neoplasms . 31 Jugular Foramen . 32 Benign Neoplasms . 32 Malignant Neoplasms . 32 Clivus and Foramen Magnum . 32 Clivus Masses . 32 Foramen Magnum Masses . 32 Infratentorial Extra-axial Masses in Children . 32 References . 33 Introduction Extra-axial neoplasms, cysts and tumor-like lesions ac- count for approximately one-third of all intracranial primary neoplasms in adults and about one-quarter of brain tumors in children. The differential diagnosis of an extra-axial mass varies significantly with both pa- tient age and geographic location [1]. In this article we consider the pathology and imaging appearance of intracranial extra-axial masses, subdividing them into supra- and infratentorial lesions by age group. Their more specific differential diagnosis by anatomic region Fig. 1. Sagittal anatomic diagram depicts common locations of su- is illustrated graphically. Special attention is given to pratentorial extra-axial tumors in adults, showing the most impor- sellar and parasellar lesions. tant lesions found in each anatomic area 28 Anne G. Osborn Sella and Parasellar Region Table 1. Sellar/parasellar masses in adults Intrasellar A variety of neoplasms and tumorlike lesions affect the Most common sella and parasellar region. The differential diagnosis of Microadenoma a mass in this anatomically complex area is extensive, Hyperplasia (physiologic, end-organ failure) with more than thirty different pathologic entities re- Less common ported in this area. However, just five lesions (“The Big Rathke cleft cyst Other nonneoplastic cysts Five”) account for the vast majority of masses that occur Metastasis to pituitary gland in and around the sella (Table 1). Rare Three neoplasms account for the vast majority of sel- Craniopharyngioma lar and parasellar tumors in adults: (1) pituitary adeno- Suprasellar ma; (2) meningioma; and (3) schwannoma (in the cav- Most common ernous sinus). Less common lesions in adults include Pituitary macroadenoma craniopharyngioma and metastasis.A spectrum of non- Meningioma neoplastic cysts also can be found in this location [2]. Aneurysm Dividing lesions into intra-, supra-, and parasellar Less common Astrocytoma masses is helpful in establishing a more limited diag- Rathke cleft cyst nosis. Other nonneoplastic cysts (parasitic, arachnoid, epidermoid, dermoid) Metastasis (infundibular stalk, hypothalamus) Lymphoma Intrasellar Masses Hypophysitis Sarcoid Lipoma Pituitary microadenomas (by definition, these are Rare 10 mm or less in diameter) and nonneoplastic cysts are Craniopharyngioma the most common intrasellar masses. Other lesions Germinoma (e.g., craniopharyngioma, meningioma, and metastasis) Parasellar (cavernous sinus) are rare. Dynamic contrast-enhanced MR scans are the Most common best imaging technique for identifying intrasellar Meningioma masses. Most microadenomas enhance more slowly Schwannoma than the surrounding normal pituitary gland. They are Metastasis seen as an area of relative hypointensity compared to Less common Lymphoma the intensely enhancing pituitary gland and cavernous Aneurysm sinus. Rare Chordoma Osteocartilaginous tumor Suprasellar Masses Infrasellar (basisphenoid) Most common Overall, pituitary adenoma is the most common sellar Metastasis (hematogenous) region mass, accounting for at least half of all tumors in Common this location. As they enlarge, small pituitary adenomas Metastasis (nasopharyngeal carcinoma) Inflammatory disease (osteomyelitis) often grow upwards through the diaphragma sellae and Less common acquire a figure of eight appearance. On MR scans, these Plasmacytoma pituitary macroadenomas demonstrate variable signal Lymphoma intensity and strong but heterogenous enhancement fol- Invasive adenoma lowing contrast administration. Hemorrhage and cyst Rare Nonfungal granuloma formation are not uncommon. Mucocele At surgery, microscopic dural invasion is identified with the vast majority of macroadenomas. However, identifying this finding preoperatively is difficult on im- aging studies alone. Occasionally macroadenomas at- Meningioma is the second most common suprasellar tain striking size, extending into the anterior and mid- tumor in adults (see below). It may arise from the dia- dle cranial fossae. Some adenomas exhibit frank inva- phragma sellae, tuberculum, dorsum, or cavernous si- sion of the skull base. Despite their aggressive appear- nus and secondarily involve the sella itself. ance, nearly all of these “invasive adenomas” are histo- Craniopharyngiomas are most commonly seen in logically benign; true pituitary carcinoma is exception- children. However, a second peak occurs in adults ally rare [3]. between the fourth and sixth decades. Metastases in the Chapter 1.2 Extra-Axial Neoplasms, Cysts and Tumor-Like Lesions 29 sellar/juxtasellar region most commonly involve the displacing the cortex and creating a surrounding cleft of skull base and cavernous sinus (see below). Hematoge- cerebrospinal fluid and vessels that is easily identified nous metastases to the pituitary gland may cause an on MR imaging. intrasellar mass; metastases that involve the infundibu- lar stalk or hypothalamus are an uncommon but impor- tant cause of a suprasellar mass. Metastases Lymphoma (almost always non-Hodgkin type) is an- other tumor that has a predilection for the sellar region. Metastases account for between one-quarter and one- Any site (pituitary gland, infundibular stalk, hypothala- third of all brain tumors in adults. Several varieties are mus, cavernous sinus) may be involved. recognized: (1) direct geographic extension of local tu- A variety of nonneoplastic cysts and tumor-like le- mors; (2) hematogenous metastasis; and (3) CSF dis- sions involve the suprasellar region. These include Rath- semination from extra- or intracranial neoplasms. ke cleft cyst, dermoid cyst, epidermoid inclusion cyst and Common regional malignant tumors that may arachnoid cyst. spread directly to the adjacent skull and dura include squamous cell carcinoma (of the nasopharynx and par- anasal sinuses) and adenoid cystic carcinomas (of the Parasellar Masses salivary or mucous glands). Scalp carcinomas (e.g., basal cell carcinoma) occasionally extend through the under- Schwannomas are comparatively rare in intracranial lo- lying calvarium and may even involve the dura. cations other than the cerebellopontine angle cistern Hematogenous metastases from extracranial pri- (see below). The most common supratentorial site is the mary tumors such as carcinoma of the lung or breast cavernous sinus; the trigeminal (cranial nerve V) nerve most commonly involve the brain parenchyma. The cal- is most commonly involved. Meningiomas and metasta- varial vault, central skull base, and dura are also fre- ses often also involve the cavernous sinus. quently affected. Skull and Meninges Supratentorial Extra-axial Tumors in Children Meningioma Extra-axial tumors in children are less common than their intra-axial counterparts. The most common loca- Meningioma is the second most common primary brain tion of a supratentorial extra-axial mass in a child is the tumor in adults, representing between 15% and 20% of sella, followed by the pineal region and ventricles (Fig. these neoplasms. Most typical meningiomas are slowly 2) [5]. The skull and meninges are a less common but growing neoplasms that are histologically benign al- nevertheless important site. though their location at the skull base may make com- plete resection difficult. Three microscopic subtypes of typical meningioma Sella and Parasellar Region are recognized: (1) A meningothelial (or syncytial) type; (2) a fibrous (fibroblastic) type; and (3) a transitional Pituitary adenoma, the most common sellar lesion in form. Atypical and anaplastic (malignant) meningiomas adults, is very rare in children. Only two sellar lesions are biologically more aggressive tumors that account for commonly occur in the pediatric age group; one (crani- 10%-15% of cases. The rare tumor formerly known as opharyngioma) is truly an extra-axial mass whereas the “angioblastic meningioma” is now designated as he- other (an exophytic astrocytoma of the optic chiasm or mangiopericytoma of the meninges [4]. hypothalamus) is considered an intra-axial neoplasm. More than three-quarters of meningiomas are supra- As with adults, dividing lesions into intra-, supra-, tentorial; the most common locations are the convexity and parasellar locations is helpful in establishing an ap- and falx cerebri. Other favored sites include the sphe- propriate differential diagnosis (Table 2). noid ridges, olfactory grooves, parasellar region (tuber-
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