SELLAR AND SUPRASELLAR MIMICSA CASE REVIEW SERIES

ANGELA SNEIDER, D.O., NATALYA NAGORNAYA, M.D. CHARIF SIDANI M.D., RITA BHATIA, M.D.

Presentation #eEdE-140-7366

Disclosures

The authors have no disclosures

Purpose

Mimics of typical sellar and suprasellar lesions are relatively rare and can present a diagnostic dilemma with vital therapeutic implications.

The authors report a pictorial review of various common and uncommon mimics of sellar and suprasellar lesions.

Also included are atypical presentations of common lesions.

Approach/Methods

Retrospective case review of patients presenting to the Department of Radiology at Jackson Memorial Hospital and the University of Miami MRI Center.

Included are atypical lesions of the sellar and suprasellar spaces, as well as more common lesions found at these locations with atypical imaging characteristics.

Findings

We present multiple cases in which uncommon sellar or suprasellar lesions have the appearance of other more often encountered masses of this location.

We also present atypical imaging characteristics of more common lesions.

Case #1

Optic Chiasm Cavernoma

Clinical May be Asymptomatic (>30% become symptomatic, usually in 20s or 30s)

Acute: Visual disturbance, subarachnoid hemorrhage, seizures Chronic: Progressive visual loss, headache, endocrine dysfunction

Behave as all cavernomas Hemorrhage rate of all cavernomas: 0.7-1.7%/lesion/year 20% familial, autosomal dominant

Imaging CT: Well-delineated hyperdense mass +/- calcification, negative in up to 50% MRI: Popcorn appearance on T1 and T2 common, hypointense hemosiderin ring on

T2, blooming on GRE, minimal/no enhancement, look for associated developmental venous anomaly

DDx: AVM, aneurysm, optic glioma, craniopharyngioma, pituitary apoplexy Tx: Somewhat controversial; 50% stable vision and 50% progressive visual

loss With resection (partial or complete): 87% improved vision Stereotactic radiation therapy proposed but not well documented; anterior optic

pathway sensitive to radiation“Cavernous Malformations,” American Association of Neurological Surgeons, 2008 Jul. http://www.aans.org/Patient%20Information/Conditions

%20and%20Treatments/Cavernous%20Malformations.aspx Accessed September 22, 2014

Crocker, M, DeSouza R, King A, at el. Cavernous Hemangioma of the Optic Chiasm: A Surgical Review, Skull Base 2008:18(3):201-12.

Case #2

Case #2 (continued)

Early Right ICA

Late Right ICA

Arteriovenous Malformation

Clinical About half present with headache secondary to hemorrhage More than half have an associated aneurysm ~1/4 have associated seizures Patients may have progressive neurologic dysfunction secondary to related hypoperfusion

or microhemorrhage Imaging

“Bag of worms,” tangle of vessels MRI: “Honeycomb” of flow voids, may have blooming on GRE if hemorrhage, nidus

enhances, enlarged feeding artery/-ies and early draining vein(s) DDx: Other vascular malformation such as dural AV fistula or cavernoma, vascular

neoplasm/metastasis, meningioma Tx:

Embolization Stereotactic radiation therapy Surgical resection

Spetzler-Martin score utilized (surgery typically avoided with a score of 4 or 5) Based on size of nidus, venous drainage and involvement of eloquent cortex

Geibprasert S, Pongpech S, Jiarakongmun P, et al. Radiologic assessment of brain arteriovenous malformations: what clinicians need to know. RadioGraphics 2010:30(2):483-501

Santos M, Demartini Z, Matos L, et al. Angioarchitecture and clinical presentation of brain arteriovenous malformations. Arq Neuropsiquiatr 2009:67(2-A):316-21

Case #3

Case #3 (continued)

Bilateral Petrous Apex ICA Aneurysms, Partially Thrombosed

Clinical May present with sensorineural hearing loss (most common), cranial neuropathy,

pulsatile tinnatus, epistaxis, stroke Underlying causes (i.e. connective tissue disorder) are atypical Possible causes of acquired aneurysm: radiation therapy to the skull base, prior

trauma, skull base infection Important to evaluate for additional intracranial aneurysms, especially with those

suspected to be congenital Imaging

CT: Osseous erosion, curvilinear calcification, enhancement MRI: T1 and T2 heterogeneous, may see a swirl pattern on T2, clot may bloom on

GRE, enhancement CTA preferred diagnostically; MRA can demonstrate hyperintense T1 signal with

cholesterol granuloma that appears as flow-related signal DDx: Cholesterol granuloma, fibrous dysplasia, encephalocele, petrous

apicitis Tx: Balloon trapping, stent placement, sacrifice of ICA with ECA to MCA

bypassChapman P, Shah R, Curé J, et al. Petrous apex lesions: pictorial review. American Journal of Roentgenology 2011:196:WS26-37

Razek A, Huang B. Lesions of the petrous apex: classification and findings at CT and MR imaging. Radiographics 2012:32(1):151-73

Case #4a

Case #4b

Macroadenoma (mimicking tuberculum sella meningioma (4a) & craniopharyngioma (4b)) Clinical

Typically present with endocrinopathy or visual disturbance Most common suprasellar mass in adults “snowman” appearance End-organ failure in adolescent males can lead to pituitary hyperplasia with

an adenoma-like mass Imaging

No separate pituitary gland identified MRI: T1 and T2 isointense, heterogeneously enhancing By definition, >10 mm; if >4 cm, giant macroadenoma May appear invasive, however pituitary carcinoma is extremely rare

DDx: Pituitary hyperplasia, metastasis, meningioma, craniopharyngioma, aneurysm

Tx: Surgical resection, typically transsphenoidal Transsphenoidal resection likely to fail when lesions are solid and enhancing

with restricted diffusion ~35% recurrence rate over 20 years

Boxerman J, Rogg J, Donahue J, et al. Preoperative MRI evaluation of pituitary macroadenoma: imaging features predictive of successful transsphenoidal surgery. Amer J Roentgen 2010:195(3):720-8

Ehirim P, Kerr D, Cohen A. Primary hypothyroidism mimicking a pituitary macroadenoma. Pediatric Neurosurgery 1998:28(4):195-7

Case #5

Macroadenoma with Apoplexy

Clinical Typically present with sudden onset headache, severe nausea, visual disturbance,

altered mental status Most patients have an underlying macroadenoma

~1% of macroadenomas will hemorrhage Usually middle-aged, more common in males; peripartum females also at risk

Imaging CT hyperdensity, MRI blooming within the sella; rim enhancement suggests

underlying hemorrhage; may have associated subarachnoid hemorrhage MRI characteristics depend on age of hemorrhage; acute hemorrhage is T1

isointense and T2 hypointense; may see diffusion restriction early Commonly see associated dural thickening/enhancement and/or mucosal

thickening of the sphenoid sinuses DDx: Craniopharyngioma, Rathke cleft cyst, nonhemorrhagic

macroadenoma, aneurysm, abscess Tx: Surgical decompression/resection of underlying macroadenoma,

supportive therapyBoellis A, Napoli A, Romano A, et al. Pituitary apoplexy: an update on clinical and imaging features. Insights into Imaging 2014:5(6):753-62

Semple P, Jane J, Lopes M, et al. Pituitary apoplexy: correlation between magnetic resonance imaging and histopathological results. Journal of Neurosurgery 2008:108(5):909-15

Case #6 (two different patients)

Meningioma

Clinical 1/3 of all incidental intracranial neoplasms ~3-10% of meningiomas arise from the tuberculum sellae More common in women, typically between 30-60 years old Sx: Headache, visual disturbance, anosmia Up to 2/3 involve at least one of the optic canals

Imaging CT: 3/4 hyperdense, 1/4 at least partially calcified, underlying bone hyperostotic MRI: Hypo- to isointense on T1 and T2, look for dural tail, early and prolonged

enhancement (mother-in-law sign) DDx: Macroadenoma, metastasis, sarcoid, nerve sheath tumor Tx: Watchful waiting if asymptomatic, surgical resection if symptomatic

Immediate postoperative improvement in vision in ~2/3 presenting with visual disturbance

Resection complicated by proximity to adjacent arteries, optic nerves/chiasm and hypothalamus

Sklar E, Schatz N, Glaser J, et al. Optic tract edema in a meningioma of the tuberculum sellae. AJNR Am J Neuroradiol 2000:21:1661-63

Nakamura M, Roser, F, Struck M, et al. Tuberculum sellae meningiomas: clinical outcome considering different surgical approaches. Neurosurgery 2006:59(5):1019-28

Mahmoud M, Nader R, Al-Mefty O. Optic canal involvement in tuberculum sellae meningiomas: influence on approach, recurrence, and visual recovery. Neurosurgery 2010:67(3 Suppl Operative):108-19

Case #7

Chondrosarcoma

Clinical Only ~7% of all chondrosarcomas are craniocervical Sx: Headache, cranial nerve palsies (CN6 most common) Symptoms usually present long before diagnosis

Imaging Typically centered at the petro-ocipital fissure Chondroid matrix calcification on CT, osseous destruction

in >50% MRI: T1 iso- to hypointense, T2 hyperintense, heterogeneously enhancing (may

have whorls of enhancement) ADC values may show benefit in distinguishing from chordoma

Chondrosarcoma typically has a much higher ADC value (~2000 mm2/s compared to ~1500 mm2/s for classic chordoma and ~900 mm2/s for poorly differentiated chordoma)

DDx: Chordoma, chondromyxoid fibroma, metastasis, plasmacytoma, meningioma

Tx: Typically combined radical resection and adjuvant radiotherapyBloch O, Parsa A. Skull base chondrosarcoma: evidence-based treatment paradigms. Neurosurgery Clinics of North America 2013:24(1):89-96

Günes M, Günaldi O, Tuğcu B, et al. Intracranial chondrosarcoma: a case report and review of the literature. Minim Invasive Neurosurg 2009:52(5-6):238-41

Yeom K, Lober R, Mobley B, et al. Diffusion-weighted MRI: distinction of skull base chordoma from chondrosarcoma. AJNR Am J Neuroradiol 2012 Nov 1. [Epub ahead of print]

Case #8

Case #8 (continued)

Chondromyxoid Fibroma

Clinical 2/3 occur in long bones, 1/4 in flat bones, craniofacial extremely rare Slow-growing and locally destructive, no tendency to metastasize About half present in the 2nd decade, males more common than

females Most present with very slowly worsening bone pain

Imaging CT: Geographic lesion with a sclerotic margin, thinning of cortex,

usually expansile MRI: T1 isointense, T2 hyperintense with lobulations, periphery T1

and T2 hypointense, minimal diffuse central contrast enhancement DDx: Chondrosarcoma, aneurysmal bone cyst, fibrous dysplasia Tx: Surgical excision with curettage and bone grafting – recur

in~20%, may radiate if recurrentCrocker M, Corns R, Bodi I, et al. Chondromyxoid fibroma of the skull base invading the occipitocervical junction: report

of a unique case and discussion. Skull Base 2010:20(2):101-4

Haygood T, Herndon M, Chitkara P, et al. Chondromyxoid fibroma involving the sphenoid sinus: case report and literature review. Radiology Case Reports 2010:5(2)

Case #9

Primary Pituitary Lymphoma

Clinical Incidence of primary CNS lymphoma has significantly increased over the last 30

years due to increased survival rates of patients with AIDS and increased rate of successful organ transplantation ~2.5% of AIDS patients will develop primary CNS lymphoma

Based on the 28 reported cases of primary pituitary lymphoma as of 2012 Equal incidence in men and women Typically in the 6th decade Presenting symptoms included endocrine dysfunction (50%), headache, visual disturbance,

and other cranial neuropathies Imaging

Typically hyperdense on CT, although may be isodense; may see areas of hemorrhage and/or necrosis

T1 and T2 iso- or hypointense, may show diffusion restriction, may homogeneously enhance except for possible areas of necrosis

DDx: Metastasis, sarcoid, lymphocytic hypophysitis (in our patient); toxoplasmosis, glioblastoma multiforme, metastasis

Tx: Chemotherapy +/- radiation therapy +/- surgical resectionMansour, A, Qandeel M, Abdel-Razeq H, et al. MR imaging features of intracranial primary CNS lymphoma in immune competent patients. Cancer

Imaging 2014:14:22-30

Rainsbury P, Mitchell-Innes A, Clifton N, et al. Primary lymphoma of the pituitary gland: an unusual cause of hemianopia in an immunocompetent patient. JRSM Short Rep 2012:3(8):55-9

Case #10 (two different patients)

a b

Metastatis (lung cancer (10a) & GBM (10b))

Clinical Only 1-2% of intracranial metastases are extraparenchymal or non-meningeal;

typically more infiltrative Breast cancer is most common, followed by lung, kidney and colon; glioblastoma

multiforme and anaplastic astrocytoma are the most common primary sites from the brain

May present with diabetes insipidus, anterior pituitary insufficiency, retroorbital pain

Median survival after diagnosis is 4.1 months Positive prognostic indicators include solitary pituitary metastasis, good performance

status and receipt of local therapy; NOT age, gender or primary cancer type Imaging

Enhancing lesion; typically small, round and infiltrating May mimic many other lesions; no pathognomonic distinguishing feature Must include in patients with a known primary and enhancing pituitary mass

DDx: Macroadenoma, lymphoma, sarcoid, lymphocytic hypophysitis Tx: Radiation therapy and/or chemotherapy and/or surgical resectionAkio M, Meyer F, Laws E. Symptomatic pituitary metastases. Journal of Neurosurgery 1998:89(1):69-73

Lin C, Huang W, Chung F, et al. Prognostic factors in cancer patients with symptomatic pituitary metastasis: a clinical case study. Anticancer Research 2015:35(2):983-7

Hermet M, Trouillier S, André M, et al. Pituitary metastasis presenting as diabetes insipidus: a report of four cases and literature review. Review of Internal Medicine 2009:30(5):425-9

Case #11

Case #11 (continued)

Epidermoid

Clinical May present with headache, cranial neuropathy, visual disturbance, hypopituitarism,

diabetes insipidus Mean age of presentation 40 years, equally common in males and females Usually very slow growing, rarely degenerates into squamous cell carcinoma Rupture may lead to chemical meningitis ~10% are parasellar

Imaging Predominantly nonenhancing – may have minimal peripheral enhancement CT: Lobulated, hypodense mass; ~3% hyperdense due to prior hemorrhage/high

proteinaceous content MRI: Cauliflower-like lobulated mass, slightly hyperintense to CSF on T1 and T2,

diffusion restricting, insinuates cisterns and encases neurovascular structures “White” epidermoids hyperintense to grey matter on T1 due to high triglyceride/fatty acid

content “Black” epidermoids hypointense to CSF on T1 due to solidification of cholesterol and keratin

DDx: Rathke cleft cyst, craniopharyngioma, neurocysticercosis, abscess Tx: Surgical resection with tendency to recurCosta F, Fornari M, Felisati G, et al. Epidermoid cyst of the pituitary stalk: case report and review of the literature. Neurosurgery Quarterly

2013:23(2):108-11

Law E, Lee R, Ng A, et al. Atypical intracranial epidermoid cysts: rare anomalies with unique radiological features. Case Reports in Radiology 2015:2015:4

Case #12

Mucoepidermoid Carcinoma (mimicking craniopharyngioma)

Clinical Typically a salivary gland (parotid most commonly) neoplasm containing mucous-

secreting cells and epidermoid cells Most common primary malignancy of the parotid gland Those with prior radiation therapy are predisposed May present with facial nerve paralysis, facial pain, enlarging facial mass

Imaging Low-grade lesions are well-circumscribed, while high-grade lesions are invasive

and irregular with associated lymphadenopathy Low-grade lesions tend to have cystic areas while high-grade lesions do not Hypointense T2 areas are characteristic Heterogeneous enhancement Must evaluate for perineural spread (CN7)

DDx: Craniopharyngioma, macroadenoma or Rathke cleft cyst (in our patient), benign mixed tumor, Warthin tumor, lymphoma, adenoid cystic carcinoma

Tx: Surgical resection +/- adjuvant radiation, neck dissection if high-gradeTriantafillidou K, Dimitrakopoulos J, Iordanidis F, et al. Mucoepidermoid carcinoma of minor salivery glands: a clinical study of 16 cases and

review of the literature. Oral Diseases 2006:12(4):364-70

Van Furth W, Smyth H, Horvath E, et al. Salivery gland-like tumor of the sella. The Canadian Journal of Neurological Sciences 2007:34(4):478-82

Case #13

Lymphocytic Hypophysitis

Clinical Inflammation of the anterior pituitary gland which is idiopathic Much more common in females, typically in the 4th decade Up to 40% misdiagnosed as adenoma

Imaging Features more indicative of LH: symmetric enlargement of pituitary gland,

thickened pituitary stalk (>2 mm), intact sellar floor Homogeneous enhancement Parasellar T2 dark sign – hypointense T2 signal around the pituitary gland and

cavernous sinus, NOT seen in patients with adenoma and often progresses with chronicity in patients with LH (our patient did not display this sign)

DDx: Adenoma, pituitary hyperplasia, metastasis, sarcoid Tx

Steroids and hormone replacement therapy Secondary panhypopituitarism may lead to death if untreated, however can be self-

limitingGutenberg A, Larsen J, Lupi I, et al. A radiologic score to distinguish autoimmune hypophysitis from nonsecreting pituitary adenoma preoperatively.

AJNR Am J Neuroradiol 2009:30:1766-72

Nakata Y, Sato N, Masumoto T, et al. Parasellar T2 dark sign on MR imaging in patients with lymphocytic hypophysitis. AJNR Am J Neuroradiology 2010:31:1944-50

Quencer R. Lymphocytic adenohypophysitis: autoimmune disorder of the pituitary gland. AJNR Am J Neuroradiol 1980:1:343-5

Case #14

Rosai Dorfman (Sinus Histiocytosis)

Clinical Unknown etiology; may be autoimmune or infectious Typically present in 1st or 2nd decade with painless neck mass(es) Massive bilateral cervical lymphadenopathy Other Sx: anosmia, facial pain, epistaxis, visual disturbance Benign entity, however morbidity increases if superimposed immune disorder

present More common in males; African and West Indian heritage predominance

Imaging May see involvement of dura, orbits, bone, skin and salivary glands; rarely

involves airway, mediastinum Huge, enhancing lymph nodes MRI: T1 and T2 hypointense, enhancing and infiltrating extranodal lesions

DDx: Lymphoma, meningioma, metastasis, histiocytosis Tx: Watchful waiting, steroids, surgical debulking if compression of

adjacent neurovascular structuresLa Barge D, Salzman K, Harnsberger H, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease): imaging

manifestations in the head and neck. American Journal of Roentgenology 2008:191(6):W299-W306

Symss N, Cugati G, Vasudevan M, et al. Intracranial Rosai Dorfman disease: report of three cases and literature review. Asian Journal of Neurosurgery 2010: 5(2):19-30

Case #15

Case #15 (continued)

Prior to Tx

5 days later, after steroids

Paraneoplastic Syndrome

Clinical Less than 1% of cancer patients develop paraneoplastic syndrome; most do not

have imaging findings Most commonly due to small cell lung cancer, followed by breast cancer,

gynecologic tumors and hematologic malignancies May present with seizure, altered mental status, memory loss Immune-mediated response; >50% have autoantibodies and/or specific surface

antigens (as were found in this patient) NMDA receptor Ab, mGluR1 Ab, voltage-gated K+ channel Ab, Hu Ab, Yo Ab, Ri Ab, CV2 Ab

Imaging Limbic encephalitis: T2 hyperintensity throughout the mesial temporal lobes and

limbic system Brainstem encephalitis: T2 hyperintensity throughout the brainstem, as well as the

cerebellar peduncles and basal ganglia Cerebellar degeneration: atrophy

DDx: Metastasis/lymphoma (in our patient), herpes encephalitis, glioma Tx: Treatment of primary tumor, supportive therapy including

immunosuppression and electrolyte/hormonal correctionDalmau J, Rosenfeld M. Paraneoplastic syndromes of the CNS. Lancet Neurol 2008:7(4):327-40

Pelosof L, Gerber D. Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clinic Proceedings 2010:85(9):838-54

Key Points

Cavernoma CT: Well-delineated hyperdense mass +/- calcification; MRI: popcorn

appearance, hypointense hemosiderin ring on T2, blooming on GRE, minimal/no enhancement, look for associated DVA

Arteriovenous Malformation “Bag of worms,” tangle of vessels; MRI: “Honeycomb” of flow voids, enlarged

feeding artery/ies and early draining vein(s) Petrous apex aneurysms

CTA preferred diagnostically; MRA can demonstrate hyperintense T1 signal with cholesterol granuloma that appears as flow-related signal

Macroadenoma No separate pituitary gland identified

Pituitary apoplexy CT hyperdensity, MRI blooming within the sella; rim enhancement suggests

underlying hemorrhage; may have associated subarachnoid hemorrhage Meningioma

CT: most hyperdense, may be partially calcified, underlying bone hyperostotic; MRI: Hypo- to isointense on T1 and T2, look for dural tail, early and prolonged enhancement (mother-in-law sign)

Key Points

Chondrosarcoma Typically centered at the petro-ocipital fissure, chondroid matrix calcification on

CT; MRI may demonstrate whorls of enhancement, ADC values may show benefit in distinguishing from chordoma

Chodromyxoid fibroma CT: Geographic lesion with a sclerotic margin, thinning of cortex, usually

expansile; MRI: T1 isointense, T2 hyperintense with lobulations, minimal enhancement

Pituitary lymphoma Typically hyperdense on CT; MRI: T1 and T2 iso- or hypointense, may restrict,

homogeneous enhancement except for possible areas for necrosis Metastasis

Must include in patients with a known primary and enhancing pituitary mass Epidermoid

Nonenhancing, cauliflower-like lobulated mass, slightly hyperintense to CSF on T1 and T2, diffusion restricting, insinuates cisterns and encases neurovascular structures “White” epidermoids hyperintense to grey matter on T1 due to high triglyceride/fatty acid

content “Black” epidermoids hypointense to CSF on T1 due to solidification of cholesterol and keratin

Key Points

Mucoepidermoid carcinoma Hypointense T2 areas are characteristic, must evaluate for

perineural spread (CN7) Lymphocytic Hypophysitis

Symmetric enlargement of pituitary gland, thickened pituitary stalk (>2 mm); homogeneous enhancement; parasellar T2 dark sign – low T2 signal around the pituitary gland/cavernous sinus

Rosai Dorfman Huge, enhancing lymph nodes; MRI: T1 and T2 hypointense,

enhancing and infiltrating extranodal lesions Paraneoplastic syndrome

Limbic/brainstem encephalitis: T2 hyperintensity; cerebellar degeneration with atrophy; serum markers necessary in atypical cases

Summary

Many less commonly encountered pathologies of the sellar and suprasellar spaces may mimic those lesions which present more commonly in practice.

In contrast, typical lesions of the sellar and suprasellar spaces may have atypical imaging characteristics, altering our differentials.

Here, we review a set of cases which challenge both our knowledge of sellar and suprasellar lesions, as well as our eyes.

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